A twelve-month-old infant is followed with a continuous murmur, and echocardiographic confirmation of a Patent Ductus Arterosus (PDA). There is left atrial and left ventricular enlargement, normal right-sided pressures, runoff from the abdominal aorta, and

transcatheter closure is recommended as spontaneous closure is unlikely beyond this age. The patient weighs 8.5kg. Access to the femoral artery is made with a 4F sheath, and to the femoral vein with a 5F sheath. Cardiac catheterization confirms normal pulmonary artery pressure with a large left-to-right shunt. Aortography shows a conical ductus (Figure 1)

Patent Ductus Arteriosus and the Nit-Occlud PDA Device

January 2016; Volume 14; Issue 1North American Edition

IN THIS ISSUE

Patent Ductus Arteriosus and the Nit-Occlud PDA Device By Gurumurthy Hiremath, MD; John L. Bass, MD~Page 1

Highlights from the Second Fetal Cardiac Symposium at Rush University in Chicago - September 10th-12th, 2015By Karim Diab, MD ~Page 9

Atrioventricular Septal Defect: Case Report By Felipe Guimarães Machado, MD; Éric Guimarães Machado, MD; Maria Clara Menezes de Jesus Lisboa, MD; Léo Guimarães Soares, MD; Paulo Sérgio Lopes Soares, DDS, PhD; Gabriel Porto Soares, MD, PhD~Page 12

The 48th Annual Southeast Pediatric Cardiology Society Conference Meeting ReviewBy F. Bennett Pearce, MD~Page 16

Medical News, Products & Information ~Page 18

CONGENITAL CARDIOLOGY TODAYEditorial and Subscription Offices16 Cove Rd, Ste. 200Westerly, RI 02891 USAwww.CongenitalCardiologyToday.com

Official publication of the CHIP Network - www.chipnetwork.org

© 2016 by Congenital Cardiology Today Published monthly. All rights reserved.

Recruitment ads pages: 3, 4, 7, 13, 17,18, 19

By Gurumurthy Hiremath, MD; John L. Bass, MD

CONGENITAL CARDIOLOGY TODAYTimely News and Information for BC/BE Congenital/Structural Cardiologists and Surgeons

Upcoming Medical Meetings

Cardiology 2016Feb. 24-28, 2016; Orlando, FL USA

www.chop.edu/events/cardiology-2016

PICS-CSI Asia 2016 - Catheter Interventions in Congenital,

Structural and Valvular Heart DiseaseMar. 3-5, 2016; Dubai UAE

http://www.csi-congress.org/pics-csi-asia.php?go=0

Figure 1. Lateral view of aortogram in a patient with a Type A Patent Ductus Arteriosus (A). The narrowest portion at the pulmonary end measures 1.8mm, and the ampulla at the descending aorta just under 9mm, nearly the same diameter as the descending aorta at the level of the ductus.

CONGENITAL CARDIOLOGY TODAY

CALL FOR CASES AND OTHER ORIGINAL ARTICLES

Do you have interesting research results, observations, human interest stories, reports of meetings, etc. to share?

Submit your manuscript to: RichardK@CCT.bz

Nit-Occlud Coil System for PDA Closure

a safer, easier way

to closeThe Nit-Occlud® coil system for PDA closure is designed to match individual morphologies and sizes

The delivery system facilitates optimal device positioning

The Nit-Occlud® PDA coil is repositionable and retrievable prior to release

Tight and compact windings ensure efficient occlusion

The Nit-Occlud® PDA coil was determined to be MRI conditional

Radiopaque

®

For more information or to place an order, contact your B. Braun Interventional Systems Inc. representative or call 1-877-VENA-CAV (836-2228)

Distributed by:B. Braun Interventional Systems Inc.824 Twelfth AvenueBethlehem, PA 18018 USATel: 1-877-VENA CAV (836-2228) (USA)Fax: 1-610-849-1334www.bisusa.org

Manufacturer:pfm medical, ag Wankelstraße 6050996 Köln, GermanyT +49 (0)2236 9641-10F +49 (0)2236 9641-20

Indication: The Nit-Occlud® PDA coil is a permanently implanted prosthesis indicated for percutaneous, transcatheter closure of small to moderate size patent ductus arteriosus with a minimum angiographic diameter less than 4mm. Nit-Occlud Brief Statement: Do not implant the Nit-Occlud PDA into patients who have endocarditis, endarteritis, active infection, pulmonary hypertension (calculated PVR greater than 5 Wood Units), thrombus in a blood vessel through which access to the PDA must be obtained, thrombus in the vicinity of the implantation site at the time of the implanttion or patients with a body weight < 11 lbs (5 kg). An angiogram must be performed prior to implantation for measuring length and diameter of the PDA. Only the pfm medical implantation delivery catheter should be used to implant the device. Administration of 50 units of heparin per kg body weight should be injected after femoral sheaths are placed. Antibiotics should be given before (1 dose) and after implantation (2 doses) in order to prevent infection during the implant procedure. Do not implant the Nit-Occlud PDA in an MR environment. Do not pull the Nit-Occlud coil through heart valves or ventricular chambers. Contrast media should not be injected through the implantation catheter. The catheter must not be connected to high pressure injectors. Patients may have an allergic response to this device due to small amounts of nickel that has been shown to be released from the device in very small amounts. If the patient experiences allergic symptoms, such as difficulty in breathing or swelling of the face or throat, he/she should be instructed to seek medical assistance immediately. Antibiotic prophylaxis should be performed to prevent infective endocarditis during first 6 months after coil implantation. Potential Adverse Events: Air embolism, Allergic reaction to drug/contrast, Apnea, Arrhythmia requiring medical treatment or pacing, Arteriovenous fistula, Bacterial endocarditis, Blood loss requiring transfusion, Chest pain, Damage to the tricuspid or pulmonary valves, Death, Embolization of the occluder, requiring percutaneous or surgical intervention, Endarteritis, False aneurysm of the femoral artery, Fever,Headache/migraine, Heart failure, Hemolysis after implantation of the occluder, Hypertension, Hypotension or shock, Infection, Myocardial infarction, Occluder fracture or damage, Perforation of the heart or blood vessels, Stenosis of the left pulmonary artery or descending thoracic aorta, Stroke/TIA, Thromboembolism (cerebral or pulmonary), Valvular Regurgitation, Vessel damage at the site of groin puncture (loss of pulse, hematoma etc.)

The Nit-0cc|ud® coil system for The Nit-0cclud® PDA coil is The Nit—0cc|ud® PDA coilPDA closure is designed to match repositionable and retrievable was determined to be MRIindividual morphologies and sizes prior to release conditional

The delivery system facilitates Tight and compact windings Radiopaqueoptimal device positioning ensure efficient occlusion

For more information or to place an order, contact your B. Braun lnterventional Systems Inc.representative or call 1-877-VENA-CAV (836-2228)

, II1teI'vei1tiom1I “= Systems nite:-IiIIi1!l~

Distributed by:Manufacturer: B. Braun lnterventional Systems Inc.pfm medical, ag 824 Twelfth AvenueWankelstra e60 Bethlehem, PA 18018 USA50996 Kiiln, Germany Tel: 1-877-VENA CAV (836-2228) (USA)T +49 (0)2236 9641-10 Fax: 1-610-849-1334F +49 (0)2236 9641-20 www.bisusa.org

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lcalculaied PVR greaier man 5 wood uniis). Lhmmhus in a blood vessel inrougli wnicii access to the PDAmlisl he amainedy inrornous in the vicinity oline implanraiion 5118 ac me lime or me iiiiplalllllan or patients vvilii a body weight < 11 lbs (5 kg). An angiograrn rnusi be perlnrllied prior to irnplaniaiion ior measuring lengin and diameler oline PDA. only me pirn medicalirnplaniaiion delivery caineier snculd be used lo implannne device. Administration oi so iiliils oineparin per kg body weight should he inyecled aler lemoral sneaiiis are placed Aniioiciics snould oe given oelore ll dose) and aiier implanraiion (2 doses) in orderio preveni liilecllorl during ine implani procedure Do nolimplaiinhe Nii.oocirrd FDA in an MR environrneni. Do rimpull ilie liii.occlud coil inrcugn lieari valves or velilrlcularchambels Culllld r ' iirjecree ' r r ml to ' injectors i=aiienis may * iiiuxei uiai

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experiences allergic symptoms‘ such as dimcclry in oreailiing or swelling oi ine lace or throat, helshe slroirld be instructed lo seek medical assistance imrnediaiely. Anllhiollc prophylaxis should be rierlorrried to prevent" 4 -4 4g nrsie monins airei

' F -M Eveiils Aireriiirclism, ' Apnea‘ Aniiyilimiarerruin'ng medical ireaimeni or racing Ariencvenoirs nsiula, Bacterial endooardirisr Blood lcss requiring iranslusicn. cliesi pain, Damage lo ilie llioiispid or pulmonary valves, neain, Eiiihollzallarl cl ine cccluder, iequirllig percuraneous or surgical inlerveniion, Endaneriiis. False aneurysm or me ienicral artery. Fever, eadaciielrnigraine, Heart lailliler iiernolysis aiierimplalilallorl oi ine occluden Hyperlerlslorl. Hypnlenslon or snack, llileciioli, Myocardial iniarclion, occluder iraclure or damage‘ Perforation cl me lieari or blood vessels, svencsis ol the leli pulmonary ariery or descending inoracic aorta‘ SlrokeiT|A. Thloliiboeliibolism lcereoral or pirlrnonarylr valvular Regurgilalionr vessel damage ai ilre siie cl groin rrunciure llcss oipulse. herlialoliia eic.l

narrowed at the pulmonary end. A decision is made to implant a Nit-Occlud® PDA device to close the ductus. Measurements show a 1.8mm minimum diameter at the pulmonary artery, and 8.5mm at the aorta (Figure 1). A 9x6 Nit-Occlud® device is chosen, a 0.025in. guidewire is advanced from the pulmonary artery into the descending aorta, and the 5F delivery catheter advanced through the ductus. The 9x6 Nit-Occlud® device is flushed, is advanced through the delivery catheter, the aortic end exposed and pulled into the aortic PDA ampulla, and the pulmonary end exposed. Aortography confirms good pos i t ion , the co i l i s detached, and the aor togram is repeated,confirming repositioning of the device after release and a moderate residual shunt (Figure 2). Catheters are removed with a good pulse distal to the arterial sheath site. Before discharge 4-hours later, the residual PDA shunt is gone.

This relatively straightforward procedure raises a number of questions:1. Why choose a Nit-Occlud® PDA device?2. What is different about this device and its implantation?3. How can you leave this residual shunt behind in the

cardiac catheterization laboratory?4. Are there any disadvantages to using this device?

Why Choose a Nit-Occlud®?

Transcatheter closure of the PDA was reported over 40 years ago.1 Yet today, there are only three transcatheter PDA closure devices with FDA approval, most recently the Nit-Occlud® device. The first two are the Amplatzer PDA Occluders (Types 1 and 2; ADO1 and ADO2). There are minor disadvantages with these two devices. The ADO1 device aortic disc can protrude into the aorta, particularly in small infants, and it requires a relatively large delivery sheath (6F or more).

CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 3

Figure 2. Lateral view of aortogram in the same patient as Figure 1. A 9x6 Nit-Occlud® ductus coil is in the aortic ampulla still attached to the retrieval cable (A). The coil lies slightly angled in the ampulla. In B the coil has been released. The coil has reoriented towards the aorta, but still is not in the true lumen. Approximately ½ turn of the coil is within the pulmonary artery. There is a modest amount of residual flow through the ductus that was completely occluded 4 hours later on echocardiography.

Florida - Pediatric Cardiology - The Department of Pediatrics at the University of Florida College of Medicine-Jacksonville is recruiting a full-time faculty member to the Division of Pediatric Cardiology (# 00030335) as a clinician-educator on the non-tenure, multi-mission academic track. We seek an excellent general cardiologist who will divide duties between attending on the inpatient service at Wolfson Children’s Hospital and participating in our expanding outpatient satellite clinics. Night and weekend call responsibilities will be shared equitably with other division faculty. The successful candidate is expected to provide outstanding clinical care in a patient and family centered environment. The successful candidate must be able to evaluate and manage children with complex congenital heart disease and to interpret transthoracic echocardiograms accurately. Excellent interpersonal and communication skills are essential. Prior experience in telemedicine is desirable.

The Division follows approximately 7,500 children per year. Full participation in all other divisional activities such as the education of residents, fellows and medical students and attendance at divisional conferences are required. The appointment will be at the Assistant/Associate Professor level depending upon experience and qualifications. The congenital heart program at consists of 10 pediatric cardiologists and 2 congenital heart surgeons who provide care to children from northeast Florida, southeast Georgia as well as children in the international community. Jacksonville is a vibrant, young, and growing community. The catchment population for Wolfson Children’s Hospital exceeds 1.5 million.

Applicants must possess a MD/DO degree, be BE/BC in pediatric cardiology, and be eligible for Florida medical licensure. Applications will continue to be considered until the position is filled.

To apply for this position visit https://jobs.ufl.edu/ and search for job number 494973.

Attach curriculum vitae, the names and addresses of three references and a letter of intent addressed to:

Frank J. Genuardi, MD, Search Committee Chairman

University of Florida College of Medicine - Jacksonville653-1 West 8th StreetJacksonville, FL 32209

The University of Florida is an equal opportunity institution dedicated to building a broadly diverse and inclusive faculty and staff.

Please see our website at www.hscj.ufl.edu/pediatrics.

Pediatric Cardiology

CONGENITAL CARDIOLOGY TODAY

CALL FOR CASES AND OTHER ORIGINAL ARTICLESDo you have interesting research results, observations, human interest stories, reports of meetings, etc. to share?

Submit your manuscript to: RichardK@CCT.bz

Department of PediatricsCollege of Medicine — JacksonvilleUF

The ADO2 device was designed to overcome this with discs that rotate to lie flat against the aorta and pulmonary artery, passing through a smaller delivery system, and horizontally symmetrical so that it can be implanted from either the pulmonary artery or aortic side. The animal model for the ADO2 was a tubular PDA that was filled by the connecting waist, yet 65% of PDAs in humans are conical PDAs. The aortic disc of the ADO2 can lie horizontally in the PDA ampulla with residual shunting, or with a shorter PDA, the waist and aortic disc may remain floating in the aorta despite successful occlusion. For decades the primary method of transcatheter occlusion of the PDA was the Gianturco stainless steel coil,2 and this worked well for smaller PDAs. But this is off-label use; the coil is not designed to occlude a PDA. Tubular PDAs can also be closed with implantation of the Amplatzer Vascular Plug 2, usually quite effectively, but still an off-label use of the device. It is into this milieu of off-label use for transcatheter PDA occlusion, occasional obstruction of the aorta, and some hemodynamic instability with a large delivery sheath, that the Nit-Occlud® PDA device was introduced and reached FDA approval in August 2013. The advantages of this device are the smaller delivery catheter (4-5F), less chance of protrusion into the aorta or pulmonary artery especially in conical PDAs, FDA approval (on-label use of a device designed specifically to close the PDA), and a high degree of effectiveness. The cost of the Nit-Occlud® PDA device is competitive with, or cheaper than, the alternative FDA-approved PDA closure devices. PDAs with minimum diameters up to 4mm can be effectively closed with this device.

What Is Different about the Nit-Occlud®?

Unlike other coils placed in the PDA, the Nit-Occlud® device has no fibers attached. The Nitinol surface of the coil leads to rapid thrombosis and occlusion. It is designed to be deployed from the venous side, and the delivery catheter must be placed from the femoral or internal jugular vein. The attachment mechanism uses friction between coils of the proximal end of the device and a mandrel (Figure 3) to maintain the connection, allowing easy retrieval once deployed. The Amplatzer device family and the Flipper coils use a screw attachment. In the unlikely event of embolization of the Nit-Occlud® PDA device, it is easily snared and retrieved through a 6F sheath (through the PDA if embolized to the aorta).

Sizing the device may be a little uncertain for beginning users. Device sizes are given as two numbers, e.g. 6x5. The second number is the diameter of the proximal-pulmonary end of the conformed coil, and the first the diameter of the distal-aortic end of the conformed coil (Figure 4). A sizing table is supplied by the manufacturing company (PFM) (Figure 4) with the distal/aortic end designed to be at least 3-4mm larger than the narrow pulmonary end of the PDA, and no more than 2mm larger than the aortic ampulla. This guide is a starting point. In general we have chosen a proximal/pulmonary diameter 2-4mm larger than the minimum PDA diameter to maximize the occlusion rate, and a distal/aortic diameter that will fit into the PDA ampulla keeping it out of the aortic lumen. In the case presented here, with a nearly 9mm distal aortic diameter and a 1.8mm pulmonary diameter, an 11x6 device could have been chosen. But an 11mm distal coil would have protruded into the aorta, even if it configured when exposed with a descending aortic diameter of 9mm. A 7x6 device would have worked as well, and perhaps even a 6x5. So why not choose a minimum distal coil diameter? A smaller distal diameter may leave the coil “rattling around” in the PDA ampulla, and changing position could influence occlusion.

CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 4

Volunteer / Get Involved www.chimsupport.com

HOW WE OPERATE

The team involved at C.H.I.M.S. is largely a volunteer group of physicians, nurses and technicians who are involved in caring for children with congenital heart disease.

The concept is straightforward. We are asking all interested catheter laboratories to register and donate surplus inventory which we will ship to help support CHD mission trips to developing countries.

Figure 3. A Nit-Occlud® coil is seen with arrows pointing to the terminal portion of the coil that has no core (A) allowing the outer winding to wrap around the inner mandrel of the delivery cable (B). This attachment mechanism relies on the friction between the winding and the mandrel.

Figure 4. Sizing table supplied by PFM Medical for selecting the appropriate sized Nit-Occlud® coil for occlusion of a Patent Ductus Arteriosus. D2 in the left upper panel is the measure of the diameter of the aortic ductal ampulla matches to largest diameter D of the Nit-Occlud ® ductal coil in the upper right. D1 is the narrowest portion of the ductus and matches to the smallest coil diameter of the rewound portion (P). The device size-designation (e.g. 9x6) indicates the larger coil diameter as the first number by the smaller rewound coil diameter as the second number. Recommendations are that the larger coil diameter be no more than 1-2mm larger than the aortic ampulla, and at least 3-4mm larger than the minimum ductal diameter. PDA = Patent Ductus Arteriosus.

D1 D2 Device1mm ≤ 3mm 4x41mm 4mm 5x41mm ≥ 5mm 6x51mm ≤ 5mm 6x52mm 6-7mm 7x62mm ≥8mm 9x6

D1 D2 Device3mm ≤7mm 7x63mm 8-9mm 9x63mm ≥ 9mm 9x6 or 11x6

< 4mm 9mm 11x6< 4mm 10-11mm 11x6< 4mm ≥ 12mm 11x6

Table: Selection of Nit-Occuld PDA coil (according to angiographic PDA dimensions).

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What about the apparent “hole” in the device (Figure 5)? Doesn’t this work against occlusion? The actual hole in the coil is never pointed into the PDA communication on the pulmonary end, as the coil passing through the PDA is “above” it. When the device lies sideways in the ampulla, occlusion is just as certain, and the device does not need to be reimplanted. The rewound proximal coils of the device are the most important to occlusion of the PDA, and only need to be in the pulmonary end of the PDA regardless of orientation.

The Angiographic Residual Shunt?

Only the tiniest of PDAs (≤1mm minimum diameter) will leave the cardiac catheterization laboratory with complete occlusion of the PDA after deployment of a Nit-Occlud® PDA device. In our 30 patients who

underwent closure with Nit-Occlud® PDA device in the last 2 years, all but 3 had complete occlusion before same day discharge. The other three were occluded at follow-up two months later. Fourteen have returned for 6-month follow-up, and all were occluded including the three discharged with a residual shunt. This parallels the experience reported from the pivotal study where there was 83% occlusion at discharge, and over 96% at both 6-month (Personal Communication, Dave Mittl, B. Braun Interventional Systems Inc. Bethlehem PA) and 1-year fol low-up on echocardiogram (Figure 6).3 Over ¾ of residual shunts at discharge closed after 6 months. This is in contrast to the experience with Gianturco coils, where no patient is left with a residual shunt as additional coils would usually be required later. The Amplatzer devices may have flow through the device after deployment, but no discrete jets. Residual shunts are the rule with the Nit-Occlud® PDA device at the end of the procedure, but these will close, usually within 24 hours.

The pivotal study showed a definitive difference in effectiveness by type of PDA.4 There were too few tubular PDAs closed to draw a conclusion (though all were closed), but only 85% of ‘window-type’ PDAs were effectively closed (Figure 7). And the device is not recommended for transcatheter occlusion of this type of PDA.

Disadvantages

There are few disadvantages to the Nit-Occlud® PDA device. In using the device, it is important to remember that friction between the device coils and mandrel are responsible for retrievability. The mechanism of release is pushing the coils off the end of the mandrel. Prior to retrieval or once the device has been retrieved before release, a separation or “gap" may develop between the coils and the pusher winding (Figure 8) to allow a smooth retrieval into the catheter. The gap at the proximal device coil leaves a step that could catch on retrieval into the delivery catheter, stripping the coil off the mandrel and leading to premature detachment. The manufacturer recommends looking for this gap prior to and during deployment (especially prior to retrieval) of the device into the delivery catheter, and pushing the outer winding up to the device when it is observed (Figure 8). It is also recommended that testing the attachment of the device to the mandrel be confined to pulling the device back within the delivery catheter, rather than exposing the coils from the loader, which could loosen the attachment.

CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 5

Figure 5. Cineradiography in a patient with a Type A ductus. In A the camera is positioned RAO 30° and there in an apparent hole through the device in the long axis of the ductus. In B, however, the long axis of the device (solid line) does not point to the communication through the ductus (arrow) where the proximal Nit-Occlud® coil passes into the pulmonary artery.

Figure 7. Final closure rates by type of Patent Ductus Arteriosus. There were no statistical differences of closure rates for conical, complex or elongated ductuses (96.6-100%). However, just under 85% of window/short ductuses were closed with one device. There were too few tubular ductuses to allow analysis.

Conical Window Complex Elongated

100

95

90

85

80

75

Figure 6. Echocardiographic closure rates over time from the combined pivotal and continued access trials (3). Only 83% of patients had closure at discharge (D/C). By 6 months follow-up there was almost 97% closure (Personal Communication, Dave Mittl, B. Braun Interventional Systems Inc. Bethlehem PA). Of the residual shunts at discharge, 77% had closed by 6 months. There was no further change by 12 months.

100

95

90

85

80

75

96.60

94.10

84.60

955 95.3

DIC 6 Month 12 Month

Data from US IDE Study

Proven Performance. Simply DelivereD.

melody® Transcatheter Pulmonary valve Therapy

melody TPv delays conduiT rePlacemenT

98%Patients at 2 years

Patients at 5 years91%Data from US IDE Study

medtronic | minneapolis, mn 55432-5604 Toll-free: 1 (800) 328-2518

melody-Tpv.com

uc201601525a en ©2015 medtronic. all rights reserved 08/2015

MeottronicFurther,Together

Important Labeling Information for United States

Indications: The melody TPv is indicated for use as an adjunct to surgery in the management of pediatric and adult patients with the following clinical conditions:

• Existenceofafull(circumferential)RVOTconduitthatwasequaltoorgreater than 16 mm in diameter when originally implanted and

• DysfunctionalRVOTconduitswithaclinicalindicationforintervention,and:

-regurgitation:≥moderateregurgitation,AND/OR

-stenosis:meanRVOTgradient≥35mmHg

Contraindications: none known.

Warnings/Precautions/Side Effects

• DONOTimplantintheaorticormitralposition.PreclinicalbenchtestingoftheMelodyvalvesuggeststhatvalvefunctionanddurabilitywillbeextremelylimitedwhenusedintheselocations.

• DONOTuseifpatient’sanatomyprecludesintroductionofthevalve,ifthe venous anatomy cannot accommodate a 22-fr size introducer, or if thereissignificantobstructionofthecentralveins.

• DONOTuseifthereareclinicalorbiologicalsignsofinfectionincludingactiveendocarditis.Standardmedicalandsurgicalcareshouldbestrongly considered in these circumstances.

• Assessmentofthecoronaryarteryanatomyfortheriskofcoronaryarterycompressionshouldbeperformedinallpatientspriortodeployment of the TPv.

• Tominimizetheriskofconduitrupture,donotuseaballoonwithadiameter greater than 110% of the nominal diameter (original implant size) of the conduit for pre-dilation of the intended site of deployment, or for deployment of the TPv.

• Thepotentialforstentfractureshouldbeconsideredinallpatientswho undergo TPv placement. radiographic assessment of the stent withchestradiographyorfluoroscopyshouldbeincludedintheroutinepostoperative evaluation of patients who receive a TPv.

• Ifastentfractureisdetected,continuedmonitoringofthestentshouldbeperformedinconjunctionwithclinicallyappropriatehemodynamicassessment.InpatientswithstentfractureandsignificantassociatedRVOTobstructionorregurgitation,reinterventionshouldbeconsideredin accordance with usual clinical practice.

Potential procedural complications that may result from implantation of the melody device include the following: rupture of the rvoT conduit, compressionofacoronaryartery,perforationofamajorbloodvessel,embolizationormigrationofthedevice,perforationofaheartchamber,arrhythmias,allergicreactiontocontrastmedia,cerebrovascularevents (Tia, cva), infection/sepsis, fever, hematoma, radiation-induced erythema,blistering,orpeelingofskin,pain,swelling,orbruisingatthecatheterization site.

Potential device-related adverse events that may occur following device implantation include the following: stent fracture,* stent fracture resulting inrecurrentobstruction,endocarditis,embolizationormigrationofthedevice, valvular dysfunction (stenosis or regurgitation), paravalvular leak, valvularthrombosis,pulmonarythromboembolism,hemolysis.

*The term “stent fracture” refers to the fracturing of the Melody TPV. However, in subjects with multiple stents in the RVOT it is difficult to definitively attribute stent fractures to the Melody frame versus another stent.

for additional information, please refer to the instructions for use provided with the product.

CAUTION: Federallaw(USA)restrictsthisdevicetosalebyorontheorderof a physician.

melody is a registered trademark of medtronic.

Medical City Children’s Hospital has an unwavering focus on patient care and offers world-renowned excellence in comprehensive pediatric services. Since 1996, our specialists haven’t let anything distract them from serving children. As a result, we’ve helped thousands of children from more than 75 countries. We are a comprehensive children’s hospital with specialists in virtually every pediatric subspecialty. Medical City is the only facility in north Texas where fetal diagnosis, maternal, neonatal and pediatric transport, high risk delivery stabilization in the NICU, corrective surgery, state of the art postoperative monitoring and care and long term follow-up of patients with complex congenital heart disease can all be delivered under one roof.

The Congenital Heart Surgery Unit (CHSU) accommodates around 400 children annually who undergo heart operations performed by Dr. Eric Mendeloff. 30% of our cases are neonates and 58% are under the age of 2 years. Cases range in complexity from palliation of hypoplastic left heart syndrome to closure of atrial and ventricular septal defects. Highly specialized care in the CHSU is provided by subspeciality-trained physicians and an excellent group of long term nurses and respiratory therapists. This focus on pediatric cardiac critical care has resulted in superlative patient outcomes that exceed national norms. The heart program’s success has attracted referrals from across the country. With the addition of a second Congenital Heart Surgeon to our already robust program, we anticipate growth that will require a sixth member for our CICU team in addition to our need for a Medical Director of the Unit. Preferred candidate for the director level position will possess leadership attributes with evidenced experience, along with a strong clinical skill set.

All candidates are preferred to be BC/BE in Pediatric Cardiology and Pediatric Critical Care or boarded in one of these with additional training in Pediatric Cardiac Critical Care. Those with certification in one discipline and solid experience in the alternate subspecialty should also apply. Positions are employed and offer a competitive salary and excellent benefits packet.

Our hospital has immense current capabilities and is positioned to grow.

Kathy KyerNational Director of Pediatric Subspecialty Recruitment

Kathleen.Kyer@HCAHealthcare.com937.235.5890

Medical Director and Staff Level Pediatric Cardiovascular Critical Care Physicians

General Pediatric Cardiologist Pediatric Cardiac Interventionalist

Geneticist

CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 7

MedtronicFurther,Together

13- MedicalCityChildren‘; HospitarThe City Just for Kids

Advantages

The Nit-Occlud® device enters a space (transcatheter PDA closure devices) that is already occupied by several devices specifically designed for PDA closure, as well as some off-label devices that are, nevertheless effective. It’s ease of use and effective closure rates outweigh unfamiliarity with the device, anxiety over residual shunting in the cardiac catheterization laboratory, and the remote possibility of premature release. The delivery system is smaller than for the ADO1 device for transvenous approach. It can be used with a 3.3F arterial sheath. The cost is competitive. In our hands the device has been easy to use and effective. Not all

PDAs can be addressed with the Nit-Occlud® PDA device. However, it significantly expands the options for treating children with a persistentl Patent Ductus Arteriosus.

References

1. Porstman W, Wierny L, Warnke H, Gerstberger G, Romaniuk PA. Catheter closure of patent ductus arteriosus: 62 cases treated without thoracotomy. Radiol Clin North Am. 1971;9:203-218.

2. Cambier PA, Kirby WC, Wortham D, Moore JW. Percutaneous closure of the small (less than 2.5 mm) patent ductus a r te r iosus us ing co i l embo l ia t i on . Am J Card io l . 1992;69:815-816.

3. Summary of safety and effectiveness data (SSED), Device Generic Name: Transcatheter Patent Ductus Arteriosus (PDA) Occlusion Device, Device Trade Name: Nit-Occlud® PDA, h t t p : / / w w w. a c c e s s d a t a . f d a . g o v / c d r h _ d o c s / p d f 1 2 /P120009b.pdfww.

4. Moore JW, Greene J, Palomares S, Javois A, Owada CY, Cheatham JP, Hoyer MH, Jones TK, Levi DS. Results of the combined U.S. multicenter pivotal study and the continuing access study of the Nit-Occlud PDA Device for percutaneous closure of patent ductus arteriosus. J Am Coll Cardiol: Intv. 2014;7:1430-1436.

CCT

CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 8

Figure 8. Photographs of the Nit-Occlud® device attached to the mandrel of the delivery system after full deployment, but prior to device release. In panel A, there is a “gap” shown between the arrows. The "gap" is a length of mandrel between the pusher winding and the end of the device, attached to the end of the mandrel. Prior to release or recapture of the device, as shown at the arrow in Panel B, the "gap" should be eliminated by advancing the pusher winding to the end of the device.

John L. Bass, MDDirector Interventional CatheterizationUniversity of MinnesotaMasonic Children’s Hospital2450 Riverside Ave.East Building, Rm. MB547Minneapolis, MN 55454 USAPhone: 612.626.2755; Fax: 612.626.2467bassx001@umn.edu

“Not all PDAs can be addressed with the Nit-Occlud® PDA device. However, it significantly expands the options for treating children with a persistently Patent Ductus Arteriosus.”

Biosketch of Corresponding Author

Dr. Gurumurthy Hiremath is an Assistant Professor of Pediatrics at the University of Minnesota Masonic Children’s Hospital. He did his Pediatric Residency at Wayne State University in Detroit MI, and Pediatric Cardiology Fellowship at USCF in San Francisco CA. Dr. Hiremath completed a 4th year of Interventional training at UCSF, where he pursued a study of the effects of relief of unilateral pulmonary artery stenosis on exercise capacity, and late outcomes after Atrial Septal Defect (ASD) device closure in infants under 15kg. His early efforts in Minnesota center around Interventional Catheterization with stent placement and treatment of paravalvar leaks. He continues a multicenter study on the effects of relieving unilateral branch pulmonary artery stenosis.

Corresponding Author

Gurumurthy Hiremath, MDDepartment of PediatricsPediatric CardiologyUniversity of MinnesotaMasonic Children’s Hospital2450 Riverside Ave.East Building, Rm. MB547Minneapolis, MN 55454 USAPhone: 612.626.2755; Fax: 612.626.2467hiremath@umn.edu

The Second Fetal Cardiac Symposium took place in the amazing summertime that the windy city offers. The meeting was held at Rush University Medical Center in Chicago and was expanded from last year to two-and-a-half days, and included a total of 15 faculty members from various institutions. With the persistence of the low nat ional prenata l detect ion rate of congeni ta l cardiac defects, despi te universal screening during pregnancy, the main goal of the conference was to boost the education in the field in order help improve the status of prenatal diagnosis of Congenital Heart Disease. This was done through a series of didactic lectures, as well as by focusing on improving the technical skills in scanning the fetal heart through hands-on sessions.

As was the case with the first year of launching the meeting, the symposium continued to be a tremendous success and was nearly sold-out with an audience of 140 registrants who came from 10 different countries and more than 19 states within the U.S. Fifteen percent of the registrants came from overseas, including countries such as: Canada, Japan, Brazil, Mexico, China, Columbia, The Netherlands, Iran, Qatar and Saudi Arabia.

Most of the attendees (~65%), however, came from the Midwest states, highlighting the need for such a conference focusing on the fetal heart in this region. Although more than 50% of the attendees were physicians, there were about 30% sonographers attending the meeting, likely reflecting the attractiveness of hands-on workshops included in the meeting and that provide the a t t endees w i t h p rac t i ca l scann ing opportunities rather than only didactic lectures. The attendees came from various

specialties including: Pediatric Cardiology, OB and MFM as well as other specialties such as Neonatology and Radiology.

The conference featured a two-and-a-half day meeting that offered thorough and updated presentations on scanning the fetal heart and diagnosing and managing various common fetal congenital heart disease malformations. The activity was designated for a maximum of 19.25 AMA PRA Category 1 continuing medical education credit, 19.25 continuing Nursing Education credits and 19.25 CME credits in medical sonography (SDMS). Lectures, given by an internationally acclaimed facul ty in Pediatr ic Cardiology and Maternal-Fetal Medicine special ists, emphasized the basics of fetal cardiac s c a n n i n g c o u p l e d w i t h l i v e c a s e demonstrations and tips for diagnosing various anomalies. There was intensive focus on anomalies of the four-chamber and outflow-tracts views, reflecting the recently published guidelines for screening for fetal heart disease. In addition, there was specific focus on the 3-vessel and tracheal view that Dr. Alfred Abuhamad lectured on very extensively with ample video clips and examples to the point that he almost called it the most important view, and likely the only view needed for screening for Critical Congenital Heart Disease (CCHD)! The symposium featured a unique two-hour workshop on both days of the meeting, which gave the attendees a unique opportunity to scan pregnant volunteers with both normal hearts and cardiac pathology. The pathologies included: right-sided and left-sided critical lesions that helped attendees practice scanning fetuses with anomalies. This provided an excellent opportunity for becoming more familiar with the required cardiac views, including the four-chamber, the outflow tracts and the three-vessel v iews. I t a lso al lowed participants to experiment scanning using

CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 9

Highlights from the Second Fetal Cardiac Symposium at Rush University in Chicago - September 10th-12th, 2015By Karim Diab, MD

various technological instruments and machines that are currently on the market. All this was done under the supervision of expert faculty in the field of Fetal Cardiology and Maternal Fetal Medicine.

The first day of the meeting started with an o v e r v i e w o f t h e b a s i c s o f f e t a l cardiovascular physiology and the current

guidelines and indications for performing fetal echocardiography by Drs. Abdulla and Young. Then, Dr. Mark Sklansky from UCLA went over the impact of prenatal diagnosis of CHD on the outcome for these patients. This was followed by a live scanning demonstration of a complete fetal e c h o c a r d i o g r a p h i c s t u d y. I t t h e n sequentially focused on the essential

screening views of the fetal heart, including the four-chamber and the outflow tract views, as well as the three-vessel view. This demonstrated the normal f indings, as well as typical cardiac lesions diagnosed with the particular view which helped give the audience practical tips for scanning and d i a g n o s i n g v a r i o u s c a r d i a c ma l fo rma t ions . Add i t i ona l l ec tu res focused on specif ic lesions. Dr. E. Alboliras went over Ventricular Septal Defect (VSD) and AVC lesions, and Dr. W. Tworetzky talked about Ebstein’s A n o m a l y a n d s o m e o f t h e n e w l y suggested in-utero treatment modalities in some cases using NSAIDs to close the ductus. He also talked about HLHS with the skinny LV vs. the fat and globular LV. Dr. E. Jaeggi went over Total Anomalous Pulmonary Venous Return (TAPVR), which continues to be tricky to diagnose and is missed on prenatal screens, as well as DILV and MV anomalies. Other lesions that were discussed included: D-TGA and cc-TGA (Dr. Sklansky), ductal anomalies and conotruncal anomalies, as well as coarctation and arch anomalies. The first day ended with the first hands-on session of the symposium with 6 stat ions for hands-on scanning on fetuses with normal hearts as well as cardiac pathologies.

The second day of the symposium started with a session focusing on the national guidelines for screening for CHD by Dr. Abuhamad. Additional lectures went over various cases and tips in imaging (Dr. Awad), 3-D and 4-D imaging by Dr. Sklansky, cardiomegaly and CM (Dr. Young), fetal cardiac tumors (Dr. Jaeggi) and hydrops and its management (Dr. Hornberger).

Dr. Abuhamad lectured extensively on the three-vessel and tracheal view and Dr. Tworetzky talked about the various heterotaxy lesions.

Midday on the second day of the meeting there was another hands-on session with an additional 6 stations for scanning and practice.

There was also a session on the “Most Interesting Case I’ve Seen,” where the audience presented interesting, and u n u s u a l f e t a l c a s e s w i t h c a r d i a c pathology that included cases such as

CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 10

Archiving Working GroupInternational Society for Nomenclature of Paediatric and Congenital Heart Disease

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fe ta l aor t ic -LV tunne l , c r i t i ca l PS, aneurysm of the intervalvular fibrosa and AVC, Ebstein’s Anomaly, fetal AF, and a case of a giant LA pseudoaneurysm.

There were two winners of a special prize and a free registration to next year's symposium which was awarded to two of the presenters. This gave the audience ample opportunity to present challenging fetal cardiac cases and discuss them with the faculty.

The third day of the meeting focused on fetal rhythm abnormalities, with lectures on the techniques to evaluate the fetal r h y t h m b y e c h o a n d b y f e t a l m a g n e t o c a r d i o g r a p h y, a s w e l l a s diagnosing and managing fetal tachy and brady-arrhythmias and heart block. The last session of the meeting focused on fetal cardiac imaging in early gestation, genetic evaluation of the fetus with CHD and neurologic development of the fetus with CHD.

In addition to an extensive series of didactic presentations and live-scanning sessions, the symposium offered a variety of exhibitors that showcased the latest in ultrasound technology. The symposium also offered an exceptional opportunity for fellows and attendees to network and meet with pioneers in the field, as well as an opportunity to catch up wi th col leagues and meet new friends.

Given the recent updates and revisions to the North American guidelines, as well as the International Society of U l t r a s o u n d i n O b s t e t r i c s a n d G y n e c o l o g y ( I U S O G ) p r a c t i c e guidelines on sonographic screening examination of the fetal heart, the need for such annual fetal symposia in different regions is a must, without any doubt, in order to improve the skills of various practitioners in the field and u l t i m a t e l y i m p r o v e t h e p r e n a t a l detection of CHD!

Keep an eye out for next year’s meeting information as the registration sold out t h i s yea r ! ! Da tes f o r nex t yea r ’s symposium will be announced in the near future; for more information, you can reach Dr. Diab at Karim_Diab@rush.edu o r v i s i t t h e m e e t i n g w e b s i t e a t www.FetalCardiacSymposium.com.

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CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 11

Corresponding Author

Karim A. Diab, MD, FACCRush Center for Congenital Heart DiseaseRush University Medical Center1653 W. Congress Parkway661PAVChicago, IL 60612 USA

Karim_Diab@rush.edu

C o l l a b o r a t e , L e a r n & E n g a g e

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“Given the recent updates and revisions to the North American guidelines, as well as the International Society of Ultrasound in Obstetrics and Gynecology (IUSOG) practice guidelines on sonographic screening examination of the fetal heart, the need for such annual fetal symposia in different regions is a must, without any doubt, in order to improve the skills of various practitioners in the field and ultimately improve the prenatal detection of CHD!”

ASSISTANT/ASSOCIATE PROFESSOR

PEDIATRIC CARDIOLOGIST UNIVERSITY OF NEW MEXICO

Ful l - t ime facul ty posi t ions are available at the level of Assistant/Associate Professor in the Pediatric Cardiology Division at the School of Medicine, University of New Mexico.

Applicants must be authorized to work in the United States, BC/BE in Pediatric Cardiology, and eligible to be licensed in the State of New M e x i c o . P r e f e r e n c e s i n c l u d e ; demonstrated excel lence as a general clinical pediatric cardiologist and cardiac imaging and a record of teaching experience.

This position is subject to criminal records screening in accordance with New Mexico law and clearance by the New Mexico Department of Health is required as a condition of employment.

The University of New Mexico is an Equal Opportunity/Affirmative Action

Employer and Educator.

" U N M ' s c o n f i d e n t i a l i t y p o l i c y ("Recruitment and Hiring," Policy #3210), which includes information about public disclosure of documents submitted by applicants, is located at http://www.unm.edu/~ubppm."

For complete details of this position or to apply, please visit this website:https://unmjobs.unm.edu/

Please reference Posting Number: #0832756.

For additional information you may contact Lynda Easter, Search Coordinator, at leaster@salud.unm.edu

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Introduction

The Atrioventricular Septal Defect (AVSD) occurs in 2: 10.000 live births, accounting for 3% of Congenital Heart Disease (CHD), AVSD is more frequent in females and usually associated with carriers of the Down Syndrome (DS).1,2

The AVSD is characterized by the absence of the atrioventricular septum, and five or more atrioventricular valve leaflets of varying sizes are usually present.1,2

This clinical case is characterized by increased pulmonary blood flow in the first weeks of life. The natural history of this defect is survival of 54% at six months and 15% at two years.1 If significant regurgitation of the common atrioventricular valve is present, a systolic cardiac murmur and gallop rhythm are frequently heard. Over time, pulmonary hypertension and congestive heart failure (CHF) are observed.2

The main cause of death in infants is CHF; after the first year of life, occlusive pulmonary vascular disease predominates,1

The diagnosis should be suspected in patients with CHF symptoms in the first months of life. Chest radiographs show cardiomegaly. The electrocardiogram may show left-axis deviation and bi-atrial and bi-ventricular enlargement, in addition to pressure and volume overload. Echocardiography is the main tool for both the diagnosis and anatomical classification of this malformation, according to Rastelli classification (Type A, B or C). The electrocardiogram also demonstrated the Atrial Septal D e f e c t , w h i c h i s c o m m o n l y a s s o c i a t e d w i t h AV S D . Echocardiography can still observe anatomical defects, such as atrial septal defect with the underlying common atrioventricular valve and Ventricular Septal Defect (VSD).

Medical treatment is aimed at improving the signs and symptoms of CHF. Medications used may be digoxin, diuretics (such as furosemide and spironolactone), and vasodilators (often angiotensin-converting enzyme inhibitors). Administration of these medications, pre-operatively, may result in an improved post-operative course and prognosis.

The surgery to repair AVSD involves the use of two patches (an atrial and a ventricular patch), which maintains the integrity of the valvular leaflets.1

Case Report

A 24-year-old woman was admitted to the cardiology clinic from the Hospital Universitário Sul Fluminense (HUSF), reporting effort dyspnea on exertion and chest pain.

The patient reported strong chest pain, which did not radiate, but was effort-related and was relieved by rest. The patient also presented with cyanosis of fingers and lips, numbness in left hemibody, more pronounced in the upper limb. Physical examination of the cardiovascular system showed a Grade 3/6 murmur at the left-midsternal border. The patient had no cognitive impairment, nor Down Syndrome stigmata.

An electrocardiogram was conducted, which showed sinus rhythm, 1st degree atrioventricular blockage, left-anterior hemiblock and change in lower and anteroseptal repolarization. Structural and functional study of the heart was requested through Doppler echocardiography.

On Color Dopper...(Figure 1), right ventricular and right atrial enlargement were noted, as was the presence of large Atrial Septal Defects with left-to-right shunt, mild tricuspid insufficiency and mild pulmonary arterial hypertension. Other parameters were normal. Transesophageal echocardiography or cardiac MRI was recommended.

Transesophageal echocardiography revealed a partial form of AVSD (interatrial communication ostium primum), enlargement of the right ventricle and right atrium , valve regurgitation, moderate right-sided valve regurgitation, mild pulmonary hypertension,(pulmonary artery systolic pressure (PASP) estimated to be 39 mm Hg by Doppler). Bi-ventricular systolic function was preserved. There were no thrombi observed, and flow was normal in the left atrial appendage.

It was concluded that there was an AVSD with single atrioventricular valve, classified as Type A in the Rastelli classification. Consequently, the patient was referred for cardiac surgery, where Inteventricular Communication Closure (IVC), Interatrial Communication Closure (IAC), and mitral and tricuspid valvuloplasty were performed.

When she returned to the clinic, the patient reported improvement in signs and symptoms of heart failure, with mild pain in the surgical wound. Physical examination of the cardiovascular system showed a regular heart rate, accentuated P2 and no murmurs.

Two-dimensional echocardiography with Color Doppler was conducted and showed cardiac chambers of normal morphology and dimensions. Global Left Ventricular (LV) and Right Ventricular (RV) systolic function were normal. LV wall thickness was normal. Segmental contractility normal. Left atrial diameter was normal. The mitral valve had thickened leaflets. The aortic, tricuspid and pulmonary valves were normal. The inferior vena cava and pericardium were normal. There was mild mitral regurgitation. There was normal flow in the outflow tract of the left ventricle and aorta. Tricuspid and pulmonary flow were unchanged.

The patient was discharged eight days after being admitted to the hospital and is currently under follow-up at HUSF cardiology clinic.

CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 12

Atrioventricular Septal Defect: Case ReportBy Felipe Guimarães Machado, MD; Éric Guimarães Machado, MD; Maria Clara Menezes de Jesus Lisboa, MD; Léo Guimarães Soares, DDS, PhD; Paulo Sérgio Lopes Soares, MD; Gabriel Porto Soares, MD, PhD

www.giftofl i feinternational.org

A Rotarian-based organization that over the past 4 decades has helped more than 18,000 children from emerging countries receive treatment for their heart disease.

Gift of Life InternationalPO Box 650436Fresh Meadows, NY 11365Email: r ray lman@ao l . com Phone: 845-546-2104

""‘,G11:ToF L1FE@INTERNATIONAL

CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 13

Figure 1. Echocardiogram with Color Doppler. A: M mode or unidimensional left ventricle longitudinal section. B: M mode or one-dimensional aorta and left atrium longitudinal section. C: Apical 4-chamber view. D: Mitral flow demonstrated by Color Doppler. E: Aortic flow demonstratedby Color Doppler. F: Interatrial communication visualization.

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Discussion

The case reported is about a female patient, without a genetic syndrome, who became symptomatic in the third decade of l i fe. According to l i terature,1,3,4 the incidence in women is slightly higher than in men, ranging between 52% and 62%.

Typically, the clinical manifestation and surgical correction occur in early age. The scientific literature shows average ages of the correction ranging from 3.7 to 18.7 months of age.1, 3-6 However, in this case, the patient showed signs and symptoms in the third decade of life, with the surgical correction performed at that time.

Atrialventricular Septal Defect is usually associated with Down Syndrome in 64% to 89% of patients, according to previous reports. 1, 3-6 However, this is a report of a patient without Down Syndrome.

The definitive diagnosis and Rastelli classification of this case was achieved through echocardiography. Other authors also used echocardiography for diagnosis and classification. The patient has a Rastelli Type A defect, which is the most common type in the earlier studies, ranging from 58% to 80%.

Regarding medical therapy, beta-blockers were used; for surgery, the technique of two patches was used. Both were similar to other reports.2 Other authors 1, 4-6 reported use of the double-patch surgical technique in 72% of pat ients .1,3,4,5 S t u d i e s 6 c o m p a r i n g t h e s u r g i c a l techniques have concluded that the single- and double-patch technique present similar final results.

Thus, it can be concluded that this case was similar to the literature because the patient was female, and because of the methods of diagnosis and treatment. However, the age and lack of Down Syndrome are different from most reported c a s e s . F i n a l l y , t h i s c a s e r e p o r t demonstrates the importance of diagnosing and treating this disease.

References

1. Tagliari AP, Schröder DA, Filho GT, Prates PR, Sant’Anna JRM, Nesralla IA, et al. Results of simplified single-p a t c h r e p a i r f o r c o m p l e t e atrioventricular septal defect. Arq Bras Cardiol. 2013;100(3):288-93.

2. Calabrò R, Limongelli G. Complete atrioventricular canal. Orphanet J Rare Dis. 2006;1:8.

3. Crawford FA, Stroud MR. Surgical r e p a i r o f c o m p l e t e atrioventricularseptal defect. Ann Thorac Surg. 2001;72(5):1621–29.

4. Atz AM, Hawkins JA, Lu M, Cohen MS, Colan SD, Jaggers J, et al. Surgical m a n a g e m e n t o f c o m p l e t e a t r i o v e n t r i c u l a r s e p t a l d e f e c t : Associations with surgical technique, age, and t r isomy 21. J Thorac Ca rd iovasc Su rg . 2011 ;141 (6 ) :1371-79.

5. Daebritz SH. Correction of Complete Atrioventricular Septal Defects With Two Patch Technique. Operative Te c h n i q u e s i n T h o r a c i c a n d C a r d i o v a s c u l a r S u r g e r y : A Comparative Atlas. 2004;9(3):208-20.

6. Backer CL, Stewart RD, Bailliard F, Kelle AM, Webb CL, Mavroudis C. Complete atr ioventr icular canal : comparison of modified single-patch t e c h n i q u e w i t h t w o - p a t c h t e c h n i q u e . A n n T h o r a c Surg. 2007;84(6):2038–46.

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CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 14

Biographical Sketch of Corresponding Author

Felipe Guimarães Machado is currently a sixth year medical student at Universidade Severino Sombra (Vassouras, RJ/Brasil).

Éric Guimarães Machado, MD Department of Anesthesia, Therezinha de Jesus Hospital, Juiz de Fora - MG, Brazil

Léo Guimarães Soares, DDS, PhDDentistry, Universidade Estadual do Rio de Janeiro Rio de Janeiro - RJ, Brazil

Corresponding Author

Felipe Guimarães Machado, MDMedical Student from Severino Sombra University, Vassouras - RJ, Brazil

Mailing Address:Alfredo da Costa Mattos Junior St., 75. Paraíba do Sul – RJ, BrasilCEP 25850-000Phone: +5524981179649Felipe_gmachado@hotmail.com

Maria Clara Menezes de Jesus Lisboa, MDDepartment of Internal Medicine, Santa Casa de Misericórdia de Juiz de Fora - MG, Brazil

Paulo Sérgio Lopes Soares, MD Department of Cardiology, Medical School from Severino Sombra University, Vassouras - RJ, Brazil

Gabriel Porto Soares, MD, PhDDepartment of Cardiology, Medical School from Severino Sombra University, Vassouras - RJ, Brazil

The Ward Family Heart Center at Children’s Mercy Kansas City advances heart care through innovation. From fetal cardiology to the latest ventricular support devices and heart transplants, our team of dedicated experts is equipped to treat all pediatric heart conditions.

Here, evidence-based care is a reality. Our HeartCenter® database helps us transform information into action. Updated constantly, this real-time, clinical data center allows us to monitor and modify treatments for the best patient outcomes.

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The 48th Annual Southeastern Pediatric Cardiology Society (SPCS) Meeting was held in Birmingham, AL at Renaissance Birmingham Ross Bridge Golf Resort and Spa, September 25th-26th, 2015 The Society, which was founded by Dr. Arno Hohn and other Pediatric Cardiologists from the Southeast, has met yearly in various locations in the Southeastern US. The meeting occurs in the fall of each year. Generous support from industry sponsors and local institutions have helped defray the costs of the meeting.

On the evening of September 24th, a Welcome Reception was held on the Ross Bridge Patio. SPCS attendees, including team members and trainees from pediatric cardiac programs throughout the Southeast, as well as attendees from as far away as Minnesota and Seattle, were able to get reacquainted and network.

On Friday, September 25th, the scientific session began featuring prestigious guest speakers. Dr. Michael Ackerman from Mayo Clinic gave state-of-the-art presentations on “Molecular Autopsy” and “Long QT Diagnosis and Treatment.” Dr. Prince Kannakeril from Va n d e r b i l t U n i v e r s i t y p r e s e n t e d “Personalized Treatment of Arrhythmias.”

The attendees were then provided lunch at Children’s of Alabama, where two outstanding abstracts were presented by Dr. Srikant Das of Arkansas Children’s Hospital and Dr. Aamisha Gupta of Georgia Regent’s University. There was also a tour of the new Joseph A. Bruno Heart Center at Children’s of Alabama.

Additional presentations on September 25th included guest speakers: Dr. Heather T Henderson from Duke University who

presented “Mechanical Circulatory Support Options in Children,” and Dr. Iki Adachi from Texas Children’s Hospital who presented “Evolution of the Mechanical Circulatory Support Program at Texas Children’s Hospital.” Further presentations that afternoon from local faculty included: talks on “Outpatient Treatment of Children on VAD Support,” presentations on database research, new information on registry participation, and findings from some of the largest Pediatric Cardiac Registries including: PHTS (The Pediatric Heart Transplant Study), INTERMACs (The Interagency Registry for Mechanically-Assisted Circulatory Support), PediMacs, STS (Society of Thoracic Surgeons) and CHSS (Congenital Heart Surgeons' Society) databases. Panel discussions with interactive audience participation followed each of the sessions.

There were over 40 abstracts submitted and accepted by the SPCS Scientific Committee, who also selected the outstanding abstracts for oral presentation. All abstracts were displayed as posters and generated a great deal of interest.

The day’s session was followed by a fellow’s networking reception and dinner with live music on the Ross Bridge Patio.

On the morning of the second day, there were physician and nursing breakout sessions. For the physician section, local faculty provided presentations on current topics in imaging, qual i ty improvement, d iagnosis and m a n a g e m e n t o f a o r t o p a t h i e s , a n d interventional cardiology.

The nursing breakout sessions featured presentations from local nursing faculty, as well as invited speakers including: Emily Moore, ARNP, BSN, Seattle Children’s Hospital who presented, “Pain Management Following Cardiac Catheterization,” Bronwyn Bartle, DNP, MSN, of Duke University presented a talk entitled, “Care Transitions–Easy as Child’s Play–Right?” Misty Ellis, MSN, APRN, of Kosair Children’s Hospital presented, “Gas Prices on the Rise.” JoAnn Nieves, MSN, APRN of Miami Children’s Hospital presented “Interstage Monitoring: Who, Why and How.”

At the conclusion of the meeting, the location for next year’s meeting was announced. The 49th Annual Southeastern Pediatric Cardiology Society Conference will be hosted by Joe DiMaggio Children’s Hospital in sunny south Florida. The meeting will be held on September 15th-17th, 2016 at the Hyatt Regency Pier Sixty-Six in Ft. Lauderdale, FL. Dr. Maryanne Chrisant will serve as Program Director.

Information concerning the 48th annual meeting, meeting presentations described above and information for other upcoming meetings of interest can be found on the 48th annual meeting website - https://www.childrensal.org/spcs2015.

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CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 16

The 48th Annual Southeast Pediatric Cardiology Society Conference Meeting ReviewBy F. Bennett Pearce, MD

F. Bennett Pearce, MDProfessor and Medical Director-Pediatric Heart Transplant, Advanced Heart FailureLM Bargeron Division of Pediatric CardiologyUAB School of MedicineWomen and Infants Center, Ste. 91001700 6th Ave. SouthBirmingham, AL 35233 USAPhone: 205.934.3460; Fax:205.975.6291BPearce@peds.uab.edu

We are committed to the lifetime management of congenital heart disease.

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CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 18

Hearts Build New Muscle with this Simple Protein Patch An international team of researchers has identified a protein that helps heart muscle cells regenerate after a heart attack. Researchers also showed that a patch loaded with the protein and placed inside the heart improved cardiac function and survival rates after a heart attack in mice and pigs.

Animal hearts regained close to normal function within four to eight weeks after treatment with the protein patch. It might be possible to test the patch in human clinical trials as early as 2017. The team, led by Professor Pilar Ruiz-Lozano at Stanford University and involving researchers from the University of California, San Diego and Sanford Burnham Prebys Medical Discovery Institute (SBP) published their findings in the Sept. 16 online issue of Nature.

"We are really excited about the prospect of bringing this technology to the clinic," said Mark Mercola, Professor of Bioengineering at UC San Diego and a professor in the Development, Aging and Regeneration Program at SBP. "It's commercially viable, clinically attractive and you don't need immunosuppressive drugs."

High throughput technology in Mercola's lab was critical in identifying a natural protein, called Follistatin-like 1 (FSTL1), and showing that it can stimulate cultured heart muscle cells to divide. Researchers led by Ruiz-Lozano at Stanford embedded the protein in a patch and applied it to the surface of mouse and pig hearts that had undergone an experimental form of myocardial infarction or "heart attack." Remarkably, FSTL1 caused heart muscle cells already present within the heart to multiply and re-build the damaged heart and reduce scarring.

Heart muscle regeneration and scarring are two major issues that current treatments for heart attacks do not address, said Ruiz-Lozano. "Treatments don't deal with this fundamental problem--and consequently

Medical News, Products & InformationCompiled and Reviewed by Tony Carlson, Senior Editor

For all details to register, see www.nicklauschildrens.org/MiamiSCA or call 1-305-669-5873 or email: cme4mch@mch.com Featured Topics:

Associated structural and primary electrical defects The role of genetic testing Athletes and sudden cardiac arrest Sudden cardiac arrest prevention and the legal system ICD use and athletes.

Includes: Up to 13 CMEs/CEUs credits Spanish and English translation Hands on breakout sessions Nursing break out sessions

COURSE FACULTY Dominic Abrams, MBBS Micheal Ackerman, MD, PhD Anjan Batra, MD Woodrow Benson, MD, PhD Stuart Berger, MD Randall M. Bryant, MD Christine Chiu-Man, MSC Mitchell Cohen, MD Jane Crosson, MD Brynn Dechert-Crooks, CPNP Christopher Erickson, MD Peter Fischbach, MD Steven Fishberger, MD Richard Friedman, MD Debra Hanisch, CPNP Salim Idriss, MD, PhD, BME Ronald Kanter, MD Rachel Lampert, MD Ian Law, MD Melissa Olen, FNP-C John Papagiannis, MD Timothy Paterick, MD, JD Anthony Rossi, MD Elizabeth Saarel, MD Shubhayan Santani, MD Maully Shah, MD Michael Silka, MD Paul Stephens, Jr, MD Ming-Lon Young, MD Frank Zimmerman, MD

Sudden Death in the Young February 19-20, 2016 Miami, Florida, USA

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CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 19

many patients progressively lose heart function, leading to long-term disability and eventually death," she said.

Today, most patients survive a heart attack immediately after it happens. However, the organ is damaged and scarred, making it harder to pump blood. Sustained pressure causes scarring to spread and ultimately leads to heart failure. Heart failure is a major source of mortality worldwide, and roughly half of heart failure patients die within five to six years. Treatments available today focus primarily on making it easier for the heart to pump blood, and advances have extended patients' lives. But they can't help regenerate heart tissue.

The team initially looked to other species for inspiration. Lower vertebrates, such as fish, can regenerate heart muscle, and prior studies in fish suggested that the epicardium, the heart's outside layer, might produce regenerative compounds. The researchers joined forces to find a solution.

The team started with the epicardial cells themselves, and showed that they stimulated existing heart muscle cells, or cardiomyocytes, to replicate. To find whether a single compound might be responsible, the Mercola lab used mass spectrometry, a sophisticated technology, to find over 300 proteins produced by the cells that could fit the bill. They then screened a number of these candidates using high throughput assays to look for the ones that had the same activity as the cells, and found that only one did the job: Follistatin-like 1 (FSTL1).

The group at Stanford--including teams led by Ruiz-Lozano, Dan Bernstein, Manish Butte and Phil Yang-- led the development effort for a therapeutic patch made out of collagen, which was cast with FSTL1 at its core. The patch has the elasticity of fetal heart tissue and slowly releases the protein. "It could act like a cell nursery," Ruiz-Lozano said. "It's a hospitable environment. Over time, it gets remodeled and becomes vascularized as new muscle cells come in."

Testing the patch loaded with FSTL1 in a heart attack model in mice and pigs showed that it stimulated tissue regeneration even if implanted after the injury. For example, in pigs that had suffered a heart attack, the fraction of blood pumped out of the left ventricle dropped from the normal 50% to 30%. But function was restored to 40% after the patch was surgically placed onto the heart a week after injury and remained stable. The pigs' heart tissue also scarred considerably less.

Ruiz-Lozano is the co-founder of EpikaBio, a startup that aims to bring the patches to human clinical trials as soon as possible.

Research Team Identifies New Genetic Cause for Heart Arrhythmia

Newswise – Scientists at The Ohio State University Dorothy M. Davis Heart and Lung Research Institute have identified a new genetic cause for congenital heart arrhythmia. The results of their research are published online by the Proceedings of the National Academy of Sciences (PNAS).

The mechanism is due to defects in the regulation of the primary sodium channel, which controls the flow of sodium ions across the heart cell membrane. When these channels don’t work properly, it promotes abnormal heart rate (arrhythmia) and symptoms of heart failure.

Advanced Heart Failure, Transplant Cardiologist (Pediatric)

Phoenix Children’s Hospital has an opportunity for a Pediatric Cardiologist with experience in Heart Transplant and Advanced Heart Failure Management to join one of the largest heart centers in the west. Join a thriving heart center with broad multidisciplinary expertise, two dedicated pediatric cardiothoracic surgeons and collaborate with 28 cardiologists to provide cutting edge cardiac care for children throughout the region. This transplant and heart failure focused position will join three subspecialty cardiologists, three nurse practitioners and a broad team supporting clinical, administrative and academic growth. Targeting patients with advanced heart failure including those needing mechanical circulatory support and/or heart transplant care, opportunities abound for teaching, clinical leadership, as well as clinical and translational research.

Job Roles

Provide subspecialty care in the inpatient and outpatient setting for targeted patient populations: advanced heart failure including those needing mechanical circulatory support, cardiomyopathy, the failing single ventricle and heart transplant candidates/recipients.

Follow a growing population of advanced heart failure and pre/post-transplant patients.

Work with a transplant team of 2 surgeons, 3 dedicated nurse practitioners, 3 cardiologists and staff support program in a manner that fulfills the strategic goals of the Hospital while complying with state and federal laws and accreditation standards related to safety and risk management.

Support outpatient VAD Program.

Desired Skills & Experience Minimum Qualifications

Formal training or experience in pediatric heart transplantation and advanced heart failure.

Board Certified/Eligible in Pediatric Cardiology.

Willingness to apply for academic position with the University of Arizona and Mayo Clinic.

Please direct inquiries/CV to:

Steven Zangwill, MD c/o Brooke Wedding

bwedding@phoenixchildrens.comor contact me directly at:

szangwill@phoenixchildrens.com

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“The mechanism of disease was unexpected,” said Hassan Musa, a research scientist in Ohio State’s Department of Physiology and Cell Biology and first author of the publication. “As a field, we are beginning to identify genetic variants that alter the sodium channel’s association with essential regulatory proteins. However, in this case, we were surprised by the impact of this gene variant on the regulation of the sodium channel by a protein called FGF12.”

Musa and the team examined genes from a 27-year-old man who had suffered sudden cardiac arrest while moving boxes at work. In addition to the cardiac arrest, tests showed the man also has atrial fibrillation. Doctors implanted a defibrillator, which has appropriately fired several times to save the man’s life.

“Because of this man’s cardiac arrest at a young age, as well as his family history of sudden cardiac death, our team in the Inherited Arrhythmia Clinic had a high suspicion for an inherited arrhythmia disease, so we conducted genetic testing for known arrhythmia genes,” said Amy Sturm, licensed genetic counselor and a co-author on the research. “This identified a novel mutation previously undescribed in the sodium channel SCN5A gene. Mutations in SCN5A are associated with several hereditary cardiovascular diseases, so we knew it was a strong candidate for his and his family’s arrhythmia condition.”

Further screening of the man’s family showed his mother, sister and young niece also have the SCN5A gene variant.

The team tested the impact the mutation had on signaling proteins (fibroblast growth factor homologous factors – FHFs) that regulate sodium channels and found it blocked the proteins from binding and caused abnormal function consistent with human arrhythmia.

“It’s the first evidence of human arrhythmia based on gene variants that block FHFs,” said Peter Mohler, Director of the Davis Heart and Lung Research Institute, and corresponding author of the study. “This will need to be tested further as more regulatory proteins are associated with cardiac cell function.”

Other members of the Ohio State research team include: Crystal Kline, Nathaniel Murphy, Sara Adelman, Benjamin Johnson, Thomas Csepe, Dr. Ahmet Kilic, Paul Janssen, Vadim Fedorov, Dr. Raul Weiss and Thomas Hund, as well as Geoffrey Pitt and colleagues at Duke University Medical Center.

This research was supported by grants from the National Institutes of Health, the American Heart Association and the William D. and Jacquelyn L. Wells Fund for Cardiovascular Research.

Personalized Heart Models for Surgical Planning - System Can Convert MRI Scans into 3D-Printed, Physical Models in Hours

Researchers at MIT and Boston Children’s Hospital have developed a system that can take MRI scans of a patient’s heart and, in a matter of hours, convert them into a tangible, physical model that surgeons can use to plan surgery.

The models could provide a more intuitive way for surgeons to assess and prepare for the anatomical idiosyncrasies of individual

CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 20

OPPORTUNITY INPEDIATRIC CARDIOLOGY - MRI

CORPUS CHRISTI, TEXAS

Driscoll Children’s Hospital is advancing a comprehensive Heart Center to meet the healthcare needs of congenital heart patients in South Texas. The Heart Center is seeking a Pediatric Cardiologist with 6-12 months of additional training in MRI congenital heart imaging. Sub-specialty board eligible or board certification is required.

Driscoll Children’s Cardiac MRI program has completed nearly 200 pediatric/congenital heart scans on a new 1.5 Tesla Phillips Magnet over the last 19 months. Scans are supported by pediatric anesthesia. The successful candidate will spend about 30% of his/her time in direct patient care.

Pediatric Cardiology has been an integral part of Driscoll Children’s Hospital since 1962. The Hospital and the Heart Center are committed to bringing state-of-the-art technology and quality service to 31 counties in South Texas. The Heart Center incorporates all cardiology subspecialties including fetal, interventional catherization and surgical support. Driscoll Children’s Hospital is associated with three pediatric cardio-thoracic surgeons who deliver all aspects of surgical service including hybrid procedures.

Corpus Christi and the Rio Grande Valley offer a relaxed “island style” setting with miles of Gulf beaches and mild weather perfect for outdoor activities. South Texas offers world class hunting, fishing, sailing and wind surfing. Golf, cycling and tennis are enjoyed year round. The cost of living in south Texas is low, and there is no state income tax.

If you are interested in more information on this excellent opportunity, please contact:

John Brownlee, MDCardiology Medical DirectorJohn.Brownlee@dchstx.org

or Annette Shook

Physician RecruiterDriscoll Children’s Hospital Annette.Shook@dchstx.org

19th Annual Update on Pediatric and Congenital Cardiovascular DiseaseFeb. 24- 28, 2016Loews Royal Pacific Resort at Universal Orlando®

6300 Hollywood Way, Orlando, FL 32819

www.chop.edu/events/cardiology-2016#.VnMHWzYR6yI

CardiologyC2016

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patients. “Our collaborators are convinced that this will make a difference,” says Polina Golland, a professor of Electrical Engineering and Computer Science at MIT, who led the project. “The phrase I heard is that ‘surgeons see with their hands,’ that the perception is in the touch.”

This fall, seven cardiac surgeons at Boston Children’s Hospital will participate in a study intended to evaluate the models’ usefulness.

Golland and her colleagues described their new system at the International Conference on Medical Image Computing and Computer Assisted Intervention in October. Danielle Pace, an MIT graduate student in Electrical Engineering and Computer Science, is first author on the paper and spearheaded the development of the software that analyzes the MRI scans. Mehdi Moghari, a physicist at Boston Children’s Hospital, developed new procedures that increase the precision of MRI scans tenfold, and Andrew Powell, a cardiologist at the hospital, leads the project’s clinical work.

The work was funded by both Boston Children’s Hospital and by Harvard Catalyst, a consortium aimed at rapidly moving scientific innovation into the clinic.

MRI data consist of a series of cross sections of a three-dimensional object. Like a black-and-white photograph, each cross section has regions of dark and light, and the boundaries between those regions may indicate the edges of anatomical structures. Then again, they may not.

Determining the boundaries between distinct objects in an image is one of the central problems in computer vision, known as “image segmentation.” But general-purpose image-segmentation algorithms aren’t reliable enough to produce the very precise models that surgical planning requires.

Human Factors

Typically, the way to make an image-segmentation algorithm more precise is to augment it with a generic model of the object to be segmented. Human hearts, for instance, have chambers and blood vessels that are usually in roughly the same places relative to each other. That anatomical consistency could give a segmentation algorithm a way to weed out

improbable conclusions about object boundaries.

The problem with that approach is that many of the cardiac patients at Boston Children’s Hospital require surgery precisely because the anatomy of their hearts is irregular. Inferences from a generic model could obscure the very features that matter most to the surgeon.

In the past, researchers have produced printable models of the heart by manually indicating boundaries in MRI scans. But with the 200 or so cross-sections in one of Moghari’s high-precision scans, that process can take eight to 10 hours.

“They want to bring the kids in for scanning and spend probably a day or two doing planning of how exactly they’re going to operate,” Golland says. “If it takes another day just to process the images, it becomes unwieldy.”

Pace and Golland’s solution was to ask a human expert to identify boundaries in a few of the cross sections and allow algorithms to take over from there. Their strongest results came when they asked the expert to segment only a small patch —one-ninth of the total area — of each cross-section.

In that case, segmenting just 14 patches and letting the algorithm infer the rest yielded 90% agreement with expert segmentation of the entire collection of 200 cross-sections. Human segmentation of just three patches yielded 80% agreement.

“I think that if somebody told me that I could segment the whole heart from eight slices out of 200, I would not have believed them,” Golland says. “It was a surprise to us.”

Together, human segmentation of sample patches and the algorithmic generation of a digital, 3-D heart model takes about an hour. The 3-D-printing process takes a couple of hours more.

Prognosis

Currently, the algorithm examines patches of unsegmented cross-sections and looks for s imi lar features in the nearest segmented cross-sections. But Golland believes that its performance might be improved if it also examined patches that

CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 21

WHAT IS THE CHiP NETWORK? - The CHiP Network, the Congenital Heart Professionals Network, is designed to provide a single global list of all CHD-interested professionals in order to:• Connect pediatric and adult CHD-interested

professionals to events, conferences, research opportunities and employment

• Keep members up with the literature through the monthly Journal Watch service

• Increase education and provider awareness of new developments

• Bring the pediatric and adult congenital heart communities into closer contact

• Offer a communication tool for critical issues

WHO SHOULD PARTICIPATE? - The CHIP Network is all inclusive and is comprised of everyone who considers themselves a congenital heart professional or administrator, including: Pediatric cardiologists, ACHD cardiologists, RNs and APNs, Cardiac surgeons, Cardiac care associates, Trainees/fellows, Administrators, Psychologists and Mental health professionals, Researchers/scientists, Intensivists, Anesthetists, Industry representatives

OUR SUPPORTING PARTNERS:• Adult Congenital Heart Association• Asia Pacific Society for ACHD• Children’s Hospital of Philadelphia

Cardiology meeting• Cincinnati Children’s Hospital• Congenital Cardiology Today (official

publication of the CHiP Network)• Congenital Heart Surgeons Society• ISACHD• Japanese Society of ACHD• Johns Hopkins All Children’s Heart Institute• North American ACHD program• Paediatric Cardiac Society of South Africa• Pan Arab Congenital Heart Disease Association• PCICS• PICS• Specialty Review in Pediatric Cardiology• World Congress of Pediatric Cardiology and

Cardiac Surgery

JOIN US - Membership is Free!The CHiP Network management committee invites the participation of other organizations who want to communicate with all or some of the congenital heart professionals on this list. Please contact Dr. Gary Webb (gary.webb@cchmc.org) to ask that your organization’s or institution’s name be added to the list of partner organizations.

Register at: www.chipnetwork.org.

Join Us for Two Weeks to Save Children with Congenital Heart Defects!

We are looking for medical volunteers who can join us in our

missions to underdeveloped countries to perform pediatric cardiac

surgery and care while also training the local medical staff.

For more information and our 2014 mission schedule, please visit Babyheart.org2016

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ran obl iquely across several cross-sections. This and other variations on the algorithm are the subject of ongoing research.

The clinical study in the fall will involve MRIs from 10 patients who have already received treatment at Boston Children’s Hospital. Each of seven surgeons will be given data on all 10 patients — some, probably, more than once. That data will include the raw MRI scans and, on a randomized basis, either a physical model or a computerized 3-D model, based, again at random, on either human segmentations or algorithmic segmentations.

Using that data, the surgeons will draw up surgical plans, which will be compared with documentation of the interventions that were performed on each of the patients. The hope is that the study will shed light on whether 3-D-printed physical models can actually improve surgical outcomes.

What Powers the Pumping Heart?

Researchers at the Ted Rogers Centre for Heart Research have uncovered a treasure trove of proteins, which holds answers about how our heart pumps -- a phenomenon known as contractility.

Led by University of Toronto Physiology Professor Anthony Gramolini and his collaborator, Professor Thomas Kislinger in the Department of Medical Biophysics, the team used high-throughput methods to identify more than 500 membrane proteins on the surfaces of cardiac contractile cells, which are likely to have a critical role in normal heart function. The proteins may also play a part in heart failure and abnormal heartbeat patterns known as arrhythmias.

"In addition to providing a new understanding of what makes our hearts pump, these findings could also help researchers uncover new information about how heart disease affects the signal pathways in our hearts. That might pave the way to find ways to prevent or reverse those changes," says Gramolini.

During the study, the researchers found about 500 novel molecules that have been conserved throughout evolution. These

molecules haven't been studied in the heart and little is known about what they do in other tissues.

The group's research focused on a protein called transmembrane protein 65 (Tmem65). By studying human stem cells and zebrafish using cel l imaging and biochemical techniques, the researchers discovered that Tmem65 is involved in communication and electrical processes known as electrical coupling and calcium signaling. The team showed that Tmem65 regulates the connection point between adjacent cardiac contractile cells where it contributes to making the heart contract normally. R e m o v i n g t h e p r o t e i n h a d f a t a l consequences. The team also identified Tmem65 as the first critical tool for stem-cell researchers to monitor the maturation of cells in the heart's two main chambers, known as ventricles.

"These proteins are theoretically targetable for intervention as well as basic study. In this study, our focus was on Tmem65, but there are 555 proteins that we identified and showed that they are present throughout many species and are conserved throughout evolution-- at least in the mouse and the human -- in the heart's membrane-enriched contractile cells. Tmem65 was only the number-one candidate in our study, but theoretically, we have 554 other proteins to work through," says Gramolini.

T h e s t u d y , p u b l i s h e d i n N a t u r e Communications, also provides the first resource of healthy human and mouse heart-cell proteins that will help scientists develop a better understanding of the mechanisms involved in cardiac disease.

Gramolini says the findings are essential for understanding cardiac biology, and hopes they open the door for further study into health and disease in his lab and others.

"We need to figure out what all of these molecules are doing. My team and I hope our research sets the stage for other people to begin to pick up some of this work," says Gramolini. "These are molecules that haven't been studied, but must play some role in heart function. If a protein is conserved in evolution, generally it must have a critical function. We are very excited to look at the role of a number of these new proteins."

CONGENITAL CARDIOLOGY TODAY t www.CongenitalCardiologyToday.com t January 2016 22

Volunteer / Get Involved www.chimsupport.com

HOW WE OPERATE

The team involved at C.H.I.M.S. is largely a volunteering group of physicians nurses and technicians who are involved in caring for children with congenital heart disease.

The concept is straightforward. We are asking all interested catheter laboratories to register and donate surplus inventory which we will ship to help support CHD mission trips to developing countries.

CONGENITAL CARDIOLOGY TODAY

© 2016 by Congenital Cardiology Today (ISSN 1554-7787-print; ISSN 1554-0499-online). Published monthly. All rights reserved. www.CongenitalCardiologyToday.com

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Statements or opinions expressed in Congenital Cardiology Today reflect the views of the authors and sponsors, and are not necessarily the views of Congenital Cardiology Today.

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THE coMeEyIrAL HEART INTERVENTION

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Pediatrix Cardiology is seeking a highly experienced board certified interventional pediatric cardiologist to serve as the Medical Director for The Pediatric Heart Center in Long Beach, CA, part of the Memorial Healthcare System — Miller Children’s and Women’s Hospital.

This position will partner with an existing team of three board certified pediatric cardiologists who provide an array of services including interventional, fetal, adult congenital heart disease, non-invasive EP and non-invasive imaging in addition to general pediatric cardiology services. The candidate will work collaboratively with the hospital’s cardiac surgeon to develop and promote this premier program throughout Southern California.

Requirements:

• Leadership experience in a nationally recognized congenital heart center and/or large tertiary children’s hospital

• Experience in a state-of-the-art congenital cardiac cath lab • Ability to assist the PICU team in developing a new state-of-the-art CVICU• Knowledge of program development and fundraising • Strong interpersonal skills with key healthcare and practice leadership

Benefits:

• Located in desirable Southern California• 93-bed Level 3 NICU and 24 additional private NICU bays, the largest in LA and San Diego, staffed by

Pediatrix Medical Group/Mednax affiliated physicians• Working collaboratively with the hospital cardiac surgeon• Competitive salary and excellent benefits including health/dental/vision, 401(k), annual CME allowance,

employee stock purchase plan, professional liability insurance, and assistance with mandatory hospital credentialing and state licensing

Pediatrix Cardiology is an Equal Opportunity Employer

InterventionalPediatric

Cardiologist

Toapplyforthispositionorlearnaboutopportunitiesinotherlocations,pleasevisitwww.pediatrix.com/clinicalcareersorcontactJanetFriedman,PhysicianRelations

Specialist,PediatrixCardiology,at800.243.3839,ext.5589.

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he tools you need. For more than 3 decades, we've been buildingree solutions so clinicians can focus on what really matters:

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