c a r d i o l o g y g r a n d r o u n d s · obstruction of lv‐aortic pathway in the area of vsd...
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C A R D I O L O G Y G R A N D R O U N D S
Title:
Congenital Heart Disease: diagnostics past, present and future
Speaker: Charles C. Gornick, MD, FACC, FHRS Director of Cardiac Electrophysiology Minneapolis Heart Institute® at Abbott Northwestern Hospital
Kelly G. Han, MD Director of Congenital Cardiac Imaging Minneapolis Heart Institute® at Abbott Northwestern Hospital
Date: Monday, February 15, 2016
Time: 7:00 – 8:00 AM
Location: ANW Education Building, Watson Room OBJECTIVES At the completion of this activity, the participants should be able to:
1. Distinguish the changing congenital heart disease (CHD) population. 2. Associate that most morbidity and mortality in CHD is in adulthood. 3. Recognize the critical collaboration necessary between adult and pediatric cardiology to care for this
group of patients. 4. Illustrate the changing diagnostics in CHD. 5. Illustrate several examples of CHD presenting in adulthood. 6. Interpret the depth and breadth of the Midwest Adult Congenital Cardiac Program; its importance of
ongoing care for this unique group of patients; and illustrate its comprehensive program.
Physician: This activity has been planned and implemented in accordance with the accreditation requirements and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint providership of Allina Health and Minneapolis Heart Institute Foundation. Allina Health is accredited by the ACCME to provide continuing medical education for physicians.
Allina Health designates this live activity for a maximum of 1.0 AMA PRA Category 1 Credit(s)TM. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
(Continued on next page)
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C A R D I O L O G Y G R A N D R O U N D S
Title:
Congenital Heart Disease: diagnostics past, present and future
Speaker: Charles C. Gornick, MD, FACC, FHRS Director of Cardiac Electrophysiology Minneapolis Heart Institute® at Abbott Northwestern Hospital
Kelly G. Han, MD Director of Congenital Cardiac Imaging Minneapolis Heart Institute® at Abbott Northwestern Hospital
Date: Monday, February 15, 2016
Time: 7:00 – 8:00 AM
Location: ANW Education Building, Watson Room Nurse: This activity has been designed to meet the Minnesota Board of Nursing continuing education requirements for 1.2 hours of credit. However, the nurse is responsible for determining whether this activity meets the requirements for acceptable continuing education.
DISCLOSURE STATEMENTS Speaker(s): Dr. Gornick has declared that he does not have any conflicts of interest to disclose. Dr. Han has declared that she has
received grant/research support from Siemens Medical.
Planning Committee Dr. Michael Miedema, Dr. Scott Sharkey and Jolene Bell Makowesky have declared that they do not have any conflicts of interest associated with the planning of this activity. Dr. Robert Schwartz declared the following relationship - consultant: Boston Scientific.
PLEASE SAVE A COPY OF THIS FLIER AS YOUR CERTIFICATE OF ATTENDANCE
Signature: __________________________________________________________________________ My signature verifies that I have attended the above stated number of hours of the CME activity.
Allina Health - Learning & Development - 2925 Chicago Ave - MR 10701 - Minneapolis MN 55407
B. Kelly Han, MD
Charles Gornick, MD
Adult Congenital Heart Disease
The Changing PopulationPast – Present – Future
Minneapolis Heart Institute 2016
Disclosures
Research support Siemens Medical
Congenital Heart Disease Past - Present - Future
Transformation in CHD Medical treatment
Surgical intervention
Catheter based intervention
Cardiac diagnostics
CHD Historical perspective Cardiac Surgery
• First Aortopulmonary shunt 1944 (Blalock & Taussig)
• First open heart surgery for ASD closure 1952
• First Fontan palliation for tricuspid atresia 1968
• First Arterial Switch 1975 (Jatene)
• First Norwood for HLHS 1979
• First Fontan for HLHS 1983
CHD Historical PerspectiveCardiac Catheterization
• First described in 1929 (Forssmann, Germany)
• First diagnostic catheterization 1941
• First coronary balloon angioplasty 1977
• First pulmonary balloon Valvuloplasty 1982
• First aortic balloon Valvuloplasty 1985
• First ASD closure with an Amplatzer device (1995)
• Transcatheter Pulmonary valve (Melody) approved by FDA 2010
Cardiac Diagnostics
Catheterization Echocardiography MRI CT
1950
2016
CHD in Adults: The Changing Population
CHD survival 1960: 1% of d-TGA survived to adulthood 10% of TOF survived to adulthood No HLHS survived to adulthood
2013 95 % surgical survival for complex CHD 90% survival to adulthood 70% of single ventricle patients expected to survive to adulthood
Estimated that 66% of patients with CHD are now adults Most morbidity (and mortality) for CHD is now adulthood
32nd Bethesda Conference: “Care of the Adult with Congenital Heart Disease” JACC Vol 37,No5, 2001: 1161-98.Khairy et al. JACC 2010; 56:1149-1157.
CHD in Adults: The Changing Population
30% decrease in mortality Mortality shifted from
children to adults Median age of death was 57
years Complex CHD: median age of death changed
from 2 to 23 years during the study
Khairy et al. JACC 2010; 56:1149-1157.
Mortality in CHD 1987-2005
prevalence of congenital heart disease (CHD) in the European Union by age group. prevalence of congenital heart disease (CHD) in the European Union by age group.
Helmut Baumgartner Eur Heart J 2014;35:683-685
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2013. For permissions please email: [email protected]
Changing prevalence of congenital heart disease (CHD) in the European Union by age group.
CHD in Adults: The Changing Population
ACHD Rate of Intervention & Arrhythmia by Diagnosis
RE-OP ATRIAL vt
Ebstein’s anomaly 30-50% 33-60% >2% Single ventricle >25% 40-60% >5% Tetralogy of Fallot 26-50% 15-25% 10-15% TGA, atrial switch 15-27% 26-50% 7-9% TGA, arterial switch 12-20% <2% 1-2% CC-TGA 25-35% >30% >2% Truncus arteriosus 5-89% >25% >2% AVSD 19-26% 5-10% <2% Atrial septal defect <2% 16-28% <2%
PACES/HRS expert concensus statement on the recognition and management of arrhythmia in adult congenital heart disease. Khairy et al. May 2014. DOI: 10.1016/j.hrthm.2014.05.009
Most Common CHD diagnosis followed in Adulthood Tetralogy of Fallot
Aortic Coarctation
Transposition of the Great Arteries Atrial switch
Arterial Switch
Nikaidoh/Rastelli
Single Ventricle Heart Disease
TOF Anatomic Variants
Han, Leipsic, Lesser et al. Computed Tomography Imaging in Patients with Congenital Heart Disease, Part 2: Technical Recommendations. An Expert ConsensusDocument of the Society of Cardiovascular Computed Tomography (SCCT). Endorsed by the Society of Pediatric Radiology. JCCT 2015
Tetralogy of Fallot:Residual Hemodynamic Lesions
TOF with Pulmonary stenosis pulmonary stenosis, pulmonary insufficiency, RV
dilation and dysfunction, aortic root dilation
TOF with Pulmonary artery atresia RV‐PA conduit, branch pulmonary artery or distal
pulmonary artery stenosis, conduit insufficiency,
RV dilation and dysfunction, aortic root dilation
TOF with Absent pulmonary valve RV‐PA conduit stenosis or insufficiency,
airway abnormalities,
pulmonary artery dilation
Han, Leipsic, Lesser et al. Computed Tomography Imaging in Patients with Congenital Heart Disease, Part 2: Technical Recommendations. An Expert ConsensusDocument of the Society of Cardiovascular Computed Tomography (SCCT). Endorsed by the Society of Pediatric Radiology. JCCT 2015
Copyright © The American College of Cardiology. All rights reserved.
From: Long-Term Survival in Patients With Repair of Tetralogy of Fallot: 36-Year Follow-Up of 490 Survivors of the First Year After Surgical Repair
J Am Coll Cardiol. 1997;30(5):1374-1383. doi:10.1016/S0735-1097(97)00318-5Nollert et al. J Am Coll Cardiol. 1997;30(5):1374-1383Arrhythmia Burden in Adults With Surgically Repaired Tetralogy of Fallot: A Multi-Institutional Study. Khairy et al, (Circulation. 2010;122:868-875.)
Long-term survival after correction of TOF. All patients who died within the first year after correction were excluded for calculation of long-term survival. The curve shows two different phases that are distinct. The early, low risk phase lasts 25 years; thereafter, the risk increases significantly. Mortality risk (r) per year, as a linearized number, is calculated for each phase. Note the break in the y axis. OP = operation; p.o. = postoperatively.
Mortality< 25 post op: 0.25%/yr> 25 post op: 0.94%/yr
Risk for sudden death:
Older age at repair QRS duration > 180 msec Severe dilation of the right ventricle RVDD/m2 > 150 ml/m2 RVESV/m2 > 80-95 ml/m2
RV or LV dysfunction (lv fxn abn in 21% of adult tof) Arrhythmia Multiple surgeries Initial palliative shunting Level of pre operative polycythemia Ventriculotomy or RVOT patch
History of Intervention: d-TGA
1940 1950 1960 1970 1980 1990 2000 2010
Atrial septectomy (Blalock Hanlon)1950Atrial switch 1958 (Senning)
Atrial switch 1964 (Mustard)Balloon septostomy (Rashkind)1966
Rastelli procedure 1969Arterial switch operation(Jatene) 1975
Nikaidoh procedure 1984
Surgical Approach to d-TGA
Han, Leipsic, Lesser et al. Computed Tomography Imaging in Patients with Congenital Heart Disease, Part 2: Technical Recommendations. An Expert ConsensusDocument of the Society of Cardiovascular Computed Tomography (SCCT). Endorsed by the Society of Pediatric Radiology. JCCT 2015
D-TGA: Residual Hemodynamic Abnormalities
Atrial switch Systemic or pulmonary venous baffle narrowing or occlusion
RV failure (systemic ventricle)
Tricuspid regurgitation (quantify by stroke volume differences)
Arterial switch Neo‐pulmonary root or branch pulmonary artery stenosis, pulmonary insufficiency
Neo‐aortic root dilation, stenosis or insufficiency
Compromise of re‐implanted coronary arteries
Rastelli Obstruction of the RV‐PA conduit or branch pulmonary arteries, pulmonary insufficiency
Obstruction of LV‐Aortic pathway in the area of VSD patch
Nikaidoh Native RVOT or RVOT conduit stenosis or insufficiency
Right coronary artery compromise due to translocation of the aorta leftward
Procedure Residual Hemodynamic Abnormalities
Han, Leipsic, Lesser et al. Computed Tomography Imaging in Patients with Congenital Heart Disease, Part 2: Technical Recommendations. An Expert ConsensusDocument of the Society of Cardiovascular Computed Tomography (SCCT). Endorsed by the Society of Pediatric Radiology. JCCT 2015
Neonatal Arterial Switch for D-TGA
J Am Coll Cardiol. 2014;64(5):498-511. doi:10.1016/j.jacc.2014.06.1150
S/P Arterial Switch Operation
Quality of life and health status 11-15 years later same as normal children, better than atrial baffle
Earliest survivors now young adults Coronary event free survival 93% at one year 88% at 15 years
Hemodynamic sequelae: Coronary insufficiency Ventricular dysfunction Stenosis of great arterial anastomotic sites AI or PI Neo-aortic root dilation
Coronary Artery after ASO
Ostial Lesions Potential for Compression
Coronary anatomy and Relationship to RVOT and Sternum
Date of download: 2/3/2015
Copyright © The American College of Cardiology. All rights reserved.
From: Coarctation of the Aorta: Lifelong Surveillance Is Mandatory Following Surgical Repair
J Am Coll Cardiol. 2013;62(11):1020-1025. doi:10.1016/j.jacc.2013.06.016
Survival of the entire cohort compared with an age- and sex-matched Minnesota population (p < 0.001).
Aortic Coarctation
Long Term Follow-up of Simple Coarctation Repair Systemic Hypertension
Premature coronary artery disease
Aortic valve abnormalities
Aortic aneurysm (often with patch repair)
Re-coarctation
1/3 of patients in long-term follow-up had significant cardiovascular abnormalities
The most predictive factor of late complications was AGE AT OPERATION
Long-term follow-up of patients after coarctation of the aorta repair. Toro-Salazar et al, AJC Vol 89(5)March 1 2002
Long Term survival in Coarctation
Age at operation < 1 year old Long term survival 91%
Age at operation 5-10 years old Long term survival 87%
Age at operation > 10 years old Long term survival 60%
Long-term follow-up of patients after coarctation of the aorta repair. Toro-Salazar et al, AJC Vol 89(5)March 1 2002
Undiagnosed COA
13 year old:cerebral aneurysm ruptureIR couldn’t access aneurysm
25 year old: pneumoniaCXR rib notching
45 year oldPregnancy induced htnTreated for 20 years
Aneurysm Formation
Single Ventricle Intervention
1940 1950 1960 1970 1980 1990 2000 2010
AP shunt 1940s
Fontan for TA 1971Norwood Procedure for HLHS 1981
Fontan for HLHS 1983Lateral tunnel Fontan 1980s
Extracardiac Fontan 1990s
Glenn shunt 1950s
Single Ventricle Intervention
Passive systemic venous flow to the lungs
Unobstructed systemic arterial flow from single V Stage 1: BT shunt or Sano
Norwood or DKS
Stage 2: Glenn (TCPC)
Stage 3: Fontan
Classic Fontan Bjork Kawashima Atrio-pulmonary
Lateral tunnel
Extracardiac
Han, Leipsic, Lesser et al. Computed Tomography Imaging in Patients with Congenital Heart Disease, Part 2: Technical Recommendations. An Expert ConsensusDocument of the Society of Cardiovascular Computed Tomography (SCCT). Endorsed by the Society of Pediatric Radiology. JCCT 2015
Date of download: 2/3/2015
Copyright © The American College of Cardiology. All rights reserved.
From: Predictors of Survival After Single-Ventricle Palliation: The Impact of Right Ventricular Dominance
J Am Coll Cardiol. 2012;59(13):1178-1185. doi:10.1016/j.jacc.2011.11.049
Kaplan-Meier Survival Curves
Patients were separated into 2 groups according to era of birth. Era 1 consisted of patients born from 1990 to 1999; era 2 consisted of patients born from 2000 to 2008. HLHS = patients with hypoplastic left heart syndrome; LV = patients with a dominant left ventricle; RV no HLHS = patients with a dominant right ventricle but not a diagnosis of HLHS.
Figure Legend:
Long-Term Survival, Modes of Death, and Predictors of Mortality in Patients with Fontan Surgery. Khairy et al, Circulation 2008;117:85-92
Late Fontan Survival
261 patients Born 1985 or earlier Median age at Fontan
7.9 Freedom from death or
transplant 5 yrs: 93.7% 10 yrs: 89.9% 15 yrs: 87.3% 20 yrs: 82.6%
Common Residual Hemodynamic Lesions after the Fontan procedure SVC to PA, IVC to PA or branch pulmonary artery narrowing Atrial dilation (most commonly in older atriopulmonary Fontan) Systemic venous occlusion, collaterals or aortic bronchial collaterals Clot within the Fontan system, pulmonary embolism Ventricular to aortic obstruction if aorta from an outlet chamber Ventricular dysfunction Valvular regurgitation (regurgitation can’t be determined from CT) Recurrent arch obstruction Fenestration or leak in lateral tunnel Fontan Hepatic disease Plastic bronchitis
Diagnostic in CHD
CHD patients need repeat diagnostic testing throughout life
Diagnostic Risk is considered cumulative Vascular access
Radiation
Anesthesia
Risk of Diagnostics in CHD
+/- PIV
Anesthesia < 8 yo No radiation
Gadolinium (NSF & neuro)
Resolution vs signal in small patients
45-75 minutes for complete study
PIV
Limited or no anesthesia
Radiation Iodinated Contrast
Resolution independent of ptsize (< 1 mm, isotropic)
< 1 second, one breath hold
Central Vascular Access
Anesthesia
Radiation
Iodinated contrast
Long procedural time
Echo
Diagnostic CatheterizationCardiac CTA CMRI
Vascular Access Risk in Pediatric Patients
Children’s Hospital Philadelphia 2005-2010• 3254 patients underwent 5715 cath procedures
• Acute occlusive arterial injury 4.3% of 721 patients < 6 months of age – 18%
of 118 patients < 6 months, > 4Fr – 36%
Prevalence and Risk Factors for Acute Occlusive Arterial Injury Following Pediatric Cardiac Catheterization in the Modern Era at a Large Volume Center. Andrew C. Glantz et al, ACC 2012
Anesthesia risk in CHD is Twofold• Procedural Risk of Adverse Event:
• MRI with Anesthesia, odds ratio of adverse event 3.9• Anesthesia risk higher when performed outside of the OR and in
hospitalized patients• Cardiac arrest with anesthesia occurs most commonly in CHD ASA III-
V (single ventricle, unrepaired and palliated CHD)
• Long term risk of adverse Neurodevelopmental Outcome• Repeat or prolonged anesthesia may increase risk of learning disability,
developmental disability, and behavioral problems• Most relevant in period of rapid brain growth < age 2
Risk factors for adverse events during cardiovascular magnetic resonance in congenital heart disease (JCMR 2007;9(5):793-8)Anesthesia-related cardiac arrest in children with heart disease: data from the Pediatric Perioperative Cardiac Arrest registry.(Anesth Analg 2010 May 1;110 (5): 1376-82)
• Anesthesia exposure < 2 yo independent risk factor for development of learning disability
Flick et al. Pediatrics. 2011:128 (5): e1053-61.
• Children exposed to anesthesia < 3 years were twice as likely to have a developmental disorder as non exposed children
DiMaggio. Journal of Neurosurgical Anesthesiology 2009;21:286-291
Children exposed to anesthesia < 3 had higher risk of deficits in language and abstract reasoning at age 10 than unexposed children
Ing et al. Pediatrics 2012 Sep;130(3):e476-85
RR of developmental and behavioral disorders anesthesia< 3 yo
DiMaggio et al AnesthAnalg 2011;113(5):1143-51
RR 1.1 for one anesthetic RR 2.9 for two anesthetic RR 4.0 for > anesthetic
Prospective studies underway (GAS,PANDA)
Diagnostic Risk in CHD - AnesthesiaNeurodevelopmental Outcome
Diagnostic Risk in CHD - Radiation Exposure
• The average CHD patient : 20 mSv of radiation exposure by adolescence (unadjusted for age/size)
• Radiation exposure primarily from cath procedures• Electrophysiology studies – 17-25 mSv (unadjusted)
• 22% of studies exceeded dose to cause skin injury
• Pediatric Cardiac Catheterization• Median effective dose 6.4 mSv (0.5 – 53 mSv)• Age adjusted mSv (13 mSv for neonate, 8 mSv for a 1 year old )• Catheterization exposure 10-15 fold higher than CT exposure
using modern equipment Rosenthal et al. Am J Cardiol 1998;82:451-458 Akash et al. Circulation 2010;122:A18535Andreassi et al. , Circulation 2009;120:1847-1849Beels et al. Circulation 2009:120:1903-1909Hvlacek et al. Pediatric Cardiology 2012. DOI: 10.1007/s00246-012-0486-2Han & Lesser et al. JTCVS , in press
Background Radiation 3-6 mSv/year0.24 mSv/Month
CXR (2 view) 0.10 mSvNew York – Hong Kong flight 0.09 mSv
Cumulative Radiation Exposure < 6 yo
ASD 6 (4, 30) 0.09 (0.02, 6.9) 0.19 (0.07,29.3)
VSD 9 (4, 79) 0.20 (0.01, 12.01) 0.36 (0.06, 23.6)
AVCD 11 (4, 111) 0.26 (0.02, 29.45) 0.49 (0.07, 30.4)
TOF 10 (5, 63) 0.27 (0.02, 22.59) 0.55 (0.08, 33.3)
ASO 18 (10, 47) 0.29 (0.05, 3.56) 0.60 (0.18, 8.2)
TX 117 (50, 247) 42.5 (8.34, 396) 63.79 (9.93, 190)
Single V 63 (19, 271) 20.08 (0.66, 182) 28.93 (0.70, 113)
Total 17 (4, 158) 1.34 (0.03, 83.24) 2.67 (0.08, 76.93)
Diagnosis # exams Annual mSv Cumulative
Johnson J N et al. Circulation. 2014;130:161-167
Background Radiation 3-6 mSv/year0.24 mSv/Month
CXR (2 view) 0.10 mSvNew York – Hong Kong flight 0.09 mSv
Cumulative Radiation Exposure and Cancer Risk Estimation in Children With Heart Disease
The lifetime
Johnson J N et al. Circulation. 2014;130:161-167
Copyright © American Heart Association, Inc. All rights reserved.
Chest CT 4.7 mSvGated Cardiac Cta 18 MsVDiagnostic cath 9 mSv
Background Radiation 3-6 mSv/year0.24 mSv/Month
CXR (2 view) 0.10 mSvNew York – Hong Kong flight 0.09 mSv
Pediatric CCTA Radiation Dose Estimates by Scan Mode and Tube Voltage Scan Mode kVp* DLP† mSv‡ Age Adjusted
mSv
High Pitch (n=19) (prospective ECG)
17 (3‐122) 0.24 (0.04‐1.7) 0.34 (0.08‐1.7)
80 (n=13) 15 (3‐19) 0.21 (0.04‐1.7) 0.26 (0.08‐0.39)
100 (n=5) 60 (33‐69) 0.89 (0.46‐0.97) 0.89 (0.46‐0.97)
120 (n=1) 122 1.7 1.7
Sequential (n=35) (prospective ECG)
62 (6‐471) 0.86 (0.2‐6.59) 1.05 (0.2‐6.59)
80 (n=29) 57 (13‐111) .079 (0.18‐1.54) 0.99 (0.34‐1.98)
100 (n=5) 145 (48‐170) 2.03 (0.67‐2.38) 2.03 (0.67‐2.38)
120 (n=1) 471 6.6 6.6
Spiral (n=22) (retrospective ECG)
122 (32‐344) 1.7 (0.6‐4.8) 1.8 (0.8‐4.8)
80 (n=17) 106 (32‐212) 1.48 (0.6 – 3.74) 1.64 (0.77‐3.74)
100 (n=5) 306 (263‐344) 4.28 (3.44‐4.68) 4.28 (3.44‐4.68)
*kVp = Tube voltage in kilovolt peak, † DLP = dose length product in mGy.cm, ‡ mSv= miliSievert. The median and range of the DLP, mSv and age adjusted mSv is listed for each scan mode and is subdivided by tube voltage.
Han & Lesser et all. Safety and accuracy of dual-source coronary computed tomography angiography in the pediatric population. JCCT Jul-Aug 2012
Background Radiation 3-6 mSv/year0.24 mSv/Month
CXR (2 view) 0.10 mSvNew York – Hong Kong flight 0.09 mSv
Symptomatic Coronary Anomaly
Anomalous RCA, 2 year old
(syncope)
Ostial narrowing
Intraarterial course
Acute angulation takeoff
Anomalous RCA
12 year old
Acute angulation takeoff
Sequential scan, DLP 32High pitch scan, DLP 12
Anomalous RCA, 6 month old (6 kg)
2 “SIDS like” episodes
Intraarterial course
Acute angulation takeoff
Right dominant
Sequential scan, DLP 35 HR 98 bpm
CTA: Coronary Lesions < 5 years
3 yo - Nikaidoh 3 yo – h/o Kawasaki4 yo – h/o Kawasaki & CP
3 month old (3.5 kg, s/p Arterial Switch Operation)
Diagnostic Risk in CHD
Advanced Diagnostics Venous/Arterial anomalies
Critically ill patients
Single ventricle patients prior to second stage palliation
Radiation Exposure – Neonatal Imaging
#pts DLP 0.014 Age adj mSv length
64 slice
MDCTA
29 66 (29‐272) 1.1 (0.4‐3.8) 2.6 (1.1‐10.6) 15.2 cm
Dual source
MDCTA
Variable pitch
(2.25‐3.0)
14 28 (8‐50) 0.39 (0.1‐0.6) 1.05 (0.3‐ 1.9) 16.2 cm
Dual source
MDCTA
High Pitch
(3.4)
18 5.4 (3‐12) 0.07 (.04‐.11) 0.2 (0.12‐0.17) 15.8 cm
10 fold dose reduction with highest pitch scans
Han & Lesser et all. Accuracy and Safety of High Pitch Computed tomography Imaging in Young Children with Congenital Heart Disease. Am J Cardiol 2011
64 slice vs High Pitch DS CTA (1st generation)patients < 2 yo
Neonatal Unsedated, Free breathing CTA
Han and Lesser et all. Non-sedated, free breathing cardiac CT for evaluation of complex congenital heart disease in neonates. JCCT 2013.
N=19 Median (lower quartile, upper quartile)
Angiogram DLP 8 (5,9)
Angiogram effective mAs 99.5 (66, 110)
Angiogram length (cm) 10 (9.4, 12.1)
Angiogram CTDIvol 0.47 (0.31, 0.5)
Total procedural DLP 11 (10, 14)
mSv unadjusted (procedural) 0.15 (0.14, 0.2)
mSv age/size adjusted (procedural) 0.86 (0.78, 1.1)
DLP= dose length product, CTDIvol= CT dose index volume, mSv= milliSievert
Background Radiation 3-6 mSv/year0.24 mSv/Month
CXR (2 view) 0.10 mSvNew York – Hong Kong 0.09 mSv
Loeys Dietz, PDA
Neonatal CT
IAA type B
TOFL-TGA, IAA
IAA type C
Critically Ill Patients
3 day old (2.5 kg, HR 150) s/p TAPVR repair DOL #1 Open mediastinum Scan - 0.25 seconds 3 minutes procedural time No change in Vent mgmt
(PEEP 8, right pneumo) DLP 6
0.08 mSv (chest conversion .014)
Critically Ill patients:
AO
PA
2 Month old (3.5 kg, HR 160)s/p Truncus Repair, Pseudoaneurysm from truncal root
Neonate for venous anatomy
HLHS + Scimitar
HLHS + TAPVR
CT Provides Better Information: Extra-Cardiac Anomalies
CTA in Infants and Neonates (MHI/CHCM) 62 scans in neonates (< 30 days of age) Median DLP 14 (11, 18)
Median mSv 0.19
Free breathing, no sedation
178 Scans in infants (< 1 year of age) Median DLP 17 (13, 24)
Median mSv 0.23 Background Radiation 3-6 mSv/year
0.24 mSv/MonthCXR (2 view) 0.10 mSvNew York – Hong Kong flight 0.09 mSv
Diagnostic vs interventional catheterization in CHD
CT vs Diagnostic Cath in CHD patients < 1 Year
Han, Lesser et al. Cardiovascular imaging trends in congenital heart disease: A single center experience. JCCT 2013. (6):361-6
Evaluation of Single Ventricle Heart Disease
Stage 1
“Norwood”Aorto-pulmonary Shunt/ Sano
Stage 2
“Glenn”
SVC- PA connection
Stage 3
“Fontan”
IVC – PA connection
CathCath
?
Average single Ventricle Patient Diagnostic Radiation exposure 20 mSv/year
CT in Single Ventricle CHD: Pre-Glenn Cath vs CT
Cardiac CTA
(n=16)
Diagnostic Cardiac Catheterization(n=16)
p Value
Gender (male/female) (8/8) (10/6) 0.4760Age at time of imaging procedure, mo 3.7 (2.6, 5.4) 3.6 (3.3, 5.0) 0.6144Weight, kg 5.3 (4.5, 6.8) 5.6 (5.3, 6.4) 0.7650Body surface area, m2 0.29 (0.25, 0.32) 0.30 (0.27, 0.32) 0.6409Time between imaging procedure and Glenn surgery, days 37.5 (10.5, 71.5) 36.5 (23.0, 51.0) (n=14) 0.5786Primary Diagnosis
HLHSUnbalanced AV canal
73
8
DILVDORVTAHeterotaxy
1212
2141
Han & Lesser et all. Selective use of cardiac computed tomography angiography: An alternative diagnostic modality before second-stage single ventricle palliation. JTCVS 2013.
Patient Characteristics
Pre- Glenn CT vs Cath: Procedural Risk
Cardiac CTA(n=16)
Diagnostic Cardiac Catheterization (n=16)
p Value
Anesthesia & Procedural timeTotal anesthesia/sedation time, min Procedural time (min)Image Acquisition time
55 (45, 70.5)8 0.25 seconds
166 (119.5, 197)81.5
<0.0001<0.0001
General anesthesia (N /total) 1/16 16 /16 <0.0001
Vascular AccessPeripheral IV (22g, 24 g) 16/16 ‐‐
Central venous 0/16 16/16 ‐‐
Central arterial 0/16 12/16 ‐‐
Radiation DoseRadiation dose adjusted for age/size, mSv* 1.12 (0.96, 1.31) 13.95 (8.86‐17.93) <0.0001
Number of Angiograms 1 (1,1) 7.5 (5.0, 10) <0.0001
Minutes of fluoroscopy N/A 19.5 (16.5, 29.0) ‐‐
ContrastVolume contrast administered in ml/kg 2.06 (1.98, 2.20) 4.82 (3.78, 5.90) <0.0001
Adverse Events 0 6 <0.0001
CTA visualization of Shunt, Sano
CT visualization of Systemic Venous Anatomy
1E 1F
SVC
RPA
S/p Fontan
Visualization of Fenestration
Radiation Exposure in single ventricle CHD
Single Ventricle Cumulative Radiation exposure 38 SV patients followed for 33 months
Median cumulative exposure : 25.7 mSv (cath 78%)
Downing et al. Cumulative medical radiation exposure throughout staged palliation of single ventricle congenital heart disease. Pediatr Cardiol. 2015 Jan;36(1):190-5
Johnson et al. Cumulative radiation exposure and cancer risk estimation in children with heart disease. Circulation. 2014 Jul 8;130(2):161-7. 2014 Jul 8;130(2):161-7
Glatz et al. Cumulative exposure to medical radiation for children requiring surgery for congenital heart disease. J Pediatr. 2014 Apr;164(4):789-794
CT evaluation of Single V CHD
79/132 underwent intervention – 4 minor discrepancies
N DLP mSv
Total 132 24 0.33 anatomy
Neonate 20 18 0.25 anatomy
Post Norwood 52 16 0.22 anatomy
Post Glenn 15 33 0.46 anatomy
Post Fontan 45 158 2.2 Anatomy + function
ACC 2016
Ventricular Function
ECG pulsed modulation Full radiation for a portion of the cardiac cycle
20% radiation for the remainder of the cardiac cycle
Narrow acquisition window
Low kVp
Higher noise tolerated – reconstructed in thicker slice for analysis
70 kVp functional imaging in CHD
Age <10(n=19)
Age 10-18(n=17)
Age >18(n=36)
BMI 15.6 (14.5, 18.0) 18.1 (16.2, 19.4) 24.2 (21.4, 27.5)
Weight (kg) 11.3 (6.6, 20.0) 43.5 (33.4, 56.2) 72.1 (64.2, 83.0)
Functional Scan DLP 35 (27, 47) 65 (59, 97) 95 (83.5, 106.5)
Functional Scan CTDIvol 2.2 (2.0, 3.6) 4.2 (3.9, 6.0) 5.6 (4.8, 5.9)
Functional mSv (Unadjusted) 0.49 (0.38, 0.66) 0.91 (0.83, 1.36) 1.3 (1.2, 1.5)
Functional mSv (Age-Adjusted) 0.85 (0.66, 1.05) 0.91 (0.83, 1.36) 1.3 (1.2, 1.5)
72 patients
Median age 19.5 years
Median mSv for functional scan < 1 mSv
JCCT 2016 Han, A Lesser et all
86 kgScan dlp 98Effective mas 102Scan range 15.2 cmCtdi vol 5.68
1.5 mm, systole 8 mm systole
1.5 mm diastole 8 mm diastole
MACC: Midwest Adult Congenital Center
Collaboration between CHC, CHCM,MHI
400 clinic visits in 2015
Drs Han, Burton, Carter
Drs Gornick, Lesser, Chu, Samara, Sorajja
Drs Farivar and Flavin
Clinic visits every Monday Jan 1st
ACHD director needed
2016: comprehensive ACHD center certification
Clinical Cases
59 yo white male
Seen North Memorial
Surgeries at Mayo 1960, 1974 Tetralogy of Fallot repair
No cardiology fu since late 1970’s
9/2015 presents with atrial flutter
Subsequent evaluation with evidence of right heart enlargement and to and fro murmur on exam
CT
No coronary lesions
RV EF 47%
RVEDV 204 ml/m2
RVESV 108 ml/m2
PI: 30%
LV EF normal
No significant Ao root dilation
The proportion of CHD patients in contact with the healthcare system by age
Andrew S. Mackie et al. Circulation. 2009;120:302-309
Copyright © American Heart Association, Inc. All rights reserved.
Sees Cardiologist
Sees Primary Care
By age 15 50% of patients did not receive cardiology care
PVR after TOF
Not shown to change VT or mortality Herrild et al. circ 2009. 111:445-51
No agreed upon criteria for valve replacement RVEDV > 150 – 160 ml/m2 RVESV > 80-95 ml/m2
Meta-analysis of 48 studies (3118 pts) Decreased pulmonary regurgitation Improved RV indexed volumes but not EF (unless “corrected” EF Improved LV fxn by larger LVEDV Decreased QRS duration Improved symptoms Largest RVs responded best but not as much decrease in symptoms Largest reduction in PI had best RV response
Pulmonary valve replacement after operative repair of TOF.Cavalcanti et al. JACC vol 62, no 23, 2013.
Surgical Pulmonary Valve replacement
945 RVOT operations (Toronto) Median followup 8.3 yrs (birth – 31 yrs) Median age 6 yrs
25% underwent at least 2 Freedom from RVOT valve replacement
5 yrs 82% 10 years 58% 15 years 41%
Risk factors 13-65 yo Younger age at initial valve placement Smaller valve size Need for endovascular stents Increased # of previous valve replacements
Indications Stenosis PI if increased RVEDV
Caldarone et al. JTCVS 2000.Volume 120 (6) 1022–1031.
45 yo Spanish speaking female
Htn history for 16-17 years on diuretic and ACE
Sharp chest pain intermittent in past
More recently chest heaviness and some difficulty breathing with exertion
HCMC referral to MACC
Bicuspid aortic valve
40-50 mmHg gradient between arms and legs bp RUE BP 142/92
Rib notching noted on chest xray
35 yo white male
History as child of coarc repair and balloon valvuloplasty for aortic stenosis bicuspid valve
Seen by Dr Sutton Children’s Heart Clinic 2004
CMR 2005 AAO 3.6 x 3.6 cm
Lost to FU for 10 years
No symptoms reported, cuts wood for living
Echo done demonstrating mild as/mod ai
Sent for advanced imaging to quantify AI
MRI: LV EF 41%
LVDD 132 ml/m2
30% AI
CT and MRI Pseudoaneurysm with
mural thrombus
6 x 5.8 x 5 cm
Bicuspid AO valve with calcification
Dr. Mudy 21 mm St Judes with 22 mm hemashield graft
53 yo white female
Followed by Dr Gornick since retirement of Dr Gobel Hx of Ragib syndrome (persistent left svc connected
to la along with asd. As child age 12 underwent repair by Dr Aldo
Castenada consisting of tunneling of left svc to RA and closure asd
Subsequent development sinus and av nodal dysfunction and atrial arrhythmias
Clinically did well over the years, annual to bi annual echoes including TEE at one point in time
Occasional cardioversion but for the most part stable on propafenone therapy
C.U. Hx continued(2)
Scheduled ct for structure and volumes but not done due to insurance issues 2013
At fu in 2014 noted to have new evidence of right sided venous distention on exam and gradual reduction in exercise tolerance confirmed by CPST 18 ml/kg/min
Ct scan for structure and function obtained Note complexity 1)hemodynamic problems
2)arrhythmia issues 3) pacemaker leads with chronic failure
C.U. Hx continued (3)
3 pronged plan Arrhythmia ablation by Dr Melby and Dr
Gornick. Typical atrial flutter and 2 other scar mediated flutters on right side. Note access to la via venosus defect
Surgery by Dr Flavin and Moga with venosusdefect closed and tricuspid ring placed, old pacing leads transected and left in svc
Dr Gornick removed transected leads and placed new dual chamber pacing system in post op period
AO
RV