advancesin%achd · 2015-09-24 · marcg cribbs,md%% director,alabama%adult%congenitalheart%program%...
TRANSCRIPT
Marc G Cribbs, MD Director, Alabama Adult Congenital Heart Program 2015 Southeastern Pediatric Cardiology Society
September 26th, 2015
Advances in ACHD a narrative of thinking outside the box
§ I have no relevant financial relationships with any commercial interests to disclose.
“Congenital affections of the heart are of only limited clinical interest,
as in a large proportion of cases the anomaly is not compatible with life,
and in the others nothing can be done to remedy the defect
or even to relieve the symptoms.” Sir William Osler: The Principles and Practice of Medicine. 1894
§ 34 year old male with tetralogy of Fallot
§ Status post: ü Right Blalock Taussig shunt (age 6 mo) ü Left BT shunt (3 yrs) ü Transannular patch & VSD closure (6 yrs)
§ Presents with NYHA class III-‐IV
symptoms
Case 1: AH
§ Severe pulmonary insufficiency & RV dilation (age 11 yrs) § Surgery recommended but… § …lost to follow-‐up.
§ AICD for primary prevention (age 28) § LVEF 25% § Lost to follow-‐up
§ Past Medical History also includes…
Case 1: additional history
BMI = 41
§ Referred for heart transplant § Quickly deemed ineligible due to:
§ Morbid obesity § Continued tobacco abuse (1 ppd since 12 yrs of age) § Medical non-‐compliance
§ Referred to the ACHD program for further management
Case 1: AH
§ Growing number of Adults with Congenital Heart Disease
§ Lost to follow-‐up: an ongoing problem
§ Care of the ACHD patient § Alabama Adult Congenital Heart Program Alabama Adult Congenital Heart Program
Advances in ACHD: a narrative of thinking outside the box
The growing number of Adults with Congenital Heart Disease
Incidence of CHD
Expanding Population of Adolescents & Adults with CHD
Advanced Post-‐Op Care Early Complete
Repair Diagnostic Advances
Increased Early Survival Improved Peri-‐Op
Survival
Increased Mid & Late-‐Term Survival
Improved Surgical Techniques
0.4—1.2% of all live births § #1 congenital malformation § Blamed in 20% of miscarriages
1980 –
1970 –
1960 –
1940 –
0 10 20 30 40
Percent Survival to 18 Years Old
Decade Born with
CHD
50 60 70 80 90 100
20
JACC. 2001;37(5):1170-‐1175
1990
Circulation 2010.122:2264-‐2272
40
80
75
90+
Survival to 18 years of age with CHD
% Survival
Survival is expected – from the crib to old age
JACC. 2006; 47(4): 701-‐707
Percentage of Adults with Congenital Heart Disease
PEDIATRIC ADULT
2010
1965
2000
30%
65% 50%
70%
35% 50%
20,000 NEW ACHD patients per year
Circulation. 2015; 131: 1884-‐1931
ASD
AVSD
BAV
Coarctation
Chest pain
ccTGA
dTGA s/p Mustard
Ebstein
Fontan
Marfan, CTD
Pulm Stenosis
Tetralogy
Truncus
VSD
Alabama Adult Congenital Heart Program distribution of patients
Tetralogy of Fallot
dTGA
Fontan
Alabama ACHD Program
1560 pt visits (2014-‐2015)
…60% of patients are Lost to Follow-‐up
by age 18.
Circulation. 2009;120:302-‐309
“I have a history of tetralogy of Fallot repair and didn’t think I’d need another surgery…”
§ Misconception the disease has been “cured”
§ Lack of cardiac symptoms
§ Lack of appropriate healthcare access
Lost to follow-‐up: why does it happen?
Circulation. 2010; 122:868-‐875
Tachy-‐Arrhythmia by age in Tetralogy of Fallot
Step 1 Step 2
Step 4 Step 3
Medical School
Fellowship #2: Adult Congenital Cardiology
Fellowship #1: Adult Cardiology Pediatric Cardiology
Residency: Medicine/Pediatrics Internal Medicine Pediatrics
4 years 7-‐8 years 11-‐12 years 13-‐14 years
Number of years after college
Training: Adult Congenital Cardiology
Cost: ~$10,000.00
Lost to Follow-‐up why does it matter?
EHJ. 2010.31:1220-‐29.
Non Cardiac (23%) M
ultip
le (3
.6%)
Arrhythmia (21.9%)
Heart Failure (24.5%)
Vascular (14.3%)
Many of these can be prevented IF
UNinterrupted care is maintained
Therefore, exploring innovative ways to improve ACHD care is necessary
Care of the ACHD patient
Case 1: AH
Back to …
§ 32 yo with tetralogy of Fallot status post:
ü Right BT shunt ü Left BT shunt ü Trans-‐annular patch, VSD closure
§ History of: ü Morbid Obesity Tobacco Abuse ü Non-‐compliance AICD ü NYHA class III-‐IV
§ Referred to the ACHD Program for further management
Right Ventricle: RV EDVi 649ml (250ml/m2); RVEF 17% Left Ventricle: LV EDV 299ml, LV ESV 236ml; LVEF 21% Cardiac CT
Dilated MPA to ~32mm Proximal narrowing of the RPA and LPA Cardiac CT
§ Case management conference § Surgical pulmonary valve replacement?
Case 1: Decision making
§ Length of Stay > 7 days: 19.7%
§ Major Adverse Event (MAE): 20.5% § Re-‐exploration Deep sternal infection Stroke § Renal failure Ventilation > 48 hours
§ Hospital Mortality: 3.49%
Ann Thorac Surg. 2013; 95: 1377-‐82
§ AH’s Past Medical History: § Male § NYHA functional class III – IV § Severe restrictive lung disease
§ Tobacco abuse
§ Agreed patient was High Risk for surgical PVR
§ Continue medical vs. “Other” management? § Bilateral branch PA
percutaneous PVR pursued
Case 1: Decision making
Disclaimer: The procedure presented here is not included in the Medtronic Melody Valve IFU (Information for Use). IRB approval and appropriate consent was obtained.
Pre-‐procedure angiography
Right Pulmonary Artery Left Pulmonary Artery
Post-‐procedure angiography
Right Pulmonary Artery Left Pulmonary Artery
§ No pulmonary insufficiency
§ NYHA functional class II-‐III
§ Tobacco use: § Still smoking ½ ppd
Case 1: Follow-‐up visit
Heart Failure (24.5%)
On to Case 2
Case 2: TH
§ 45 year old male with dTGA: ü S/P Mustard procedure ü Recent Sinus Node Dysfunction and
Ventricular Tachycardia s/p pacemaker/AICD
§ Presents with progressive dyspnea
despite maximal medical therapy
Mustard: Despite excellent early outcomes…
…there remains a concerning incidence of long-‐term complications
§ Sinus node dysfunction (>50%) § Arrhythmia (IART; ~30%) § Baffle leaks (25%) § Baffle obstruction (20%) § Tricuspid Insufficiency § Right ventricular failure
§ Primary long-‐term concern
§ Sudden death (6 – 17%) § Tachyarrhythmia and CHF § During exercise (81%)
Treatment of Systemic (RV) dysfunction
§ Conventional heart failure meds § Do they help?
§ Tricuspid valve replacement § Outcomes dependent upon good RV function
§ Heart transplant
§ Cardiac Resynchronization Therapy (CRT) § Could it help the Systemic RV?
Cardiac MRI PRIOR to AICD
Systemic (Right) Ventricle EF <20% Severe tricuspid insufficiency
§ Patient not interested in cardiac transplant
§ Case management conference § Tricuspid valve replacement felt to be high risk for
questionable reward § Continue medical vs. “Other” management?
Case 2: Decision making
§ 8 patients § Age 6.9 to 29.2 years
§ Congenital heart disease with a Systemic RV
§ Mustard or Senning: 4 patients
§ QRS duration 161 + 21 msec
JACC. 2004;44:1927 – 31 § QRS interval:
§ Decreased from 161 to 116msec (p<0.005)
§ RVEF%: § Increased from 41.5 to 45.5 (p<0.04)
§ NYHA functional class: § Improved from II to I (p<0.008)
CRT in Congenital Heart Disease
Heart Rhythm. 2014;11:e102–e165
§ Continue medical vs. “Other” management? § Cardiac Resynchronization Therapy pursued
Case 2: Decision making
Transvenous leads to the left atrium and left ventricle Surgically placed leads to the systemic right ventricle
§ ECG: QRS 166 msec § Previously, 188 msec
§ Systemic RVEF% unchanged
§ Improved shortness of breath and a 15 pound weight loss
§ NYHA functional class II-‐III
Case 2: Follow-‐up visit
Alabama Adult Congenital Heart Program And lastly…
Alabama adult congenital patients
Estimated 9,000 adults with congenital heart disease Where are they now?
60% of patients are Lost to follow-‐up…
§ Team: § ACHD Physicians § ACHD RNs § ACHD Imaging (echo, CT, MRI) § Interventional Cardiology § Electrophysiology § Heart Failure/Transplant § CV Surgery § Maternal Fetal Medicine
MFM & ACHD multidisciplinary team
Cardiology physicians Maternal Fetal Medicine physicians
OB Anesthesia physicians OB Anesthesia nurses Labor & Delivery nurses
§ Meet each month
§ Discuss each patient who is currently pregnant
§ Review history, recent imaging, and exam
§ Formulate optimal plan of care and delivery
Alabama Adult Congenital Heart Program
The Kirklin Clinic
Birmingham
Huntsville
Montgomery
Mobile
Alabama ACHD clinic locations
§ Growing number of Adults with Congenital Heart Disease
§ Lost to follow-‐up: an ongoing problem
§ Care of the ACHD patient § Alabama Adult Congenital Heart Program Alabama Adult Congenital Heart Program
Advances in ACHD: a narrative of thinking outside the box
“Congenital affections of the heart are of only limited clinical interest,
as in a large proportion of cases the anomaly is not compatible with life,
and in the others nothing can be done to remedy the defect
or even to relieve the symptoms.” Sir William Osler: The Principles and Practice of Medicine. 1894
“Congenital affections of the heart are of significant clinical interest,
as in a large proportion of cases the anomaly is compatible with life,
and much can be done to remedy the defect and
to relieve the symptoms.”
§ Dr. Larry Markham § Director, Vanderbilt Adult Congenital Heart Program
§ Dr. Curt Daniels § Director, Columbus Ohio Adult Congenital Heart (COACH) program
§ Dr. Ali Zaidi § Director, Montefiore Adult Congenital Heart program
§ Dr. Sarah Cribbs § Med/Peds and Sports Medicine, UAB
Acknowledgements
Marc G Cribbs, MD Director, Alabama Adult Congenital Heart Program 2015 Southeastern Pediatric Cardiology Society
September 26th, 2015
Advances in ACHD a narrative of thinking outside the box Thank you