Special Considerations for Special Populations: Congenital Heart Disease Valerie Bosco, FNP, EdD Alison Knauth Meadows, MD, PhD University of California San Francisco Adult Congenital Heart Program Outline The exploding population Pre-transplant considerations Outcomes Cases Conclusions Congenital Heart Disease: Spectrum of Disease Congenital Heart Disease CHD occurs in 8:1000 live births CHD represents a spectrum simple complex 1
Congenital Heart Disease 1938: First PDA ligation 1944: First aortic coarctation repair 1945: First Blalock-Taussig shunt 1950 s: Introduction of cardiopulmonary bypass - facilitated repair of TOF, CAVC, D-TGA, VSDs 1970 s: Fontan procedure introduced 1980 s: Neonatal surgery began - allowed repair of D- TGA with arterial switch procedure and palliation for HLHS Congenital Heart Disease Advances in catheter based interventions Advances in imaging 2D and 3D echocardiography Cardiac MRI Cardiac CT Advances in intensive care management Balloon pump ECMO Ventricular assist devices Advances in medical management Adults with Congenital Heart Disease More are surviving to adulthood 85% are predicted to survive to adulthood 85% increase since 1985 >1,000,000 ACHD patients in the USA Adults with CHD > children with CHD Adults with Congenital Heart Disease Sicker patients are surviving to adulthood More than 1/3 have moderate to severe disease Fewer than 30% are getting specialty care Many young adults will reach end stage without good options 2
Heart Transplant Transplant Heart-Lung Transplant Options for ACHD Patients yes Perform appropriate intervention Are there surgical or transcatheter options to treat residual hemodynamic burden? yes Consider transplant evaluation no Medical management no Consider transplant evaluation Pre-transplant considerations in ACHD Multiple surgeries Antibodies Scar Chest wall collaterals Access issues Anatomic issues Heterotaxy Great vessel anatomy distortion Venous anomalies Pre-transplant considerations in ACHD Long standing heart failure (low output, congestion) Renal failure Liver failure PLE Pulmonary arterial hypertension Chronic cyanosis bleeding risk thrombosis risk 3
DIAGNOSIS IN ADULT HEART TRANSPLANTS ADULT HEART TRANSPLANTATION Kaplan-Meier Survival by Diagnosis (Transplants: 1/2002-6/2007) % with congenital heart disease CHD = 1.8% (656) 1992-2001 CHD = 2.4% (451) 2002-2008 ADULT HEART TRANSPLANTATION Kaplan-Meier Survival by Era (Transplants: 1/1982 6/2007) Diagnosis: Congenital Type of study Single center (Chen et al., Ann Thorac Surg, 2004) Transplant in CHD Date # of Patients 1984-2004 106 with CHD (vs. 1419 without) Outcomes Predictorsof adverse outcomes 35% died -prior shunt -need for PA reconstruction -neonates -female -year of tx UNOS data base (Karamlouet al., J Thorac Cardiovasc Surg, 2010) 1990-2008 575 with CHD (vs. 7921 without) 38% died; 24% at 1 yr, 48% at 10 yrs(13% and 45% for non- CHD) -female -younger age -status 1 -longer ischemic times -absence of induction or steriod maintenance PHTS and CTRD (Chen et al., Ann Thorac Surg, 2004) 1990-2002 488 children and adults with CHD 14%died at 3 mos, 20% died at 5 years -previous Fontan -younger age (late) -older age (early) -longer ischemic times -higher transpulmonary gradient -CMV + donor/- recipient 4
DIAGNOSIS IN ADULT HEART-LUNG TRANSPLANTS (January 1982 - June 2008) ADULT HEART-LUNG TRANSPLANTATION Kaplan-Meier Survival By Diagnosis (Transplants: January 1990 June 2007) Other includes cancer, LAM, OB, sarcoidosis, bronchiectasis 24 yo female Heterotaxy, dextrocardia, {I,L,L} CAVC unbalanced to the RV, LV hypoplasia, pulmonary atresia, transposed great vessels, bilateral SVCs without a bridging vein, left sided IVC Status post LmBTS Status post RmBTS Status post bilateral BDG procedure Status post RSVC coiling Status post placement of left axillary AV fistula 5
History of atrial flutter controlled on sotalol Bilateral iliac venous occlusions Now with profound symptoms Resting and exertional cyanosis (rest 78%, with brief exercise 50%) Severe headaches Disfiguring AV malformation in left arm Risk factors Single ventricle Chronic cyanosis Limited venous access Heterotaxy and dextrocardia Multiple previous surgeries Case #2: DB Transplant candidate now, but can t wait 23 yo female Shone s syndrome including Parachute MV with MS Subvalvar and valvar aortic stenosis Coarctation of the aorta Severe LV diastolic dysfunction Case #2: DB Transplant candidate now, but can t wait Status post surgical aortic valvuloplasty Status post percutaneous aortic valvuloplasty Status post CoA repair and subas resection Status post AVR w/ St Jude s mechanical valve Left with hemodynamic burdens Mild MS Severe LV diastolic dysfunction with LVEDp30 and rising PVR NYHA Class III-IV 6
Case #3: LH Good transplant candidate 38 yohispanic male Tetralogy of Fallot Status post repair (8 years of age) Developed severe cardiomyopathy(lvef 7-25%) -? alcohol toxicity Status post ICD for syncope and NSVT NYHA Class III, CHF management, multiple hospital admissions Considered for transplant in 2003 Case #3: LH Good transplant candidate Admitted in cardiogenic shock (2005) 8/2005: To OR, but aborted transplant 9/2005: Successful heart transplant Follow-up at 1 and 5 years with no evidence of rejection, clinically well Co-morbidities Diabetes Osteoporosis Hepatic congestion Skin lesions Case #3: LH Good transplant candidate Predictors of favorable outcomes Single sternotomy Low PRA Relatively acute onset of heart failure Compliant with good family support Summary Adults with CHD represent a unique and complex population of transplant candidates Many ACHD are not good candidates Transplant should be considered early in ACHD patients To determine early whether transplant is an option in the future To determine whether other interventions will help or hurt candidacy To avoid detrimental effects of congestion, low output, and PAH on future candidacy 7