Adult Congenital Heart Disease Carlos Macias, MD Arizona Pediatric Cardiology Consultants
Congenital Heart Disease • An abnormality of the heart that is present at birth. It may be hereditary or due to some influence occurring during pregnancy.
Congenital Heart Disease • 95% has no identifiable cause. • Ranges in severity from fairly simple to and curable to very complex and potentially lethal.
Congenital Heart Disease • The most common birth defect. • Occurs in @ 1/100 births
History of Congenital Heart Surgery • 1940’s-1950’s Maude Abbott and Helen Taussig defined the anatomy and physiology of Congenital Heart Disease. • Early 1950’s, first closed heart surgery using bathtub hypothermia. • 1953, First attempts at heart “bypass” surgery. • 1955, first bypass surgery at Mayo Clinic.
Congenital Heart DefectsPrevalence • 32,000 new cases of CHD / year in the U.S. • 1.5 million, world wide. • 20,000 open-heart operations / year in U.S. • > 85% reaching adulthood • Approx.. 1,000,000 adults with CHD in U.S. • More adults than children with CHD
Changes in Technology • Preoperative diagnosis • Intraoperative care (cardiopulmonary bypass). • Surgical technique • Postoperative care
ACHD • For all but the most simple defects, surgical and catheter procedures are palliative rather that curative.
Acyanotic CHD(left to right shunt) • Atrial septal defect (ASD) • Ventricular septal defect (VSD) • Endocardial cushion defect • Patent ductus arteriosus (PDA)
Cyanotic CHD(right to left shunt) • Obstruction to pulmonary blood flow • Tricuspid, pulmonary atresia • Intracardiac mixing of oxygenated and unoxygenated blood • Tetralogy of Fallot • Transposition of the great Vessels • Single ventricle
Obstructive Valvar Lesions • Pulmonary valve stenosis • Aortic valve stenosis. • Coarctation of the thoracic aorta
Amplatzer Occlusion of Atrial Septal Defect Clockwise from above: Transcatheter delivery of Amplatzer device, which is positioned across the atrial septal defect Left: Amplatzer device in place
ASD, Survival Patterns • Normal, if closed before 20 years of age. • Good, but shorter than normal 25-41 yrs. • Increased morbidity and mortality after 41 yrs.
ASD and PHT • Occurs in two chronologically disparate age groups. • Females, late teens and 20s. • Primary PHT with coincidental ASD • Don’t close the ASD !! • Patients > 40 years of age • Mild to mod. PHT secondary to longstanding left to right shunt. • Likely to benefit from ASD closure
Ventricular Septal Defect VSD before surgical repair VSD after surgical repair
Occlusion of Intracardiac and Vascular ShuntsVentricular Septal Defect Occlusion Above: Echocardiogram of muscular VSD Upper right: Fluoro image of CardioSEAL device occlusion of a VSD. Transesophageal echo probe (TEE) and pigtail catheter in place. Lower right: Amplatzer muscular ventricular septal occluder Illustration courtesy AGA Medical Group
Eisenmenger Complex Ventricular Septal Defect (VSD) in conjunction with Pulmonary Vascular Obstructive Disease (PVOD)Arrow – right to left shunt
Atrioventricular Canal Defect - Complete Above: view of heart from above showing joined mitral and tricuspid valvesRight: ASD – atrial septal defectVSD – ventricular septal defect
Occlusion of Intracardiac and Vascular ShuntsCoil embolization of PDA Left, top: Catheter crosses the PDA from the aortic side and delivers a coil. Left, bottom: Withdrawal of catheter, leaving coil in PDA
Severe Aortic Stenosis in an infant RV RA LA 1) Valvular Stenosis with a bicuspid aortic valve
Repair of Aortic Stenosis Ross Procedure Konno Procedure
Marfan’sSyndrome1 – enlarged ascending aorta2 – stretched aortic valve3 – mitral valve prolapseSV – Sinuses of Valsalva
ValvuloplastyPulmonary Balloon Valvuloplasty Left: Arrow indicates stenotic pulmonary valve
Coarctation of the AortaSA – subclavian artery COA – coarctationAV - aortic valve
Coarctation of the Aortawith severe aortic stenosis in an infant 1. Coarctation of the aorta, distal to the left subclavian artery 2. Severe aortic stenosis
AngioplastyAortic Coarctation Angioplasty Illustrations showing (left) uninflated and (right) inflated angioplasty balloon positioned within coarctation of the descending aorta
Intravascular StentsCoarctation of the Aorta Left: uninflated angioplasty balloon and stent within coarctation Middle: expansion of balloon and stent Right: deflation of balloon leaving stent wide open
Transposition of the Great Arteries – L TypeMV – mitral valveTV – tricuspid valve
Ebstein’s AnomalyASD – atrial septal defect1 – atrialization of the right ventricle