Adult Congenital Heart Disease “Common” Complex Lesions Jeffrey D. Solomon, MD November 14 & 19, 2008 Objectives • Describe the changing profile of ACHD • Describe the challenges in the transfer of care from pediatric to adult practitioners • Review the development of the heart • Describe the anatomy, natural history, surgical repair, and common adult presentations of tetralogy of Fallot, transposition of the great arteries, and hypoplastic left heart syndrome ACHD • A gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance • Overall incidence: 75 of every 1000 live births ACHD • A gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance • Overall incidence: 75 of every 1000 live births • Incidence of moderate and severe CHD: 8 of every 1000 live births ACHD • A gross structural abnormality of the heart or intrathoracic great vessels that is actually or potentially of functional significance • Overall incidence: 75 of every 1000 live births • Incidence of moderate and severe CHD: 8 of every 1000 live births • Over 90% of children survive to adulthood • 20,000 operations for CHD in the US each year • Over 1 million adults in the US with CHD Transfer/Transition of Care • Challenges – Complex CHD is seldom cured – Patients have a poor understanding – Timing of transition – Lack of exposure among adult cardiologists Transfer/Transition of Care • 32nd Bethesda Conference (2000) – Regular evaluation at an ACHD center – Health care passport – Structured transfer/transition plan – Co-management with cardiologists having undergone advanced training Tetralogy of Fallot (TOF) • Named by Etienne-Louis Arthur Fallot in 1888 • Approximately 10% of all complex CHD • Single developmental error of the terminal portion of the spiral truncoconal septum • Four distinct components: subpulmonic stenosis, VSD, overriding aorta, and RV hypertrophy • Often accompanied by other anomalies Natural History • Depends on severity of RVOT obstruction • Cyanosis and exertional dyspnea • “Tet spell” – profound cyanotic episode secondary to augmented right-to-left shunt • Without surgical correction, patients do poorly Surgical Repair • Longest surgical history/most studied outcomes • Palliative surgical shunts Surgical Repair • Longest surgical history/most studied outcomes • Palliative surgical shunts • Classic/complete repair – Infundibular muscle resection, VSD patch, & RVOT repair – Typically preformed between 4-6 months of age – Surgical risk: < 5% • Survival rates: 85% at 30+ years Adult Presentation • Repaired – RVOT obstruction – Pulmonary or tricuspid regurgitation Adult Presentation • Repaired – RVOT obstruction – Pulmonary or tricuspid regurgitation – LV/RV dysfunction – Atrial/ventricular arrhythmias • Unrepaired – Significant morbidity – Consider later repair Transposition of the Great Arteries (TGA) • Complete TGA or D-TGA – Embryological inversion of the great arteries – Ventriculoarterial discordance Transposition of the Great Arteries (TGA) • Complete TGA or D-TGA – Embryological inversion of the great arteries – Ventriculoarterial discordance • Congenitally corrected TGA or L-TGA – Embryological inversion of the ventricles – Atrioventricular & ventriculoarterial discordance (double discordance) Surgical Repair • Balloon atrial septostomy – Developed by Rashkind (1965) – Enlarges the atrial communication • Atrial switch – Performed first by Senning (1958) and later modified by Mustard (1964) – Atrial baffle is created to direct venous return to the contralateral ventricle Adult Presentation • RV dysfunction • Tricuspid insufficiency • Bradyarrhythmias • Atrial tachycardias • Obstructed/leaky atrial baffle Surgical Repair • Arterial switch – Performed first by Jatene (1976) – Great arteries transected and reattached to appropriate AV valve – Coronary ostia also transplanted – Surgical treatment of choice – Excellent outcomes so far Adult Presentation • Coronary perfusion issues • Supravalvar aortic and pulmonic stenosis • Aortic root dilatation and valvular insufficiency • Branch pulmonary stenosis Single Ventricle Physiology • Hypoplastic left-heart syndrome • Hypoplastic right-heart syndrome (pulmonary atresia with intact ventricular septum) • Tricuspid valve atresia • Mitral valve atresia • Double-inlet ventricle • Atrioventricular canal defects • Double outlet right ventricle Surgical Repair • Staged rerouting of the systemic venous return • Step One – Complete mixing of systemic and pulmonary venous return – Unobstructed outflow tract to the systemic arterial circulation – Reliable source of pulmonary blood flow Adult Presentation • RV dysfunction • Cyanosis • Protein losing enteropathy Key Points • The majority of children born with CHD survive into adulthood • Infants born with complex CHD are seldom cured; residual problems are common and predictable • There are many challenges in the transition of care from pediatric to adult practitioners • Adults with moderate and complex CHD should undergo regular evaluation at a regional ACHD center Selected References Bashore TM. Adult congenital heart disease: right ventricular outflow tract lesions. Circulation 2007 115: 1933-1947. Gewillig M. The Fontan circulation. Heart 2005 91: 839-846. Hoffman JIE, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002 39: 1890-1900. Sommer RJ, Hijazi ZM, Rhodes JF. Pathophysiology of congenital heart disease in the adult: part III: complex congenital heart disease. Circulation 2008 117: 1340-1350. Warnes CA. Transposition of the great arteries. Circulation 2006 114: 2699-2709. Warnes CA, Liberthson R, Danielson GK, Dore A, Harris L, Hoffman JIE, Somerville J, William RG, Webb GD. Task force 1: the changing profile of congenital heart disease in adult life. J Am Coll Cardiol 2001 37: 1170-1175.
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