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 theGreat Arteries (TGA)

• Complete TGA or D-TGA– Embryological inversion of the great arteries– Ventriculoarterial discordance

Transposition of theGreat 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.