Congenital heart defects in adulthood

Abstract

Background: More than 90% of children with congenital heart defects now survive into adulthood; just a few decades ago, survival was rare, particularly among patients with complex defects. The new population of adults with congenital heart disease presents a special challenge to physicians from all of the involved specialties.

Methods: Selective literature review.

Results and conclusion: A complete cure of the congenital heart defect in childhood is exceptional, and most adult patients continue to suffer from residual problems and sequelae. Further surgery or catheter interventions may be needed. Potential late complications include arrhythmias, heart failure, pulmonary hypertension, endocarditis, and thromboembolic events. The management of these patients during pregnancy or non-cardiac surgery remains a challenge. If this evolving patient population is to receive the best possible care, the adequate provision of specialized medical services is a necessary, but not sufficient, condition: patients and their referring physicians will also need to be aware that these services are available, and then actually make use of them. Moreover, optimal communication among all of the involved physicians is essential.

Figure 1

Overview of the localizations (view from right atrium and ventricle to interatrial and interventricular septum) of atrial septal defects (ASD), atrioventricular defects (AVSD), and ventricular septal defects (VSD). The figure shows that especially in upper sinus venosus defects, attention should be paid to associated anomalous pulmonary venous connection. TV, tricuspid valve

Figure 2

Overview of the anatomy in uncorrected (left) and corrected tetralogy of Fallot. Common problems in adult patients after correction of a tetralogy of Fallot include relevant pulmonary valve regurgitation, right ventricular dilatation and functional impairment, and a tendency to arrhythmias

Figure 3

Overview of the anatomy in patients with transposition of the great arteries (TGA or D-TGA) and congenital corrected TGA (ccTGA or L-TGA). ccTGA and TGA (after atrial switch operation). Both have a morphologically right systemic ventricle in common. This is associated with the development of heart failure in the long term and with increased morbidity/mortality. By means of atrial switch surgery, the venous blood is redirected at the atrial level. This surgical method has been superseded in the past 20 years by arterial switch surgery, which has the advantage of a morphologically left systemic ventricle. In ccTGA, a combination of atrioventricular and ventriculoarterial discordance is present. The systemic ventricle is hatched. LV, left ventricle; RV, right ventricle

Figure 4

Overview of different modifications of Fontan palliation in patients with a univentricular heart. In patients with Fontan-type repair, the systemic venous return is re-directed to the pulmonary circulation (without a subpulmonary ventricle). Over time, several modifications have been developed. The “classic” Fontan repair: direct connection between right atrium and pulmonary artery. In total cavopulmonary anastomosis (TCPC), a connection is made between the superior and inferior vena cava and the right pulmonary artery. The superior vena cava is anastomosed directly end-to-side with the pulmonary artery, the connection to the inferior vena cava can be established either via a lateral tunnel within the right atrium or via an extracardiac conduit. TCPC is the preferred procedure because of better hemodynamics, a lower incidence of arrhythmias, and better long-term survival. Typical problems in the long term are ventricular dysfunction, intraatrial thrombi, and a proneness to arrhythmias. These problems are encountered in particular after the “classic” Fontan repair