The natural and unnatural history of the systemic right ventricle in adult survivors

Abstract

Those patients who survive to adulthood with a systemic right ventricle experience low mortality and good functional status up to 40 years of age. However, there is a substantial burden of atrial tachyarrhythmia, and this occurs significantly earlier in those with transposition of the great arteries-atrial switch. Management of atrial tachyarrhythmia, along with systemic right ventricular dysfunction and systemic atrioventricular valve regurgitation, is likely to be the major challenge for this group of patients over the next decade.

Congenital Heart Disease in the Older Adult

“…However, high mortality with failure to complete LV retraining among adult patients undergoing the double switch coupled with the recent evidence of excellent survival (84%) at 40 years achieved in patients with nonanatomic repairs has underscored the need for further comparative studies. [367][368][369][370] Nonanatomic repair consists of simple VSD closure (when intraventricular tunnel [SenningRastelli] is prohibited) with relief of pulmonary stenosis with concomitant replacement of the systemic AV valve.…”

Management of Pregnancy in Patients With Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association

“…205 Baffle obstruction or leak can occur; heart failure caused by systemic ventricular dysfunction is a significant concern; and pulmonary hypertension is seen in a minority of patients. 206,207 Pregnancy in a patient with a systemic RV, whether D-TGA with an atrial switch or CC-TGA, may be poorly tolerated. The increased blood volume, heart rate, stroke volume, and cardiac output that occur with pregnancy may unmask heart failure, worsen tricuspid regurgitation, or exacerbate arrhythmias.…”

Long‐Term Outcomes of Tricuspid Valve Surgery in Patients With Congenitally Corrected Transposition of the Great Arteries

“…Given that patients without significant associated intracardiac lesions could remain asymptomatic for a long period,1, 2, 3, 4, 5 these patients can be unrecognized until their adulthood, especially in developing countries like China. Meanwhile, even if they are diagnosed in early childhood, more surgeons tend not to intervene surgically in the absence of hemodynamic lesions, but closely monitor them instead 6, 7.…”

“…Consequently, many patients with CCTGA are referred in adulthood. In these patients, incidence and degree of tricuspid regurgitation (TR) increase over time,5, 8 and TR has been identified as a major contributor to right ventricular (RV) failure and decreased survival 2, 9, 10, 11, 12. Traditionally, TR was considered secondary to dysfunction of morphological RV, which cannot sustain the systemic circulation, and tricuspid valve (TV) surgery was mostly described as unbeneficial in the early days 10, 13, 14, 15.…”