Long-term survival of patients with unoperated single ventricle heart defect: Four case reports and literature review.

ABSTRACT

A single ventricle (SV) heart defect is a rare complex congenital cardiac malformation, accounting for approximately 1%-2% of congenital heart diseases. Surgical intervention is the mainstay treatment for SV patients, although patients who do not receive surgical intervention may also survive. We followed up four adult patients who had SV since birth without surgical intervention and they had a good prognosis. The common characteristics of four long-term SV survivors were investigated by reviewing their medical records and the literature, and the current treatment methods for SV patients were also reviewed. The clinical presentation and long-term prognosis of SV patients without surgical intervention depend on the presence or absence of pulmonary blood flow obstruction, pulmonary vascular resistance, ventricular shape and function, aortic blood flow obstruction, and the atrioventricular valve shape and function. While the Fontan operation has become a common and effective method for SV treatment, long-term outcomes are fraught with morbidity and mortality. In our opinion, such patients with balanced hemodynamic condition could be followed and treated conservatively. Major cardiac surgery which leads to gross hemodynamic adjustments should be avoided. However, additional prospective study will be necessary to verify this assertion. This report aims to improve the prognosis as well as quality of life of SV patients.