Definitive palliation with cavopulmonary or aortopulmonary shunts for adults with single ventricle physiology

Abstract

Objective-To compare the relative merits of cavopulmonary or aortopulmonary shunts, or both, as definitive non-Fontan palliations for patients with single ventricle physiology. Design-Clinical data, ECG, echocardiographic data, surgical records, and available postmortem material were reviewed in all patients with single ventricle physiology identified from the University of Toronto Congenital Cardiac Centre for Adults (UTCCCA) database who had not undergone a Fontan operation. Current status of patients was assessed from clinic reviews and patient contact. Two groups of patients were identified: those with cavopulmonary shunt (group 1, n = 35); and those with aortopulmonary shunt(s) only (group 2, n = 15). Results-50 adults (21 male/29 female) who underwent the last palliation at a median age of 11 years (range 1 day to 53 years) were identified. During a mean (SD) follow up of 13.0 (6.2) years at the UTCCCA, 19 patients died. Survival is 89.4% and 51.9% at 10 and 20 years, respectively, from the time patients were first seen at UTCCCA, with no diVerences between the groups. Most recent New York Heart Association (NYHA) classification was I-II in 21 patients, III in 25, and IV in four patients; mean haemoglobin was 190 (28) g/l, and oxygen saturation was 82 (4)%, with no group diVerences. Arrhythmia developed in 25 patients (atrial flutter/fibrillation in 20 and/or sustained ventricular tachycardia in 11). Atrial flutter/fibrillation was more common in patients in group 2, who also showed a greater decline in ventricular function with time. Age at last palliation, cardiothoracic ratio, and inclusion in group 2 were predictive of atrial flutter/fibrillation, poor ventricular function predictive of ventricular tachycardia, NYHA class > III, and prior ventricular tachycardia predictive of death. Conclusions-Cavopulmonary or aortopulmonary shunts, or both, provide sustained palliation for selected patients with single ventricle physiology. Survival for both compares favourably with published Fontan series. Compared to aortopulmonary shunts, cavopulmonary shunts convey a beneficial long term eVect on ventricular function. Arrhythmia is a major cause of late morbidity in these patients, relating to both ventricular dysfunction and death. Onset of sustained ventricular tachycardia is an ominous sign. (Heart 2000;83:51-57)

Predictive factors for long-term prognosis in adults with cyanotic congenital heart disease — Japanese multi-center study

“…Several articles focused on the mortality and morbidity in adult patients with CCHD such as Eisenmenger syndrome [3][4][5][6][7][8][9][10][11][12][13], tricuspid atresia [14], pulmonary atresia with ventricular septal defect [15], Glenn shunts [16], palliation with cavopulmonary or aortopulmonary shunts [17], Ebstein's anomaly [18] or univentricular heart [19]. However, most of these studies were conducted by a single center and the number of patients was relatively small.…”

Clinical Use of Electrocardiography in Adults With Congenital Heart Disease

“…12 Atrial arrhythmias may be associated with substantial morbidity and death, as hemodynamic deterioration can ensue. Depending on the type of repair, intra-atrial reentrant tachycardias or atrial fibrillation may occur in up to 57% of patients, 89 with often complex and/or multiple circuits. 90 At mid-term follow-up, sinus node dysfunction occurs in 13% to 16% of patients with classic modified Fontans (ie, right atrium to pulmonary artery anastomosis) and increases with duration of follow-up.…”

Univentricular Heart

“…75 Although the occurrence of atrial tachycardias increase with follow-up duration and depend on the particular type of repair, they have been reported in up to 57% of patients. 60 The most common form is a macro-reentrant circuit, termed intraatrial reentrant tachycardia. These circuits may be complex or multiple.…”