Survival in atrioventricular discordance

Abstract

Limited information is available concerning the long-term survival of patients with atrioventricular discordance, ventriculoarterial discordance, and two ventricles (corrected transposition). The long-term follow-up of 107 patients examined at the Mayo Clinic over a 30-year period between 1951 and 1981 was reviewed. Overall survival from the date of Mayo Clinic diagnosis was 70% at five years and 64% at ten years. Associated variables were analyzed for their effect on survival including sex, age at diagnosis, presence of ventricular septal defect, pulmonary stenosis, dextrocardia, left atrioventricular valve insufficiency, and complete heart block. There was no significant difference between those with and without a ventricular septal defect (VSD). Pulmonary stenosis was protective when a VSD was present but was not a significant predictor of long-term survival. The only variable that consistently correlated with decreased survival was left atrioventricular valve insufficiency (p less than 0.04 for univariate and stepwise Cox, and p = 0.08 for multivariate analysis). A logistic model for survival after open-heart surgery failed to identify any significant variable. We conclude that the presence of left atrioventricular valve insufficiency in association with atrioventricular discordance significantly alters the long-term outcome. Atrioventricular valve replacement should be considered in such patients when insufficiency becomes hemodynamically significant.

The prenatal diagnosis of, and short-term outcome for, patients with congenitally corrected transposition

“…This seems to support the hypothesis that postnatal tricuspid regurgitation in congenitally corrected can be related to malformations of the valve in themselves, 11 and to the increased load on the morphologically right ventricle. 8,27 Moreover, tricuspid insufficiency in our first patient, possibly related to complete atrioventricular block through ventricular dilation and higher stroke volume, was not reversed by dual-chamber pacing. Our seventh patient had long episodes of sustained supraventricular tachycardia refractory to different drug therapies.…”

A rare case report of corrected transposition of the great arteries in association with tuberous sclerosis and cardiac rhabdomyomas

“…Survival is then relatively constant, with an attrition rate of ∼1% to 2% a year. 10 However, the associated anomalies have a great impact on prognosis and outcome. Multiple rhabdomyomas may have an impact on the ultimate outcome in this infant and hence needs long-term follow-up.…”

Midterm evaluation of echocardiographic examination, cardiac catheterization findings and surgical results of patients with congenitally corrected transposition of great arteries: A single center experience

“…There is a heterogeneous group of patients and highly variable clinical course associated with the presence and severity of accompanying abnormalities such as ventricular septal defect (VSD), pulmonary stenosis (PS)/atresia, Ebstein-like malformation of the tricuspid valve (2) . Long-term survival and, if possible, morphological right ventricular dysfunction and tricuspid regurgitation counter-acting the systemic circulation are important prognostic determinants in these patients (3)(4)(5) . Surgical procedures include conventional repair (atrial septal defect and VSD closure, elimination of PS, isolated tricuspid valve repair), anatomic repair (double switch operation; atrial switch and arterial switch), and Fontan-type repair options (19) .…”