SITUS SOLITUS WITH DEXTROCARDIA

SITUS SOLITUS WITH DEXTROCARDIA. This cardiac malposition occurs only occasionally with a structurally normal heart, which permits adult survival but delays clinical recognition.5 A routine chest radiograph may provide the first evidence of the malposition. Coexisting congenital cardiac anomalies, which are usually present, determine survival.

CONGENITAL COMPLETE HEART BLOCK

CONGENITAL COMPLETE HEART BLOCK (see also [For More Information] and 904). Adult survival is the rule, although the ultimate fate of large numbers of older patients with congenital complete heart block dampens optimism, and mortality even in infancy and childhood is not negligible.41–43 A substantial majority of young patients are asymptomatic, but mild, serious, or even fatal sequelae sometimes occur.5 Key determinants of clinical stability in uncomplicated congenital complete heart block are the ventricular rate, the hemodynamic adjustments at rest and with exercise, and the presence of intrinsically normal myocardium.5

CONGENITALLY CORRECTED TRANSPOSITION OF THE GREAT ARTERIES

CONGENITALLY CORRECTED TRANSPOSITION OF THE GREAT ARTERIES (see also [For More Information] and Fig. 29–62 Fig. 29–62 ). When this malformation is isolated (uncomplicated), survival is good but not normal because of the vulnerability of a morphological right ventricle in the systemic location.44–47 A major variable that decreases long-term survival is the presence and degree of incompetence of the inverted left atrioventricular valve (Ebstein-like anomaly) that may be mistaken for acquired mitral regurgitation.5 Survival into the sixth or seventh decade is uncommon, but not unknown, with an occasional patient reaching the eighth decade.48 Complete atrioventricular block accrues at a rate of about 2 per cent per year and may become manifested with a Stokes-Adams attack or sudden death.49

EBSTEIN’S ANOMALY OF THE TRICUSPID VALVE

EBSTEIN’S ANOMALY OF THE TRICUSPID VALVE (see also [For More Information] and Figs. 29–56 Figs. 29–56 , and 29–57 29–57 ). Longevity ranges from intrauterine or neonatal death to asymptomatic survival into late adulthood.50–52 The majority of patients fall between these extremes. Factors chiefly limiting survival in adults are functional class, marked increase in cardiac size, cyanosis, and recurrent paroxysmal rapid heart action, especially when accompanied by accelerated conduction through accessory pathways (Fig. 30–7 Fig. 30–7 ).53,54 Left ventricular function is an additional matter of concern.55,56 Syncope heightens suspicion of accelerated bypass conduction (rapid atrial fibrillation or one-to-one atrial flutter). The rapid ventricular response has been responsible for sudden death. For patients who survive the first year of life, there is a cumulative mortality of approximately 12 per cent, which is distributed about evenly throughout childhood and adolescence.53,54 Nevertheless, there are accounts of patients with Ebstein’s anomaly who have survived into their eighth decade.57,58 The oldest patient recorded with the anomaly lived to age 85 and had no cardiac symptoms until age 79.59

Date: 2014-12-21; view: 1156