Abstract:Ebstein's anomaly is an uncommon congenital heart defect, with a prevalence of 0.3-0.5%. Its association with an imperforate tricuspid valve is an even more rare situation (less than 10% of cases). Prenatal diagnosis of this association by means of fetal echocardiography has not been reported. We describe here this association diagnosed before birth and confirmed after birth. The diagnostic potential and importance of fetal echocardiography during prenatal evaluation of cardiac malformations allows for adequat… Show more
“…Ebstein's anomaly is a rare congenital cardiac defect with a prevalence of 0.3–0.5% 1 . It is a complex congenital anomaly of the tricuspid valve leading to variable pathological features according to associated heart defects and severity of lesion 1,2 . The average life expectancy of patients with Ebstein's anomaly is 25–30 years 3,4 .…”
Section: Introductionmentioning
confidence: 99%
“…Survival into adulthood is common, and patients present with cyanosis, dyspnea and palpitations 1,6,7 . During pregnancy, the cardiac symptoms may be variable, from asymptomatic to cyanosis, congestive heart failure, right heart failure, systemic or pulmonary embolism, and tachyarrhythmia 4,5 .…”
When a woman with Ebstein's anomaly reaches childbearing age, fertility is not affected, even in cyanotic women. Under close supervision by the woman's obstetrician and cardiologist, the pregnancy outcome is usually favorable.
“…Ebstein's anomaly is a rare congenital cardiac defect with a prevalence of 0.3–0.5% 1 . It is a complex congenital anomaly of the tricuspid valve leading to variable pathological features according to associated heart defects and severity of lesion 1,2 . The average life expectancy of patients with Ebstein's anomaly is 25–30 years 3,4 .…”
Section: Introductionmentioning
confidence: 99%
“…Survival into adulthood is common, and patients present with cyanosis, dyspnea and palpitations 1,6,7 . During pregnancy, the cardiac symptoms may be variable, from asymptomatic to cyanosis, congestive heart failure, right heart failure, systemic or pulmonary embolism, and tachyarrhythmia 4,5 .…”
When a woman with Ebstein's anomaly reaches childbearing age, fertility is not affected, even in cyanotic women. Under close supervision by the woman's obstetrician and cardiologist, the pregnancy outcome is usually favorable.
“…EA may manifest clinically at any age and has a highly variable clinical course. Adults often present with cyanosis, dyspnea, palpitations, decreasing exercise tolerance, fatigue [4][5][6][7][8][9]. In the presence of an interatrial communication the risk of paradoxical embolization, brain abscess and sudden cardiac death increases.…”
Literature reviewEbstein's anomaly (EA) is a rare congenital heart defect with a prevalence of 0.3-0.5% (without any gender predilection) characterized primarily by abnormalities of the tricuspid valve and right ventricle [1]. The survival of patients with EA during childhood and adolescence has improved dramatically: after 35 months of diag-
“…Indeed, in rare circumstances, the valve deformed by Ebstein's malformation can be imperforate, producing a variant of tricuspid atresia. 32,[37][38][39][40] The hearts seen with tricuspid stenosis or atresia, however, do not typically show exorbitant cardiac enlargement, and hence do not fall within the category of "wall-to-wall" heart.…”
Section: Segmental and Sequential Analysismentioning
D ISCUSSION AND DEBATE CONTINUES AS TO THE congenital cardiac malformation currently warranting the dubious distinction as representing the worst disease. At one time, Roberts et al. 1 considered hearts with aortic atresia as constituting the worst disease, at least in terms of survival. The emergence of strategies for functionally univentricular palliation, along with transplantation, for these patients, with ever improving outcomes, has markedly altered this perception. 2 In a recent review, 3 we discussed the continuing disappointing outcomes for patients with isomerism of the right atrial appendages, certainly justifying the nomination of this lesion as one of the current worst forms of disease. Few would deny, however, that the subset of patients with pulmonary atresia and intact ventricular septum with florid tricuspid regurgitation, the complication producing the so-called "wall-to-wall" heart (Figs 1 and 2), also have a very grim prognosis. 4-6 The outcomes for the patients making up this subset, however, have received scant attention in comparison to that devoted to the more commonly encountered patients with hypoplastic ventricular cavities, severe right ventricular hypertension, and ventriculo-coronary arterial connections, another fascinating combination that received our recent attention. 7 It is our intention to correct the neglect for those with dilated right ventricles in the present review. Historical Comment As indicated above, we recently reviewed the findings in those patients with pulmonary atresia and intact ventricular septum complicated by ventriculocoronary arterial connections, emphasizing their Continuing Medical Education The "wall-to-wall" heart in the patient with pulmonary atresia and intact ventricular septum
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.