Diagnosis and natural history of Ebstein's anomaly.

Abstract

Thirty five cases of Ebstein's anomaly were diagnosed by cardiac catheter study or echocardiography between 1962 and 1984. The anomaly was an isolated abnormality in 27 patients; the remainder had additional heart lesions. Six patients have died and four of these had other cardiac lesions. Thirteen are currently symptom free. Twelve cases presented with cyanosis and heart murmur on the first day of life. Eight of these had associated thrill which is a rare finding in neonates. As pulmonary vascular resistance fell there was clinical improvement. This was assisted by oxygen treatment. Another two cases presented in the first week of life and 10 in the first decade with a murmur or supraventricular tachycardia. Eleven cases presented as adolescents or adults with a murmur, cardiomegaly, or cardiac symptoms. Three patients were seen initially in the sixth decade. Clinical diagnosis was correct in only one adult. Twenty five patients had cardiac catheterisation. Important arrhythmias occurred in 10 cases, and two required cardioversion. Difficulty was experienced in entering the pulmonary artery in 11 infants and children. Diagnosis at initial catheter study was incorrect in four patients. Echocardiograms were recorded in 29 cases. Earlier M mode measurements of time delay of tricuspid closure compared with mitral closure did not always lead to the correct diagnosis. Cross sectional studies gave good images of tricuspid leaflet displacement, its tethering, and the atrialised portion of the right ventricle and have facilitated the diagnosis of more cases in recent years. Echocardiography is the procedure of choice for diagnosis of Ebstein's anomaly.

Case Report of Symptomatic and Asymptomatic Ebstein’s AnomalyCardiac MR Imaging

“…In 1866, Wilhelm Ebstein performed and reported the findings of necropsy in a 19-year-old cyanosed laborer. 4 Although the classical findings are inferior displacement of tricuspid valves and atrialization of right ventricle, the extent of both varies widely. Other factors are leaflet structure, size and extent of adherence to the right ventricle wall, right ventriclecontractility and myocardial thickness.…”

Ebstein's anomaly with coarctation of the aorta. An unusual association

“…Coarctation of the aorta is one of the most unusual abnormalities associated with Ebstein's anomaly 5 .…”

A Case of Pregnancy with Ebstein’s Anomaly

“…8 However the prognosis for neonatal Ebstein's anomaly has improved with modern surgical techniques. 9 There are few published reports on pregnancy in women with Ebstein's anomaly. The largest series was reported by Radford et al, where successful pregnancy occurred in six women who had a total of 12 children.…”

“…The largest series was reported by Radford et al, where successful pregnancy occurred in six women who had a total of 12 children. 9 In two large studies on congenital heart disease in pregnancy (total 357 women) five women had Ebstein's anomaly and three of them had cyanosis. 23 They had eight pregnancies and there were two fetal deaths, both in infants born to women with cyanosis.…”