Ebstein’s Anomaly with Refractory Right-Side Heart Failure and Leg Ulcers

Abstract

Ebstein's malformation is a congenital heart disease. The principal pathological change is displacement of the septal leaflet of the tricuspid valve toward the apex of the right ventricle of the heart. The resulting hypoplasia, dysfunction of the right ventricle, and tricuspid regurgitation increase the volume load on the right side of the heart. The clinical features are chest tightness, shortness of breath, fatigue after activity, palpitations, cyanosis, and heart failure. We here report a case of Ebstein's anomaly with refractory right-side heart failure and leg ulcers.Keywords: Ebstein's anomaly; Right-side heart failure; Cardiogenic liver damage; Leg ulcer CaseOur male patient was 44 years old and was admitted to hospital for the fifth time since he had been recorded as suffering from "Chest tightness after activity for more than 30 years with abdominal distension, leg edema, and canker 1 year in duration, with an increase in symptom severity commencing 1 month prior". Physical examinationThe patient appeared chronically ill, with facial swelling, mild scleral jaundice, lip cyanosis, engorgement of the jugular vein, and a reflux-positive liver jugular vein. Lung percussion was voiceless, the breath sounds coarse, and no lung rale was apparent. No precordial bulge was noted; cardiac dullness was significantly expanded to the left; the heart rate was 84 beats/min; arrhythmia was evident; the intensity of the first heart sound differed from that of the others; threeto-six systolic murmurs could be heard in the pulmonary valve auscultation area; and no pericardial friction sound was apparent. Abdominal distension was noted; the abdominal veins; the liver was palpable 2 cm below the ribs; and the "shifting dullness" test was positive. Severe edema of the lower limbs was noted; skin ulcers 5-8 cm in diameter were present on the right lower limb, and large purplebrown areas of pigmentation were noted on both lower limbs ( Figure 1).