Case of Ebstein Anomaly Complicated by Left Ventricular Outflow Tract Obstruction Secondary to Deformed Basal Septum Attributable to Atrialized Right Ventricle

A 59-year-old woman presented to Okinawa Chubu Hospital with a several-month history of progressive dyspnea. The patient initially noted exertional dyspnea after walking only a half-mile or when lifting heavy objects. Dyspnea worsened progressively, resulting in multiple syncopal episodes. The patient denied chest pain or dyspnea at rest. Her past history was significant for Ebstein anomaly, hypertension, hyperlipidemia, and duodenal ulcer. Her current medication included furosemide 20 mg twice a day, spironolactone 25 mg every day, losartan 25 mg every day, and pravastatin 10 mg every day.On initial examination, her blood pressure was 100/75 mm Hg, her heart rate was 75 bpm, and her respiratory rate was 18 breaths/min. A grade 4 systolic ejection murmur was noted throughout the precordium. Jugular venous pressure was not elevated and peripheral edema was not present. A chest roentgenogram showed cardiomegaly(Figure 1A)without pulmonary vascular congestion. An ECG showed normal sinus rhythm and marked left ventricular hypertrophy as manifested by high voltage and strain-type ST-T changes in I, aVL, V4 through V6(Figure 1B). Laboratory tests showed normal kidney and liver functions and no evidence of anemia, and brain natriuretic peptide was 399 pg/mL (u003c18.4 pg/mL). An echocardiogram showed the apically displaced septal leaflet of the tricuspid valve in comparison with the anterior leaflet (Figure 2A and 2B), consistent with Ebstein anomaly. Functional right ventricle was so diminutive, and there …