Case ReportIn August 2013, a 58-year-old woman with a history of atrial arrhythmias and severe tricuspid regurgitation presented with fatigue and right-sided heart failure. Her medical history also included ulcerative colitis; superficial thrombophlebitis; and atrial flutter and atrioventricular nodal reentrant tachycardia, for which she had undergone successful radiofrequency ablation 3 years earlier. Results of a clinical examination included elevated jugular venous pressure, peripheral edema, and hepatomegaly.The patient's treadmill exercise test results included average exercise capacity, normal heart-rate and blood-pressure responses to exercise, normal heart-rate recovery, no arterial desaturation, and a functional aerobic capacity of 101% of predicted value. A chest radiograph showed cardiomegaly. A transthoracic echocardiogram revealed reduced RV size and substantial hypoplasia of the RV apex (Fig. 1). The right atrium was markedly enlarged. Abnormal TV function included incomplete valvular coaptation, severe regurgitation, and annular dilation. Pulmonary valve cusp mobility was abnormal without regurgitation. The left ventricle (LV) was of normal size but occupied the apical portion of the heart, wrapping around the hypoplastic RV apex. The LV systolic function was normal; however, paradoxical septal motion was apparent.Cardiac magnetic resonance images similarly revealed an enlarged right atrium, severe TV insufficiency, and RV apical hypoplasia (Fig. 2). The patient's LV ejection fraction was 0.59, and her RV ejection fraction was mildly depressed (0.46). Liver magnetic resonance elastograms revealed elevated stiffness at a value of 3.9 kPa, consistent with moderate hepatic fibrosis.Preoperative cardiac catheterization was performed to delineate the patient's RV hemodynamic status and coronary anatomy. An RV angiogram showed RV apical hypoplasia with mildly to moderately decreased systolic function, severe tricuspid regurgitation, and a massively dilated right atrium (Fig. 3). On levophase, the patient's LV function was normal with no regional wall-motion abnormalities, and her coronary artery anatomy was normal. Atrialization of RV pressure waves was noted, and her RV end-diastolic pressure was elevated at 14 mmHg. Her pulmonary vascular resistance index was borderline elevated (4.7 WU*m 2 ), and her cardiac index was low (2 L/min/m 2 ).
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