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Abstract

Abstract Background: There have been only at least seven cases of isolated right ventricular non-compaction reported in literature, hence, there are no definite criteria for diagnosis and recommendations for management of these patients. The reported clinical manifestations include heart failure, arrhythmias and cardioembolic events. Left ventricular and biventricular non-compaction are likewise rare but more common than isolated right ventricular non-compaction. Pulmonary hypertension is associated with biventricular non-compaction more commonly but not with isolated right ventricular non-compaction. Setting: University of Santo Tomas Hospital Case report: We report a case of a 25 year old female who presented with progressive dyspnea for one year accompanied by easy fatigability. She denied chest pain, palpitations, pedal edema, paroxysmal nocturnal dyspnea and orthopnea. She had a brother who died at a young age reportedly due to a cardiac illness which was not fully worked-up. Her 2D echocardiogram showed excessive prominent trabeculations and deep inter-trabecular recesses in the right ventricular wall with depressed right ventricular systolic function by tricuspid annular plane systolic excursion and fractional area change. Color Doppler studies also showed severe tricuspid regurgitation, pulmonic regurgitation and severe pulmonary hypertension. Secondary causes of pulmonary hypertension like connective tissue diseases, left heart disease, chronic thrombotic/embolic disease, anomalous cardiac and pulmonary shunts and lung disease were excluded. She was given diltiazem, sildenafil, digoxin, warfarin overlapped with subcutaneous enoxaparin and oxygen supplementation which provided symptomatic relief.