A giant right-sided heart due to idiopathic pulmonary hypertension.

A 46-year-old man with long standing idiopathic pulmonary hypertension (IPAH) was referred for further cardiac investigation due to clinical deterioration associated with recurrent episodes of syncope and progressive dyspnea, NYHA class IV. An underlying lung disease or chronic thromboembolic disease were excluded. Transthoracic echocardiography (Figure 1A–C) revealed a giant right dilated atrium and ventricle with squashed left heart chambers (echocardiography: 4-chamber view during diastole (A) and systole (B), and supplemental video), which indicated an ‘ acute-on-chronic ’ severe cor pulmonale. An elevated right ventricular systolic pressure (107 mmHg) over right atrial pressure was determined by continuous wave Doppler echo imaging (Figure 1D) with a tricuspid regurgitation peak velocity of 518 cm/s (C). Th e tricuspid regurgitation was moderate. ECG at rest indicated right ventricular hypertrophy