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A 35-year-old-young man from Guatemala presented with chronic cough and progressive fatigue. He was told that he had a "hole in his heart" when he was a child but never underwent surgical repair. His oxygen saturations at rest were 84%, heart rate of 106 bpm, and a BP of 118/78 mm Hg. He had equal saturations in all 4 extremities. He had a loud second heart sound, and a III/VI holosystolic murmur at the left sternal border (LSB) with a mild right parasternal heave on palpation and clear lungs. He had only trivial edema around his ankles. On echocardiography he was found to have a large perimembranous ventricular septal defect (VSD) with predominantly right-to-left shunting with color flow Doppler assessment (Figures 10-1A, 10-1B, and 10-2).There was no evidence of any right ventricular outflow tract obstruction. He underwent a cardiac catheterization and was found to have irreversible pulmonary vascular disease with a pulmonary vascular resistance of 14 Wood units (WU) and no reversibility with a vasodilator challenge (Figure 10-3). He was not a candidate for late surgical closure of the defect. He was treated with pulmonary vasodilators for symptomatic relief. His symptoms of chronic cough did improve. He also had significant erythrocytosis and an elevated hematocrit. He did not manifest any symptoms of hyperviscosity and therefore did not undergo any phlebotomy.
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