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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.


Parasternal long image on a 2D transthoracic echocardiographic image showing a large-sized perimembranous ventricular septal defect (arrow). Ao, aorta; LV, left ventricle; RV, right ventricle.

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Parasternal long-axis image of a large perimembranous ventricular septal defect (VSD) with color flow Doppler with predominantly right-to-left shunting across the defect.

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Side-by-side parasternal short-axis echocardiographic images showing the perimembranous ventricular septal defect (VSD) (arrow) in 2D and with color flow across the defect. Ao, aorta; LA, left atrium; RA, right atrium; RV, right ventricle.

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Hemodynamic cardiac catheterization data in a 35-year-old patient with an unrepaired ventricular septal defect (VSD). The hemodynamic data shown in the image is after vasoreactive testing using iNO and 100% Fio2.

  1. There were systemic RV and PA pressures in all phases.

  2. Baseline hemodynamic measurements on room air are as follows:

    • Qp/Qs = 0.25:1, PVR = 30.2 WU

    • Saturations: SVC 73%, LPA 74%, aAo 79%

    • Pressures: RA 3 mm Hg, RV 126/8 mm Hg, PA 120/50/86 mm Hg, LPA 120/50/86 mm Hg, LPCW 24 mm Hg

    • LV 126/24 mm Hg, dAo 126/82/96 mm Hg

  3. Hemodynamic measurements after 100% oxygen are as follows:

    • Qp/Qs = 1.24:1 PVR 30.9 WU

    • Saturations: SVC 71%, LPA 85%, aAo 96%

    • Pressures: RA 4 mm Hg, RV 111/8 mm Hg, PA ...

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