Exceptional survival of a patient with large ventricular septal defect, bidirectional shunt, and severe pulmonary valve stenosis

A 74-year-old man has survived in good health for an exceptionally long time despite the presence of a moderate-to-large-sized membranous ventricular septal defect (VSD). He has remained acyanotic with New York Heart Association class I function. Transthoracic and transesophageal echocardiography with color flow Doppler demonstrated a membranous VSD with left-to-right and right-to-left bidirectional shunts during ventricular systole and diastole, respectively, with an right ventricular systolic pressure of 93 mm Hg, dilation of the atria and the right ventricle, and right ventricular hypertrophy. The pulmonary valve was severely stenotic with transpulmonary valve peak velocity of 6.1 m/s and a peak pressure gradient of 147 mm Hg. The pulmonary artery and inferior vena cava were mildly dilated, and the left ventricular dimension and systolic function were normal. Transesophageal echocardiography with saline solution microbubble injection demonstrated positive contrast effect in the left ventricle in diastole confirming a right-to-left shunt at the ventricular level. This man is currently the oldest survivor with a moderate-to-large-sized membranous VSD reported in the literature.