Background Fifty years ago, Paul Wood described a small subset of patients with rheumatic mitral valve disease who had a marked rise in pulmonary vascular resistance (PVR) and which was disproportionate to their elevated pulmonary venous pressure. Termed a “hyperactive” vasculature, the elevation in PVR fell dramatically and promptly after pulmonary venous pressure was reduced by mitral valve replacement. Herein we report a patient with valvular heart disease and a disproportionately elevated PVR that responded to pharmacologic management alone.
Case Presentation A 55-year-old man presented to the cardiology clinic complaining of profound exertional dyspnea of several months' duration. He was found to have jugular venous distention, a nondisplaced apical impulse, a normal first sound, accentuated P2 with physiologic splitting and no gallop sounds. Murmurs of aortic valvular stenosis (AS) and mitral regurgitation (MR) were appreciated. Lung fields were clear and there was no cyanosis, clubbing or edema. Left ventricular hypertrophy (LVH) without left atrial (LA) enlargement was seen on ECG. Based on these findings, chronic AS was suspected with functional MR and pulmonary hypertension considered to be of more recent onset. Echocardiography identified concentric LVH with normal-sized LV and LA chambers; AS and MR; and elevated mean pulmonary artery pressure confirmed at right heart catheterization (60 mm Hg), which also revealed a marked elevation in total pulmonary resistance (975 dynes•s•cm-5) and elevated wedge pressure (mean 37 and peak v wave of 53 mm Hg). Given normal-sized LV and LA chambers, respectively, this suggested abnormal LV stiffness and recent onset MR. Calculated aortic valve area was 1.2 cm2. ACE inhibition, together with loop diuretic and spironolactone, were initiated. At follow-up 1 month later, right heart catheterization revealed PCW of 25 mm Hg, PVR 63 dynes•s•cm-5, and loss of V wave.
Conclusion A hyperactive pulmonary vasculature in patients with valvular heart disease may be responsive to medical management and therefore should be so assessed prior to considering valve replacement.