Journal of Rare Cardiovascular Diseases

In idiopathic pulmonary arterial hypertension (IPAH) nitric oxide (NO) is an agent of choice for pulmonary artery (PA) reactivity testing. Contrary, currently there is no standardized protocol for PA reactivity testing in patients with pulmonary hypertension (PH) associated with left heart disease and several vasodilator agents are suggested for this procedure. We aimed to assess whether standard protocol of PA reactivity testing used in IPAH is useful in patients with severe PH due to severe systolic dysfunction of a left ventricle. During right heart catheterization we assessed hemodynamic parameters in 14 patients at baseline, during NO inhalation and 30 minutes after oral administration of sildenafi l. We observed a significant decrease in PA pressure and resistance, and increase in cardiac index after sildenafil but not after NO administration. We conclude that standard protocol for PA reactivity in IPAH is not useful in patients with PH due to severe left ventricular systolic dysfunction. JRCD 2012; 1: 7–9

Pulmonary hypertension due to left heart disease; Qualification for heart transplant

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