Comparison of intravenous sildenafil with inhaled nitric oxide for acute vasodilator testing in pulmonary arterial hypertension.

Acute vasodilator testing (AVT) identifies acute responders for initiation of calcium channel blockers in pulmonary arterial hypertension (PAH) and operability in congenital heart disease (CHD). We sought to determine the feasibility of intravenous sildenafil (ivS) as an alternative to inhaled nitric oxide (iNO) in AVT. All patients with PAH undergoing cardiac catheterization for AVT (November 2015 to December 2020) were prospectively enrolled. Hemodynamic data were obtained at baseline, with iNO 20 ppm and ivS (0.25 mg/kg for children and 10 mg for adults). We studied 44 patients with a mean age of 20.5 ± 14.4 years (27 [61%] females and 20 [45%] children). There were 17 (38.6%) patients in the CHD group for operability assessment and 27 patients in non-CHD group (idiopathic pulmonary arterial hypertension-16 [36.3%], residual PAH after shunt closure-7 [15.9%], and 2 cases [4.5%] each of familial PAH and portopulmonary hypertension). There was an excellent intraclass correlation for mean pulmonary artery pressures (0.903, 95% confidence interval, CI: 0.809-0.949, p < 0.001), mean aortic pressures (0.745, 95% CI: 0.552-0.858, p < 0.001), pulmonary vascular resistance index (0.920, 95% CI: 0.858-0.956, p < 0.001), systemic vascular resistance (SVR) index (0.828, 95% CI: 0.706-0.902, p < 0.001), and the ratio of pulmonary and SVR indices (0.857, 95% CI: 0.752-0.919, p < 0.001) between the two agents. There were two responders, both in non-CHD group, and were identified by iNO and ivS. The hemodynamic effects of ivS show excellent correlation with iNO and could be a potential alternative agent for identifying acute responders during AVT.

Pulmonary Hemorrhage Due to Unrecognized Bronchial Collateral After Arterial Switch Operation.

We report a neonate with transposition of great arteries and intact ventricular septum who had a massive pulmonary hemorrhage soon after an arterial switch operation. An emergency cardiac catheterization revealed a large bronchial collateral artery from the descending aorta feeding the right lung. The hemorrhage was controlled by coil embolization of the collateral, and the patient recovered after prolonged intensive care.