No abstract

AFOP and GOP, a newly described OP form, possess clinical and pathological findings that set it apart from a COP, suggesting an emerging spectrum of OP.

Inhaled nitric oxide (iNO) is used for acute vasoreactivity testing in pulmonary arterial hypertension (PAH) patients. Inhaled epoprostenol (iPGI2) has pulmonary selectivity and is less costly. We sought to compare acute hemodynamic effects of iNO (20 ppm) and iPGI2 (50 ng/kg/min) and determine whether their combination has additive effects. We conducted a prospective, single center, randomized, cross-over study in 12 patients with PAH and seven with heart failure with preserved ejection fraction (HFpEF). In PAH patients, iNO lowered mean pulmonary artery pressure (mPAP) by 9 ± 12% and pulmonary vascular resistance (PVR) by 14 ± 32% (mean ± SD). iPGI2 decreased mPAP by 10 ± 12% and PVR by 12 ± 36%. Responses to iNO and iPGI2 in mPAP and PVR were directly correlated (r2 = 0.68, 0.70, respectively, P < 0.001). In HFpEF patients, mPAP dropped by 4 ± 7% with each agent, and PVR dropped by 33 ± 23% with iNO, and by 25 ± 29% with iPGI2 (P = NS). Pulmonary artery wedge pressure (PAWP) increased significantly with iPGI2 versus baseline (20 ± 3 vs. 17 ± 2 mmHg, P = 0.02) and trended toward an increase with iNO and the combination (20 ± 2, 19 ± 4 mmHg, respectively). There were no additive effects in either group. In PAH patients, the vasodilator effects of iNO and iPGI2 correlated at the doses used, making iPGI2 a possible alternative for testing acute vasoreactivity, but their combination lacks additive effect. Exposure of HFpEF patients to inhaled vasodilators worsens the PAWP without hemodynamic benefit.

Platypnea-orthodeoxia (P-O) syndrome is an underdiagnosed condition characterized by dyspnea and deoxygenation accompanying a change from a recumbent to an upright position. It is caused by increased right-to-left shunting of blood on assuming an upright position. The diagnosis of this shunt is often challenging. A case where a diagnosis was missed despite performing a tilt transesophageal echocardiogram with bubble study and a technetium labeled macroaggregated albumin scan is presented. However, a large patent foramen ovale (PFO) was found on autopsy. A brief overview of the diagnostic workup and management of this condition along with methods to increase the sensitivity of diagnostic tests is discussed.