Pulmonary Thromboembolism Superimposed on a Congenital Ventricular Septal Defect in a 50-Year-Old Man: Inhaled Nitric Oxide and Sildenafil to the Rescue

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We report the combined use of inhaled nitric oxide (iNO) and sildenafil to selectively lower pulmonary vascular resistance (PVR) and reverse right-to-left shunt flow across a congenital ventricular septal defect (VSD) in a 50-year-old man. The patient developed right-to-left shunt flow with profound hypoxia because of an acute pulmonary embolism superimposed on underlying pulmonary hypertension. The dramatic improvement in oxygenation and PVR with sildenafil plus iNO has been sustained with sildenafil monotherapy. To our knowledge, use of this combination therapy in patients with decompensated pulmonary arterial hypertension (PAH) and a predisposition to right-to-left shunting has not been previously reported.

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