sildenafil-citrate and Foramen-Ovale--Patent

We describe the case of a woman who presented to the intensive care unit with acute respiratory failure that required mechanical ventilation. She had severe pulmonary hypertension secondary to interstitial lung disease, and her history included sarcoidosis and tuberculosis. She was dependent on inhaled nitric oxide (INO) to maintain safe arterial oxygen saturation and could not be weaned from mechanical ventilation. Echocardiography revealed a patent foramen ovale with substantial right-to-left shunt, which probably contributed to her hypoxemia. Sildenafil enabled weaning from INO and substantially reduced the flow through the patent foramen ovale. She was successfully extubated and discharged home. To our knowledge, this is the first report of weaning from INO and mechanical ventilation in a patient with both severe secondary pulmonary hypertension and a right-to-left shunt through a patent foramen ovale.

Topics: Aged; Bronchodilator Agents; Comorbidity; Dyspnea; Female; Foramen Ovale, Patent; Humans; Hypertension, Pulmonary; Nitric Oxide; Phosphodiesterase 5 Inhibitors; Piperazines; Purines; Respiratory Insufficiency; Sarcoidosis, Pulmonary; Sildenafil Citrate; Sulfones; Vasodilator Agents; Ventilator Weaning

Topics: Administration, Oral; Adult; Aged; Aged, 80 and over; Brain Ischemia; Echocardiography, Transesophageal; Female; Foramen Ovale, Patent; Hemodynamics; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Phosphodiesterase 5 Inhibitors; Piperazines; Purines; Sildenafil Citrate; Stroke; Sulfones; Treatment Outcome; Ultrasonography, Doppler, Transcranial

Pulmonary arterial hypertension (PAH) is a life-threatening disease of the pulmonary arterioles, which, in the absence of effective therapy, progresses rapidly to right heart failure and death. Opening of a patent foramen ovale (PFO) is common in patients with severe pulmonary hypertension (PH), resulting in resistive hypoxemia. We report the case of a 40-year-old woman with idiopathic pulmonary hypertension (iPAH) in New York Heart Association (NYHA) class III to IV, who was admitted in the intensive care unit with hemodynamic compromise and severe hypoxemia due to right-to-left shunt throughout a PFO. Combination therapy initially with inhaled iloprost and sildenafil, and then addition of an endothelin A receptor-selective antagonist (sitaxsentan), resulted in impressive improvement in oxygenation with reversal of the right-to-left shunt and marked improvement in functional class of the patient.

Topics: Adult; Antihypertensive Agents; Drug Therapy, Combination; Endothelin A Receptor Antagonists; Epoprostenol; Female; Foramen Ovale, Patent; Humans; Hypertension, Pulmonary; Iloprost; Isoxazoles; Piperazines; Purines; Sildenafil Citrate; Sulfones; Thiophenes; Treatment Outcome; Vasodilator Agents