Unilateral absence of the pulmonary artery is a rare congenital cardiovascular anomaly that can lead to pulmonary hypertension and poor outcomes. We report the case of a 1-month-old infant with isolated unilateral absence of the pulmonary artery and severe pulmonary hypertension on the right and left sides, respectively. The patient was unresponsive to multiple medications for pulmonary hypertension, and surgical revascularisation was unfeasible. However, iloprost inhalation was effective.

We report a patient who developed sudden, bilateral, sensorineural hearing loss during therapeutic use of iloprost for Raynaud's phenomenon. The sudden hearing loss was attributed to iloprost use and completely reversed in eight days with conservative therapy. Iloprost may be a potentially ototoxic drug, causing sudden hearing loss.

Perioperative acute right heart failure after orthotopic heart transplantation contributes significantly to morbidity and mortality. Vasodilators administered intravenously may decrease pulmonary vascular resistance, but also affect systemic circulation. A decrease of mean arterial pressure will result in reduced right coronary blood flow and deterioration of right ventricular performance. Vasodilators administered by inhalation, i.e. nitric oxide and prostacyclin, are attractive due to a more selective effect on the pulmonary circulation. This is the first report on the use of aerosolized iloprost, a long-acting prostacyclin analogue, applied for treatment of postoperative right heart failure in cardiac transplantation. After uneventful surgery, a heart transplant recipient was extubated on the first postoperative day and exhibited right heart failure on the following day. Standard treatment did not resolve this clinical problem sufficiently. A trial with inhalation of iloprost (16 μg per inhalation cycle, applied six times per day) was initiated. Iloprost inhalation resulted in a sufficient decrease of pulmonary vascular resistance (–23.5%), while cardiac index (+24.0%) and mixed venous saturation (+9.0%) increased. No profound effect on the systemic vascular resistance was observed (−2.8%). Iloprost inhalation may be an effective alternative to nitric oxide in the intensive care management of acute right heart failure after orthotopic heart transplantation. Due to the prolonged effect on the pulmonary vascular resistance, iloprost is especially useful for extubated patients.