Inhaled iloprost as a bridge to heart transplantation in a patient with endomyocardial fibroelastosis and severe pulmonary hypertension

Abstract

Background: Endomyocardial fibrosis (EMF) is an endemic disease which can lead to partial obliteration of one or both ventricles with decreased ventricular distensibility and impaired diastolic filling. Results: We describe the intra- and postoperative course of an 18-year-old female patient undergoing orthotopic heart transplantation (HTx) because of EMF. Her body surface area was 1.18 m2. Blood pressure was 106/63mmHg during paroxysmal atrial fibrillation. She continously received an i.v. medication of 5µg/kg/min dopamine and 0.75µg/kg/min milrinone. Pulmonalis catheter examination revealed a PA of 83/39mmHg; mean pulmonary artery pressure (PAP) was 53mmHg; post-capillary wedge pressure was 15mmHg; trans-pulmonary gradient was 38mmHg; pulmonary vascular resistance (PVR) was 1113 dyn, systemic vascular resistance (SVR) was 2051 dyn. She received 50µg/day inhaled iloprost (Ilomedin®). This therapy was able to keep the preoperative PVR/SVR ratio below 30%. On day 33 of her hospitalisation, HTx was performed. Postoperative hemodynamics and respiratory conditions were stable without inhaled iloprost or nitric oxide. On postoperative day 3, however, iloprost therapy had to be restarted because of an increase in PAP levels. On postoperative day 24, the patient was discharged. Iloprost therapy was continued for additional 2 months then stopped since right heart catheter examination revealed hemodynamics within normal ranges and routine biopsy was grade 0. Conclusion: Inhaled iloprost, a long-acting prostacyclin analogue, can ameliorate pulmonary hypertension very successfully. In this case inhaled iloprost therapy was able to avoid the previously considered combined heart-lung transplantation. The 1-year follow data demonstrate the complete disappearance of pulmonary hypertension.