Heart Transplantation in a 14-Year-Old Boy in the Presence of Severe Out-of-Proportion Pulmonary Hypertension due to Restrictive Left Heart Disease: A Case Report.
A 14-year-old boy after balloon valvuloplasty of severe aortic valve stenosis in the neonatal period was referred for heart-lung transplantation because of high grade pulmonary hypertension and left heart dysfunction due to endocardial fibroelastosis with severe mitral insufficiency. After heart catheterization, hemodynamic parameters were invasively monitored: a course of levosimendan and initiation of diuretics led to a decrease of pulmonary capillary wedge pressure (from maximum 35 to 24 mmHg). Instead of an expected decrease, mean pulmonary artery pressures (mPAP) increased up to 80 mmHg with increasing transpulmonary pressure gradient (TPG) up to 55 mmHg. Oral bosentan and intravenous epoprostenol then led to a ~50% decrease of mPAP (TPG between 16 and 22 mmHg). The boy was listed solely for heart transplantation which was successfully accomplished 1 month later.
Department of Pediatrics, Division of Cardiology, Pulmology, Allergology and Cystic Fibrosis, Innsbruck Medical University, 6020 Innsbruck, Austria.
Martin
M
Schwienbacher
Department of Pediatrics, Division of Cardiology, Pulmology, Allergology and Cystic Fibrosis, Innsbruck Medical University, 6020 Innsbruck, Austria.
Ulrich
U
Schweigmann
Department of Pediatrics, Pediatric Intensive Care Unit, Innsbruck Medical University, 6020 Innsbruck, Austria.
Nikolaus
N
Neu
Department of Pediatrics, Division of Cardiology, Pulmology, Allergology and Cystic Fibrosis, Innsbruck Medical University, 6020 Innsbruck, Austria.
Elisabeth
E
Schermer
Department of Anaesthesiology and Critical Care Medicine, Innsbruck Medical University, 6020 Innsbruck, Austria.
Corinna
C
Velik-Salchner
Department of Pediatric Cardiology, Medical University of Vienna, 1090 Vienna, Austria.
Ina
I
Michel-Behnke
Department of Pediatrics, Salzburg State Hospital, 5020 Salzburg, Austria.
Erentraud
E
Irnberger
Department of Pathology, Innsbruck Medical University, 6020 Innsbruck, Austria.
Christina Maria
CM
Steger
Department of Pediatrics, Division of Cardiology, Pulmology, Allergology and Cystic Fibrosis, Innsbruck Medical University, 6020 Innsbruck, Austria.
Jörg Ingolf
JI
Stein
Department of Pediatrics, Division of Cardiology, Pulmology, Allergology and Cystic Fibrosis, Innsbruck Medical University, 6020 Innsbruck, Austria.
Ralf
R
Geiger