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J Thorac Cardiovasc Surg 2008;136:529-531
© 2008 The American Association for Thoracic Surgery

Brief Communication

Beyond Berlin: Heart transplantation in the "untransplantable"

Sanjiv K. Gandhi, MDa,*, R. Mark Grady, MDb, Charles B. Huddleston, MDa, David T. Balzer, MDb, Charles E. Canter, MDa

a Division of Pediatric Cardiothoracic Surgery, Saint Louis Children's Hospital, Washington University School of Medicine, St Louis, Mo
b Division of Pediatric Cardiology, Saint Louis Children's Hospital, Washington University School of Medicine, St Louis, Mo

Received for publication November 14, 2007; accepted for publication January 9, 2008.

* Address for reprints: Sanjiv K. Gandhi, MD, Division of Cardiothoracic Surgery, Saint Louis Children's Hospital, Suite 5S50, 1 Children's Place, St Louis, MO 63110. (Email: gandhis@wustl.edu).

The first 20% of the full text of this article appears below.

Severe pulmonary hypertension unresponsive to acute pulmonary vasodilators—"fixed" or "irreversible" pulmonary hypertension—has traditionally been regarded as a contraindication to an orthotopic heart transplant. We report a novel strategy of combining mechanical biventricular assist device (BiVAD) support provided by the Berlin Heart EXCOR device (Berlin Heart AG, Berlin, Germany) with medical pulmonary vasodilator therapy in the cases of 2 children initially referred to our institution for a heart–lung transplant because of heart failure and presumed irreversible pulmonary hypertension. In both cases, our approach significantly improved the pulmonary hypertension, permitting an orthotopic heart transplant alone, a procedure with a much better long-term prognosis than a heart–lung transplant.

Case Reports

Patient 1
Patient 1 was a 2-year-old boy with congenitally corrected transposition of the great arteries, congenital heart block, and a small ventricular septal defect. He had undergone implantation of a single-chamber epicardial pacemaker as a neonate. He was seen at 2 years with worsening ventricular failure. Cardiac catheterization at that time demonstrated a pulmonary vascular resistance (PVR) of 15 Woods units/m2 and a transpulmonary gradient of 40 mm Hg, unresponsive to oxygen or inhaled nitric oxide (Go Table 1).


View this table:



  		Table 1 Patient 1's hemodynamic variables at baseline and after interventions
 
Initiation of intravenous epoprostenol therapy and placement of a dual-chamber biventricular pacemaker in an attempt to optimize ventricular function and reduce PVR were poorly tolerated and resulted in significant cardiac instability. Ultimately, the child was placed on extracorporeal membrane oxygenation support. After 8 days of extracorporeal membrane oxygenation, the patient was transitioned to mechanical BiVAD support with the EXCOR system. The patient was weaned from extracorporeal circulation without difficulty and extubated at 1 week. He was maintained on a regimen . . . [Full Text of this Article]




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