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Updated: Jul 10, 2026
A Simplified Model for Heterotopic Heart Valve Transplantation in Rodents
Published on: September 21, 2021
Twenty-year experience with heart transplantation for infants and children with restrictive cardiomyopathy: 1986-2006
A J Bograd1, S Mital, J C Schwarzenberger
1Division of Cardiothoracic Surgery, Department of Surgery, College of Physicians and Surgeons, Columbia University, New York, NY, USA.
Insights
Heart transplantation (HTX) is a viable option for children with restrictive cardiomyopathy (RCM), even those with high pulmonary vascular resistance indices (PVRI). This study shows excellent survival rates, challenging previous exclusion criteria for HTX in RCM patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Transplantation Medicine
Background:
- Idiopathic restrictive cardiomyopathy (RCM) in children presents with severe diastolic dysfunction and elevated pulmonary vascular resistance (PVR).
- Historically, patients with pulmonary vascular resistance indices (PVRI) exceeding 6 Wood Units x m² were excluded from heart transplantation (HTX).
Purpose of the Study:
- To evaluate the clinical outcomes and survival rates of pediatric patients with RCM who underwent heart transplantation.
- To assess the impact of preoperative pulmonary vascular resistance indices (PVRI) on post-transplant outcomes.
Main Methods:
- Retrospective review of 23 pediatric patients who underwent HTX for RCM between 1986 and 2006.
- Analysis of preoperative, intraoperative, and postoperative variables, including PVRI and pulmonary artery (PA) pressure.
- Evaluation of short-term and long-term survival rates and factors associated with outcomes.
Main Results:
- Mean age at HTX was 8.8 years; mean follow-up was 5.7 years.
- Preoperative PVRI averaged 5.9 Wood Units x m², decreasing to 2.0 Wood Units x m² at follow-up.
- Higher preoperative PVRI (>6 Wood Units x m²) and mean PA pressure were associated with the need for ECMO postoperatively, but not with mortality.
- 30-day and 1-year actuarial survival rates were 96% and 86%, respectively.
- Five of seven patients with preoperative PVRI >6 Wood Units x m² survived the first postoperative year.
Conclusions:
- Heart transplantation can achieve excellent survival rates in pediatric patients with RCM, including those previously considered high-risk due to elevated PVRI.
- Preoperative PVRI and mean PA pressure are not absolute contraindications for HTX in RCM and should not preclude consideration for transplantation.
- These findings support expanding eligibility criteria for HTX in pediatric RCM patients with significant pulmonary hypertension.
Abstract:
Idiopathic restrictive cardiomyopathy (RCM) is a rare cardiomyopathy in children notable for severe diastolic dysfunction and progressive elevation of pulmonary vascular resistance (PVR). Traditionally, those with pulmonary vascular resistance indices (PVRI) >6 W.U. x m(2) have been precluded from heart transplantation (HTX). The clinical course of all patients transplanted for RCM between 1986 and 2006 were reviewed. Preoperative, intraoperative and postoperative variables were evaluated. A total of 23 patients underwent HTX for RCM, with a mean age of 8.8 +/- 5.6 years and a mean time from listing to HTX of 43 +/- 60 days. Preoperative and postoperative (114 +/- 40 days) PVRI were 5.9 +/- 4.4 and 2.9 +/- 1.5 W.U. x m(2), respectively. At time of most recent follow-up (mean = 5.7 +/- 4.6 years), the mean PVRI was 2.0 +/- 1.0 W.U. x m(2). Increasing preoperative mean pulmonary artery pressure (PA) pressure (p = 0.04) and PVRI > 6 W.U. x m(2) (chi(2)= 7.4, p < 0.01) were associated with the requirement of ECMO postoperatively. Neither PVRI nor mean PA pressure was associated with posttransplant mortality; 30-day and 1-year actuarial survivals were 96% and 86%, respectively. Five of the seven patients with preoperative PVRI > 6 W.U. x m(2) survived the first postoperative year. We report excellent survival for patients undergoing HTX for RCM despite the high proportion of high-risk patients.
