Introduction: Long-term benefits of medical treatment and mechanical support devices for restrictive cardiomyopathy (RCMP) patients remain unproven, emphasizing heart transplantation as the sole treatment to enhance survival and quality of life in this patient group. The aim of this study is to analyze pediatric patients diagnosed with RCM who underwent heart transplantation at our clinic.
Material and Methods: Data of 18 patients diagnosed with RCM who underwent heart transplantation at Başkent University Ankara Hospital between June 2007 and July 2023 were retrospectively reviewed. Of our patients, 7 were female and 11 were male, with mean age 10,5 (2, 17) years at the time of transplantation. The time interval from diagnosis to transplantation mean 44,9 (2-240) months. Patients with a measured pulmonary vascular resistance (PVR) at the upper limit for heart transplantation were listed for transplantation to prevent missing the chance for transplantation, even if heart failure symptoms were absent. Patients with low PVR were listed in an urgent status on the waiting list due to advanced stages of heart failure. The bicaval heart transplantation technique was used in all patients, with the left atrium plicated in two patients to reduce the size of the left atrium.
Results: The mean left ventricular ejection fraction of our patients was 52.9% (23-80), with both atrial sizes being larger than normal. Mean values from pre-transplant catheterization included mean pulmonary artery pressure of 27.8 (11-48) mmHg, mean transpulmonary gradient of 8.4 (1-23) mmHg and mean pulmonary vascular resistance index (PVRI) of 1.9 (0.29-5.63) WU·m². The total ischemia time and aortic cross-clamp time were 207.8 (121-274) minutes and 102.9 (59-153) minutes. In the postoperative period, temporary support devices were used in 4 patients, including ECMO in 3 patients and a ventricular assist device in 1 patient. In the early postoperative period, two patients with ECMO support unfortunately died. Additionally, within the first year following transplantation, four patients were lost, all exhibiting PVRI at the upper limit.
Conclusion: Due to long waiting times on the transplant list, many patients may develop irreversible pulmonary hypertension, resulting in loss of transplantation opportunity. Particularly in patients with RCM with PVRI above normal values, listing them on the waiting list in an urgent status without waiting for advanced heart failure stages after diagnosis is necessary to increase the chance of transplantation and improve survival.