Author + information
- K. Anitha Jayakumar, MD⁎ ( and )
- Daphne T. Hsu, MD, FACC
- ↵⁎Columbia University College of Physicians and Surgeons Pediatric Cardiology Children’s Hospital of New York 3959 Broadway BH 2 North New York, NY 10032
We appreciate the thoughtful comments from Drs. Pereira and Shirali on our recent article (1) and would like to take this opportunity to respond.
The power to detect significant differences in posttransplant survival between the Fontan patients and other etiologies was limited by the small number of Fontan patients (n = 24), and further subanalyses by type of surgery and era of transplant were not feasible. Congenital heart disease has consistently been identified as a risk factor for one-year mortality according to reports of the International Society for Heart and Lung Transplantation (2). The majority of patients with congenital heart disease undergoing transplantation have complex disease and single-ventricle physiology. Similar to our data, conditional survival after one year was not significantly different for the congenital heart disease patients compared to others.
Pulmonary resistance is often impossible to determine in this group of single-ventricle patients with multiple sources of pulmonary blood flow; however, mortality from right heart failure was not a cause of death in our series, except in the single patient who had undergone a Fontan takedown procedure for pulmonary hypertension. It is difficult to imagine that a patient who is surviving, even poorly, with Fontan physiology would have significant enough pulmonary hypertension leading to right heart failure following implantation of a two-ventricle heart.
Long-standing hepatic venous congestion was common among patients in our series. The mean pretransplant prothrombin time was 14.6 ± 2 s, with a range of 11 to 18 s and an international normalized ratio of 1.7 ± 0.7, with a range of 1 to 2.9. There was no difference in the preoperative coagulation profile among the three patients who died from hemorrhage and the other Fontan patients. Subtle abnormalities of coagulation may have been present in these patients; however, other causes of postoperative hemorrhage such as prolonged cardiopulmonary bypass time and perfusion injury to the liver may also have played a role.
Additional data from multicenter registries, such as the Pediatric Heart Transplant Study Group, will soon be available that allows further risk stratification for posttransplant outcome in these complex patients, and continued efforts to define the criteria for transplantation are essential (3). We appreciate the opportunity to respond to the letter by Drs. Pereira and Shirali.
- American College of Cardiology Foundation
- Jayakumar K.A.,
- Addonizio L.J.,
- Kichuk-Chrisant M.R.,
- et al.
- Bernstein D.,
- Naftel D.,
- Chin C.,
- et al.