Author + information
- Naveen L. Pereira, MD, FACC⁎ ( and )
- Girish Shirali, MD, FACC
- ↵⁎Medical University of South Carolina, Cardiology/Medicine, 135 Rutledge Avenue, Suite 1201, P.O. Box 250592, Charleston, SC 29425
It is with great interest that we read the study by Jayakumar et al. (1) on the outcome following cardiac transplantation after a failed Fontan or Glenn procedure. Because adults and children were included in the study population, it is difficult to draw meaningful comparisons to posttransplant survival data. Nevertheless, in the more recent era of overall pediatric and adult cardiac transplantation (1994 to 1998). International Society for Heart and Lung Transplantation (ISHLT) Kaplan-Meier survival rates are reported at 80% and 82% at one year and 74% and 70% at five years’ posttransplant, respectively (2,3). In this context, patient survival with cardiac transplantation after a failed Fontan procedure, reported by Jayakumar et al. as 63% at one year, and 57% at five years, is of concern (1). The investigators have reported five-year combined survival rates in both groups (post-Fontan and post-Glenn) in a later era (1996–2001) as 77%, but it would be useful to report the posttransplant survival in the failed Fontan patients separately in this later period as these patients seem to have a worse outcome than those transplanted after a Glenn procedure. The majority of deaths occurred within seven days after transplantation, and they were due to hemorrhage, multiorgan failure, sepsis, and right ventricular failure. The increased mortality posttransplant in the failed Fontan physiology patients might be due to various reasons.
First, an accurate determination of pulmonary vascular resistance is difficult in the failed Fontan patient population because of methodological inaccuracies that are implicit in the use of assumed oxygen consumption. Pretransplant pulmonary hypertension even in this modern era has consistently been a risk factor for poor outcomes.
Second, in our experience cardiac cirrhosis of the liver can occur especially in adults with failed Fontan physiology. Cardiac cirrhosis is variably encountered in adults with long-standing right heart failure and can be insidiously present and difficult to diagnose in the post-Fontan population owing to overlapping symptoms and signs attributable to the failed Fontan state, such as ascites, pleural effusions, and hypoproteinemia (4). Abdominal imaging studies such as computed tomography and ultrasound are useful in making the diagnosis by demonstrating a nodular liver. In selected cases, liver biopsy may be needed because the outcome of heart transplantation in patients with heart failure and dysfunction due to passive congestion of the liver is best determined by the extent of hepatic fibrosis demonstrated by histology (5).
Moreover, patients with significant cirrhosis have abnormal coagulation profiles, are prone to infections, and can suffer from hepatic decompensation postoperatively, thus adversely impacting survival following heart transplantation. It is therefore important to recognize the concept of cardiac cirrhosis and seek it in the failed Fontan patient who is being considered for heart transplantation. This may enable not only risk stratification but also, in severe cases, consideration of aggressive approaches such as combined heart and liver transplantation to help improve posttransplant survival in these high-risk patients.
- American College of Cardiology Foundation