Clinical lung and heart/lung transplantation
Heart–lung or lung transplantation for Eisenmenger syndrome

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Abstract

Background:

The optimal therapy for end-stage Eisenmenger syndrome (ES) is unknown. We analyzed the United Network for Organ Sharing/International Society for Heart and Lung Transplantation Joint Thoracic Registry to determine predictors of survival.

Methods:

Univariate analysis was performed using Kaplan–Meier survival curves. Groups were compared using the log-rank test. Multivariate analysis was performed using a proportional hazards model.

Results:

There were 605 transplants performed between 1988 and 1998. The causes of ES included atrial septal defect (ASD) in 171, ventricular septal defect (VSD) in 164, multiple congenital anomalies (MCA) in 68 and patent ductus arteriosus (PDA) in 32. Procedures included 430 heart–lung (HLT), 106 bilateral lung, and 69 single lung transplants (LT). Survival after HLT was better than after LT on univariate analysis (p = 0.002). For HLT, survival at 30 days and 1 year was 80.7% and 70.1% compared with 68% and 55.2% for LT. Diagnosis was also a significant predictor of survival (p = 0.011), being best for VSD and MCA (1-year survival 71.4% and 77.6%). There was a highly significant benefit of HLT over LT for VSD patients (p = 0.0001). Diagnosis, the combination of diagnosis and procedure, recipient age, recipient gender, donor age, ischemic time and recipient status were significant in a multivariate model. Multivariate analysis confirmed the superior prognosis of patients with VSD or MCA (p = 0.007 and p = 0.022, respectively) and suggested that the adverse effect of LT was predominately in patients with VSD (risk ratio 1.817, p = 0.035).

Conclusions:

This analysis suggests that ES recipients are not a homogeneous group. Patients with VSD and MCA have the best prognosis. HLT appears to offer a survival benefit for patients with ES secondary to VSD and should be re-considered as the operation of choice.

Section snippets

Methods

The ISHLT/UNOS Joint Thoracic Registry prospectively collects demographic and outcome data on heart, heart–lung and lung transplantation performed around the world. The Registry contains data on 605 patients with ES transplanted between January 1988 and December 1998. Three procedures were performed as living-related transplants and were excluded from further analysis. Follow-up data were available for 95% of patients (576). Survival analysis was performed on only those patients with available

Results

The causes of ES are shown in Figure 1A. Atrial septal defect was the most common cause. The cause was not indicated to the Registry in 28% of the cases. Heart–lung transplantation was the most commonly employed procedure, constituting 71%, as shown in Figure 1B. Bilateral lung transplantation was used more commonly than SLT.

As expected, lung transplantation was applied more commonly in cases with simple cardiac problems. Recipients with patent ductus arteriosus (PDA) received lung

Discussion

There are three important findings of this study. First, patients with ES are not functionally equivalent to patients with PPH. Second, patients with ES comprise a heterogeneous group, with significant differences in survival after transplantation depending on the cause of ES. Third, HLT appears to be the operation of choice, especially for patients with VSD.

The strengths of this study include the large number of patients, the fact that the data were collected prospectively by data managers

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