Heart and unilateral lung transplantation in patients with end-stage cardiopulmonary disease and previous thoracic operations

Orthotopic en bloc transplantation of the heart and one lung has been done in two patients with end-stage cardiopulmonary disease and a prior thoracic operation. The first patient had undergone right pulmonary thromboembolectomy with caval ligation 5 years earlier, and the second had had left lower lobectomy for bronchiectasis 15 years before the heart and contralateral lung transplantation. Surgical procedures followed the techniques that had been developed in animals. Transplantation of the unoperated contralateral lung made it possible to avoid dissection in the obliterated pleural space and to minimize bleeding, which simplified the procedure considerably. Dramatic reduction in pulmonary artery pressure and improved respiratory function allowed both patients to be weaned from cardiopulmonary bypass without problems. Although the first patient died of liver and renal failure soon after the operation, an intact cough reflex facilitated recovery in the second patient, who has been discharged with essentially normal respiratory function. This report describes heart and unilateral lung transplantation as a procedure of choice for patients with extensive pleural adhesions that made total cardiopulmonary replacement unfeasible.     

A new method for reliable fenestration in extracardiac conduit Fontan operations

A fenestrated extracardiac conduit Fontan operation was performed with a new method in 5 patients by means of a pericardial tube anastomosed end to end with the inferior inlet of the right atrium.     

Improving results of the modified Fontan operation in patients with heterotaxy syndrome

BACKGROUND: Historically the Fontan operation in patients with single ventricle heterotaxy syndrome and atrial isomerism has been associated with high mortality. We studied whether recent modifications of the surgical technique have improved outcome. METHODS: A retrospective review of 135 patients with heterotaxy syndrome who underwent a Fontan operation between 1981 and 2000 was performed. RESULTS: There were 93 patients with right isomerism and 42 with left isomerism. Anomalies of venous return included 25 patients with extracardiac pulmonary venous connection (19%) and 37 patients with an interrupted inferior vena cava (27%). Thirty-six patients (27%) had at least moderate atrioventricular valve regurgitation. The type of Fontan procedure included 17 patients with an atriopulmonary Fontan connection, 67 with a lateral tunnel modification, 19 with an intraatrial tube graft, 25 with an extracardiac tubegraft, and 7 with an intra-extra atrial tube graft. A fenestration was placed in 93 patients (78%). Early mortality was 19% before 1991, 3% since 1991, and no patient has died early since 1993. Ten-year survivals were 70% for Fontan operations before 1990 and 93% for Fontan operations after 1990. Thirty-two patients (23%) had prolonged pleural effusions. Risk factors for death included anomalous pulmonary venous connection (p = 0.02) and higher preoperative pulmonary vascular resistance (p = 0.002). Sixty-two patients (47%) had some form of early postoperative arrhythmia. At 10 years, freedom from late bradyarrhythmia and late tachyarrhythmia were 78% and 70%, respectively. Preoperative arrhythmias, older age at operation, and anatomic features were each independent predictors of late arrhythmia. CONCLUSIONS: The Fontan operation can now be performed in patients with heterotaxy syndrome with excellent survival. However, morbidity in terms of postoperative arrhythmias and prolonged pleural effusions remains significant. Fontan staging, appropriate choice of Fontan modification, aggressive treatment of concomitant malformations, and use of a baffle fenestration contribute to improved outcome.     

Heart transplantation in diabetic recipients: a decade review of 161 patients at Columbia Presbyterian

OBJECTIVE: Diabetes is considered by some transplant centers to be a relative contraindication for cardiac transplantation because of concerns regarding decreased survival, as well as increased incidence of infection and transplant coronary artery disease. We evaluated our experience with diabetic recipients over the last 10 years. METHODS: From January 1992 through June 2002, 881 patients underwent cardiac transplantation at New York Presbyterian Hospital. Of these, 161 (18.3%) were diabetic patients. Diabetic recipients were compared with a control group of 161 nondiabetic recipients matched for age, sex, cause of heart failure, United Network for Organ Sharing status, and immunosuppression era. Outcome measures included posttransplantation survival, incidence of infection, rejection, and transplant coronary artery disease. RESULTS: There was no statistically significant difference in survival between diabetic and nondiabetic recipients, with actuarial survival at 1, 5, and 10 years of 89.3%, 66.9%, and 45.6%, respectively, for diabetic patients and 87.4%, 78.8%, and 59.1%, respectively, for nondiabetic patients (P =.168). There was no significant difference in freedom from infection, rejection, or transplant coronary artery disease between the groups. By using Cox proportional hazard models, development of infection, rejection, and transplant coronary artery disease were independent predictors of decreased survival (P <.001, P =.004, and P =.004, respectively). CONCLUSIONS: These results demonstrate similar short-term and long-term survivals, as well as similar risks for infection and transplant coronary artery disease, in diabetic and nondiabetic patients undergoing cardiac transplantation. The trend toward worse survival in the diabetic cohort, however, raises the possibility that if a greater number of diabetic patients were evaluated, a significant difference in survival might be observed, suggesting the need for a multicenter analysis to validate these outcomes.     

Heart transplantation in a patient with isolated noncompaction of the left ventricular myocardium

We describe a patient with isolated noncompaction of the left ventricle who presented with worsening congestive heart failure and was successfully treated with heart transplantation. The prognosis for these patients is poor because of accelerated event rates of fatal arrhythmias, thromboemboli, and profound left ventricular decompensation. Only 7 patients with isolated noncompaction of the left ventricle have been reported to have undergone heart transplantation. Herein we describe a patient with isolated noncompaction of the left ventricle who underwent successful heart transplantation.     

Coagulation factor abnormalities in patients with single-ventricle physiology immediately prior to the Fontan procedure

Background. Coagulation abnormalities have been reported following the Fontan operation and have been attributed to various aspects of Fontan-associated physiology. Using age-matched controls, this study evaluated coagulation abnormalities in children who had undergone a bidirectional Glenn procedure to test the hypothesis that coagulation abnormalities are present before the Fontan operation.

Methods. Coagulation factors were assayed in 38 children (mean age 34.4 ± 15 months) immediately before the Fontan operation; 37 healthy children (mean age 33 ± 17 months) were assayed as controls. Concentration of factors II, V, VII, VIII, IX, and X and of antithrombin III, plasminogen, proteins C and S, fibrinogen, serum albumin, and liver enzymes were measured. Normal reference intervals based on the control patients were determined using 95% confidence limits. Patient demographic data, hemodynamic variables, and elapsed time after the Glenn procedure were evaluated as possible predictors of coagulation abnormalities.

Results. Concentrations of protein C; factors II, V, VII, and X; plasminogen; and antithrombin III were significantly lower before the Fontan operation compared with age-matched controls (p < 0.01); no specific hemodynamic variables were predictive of a pro- or anticoagulant deficiency. There were significant positive correlations between patients who had abnormally low factor VII, protein S, and protein C levels and a longer interval between the bidirectional Glenn procedure and the Fontan operation (p < 0.001).

Conclusions. Coagulation abnormalities that could predispose patients to increased risk for clotting or bleeding are evident early in the course of staged single-ventricle repair.