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Cardiac transplantation for end-stage congenital heart defects: the Mayo Clinic experience. Mayo Cardiothoracic
G Speziali1, D J Driscoll, G K Danielson
1Division of Thoracic and Cardiovascular Surgery, Mayo Clinic Rochester, MN 55905, USA.
Insights
Cardiac transplantation for congenital heart disease offers excellent survival rates. This study shows successful outcomes in patients with complex congenital heart defects, despite surgical challenges and the need for lifelong medication.
Area of Science:
- Cardiology
- Cardiac Surgery
- Transplantation Medicine
Background:
- Congenital heart defects (CHDs) represent a significant challenge in cardiac surgery.
- Many patients with CHDs require complex, multi-stage palliative procedures.
- Cardiac transplantation is an option for end-stage heart failure in CHD patients.
Purpose of the Study:
- To evaluate the outcomes of cardiac transplantation in patients with congenital heart defects.
- To assess the feasibility and survival rates of this procedure in a complex patient population.
Main Methods:
- A review of 16 patients with various congenital heart diseases who underwent cardiac transplantation between 1991 and 1998.
- Patients had a history of one to five previous palliative operations.
- Preoperative diagnoses included a range of complex CHDs such as univentricular heart and complete transposition of the great arteries.
Main Results:
- Actuarial 1-, 2-, and 5-year survival rates were 86.2 +/- 9.1%.
- Two early deaths occurred within the first year due to vascular and cellular rejection.
- Most patients experienced functional rehabilitation, with a mean follow-up of 26.1 months.
Conclusions:
- Cardiac transplantation is a viable option for patients with end-stage congenital heart disease.
- The procedure can be performed with low perioperative mortality and excellent medium-term survival.
- Despite technical challenges and the need for long-term immunosuppression, outcomes are favorable.
Objective:
To review the outcome of cardiac transplantation undertaken in patients with congenital heart defects.
Material And Methods:
Between November 1991 and March 1998 at our institution, cardiac transplantation was performed in 16 patients with congenital heart disease (age range, 3 to 57 years; mean, 26.1). Preoperative diagnoses included univentricular heart (N = 4); complete transposition of the great arteries (N = 3); Ebstein's anomaly (N = 2); tetralogy of Fallot (N = 2); levotransposition (N = 2); dextrocardia, corrected transposition, ventricular and atrial septal defects, and pulmonary stenosis (N = 1); double-outlet right ventricle (N = 1); and hypertrophic obstructive cardiomyopathy (N = 1). All patients had undergone from one to five previous palliative operations.
Results:
Four patients required permanent pacemaker implantation during the first month postoperatively because of bradycardia; more than 2 years later, another patient required a permanent pacemaker because of sick sinus syndrome. In addition, one patient had an automatic implantable cardioverter-defibrillator. Three patients required reconstruction of cardiovascular structures with use of prosthetic material (Teflon patches or donor tissue) at the time of cardiac transplantation. Actuarial 1-, 2-, and 5-year survival was 86.2 +/- 9.1%. During the first year after transplantation, two deaths occurred--one at 41 days of putative vascular rejection and the second at 60 days of severe cellular rejection. All other patients are alive and functionally rehabilitated; the mean follow-up period has been 26.1 months (range, 2 to 89.6).
Conclusion:
Cardiac transplantation for patients with congenital heart disease can be accomplished with a low perioperative mortality and an excellent medium-term survival despite the challenges presented by the technical difficulties during invasive diagnostic procedures and at operation and the need for adherence to long-term multiple-drug therapy in this patient population.
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