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Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Heart transplantation in the setting of complex congenital heart disease and physiologic single lung
Warren A Zuckerman1, Marc E Richmond1, Teresa M Lee1
1Division of Pediatric Cardiology, Columbia University Medical Center, New York, NY.
Insights
Heart transplantation is a successful option for patients with complex congenital heart disease and single lung physiology. Outcomes are comparable to those with dual lung physiology, demonstrating excellent long-term results.
Area of Science:
- Cardiology
- Thoracic Surgery
- Transplantation Medicine
Background:
- Complex congenital heart disease (CCHD) presents unique challenges for heart transplantation.
- Physiologic single lung in CCHD patients requires specialized transplant considerations.
Purpose of the Study:
- To update on the largest reported cohort of heart transplantation in patients with CCHD and single lung physiology.
- To evaluate the success and outcomes of heart transplant to single lung.
Main Methods:
- Retrospective data collection on patients undergoing heart transplant to single lung since 1992.
- Comparison of single lung transplant cohort with controls undergoing transplants for single ventricle or tetralogy of Fallot.
Main Results:
- Twenty-two patients underwent heart transplant to single lung, with comparable demographics and pre-transplant support to controls.
- Similar median extubation time, inotrope use, and length of stay were observed.
- No significant difference in survival between the single lung group and controls.
Conclusions:
- Heart transplantation alone is an excellent option for CCHD patients with single lung physiology.
- Outcomes are comparable to patients with similar cardiac anatomy and dual lung physiology.
- This approach offers a viable solution for complex cardiac conditions.
Objective:
To highlight the success of heart transplantation in patients with complex congenital heart disease and physiologic single lung by providing an update on the world's largest reported cohort.
Methods:
Demographic, perioperative, postoperative, and outcomes data were collected retrospectively on all patients undergoing heart transplant to single lung at Columbia University Medical Center since 1992, and compared with all other patients undergoing transplants performed for single ventricle or tetralogy of Fallot during that time.
Results:
Twenty-two patients (mean age, 20.6 years; range, 5 months-47 years) underwent heart transplant to single lung. Compared with controls (n = 67), the single lung group had more male patients and a greater proportion of tetralogy compared with single ventricle patients, although the single lung group had fewer post-Fontan patients. Age, weight, and body surface area were similar between the groups as were use of mechanical circulatory support and mechanical ventilation before transplant. Median time to extubation, time on inotropes, and length of stay were similar. There were 3 perioperative deaths, including a patient who died during postoperative day 1 from primary graft failure, likely related to a combination of elevated pulmonary vascular resistance and volume load. There were 5 additional mortalities during intermediate- and long-term follow-up, none of which were related to single-lung physiology. There was no significant survival difference between the groups.
Conclusions:
In patients with complex congenital heart disease and single lung physiology, heart transplant alone remains an excellent option, with comparable outcomes to patients undergoing transplant with similar cardiac anatomy and dual lung physiology.

