Zuckerman et al

Congenital Heart Disease: Transplantation

Heart transplantation in the setting of complex congenital heart disease and physiologic single lung Warren A. Zuckerman, MD,a Marc E. Richmond, MD, MS,a Teresa M. Lee, MD,a Emile A. Bacha, MD,b Paul J. Chai, MD,b Jonathan M. Chen, MD,c and Linda J. Addonizio, MDa ABSTRACT

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 ¼ 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. (J Thorac Cardiovasc Surg 2015;150:1465-72)

Orthotopic heart transplantation (OHT) is a successful option for treatment of end-stage heart failure due to congenital heart disease (CHD). An International Society of Heart and Lung Transplantation (ISHLT) review of pediatric heart transplants performed in North America between 2000 and 2012 found approximately 40% occurred in patients with From the aDivision of Pediatric Cardiology, Columbia University Medical Center, New York, NY; bDepartment of Pediatric Cardiothoracic Surgery, Columbia University Medical Center, New York, NY; and cDepartment of Pediatric Cardiothoracic Surgery, Seattle Children’s Hospital, Seattle, Wash. Received for publication April 1, 2015; revisions received May 22, 2015; accepted for publication June 6, 2015; available ahead of print July 16, 2015. Address for reprints: Warren A. Zuckerman, MD, Division of Pediatric Cardiology, Columbia University Medical Center, Babies Hospital, 2 N, Rm 229, 3959 Broadway, New York, NY 10032 (E-mail: [email protected]). 0022-5223/$36.00 Copyright Ó 2015 by The American Association for Thoracic Surgery http://dx.doi.org/10.1016/j.jtcvs.2015.06.021

CHD

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.

Kaplan-Meier survival analysis for patients undergoing orthotopic heart transplantation to single lung (n ¼ 22) versus controls (n ¼ 67). Central Message Heart transplantation alone is an excellent option in patients with congenital heart disease with physiologic single lung. Perspective Patients with congenital heart disease may have impaired blood flow to 1 lung. Outcomes after heart transplant in these patients are comparable to patients with similar anatomy and dual lung physiology, and superior to heart–lung transplant. With careful preoperative evaluation and meticulous postoperative management, physicians should feel comfortable referring these patients for heart transplant.

See Editorial Commentary page 1473.

CHD.1 Due to improvements in congenital heart surgery, CHD has been the indication for OHT less often in pediatrics in the recent era; however, with increasing numbers of teens and young adults with CHD, OHT has become increasingly more common in these patients.1-3 Although outcomes in patients with CHD across all age groups are slightly worse than in patients with cardiomyopathy, 10year survival is well >50% in adults and adolescents, and >60% in infants and children, with era-effect improvements noted.1-3 Patients with complex CHD may have impaired blood flow to 1 lung. This may occur due to congenital or acquired absence/stenosis of the right pulmonary artery (RPA) or left pulmonary artery (LPA), branch pulmonary artery (PA) thrombosis, unilateral pulmonary hypertension as a result of prior aortopulmonary shunt or collaterals, or as a result

The Journal of Thoracic and Cardiovascular Surgery c Volume 150, Number 6

1465

Congenital Heart Disease: Transplantation

CHD

Abbreviations and Acronyms CHD ¼ congenital heart disease iNO ¼ inhaled nitric oxide ISHLT ¼ International Society of Heart and Lung Transplantation LPA ¼ left pulmonary artery LV ¼ left ventricle OHT ¼ orthotopic heart transplantation PA ¼ pulmonary artery PRA ¼ panel reactive antibody PVR ¼ pulmonary vascular resistance PVRI ¼ pulmonary vascular resistance indexed to body surface area RPA ¼ right pulmonary artery RV ¼ right ventricle TOF ¼ tetralogy of Fallot of partial pulmonary venous obstruction.4,5 In these cases, the unaffected lung may have relatively normal resistance in the setting of increased blood flow, leading to effective single lung physiology. Although rare, this most often occurs in patients with tetralogy of Fallot (TOF) and pulmonary atresia or shunted single ventricle lesions. These patients were often referred for heart–lung transplantation in the past, which carries high morbidity and mortality risks. An alternative is OHT to the physiologic single lung, which was reported in 11 patients from our institution more than a decade ago,4 finding similar survival to patients with complex CHD with dual lung physiology, and improved survival compared with patients undergoing heart–lung transplant. Additional reports of OHT to physiologic single lung remain absent from the literature, and with now more than 20 years of experience using this therapeutic strategy, we seek to update the world’s largest reported cohort, demonstrating the lessons learned and favorable outcomes of OHT in the setting of single lung physiology. METHODS A review of all patients at our institution who underwent OHT for CHD and physiologic single lung was performed. Single lung physiology was defined as absence of flow unilaterally by catheterization or

Heart transplantation in the setting of complex congenital heart disease and physiologic single lung.

To highlight the success of heart transplantation in patients with complex congenital heart disease and physiologic single lung by providing an update...
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