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CLINICAL STUDIES

Posttransplant recoarctation of the aorta: a twelve year experience

Girish S. Shirali, MD, FACC^a, Constance E. Cephus, RN, MSN, NNP, Micheal A. Kuhn, MD, FACC^a, Kay K. Ogata, RN, MS, Laura K. Vander Dussen, RN, BS, Richard E. Chinnock, MD^a, Neda F. Mulla, MD, FACC^a, Joyce K. Johnston, RN, BS, Leonard L. Bailey, MD, FACC, Steven R. Gundry, MD, FACC, Anees J. Razzouk, MD, FACC and Ranae L. Larsen, MD, FACC^a

^a Department of Pediatrics, Loma Linda University Children’s Hospital, Loma Linda, California, USA
Department of Nursing, Loma Linda University Children’s Hospital, Loma Linda, California, USA
Department of Surgery, Loma Linda University Children’s Hospital, Loma Linda, California, USA

Manuscript received October 16, 1997; revised manuscript received April 3, 1998, accepted April 22, 1998.

Address for correspondence: Girish S. Shirali, MD, Pediatric Cardiology, Loma Linda University School of Medicine, 11234 Anderson Street, Rm. 4433, Loma Linda, California 92354
gshirali{at}ccmail.llu.edu

Objectives. This study was undertaken to investigate the incidence of posttransplant recoarctation of the aorta, delineate the mode of presentation, identify risk factors that predict recoarctation and examine the results of intervention for posttransplant recoarctation.

Background. Patients with aortic arch hypoplasia require extended arch reconstruction at transplant, with an inherent possibility of subsequent recoarctation of the aorta.

Methods. This was a retrospective review of all children (age <18 years) who underwent cardiac transplantation over a 10-year period. Collected data included pretransplant diagnosis, details of the transplant procedure and posttransplant data including development of recoarctation of the aorta, interventions for recoarctation and the most recent follow-up assessment of the aortic arch.

Results. Two hundred eighty-eight transplants were performed on 279 children (follow-up = 1,075 patient-years; range 0 to 133 months, median 43.7). Thirty-two of 152 patients (21%) who underwent extended aortic arch reconstruction subsequently developed recoarctation. All but one patient developed recoarctation within 2 years after transplant; 87% were hypertensive at presentation. Of 30 patients who underwent intervention for recoarctation (balloon angioplasty [n = 26] and surgical repair of recoarctation [n = 4]), 26 (87%) have remained recurrence-free (follow-up = 133 patient-years; range 8 to 106 months, median 47).

Conclusions. The high frequency of recoarctation after cardiac transplantation with extended aortic arch reconstruction mandates serial echocardiographic evaluation of the aortic arch. Patients typically present with systemic hypertension within the first two years after transplantation. Balloon angioplasty is a safe, effective and durable method of treatment.

Abbreviations and Acronyms   HLHS = hypoplastic left heart syndrome