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J Thorac Cardiovasc Surg 2001;122:169-177
© 2001 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease (CHD)

Pulmonary atresia with ventricular septal defect and persistent airway hyperresponsiveness

Michael J. Ackerman, MD, PhD, Mark E. Wylam, MD, Robert H. Feldt, MD, Coburn J. Porter, MD, Gordon Dewald, PhD, Paul D. Scanlon, MD, David J. Driscoll, MD

From the Departments of Pediatric and Adolescent Medicine, Cytogenetics, and Internal Medicine, Mayo Eugenio Litta Children's Hospital, Mayo Clinic/Mayo Foundation, Rochester, Minn.

This study was supported by a Resident Research award (M.J.A.) from the American Academy of Pediatrics and a Beginning Grant-in-Aid research award (M.J.A.) from the Minnesota Affiliate of the American Heart Association.

Preliminary findings from this study were presented at the 1998 Annual Meeting of the Pediatric Academic Society (May 3, 1998) in New Orleans, La.

Received for publication Nov 22, 2000. Revisions requested Jan 22, 2001; revisions received Feb 2, 2001. Accepted for publication Feb 7, 2001. Address for reprints: Michael J. Ackerman, MD, PhD, Department of Pediatric and Adolescent Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905. (E-mail: ackerman.michael{at}mayo.edu).

Abstract

Objective: We and others have observed significant hyperinflation and airflow obstruction after the surgical repair of pulmonary atresia and ventricular septal defect. This study sought to objectively characterize this problem and determine the prevalence of airway hyperresponsiveness in these patients.
Methods: We performed a prospective study of children and young adults with pulmonary atresia and ventricular septal defect between June 1996 and December 1998. The participants were stratified into 2 distinct molecular genotypes on the basis of chromosome 22q11.2 microdeletion. A clinical diagnosis of asthma and an objective assessment of airway hyperresponsiveness were determined by means of an asthma inventory scale and methacholine challenge testing, respectively. Thirty-three patients were enrolled. Thirteen had velocardiofacial syndrome, each with chromosome 22q11.2 microdeletion.
Results: None of the nonsyndromic patients had evidence for haploinsufficiency. Overall, 66.7% (22/33) met criteria for a clinical diagnosis of airway hyperresponsiveness: 62% (8/13) from the microdeletion genotype and 70% (14/20) from the nonsyndromic group.
Conclusions: We have identified an extremely strong association between pulmonary atresia and ventricular septal defect and persistent airway hyperresponsiveness. Haploinsufficiency at chromosome 22q11.2 did not contribute to this predilection for airway hyperresponsiveness. These results provide a basis to anticipate persistent respiratory difficulties after operations in patients with pulmonary atresia and ventricular septal defect. Moreover, this at-risk patient population may yield unique insights into fundamental mechanisms involved in the pathogenesis of airway hyperresponsiveness.




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