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Christopher A. Caldarone
Hani K. Najm
William G. Williams
John G. Coles
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Ann Thorac Surg 1998;66:1514-1520
© 1998 The Society of Thoracic Surgeons

Relentless pulmonary vein stenosis after repair of total anomalous pulmonary venous drainage

Christopher A. Caldarone, MDa, Hani K. Najm, MDa, Margit Kadletz, MDa, Jeffrey F. Smallhorn, MDa, Robert M. Freedom, MDa, William G. Williams, MDa, John G. Coles, MDa

a Division of Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto and University of Toronto Faculty of Medicine, Toronto, Canada

Address reprint requests to Dr Caldarone, Division of Cardiovascular Surgery, The Children’s Hospital of Iowa at the University of Iowa Hospitals and Clinics, 200 Hawkins Dr 1616A JCP, Iowa City, IA 52242-1083
e-mail: (Chris-Caldarone{at}UIowa.edu)

Presented at the Thirty-fourth Annual Meeting of The Society of Thoracic Surgeons, New Orleans, LA, Jan 26–28, 1998.

Background. Progressive stenosis of the pulmonary veins after repair of total anomalous pulmonary venous drainage is frequently refractory to surgical therapy.

Methods. Retrospective review of 170 consecutive patients treated for total anomalous pulmonary venous drainage identified 13 patients with postrepair pulmonary vein stenosis. Preoperative and operative data were analyzed to define the patterns of progression and efficacy of surgical techniques.

Results. Seventeen reoperations were performed in 13 patients. Postrepair pulmonary vein stenosis was most common in the infracardiac and mixed subtypes (p < 0.02). All 4 patients with unilateral stenosis, 2 of whom had progression of stenosis resulting in nearly complete unilateral pulmonary vein occlusion, survived. Six of 9 patients with bilateral disease died (p < 0.05 versus unilateral); 2 of the 3 survivors were repaired with a novel technique creating a sutureless neoatrium without evidence of restenosis at 1.8 years’ follow-up. Stenting was uniformly unsuccessful.

Conclusions. In unilateral stenosis, progression of disease may be survivable with loss of single-lung perfusion. Although bilateral disease is lethal in most cases, creation of a sutureless neoatrium has demonstrated short-term freedom from disease progression.




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