Elsevier

The Annals of Thoracic Surgery

Volume 66, Issue 5, November 1998, Pages 1514-1519
The Annals of Thoracic Surgery

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

Presented at the Thirty-fourth Annual Meeting of The Society of Thoracic Surgeons, New Orleans, LA, Jan 26–28, 1998.
https://doi.org/10.1016/S0003-4975(98)00952-7Get rights and content

Abstract

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.

Section snippets

Methods

Review of the cardiovascular surgical database at the Hospital for Sick Children of Toronto between July 1982 and July 1996 identified 170 patients undergoing primary surgical repair of total anomalous pulmonary venous return. Of these 170 patients, 129 survived more than 30 days after initial repair (24% early mortality). Thirteen of the 129 survivors (10%) experienced PR-PVS, defined as pulmonary vein stenosis requiring intervention and presenting more than 30 days after initial TAPVD repair.

Results

A total of 170 patients were identified with TAPVD. Of this group, 129 survived more than 30 days after repair, and of these survivors, 13 patients had recurrent pulmonary vein stenosis. The anatomic breakdown of the entire cohort and the patients with pulmonary vein stenosis is summarized in Table 2. Within the smaller group of 13 patients with PR-PVS, the infracardiac and mixed subtypes were most common (p < 0.05). Of note, 1 of the 2 patients with a mixed subtype who had recurrent stenosis

Comment

Repair of total anomalous pulmonary venous return has been noted by Bando and coworkers [2] to be associated with decreasing perioperative morbidity in more recent years. This improvement in early mortality is thought to reflect evolving management strategies and operative technique. Despite such advances, however, in most series a small but persistent percentage of patients will return after repair with pulmonary vein stenosis. In the series by Bando and associates [2], 99 patients had

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