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J Thorac Cardiovasc Surg. 2015 Oct;150(4):911-7. doi: 10.1016/j.jtcvs.2015.06.050. Epub 2015 Jun 30.

Outcomes of surgery for young children with multivessel pulmonary vein stenosis.

Author information

1
Department of Cardiac Surgery, Boston Children's Hospital, Boston, Mass. Electronic address: luis.quinonez@cardio.chboston.org.
2
Department of Cardiology, Boston Children's Hospital, Boston, Mass.
3
Department of Cardiac Surgery, Boston Children's Hospital, Boston, Mass.

Abstract

OBJECTIVE:

We pursued a multimodality approach to the treatment of patients with pulmonary vein stenosis, incorporating sutureless surgical repair, catheter interventions, and adjunctive chemotherapy. We report our outcomes after surgery.

METHODS:

Between January 2007 and August 2013, 49 patients with multivessel pulmonary vein stenosis underwent operations at our institution. We retrospectively reviewed data from a pulmonary vein stenosis registry and the medical records.

RESULTS:

At the time of the index operation, the median patient age was 6 months (range, 32 days-48 months) and weight was 4.9 kg (range, 2.1-13.4 kg). Fourteen patients (28%) died during the follow-up period (median follow-up was 0.5 years [range, 0.04-4.9 years]). There were 2 deaths (4%) within 30 days. Age at repair <6 months, weight at repair <3 kg, and a preoperative right ventricular systolic pressure < ¾ systemic were found to be associated with mortality. One patient required repeat operation for recurrent stenosis. Thirty-nine patients (80%) underwent postoperative catheterizations. The median number of catheterizations per patient was 2 (range, 0-14). Twenty-nine patients (59%) underwent catheterizations with pulmonary vein intervention. The median number of catheterizations with intervention per patient was 1 (range, 0-14). There were no identifiable associations with need for or number of catheterizations with intervention. Ten patients were listed for lung transplantation: 4 patients were de-listed, 3 patients died waiting, and 3 patients underwent transplant.

CONCLUSIONS:

Using a multimodality approach, we observed acceptable early survival after operation in patients with pulmonary vein stenosis, despite the need for catheter reinterventions. Lung transplantation remains a viable option.

KEYWORDS:

congenital heart disease; lung transplanation; pulmonary hypertension; pulmonary vein; pulmonary vein dilation; pulmonary vein operation; pulmonary vein stenosis

Comment in

PMID:
26215353
DOI:
10.1016/j.jtcvs.2015.06.050
[Indexed for MEDLINE]
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