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A 30-year experience with mixed-type total anomalous pulmonary venous connection: a word of caution

Published online by Cambridge University Press:  20 September 2016

Brian Kogon*
Affiliation:
Department of Cardiothoracic Surgery, Emory University, Atlanta, Georgia, United States of America
Jan Fernandez
Affiliation:
Department of Cardiothoracic Surgery, Emory University, Atlanta, Georgia, United States of America
Subhadra Shashidharan
Affiliation:
Department of Cardiothoracic Surgery, Emory University, Atlanta, Georgia, United States of America
Kirk Kanter
Affiliation:
Department of Cardiothoracic Surgery, Emory University, Atlanta, Georgia, United States of America
Bahaaldin Alsoufi
Affiliation:
Department of Cardiothoracic Surgery, Emory University, Atlanta, Georgia, United States of America
*
Correspondence to: B. E. Kogon, MD, Emory University, Children’s Healthcare of Atlanta, Egleston, Atlanta, Georgia, United States of America. Tel: 678 372 7324; Fax: 404 785 6266; E-mail: Bkogon@emory.edu

Abstract

Background

Patients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome.

Methods

We reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015.

Results

A total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04).

Conclusion

The surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.

Type
Original Articles
Copyright
© Cambridge University Press 2016 

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