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Congenital pulmonary lymphangiectasia and early mortality after stage 1 reconstruction procedures*

Published online by Cambridge University Press:  13 March 2017

Jeremy L. Herrmann*
Affiliation:
Division of Cardiothoracic Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Mallory L. Irons
Affiliation:
Department of Surgery, Perelman School of Medicine, Division of Cardiovascular Surgery, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
Christopher E. Mascio
Affiliation:
Division of Cardiothoracic Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Jack Rychik
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Thomas L. Spray
Affiliation:
Division of Cardiothoracic Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
J. William Gaynor
Affiliation:
Division of Cardiothoracic Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Jennifer E. Pogoriler
Affiliation:
Department of Pathology and Laboratory Medicine, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
*
Correspondence to: J. L. Herrmann, MD, Indiana University School of Medicine, 545 Barnhill Drive, Emerson 215, Indianapolis, IN 46202, United States of America. Tel: +1 317 944 7150; Fax: +1 317 274 2940; E-mail: jlherrma@iupui.edu

Abstract

Objectives

Pulmonary lymphangiectasia associated with hypoplastic left heart syndrome with an intact or restrictive atrial septum may result from increased left atrial pressure, and is associated with worse outcomes following staged reconstruction due to lung dysfunction and significant hypoxaemia. Our objective was to characterise the incidence of pulmonary lymphangiectasia in cases of early mortality following stage 1 reconstructions.

Methods

An institutional cardiac surgical database was retrospectively searched for patients who died within 30 days following a stage 1 reconstruction between 1 January, 1984 and 31 December, 2013. During that period, 1669 stage 1 procedures were performed. Autopsy lung specimens were reviewed by a paediatric pathologist. Patients who died of suspected technical issues were excluded.

Results

A total of 54 patients were included, and of these seven cases (8.5%) of pulmonary lymphangiectasia were identified. The mean estimated gestational age was 38.2±2.4 weeks, and the mean birth weight was 3.0±0.6 kg. The median interval between surgery and death was 1 day (with a range from 0 to 18 days). The atrial septum was intact in one patient (14.3%), restrictive in three patients (42.9%), and unrestrictive in three patients (42.9%).

Conclusions

Pulmonary lymphangiectasia may develop in hypoplastic left heart syndrome with or without a restrictive atrial septum. As standard prenatal diagnostic evaluations and treatment methods for pulmonary lymphangiectasia are limited, this may be an important contributor to early and late mortality following stage 1 reconstruction for hypoplastic left heart syndrome.

Type
Original Articles
Copyright
© Cambridge University Press 2017 

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Footnotes

*

Meeting presented at the 30th Annual Meeting of the European Association for Cardio-Thoracic Surgery, Barcelona, Spain, 3 October, 2016.

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