Pulmonary Interstitial Glycogenosis: A Reversible Underlying Condition Associated With D-Transposition of the Great Arteries and Severe Persistent Pulmonary Hypertension

被引：5

作者：

Sanchez-de-Toledo, Joan ^{[1
]}

Gonzalez-Peris, Sebastia ^{[2
]}

Gran, Ferran ^{[3
]}

Gregoraci, Angela ^{[4
]}

Carles Ferreres, Joan ^{[5
]}

Ruiz, Cesar W. ^{[4
]}

Balcells, Joan ^{[2
]}

Abella, Raul F. ^{[6
]}

机构：

[1] Univ Pittsburgh, Childrens Hosp Pittsburgh, Dept Crit Care Med, Cardiac Intens Care Div, 4401 Penn Ave, Pittsburgh, PA 15224 USA

[2] Univ Autonoma Barcelona, Hosp Univ Vall dHebron, Dept Pediat Intens Care Med, Barcelona, Spain

[3] Univ Autonoma Barcelona, Hosp Univ Vall dHebron, Dept Pediat Cardiol, Barcelona, Spain

[4] Univ Autonoma Barcelona, Hosp Univ Vall dHebron, Dept Neonatol, Barcelona, Spain

[5] Univ Autonoma Barcelona, Hosp Univ Vall dHebron, Dept Pathol, Barcelona, Spain

[6] Univ Autonoma Barcelona, Hosp Univ Vall dHebron, Dept Pediat Cardiothorac Surg, Barcelona, Spain

来源：

WORLD JOURNAL FOR PEDIATRIC AND CONGENITAL HEART SURGERY | 2015年 / 6卷 / 03期

关键词：

transposition of the great arteries; pulmonary hypertension; ECMO; pulmonary interstitial glycogenosis;

D O I：

10.1177/2150135115577433

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Transposition of the great arteries with intact ventricular septum and persistent pulmonary hypertension (TGA-IVS PPHN) is a rare association with a poor prognosis. We report the case of a term newborn with TGA-IVS PPHN successfully managed with perioperative extracorporeal membrane oxygenation (ECMO) and aggressive pulmonary vasodilation therapy that underwent successful arterial switch procedure. A lung biopsy obtained during the surgical procedure showed pulmonary interstitial glycogenosis, a reversible condition. Concerns over left ventricle deconditioning after ECMO could be minimized with appropriate management and monitoring of the ductus arteriosus and appropriate timing of surgery.

引用

页码：480 / 483

页数：4