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J Pediatr Intensive Care 2021; 10(03): 228-231
DOI: 10.1055/s-0040-1713437
Case Report

Successful Management of an Infant with Atypical Presentation of Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins

Christoph Armin Neuhäuser
1   Department of Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital, Justus Liebig University, Giessen, Germany
,
Karsten Grosse Kreymborg
1   Department of Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital, Justus Liebig University, Giessen, Germany
,
Carsten Müller
2   Department of Pediatric Pneumology and Neonatology, University Children's Hospital, Hannover Medical School, Hannover, Germany
,
Markus Khalil
1   Department of Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital, Justus Liebig University, Giessen, Germany
,
Christian Jux
1   Department of Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital, Justus Liebig University, Giessen, Germany
,
Csaba Galambos
3   Department of Pathology and Laboratory Medicine, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado, United States
,
Dietmar Schranz
1   Department of Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital, Justus Liebig University, Giessen, Germany
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Abstract

A newborn infant patient presented with persistent pulmonary hypertension. For right ventricular decompression, the ductus arteriosus was kept open by prostaglandin E1 infusion and was stented at the age of 4 weeks during heart catheterization. The child was weaned from mechanical ventilation, since pulmonary functions were adequate. A small atrial septal defect was identified and closed in cardiac catheterization laboratory to decrease preductal hypoxemia. Diagnostic workup led to the diagnosis of alveolar capillary dysplasia with misalignment of the pulmonary veins. Suprasystemic pulmonary arterial hypertension with persisting nitric oxide dependency remained the leading symptoms. The child underwent bilateral lung transplantation at the age of 28 months. He is well at the age of 44 months.

Keywords

alveolar capillary dysplasia - pulmonary arterial hypertension - inhaled nitric oxide - ductal stent - riociguat


Publication History

Received: 29 March 2020

Accepted: 10 May 2020

Article published online:
19 June 2020

© 2020. Thieme. All rights reserved.

Georg Thieme Verlag KG
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