CC BY-NC-ND 4.0 · AJP Rep 2019; 09(03): e310-e314
DOI: 10.1055/s-0039-1697960
Case Report
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Two Extremely Low Birth Weight Infants Who Survived Functional Pulmonary Atresia with Normal Intracardiac Anatomy

Shuntaro Oka#
1  Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Saitama, Japan
,
Utako Kondo#
2  Department of Neonatology, Tokyo Metropolitan Bokutoh Hospital, Tokyo, Japan
,
Ayumi Oshima
1  Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Saitama, Japan
,
Shun Matsumura
1  Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Saitama, Japan
,
Masayo Kanai
1  Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Saitama, Japan
,
Akio Ishiguro
1  Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Saitama, Japan
,
Yoichi Iwamoto
1  Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Saitama, Japan
,
Hirotaka Ishido
1  Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Saitama, Japan
,
Isaku Omori
2  Department of Neonatology, Tokyo Metropolitan Bokutoh Hospital, Tokyo, Japan
,
Satoshi Masutani
1  Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Saitama, Japan
› Author Affiliations
Further Information

Publication History

15 December 2018

07 June 2019

Publication Date:
19 September 2019 (online)

  

Abstract

For the first time, we report about two extremely low birth weight infants who were born at 25 and 22 weeks' gestation and who survived functional pulmonary atresia (fPA) with normal intracardiac anatomy. A slow, reflected, and bimodal blood flow pattern in the pulmonary artery (both cases) and the presence of pulmonary regurgitation (1 case) were useful for diagnosing fPA. Timely use of lipo-prostaglandin E1 to maintain adequate pulmonary flow and reduce pulmonary arterial resistance and sodium bicarbonate to improve acidosis were effective treatments to attain forward flow. As optimal management is essential for the intact survival of extremely early preterm infants and the accurate diagnosis of fPA is difficult without the awareness of the disease entity, our cases underline the importance of recognizing that fPA can occur even in extremely low birth weight infants with normal intracardiac anatomy.

# The first two authors equally contributed to this work.