Prenatal diagnosis of fetal unilateral lung agenesis complicated with cardiac malposition

Ying Zhang,Wei Sun,Chang-wei Ding,Wei-dong Ren,Miao Fan,Yu Wang,Li-mei Xie,Ya-jun Guo,Ai-lu Cai

doi:10.1186/1471-2393-13-79

Abstract

BackgroundFetal unilateral lung agenesis, complicated with cardiac shifting, is a rare anomaly, the diagnosis of which remains a challenge for many sonographers in routine screening programs. The present study describes a systematic approach for the diagnosis of fetal unilateral lung agenesis and cardiac malpositions in routine prenatal screening.MethodsA total of 18 cases of fetal unilateral lung agenesis complicated with cardiac malposition were reviewed. A systematic method was proposed to identify the fetal left side and right side according to the fetal head position and posture by acquiring a long axis and transverse view of the fetus. Fetal unilateral lung agenesis was diagnosed by evaluation of the ipsilateral pulmonary artery. The diagnosis was confirmed by postnatal echocardiography, digital radiology, and computed tomography after birth or by autopsy findings.ResultsThe left-sided fetal heart with the cardiac apex rotating to the left and posterior were confirmed in all 7 left lung agenesis cases, while the rightward shifting of the fetal heart together with the cardiac axis deviating to the right were confirmed in all 11 cases of right lung agenesis. The disappearance of the ipsilateral pulmonary artery was confirmed in all 18 cases of unilateral lung agenesis. Cardiac anomalies were present in a total of 7 of the18 cases of lung agenesis with 4 of 7 in cases of left lung agenesis and 3 of 11 in cases of right agenesis.ConclusionsThe systematic approach introduced in the current report is helpful in the diagnosis of fetal unilateral lung agenesis complicated with cardiac malposition. The information provided by this study may be helpful to better understand unilateral lung agenesis anatomically and to facilitate its potential examination.

Highlights

Fetal unilateral lung agenesis, complicated with cardiac shifting, is a rare anomaly, the diagnosis of which remains a challenge for many sonographers in routine screening programs
Fetal cardiac malposition can be classified into two main types [1,2]: One is caused by intrinsic congenital heart diseases, such as isolated dextrocardia, situs inversus, isolated levocardia, etc., while others are caused by extracardiac malformations such as unilateral lung agenesis, diaphragmatic hernia, etc
The primary objective of the current study is to describe a systematic approach to diagnose fetal unilateral lung agenesis complicated with cardiac malposition

Summary

Introduction

Fetal unilateral lung agenesis, complicated with cardiac shifting, is a rare anomaly, the diagnosis of which remains a challenge for many sonographers in routine screening programs. Fetal cardiac malposition can be classified into two main types [1,2]: One is caused by intrinsic congenital heart diseases, such as isolated dextrocardia, situs inversus, isolated levocardia, etc., while others are caused by extracardiac malformations such as unilateral lung agenesis, diaphragmatic hernia, etc. Lung agenesis is a rare developmental anomaly and prenatal diagnosis is difficult for many sonographers during routine screening It is characterized by a complete absence of a lung and bronchi, and no blood supply to the affected side, either the left, the right, or bilateral side. The estimated incidence is 1 per 10000–15000 autopsies [5]

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