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Pulmonary hypoplasia in fetuses with congenital conotruncal defects
Qian Wang1, Ying Zhao1, Ling Han1
1Beijing Key Laboratory of Maternal-Fetal Medicine and Fetal Heart Disease, Beijing Anzhen Hospital, Capital Medical University, Beijing, China.
Insights
Congenital conotruncal defect (CTD) in fetuses is linked to smaller pulmonary arteries and lung hypoplasia. Risk factors include specific defect types and right ventricular outflow tract obstruction.
Area of Science:
- Fetal Cardiology
- Pediatric Radiology
- Congenital Heart Disease Research
Background:
- Congenital conotruncal defect (CTD) encompasses a group of complex heart abnormalities.
- Pulmonary hypoplasia (PH) is a known complication, but its specific risk factors in CTD require further elucidation.
Purpose of the Study:
- To investigate pulmonary hypoplasia (PH) in fetuses with congenital conotruncal defect (CTD).
- To identify associated risk factors for PH in fetuses with CTD.
Main Methods:
- Fetal echocardiography was used to assess 75 fetuses with CTD and 150 controls.
- Measurements included pulmonary artery (PA) diameters, Z-scores, and various lung area indices (LCR, LHR, QLI).
Main Results:
- Fetuses with CTD exhibited significantly smaller PA dimensions and lung areas (except QLI) compared to controls.
- Subgroup analysis revealed smaller lung areas in CTD with right ventricular outflow tract obstruction (RVOTO), pulmonary atresia, double outlet right ventricle (DORV), and tetralogy of Fallot (TOF).
- Transposition of the great arteries (TGA) did not show a significant difference in lung areas.
Conclusions:
- CTD is associated with hypoplastic pulmonary arteries and PH.
- Risk factors for PH in CTD include specific defect types (DORV, TOF) and RVOTO.
- Pulmonary blood flow may be a more critical determinant of PH than defect type, RVOTO, or PA dimensions.
Objectives:
We sought to investigate pulmonary hypoplasia (PH) and associated risk factors in fetuses with congenital conotruncal defect (CTD).
Methods:
A total of 75 fetuses with CTD (gestational age (GA): 22-32 weeks) and 150 normal GA-matched fetuses as the control group were studied. We measured diameters of aorta (Ao); main, left, and right pulmonary artery (PA); and their Z-scores by fetal echocardiography (FE). We also measured the lung area, lung area/chest area ratio (LCR), lung-to-head circumference ratio (LHR), right lung area/head circumference2 (quantitative lung index, QLI), and Z-scores.
Results:
The PA, left pulmonary artery (LPA), RPA, and their Z-scores and the lung area measurements (except for QLI) were significantly smaller in the CTD group, compared with the normal control group. Subgroup analysis showed the following: (1) CTD with right ventricular outflow tract obstruction (RVOTO) had smaller main and branch PA dimensions and small lung areas in contrast to CTD without RVOTO. (2) CTD with pulmonary atresia had smaller LPA and RPA dimensions but no difference in lung areas. (3) In the common types of CTD, lung area variables were mostly smaller in double outlet right ventricle (DORV) and tetralogy of Fallot (TOF), but there was no difference in transposition of the great arteries (TGA).
Conclusions:
CTD is associated with hypoplastic main and branch pulmonary arteries as well as PH. Risk factors for PH included defect types (DORV and TOF but not TGA) and RVOTO. It is plausible that pulmonary blood flow, rather than the type of CTD, RVOTO, or pulmonary artery dimensions, plays a key role in PH.
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