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. 2018 Jul;8(3):e174-e179.
doi: 10.1055/s-0038-1669429. Epub 2018 Sep 14.

Cardiothoracic Area Ratio Predicts Lethal Pulmonary Venous Obstruction in Patients with Single Ventricle and Total Anomalous Pulmonary Venous Connection

Misugi Emi  1 , Noboru Inamura  2
Affiliations

Cardiothoracic Area Ratio Predicts Lethal Pulmonary Venous Obstruction in Patients with Single Ventricle and Total Anomalous Pulmonary Venous Connection

Misugi Emi et al. AJP Rep. 2018 Jul.

Abstract

Background and Objectives When single ventricle (SV) is complicated with total anomalous pulmonary venous connection (TAPVC), the pulmonary vein obstruction (PVO) occurs at a high rate. There are some patients who died from the lethal PVO (l-PVO) which needed PVO release dead due to severe desaturation within 24 hours after birth. The purpose of this study was to find a predictive marker for l-PVO during the fetal period. Methods We enrolled 21 patients diagnosed with SV associated with TAPVC in the antenatal period. Ten patients had supracardiac, five had cardiac, five had infracardiac, and one had mixed TAPVC. We reviewed fetal echocardiography and measured cardiothoracic area ratio (CTAR) and total cardiac dimension (TCD). We divided 21 cases into l-PVO group (6) and non-l-PVO group (15) and compared the fetal echocardiography findings and postnatal prognoses between the groups. Results CTAR at the final fetal echocardiography was 16 to 29% (median: 21) in the l-PVO group and 22 to 38% (median: 28) in the non-l-PVO group ( p = 0.01). TCD/week at the final echocardiography was 0.67 to 1.0 (median: 0.77) in the l-PVO group and 0.78 to 1.2 (median: 0.96) in the non-l-PVO group ( p = 0.02). Conclusion Reduced CTAR in the antenatal period is a good predictor of l-PVO after birth.

Keywords: CTAR; Fetal echocardiography; TAPVC; pulmonary venous obstruction; single ventricle.

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Figures

Fig. 1
Fig. 1
Procedure for measuring cardio-thoracic area ratio.
Fig. 2
Fig. 2
Changes in cardio-thoracic area ratio (CTAR) by gestational week. The open circle shows the change in patients with lethal pulmonary vein obstruction (l-PVO). The closed circle shows the change in non-l-PVO patients. CTAR gradually decreased in cases of l-PVO with no or trivial regurgitation of the atrioventricular valve.
Fig. 3
Fig. 3
Changes in combined cardiac output by gestational week showing the CCO of patients with lethal pulmonary vein obstruction (l-PVO) and non-l-PVO with single ventricle, total anomalous pulmonary venous connection, and pulmonary atresia. The open circle shows the CCO of patients with l-PVO. The closed circle shows the CCO of patients with non-l-PVO. CCO; combined cardiac output.
Fig. 4
Fig. 4
Histological findings from a lung sample from case 20 (who died 16 hours after birth due to pulmonary vein obstruction). (A) The elastic fiber is hypertrophic, especially in the pulmonary vein. (B) The media of the pulmonary artery is hypertrophic. Heath-Edwards grade = I. (C) The interstitial wall of the pulmonary alveolus is hypertrophic and the lymphatic vessels are dilated. (D) The wall of the pulmonary alveolus is edematous and there are many cellular components and thickening.
See this image and copyright information in PMC

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