Unusual Combination of Anomalous Connection of the Left Pulmonary Veins and Aortic Arch Anomaly Presenting as Abnormal T
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ORIGINAL ARTICLE
Unusual Combination of Anomalous Connection of the Left Pulmonary Veins and Aortic Arch Anomaly Presenting as Abnormal Three Vessel View in the 2nd Trimester US Scan Geetanjli Behl1 • Smita Mishra2
Received: 16 March 2020 / Accepted: 1 August 2020 Ó Society of Fetal Medicine 2020
Abstract Partial anomalous pulmonary venous drainage is often missed antenatally unless a complex congenital heart defect is associated. Likewise, isolated fetal coarctation of aorta has only a 60–70% chance to be diagnosed in the second trimester. The combination of these two conditions (PAPVC and COA), in the absence of situs abnormality or syndromic association is rare and not much reported in the literature. We are reporting a fetal heart which presented with apparent hypoplasia of the left ventricle and ascending aorta, as well as significant dilatation of the right atrium, right ventricle and pulmonary artery at 22 weeks of gestation. To begin with, we strongly suspected a left sided obstructive lesion until we noticed a 4th channel in the three vessel view (3VV) and dilated innominate vein in the absence of a dilated coronary sinus in the 4 chamber view. This led to a diagnosis of supracardiac PAPVC in combination with an arch anomaly. The pregnancy was continued up to 37–38 weeks when she underwent C-section for an obstetric indication. The baby underwent surgical intervention postnatally. Keywords PAPVC Coarctation of Aorta, Fetal Cardiac Anomaly
Electronic supplementary material The online version of this article (https://doi.org/10.1007/s40556-020-00263-6) contains supplementary material, which is available to authorized users. & Geetanjli Behl [email protected] 1
Department of Fetal Medicine, Manipal Hospital, Dwarka Sector 6, New Delhi, Delhi 110075, India
2
Department of Pediatric Cardiology, Manipal Hospital, Dwarka Sector 6, New Delhi, Delhi 110075, India
Introduction The combination of supracardiac partial anomalous pulmonary venous connection and coarctation of the aorta is an unconventional fetal diagnosis in the absence of situs anomalies and complex heart defects and is hardly reported in literature [1]. After birth blood volume in the pulmonary circulation increases and receives almost 50% of combined ventricular cardiac output (CVCO), unlike the 20% of CVCO received in fetal life. Abnormal post-left atrial space index [2] is one of the major clues for suspicion of a total anomalous pulmonary venous connection while in isolated PAPVC the findings are often subtle. Isolated fetal PAPVC is insignificant hemodynamically and remains mostly obscured before the birth. On the other hand, coarctation of aorta (COA), narrowing of the distal aortic arch, constitutes 6–8% of all CHD. Its antenatal diagnosis is challenging and carries a high false positive rate. Prenatal diagnosis of coarctation of the aorta is critically important to avoid morbidity and mortality postnatally by facilitating ‘in utero transfer’ to a neonatal tertiary cardiac set-up. We report an antenatal diagnosis of PAPVC and forme-fro
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