Hypoplastic left heart syndrome associated with left ventricle–coronary artery–pulmonary artery fistula: prenatal diagnosis and histological correlation

• Published in: Ultrasound in obstetrics & gynecology Vol. 26; no. 1; pp. 78 - 80
• Main Authors: Paladini, D., Caruso, G., Sglavo, G., Lapadula, C., Parisi, G., Martinelli, P.
• Format: Journal Article
• Language: English
• Published: Chichester, UK John Wiley & Sons, Ltd 01.07.2005; Wiley
• Subjects: Abortion, Therapeutic; Adult; Arterio-Arterial Fistula - complications; Arterio-Arterial Fistula - diagnostic imaging; Arterio-Arterial Fistula - pathology; Biological and medical sciences; Cardiology. Vascular system; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; coronary fistula; Coronary Vessels - diagnostic imaging; Coronary Vessels - pathology; Echocardiography, Doppler, Color; Female; fetus; Gynecology. Andrology. Obstetrics; Heart; Heart Septal Defects, Ventricular - complications; Heart Septal Defects, Ventricular - diagnostic imaging; Heart Septal Defects, Ventricular - pathology; Heart Ventricles - diagnostic imaging; Heart Ventricles - pathology; histology; Humans; hypoplastic left heart syndrome; Hypoplastic Left Heart Syndrome - complications; Hypoplastic Left Heart Syndrome - diagnostic imaging; Hypoplastic Left Heart Syndrome - pathology; Medical sciences; Pregnancy; Pregnancy Trimester, Second; prenatal diagnosis; Pulmonary Artery - diagnostic imaging; Pulmonary Artery - pathology; Regional Blood Flow; Ultrasonography, Prenatal - methods; ultrasound; Sonography; hypoplastic left heart syndrome; Prenatal Diagnosis; Gynecology; Coronary artery; Cardiovascular disease; Left Heart Hypoplasia; Fistula; Histology; coronary fistula; Congenital disease; Coronary heart disease; Obstetrics; Pregnancy; Echography; Fetus; Ultrasound; Left pulmonary artery
• ISSN: 0960-7692; 1469-0705
• DOI: 10.1002/uog.1932

We describe an unusual case of left ventricle–left coronary artery–pulmonary artery fistula associated with hypoplastic left heart syndrome (HLHS) and a ventricular septal defect, in which findings on echocardiography and histological analysis were correlated. The diagnosis was made at 22 weeks of gestation. We speculated that the presence of the fistula was the primary cause of the HLHS due to the existence of the ventricular septal defect that nullified the intraventricular pressure gradient, which is typical of hypoplastic ventricles and thought to be responsible for the secondary development of fistulas in other cases. Copyright © 2005 ISUOG. Published by John Wiley & Sons, Ltd.