Totally anomalous pulmonary venous connection and complex congenital heart disease - Prenatal echocardiographic diagnosis and prognosis
Acoustics; anomalous pulmonary venous connections; blood-flow; congenital heart diseases; fetal echocardiography; fetus; heterotaxy syndrome; Nuclear Medicine & Medical Imaging; pregnancy; prenatal diagnosis; Radiology; right isomerism; totally
Objective. The purpose of this study was to determine the accuracy of prenatal cardiac diagnosis, prognosis, and outcome of totally anomalous pulmonary venous connection (TAPVC) and to determine echocardiographic clues in the prenatal diagnosis of isolated TAPVC or TAPVC in association with other complex congenital heart disease (CHD). Methods. We reviewed our 13-year experience of prenatal diagnosis of TAPVC. Thirteen fetuses were identified with the diagnoses of TAPVC. We systematically analyzed the individual pulmonary veins by color and pulsed Doppler imaging, the presence of a pulmonary venous confluence, the pulsed and color Doppler evaluation of the vertical vein, and sites of connections. Prenatal diagnosis was confirmed by postnatal echocardiography, cardiac catheterization, surgery, or autopsy. Results. The mean gestational age at diagnosis of TAPVC was 26.3 weeks (range, 20-33 weeks). There were 8 fetuses with TAPVC and right isomerism, 3 fetuses with other associated CHD, and 2 with isolated TAPVC. There were 7 fetuses with supracardiac TAPVC, 4 with infracardiac TAPVC, and 2 with mixed TAPVC. Pulmonary vein color and pulsed Doppler data were available in 10 of 13 fetuses. The pulmonary venous confluence was visualized in all fetuses except 1. The vertical vein was visualized in all fetuses. Five fetuses had suspected signs of obstruction. The diagnosis was confirmed postnatally or at autopsy in 12 cases. Eight patients underwent surgery; 6 died, and 2 were alive. Two patients had compassionate care and died; 3 pregnancies were terminated. Conclusions. It is possible to diagnose accurately complex CHD, including the pulmonary venous connections. When diagnosed prenatally, TAPVC carries a poor prognosis.
Patel C R; Lane J R; Spector M L; Smith P C; Crane S S
Journal of Ultrasound in Medicine
2005
2005-09
Journal Article
<a href="http://doi.org/10.7863/jum.2005.24.9.1191" target="_blank" rel="noreferrer noopener">10.7863/jum.2005.24.9.1191</a>
Fetal echocardiographic diagnosis of vascular rings
Acoustics; anomalies; aortic-arch; fetal echocardiography; low-risk; Nuclear Medicine & Medical Imaging; prenatal diagnosis; Radiology; sonographic diagnosis; vascular ring; view
Objective. The purpose of this series is to describe the prenatal echocardiographic findings of vascular rings. Methods. The 3-vessel and trachea view consists of the axial view of the upper mediastinum. The normal left aortic arch appears as a V-shaped confluence of the ductus arteriosus and aortic arch, with the trachea situated posterior and to the right. No vessel should encircle the trachea. The diagnoses of vascular rings were made prenatally and were confirmed in all patients postnatally. Results. Six fetuses had diagnoses of vascular rings. The mean gestational age at diagnosis was 23.3 weeks (range, 18-31 weeks). The indications for fetal echocardiography were family history of congenital heart disease, echogenic focus in the left ventricle, and abnormal 4-chamber view. There were 2 fetuses with a double aortic arch; 3 fetuses with a right aortic arch, an aberrant left subclavian artery, and a left ductus arteriosus; and 1 with a right circumflex aortic arch with a left ductus arteriosus and an aberrant left subdavian artery. Two fetuses had associated structural cardiac defects, 1 with an unbalanced atrioventricular septal defect and trisomy 21 and the other with a double-outlet right ventricle, pulmonary atresia, and multiple other congenital anomalies. Conclusions. Vascular rings can be accurately diagnosed prenatally with recognition of a vascular structure that courses around the trachea and absence of the usual V-shaped relationship of the aortic and ductal arches. The color Doppler findings and the presence of a ductus arteriosus aid in identifying various components of the vascular ring.
Patel C R; Lane Jr; Spector M L; Smith P C
Journal of Ultrasound in Medicine
2006
2006-02
Journal Article
<a href="http://doi.org/10.7863/jum.2006.25.2.251" target="_blank" rel="noreferrer noopener">10.7863/jum.2006.25.2.251</a>
Prenatal diagnosis of ventriculocoronary arterial communication in fetuses with hypoplastic left heart syndrome
Acoustics; arterial communication; fistula; hypoplastic left heart syndrome; Nuclear Medicine & Medical Imaging; prenatal diagnosis; Radiology; ventriculocoronary
Objective. The purpose of this series was to describe the fetal echocardiographic findings in hypoplastic left heart syndrome with aortic atresia and ventriculocoronary arterial communication and implications of these findings. Methods. We describe 2 fetuses with hypoplastic left heart syndrome with ventriculocoronary arterial communication diagnosed at 29 and 20 weeks' gestation, respectively. The underlying cardiac anatomy consisted of a hypoplastic left heart and mitral stenosis with aortic atresia. We used color Doppler and pulsed Doppler sonography on the surface of the myocardiurn to specifically look for coronary arterial flow. Results. By color Doppler sonography, ventriculocoronary arterial communication was shown between the left ventricular cavity and the left coronary artery with characteristic bidirectional flow on pulsed Doppler examination. There was no mitral regurgitation. The left ventricular myocardium was substantially hypertrophied. The first patient underwent surgical Norwood palliation and died after a prolonged postoperative course. The second patient underwent stenting of the arterial duct and bilateral pulmonary artery banding in the catheterization laboratory but died after a few weeks. implications of ventriculocoronary arterial communication in association with hypoplastic left heart syndrome are discussed. Conclusions. It is possible to accurately diagnose ventriculocoronary arterial communication on fetal echocardiography. The presence of ventriculocoronary arterial communication is seen exclusively in a subgroup of patients with an aortic atresia and mitral stenosis variant of hypoplastic left heart syndrome. The prognosis is poor in this subgroup of patients.
Patel C R; Lane Jr; Spector M L; Smith P C; Waight D J
Journal of Ultrasound in Medicine
2006
2006-02
Journal Article
<a href="http://doi.org/10.7863/jum.2006.25.2.245" target="_blank" rel="noreferrer noopener">10.7863/jum.2006.25.2.245</a>