EP04.20: Prenatal subtyping of ventriculocoronary connections in hypoplastic left heart syndrome: review of the literature and a case report

Abstract

Results: Four-chamber view and three vessels and trachea view were successfully achieved in 78% (104/133,78%)and 77% (103/133,77%)cases, respectively. Ten cases of congenital heart disease (CHD) were suspected by early fetal echocardiography. One case was lost at follow-up and the rest 9 cases were confirmed by autopsy. Among the 9 cases, 7 were diagnosed correctly and 2 were misdiagnosed. Additional 2 cases of CHD were detected during follow-up. The accuracy of early fetal echocardiography in the diagnosis of complex congenital heart disease was 63.6%(7/11,63.6%). A severe form of TOF has pathological vessels arising from the aorta or its branches and caring the blood flow to the lungs, instead of the pulmonary arteries. Those arteries call Major Aortopulmonary Collateral Arteries (MAPCAs). This anomaly usually is diagnosed only postpartum and has poor prognosis. ConclusionsWe present here for the first time prenatal detection of MAPCAs. The diagnosis was done in four cases in two Medical Centres:First case: TOF with pulmonary atresia was diagnosed in one twin of monoamniotic pregnancy at 27 weeks. The pulmonary arteries could not be detected, and total diversion of blood from both ventricles into the aorta was demonstrated. Careful examination reviled MAPCAs arising from the descending aorta to the right lung. Selective termination of pregnancy using Radiofrequency Ablation was done at 31 week gestation and Caesarean delivery for the second twin.Second case: A 37 week fetus diagnosed with TOF and Pulmonic Atresia. The pulmonary arteries could not be detected. At the age of 3 days CT scan demonstrate two MAPCAs arising from the subclavian artery from each side. The child was operated (unifocalisation) and died at the age of 5 month from lung complication.Third case: The diagnosis of TOF with pulmonary atresia and MAPCAs was made at 28 week gestation. The MAPCAs arise from the descending aorta to both lungs. This pregnancy was terminated.Forth case: A 19 week fetus was diagnosed with TOF, absent pulmonary valve, small right PA, hypoplastic left PA, absent ductus arteriosus and MAPCAs arising from the descending aorta. The pregnancy was terminated. Conclusion: Prenatal diagnosis of MAPCAs is possible, and important due to the grave prognosis.Supporting information can be found in the online version of this abstract Introduction: Fetal critical aortic stenosis (CAS) is a challenging lesion with poor prognosis such as fetal hydrops and intrauterine fetal death. It has been known that the double reverse pattern in pulmonary veins relates to poor prognosis. We had a case with CAS with fetal hydrops, and spontaneously resolved the hydrops with the increased forward flow in aorta. We evaluated the hemodynamic changes with pulmonary venous Doppler pattern as well as the left atrium area/ cardiac area ratio (LA/CA). Case: A 30 year old woman, gravida 2, para 1, was referred at 28 weeks of gestation for polyhydramnios, fetal hydrops and cardiac abnormality. Markedly enlarged left atrium and severe mitral regurgitation wit...