A. Kempe

Levoatrial Cardinal Vein in a Series of Five Prenatal Cases with Hypoplastic Left Heart Syndrome and Intact Atrial Septum.

Purpose The rare finding of a levoatrial cardinal vein (LACV) represents a pulmonary-systemic connection providing an alternative egress from the left atrium for pulmonary venous blood in fetal cardiac malformations with severe left heart obstruction and intact atrial septum (IAS). The purpose of the study was the description of the various sonographic and Doppler findings, the peripartal management and neonatal outcome of this rare cardiovascular anomaly. Materials and Methods Retrospective review of 53 967 echocardiograms in our fetal database between 2002 and 2013 for cases with an LACV. The various sonographic findings of the LACV, the associated cardiac findings and the perinatal management were assessed. Results The presence of a decompressing LACV was documented in 5/27 (18.5 %) of all fetuses with hypoplastic left heart syndrome and intact atrial septum. All five fetuses were diagnosed with an LACV originating from the left atrium and draining either into the innominate vein (two cases), the azygos vein (one case), or directly into the vena cava superior (two cases). Elevated pressure in the pulmonary veins was present in the three cases with obstruction of the LACV. Two pregnancies were terminated, two neonates received compassionate care and one neonate died despite atrioseptectomy. Conclusion LACV in the presence of HLHS and IAS is a rare condition with various forms of pathway and degree of obstruction and generally has an unfavorable prognosis. The presence of obstruction can be diagnosed by spectral Doppler of the LACV. Therefore, in cases of HLHS and IAS, careful sonographic evaluation for aberrant vessels is mandatory.

OP21.08: Outcome for prenatally diagnosed pulmonary atresia/stenosis with intact ventricular septum

underwent the Ist stage of Norwood procedure in mean 12 (3–50) day of life. The mean time of hospitalization was 34 (1–156) days. Out of 55 operated patients, 20 (23%) died in the postoperative period. Survival of the Ist stage of Norwood procedure was 65%. In the fetuses with prenatally diagnosed HLHS in our center the overall survival rate was 42%. Conclusions: Although the results of the heart surgery are good, mortality and morbidity of the patients with HLHS remain one of the highest in all congenital heart diseases, in our study it was 65%. Extracardiac anomalies have significant impact on the fetal outcome and in our group they complicated 20 (23%) prenatally diagnosed HLHS.

P03.09: Ventriculocoronary connections in fetuses with pulmonary or aortic atresia with intact ventricular septum

Objective: Ventriculocoronary connections (VCC) can be prenatally visualized in fetuses with pulmonary (PA) and aortic atresia (AA) and intact ventricular septum (IVS). After prenatal diagnosis an improvement of outcome may be achieved by modification of perinatal and operative management. Method: We identified retrospectively 13 fetuses with prenatally diagnosed VCC, 10 with PA-IVS and 3 with AA-IVS in our database between 2003 and 2008. Prenatal findings, perinatal management and outcome were analysed. Results: In fetuses with PA-IVS prenatal diagnosis of VCC was correlated with small right ventricle and tricuspid annulus and absence of tricuspid regurgitation. One fetus had an additional tricuspid atresia resulting into a back-and-forth blood flow via VCC between the hypoplastic right ventricle (RV) and thickened coronary arteries. Three fetuses with AA-IVS showed a hypoplastic left ventricle with endocardial fibroelastosis and two of those had a restrictive foramen ovale. Of the ten fetuses with PA-IVS, termination of pregnancy was performed in 1 fetus; 1 severely growth restricted fetus died as consequence of uteroplacental dysfunction and acute CMV-infection, 1 fetus died postoperatively during surgical commissurotomy: 1 fetus died days after BT anastomosis; four fetuses had single ventricle palliation and two fetuses a biventricular repair. Norwood procedure was successfully performed in the three fetuses with severe obstruction of the left ventricular outflow tract. Conclusions: Prenatal diagnosis of VCC has important implications for prenatal counselling. A special perinatal approach is required in fetuses with PA-IVS because hypoplastic RV and RV-dependent coronary circulation do not allow opening of the obstructed pulmonary valve resulting in a single ventricle repair. In fetuses with hypoplastic left heart the presence of VCC seems not to worsen the outcome of Norwood procedure.

Spezielle Aspekte der Entbindungsplanung bei Zwillingen

ZusammenfassungJede Zwillingsschwangerschaft ist engmaschig zu überwachen, besonders unter dem Aspekt, dass sich bei „unkomplizierten“ dichorialen Schwangerschaften eine relative uteroplazentare Dysfunktion früher als bei Einlingen manifestiert. In „komplizierten“ dichorialen Schwangerschaften, insbesondere bei Symptomen einer uteroplazentaren Dysfunktion oder einer Präeklampsie, sollten frühzeitig eine Geburtseinleitung oder elektive Sectio caesarea erfolgen. Bei Zeichen einer uteroplazentaren Dysfunktion und anderen Einstellungen als Schädellage/Schädellage sollte eine vaginale Entbindung nur sehr zurückhaltend indiziert werden. Über die – für die Kinder wohl sichereren – Alternative einer elektiven Sectio caesarea ist aufzuklären. Besprochen werden Kriterien für Entbindungszeitpunkt und Entbindungsmodus bei verschiedenen Konstellationen sowie besondere Aspekte monochorialer Zwillinge.AbstractClose antenatal surveillance is required in each twin pregnancy. Relative uteroplacental dysfunction manifests earlier in twin than singleton pregnancies, even if they are uncomplicated. If dichorionic twin pregnancies are complicated by uteroplacental dysfunction or preeclampsia premature delivery is inevitable and usually accomplished by elective cesarian section or induction of labor. Each vaginal delivery in twin pregnancies must be monitored closely. A vaginal delivery should be cautiously indicated in the case of uteroplacental dysfunction and other settings than vertex/vertex presentation. Parents should be counselled that the only way to avoid additional risk of fetal hypoxemai during delivery is to perform elective caesarean section. Here we discuss criteria for time and mode of delivery of twins as well as special aspects of monochorionic twins.