The detection of multiple cardiac tumors during fetal echocardiography allowed us to make the diagnosis of tuberous sclerosis complex in the mother and establish the reason of her first epileptic seizures.
Objectives
To analyze pregnancy management and postnatal follow-up in monochorionic monoamniotic (MCMA) pregnancies complicated by a congenital heart defect (CHD) in one twin and to compare the results with current recommendations concerning time and mode of delivery in MCMA pregnancies.
Cases presentation
Perinatal medical records of five pairs of monoamniotic twins referred to Fetal Cardiology Department were analyzed. 5 out of 23 MCMA pregnancies (21.7%) were complicated by CHD in one fetus. Cesarean section (CS) was performed between 32 and 35 weeks of gestation (WoG). 9 out of 10 neonates had respiratory failure, including all patients with CHD. Twins without congenital abnormalities spent median 21 days (range 10–40 days) in neonatal units. Patients with CHD were transferred to cardiology departments on average 6th day of life. All were operated on later than term-born neonates, 4 out of 5 required stage surgery and their median stay in the hospital was 75 days (range 48–106 days).
Conclusions
According to current recommendations, delivery in MCMA pregnancies should be scheduled at 32–34 weeks. In cases complicated by CHD in one twin, such early delivery complicates surgical treatment and may affect the final outcome. Low body weight and respiratory disorders increase the risk of complications in the perioperative period and prolong hospitalization.
Functional analysis of the fetal cardiovascular system is crucial for the assessment of fetal condition. Evaluation of the right ventricle with standard 2D echocardiography is challenging due to its complex geometry and irregular muscle fibers arrangement. Software package TOMTEC 4D RV-Function is an analysis tool which allows assessment of right ventricular function based on volumetric measurements and myocardial deformation. The aim of this study was to determine the feasibility of this method in fetal echocardiography. The retrospective study was conducted in the high-flow Referral Center for Fetal Cardiology. We recorded 4D echocardiographic sequences of 46 fetuses with normal hearts. Following parameters were calculated: end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV) and ejection fraction (EF), right ventricle longitudinal free-wall (RVLS free-wall) and septal strain (RVLS septum). Tei index was calculated as a standard measure or RV function for comparison. 4D assessment was feasible in 38 out of 46 fetuses (83%). RV volumetric parameters—EDV, ESV and SV—increased exponentially with gestational age. Functional parameters—RV Tei index, EF and strains—were independent of gestational age. Mean EF was 45.2% (± 6%), RV free-wall strain was − 21.2% and RV septal strain was − 21.5%. There was a statistically significant correlation between septal and free-wall strains (r = 0.51, p = 0.001) as well as between EF and RV free-wall strain (r = − 0.41, p = 0.011). 4D RV assessment is feasible in most fetuses. Its clinical application should be further investigated in larger prospective studies.
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