Interobserver agreement in detailed prenatal diagnosis of congenital heart disease by telemedicine using four-dimensional ultrasound with spatiotemporal image correlation.

OBJECTIVE: To evaluate the clinical accuracy of four-dimensional (4D) echocardiography in the detailed prenatal diagnosis of congenital heart disease (CHD) in a telemedicine setting. METHODS: Ten second-trimester spatiotemporal image correlation (STIC) volumes were sent to three observers in different tertiary care centers with expertise in 4D echocardiography. The 10 volumes were selected based on the type of diagnosis to cover a wide spectrum of CHD anomalies, and also included one normal fetal heart. Observers were asked to provide the diagnosis, the postprocessing modalities used and the time spent on examination, and to give a rating of the confidence for the diagnosis on a 5-point Likert scale. They were free to consult other colleagues, including pediatric cardiologists, but were blinded to the prenatal diagnosis and the neonatal outcome. A diagnostic scoring system was used to evaluate different aspects of the heart defects. The results were compared with neonatal echocardiography or postmortem findings ('gold standard'). RESULTS: In two cases all observers correctly diagnosed all details of the volume datasets. The observer with the best performance reached perfect agreement in six cases and nearly perfect agreement in three. The volumes were most frequently studied by sectional planes and were analyzed in a median time of 11.0 (range, 2.5-30.0) min. The median confidence score was 4.0 (range, 1.0-5.0). CONCLUSIONS: In a telemedicine setting using STIC volumes, fetal cardiac anomalies can be diagnosed correctly by an expert. However, details required for adequate counseling and planning of postnatal care may be missed. STIC by telemedicine is a promising modality, although not accurate enough for exclusive use in clinical decision making regarding treatment, prognosis or termination of pregnancy.

Twin anemia-polycythemia sequence: diagnostic criteria, classification, perinatal management and outcome.

Monochorionic twins share a single placenta with intertwin vascular anastomoses, allowing the transfer of blood from one fetus to the other and vice versa. These anastomoses are the essential anatomical substrate for the development of several complications, including twin-twin transfusion syndrome (TTTS) and twin anemia-polycythemia sequence (TAPS). TTTS and TAPS are both chronic forms of fetofetal transfusion. TTTS is characterized by the twin oligopolyhydramnios sequence, whereas TAPS is characterized by large intertwin hemoglobin differences in the absence of amniotic fluid discordances. TAPS may occur spontaneously in up to 5% of monochorionic twins and may also develop after incomplete laser treatment in TTTS cases. This review focuses on the pathogenesis, incidence, diagnostic criteria, management options and outcome in TAPS. In addition, we propose a classification system for antenatal and postnatal TAPS.

TAPS and TOPS: two distinct forms of feto-fetal transfusion in monochorionic twins.

Monochorionic twins share a single placenta with inter-twin vascular anastomoses, allowing the transfer of blood from one fetus to the other and vice versa. These anastomoses are the essential anatomical substrate for the development of severe complications, including twin-twin transfusion syndrome (TTTS) and twin-anemia-polycythemia sequence (TAPS). TTTS and TAPS are both chronic forms of feto-fetal transfusion. TTTS is characterized by the twin oligo-polyhydramnios sequence (TOPS), whereas TAPS is characterized by large inter-twin hemoglobin differences in the absence of amniotic fluid discordances. TAPS may occur spontaneously in a minority of monochorionic twins or in TTTS cases after laser treatment. This review focuses on the differences between TAPS and TTTS in terms of pathogenesis, incidence, diagnostic criteria, treatment modalities, perinatal outcome and long-term outcome.

Double fatal outcome after ruptured vasa previa in monochorionic twins: case report and review of the literature.

Vasa previa is a condition in which one or more fetal blood vessels run through the amniotic membranes and cross or run near the external orifice of the uterus. Rupture of membranes can lead to tearing of these vessels and cause acute fetal exsanguination. In monochorionic twin (MC) pregnancies, acute exsanguination in one twin can lead to severe complications in the co-twin due to the presence of inter-twin placental vascular connections. We report a MC pair with severe perinatal asphyxia due to acute exsanguination after prenatally undetected ruptured vasa previa. This resulted in severe hemorrhagic shock in both twins with double fatal outcome. Antenatal detection of vasa previa is of paramount importance to prevent severe morbidity and mortality, especially in MCs. A review of the literature is presented.