Post-Laser Twin Anemia Polycythemia Sequence: Diagnosis, Management, and Outcome in an International Cohort of 164 Cases.

The aim of this study was to investigate the management and outcome in the post-laser twin anemia polycythemia sequence (TAPS). Data of the international TAPS Registry, collected between 2014 and 2019, were used for this study. The primary outcomes were perinatal mortality and severe neonatal morbidity. Secondary outcomes included a risk factor analysis for perinatal mortality and severe neonatal morbidity. A total of 164 post-laser TAPS pregnancies were included, of which 92% (151/164) were diagnosed antenatally and 8% (13/164) postnatally. The median number of days between laser for TTTS and detection of TAPS was 14 (IQR: 7-28, range: 1-119). Antenatal management included expectant management in 43% (62/151), intrauterine transfusion with or without partial exchange transfusion in 29% (44/151), repeated laser surgery in 15% (24/151), selective feticide in 7% (11/151), delivery in 6% (9/151), and termination of pregnancy in 1% (1/151). The median gestational age (GA) at birth was 31.7 weeks (IQR: 28.6-33.7; range: 19.0-41.3). The perinatal mortality rate was 25% (83/327) for the total group, 37% (61/164) for donors, and 14% (22/163) for recipients (p < 0.001). Severe neonatal morbidity was detected in 40% (105/263) of the cohort and was similar for donors (43%; 51/118) and recipients (37%; 54/145), p = 0.568. Independent risk factors for spontaneous perinatal mortality were antenatal TAPS Stage 4 (OR = 3.4, 95%CI 1.4-26.0, p = 0.015), TAPS donor status (OR = 4.2, 95%CI 2.1-8.3, p < 0.001), and GA at birth (OR = 0.8, 95%CI 0.7-0.9, p = 0.001). Severe neonatal morbidity was significantly associated with GA at birth (OR = 1.5, 95%CI 1.3-1.7, p < 0.001). In conclusion, post-laser TAPS most often occurs within one month after laser for TTTS, but may develop up to 17 weeks after initial surgery. Management is mostly expectant, but varies greatly, highlighting the lack of consensus on the optimal treatment and heterogeneity of the condition. Perinatal outcome is poor, particularly due to the high rate of perinatal mortality in donor twins.

The effect of pregnancy on growth-dynamics of neurofibromas in Neurofibromatosis type 1.

INTRODUCTION: Patients with Neurofibromatosis type 1 (NF1) develop plexiform neurofibromas (PNF) and cutaneous neurofibromas. These tumors are a major cause of the patient's morbidity and mortality. An influence of estrogen and progesterone on tumor growth has been suggested but reports on growth or malignant transformation of tumors during pregnancy remain anecdotal. The purpose of this study was to quantify growth of cutaneous and plexiform neurofibromas in NF1 patients during pregnancy, and to assess the onset of NF1 related symptoms. MATERIAL AND METHODS: Retrospectively, 13 mothers with NF1 were included and compared to nullipara, nulligravida, age-matched women with NF1. All women received whole-body magnetic resonance imaging (MRI) before and after pregnancy or after a matched time period. Presence of plexiform and cutaneous neurofibromas was evaluated. PNF were subjected to semi-automated volumetry (MedX). The sum of the longest diameters (SLD) of representative cutaneous neurofibromas was determined for both groups. Clinical symptoms and subjective tumor growth were assessed. RESULTS: PNF were identified in 12/26 women (46.2%). Follow up showed neither new PNF nor a significant difference in growth rate (median tumor-growth/year: pregnant group-0.38% (IQR -1.1-5.4%) vs control group 3.59% (IQR -2.1-5.5%; P = 0.69). Malignant transformation of PNF was not observed. There was a significant growth of cutaneous neurofibromas in both groups (median SLD increase: pregnant group 17mm; P = 0.0026 / control group 12mm; P = 0.0004) The difference in increase of SLD was not significant (P = 0.48). Singular cutaneous neurofibromas in the pregnant group displayed high levels of tumor growth (>20%/year). NF1-associated symptoms and subjective tumor growth were not significantly increased in pregnant patients. CONCLUSIONS: Growth of plexiform and cutaneous neurofibromas in pregnant patients is not significantly different compared to non-pregnant patients. Cutaneous neurofibromas show a significant increase in growth over time in both, pregnant and non-pregnant patients and NF1 related clinical symptoms do not significantly aggravate during the course of pregnancy.

Dynamic fetal cardiovascular magnetic resonance imaging using Doppler ultrasound gating.

BACKGROUND: Fetal cardiovascular magnetic resonance (CMR) imaging may provide a valuable adjunct to fetal echocardiography in the evaluation of congenital cardiovascular pathologies. However, dynamic fetal CMR is difficult due to the lack of direct in-utero cardiac gating. The aim of this study was to investigate the effectiveness of a newly developed Doppler ultrasound (DUS) device in humans for fetal CMR gating. METHODS: Fifteen fetuses (gestational age 30-39 weeks) were examined using 1.5 T CMR scanners at three different imaging sites. A newly developed CMR-compatible DUS device was used to generate gating signals from fetal cardiac motion. Gated dynamic balanced steady-state free precession images were acquired in 4-chamber and short-axis cardiac views. Gating signals during data acquisition were analyzed with respect to trigger variability and sensitivity. Image quality was assessed by measuring endocardial blurring (EB) and by image evaluation using a 4-point scale. Left ventricular (LV) volumetry was performed using the single-plane ellipsoid model. RESULTS: Gating signals from the fetal heart were detected with a variability of 26 ± 22 ms and a sensitivity of trigger detection of 96 ± 4%. EB was 2.9 ± 0.6 pixels (4-chamber) and 2.5 ± 0.1 pixels (short axis). Image quality scores were 3.6 ± 0.6 (overall), 3.4 ± 0.7 (mitral valve), 3.4 ± 0.7 (foramen ovale), 3.6 ± 0.7 (atrial septum), 3.7 ± 0.5 (papillary muscles), 3.8 ± 0.4 (differentiation myocardium/lumen), 3.7 ± 0.5 (differentiation myocardium/lung), and 3.9 ± 0.4 (systolic myocardial thickening). Inter-observer agreement for the scores was moderate to very good (kappa 0.57-0.84) for all structures. LV volumetry revealed mean values of 2.8 ± 1.2 ml (end-diastolic volume), 0.9 ± 0.4 ml (end systolic volume), 1.9 ± 0.8 ml (stroke volume), and 69.1 ± 8.4% (ejection fraction). CONCLUSION: High-quality dynamic fetal CMR was successfully performed using a newly developed DUS device for direct fetal cardiac gating. This technique has the potential to improve the utility of fetal CMR in the evaluation of congenital pathologies.

Evaluation of a Portable Doppler Ultrasound Gating Device for Fetal Cardiac MR Imaging: Initial Results at 1.5T and 3T.

PURPOSE: Fetal cardiac MRI has the potential to play an important role in the assessment of fetal cardiac pathologies, but it is up to now not feasible due to a missing gating method. The purpose of this work was the evaluation of Doppler ultrasound (DUS) for external fetal cardiac gating with regard to compatibility, functionality, and reliability. Preliminary results were assessed performing fetal cardiac MRI. METHODS: An MRI conditional DUS device was developed to obtain a gating signal from the fetal heart. The MRI compatibility was evaluated at 1.5T and 3T using B1 field maps and gradient echo images. The quality and sensitivity of the DUS device to detect the fetal heart motion for cardiac gating were evaluated outside the MRI room in 15 fetuses. A dynamic fetal cardiac phantom was employed to evaluate distortions of the DUS device and gating signal due to electromagnetic interferences at 1.5T and 3T. In the first in vivo experience, dynamic fetal cardiac images were acquired in four-chamber view at 1.5T and 3T in two fetuses. RESULTS: The maximum change in the B1 field and signal intensity with and without the DUS device was <6.5% for 1.5T and 3T. The sensitivity of the DUS device to detect the fetal heartbeat was 99.1%. Validation of the DUS device using the fetal cardiac phantom revealed no electromagnetic interferences at 1.5T or 3T and a high correlation to the simulated heart frequencies. Fetal cardiac cine images were successfully applied and showed good image quality. CONCLUSION: An MR conditional DUS gating device was developed and evaluated revealing safety, compatibility, and reliability for different field strengths. In a preliminary experience, the DUS device was successfully applied for in vivo fetal cardiac imaging at 1.5T and 3T.

Fetal blood flow velocimetry by phase-contrast MRI using a new triggering method and comparison with Doppler ultrasound in a sheep model: a pilot study.

OBJECT: We present the first study demonstrating the feasibility of antenatal blood flow velocimetry performing ECG triggered phase-contrast (PC)-MRI in the fetal aorta by using a newly developed Doppler ultrasound trigger. MATERIALS AND METHODS: Five pregnant sheep carrying singleton fetuses (gestational age 121 days) were anesthetized to undergo fetal 2D PC-MRI in the fetal descending aorta (1.5 T) using a newly developed MR-compatible Doppler ultrasound trigger for fetal cardiac triggering. Inter-operator variability was assessed for PC-MR measurements and reproducibility was tested by repeated scans in one fetus. Inter-modality comparison was performed by Doppler ultrasound velocimetry. RESULTS: Fetal cardiac triggering was possible in all examinations. PC-MR velocimetry revealed a mean inter-operator variability of 3 ± 5%. Average peak systolic flow velocities of 62.5 ± 4.4 cm/s were in good agreement with Doppler ultrasound measurements of 62.0 ± 9.2 cm/s (p (Lord's U test) ≫ 0.05). CONCLUSION: Fetal PC-MR velocimetry was successfully performed using the newly developed MR-compatible Doppler ultrasound trigger for intrauterine fetal cardiac triggering, demonstrating high inter-operator and inter-modality agreement. This new method has the high potential for alternative assessment of hemodynamic decompensation of the fetal circulation.