Summary Background Two-dimensional (2D) ultrasound echocardiography is the primary technique used to diagnose congenital heart disease before birth. There is, however, a longstanding need for a reliable form of secondary imaging, particularly in cases when more detailed three-dimensional (3D) vascular imaging is required, or when ultrasound windows are of poor diagnostic quality. Fetal MRI, which is well established for other organ systems, is highly susceptible to fetal movement, particularly for 3D imaging. The objective of this study was to investigate the combination of prenatal MRI with novel, motion-corrected 3D image registration software, as an adjunct to fetal echocardiography in the diagnosis of congenital heart disease. Methods Pregnant women carrying a fetus with known or suspected congenital heart disease were recruited via a tertiary fetal cardiology unit. After initial validation experiments to assess the general reliability of the approach, MRI data were acquired in 85 consecutive fetuses, as overlapping stacks of 2D images. These images were then processed with a bespoke open-source reconstruction algorithm to produce a super-resolution 3D volume of the fetal thorax. These datasets were assessed with measurement comparison with paired 2D ultrasound, structured anatomical assessment of the 2D and 3D data, and contemporaneous, archived clinical fetal MRI reports, which were compared with postnatal findings after delivery. Findings Between Oct 8, 2015, and June 30, 2017, 101 patients were referred for MRI, of whom 85 were eligible and had fetal MRI. The mean gestational age at the time of MRI was 32 weeks (range 24–36). High-resolution (0·50–0·75 mm isotropic) 3D datasets of the fetal thorax were generated in all 85 cases. Vascular measurements showed good overall agreement with 2D echocardiography in 51 cases with paired data (intra-class correlation coefficient 0·78, 95% CI 0·68–0·84), with fetal vascular structures more effectively visualised with 3D MRI than with uncorrected 2D MRI (657 [97%] of 680 anatomical areas identified vs 358 [53%] of 680 areas; p<0·0001). When a structure of interest was visualised in both 2D and 3D data (n=358), observers gave a higher diagnostic quality score for 3D data in 321 (90%) of cases, with 37 (10%) scores tied with 2D data, and no lower scores than for 2D data (Wilcoxon signed rank test p<0·0001). Additional anatomical features were described in ten cases, of which all were confirmed postnatally. Interpretation Standard fetal MRI with open-source image processing software is a reliable method of generating high-resolution 3D imaging of the fetal vasculature. The 3D volumes produced show good spatial agreement with ultrasound, and significantly improved visualisation and diagnostic quality compared with source 2D MRI data. This freely available combination requires minimal infrastructure, and provides safe, powerful, an...

Biventricular repair, especially Rastelli-type reconstruction, is associated with higher late mortality and reintervention than is Fontan repair. The wisdom of extending biventricular repair to borderline anatomic candidates with hypoplastic left-sided structures or a nonsubaortic ventricular septal defect is questionable.

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Background: Identifying fetuses at risk of severe neonatal coarctation of the aorta (CoA) can be lifesaving but is notoriously challenging in clinical practice with a high rate of false positives. Novel fetal 3-dimensional and phase-contrast magnetic resonance imaging (MRI) offers an unprecedented means of assessing the human fetal cardiovascular system before birth. We performed detailed MRI assessment of fetal vascular morphology and flows in a cohort of fetuses with suspected CoA, correlated with the need for postnatal intervention. Methods: Women carrying a fetus with suspected CoA on echocardiography were referred for MRI assessment between 26 and 36 weeks of gestation, including high-resolution motion-corrected 3-dimensional volumes of the fetal heart and phase-contrast flow sequences gated with metric optimized gating. The relationship between aortic geometry and vascular flows was then analyzed and compared with postnatal outcome. Results: Seventy-two patients (51 with suspected fetal CoA and 21 healthy controls) underwent fetal MRI with motion-corrected 3-dimensional vascular reconstructions. Vascular flow measurements from phase-contrast sequences were available in 53 patients. In the CoA group, 25 of 51 (49%) required surgical repair of coarctation after birth; the remaining 26 of 51 (51%) were discharged without neonatal intervention. Reduced blood flow in the fetal ascending aorta and at the aortic isthmus was associated with increasing angulation ( P =0.005) and proximal displacement ( P =0.006) of the isthmus and was seen in both true positive and false positive cases. A multivariate logistic regression model including aortic flow and isthmal displacement explained 78% of the variation in outcome and correctly predicted the need for intervention in 93% of cases. Conclusions: Reduced blood flow though the left heart is associated with important configurational changes at the aortic isthmus in fetal life, predisposing to CoA when the arterial duct closes after birth. Novel fetal MRI techniques may have a role in both understanding and accurately predicting severe neonatal CoA.

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Evidence indicates that patients with coarctation of the aorta (COA) suffer from increased cardiovascular morbidity and mortality in later life despite successful repair of COA in childhood. Systolic arterial hypertension is common, presenting in up to one-third of patients, and is regarded as the main driver of premature cardiovascular events in this group of patients. In this review, we discuss the prevalence and pathophysiology of hypertension in children following successful COA repair with no residual arch obstruction. The challenges in accurate blood pressure assessment at this early phase are considered and non-invasive measures of central blood pressure are discussed. Although the pathways for investigations in adults are well defined, we highlight the need to address the issues of cardiovascular surveillance in children and describe techniques which can provide complementary information for cardiovascular assessment in this group of patients such that timely treatment can occur.

A right aortic arch (RAA) with a left arterial duct (LAD) together encircle the trachea and have the potential to cause tracheobronchial compression and published guidelines recommend bronchoscopy in symptomatic patients. The aim of the study was to describe the incidence of tracheal compression in a cohort of prenatally diagnosed RAA and LAD. Retrospective review of clinical course and imaging of prenatal cases of RAA and LAD assessed with flexible bronchoscopy over an 11-year period. 34 cases of prenatally diagnosed RAA with LAD underwent bronchoscopy at median age of 9 months (range 0.4-123) of whom 11 had respiratory symptoms and 23 were asymptomatic. In the neonatal period, three cases demonstrated respiratory symptoms. An aberrant left subclavian artery (ALSA) was identified in 29 cases. Pulsatile tracheal compression was identified in 32/34 (94%) cases and two cases showed normal tracheal appearances. Significant tracheal compression (> 70% occlusion) was present in 25/34 (74%) cases of which 16 were asymptomatic. Significant carinal compression (> 70% occlusion) was identified in 14/34 (42%) cases, an ALSA was observed in 13/14. Surgical relief of a vascular ring has been performed in 27 (79%) cases at a median age of 15 months (range 0.6-128 months). At surgery, a fibrous remnant of an atretic left aortic arch was identified in 11/27 (41%) cases. Significant tracheal compression may be present in infants even without symptoms. If early relief of airway compression is to be achieved to promote normal development of tracheal cartilage, early bronchoscopy should be considered.

Objectives Prenatal diagnosis of coarctation of the aorta (CoA) is associated with reduced mortality and morbidity, however, accurate prenatal prediction remains challenging. To date, studies have used retrospective measurements of the outflow tracts to evaluate their potential to predict CoA. Our primary objective was to evaluate prospectively acquired measurements of the outflow tracts in fetuses with prenatally suspected CoA. A secondary aim was to report the postnatal prevalence of bicuspid aortic valve in this cohort. Methods Pregnancies with suspicion of isolated CoA and with a minimum of 6 months' postnatal follow‐up available were identified from the cardiac database of a tertiary fetal cardiology center in the UK, between January 2002 and December 2017. Measurement of the aortic valve, pulmonary valve, distal transverse aortic arch (DTAA) and arterial duct (AD) diameters were undertaken routinely in fetuses with suspected CoA during the study period. Z‐scores were computed using published reference ranges based on > 7000 fetuses from our own unit. Results Of 149 pregnancies with prenatally suspected CoA included in the study, CoA was confirmed within 6 months after birth in 77/149 (51.7%) cases. DTAA diameter Z‐score and the Z‐score of second‐trimester DTAA/AD diameter ratio were smaller in fetuses with postnatally confirmed CoA than those in false‐positive cases (–2.8 vs –1.9; P = 0.039 and –3.13 vs –2.61; P = 0.005, respectively). Multiple regression analysis demonstrated that the Z‐scores of DTAA and AD diameters were the only significant predictors of postnatal CoA (P = 0.001). Bicuspid aortic valve was identified in 30% of the false‐positive cases. Conclusions Measurement of DTAA and AD diameter Z‐scores can be used to ascertain risk for postnatal CoA in a selected cohort. The high incidence of bicuspid aortic valve in false‐positive cases merits further study with respect to both etiology and longer‐term significance. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.