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• W3166184546 abstract "Double-outlet left atrium (DOLA) is a rare congenital heart defect with a challenging prenatal diagnosis, in which the left atrium drains into both the left and right ventricles. We report on a prenatally diagnosed case of DOLA and highlight key differentiating features that facilitate the diagnosis of this malformation. We also summarize previously reported cases of DOLA. Schematic and sonographic images of fetal heart with double-outlet left atrium (LA). (a) Four-chamber view showing the LA communicating with both the right (RV) and left (LV) ventricles via a common atrioventricular (AV) valve and absence of direct communication between the right atrium (RA) and the RV. The flap valve of the fossa ovalis (FO) bows from right to left. The descending aorta (DAo) is seen to the right of the spine. (b) Images, obtained during systole and diastole, showing a double-outlet RV with malpositioned outflow tracts, a prominent pulmonary artery (PA) and an aorta (Ao). The LA is communicating with the LV and RV and a ventricular septal defect (VSD) is present. The flap valve of the FO bows from right to left. A 38-year-old woman, G3P2002, with a non-contributory history, was referred to our institution at 34 weeks for a fetal complex cardiac defect. Notable fetal sonographic findings included levocardia and DOLA with a common atrioventricular valve, bridging leaflets committed in a balanced fashion to both ventricles and the annulus extending between the free walls of the left and right ventricles. The ventricles had a superior–inferior relationship. A large unrestrictive inlet-to-outlet ventricular septal defect and a double-outlet right ventricle with malposed great arteries were observed (Figure 1, Videoclip S1). Examination of the aorta revealed borderline hypoplasia of the aortic annulus with unobstructed antegrade flow, a mildly hypoplastic ascending aorta and moderate-to-severe hypoplasia of the transverse aortic arch with a hypoplastic isthmus (Videoclips S2–S4). Heart failure medications (digoxin, furosemide, salbutamol) Remained in functional class-II heart failure until loss to follow-up Surgical repair, bilateral superior cavopulmonary anastomosis at 6 months of age A liveborn male was delivered at 39 weeks, weighing 3360 g and with Apgar scores of 2, 6 and 8 at 1, 5 and 10 min, respectively. The neonate required continuous positive airway pressure (FiO2, 40%) and received prostaglandin E. Postnatal echocardiography and gated cardiac computed tomographic angiography confirmed the prenatal findings and showed no significant atrial septal restriction (Figures S1–S3, Videoclips S5–S7). At 3 weeks of age, given the degree of atrioventricular valve regurgitation and absence of significant gradient through the subaortic area, the decision was made to proceed with atrial septectomy, bilateral branch pulmonary artery banding and repair of the aortic coarctation. There was no significant subaortic gradient after initial palliation. At 4 months, the infant underwent bidirectional Glenn procedure and Damus–Kaye–Stansel aortoplasty. At the time of writing, the infant was clinically well. Cardiac embryogenesis is a complex process that begins at 18 days of gestation with formation of the cardiac crescent from the precardiac mesoderm. D-loop formation of the heart tube is typically completed by 4 weeks' gestation, at which point the development of the left and right ventricles begins. At 5 weeks, the atria are fixed into position by the great vessels as the bulboventricular heart undergoes migration, the aorticopulmonary septum separates the aorta and the pulmonary artery, the mitral and tricuspid valves separate and the ventricular septum develops and moves leftward to align with the atrial septum1. If the septum does not migrate far enough to the left, a double-outlet right atrium (DORA) is created, whereas, if it migrates too far left, DOLA is created. This misalignment is the most commonly presented theory behind the development of DORA and DOLA2, 3. Concurrently, the atrial septum develops through a separate process. The septum primum forms early in development, followed by the formation of the septum secundum several weeks later1. It has been proposed that abnormal development of the septum primum resulting in improper separation of the atrioventricular canal could lead to DOLA or DORA4. However, this theory is less favored. Thirteen other cases of DOLA have been reported in the literature to date, with one case diagnosed prenatally at 20 weeks' gestation and the remaining cases diagnosed postnatally between 20 days and 41 years of age (Table 1)3, 5-12. Our report provides insight into the prenatal diagnosis and management of DOLA. While the differential diagnosis in the absence of a connection between the right atrium and the right ventricle includes first and foremost tricuspid atresia, differentiating DOLA from tricuspid atresia hinges on the commitment of the left atrium to both ventricles. In addition, atrioventricular valve atresia is typically associated with a hypoplastic ventricle. Awareness of DOLA and thorough, systematic examination of the fetal heart should facilitate the prenatal detection of this rare condition and enable proper management and optimization of neonatal outcome. Not applicable Videoclip S1 Schematic representation of the heart showing double-outlet left atrium, atrioventricular canal, double-outlet right ventricle and aortic coarctation. Blood enters the right atrium (RA) from the venae cavae. It then flows through the fossa ovalis into the left atrium (LA). From there, it enters both the left (LV) and right (RV) ventricles, which communicate via an atrioventricular septal defect. The blood then leaves from the RV via the malposed pulmonary artery and the aortic arch. There is an aortic coarctation, leading to reversal of flow through the aortic isthmus. The ductus arteriosus supplies the descending aorta due to the coarctation. Videoclip S2 Prenatal grayscale ultrasound of the fetal heart with double-outlet left atrium, showing a redundant atrial septum bowing from right to left and the atrioventricular valve committed to both ventricles in a balanced fashion. Videoclip S3 Prenatal grayscale ultrasound of the fetal heart, showing features of a double-outlet right ventricle with bilateral conus. The posterior great vessel bifurcates, consistent with a pulmonary artery. Videoclip S4 Prenatal grayscale ultrasound of the fetal heart in a sagittal plane, showing the inferior vena cava returning to the right atrium, the redundant atrial septum bowing from right to left posterior to the inferior vena cava entrance and the Eustachian valve anterior to the inferior vena cava. Videoclip S5 Postnatal echocardiography in the apical four-chamber orientation, showing a double-outlet right ventricle with malposed great vessels, the pulmonary valve and bifurcation of the main pulmonary artery and the subaortic conus to the right of the pulmonary valve, demonstrating the subaortic narrowing. The aortic valve is not seen in this videoclip. Videoclip S6 Postnatal echocardiography in the parasternal short-axis orientation, showing an enface view of the common atrioventricular valve with characteristic features, including the anterior and posterior bridging leaflets committed to both ventricles. The valve is ‘balanced’, meaning that it is relatively equally committed to both ventricles. Videoclip S7 Postnatal echocardiography in the suprasternal view, demonstrating transverse aortic arch hypoplasia, coarctation of the aorta and a large, unrestrictive ductus arteriosus with bidirectional shunting. Figure S1 Postnatal echocardiographic image in the apical four-chamber orientation, showing a balanced complete atrioventricular septal defect committed to both ventricles. Figures S2 and S3 Postnatal echocardiographic images in the parasternal short-axis orientation, showing the morphology of the common atrioventricular valve in diastole (Figure S2) and in systole (Figure S3). Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article." @default.
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• W3166184546 date "2021-12-01" @default.
• W3166184546 modified "2023-09-28" @default.
• W3166184546 title "Prenatal diagnosis of double‐outlet left atrium" @default.
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• W3166184546 doi "https://doi.org/10.1002/uog.23704" @default.
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