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Fetal echocardiography
Published in Hung N. Winn, Frank A. Chervenak, Roberto Romero, Clinical Maternal-Fetal Medicine Online, 2021
Caroline K. Lee, Erik C. Michelfelder, Gautam K. Singh
In addition to VSDs, other heart defects may be identified in the four-chamber view (20). Ebstein’s anomaly of the tricuspid valve is characterized by apical displacement of the septal leaflet of the tricuspid valve with associated tricuspid regurgitation and atrialization of the RV. Atrioventricular canal (endocardial cushion) defects result in a spectrum of defects ranging from a primum atrial septal defect (ASD) with cleft mitral valve, to a complete atrioventricular canal defect with a single common atrioventricular (AV) valve, primum ASD, and inlet VSD. A primum ASD is seen when the inferior aspect of the atrial septum is deficient. An inlet VSD is seen when the ventricular septum adjacent to the AV valves is deficient. An atrioventricular canal defect should be suspected when the AV valves appear at the same level in the four-chamber view, without the usual offset of tricuspid and mitral valves.
Cardiac surgery
Published in Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie, Bailey & Love's Short Practice of Surgery, 2018
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie
Closure is performed during the first decade of life, even in the absence of symptoms, to avoid late-onset right ventricular failure, endocarditis and paradoxical emboli. In adults, closure is still appropriate for symptomatic improvement and avoidance of complications. The traditional method of closure involves open-heart surgery with CPB and closure of the defect, either directly with sutures, as with most secundum defects, or, if the defect is large, using a pericardial or synthetic patch. Closure of small to moderate ASDs using percutaneous catheter-delivered devices in the cardiology catheter laboratory is increasingly common. Primum atrioventricular defect repairs may require additional mitral valve repair. The operative mortality rate for isolated atrioventricular defect repairs is <1%, with an excellent prognosis. Surgical correction of complete atrioventricular canal defects, with closure of the ASD and ventricular septal components and mitral valve repair, is possible, but with a higher surgical mortality rate.
Congenital cardiac anomalies
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
A mitral valve cleft may be present without associated cardiac defects but is always present in the setting of atrioventricular canal (septal) defect, which may be complete, transitional or partial. Patients with partial atrioventricular canal defect have a cleft mitral valve and a primum atrial septal defect, while those with complete atrioventricular canal defect have an unrestrictive inlet ventricular septal defect as well. Transitional atrioventricular canal defects have a restrictive ventricular septal defect, and present similarly to partial atrioventricular canal defect patients, usually at several years of age.
The prenatal exome – a door to prenatal diagnostics?
Published in Expert Review of Molecular Diagnostics, 2021
James S Castleman, Elizabeth Wall, Stephanie Allen, Denise Williams, Samantha Doyle, Mark D Kilby
Cohorts with the highest diagnostic yields are not necessarily the patients for which fetal ES adds the most value. Diagnostic yields are higher in cohorts with a more severe phenotype [26,33,60,61], consanguinity and family history of fetal structural anomaly [27,39]. One cohort study (n = 41) reported a significantly higher yield in fetuses with abnormal ultrasound and positive family history (55.6%) compared to abnormal ultrasound alone (13%). However, ES arguably has superior utility in milder phenotypes [62] where there is a higher likelihood of providing valuable information not obtainable with ultrasound. In the PAGE study, learning disabilities were a feature of 16 molecular diagnoses made in fetuses with no CNS abnormalities on ultrasound scan. This included a pathogenic ANKRD11 variant (associated with KBG syndrome postnatal phenotype), diagnosed in a fetus presenting with an atrioventricular canal defect (AVSD) [18]. By contrast, 20/52 (38.5%) of diagnoses in the PAGE study were made in pregnancies ending in termination based on the ultrasound scan alone. Ongoing evaluation of patient outcomes is important to ensure utility and that alternative methods of ES (e.g., postmortem or postnatal) are no longer appropriate.
Seckel syndrome presenting with complete heart block
Published in Baylor University Medical Center Proceedings, 2021
Mostafa Abohelwa, Mohamed Elmassry, Marina Iskandir, Brandon Rogers, Deephak Swaminath
Systemic abnormalities associated with Seckel syndrome include central nervous system, hematopoietic, endocrine, cochlear, and cardiovascular anomalies.1,2 Cardiovascular manifestations are rare in this disease, with around 12 reported cases. Most are congenital cardiac anomalies; however, arrhythmias like severe sinus bradycardia and CHB have been reported as well.4–6 Reported anomalies include tetralogy of Fallot,7 tricuspid atresia,8 atrioventricular canal defect,9 Moyamoya-like vasculopathy,10 patent ductus arteriosus,11 complex heart lesion,11 and atrial septal defect.12 Howanietz et al reported a case of congenital heart failure with atrial septal defect, ventricular septal defect, and patent ductus arteriosus.13
Wider intraoperative glycemic fluctuation increases risk of acute kidney injury after pediatric cardiac surgery
Published in Renal Failure, 2018
Guo-Huang Hu, Lian Duan, Meng Jiang, Cheng-Liang Zhang, Yan-Ying Duan
During four years, the incidence of AKI was 11.5% (118/1026) in our pediatric cardiac surgery center. Of those with AKI, 53.4% (63/118), 30.5% (36/118), and 16.1% (19/118) were categorized as AKIN stages I, II, and III, respectively. The patients’ perioperative characteristics are shown in Table 1; diseases included but were not limited to tetralogy of Fallot with/without atrial septal defect, pulmonary atresia, anomalous pulmonary veins drainage, endocardial cushion defect, transposition of the great arteries, coronary artery fistula, aorta-pulmonary window, atrioventricular canal malformation, persistent truncus arteriosus, single ventricle or atrium, hypoplastic or interrupted aortic arch, and Ebstein or other valve anomalies. Before matching, the entire sample who experienced AKI were younger and cyanotic, had a higher RACHS-1 score, and less preoperative LVEF (all p < .01) when compared with those who did not experience AKI. They also underwent CPB for a longer time, longer OA time, more frequency of multiple OA, ultrafiltration, DHCA or hypoperfusion (all p < .01) during surgery. They had higher mG, pG, intraGF, IS POD, and more transfusions during the perioperative period (all p < .01).