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Last update 08 May 2025

Endocardial Cushion Defects

Last update 08 May 2025

Basic Info

Synonyms

Atrioventricular canal, Cushion Defect, Endocardial, Cushion Defects, Endocardial

+ [37]

Introduction

A spectrum of septal defects involving the ATRIAL SEPTUM; VENTRICULAR SEPTUM; and the atrioventricular valves (TRICUSPID VALVE; BICUSPID VALVE). These defects are due to incomplete growth and fusion of the ENDOCARDIAL CUSHIONS which are important in the formation of two atrioventricular canals, site of future atrioventricular valves.

Clinical Trials associated with Endocardial Cushion Defects

NCT04106479

/ RecruitingNot ApplicableIIT

Do Cerebral and Renal Saturations Measured With Near-infrared Spectroscopy Correlate With Echocardiographic Markers of Perfusion and Cardiac Performance in Congenital Heart Disease?

Neonatal patients with congenital heart defects (CHD) have changing physiology in the context of transitional period. Patients with CHD are at risk of low perfusion status or abnormal pulmonary blood flow. Near infrared spectroscopy has been used in neonatal intensive care units (NICU) to measure end-organ perfusion. The investigator plan on monitoring newborns with CHD admitted to the NICU with NIRS and echocardiography during the first week of life and correlate measures of perfusion from Dopplers to cerebral and renal NIRS.

Start Date11 Oct 2019

Sponsor / Collaborator

Centre universitaire de santé McGill

[+1]

NCT03079401

/ Active, not recruitingPhase 1/2IIT

Mesoblast Stem Cell Therapy for Patients With Single Ventricle and Borderline Left Ventricle

Patients under the age of 5, with a diagnosis of hypoplastic left heart syndrome (HLHS), unbalanced atrioventricular canal (uAVC), or borderline left heart who are undergoing staged LV recruitment following bidirectional Glenn (BDG) or undergoing BDG with plans for LV recruitment will be considered for enrollment in this study. Those patients enrolled in the study will be randomized to either the experimental arm or control arm of the study. Those patients randomized to the experimental arm will receive mesenchymal precursor cells (MPCs) injected directly into the LV endocardium during their LV recruitment or BDG procedure. Those patients randomized to the control arm will receive normal standard of care during their procedure with no injection of MPCs.
It is believed that injection of MPCs will help improve the chances of those patients with single ventricle or borderline left ventricle being converted to biventricular circulation which could improve quality of life and longevity over palliation.

Start Date27 Nov 2017

Sponsor / Collaborator

The Children's Hospital Corp.

NCT00199771

/ CompletedPhase 2IIT

The Use of Hypertonic Saline Dextran in Cardiac Surgery Utilizing Cardio Pulmonary Bypass in Children Less Than 17 Weeks Old.

The purpose of this study is to determine whether infusion of hypertonic saline dextran attenuates the inflammatory response and the water overload, during and after major cardiac surgery in small children.

Start Date01 Jun 2003

Sponsor / Collaborator

Oslo universitetssykehus HF

[+1]

100 Clinical Results associated with Endocardial Cushion Defects

100 Translational Medicine associated with Endocardial Cushion Defects

0 Patents (Medical) associated with Endocardial Cushion Defects

604

Literatures (Medical) associated with Endocardial Cushion Defects

01 Mar 2025·The Journal of Thoracic and Cardiovascular Surgery

Single ventricle palliation in congenitally corrected transposition of the great arteries: An international multicenter study

Article

Author: Baruteau, Alban-Elouen ; Belli, Emre ; Kowalik, Ewa ; Bacha, Emile A. ; Kalfa, David M. ; Elder, Robert W ; Barron, David J ; Kalfa, David M ; Babu-Narayan, Sonya V ; Elder, Robert W. ; Barron, David J. ; Bacha, Emile A ; Khairy, Paul ; Babu-Narayan, Sonya V. ; Buratto, Edward ; Nield, Lynne ; McLeod, Christopher ; Ly, Mohamed

BACKGROUNDSingle ventricle palliation may be performed in patients with congenitally corrected transposition of the great arteries due to hypoplasia of 1 ventricle or anatomic complexity rendering biventricular repair unfeasible. There have been only a few small studies of the outcomes of single ventricle palliation in the setting of congenitally corrected transposition of the great arteries.METHODSA multicenter, international, retrospective cohort study of patients with congenitally corrected transposition of the great arteries undergoing single ventricle palliation was conducted in 29 tertiary hospitals in 6 countries from 1990 to 2018.RESULTSA total of 194 patients with congenitally corrected transposition of the great arteries underwent single ventricle palliation. A functionally univentricular heart was present in 62.4% (121/194). Patients with 2 good-sized ventricles had more laterality defects (50.7% vs 28.1%, P = .002). Fontan completion was achieved in 80.3% (155/194). A tricuspid valve surgery was performed in 9.3% (18/194). Survival was 97.4% (95% CI, 92.1-99.2) at 15 years in the univentricular cohort and 89.8% (95% CI, 78.0-95.5) at 15 years in those with 2 adequate ventricles (P = .05). At last follow-up, 11.5% of patients had heart failure, whereas 6.0% had moderate or greater systemic right ventricle dysfunction and 8.4% exhibited moderate or greater tricuspid regurgitation. In multivariable analysis, aortic coarctation or hypoplasia (hazard ratio, 7.7; P = .005) was associated with mortality.CONCLUSIONSSingle ventricle palliation in patients with congenitally corrected transposition of the great arteries is associated with excellent long-term survival and low rates of heart failure and atrioventricular valve failure. In patients who would require complex surgery to achieve a biventricular repair, single ventricle palliation appears to be a good alternative.

01 Dec 2024·The Journal of Thoracic and Cardiovascular Surgery

Concomitant ablation of atrial fibrillation: New pacemakers and early rhythm recovery

Article

Author: Passman, Rod S ; Cox, James L ; Kaplan, Rachel ; Kruse, Jane ; Andrei, Adin-Cristian ; Churyla, Andrei ; McCarthy, Patrick M

OBJECTIVENew permanent pacemaker (PPM) implantation after concomitant atrial fibrillation (AF) ablation has been associated with surgical ablation (SA). We sought to determine factors for PPM use as well as early rhythm recovery.METHODSFrom 2004 through 2019, 6135 patients underwent valve surgery and were grouped: No AF (n = 4584), AF no SA (n = 346), and AF with SA (n = 1205) to evaluate predischarge PPM and 3-month rhythm recovery (intrinsic heart rate >40 beats per minute).RESULTSOverall, 282 (4.6%) patients required a predischarge PPM: atrioventricular node dysfunction in 75.3%, sick sinus syndrome in 19.1%, both (5%), and indeterminate (0.7%). Patients with AF had more PPMs: AF with SA (7.9%) versus AF no SA (6.9%) versus No AF (3.6%) (P < .001). For patients with AF, PPM rates were not significantly higher for ablation patients (7.6% SA vs 6.9% AF no SA; P = .56). There were differences in PPM by SA lesion set (biatrial 12.8%; left atrial only 6.1%; pulmonary vein isolation 3.0%; P < .001). Among patients with AF treated with 3-month PPM follow-up, rhythm recovery was common (35 out of 62 [56.5%]) and did not differ by lesion set. Rhythm recovery was seen in 63 out of 141 (44.7%) in the atrioventricular node dysfunction group versus 24 out of 35 (68.6%) in the sick sinus syndrome group (P = .011). In propensity score-matched groups, late survival was similar (P = .63) for new PPM patients.CONCLUSIONSAvoiding conduction system trauma and delaying implantation reduces the need for postoperative PPM. Rhythm recovery within 3 months is frequent, especially for patients with sick sinus syndrome. A conservative approach to the implantation of a new PPMs is warranted.

01 Nov 2024·Taiwanese Journal of Obstetrics and Gynecology

Prenatal diagnosis of Jacobsen syndrome associated with a distal 11q deletion and a distal 8q duplication by chromosome microarray analysis in a fetus with a de novo unbalanced translocation of 46,XX,der(11)t(8;11)(q24.13;q23.3) and multiple congenital anomalies on fetal ultrasound

Article

Author: Chen, Wen-Lin ; Lee, Chen-Chi ; Wu, Fang-Tzu ; Wu, Peih-Shan ; Huang, Jian-Pei ; Wang, Wayseen ; Chen, Chih-Ping ; Pan, Yen-Ting

OBJECTIVEWe present prenatal diagnosis of Jacobsen syndrome associated with a distal 11q deletion and a distal 8q duplication by chromosome microarray analysis (CMA) in a fetus with multiple congenital anomalies on fetal ultrasound.CASE REPORTA 41-year-old, gravida 2, para 1, woman underwent amniocentesis at 25 weeks of gestation because of intrauterine growth restriction, endocardial cushion defect, clenched hands, arthrogryposis, rocker bottom feet and craniosynostosis on fetal ultrasound. Amniocentesis revealed a karyotype of 46,XX,add(11)(q23.3). Array comparative genomic hybridization (aCGH) analysis of the DNA extracted from the uncultured amniocytes revealed the result of arr 8q24.13q24.3 × 3, 11q23.3q25 × 1. Analysis of FGFR2 revealed no mutation. The karyotype was 46,XX,der(11)t(8;11)(q24.13;q23.3). The parental karyotypes were normal. The pregnancy was subsequently terminated, and a dead malformed fetus was delivered with craniofacial dysmorphism of low-set malformed ears, depressed nasal bridge, hypertelorism, small mouth, clenched hands and rocker bottom feet. Cytogenetic analysis of the placenta revealed a karyotype of 46,XX,der(11)t(8;11)(q24.13;q23.3). aCGH analysis of the DNA extracted from the umbilical cord showed the result of arr 8q24.13q24.3 (126,302,369-146,280,020) × 3.0, arr 11q23.3q25 (120,469,928-134,868,407) × 1.0 [GRCh37] with a 19.978-Mb duplication of 8q24.13-q24.3 and a 14.398-Mb deletion of 11q23.3-q25 encompassing the genes of BSX, ETS1, FLI1 and ARHGAP32.CONCLUSIONCMA is useful for detection of de novo chromosomal rearrangement in the fetus with multiple congenital anomalies on fetal ultrasound.

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Endocardial Cushion Defects

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