Association Between Mutations in the NKX2.5 Homeobox, Atrial Septal Defects, Ventricular Noncompaction and Sudden Cardiac Death

2018 ◽  
Vol 71 (1) ◽  
pp. 53-55 ◽  
Author(s):  
Julián Palomino Doza ◽  
Rafael Salguero-Bodes ◽  
María de la Parte ◽  
Fernando Arribas-Ynsaurriaga
2011 ◽  
Vol 412 (1-2) ◽  
pp. 170-175 ◽  
Author(s):  
Ping Ouyang ◽  
Elizabeth Saarel ◽  
Ying Bai ◽  
Chunyan Luo ◽  
Qiulun Lv ◽  
...  

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Michael McGee ◽  
Luke Warner ◽  
Nicholas Collins

Ebstein’s anomaly is a congenital disorder characterized by apical displacement of the septal leaflet of the tricuspid valve. Ebstein’s anomaly may be seen in association with other cardiac conditions, including patent foramen ovale, atrial septal defect, and left ventricular noncompaction (LVNC). LVNC is characterized by increased trabeculation within the left ventricular apex. Echocardiography is often used to diagnose LVNC; however, magnetic resonance (MR) imaging offers superior characterization of the myocardium. We report a case of sudden cardiac death in a patient with Ebstein’s anomaly with unrecognized LVNC noted on post mortem examination with screening documenting the presence of LVNC in one of the patient’s twin sons.


2012 ◽  
Vol 36 (5) ◽  
pp. e153-e155 ◽  
Author(s):  
WILLIAM LEE ◽  
DENNIS L. KUCHAR ◽  
BRUCE D. WALKER ◽  
RAJESH N. SUBBIAH

2012 ◽  
Vol 7 (1) ◽  
pp. 39-41
Author(s):  
Nilufar Fatema ◽  
Ahmed Saiful Bari ◽  
Naznin Sultana ◽  
Mohammad Al Mamun ◽  
SM Mustafa Sultan ◽  
...  

Ventricular noncompaction is a kind of cardiomyopathy which is called Noncompaction Cardiomyopathy (NCC). In this condition in which the muscular wall of the main pumping chamber of the heart -the left ventricle (LV) appears to be spongy and “non-compacted”, consisting of a meshwork of numerous muscle bands called trabeculations. Here, anatomically LV wall has deep trabeculations. This condition is associated major clinical problems like systolic and diastolic dysfunction, arrhythmia and even systemic embolism. Sudden cardiac death may occur in this group. Early detection of this condition may help to plan the management. DOI: http://dx.doi.org/10.3329/uhj.v7i1.10209 UHJ 2011; 7(1): 39-41


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
D Cortez ◽  
H Morrell

Abstract Background The most common cause of sudden cardiac death in the United State of America is hypertrophic cardiomyopathy, while other causes such as left ventricular hypertrophy, ion-channelopathies, and other causes are also significant. Screening programs typically involve electrocardiograms with mainly false positive results. Little data regarding EKG and echocardiographic screening combined have been published. Methods During 2016–2019, over 15000 patients were screened by the Heartfelt Cardiac screening program. Screening electrocardiograms and echocardiograms including parasternal short/long axis, 4-chamber views, coronary assessment with color doppler and subcostal views with color doppler for assessment of septal defects, were performed. Results 15,329 patients were screened under 35 years of age, mean age 17 years, with 36 hypertrophic cardiomyopathy patients, 61 borderline left ventricular hypertrophy patients noted, 30 patients with left ventricular dysfunction were noted, 47 patients with mitral valve prolapse, 27 patients with atrial septal defects, 39 patients with Wolf-Parkinson-White, 21patients with ideopathic dilated cardiomyopathy were discovered. Screening electrocardiography was helpful for Wolf-Parkinson-White identification, while echocardiogram was most helpful for detection of the above other defects. Screening EKG only identified 45% of those patients with myocardial disease (per above) with the modified Seattle Screening and ESC screening criteria. Conclusion Screening echocardiogram adds value for screening athletes under 35 years at risk for sudden cardiac death. Consideration of cost-effective echo screening should be considered as part of routine screening.


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