Alex J. Thompson¹, Joseph A. Dearani², Jonathan N. Johnson^1,3, Hartzell V. Schaff², Eric C. Towe¹, Jared Palfreeman¹, Philip L. Wackel¹, Frank Cetta^1,3

Congenital Heart Disease, Vol.13, No.4, pp. 617-623, 2018, DOI:10.1111/chd.12618

Abstract Background: The transapical approach has been utilized in adult HCM patients with either midventricular obstruction or a small LV cavity; however, there are little data on its use in children.
Methods: We retrospectively reviewed all patients (age <21 years) with HCM who underwent a transapical myectomy from January 2002 to December 2016. Indication for surgery was midventricular obstruction in 19/23 (83%) and small LV cavity in 4 (17%). Preoperative symptoms included: dyspnea (96%), chest pain (65%), presyncope (61%), and syncope (35%). The mean age at the time of operation was 14 ± 4.0 years (range, 4–20).
Results: Overall,… More >

Long Deng¹, Xueying Huang^2,3,*, Heng Zuo⁴, Yuan Zheng², Chun Yang⁵, Yunhu Song¹, Dalin Tang⁶

CMES-Computer Modeling in Engineering & Sciences, Vol.116, No.2, pp. 115-125, 2018, DOI:10.31614/cmes.2018.04076

Abstract The mechanisms of systolic anterior motion (SAM) of the mitral valve in hypertrophic obstructive cardiomyopathy (HOCM) remain unclear. To investigate the angle of attack between blood flow and mitral valve leaflets at pre-SAM time point, patient-specific CT-based computational models were constructed for 5 patients receiving septal myectomy surgery to obtain pre- and post-operative 2D vector flow mapping. The comparisons between pre- and post-operative angles of attack based on 2D vector flow mapping of 5 patients were performed. It was found that there was no statistically significant difference between pre- and post-operative angles of attack (61.1±t More >

Monique M. Gardner, Meryl S. Cohen

Congenital Heart Disease, Vol.12, No.6, pp. 783-786, 2017, DOI:10.1111/chd.12506

Abstract Background: Noncompaction is a poorly understood form of cardiomyopathy that typically affects the left ventricle and may be associated with congenital heart disease. Right ventricular noncompaction (RVNC) may occur when the left ventricle is affected but is rarely seen in isolation. RVNC may have clinical significance affecting surgical and long-term outcomes. We describe the diagnosis and clinical course in three patients at our institution.
Methods: We performed a retrospective review of patients diagnosed with RVNC over a 12- month period at our institution and reviewed their imaging and clinical course.
Results: Three patients were identified. All had diagnosis… More >

Mark S. Link

Congenital Heart Disease, Vol.12, No.5, pp. 597-599, 2017, DOI:10.1111/chd.12494

Abstract Sudden cardiac death (SCD), particularly in the young athlete, is a rare though devastating event for families, institutions, and communities at large. It can also affect the nonathlete and occur at rest, although most commonly associated with exercise activities and/or sports participation. Common causes of SCD include cardiomyopathies, particularly hypertrophic cardiomyopathy in the United States, congenital coronary artery anomalies, channelopathies, among others. This report will explore an overview of the prevalence and causes of SCD in the young. More >

Kari L. Turkowski¹, David J. Tester^2,3, J. Martijn Bos^2,4, Kristina H. Haugaa⁵, Michael J. Ackerman^2,3,4

Congenital Heart Disease, Vol.12, No.2, pp. 226-235, 2017, DOI:10.1111/chd.12462

Abstract Background: Arrhythmogenic cardiomyopathy (ACM) is a heritable disease characterized by fibrofatty replacement of cardiomyocytes, has a prevalence of approximately 1 in 5000 individuals, and accounts for approximately 20% of sudden cardiac death in the young (≤35 years). ACM is most often inherited as an autosomal dominant trait with incomplete penetrance and variable expression. While mutations in several genes that encode key desmosomal proteins underlie about half of all ACM, the remainder is elusive genetically. Objective: Here, whole exome sequencing (WES) was performed with genomic triangulation in an effort to identify a novel explanation for a… More >

S.S. Bombicino^*, D.E. Iglesias, I.A. Rukavina Mikusic, A. Boveris, L.B. Valdez

BIOCELL, Vol.40, No.1, pp. 7-10, 2016, DOI:10.32604/biocell.2016.40.007

Abstract Diabetic cardiomyopathy, i.e. the ventricular dysfunction in the absence of hypertension or coronary arterial disease, is a common complication of diabetes mellitus that leads to a heightened risk of heart failure and death among diabetic patients. This contractile dysfunction could be associated to mitochondrial dysfunction, in which mitochondrial biogenesis could emerge as a compensatory mechanism triggered in response to hyperglycemia. It has been proposed that nitric oxide synthase activities with enhanced NO production are involved in this process. Alterations in the contractile response and lusitropic reserve were observed in streptozotocin diabetic rats after β-adrenergic stimuli. Additionally,… More >