HYPERTROPHIC CARDIOMYOPATHY WITH A COMPLEX CLINICAL COURSE LEADING TO HEART TRANSPLANTATION

2022 ◽  
pp. 107406
Author(s):  
Uttam Savariya ◽  
Maria M. Patarroyo Aponte ◽  
Sriram Nathan ◽  
Bihong Zhao ◽  
Rajko Radovancevic ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Heart Transplantation ◽  
Clinical Course

Hypertrophic cardiomyopathy and mtDNA depletion. Successful treatment with heart transplantation

2002 ◽  
Vol 12 (1) ◽  
pp. 56-59 ◽  
Author(s):  
F.M. Santorelli ◽  
M.G. Gagliardi ◽  
C. Dionisi-Vici ◽  
F. Parisi ◽  
A. Tessa ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Heart Transplantation ◽  
Successful Treatment ◽  
Mtdna Depletion

Clinical course of middle-aged asymptomatic patients with hypertrophic cardiomyopathy

1992 ◽  
Vol 69 (9) ◽  
pp. 935-940 ◽  
Author(s):  
Gabriela M. Hecht ◽  
Julio A. Panza ◽  
Barry J. Maron
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Clinical Course ◽  
Middle Aged ◽  
Asymptomatic Patients

Abstract 4876: LAMP2 Cardiomyopathy is a Particularly Adverse Phenocopy of Sarcomeric Hypertrophic Cardiomyopathy

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Barry Maron ◽  
William C Roberts ◽  
Michael Arad ◽  
Carolyn Y Ho ◽  
Tammy S Haas ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Death ◽  
Heart Transplantation ◽  
Systolic Dysfunction ◽  
Ventricular Tachyarrhythmia ◽  
Young Patients ◽  
Disease Process ◽  
Left Ventricular ◽  
Asymptomatic Patients

Mutations in the X-linked lysosome-associated membrane protein gene (LAMP2; Danon disease) produce a morphologic phenocopy of sarcomeric hypertrophic cardiomyopathy (HCM) in young patients, characterized by extreme left ventricular (LV) hypertrophy and pre-excitation. However, the natural history of this newly recognized cardiomyopathy is incompletely resolved. Seven young asymptomatic patients with LAMP2 cardiomyopathy were identified at ages 8 to 15 years; 6 were male. LV hypertrophy was particularly marked (septal thickness 25– 65 mm; mean 42±17) in the presence of nondilated LV cavity. On each ECG, Wolff-Parkinson-White pre-excitation pattern was associated with markedly increased voltages (74±38mm for R- or S-wave). Over the 7±3 year follow-up from initial cardiac diagnosis, all 7 patients experienced particularly adverse disease consequences associated with progressive LV wall thinning and cavity dilatation and systolic dysfunction (ejection fraction, 29±7%) by the ages of 12 – 24 years (mean 20). Of the 7 patients, 5 either died of progressive heart failure, had heart transplantation or were considered for a donor heart; 2 others had sudden death events, including one fatal ventricular tachyarrhythmia refractory to defibrillator therapy and one appropriate defibrillator shock in an asymptomatic female survivor. Pathologic examination of hearts at autopsy showed histopathologic findings compatible with both HCM due to sarcomere protein mutations (i.e., extensive myocyte disarray, intramural small vessel disease, myocardial replacement scarring), and also evidence of a storage disease process (i.e., clusters of myocytes with vacuolated sarcoplasm within fibrotic areas). Heart weights, 1266 and 1425 grams, are the most substantial recorded for hypertrophic cardiomyopathies. LAMP2 cardiomyopathy is a uniformly profound, and particularly deleterious disease entity, causing refractory heart failure with systolic dysfunction as well as sudden death in young patients < 25 years of age. This novel phenocopy of sarcomeric HCM underscores the power of molecular diagnosis for predicting prognosis, and should also raise consideration for intervention with early heart transplantation.

Dilated phase of hypertrophic cardiomyopathy: high-risk reconstructive surgery as an alternative to heart transplantation

2021 ◽  
pp. 31
Author(s):  
S.L. Dzemeshkevich ◽  
A.P. Motreva ◽  
O.V. Kalmykova ◽  
Yu.B. Martyanova ◽  
D.G. Tarasov ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Heart Transplantation ◽  
Reconstructive Surgery

Adult Monozygotic Twins With Hypertrophic Cardiomyopathy and Identical Disease Expression and Clinical Course

2020 ◽  
Vol 127 ◽  
pp. 135-138
Author(s):  
Barry J. Maron ◽  
Ethan J. Rowin ◽  
Knarik Arkun ◽  
Hassan Rastegar ◽  
Amy M. Larson ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Clinical Course ◽  
Monozygotic Twins ◽  
Disease Expression
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