Presentation
We describe a sporadic case of an infant with the A8344G mtDNA mutation who presented with failure to thrive and sudden unexpected death at 11 months of age. [ncbi.nlm.nih.gov]
Infants present with dysrhythmia or cardiac arrest. The clinical course is usually fulminant, sometimes simulating sudden infant death syndrome. [malacards.org]
Treatment
The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]
3 Histiocytoid cells have an arrhythmogenic potential and conventional treatment of arrhythmia is not effective. [revespcardiol.org]
Antiarrhythmics are generally ineffective but one patient showed a good response to treatment with amiodarone. Prognosis The prognosis in general is poor and the disease is usually fatal. Last updated: 5/1/2008 [rarediseases.info.nih.gov]
Prognosis
Prognosis The prognosis in general is poor and the disease is usually fatal. Last updated: 5/1/2008 [rarediseases.info.nih.gov]
Prognosis The prognosis in general is poor and the disease is usually fatal. The documents contained in this web site are presented for information purposes only. [orpha.net]
[…] in children, cases of WPW in combination with histiocytoid cardiomyopathy are uniformly fatal, due to the poor prognosis of histiocytoid cardiomyopathy alone. [heart.bmj.com]
Etiology
We discuss the differential diagnosis, proposed theories of etiology, and the pathology of this rare entity. [ncbi.nlm.nih.gov]
Clinically, the patient presented with suspected dilated cardiomyopathy and cardiac arrhythmias, however the etiology was unclear. [escardio.org]
This lesion is not a tumour of Purkinje cell but a cardiomyopathy of unknown etiology and pathogenesis, the possible suggestions are: - Persistence of early developmental stage of myocytes with their change into oncocytes. [histopathology-india.net]
Epidemiology
Epidemiology The prevalence is unknown but less than 100 patients have been described in the literature so far. The majority of reported cases involved children under 2 years of age with a female:male ratio of 3:1. [rarediseases.info.nih.gov]
Summary Epidemiology The prevalence is unknown but less than 100 patients have been described in the literature so far. The majority of reported cases involved children under 2 years of age with a female:male ratio of 3:1. [orpha.net]
Pathophysiology
This article, the first in a two-part series, discusses the classification, pathophysiology and presentation of cardiomyopathies. [nursingtimes.net]
ISBN 978-0-07-180215-4. ^ Pathophysiology of heart disease : a collaborative project of medical students and faculty. Lilly, Leonard S., Harvard Medical School. (5th ed.). Baltimore, MD: Wolters Kluwer/Lippincott Williams & Wilkins. 2011. [en.wikipedia.org]
[…] disease expression Sporadic HCM can reflect an inaccurate family history, incomplete penetrance or a de novo mutation which may be heritable Patients who are genotype positive may be phenotypically negative without overt hypertrophy (subclinical HCM) Pathophysiology [pathologyoutlines.com]
Cardiomyopathies were then classified according to their own peculiar pathophysiological features, such as: (a) dilated, in the setting of dilatation and poor contractility of the ventricles; (b) hypertrophic, in the presence of unexplained hypertrophy [academic.oup.com]
Prevention
How can Histiocytoid Cardiomyopathy be Prevented? Presently, there are no specific methods or guidelines to prevent Histiocytoid Cardiomyopathy. [dovemed.com]
Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention [journals.sagepub.com]
[…] identify high risk patients due to differences in penetrance and expressivity Treatment Surgical myectomy or alcohol septal ablation are performed in cases with outflow obstruction to alleviate symptoms Implantable cardioverter defibrillators (ICDs) can prevent [pathologyoutlines.com]
Am J Surg Pathol 11:503–515 PubMed CrossRef Google Scholar Kijima Y, Kamiya T, Susuki A, Hirose O, Manabe H (1982) A trial procedure to prevent aneurysm formation of the coronary arteries by steroid pulse therapy in Kawasaki disease. [link.springer.com]