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Histiocytoid Cardiomyopathy

Infantile Cardiomyopathy with Histiocytoid Change


Presentation

  • Here, we present a 19-month-old male infant who died of heart failure, and an autopsy was performed and confirmed the diagnosis of HC.[ncbi.nlm.nih.gov]
Sudden Infant Death Syndrome
  • In such instances, sudden infant death syndrome (SIDS) is often considered. Grossly, the affected heart usually shows small, multifocal, subendocardial or myocardial yellow-tan nodular lesions or poorly defined plaques.[ncbi.nlm.nih.gov]
  • The clinical course is usually fulminant, sometimes simulating sudden infant death syndrome.[malacards.org]
  • Infants present with dysrhythmia or cardiac arrest, and the clinical course is usually fulminant, sometimes simulating sudden infant death syndrome ({1:Andreu et al., 2000}). Source: Online Mendelian Inheritance in Man[diseaseinfosearch.org]
Pathologist
  • The Color Atlas of Pediatric Pathology is a practical working resource for every pathologist who sees pediatric cases as well as the pathology trainee.[books.google.com]
  • A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis.[dovemed.com]
  • (C) 1977 Royal College of Pathologists of Australasia[journals.lww.com]
  • The close association of the lesion with cardiac arrhythmias in very young infants is of special interest to pathologists. Histiocytoid cardiomyopathy is a cause of sudden death in infancy.[histopathology-india.net]
Crying
  • She could not suck well and had high serum lactate levels.Echocardiography performed just after birth revealed normal left ventricular contractility at rest, but showed impaired systolic and diastolic function at crying.[jstage.jst.go.jp]
Death in Infancy
  • Histiocytoid cardiomyopathy is a cause of sudden death in infancy. Surgical excision of nodules of histiocytoid cells can result in clinical remission.[histopathology-india.net]
Corneal Opacity
  • There was a complex malformation of the brain with hydrocephalus and bilateral corneal opacities and microphthalmos. The affected myofibres had a swollen vacuolated or granular cytoplasm and rounded nuclei giving a histiocytoid appearance.[ncbi.nlm.nih.gov]
  • opacities microphtalmos microphthalmia with linear skin defects syndrome (MLS) ( 12900578 ) cataracts cleft palate hydrocephalus agenesis of corpus callosum renal cysts Etiology missense mutation in the mitochondrial cytochrome b gene ( 10960495 ) defect[humpath.com]
Excitement
  • An unusual multifocal degeneration of the myofibres of all chambers and the conducting system of the heart was found in a 4-month-old female in whom ventricular pre-excitation (Wolff-Parkinson-White syndrome) had been demonstrated.[ncbi.nlm.nih.gov]
  • RCPA: 1977 doi: 10.3109/00313027709085249 Original Article: PDF Only Abstract Summary: An unusual multifocal degeneration of the myofibres of all chambers and the conducting system of the heart was found in a 4-month-old female in whom ventricular pre-excitation[journals.lww.com]

Treatment

  • . 3 Histiocytoid cells have an arrhythmogenic potential and conventional treatment of arrhythmia is not effective.[revespcardiol.org]
  • If the arrhythmia persists for a long period of time, then surgical treatment measures may be considered Surgical removal of the nodules Occasionally, in children with extensive heart nodules, heart transplantation may be a treatment option Observation[dovemed.com]
  • This era was followed by improved environmental factors which operate to produce methods of medical treatment, introduction congenital cardiac malformations.[books.google.com]

Prognosis

  • Prognosis The prognosis in general is poor and the disease is usually fatal. Last updated: 5/22/2008[rarediseases.info.nih.gov]
  • […] 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]
  • Association Scientific 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[journals.sagepub.com]
Sex distribution
Age distribution

Pathophysiology

  • This article, the first in a two-part series, discusses the classification, pathophysiology and presentation of cardiomyopathies.[nursingtimes.net]
  • Additionally, arrhythmias and chest pain may be present. [10] The pathophysiology of cardiomyopathies is better understood at the cellular level with advances in molecular techniques.[en.wikipedia.org]
  • 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]
  • 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]
  • […] 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[nursingtimes.net]

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