2.Cardiomyopathies with Mixed and Inapparent Morphological Features in Cardiac Troponin I3 Mutation.
Dae Won SOHN ; Hyung Kwan KIM ; Yong Jin KIM ; Seil OH ; Moon Woo SEONG ; Sung Sup PARK
Korean Circulation Journal 2017;47(3):413-417
The fact that different types of cardiomyopathies can be manifested by the same sarcomere protein gene mutation in a single family is well known. However, mixed features of different types of cardiomyopathies in a single patient have not been well appreciated. We identified a novel mutation in cardiac troponin I3 (Arg186Gly) in the present case, and two of the family members showed mixed morphologic features of hypertrophic cardiomyopathy and left ventricular non-compaction. Moreover, both the features of cardiomyopathies were not apparent for each type of cardiomyopathy. In the patient's family, four other members had unexpected deaths before the age of 30.
Cardiomyopathies*
;
Cardiomyopathy, Hypertrophic
;
Cardiomyopathy, Restrictive
;
Humans
;
Sarcomeres
;
Troponin*
4.Diagnosis and Symptomatic Treatment of Early Reactive Cardiac Amyloidosis in Systemic Sclerosis.
Rae Seok LEE ; Hyun Jin SUNG ; Jung Im JUNG ; Hea Ok JUNG ; Seung Min JUNG ; Jennifer Jooha LEE ; Seung Ki KWOK ; Ji Hyeon JU ; Sung Hwan PARK
Journal of Rheumatic Diseases 2015;22(2):132-136
Systemic sclerosis as a connective tissue disease could affect all internal organs of the body and could also manifest as a cutaneous lesion. Cardiac involvement leading to cardiac manifestations in systemic sclerosis patients is not rare. However, cardiac amyloidosis combined with systemic sclerosis is extremely rare. Although there were no definite treatment options in this case, symptomatic treatment is the cornerstone of the management plan. In this case report, we described a correct diagnosis and symptomatic medical care of early reactive cardiac amyloidosis with systemic sclerosis and summarize the current state of the relevant literature.
Amyloidosis*
;
Cardiomyopathy, Restrictive
;
Connective Tissue Diseases
;
Diagnosis*
;
Humans
;
Scleroderma, Systemic*
5.A case of restrictive cardiomyopathy.
Fu-yu PEI ; Chun-fu LI ; Yu-ming ZHANG
Chinese Journal of Pediatrics 2003;41(8):639-639
6.Idiopathic cardiomyopathies in Korean Children: A nationwide study.
Eun Jung CHEUN ; I Suck GANG ; Eun Jung BAE ; Jong Goon LEE ; Hyang Suck YOON ; Yong Wook KIM ; Hee Joo PARK ; Jae Gon KOH ; Chung Il NOH ; Heung Jae LEE
Korean Circulation Journal 2000;30(5):635-645
BACKGROUND: Although idiopathic cardiomyopathies(i-CMP) are very important in all age groups, the epidemiology of i-CMP in children has not been well defined. A retrospective study in Korean children was performed in 1998 to obtain basic data on i-CMP. MATERIAL AND METHOD: The medical records of all patients aged birth to 15 years from the hospitals where pediatric cardiologists worked were reviewed to obtain information on i-CMP. Pediatric cardiologists from a total of 22 hospitals were participated in reviewing the medical records of their patients and filling up the protocol. The data were pooled to the study committee and reviewed. RESULTS: Of the 278 cases with i-CMP, there were dilated cardiomyopathy (d-CMP) in 182 (65.4%): hypertrophic cardiomyopathy (h-CMP) in 74 (26.6%): restrictive cardiomyopathy (r-CMP) and unclassified in 17 (6.1%) and 5 (1.9%) each. The average annual occurrence of new cases as a whole was 2.65 per 100,000 (95% CI: 1.5-3.7): d-CMP, 1.73/100,000/year (95% CI: 0.73-2.73): h-CMP, 0.71/100,000/year (95% CI: 0.35-1.07): r-CMP, 0.16/100,000/year (95% CI: 0.02-0.3). The median age at the time of diagnosis was 11 months in d-CMP: 3.0 years in h-CMP: 6.9 years in r-CMP. The survival rate in d-CMP was 76% at 1 year, 72.5% at 2 year, 70% at 5 year. There was no difference in survival rate according to age (in d-CMP, between children less than 2 years of age and over 2 years of age (74% vs. 79% at 1 year: 67% vs. 76% at 5 year, p=NS): in h-CMP, between children less than 1 year of age and over 1 year of age (84% vs. 96% at 1 year: 63% vs. 81% at 5 year, p=NS)). R-CMP showed the worst survival rate (72% at 1 year, 30.2% at 5 year). CONCLUSION: In spite of the inherent defects of retrospective analysis, this study provides the useful epidemiological data in children with i-CMP. However, more systemic approach is needed to define the nature of the i-CMP in children.
Cardiomyopathies*
;
Cardiomyopathy, Dilated
;
Cardiomyopathy, Hypertrophic
;
Cardiomyopathy, Restrictive
;
Child*
;
Diagnosis
;
Epidemiology
;
Humans
;
Medical Records
;
Parturition
;
Retrospective Studies
;
Survival Rate
7.Waffle Procedure in Chronic Constrictive Epicarditis Patient with Pericardial Effusion: A Case Report.
Hee Jae JUN ; Ki Bong KIM ; Kang Joo CHOI ; Yang Haeng LEE ; Youn Ho HWANG ; Kwang Hyun CHO
The Korean Journal of Thoracic and Cardiovascular Surgery 2002;35(4):307-310
The purpose of this presentation is to demonstrate a good results of a second operation, the Waffle procedure, in a patient who did not improve following pericardiectomy. Incomplete parietal pericardiectomy, myocardial fiber atrophy, and unexpected restrictive cardiomyopathy can be considered when the patient's symptom does not improve after pericardiectomy is carried out. Constrictive epicarditis is always ruled out. In our case, the patient having constrictive pericarditis combined with pericardial effusion received a pericardiectomy. However, hemodynamics and symptoms of the patient following the operation did not improve. However, we experienced a good result following a second operation, the Waffle procedure.
Atrophy
;
Cardiomyopathy, Restrictive
;
Hemodynamics
;
Humans
;
Pericardial Effusion*
;
Pericardiectomy
;
Pericarditis
;
Pericarditis, Constrictive
;
Rabeprazole
8.Early Stage Loeffler's Endocarditis Detected by Transthoracic Echocardiography.
Min Kyu KANG ; Won Jong PARK ; Sung Yun JUNG ; Su Mi KIM ; Tae Hun KWON ; Young Ha RYU ; Jang Won SON ; Dong Gu SHIN
Yeungnam University Journal of Medicine 2012;29(2):118-120
Loeffler's endocarditis involves progressive eosinophilic infiltration of the endocardium, which leads to apical thrombotic obliteration of the ventricle and endomyocardial fibrosis, that may finally represent a characteristic feature of restrictive cardiomyopathy. This paper presents a case of a 44-year-old male with symptoms of dyspnea and peripheral hypereosinophilia, who was diagnosed with early stage Loeffler's endocarditis via multicardiac imaging modalities.
Cardiomyopathy, Restrictive
;
Dyspnea
;
Echocardiography
;
Endocardium
;
Endomyocardial Fibrosis
;
Eosinophils
;
Humans
;
Hypereosinophilic Syndrome
;
Male
;
Thrombosis
9.Embolic Infarction Associated with Cardiac Amyloidosis.
Kyung Hee CHO ; Yong Mee CHO ; Jong S KIM
Journal of Clinical Neurology 2005;1(1):92-96
Embolic cerebral infarction due to cardiac amyloidosis is rare. We report two patients with amyloidosis who developed cerebral infarcts. These embolic infarcts were probably related to cardiac involvement of amyloidosis, which was based on results of myocardial biopsy (Patient 1), and kidney biopsy and characteristic echocardiographic features including granular sparkling, restrictive cardiomyopathy and the presence of mural thrombus (Patient 2). Diffuse amyloid infiltration of the heart may have lead to impairment of myocardial function and subsequent mural thrombosis. Cardiomyopathy due to cardiac amyloidosis should be recognized as one of the causes of cardioembolic infarction.
Amyloid
;
Amyloidosis*
;
Biopsy
;
Cardiomyopathies
;
Cardiomyopathy, Restrictive
;
Cerebral Infarction
;
Echocardiography
;
Embolism
;
Heart
;
Humans
;
Infarction*
;
Kidney
;
Stroke
;
Thrombosis