Clinical features of idiopathic restrictive cardiomyopathy

Hong, Jong Chul; Kim, Min Seok; Cho, Min Su; Choi, Hyo In; Kang, Duk Hyun; Lee, Sang Eun; Lee, Ga Yeon; Jeon, Eun Seok; Cho, Jae Yeong; Kim, Kye Hun; Yoo, Byung Su; Lee, Jong‐Young; Kim, Won Jang; Kim, Kyung Hee; Chung, Wook‐Jin; Cho, Myeong Chan; Kim, Jae Joong

doi:10.1097/md.0000000000007886

Cited by 10 publications

(5 citation statements)

References 16 publications

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“…All-cause mortality for the overall cohort was 56%. The poor prognosis was consistent with a previous multicenter Korean study, where five-year overall survival was 64% [5].…”

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confidence: 91%

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Restrictive cardiomyopathies: The need for a better characterization of a deadly disease

Masarone,

Falco

2023

Kardiol Pol

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“…All-cause mortality for the overall cohort was 56%. The poor prognosis was consistent with a previous multicenter Korean study, where five-year overall survival was 64% [5].…”

supporting

confidence: 91%

“…On the other hand, only a few management recommendations were suggested, highlighting the lack of data on RCM. Indeed, except for TTR-CA, a few studies focused on RCM in recent years [4][5][6][7].…”

mentioning

confidence: 99%

Restrictive cardiomyopathies: The need for a better characterization of a deadly disease

Masarone,

Falco

2023

Kardiol Pol

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“…Moreover, our study showed a five-year survival rate of 74.1% in LVNC patients, compared with the rate of 80–91% reported by Ce Wang et al and 52% reported by William Y Shi et al [ 14 , 20 ]. For RCM patients, the five-year survival rate of 51.1% was still lower than the rate of 64.6% in adult population, suggesting that patients who are diagnosed with RCM in childhood may have a worse prognosis than those diagnosed later in life [ 21 ].…”

Section: Discussionmentioning

confidence: 99%

The clinical profile, genetic basis and survival of childhood cardiomyopathy: a single-center retrospective study

Yuan,

Jia,

et al. 2024

Eur J Pediatr

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Cardiomyopathy (CM) is a heterogeneous group of myocardial diseases in children. This study aimed to identify demographic features, clinical presentation and prognosis of children with CM. Clinical characteristics and prognostic factors associated with mortality were evaluated by Cox proportional hazards regression analyses. Genetic testing was also conducted on a portion of patients. Among the 317 patients, 40.1%, 25.2%, 24.6% and 10.1% were diagnosed with dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), left ventricular noncompaction cardiomyopathy (LVNC) and restrictive cardiomyopathy (RCM), respectively. The most common symptom observed was dyspnea (84.2%). Except for HCM, the majority of patients were classified as NYHA/Ross class III or IV. The five-year survival rates were 75.5%, 67.3%, 74.1% and 51.1% in DCM, HCM, LVNC and RCM, respectively. The ten-year survival rates were 60.1%, 56.1%, 57.2% and 41.3% in DCM, HCM, LVNC and RCM, respectively. Survival was inversely related to NYHA/Ross class III or IV in patients with DCM, HCM and RCM. Out of 42 patients, 32 were reported to carry gene mutations.Conclusions: This study demonstrates that CM, especially RCM, is related to a high incidence of death. NYHA/Ross class III or IV is a predictor of mortality in the patients and gene mutations may be a common cause.Trial registration: MR-50-23-011798. What is Known:• Cardiomyopathy (CM) is a heterogeneous group of myocardial diseases and one of the leading causes of heart failure in children due to the lack of effective treatments.• There remains scarce data on Asian pediatric populations though emerging studies have assessed the clinical characteristics and outcomes of CM. What is New:• A retrospective study was conducted and the follow-up records were established to investigate the clinical characteristics, the profile of gene mutations and prognostic outcomes of children with CM in Western China.• CM, especially RCM, is related to a high incidence of death. NYHA/Ross class III or IV is a predictor of mortality in the patients and gene mutations may be a common cause.

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“…The following prognostic factors have been described in non-amyloid RCM (na-RCM): male sex, age >70 years, the New York Heart Association Functional Classification (NYHA class) ≥III, left atrial (LA) diameter >60 mm, and low cardiac output [8]. Recent studies underline the role of left ventricular end-diastolic diameter (LVEDD) and tricuspid regurgitation [9]. GDF-15 is considered a cardioprotective hormone due to its antioxidative, anti-inflammatory, and antiapoptotic properties [10].…”

Section: Introductionmentioning

confidence: 99%

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience

Szczygieł,

Michałek,

Truszkowska

et al. 2023

Kardiol Pol

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Background: Numerous prognostic factors have been proposed for cardiac amyloidosis (CA). The knowledge about other subtypes of restrictive cardiomyopathy (RCM) is scant.Aims: This study aimed to elucidate the etiology and prognostic factors of RCM as well as assess cardiac biomarkers: high-sensitive troponin T (hs-TnT), growth differentiation factor-15 (GDF-15), N-terminal pro-B-type natriuretic peptide (NT-proBNP), and soluble suppression of tumorigenicity 2, as mortality predictors in RCM. Methods:We enrolled 36 RCM patients in our tertiary cardiac department. All patients were screened for CA. Genetic testing was performed in 17 patients without CA.Results: Pathogenic or likely pathogenic gene variants were found in 86% of patients, including 5 novel variants. Twenty patients died, and 4 had a heart transplantation during the study. Median overall survival was 29 months (8-55). The univariate Cox models analysis indicated that systolic and diastolic blood pressure, GDF-15, hs-TnT, NT-proBNP, left ventricular stroke volume, the ratio of the transmitral early peak velocity (E) estimated by pulsed wave Doppler over the early mitral annulus velocity (e'), tricuspid annulus plane systolic excursion, early tricuspid valve annular systolic velocity, the presence of pulmonary hypertension, and pericardial effusion influenced survival (P <0.05). A worse prognosis was observed in patients with GDF-15 >1316 pg/ml, hs-TnT >42 ng/l, NT-proBNP >3383 pg/ml, and pericardial effusion >3.5 mm (Kaplan-Meier analysis, log-rank test, P <0.001).Conclusions: Genetic testing should be considered in every RCM patient where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF-15, hs-TNT, NT-proBNP, and pericardial effusion are associated with worse prognosis. Further studies are warranted.

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Clinical features of idiopathic restrictive cardiomyopathy

Cited by 10 publications

References 16 publications

Restrictive cardiomyopathies: The need for a better characterization of a deadly disease

Restrictive cardiomyopathies: The need for a better characterization of a deadly disease

The clinical profile, genetic basis and survival of childhood cardiomyopathy: a single-center retrospective study

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience

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