Long-Term Outcomes and Prognosticators of Pediatric Primary Dilated Cardiomyopathy in an Asian Cohort
Background: Dilated cardiomyopathy (DCM) is the most common childhood cardiomyopathy. The epidemiological profiles and prognosticators of clinical outcomes in Asian populations are not well elucidated.Methods: Data of 104 children aged <18 years with a diagnosis of primary DCM from January 19...
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Frontiers Media S.A.
2021-11-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2021.771283/full |
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| author | Po-Yuan Wang Po-Yuan Wang Wei-Chieh Tseng Chun-Min Fu Mei-Hwan Wu Jou-Kou Wang Yih-Sharng Chen Nai-Kuan Chou Shoei-Shen Wang Shuenn-Nan Chiu Ming-Tai Lin Chun-Wei Lu Chun-An Chen |
| author_facet | Po-Yuan Wang Po-Yuan Wang Wei-Chieh Tseng Chun-Min Fu Mei-Hwan Wu Jou-Kou Wang Yih-Sharng Chen Nai-Kuan Chou Shoei-Shen Wang Shuenn-Nan Chiu Ming-Tai Lin Chun-Wei Lu Chun-An Chen |
| author_sort | Po-Yuan Wang |
| collection | DOAJ |
| description | Background: Dilated cardiomyopathy (DCM) is the most common childhood cardiomyopathy. The epidemiological profiles and prognosticators of clinical outcomes in Asian populations are not well elucidated.Methods: Data of 104 children aged <18 years with a diagnosis of primary DCM from January 1990 to December 2019 in our institutional database were retrospectively investigated. Relevant demographic, echocardiographic, and clinical variables were recorded for analysis. A P <0.05 was considered statistically significant.Results: The median age at diagnosis was 1.4 years (interquartile range = 0.3–9.1 years), and 52.9% were males. During a median follow-up duration of 4.8 years, 48 patients (46.2%) were placed on the transplantation waitlist, and 52.1% of them eventually received heart transplants. An exceptionally high overall waitlist mortality rate was noted (27.1%), which was even higher (43.5%) if the diagnostic age was <3 years. The 1-, 5-, and 10-year transplant-free were 61.1, 48.0, and 42.8%. Age at diagnosis >3 years and severe mitral regurgitation at initial diagnosis were independent risk factors for death or transplantation (hazard ratios = 2.93 and 3.31, respectively; for both, P <0.001). In total, 11 patients (10.6%) experienced ventricular function recovery after a median follow-up of 2.5 (interquartile range = 1.65–5) years. Younger age at diagnosis was associated a higher probability of ventricular function recovery.Conclusions: Despite donor shortage for heart transplantation and subsequently high waitlist mortality, our data from an Asian cohort indicated that transplant-free long-term survival was comparable with that noted in reports from Western populations. Although younger patients had exceptionally higher waitlist mortality, lower diagnostic age was associated with better long-term survival and higher likelihood of ventricular function recovery. |
| first_indexed | 2024-04-11T20:51:18Z |
| format | Article |
| id | doaj.art-b2ec28c3b152463b82eeecc1c788ac9c |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-04-11T20:51:18Z |
| publishDate | 2021-11-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-b2ec28c3b152463b82eeecc1c788ac9c2022-12-22T04:03:50ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602021-11-01910.3389/fped.2021.771283771283Long-Term Outcomes and Prognosticators of Pediatric Primary Dilated Cardiomyopathy in an Asian CohortPo-Yuan Wang0Po-Yuan Wang1Wei-Chieh Tseng2Chun-Min Fu3Mei-Hwan Wu4Jou-Kou Wang5Yih-Sharng Chen6Nai-Kuan Chou7Shoei-Shen Wang8Shuenn-Nan Chiu9Ming-Tai Lin10Chun-Wei Lu11Chun-An Chen12Department of Pediatrics, National Taiwan University Children's Hospital, Taipei, TaiwanDepartment of Pediatrics, Taipei City Hospital Renai Branch, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Children's Hospital, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Hospital Hsin-Chu Branch, Hsin-Chu City, TaiwanDepartment of Pediatrics, National Taiwan University Children's Hospital, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Children's Hospital, Taipei, TaiwanDepartment of Cardiovascular Surgery, National Taiwan University Hospital, Taipei, TaiwanDepartment of Cardiovascular Surgery, National Taiwan University Hospital, Taipei, TaiwanDepartment of Cardiovascular Surgery, Fu Jen Catholic University Hospital, New Taipei City, TaiwanDepartment of Pediatrics, National Taiwan University Children's Hospital, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Children's Hospital, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Children's Hospital, Taipei, TaiwanDepartment of Pediatrics, National Taiwan University Children's Hospital, Taipei, TaiwanBackground: Dilated cardiomyopathy (DCM) is the most common childhood cardiomyopathy. The epidemiological profiles and prognosticators of clinical outcomes in Asian populations are not well elucidated.Methods: Data of 104 children aged <18 years with a diagnosis of primary DCM from January 1990 to December 2019 in our institutional database were retrospectively investigated. Relevant demographic, echocardiographic, and clinical variables were recorded for analysis. A P <0.05 was considered statistically significant.Results: The median age at diagnosis was 1.4 years (interquartile range = 0.3–9.1 years), and 52.9% were males. During a median follow-up duration of 4.8 years, 48 patients (46.2%) were placed on the transplantation waitlist, and 52.1% of them eventually received heart transplants. An exceptionally high overall waitlist mortality rate was noted (27.1%), which was even higher (43.5%) if the diagnostic age was <3 years. The 1-, 5-, and 10-year transplant-free were 61.1, 48.0, and 42.8%. Age at diagnosis >3 years and severe mitral regurgitation at initial diagnosis were independent risk factors for death or transplantation (hazard ratios = 2.93 and 3.31, respectively; for both, P <0.001). In total, 11 patients (10.6%) experienced ventricular function recovery after a median follow-up of 2.5 (interquartile range = 1.65–5) years. Younger age at diagnosis was associated a higher probability of ventricular function recovery.Conclusions: Despite donor shortage for heart transplantation and subsequently high waitlist mortality, our data from an Asian cohort indicated that transplant-free long-term survival was comparable with that noted in reports from Western populations. Although younger patients had exceptionally higher waitlist mortality, lower diagnostic age was associated with better long-term survival and higher likelihood of ventricular function recovery.https://www.frontiersin.org/articles/10.3389/fped.2021.771283/fulldilated cardiomyopathyepidemiology - analytic (risk factors)outcomepediatricAsian - Chinese |
| spellingShingle | Po-Yuan Wang Po-Yuan Wang Wei-Chieh Tseng Chun-Min Fu Mei-Hwan Wu Jou-Kou Wang Yih-Sharng Chen Nai-Kuan Chou Shoei-Shen Wang Shuenn-Nan Chiu Ming-Tai Lin Chun-Wei Lu Chun-An Chen Long-Term Outcomes and Prognosticators of Pediatric Primary Dilated Cardiomyopathy in an Asian Cohort Frontiers in Pediatrics dilated cardiomyopathy epidemiology - analytic (risk factors) outcome pediatric Asian - Chinese |
| title | Long-Term Outcomes and Prognosticators of Pediatric Primary Dilated Cardiomyopathy in an Asian Cohort |
| title_full | Long-Term Outcomes and Prognosticators of Pediatric Primary Dilated Cardiomyopathy in an Asian Cohort |
| title_fullStr | Long-Term Outcomes and Prognosticators of Pediatric Primary Dilated Cardiomyopathy in an Asian Cohort |
| title_full_unstemmed | Long-Term Outcomes and Prognosticators of Pediatric Primary Dilated Cardiomyopathy in an Asian Cohort |
| title_short | Long-Term Outcomes and Prognosticators of Pediatric Primary Dilated Cardiomyopathy in an Asian Cohort |
| title_sort | long term outcomes and prognosticators of pediatric primary dilated cardiomyopathy in an asian cohort |
| topic | dilated cardiomyopathy epidemiology - analytic (risk factors) outcome pediatric Asian - Chinese |
| url | https://www.frontiersin.org/articles/10.3389/fped.2021.771283/full |
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