TY - JOUR
T1 - Outcomes of hypertrophic cardiomyopathy in Japanese children
T2 - a retrospective cohort study
AU - Mori, Hiroki
AU - Yoshikawa, Tadahiro
AU - Kimura, Hitomi
AU - Ono, Hiroshi
AU - Kato, Hitoshi
AU - Ono, Yasuo
AU - Nii, Masaki
AU - Shindo, Takahiro
AU - Inuzuka, Ryo
AU - Horigome, Hitoshi
AU - Miura, Masaru
AU - Hirono, Keiichi
AU - Kobayashi, Tomio
AU - Kogaki, Shigetoyo
AU - Furutani, Yoshiyuki
AU - Nakanishi, Toshio
N1 - Publisher Copyright:
© 2021, Springer Japan KK, part of Springer Nature.
PY - 2022/6
Y1 - 2022/6
N2 - There has been no multicenter study on the prognosis of pediatric hypertrophic cardiomyopathy (HCM) in Japan. Therefore, we conducted a retrospective multicenter observational study on the long-term survival rate in patients diagnosed with HCM under the age of 18 between 1990 and 2014. Twenty institutions participated. A total of 180 patients were identified. The median age at diagnosis was 5.8 years old and median duration of observation was 8.3 years. Although six patients (3%) deteriorated into the dilated phase of HCM, no patient received heart transplantation. Freedom from death at 1, 5, 10, and 20 years were 97%, 92%, 84%, and 80%, respectively. There were 26 deaths. Among them, 11 patients died suddenly, presumably due to arrhythmia, and 15 patients died of heart failure. The presence of heart failure symptoms and a greater cardiothoracic ratio were significant risk factors for heart failure-related death. There were no significant risk factors identified for arrhythmia-related death. In conclusion, the prognosis of pediatric HCM in Japan is good and similar to those reported in population-based studies in the United States and Australia. Significant risk factors for heart failure-related death were identified in pediatric patients with HCM in Japan.
AB - There has been no multicenter study on the prognosis of pediatric hypertrophic cardiomyopathy (HCM) in Japan. Therefore, we conducted a retrospective multicenter observational study on the long-term survival rate in patients diagnosed with HCM under the age of 18 between 1990 and 2014. Twenty institutions participated. A total of 180 patients were identified. The median age at diagnosis was 5.8 years old and median duration of observation was 8.3 years. Although six patients (3%) deteriorated into the dilated phase of HCM, no patient received heart transplantation. Freedom from death at 1, 5, 10, and 20 years were 97%, 92%, 84%, and 80%, respectively. There were 26 deaths. Among them, 11 patients died suddenly, presumably due to arrhythmia, and 15 patients died of heart failure. The presence of heart failure symptoms and a greater cardiothoracic ratio were significant risk factors for heart failure-related death. There were no significant risk factors identified for arrhythmia-related death. In conclusion, the prognosis of pediatric HCM in Japan is good and similar to those reported in population-based studies in the United States and Australia. Significant risk factors for heart failure-related death were identified in pediatric patients with HCM in Japan.
KW - Children
KW - Hypertrophic cardiomyopathy
KW - Mortality
KW - Risk factors
UR - http://www.scopus.com/inward/record.url?scp=85119513789&partnerID=8YFLogxK
U2 - 10.1007/s00380-021-01989-7
DO - 10.1007/s00380-021-01989-7
M3 - 学術論文
C2 - 34799788
AN - SCOPUS:85119513789
SN - 0910-8327
VL - 37
SP - 1075
EP - 1084
JO - Heart and Vessels
JF - Heart and Vessels
IS - 6
ER -