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Outcomes of Childhood Pulmonary Arterial Hypertension in BMPR2 and ALK1 Mutation Carriers

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作者：

机构： [1]Tokyo Womens Med Univ, Dept Pediat Cardiol, Tokyo, Japan; [2]Tokyo Womens Med Univ, Int Res & Educ Inst Integrated Med Sci, Tokyo, Japan; [3]Tokyo Womens Med Univ, Int Ctr Mol Cellular & Immunol Res, Tokyo, Japan; [4]Natl Def Med Coll, Dept Pediat, Tokorozawa, Saitama 359, Japan; [5]Natl Def Med Coll, Dept Prevent Med & Publ Hlth, Tokorozawa, Saitama 359, Japan; [6]Toho Univ, Dept Pediat, Omori Med Ctr, Omori Hosp, Tokyo, Japan; [7]Himeji Dokkyo Univ, Dept Med Engn, Fac Hlth Care Sci, Himeji, Hyogo, Japan; [8]Hokkaido Med Ctr Child Hlth & Rehabil, Dept Pediat, Sapporo, Hokkaido, Japan; [9]Iwate Prefectural Kitakami Hosp, Dept Pediat, Kitakami, Iwate, Japan; [10]Univ Tsukuba, Dept Child Hlth, Grad Sch Comprehens Human Sci, Tsukuba, Ibaraki, Japan; [11]Gunma Childrens Med Ctr, Dept Cardiol, Shibukawa, Japan; [12]Keio Univ, Sch Med, Dept Pediat, Tokyo, Japan; [13]Sakakibara Mem Hosp, Dept Pediat & Cardiovasc Surg, Tokyo, Japan; [14]Tokyo Med & Dent Univ, Grad Sch Med, Tokyo, Japan; [15]Shizuoka Childrens Hosp, Dept Pediat Cardiol, Shizuoka, Japan; [16]Nagano Childrens Hosp, Dept Cardiovasc Surg & Pediat Cardiol, Nagano, Japan; [17]Toyama Univ, Dept Pediat, Grad Sch Med, Toyama 930, Japan; [18]Kyoto Second Red Cross Hosp, Dept Pediat, Kyoto, Japan; [19]Okayama Univ, Div Pediat Cardiol, Dept Pediat, Okayama 7008530, Japan; [20]Org Nagasaki Med Ctr, Div Pediat, Nagasaki, Japan; [21]Oita Univ, Dept Pediat & Child Neurol, Fac Med, Oita 87011, Japan; [22]Kagoshima Univ, Div Pediat, Grad Sch Med & Dent Sci, Kagoshima 890, Japan; [23]Capital Med Univ, Dept Pediat Cardiol, Anzhen Hosp, Beijing, Peoples R China; [24]Toho Univ, Sch Med, Fac Med, Tokyo, Japan

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摘要：

Mutations in the bone morphogenetic protein receptor type 2 (BMPR2) gene and the activin receptor like kinase 1 (ALK1) gene have been reported in heritable pulmonary arterial hypertension (HPAH) and idiopathic pulmonary arterial hypertension (IPAH). However, the relation between clinical characteristics and each gene mutation in IPAH and HPAH is still unclear, especially in childhood. The aim of this study was to determine, in a retrospective study, the influence and clinical outcomes of gene mutations in childhood IPAH and HPAH. Fifty-four patients with IPAH or HPAH whose onset of disease was at <16 years of age were included. Functional characteristics, hemodynamic parameters, and clinical outcomes were compared in BMPR2 and ALK1 mutation carriers and noncarriers. Overall 5-year survival for all patients was 76%. Eighteen BMPR2 mutation carriers and 7 ALK1 mutation carriers were detected in the 54 patients with childhood IPAH or HPAH. Five-year survival was lower in BMPR2 mutation carriers than mutation noncarriers (55% vs 90%, hazard ratio 12.54, p = 0.0003). ALK1 mutation carriers also had a tendency to have worse outcome than mutation noncarriers (5-year survival rate 64%, hazard ratio 5.14, p = 0.1205). In conclusion, patients with childhood IPAH or HPAH with BMPR2 mutation have the poorest clinical outcomes. ALK1 mutation carriers tended to have worse outcomes than mutation noncarriers. It is important to consider aggressive treatment for BMPR2 or ALK1 mutation carriers. (C) 2012 Elsevier Inc. All rights reserved. (Am J Cardiol 2012;110:586-593)

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中科院(CAS)分区：

出版当年[2011]版：

大类 | 2 区

医学

小类 | 3 区心脏和心血管系统

最新[2023]版：

大类 | 3 区医学

小类 | 3 区心脏和心血管系统

JCR分区：

出版当年[2010]版：

Q1 CARDIAC & CARDIOVASCULAR SYSTEMS

最新[2023]版：

Q2 CARDIAC & CARDIOVASCULAR SYSTEMS

影响因子： 2.3 最新[2023版] 2.3 最新五年平均 3.681 出版当年[2010版] 3.767 出版当年五年平均 3.575 出版前一年[2009版] 3.368 出版后一年[2011版]

第一作者：

第一作者机构： [1]Tokyo Womens Med Univ, Dept Pediat Cardiol, Tokyo, Japan; [4]Natl Def Med Coll, Dept Pediat, Tokorozawa, Saitama 359, Japan;

通讯作者：

通讯机构： [1]Tokyo Womens Med Univ, Dept Pediat Cardiol, Tokyo, Japan;

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