Clinical manifestations and long-term follow-up in pediatric patients living at altitude with isolated pulmonary artery of ductal origin.
Clinical manifestations and long-term follow-up in pediatric patients living at altitude with isolated pulmonary artery of ductal origin.
复制标题
居住在高海拔的儿科患者的临床表现和长期随访，具有导管起源的分离肺动脉。
DOI：
10.1007/s00246-012-0213-z
复制
发表时间：
2012-06
影响因子：
1.6
通讯作者：
Ivy, David Dunbar
中科院分区：
文献类型：
--
作者：
Takatsuki, Shinichi;Darst, Jeffrey R.;Das, Bibhuti B.;Fagan, Thomas E.;Wolfe, Robert;Ivy, David Dunbar
关键词：
本研究旨在探讨高原地区小儿导管源性肺动脉孤立的临床表现及远期预后。这是一项回顾性队列研究，在单一中心连续17例孤立的导管源性肺动脉。所有患者生活在中等海拔（中位数，范围；2050米，1700-3050米）。15名儿童（88%）在就诊时出现症状。2例患者（12%）在诊断时出现高原肺水肿，只有1例患者在随访期间出现咯血发作。14例患者（82%）表现出肺动脉高压（PAH）的证据。在14例PAH患者中，有11例患者进行了手术干预。接受手术康复治疗的11例患者中有5例（45%）PAH消退。1例患者在随访期间死亡，7例患者因残留多环芳烃接受口服血管扩张剂治疗；14例患者无症状。我们的研究表明，对中等海拔地区导管源性肺动脉孤立患者进行早期干预可能会使孤立肺动脉恢复并逆转PAH。在没有PAH的情况下，这种疾病的患者是否需要手术治疗尚不清楚。
The study aim was to define the clinical manifestations and long-term outcome of pediatric patients living at altitude with isolated pulmonary artery of ductal origin. This was a retrospective cohort study of 17 consecutive cases of isolated pulmonary artery of ductal origin at a single center. All patients lived at modest altitude (median, range; 2050 m, 1700–3050 m). Fifteen children (88%) were symptomatic at presentation. High altitude pulmonary edema was present in 2 patients (12%) at diagnosis and only 1 patient had episodes of hemoptysis during follow-up. Fourteen patients (82%) demonstrated evidence of pulmonary arterial hypertension (PAH). Among 14 patients with PAH, 11 patients had surgical interventions. PAH resolved in 5 of 11 patients (45%) undergoing surgical rehabilitation. One patient died during follow-up and 7 patients are receiving oral vasodilator therapies due to residual PAH; 14 patients remained asymptomatic. Our study showed that early intervention in patients with isolated pulmonary artery of ductal origin at modest altitude can potentially rehabilitate the isolated pulmonary artery and reverse the PAH. Whether surgery is indicated for patients with this disorder in the absence of PAH is unknown.
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DOI：
10.4103/0019-5278.64608
发表时间：
2010-01
影响因子：
0.9
作者：
Paralikar SJ;Paralikar JH
通讯作者：
Paralikar JH
影响因子：
2.3
作者：
Fagan, Thomas;Kay, Joseph;Neubauer, Anne
通讯作者：
Neubauer, Anne
影响因子：
4.6
作者：
Kosaka, Y;Kurosawa, H;Tsuji, Y
通讯作者：
Tsuji, Y
影响因子：
9.6
作者：
BOUROS, D;PARE, P;SIAFAKAS, N
通讯作者：
SIAFAKAS, N
影响因子：
5.5
作者：
BARER, GR;HOWARD, P;SHAW, JW
通讯作者：
SHAW, JW