Model of chronic adaptation: right ventricular function in Eisenmenger syndrome

被引:34
作者
Diller, Gerhard-Paul [1 ,2 ,3 ]
Dimopoulos, Konstantinos [1 ,2 ,3 ]
Kafka, Henryk [4 ]
Ho, Siew Yen [5 ,6 ]
Gatzoulis, Michael A. [1 ,2 ,3 ]
机构
[1] Royal Brompton Hosp, Adult Congenital Heart Ctr, London SW3 6NP, England
[2] Royal Brompton Hosp, Ctr Pulm Hypertens, London SW3 6NP, England
[3] Univ London Imperial Coll Sci Technol & Med, Natl Heart & Lung Inst, London, England
[4] Queens Univ, Cardiovasc Lab, Kingston Gen Hosp, Div Cardiol, Kingston, ON, Canada
[5] Univ London Imperial Coll Sci Technol & Med, Natl Heart & Lung Inst, London, England
[6] Royal Brompton Hosp, London, England
关键词
pulmonary arterial hypertension; Eisenmenger syndrome; right ventricular function;
D O I
10.1093/eurheartj/sum019
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Pulmonary arterial hypertension (PAH) is commonly associated with adult congenital heart disease. Eisenmenger syndrome-severe pulmonary hypertension with shunt reversal and cyanosis-represents the extreme manifestation of PAH in patients with congenital heart disease and has become the epitome of PAH in this setting. Despite representing a multi-system disorder and being associated with numerous complications, survival prospects for patients with Eisenmenger syndrome are superior compared with patients with idiopathic pulmonary hypertension. We review aetiology, clinical presentation, and prognosis of Eisenmenger syndrome and discuss potential explanations for the remarkable resilience of the right ventricle in this setting.
引用
收藏
页码:H54 / H60
页数:7
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