A 30-YEAR-OLD WOMAN WITH KNOWN CONGENITAL HEART DISEASE PRE- sented to our clinic with a 1-year history of fatigue, hemoptysis, and pro-gressive exertional dyspnea. On examination, the toes had mild cyanosis and marked clubbing, but the fingers had subtle clubbing only. She was noted to have a left parasternal heave, a palpable second heart sound, and a systolic ejection murmur audible in the pulmonic area. Contrast echocardiography showed right atrial and right ventricular dilatation, an elevated mean pulmonary-artery pressure of 68 mm Hg, and bidirectional flow through a patent ductus arteriosus. In Eisen-menger's syndrome, a long-standing systemic-to-pulmonary circulatory shunt results in pulmonary hypertension and eventual bidirectional or pulmonary-to-systemic shunting of blood. When Eisenmenger's syndrome occurs in concert with a patent ductus arteriosus, deoxygenated blood from the right ventricle is delivered to the aorta distal to the left subclavian artery. The upper extremities are thus spared the effects of the shunt, whereas the lower extremities are not, resulting in differential clubbing and cyanosis.
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机译：一名30岁已知先天性心脏病的女性被送往我们的诊所，具有1年的疲劳，咯血和进行性劳累性呼吸困难病史。检查时，脚趾有轻度发osis和明显的杵状指，但手指仅有细微的杵状指。注意到她在肺部区域有左胸骨旁隆起，明显的第二心音和收缩期喷射性杂音。超声心动图造影显示右房和右心室扩张，平均肺动脉压升高至68 mm Hg，双向流经动脉导管未闭。在艾森曼格氏综合症中，长期存在的系统-肺循环分流会导致肺动脉高压并最终导致血液双向或肺-系统分流。当艾森曼格氏综合征与动脉导管未闭共同发生时，来自右心室的脱氧血被输送到左锁骨下动脉远端的主动脉。因此，上肢避免了分流的影响，而下肢则没有，导致了不同的杵状指和发osis。
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