Thiam M, Fall M D, Calvet P, Fall P D, Charpentier P
Service de cardiologie - Hôpital Principal de Dakar.
Dakar Med. 2006;51(3):178-80.
The authors related a right heart failure related to a primitive pulmonary arterial hypertension without past medical.
This diagnosis has been noticed on the cardiac catheterism as well as the cardiac echography (no shunt). Two years later systemic anomalies such as polyarthritis and salivary glands disease were noticed. The Biopsy revelated a primitive Gougerot-Sjorgen pseudolymphomas syndrom.
Three years under conventional treatment were a failure as the evolution of the disease resulted to death.
作者报告了一例与原发性肺动脉高压相关的右心衰竭，既往无病史。
该诊断通过心导管检查以及心脏超声心动图得以证实（无分流）。两年后出现了全身性异常，如多关节炎和唾液腺疾病。活检显示为原发性古热罗 - 施约格伦假性淋巴瘤综合征。
常规治疗三年失败，疾病进展导致死亡。
