Eisenmenger syndrome: Symptoms, Pathogenesis & Managements

Let's look at the Eisenmenger SD in chronological order. In the early phase, pulmonary arterial pressure rises with a noticeable increase in pulmonary blood flow. If this condition persists, it develops into a pulmonary vascular disease with pathological changes. This results in pulmonary hypertension. Pathological changes occur in small pulmonary arterioles and muscular arteries, and are divided into Grade I-VI according to histologic findings. In the early stage, medial hypertrophy occurs in Grade I, hyperplasia of intima in II, and obliteration in III. In IV, arterial dilation, V and VI eventually result in plexiform lesion, angiomatoid formation and fibrinoid necrosis. However, in the clinical setting, pulmonary artery resistance, pulmonary artery resistance, and systemic vascular resistance are observed rather than clinical course according to these histological changes. The retrospective study showed that the symptoms, syncope, poor functional class, low oxygen saturation (<85%), serum creatinine, serum uric acid concentration and Down syndrome were strong predictors. The above functional class is defined by NYHA (New York Heart Association), I asymptomatic, II as Symptomatic with moderate exertion, III as Symptomatic with minimal exertion, IV as Symptomatic at rest.

The extraocardial complications of Eisenmenger SD are caused by oxygen deficiency. The most careful consideration is polycythemia. Chronic hypoxemia increases erythropoietin production leading to secondary eryhrocytosis. Compensation may reduce the platelet count (low-grade DIC or thrombocytopenia). In this situation, if it is dehydrated, the viscosity increases and the probability of stroke is increased. Also, sudden changes in altitude or body temperature should be avoided. Phelebotomy is performed in patients with severe polycythemia (hematocrit> 65%), especially with symptoms. Gum disease, Gout, etc. can also be caused by polycythemia.