Fatal outcome in Eisenmenger syndrome.

AIM: To assess correlations between fatal outcome and histologic findings of pulmonary vascular disease in different situations of Eisenmenger syndrome, either during the natural course or early-late after surgery. MATERIAL AND METHODS: The clinical follow-up and fatal outcome of 20 patients affected by Eisenmenger syndrome were investigated. In addition to the pathologic report and gross reexamination of the heart, the lung tissue was studied by histology. Patients were divided into three groups: 6 non-operated patients who died during the natural course (Group 1), 11 patients who underwent correction of the congenital defect and died in the perioperative period (Group 2), and 3 patients who died late after surgery (Group 3). RESULTS: In Group 1, five patients (83%) died of cardiac arrest a few days after the onset of hypoxic attacks; in four patients histology showed Grade IV pulmonary vascular disease with diffuse fibrinoid necrosis in the distal pulmonary arterial vasculature. In Group 2, nine patients (82%) died on the first or second postoperative day after a refractory pulmonary hypertensive crisis, with histologic evidence in three patients of fibrinoid necrosis of the distal pulmonary small arteries and arterioles. In Group 3, two patients (67%) died suddenly, 6 and 18 years after cardiac surgery, following onset of dyspnea and cardiogenic shock; autopsy showed aneurysmal dilatation of the pulmonary artery with massive thrombosis in the setting of Grades III-IV pulmonary vascular disease without fibrinoid necrosis. CONCLUSION: Fatal outcome in Eisenmenger syndrome, either in the natural course or after refractory hypertensive attacks post surgery, is frequently associated with fibrinoid necrosis of the small pulmonary arteries and arterioles.

Life expectancy and quality of life in adult patients with congenital heart disease.

The survival and quality of life of patients with congenital heart disease have significantly improved in the last 20 years. This is due to more effective medical and surgical care. The new community of grown-up congenital heart patients consists of a few natural survivors with trivial congenital lesions or very rare complex cardiac abnormalities which are naturally compensated, and of more than 75% of patients who had been submitted to cardiac surgery during infancy or childhood. Clinical follow-up is however mandatory for many of them with scheduled times and types of exams to control the effects of sequelae and late complications, and to prevent deterioration and premature death because cardiac surgery may not have resulted in normality. Moreover, these patients have many needs and even more, many questions. Not giving a correct answer to each specific question reduces the entity of surgical success.