Fatal outcome in Eisenmenger syndrome.
OBJECTIVE
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.


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.


CONCLUSIONS
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.
Department of Cardiology, University of Padua Medical School, Via Giustiniani 2, 35128 Padua, Italy. luciano.daliento@unipd.it
Luciano
L
Daliento
Luca
L
Rebellato
Annalisa
A
Angelini
Carla
C
Frescura
Elisa
E
Mazzotti
Maristella
M
Rotundo
Gaetano
G
Thiene
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D004541:Eisenmenger Complex / Q000473:pathology* / Q000601:surgery; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D000860:Hypoxia / Q000209:etiology; D007223:Infant; D008297:Male; D009336:Necrosis; D011183:Postoperative Complications / Q000209:etiology; D011651:Pulmonary Artery / Q000473:pathology