1/5. 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.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/5. An autopsied case of Eisenmenger syndrome complicated by recurrent thromboembolic phenomena in postpartal period.dyspnea, back pain, edema, and cyanosis developed suddenly in a 23-year-old woman during the last trimester of her first pregnancy. Although she had been noticed to have the enlarged heart and exertional shortness of breath to a slight degree, she had been apparently in good condition without any significant heart murmurs. Clinically, recurrent episodes of disseminated intravascular coagulation, including pulmonary thrombosis, were thought to be superimposed to Eisenmenger syndrome associated with toxemia of pregnancy. Anticoagulant and fibrinolytic treatments were tried, but their effectiveness was limited by hemorrhagic diathesis. She died of respiratory and circulatory failure after delivery of a moribund baby. autopsy revealed eisenmenger complex (a defect in the membranous portion of the interventricular septum and pulmonary vascular disease) and many fresh hemmorrhages in both lungs with a lot of new and organized thrombi. Fresh thrombi were also seen in the heart, the pancreas and the kidneys. The high peripartal mortality in Eisenmenger syndrome could be attributed to pulmonary thrombosis, which may be related to DIC, as well as to peripartal changes in circulatory function.- - - - - - - - - - ranking = 0.33333333333333keywords = vascular disease (Clic here for more details about this article) |
3/5. Peripartum cardiomyopathy with recovery in a patient with coincidental Eisenmenger ventricular septal defect.Severe pulmonary vascular disease was found during pregnancy in a 31 year old woman with a large ventricular septal defect. Three months after delivery peripartum cardiomyopathy with severe biventricular failure developed. This was a chance association. Recovery of ventricular function was slow but was complete two years later. There was no advance in the pulmonary vascular disease.- - - - - - - - - - ranking = 0.66666666666667keywords = vascular disease (Clic here for more details about this article) |
4/5. Single-lung transplantation with atrial septal defect repair for Eisenmenger's syndrome.heart-lung transplantation has been used successfully for patients with pulmonary vascular disease but its application has been very limited due to the scarcity of donors. We report a patient with Eisenmenger's syndrome who underwent right single-lung transplantation with closure of atrial septal defect; postoperative convalescence was uneventful. Serial magnetic resonance imaging examinations demonstrate improved right heart function.- - - - - - - - - - ranking = 0.33333333333333keywords = vascular disease (Clic here for more details about this article) |
5/5. heart-lung transplantation: successful therapy for patients with pulmonary vascular disease.We report our initial experience with three patients who received heart-lung transplants. The primary immunosuppressive agent used was cyclosporin A, although conventional drugs were also administered. In the first patient, a 45-year-old woman with primary pulmonary hypertension, acute rejection of the transplant was diagnosed 10 and 25 days after surgery but was treated successfully; this patient still had normal exercise tolerance 10 months late. The second patient, a 30-year-old man, underwent transplantation for Eisenmenger's syndrome due to atrial and ventricular septal defects. His graft was not rejected, and his condition was markedly improved eight months after surgery. The third patient, a 29-year-old woman with transposition of the great vessels and associated defects, died four days postoperatively of renal, hepatic, and pulmonary complications. We attribute our success to experience with heart-lung transplantation in primates, to the use of cyclosporin A, and to the anatomic and physiologic advantages of combined heart-lung replacement. We hope that such transplants may ultimately provide an improved outlook for selected terminally ill patients with pulmonary vascular disease and certain other intractable cardiopulmonary disorders.- - - - - - - - - - ranking = 1.6666666666667keywords = vascular disease (Clic here for more details about this article) |