1/5. Palliative arterial repair for transposition, ventricular septal defect, and pulmonary vascular disease.Failure to repair transposition of the great arteries and ventricular septal defect in the young infant results in the early development of pulmonary vascular occlusive disease. Complete repair, preferably by an arterial switch procedure and ventricular septal defect closure, may then not be possible. We report a palliative arterial switch procedure in a 5 1/2-year-old patient with transposition, ventricular septal defect, and severe pulmonary vascular obstructive disease in whom progressive hypoxemia and exercise intolerance developed. An arterial repair without ventricular septal defect closure was performed. After the operation, the child's systemic arterial oxygen saturation and exercise tolerance have substantially improved. Although the progression of pulmonary vascular disease may not be altered, arterial repair can provide effective palliation in this subset of patients.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/5. pulmonary veno-occlusive disease. A report of four cases.Four cases of pulmonary veno-occlusive disease are described. Two patients, who were brothers, had respiratory tract infections. The third patient had chronic active hepatitis and coeliac disease suggesting an abnormality of the immune system; the fourth patient had no obvious cause but presented initially with systemic hypertension. Three of the cases had been diagnosed initially as primary pulmonary hypertension either on open lung biopsy or clinically. In all cases the pulmonary arteries were abnormal with medial hypertrophy, intimal fibrosis and, in some cases, thrombosis in elastic pulmonary arteries. These findings suggest that pulmonary veno-occlusive disease is not confined to veins and should be considered as a widespread pulmonary vascular disease. The range of aetiological factors indicate that it should not be considered as a single disease entity.- - - - - - - - - - ranking = 0.2keywords = vascular disease (Clic here for more details about this article) |
3/5. Neonatal pulmonary vascular disease in hypoplastic left heart syndrome.A term neonate with hypoplastic left heart syndrome underwent surgical palliation using the Norwood procedure on day 2 of life. The postoperative course was complicated by recurrent systemic desaturation leading to death. Postmortem histologic examination of lung tissue confirmed the clinical impression of fixed pulmonary vascular disease. Some neonates with a restrictive interatrial communication may have pulmonary vascular disease at birth, which will negatively influence survival irrespective of the type of palliation undertaken.- - - - - - - - - - ranking = 1.2keywords = vascular disease (Clic here for more details about this article) |
4/5. Pulmonary venoocclusive disease in a patient with systemic lupus erythematosus.A 26-year-old woman with systemic lupus erythematosus (SLE) developed dyspnea and hypoxemia on exertion. She died from rapidly progressive respiratory failure. autopsy revealed right ventricular hypertrophy and occlusion of the pulmonary veins compatible with pulmonary venoocclusive disease (PVOD). Although PVOD has been reported in patients with suspected collagen vascular disease, this appears to be the first reported case of PVOD occurring in a patient with SLE.- - - - - - - - - - ranking = 0.2keywords = vascular disease (Clic here for more details about this article) |
5/5. Pulmonary vascular disease in neonates with transposition of the great arteries and intact ventricular septum.BACKGROUND--Progressive pulmonary vascular disease in surgically unrepaired transposition of the great arteries with or without ventricular septal defect had been frequently described in the past. Occurrence of progressive pulmonary vascular disease has been reported even after atrial switch procedure done at three months of age. With the advent of neonatal surgical repair, this problem is virtually non-existent. There is a small subgroup of infants with transposition of the great arteries who show pulmonary vascular disease in the neonatal period that can adversely affect the surgical outcome. The clinico-pathological correlation in this group of patients was studied. OBSERVATIONS--Three patients, with transposition of the great arteries and intact ventricular septum, who showed histological evidence of pulmonary vascular disease in the neonatal period or early infancy are described. Two of these patients, continued to have poor systemic oxygenation despite adequate atrial communication. One patient had a close ductus arteriosus within the first two hours of birth while on prostaglandin E1 infusion. CONCLUSIONS--In the absence of left ventricular outflow tract obstruction, a poor response to atrial septostomy suggests pulmonary hypertension and pulmonary vascular disease. Antenatal constriction of the ductus arteriosus may contribute to such changes in pulmonary vasculature.- - - - - - - - - - ranking = 1.8keywords = vascular disease (Clic here for more details about this article) |