1/50. Progressive pulmonary vascular disease after pulmonary artery banding and total correction in a case of ventricular septal defect and pulmonary hypertension.A 7-month-old infant with ventricular septal defect and pulmonary hypertension underwent pulmonary artery banding, which resulted in a decrease in the pulmonary arterial peak pressure from 102 to 54 mmHg. Lung biopsy findings showed at most an early grade 3 Heath-Edwards classification, and an index of pulmonary vascular disease of 1.4, both of which indicated operability for total correction. Small pulmonary arteries less than 100 microns in diameter, however, showed marked hydropic changes in the medial smooth muscle cells. Total correction was performed at the age of 2 years, but the pulmonary arterial pressure failed to decrease. A lung biopsy taken just after the closure of the ventricular septal defect contraindicated operability due to progressive pulmonary vascular disease at a grade 6 Heath-Edwards classification and an index of pulmonary vascular disease of 2.4. The patient died at 8 months after the operation, and an autopsy revealed still more advanced pulmonary vascular disease at a grade 6 Heath-Edwards classification and an index of pulmonary vascular disease of 2.8. The pathogenesis of arterial changes is discussed.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/50. Persistent pulmonary hypertension of the newborn associated with pulmonary atresia and intact interventricular septum.Neonates with pulmonary atresia and intact interventricular septum (PAIVS) do not have pulmonary vascular disease secondary to their heart abnormality. Persistent pulmonary hypertension of the newborn has not been described in association with this condition. The case is reported of a female neonate born with PAIVS, who preoperatively had no clinical evidence or any risk factors for persistent pulmonary hypertension of the newborn, but whose postoperative course was highly suggestive of persistent pulmonary hypertension; necropsy confirmed the features of pulmonary vascular disease.- - - - - - - - - - ranking = 0.22222222222222keywords = vascular disease (Clic here for more details about this article) |
3/50. Diagnosing and treating primary pulmonary hypertension.Primary pulmonary hypertension (PPH) is a pulmonary vascular disease characterized by an elevation in mean pulmonary artery pressure and pulmonary vascular resistance. Recently, PPH gained national attention because of its association with appetite suppressants. PPH may also be associated with pregnancy, hypothyroidism, autoimmune disorders, human immunodeficiency virus infection, and the use of drugs such as oral contraceptives and cocaine. patients with PPH may report dyspnea on exertion and fatigue. early diagnosis is crucial. New therapeutic regimens have dramatically reduced mortality rates and improved quality of life by halting the progression of pulmonary vascular remodeling and averting right-sided heart failure. These therapies include high-dose calcium channel antagonists, anticoagulants, and continuous intravenous prostacyclin. Lung or heart-lung transplantation remains a viable therapeutic option for patients who are treated late in the disease process, who are not responsive to medical management, or who remain symptomatic and continue to deteriorate.- - - - - - - - - - ranking = 0.11111111111111keywords = vascular disease (Clic here for more details about this article) |
4/50. Pulmonary hypertension associated with pulmonary occlusive vasculopathy after allogeneic bone marrow transplantation.BACKGROUND: Pulmonary vasculature abnormalities, including pulmonary veno-occlusive disease, have been demonstrated in marrow allograft recipients. However, it is often difficult to make a correct diagnosis of pulmonary lesions. methods: An open lung biopsy was performed on a patient who developed severe pulmonary hypertension after bone marrow transplantation for T-cell lymphoma. RESULTS: An open lung biopsy specimen demonstrated pulmonary arterial occlusion due to intimal fibrosis and veno-occlusion. The most striking alteration was partial to complete occlusion of the small arteries by fibrous proliferation of the intima. CONCLUSION: High-dose preparative chemotherapy and radiation before transplantation are thought to have contributed to the development of vasculopathy in this patient, because arterial occlusion by intimal fibrosis and atypical veno-occlusion are often associated with lung injury due to chemoradiation. An open lung biopsy is essential for diagnosing pulmonary vascular disease presenting signs compatible with posttransplantation pulmonary hypertension.- - - - - - - - - - ranking = 0.11111111111111keywords = vascular disease (Clic here for more details about this article) |
5/50. Palliation of systemic sclerosis-associated pulmonary hypertension by atrial septostomy.The onset of pulmonary hypertension in patients with systemic sclerosis carries a poor prognosis. Atrial septostomy has been used successfully to palliate endstage primary pulmonary hypertension but has not been attempted in other forms of pulmonary vascular disease. We report substantial clinical improvement following atrial septostomy in a patient with systemic sclerosis complicated by severe, isolated pulmonary hypertension. After the procedure, exercise capacity was improved and exertional syncope abolished. We suggest that this procedure should be considered for other patients with this diagnosis.- - - - - - - - - - ranking = 0.11111111111111keywords = vascular disease (Clic here for more details about this article) |
6/50. Case report. Two cases of pulmonary hypertension with Raynaud's phenomenon. Primary pulmonary hypertension and systemic lupus erythematosus.Two cases of hypertensive pulmonary vascular disease with Raynaud's phenomenon are reported. One is a 32-year-old housewife of primary pulmonary hypertension with some immunological disorders and the other is a 28-year-old female of systemic lupus erythematosus. It may be considered that some immunological process could play a role in the etiology of some cases of primary pulmonary hypertension and the relationship of pathogenesis between these 2 cases was discussed.- - - - - - - - - - ranking = 0.11111111111111keywords = vascular disease (Clic here for more details about this article) |
7/50. Familial lupus anticoagulant.The antiphospholipid antibody syndrome (APS) is defined by widespread arterial and venous thromboses associated with elevated plasma levels of antiphospholipid antibodies (APLA). The primary antiphospholipid antibody syndrome (PAPS) appear to be a fairly homogeneous disease, and HLA, family and other studies provide new insights into this cause of thrombosis and vascular disease. We describe two patients with PAPS (lupus anticoagulant positive), whose family members were analyzed for clinical and laboratory abnormalities associated with APS. Familial screening seems to be important, in order to prevent the thrombotic events. Low dose aspirin is the first line treatment in asymptomatic subjects with APLA, previous or present thrombosis requiring long-term, possibly life-long anticoagulation.- - - - - - - - - - ranking = 0.11111111111111keywords = vascular disease (Clic here for more details about this article) |
8/50. Severe pulmonary hypertensive vascular disease in two newborns with aneurysmal vein of galen.Arteriovenous malformation of the vein of Galen (AVG) is a rare entity in the newborn with a high morbidity and mortality. We present two cases of fatal AVG with persistent pulmonary hypertension of the newborn and significant pulmonary hypertension documented by autopsy histopathology. The pathophysiology is reviewed and a proposed mechanism of the association between AVG and pulmonary hypertension is discussed.- - - - - - - - - - ranking = 0.44444444444444keywords = vascular disease (Clic here for more details about this article) |
9/50. Successful closure of the arterial duct in the setting of rubella syndrome.A 9-year-old boy, with significant left-to-right shunting across a large duct in the context of rubella syndrome, was tested during catheterization to establish the feasability of occluding the duct with a device. The testing, including temporary closure of the duct and monitoring of pulmonary vascular reactivity to vasodilative substances, lead to the decision to implant an Amplatzer occluder. Sixteen months later, there was no residual shunting across the duct, and pulmonary arterial pressures had normalised. It remains unclear why the patient had not developed irreversible pulmonary vascular disease.- - - - - - - - - - ranking = 0.11111111111111keywords = vascular disease (Clic here for more details about this article) |
10/50. Severe erythroderma as a complication of continuous epoprostenol therapy.epoprostenol is a vasodilator that is produced by vascular endothelial cells and is currently the "gold standard" therapy for patients with severe primary pulmonary hypertension or pulmonary hypertension secondary to collagen vascular disease. hypersensitivity to the drug has not been reported. We report a case of a patient with pulmonary hypertension and undifferentiated connective tissue disease who, after 2 months of treatment with epoprostenol, presented with rapidly progressive erythema, scaling, nausea and vomiting, and fever. Test results from a skin biopsy specimen were consistent with a drug reaction. The patient' condition improved after rapid tapering of her epoprostenol and administration of corticosteroids. epoprostenol may be associated rarely with severe erythroderma.- - - - - - - - - - ranking = 0.11111111111111keywords = vascular disease (Clic here for more details about this article) |
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