1/11. Post-Mustard procedure pulmonary venous obstruction: An opportunity for anatomic correction with a one-stage arterial switch.A 14-year-old boy after a Mustard procedure for transposition of the great arteries developed pulmonary hypertension secondary to baffle obstruction. This occurred over several years without apparent significant symptomatology. Systemic-level pressure prevailed in the left (pulmonary) ventricle and provided an opportunity to perform a successful one-stage arterial switch.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/11. Successful arterial switch operation for post-Mustard pulmonary venous obstruction and secondary pulmonary hypertension.A 16-year-old girl presented with dyspnea 15 years after the Mustard operation for transposition of the great arteries with intact ventricular septum. An echocardiogram revealed secondary pulmonary hypertension due to pulmonary venous obstruction. cardiac catheterization showed the left (pulmonary) ventricular pressure was over the systemic level. We performed a successful one-stage switch conversion. The patient is doing well 1 year after the switch conversion.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
3/11. Intrastent sonotherapy in pulmonary vein restenosis: a new treatment for a recalcitrant problem.A 2 year old boy developed recurrent pulmonary vein stenosis after surgical repair of infradiaphragmatic pulmonary venous connection. He had required implantation of stents in the left and right sided pulmonary veins at 7 and 13 months of age, respectively. By 2 years of age he had undergone three catheterisation procedures and two surgical procedures to treat recurrent pulmonary vein stenosis. His right ventricular pressure was suprasystemic and catheterisation showed severe neointimal proliferation of both left and right sided stents. At this time the stents were dilated by balloon with simultaneous intrastent sonotherapy. Three months later the patient's clinical improvement was significant, his right ventricular pressure had decreased, and Doppler velocity had decreased across both left and right sided stents.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
4/11. pulmonary veno-occlusive disease and the CREST variant of scleroderma.pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension. The authors describe a 48-year-old female with the calcinosis-Raynaud's phenomenon-Esophageal dysmotility-Sclerodactyly-Telangiectasia (CREST) variant of scleroderma who developed acute pulmonary hypertension with pulmonary infiltrates and a normal pulmonary capillary wedge pressure. At post mortem examination typical changes of pulmonary veno-occlusive disease were found. Similarities between this and other cases in the literature suggest a possible association between the CREST variant of scleroderma and pulmonary veno-occlusive disease.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
5/11. A case of pulmonary veno-occlusive disease associated with systemic sclerosis.The case of a patient with pulmonary veno-occlusive disease associated with systemic sclerosis is reported. The patient presented with progressive dyspnoea. echocardiography and cardiac catheterization study demonstrated right-sided heart failure. The CXR suggested pulmonary hypertension and interstitial pulmonary oedema. We suspected pulmonary veno-occlusive disease based on radiological and haemodynamic findings. Treatment with prednisolone resulted in a reduction in pulmonary arterial pressure and CXR findings improved 2 months later, but no further effect was observed. The patient died 7 months later and at autopsy the lungs showed prominent thickening of the interlobular septa and small branches of pulmonary veins showed intimal thickening.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
6/11. recurrence of pulmonary veno-occlusive disease after heart-lung transplantation.pulmonary veno-occlusive disease, a rare cause of pulmonary hypertension, is characterized by extensive and diffuse occlusion of pulmonary veins by fibrous tissue. Although the diagnosis can be suspected by the presence of the classic clinical triad of severe pulmonary arterial hypertension, radiographic evidence of pulmonary hypertension and edema, and normal pulmonary artery occlusion pressure, the definitive diagnosis is histopathologic. The prognosis of pulmonary veno-occlusive disease is poor with most described patients dying within 2 years of diagnosis. Although anti-coagulation, oxygen, and vasodilator therapies are effective temporarily, the definitive treatment is lung transplantation. We describe the recurrence of pulmonary veno-occlusive disease at 3 months after heart-lung transplantation in a 26-year-old man. recurrence after transplantation for this disease has not been reported previously, and lung transplantation was thought to be definitive treatment. With this 1st report of early recurrence of pulmonary veno-occlusive disease after heart-lung transplantation, we believe that extrapulmonary factors may play a role in the pathogenesis of this rare disease.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
7/11. Absent aortic valve: a complex anomaly.Seven patients (four previously cited and three new cases) with absent aortic valve cusps (leaflets), a rare and underrecognized complex congenital heart defect, are discussed. All patients were male, six full-term and one premature with nonimmunologic hydrops. None underwent operation; all died within the first week of life from low cardiac output and hypoxemia. In most instances, the only remnant of the aortic valve was a nonobstructive fibrous ridge; occasionally, it was accompanied by rudimentary leaflets or sinuses of Valsalva. Absent aortic valve was associated with other significant structural malformations in all instances, including atrioventricular valve atresia, hypoplasia or dysplasia, less commonly double outlet right ventricle, abnormal pulmonary venous connection, or left ventricular endomyocardial abnormalities. Recognition of this unusual lesion is important since it is associated with other complex malformations, causes hypoxemia (for which early positive pressure ventilation is indicated), and could be possibly palliated using the right ventricle as the systemic ventricle.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
8/11. Pulmonary vascular occlusive disease presenting as sudden death.We report a case of pulmonary vascular occlusive disease (also termed pulmonary veno occlusive disease) which caused the sudden death of an eleven-year-old girl. The post-mortem findings are described and the current theories of aetiology and pathogenesis of this unusual condition are reviewed. The clinical picture is characterized by pulmonary hypertension with marked increase of pulmonary arterial pressure but often normal or only slightly elevated wedge pressure. The pulmonary vasculature, especially the veins, show progressive fibrotic occlusion. The aetiology is unknown but an infective cause is considered most likely. Definitive diagnosis usually depends on an open lung biopsy or, more commonly, autopsy examination.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
9/11. pulmonary veno-occlusive disease in a patient with unilateral absence of right pulmonary artery.A 25-year-old patient had unilateral absence of the right pulmonary artery (UARPA) and severe left pulmonary artery hypertension. After death from congestive right heart failure, autopsy revealed histologic signs of pulmonary veno-occlusive disease (PVOD) and pulmonary hypertension (PH). An accessory arterial vessel that was thrombotically occluded was found connecting the ascending aorta and the right pulmonary hilum. There was also histologic evidence of arterial thrombi within the right lung arterial vascular bed. The PH in UARPA usually occurs very early during the course of disease. From histologic findings and medical history, it is likely that in this case, late-onset elevation of pulmonary pressures was triggered by the occurrence of PVOD. This is the first case of UARPA and PVOD--a congenital unilateral arterial malformation in the presence of bilateral involvement in a possibly acquired venous obliterative disease.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
10/11. Effect of prostacyclin on microvascular pressures in a patient with pulmonary veno-occlusive disease.Continuous-infusion prostacyclin improves symptom scores and decreases mortality in patients with primary pulmonary hypertension, but use of prostacyclin in patients with pulmonary veno-occlusive disease may precipitate pulmonary edema. A patient with pulmonary veno-occlusive disease received a graduated intravenous infusion of prostacyclin and pulmonary capillary pressures were calculated during prostacyclin dose ranging. Calculated capillary pressure increased with low-dose prostacyclin (< or = 6 ng/kg/min) but decreased with higher doses. These data suggest that the post-capillary pulmonary venules in our patient had reversible vasomotor tone, but required a higher dose of prostacyclin to vasodilate than did the precapillary arterioles.- - - - - - - - - - ranking = 6keywords = pressure (Clic here for more details about this article) |
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