1/8. Urgency of operation in infracardiac total anomalous pulmonary venous connection.BACKGROUND: Because the tendency for pulmonary venous obstruction in the infracardiac type of total anomalous pulmonary venous connection may be partially dependent on the connection of the descending vein to the portal vein, the inferior vena cava, or one of their tributary vessels, we reviewed our surgical experience with various subtypes of infracardiac total anomalous pulmonary venous connection. methods: The urgency of operation in 4 neonates with infracardiac total anomalous pulmonary venous connection was reviewed. RESULTS: Two patients with pulmonary venous obstruction in whom the descending vein connected to the portal vein were operated on immediately with successful outcome. One patient who had become critically ill after the ductus venosus had closed died before operation could be undertaken. One patient in whom the descending vein connected to the left hepatic vein was operated on electively with successful outcome. CONCLUSIONS: In hemodynamically stable patients with no clinical or echocardiographic signs of pulmonary venous obstruction, some form of differentiation with regard to urgency of operation may be appropriate. When the descending vein connects to the inferior vena cava or a hepatic vein, the operation may be performed on a semi-elective basis. In contrast, when the descending vein connects to the portal vein or the ductus venosus, operation should generally not be delayed because of the high likelihood of obstruction.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/8. Recurrent unilateral bacterial pneumonias and interstitial fibrosis associated with pulmonary vein atresia: successful treatment with endovascular stent implantation.A variety of pulmonary vascular disorders, such as hemangiomatosis, telangectasia, and veno-occlusive disease, may be involved in the pathogenesis of interstitial lung diseases. We describe the case of a girl with recurrent bacterial pneumonia and progressive interstitial fibrosis affecting the right lung. Morphologic evaluation of the lung biopsy showed structural changes of the vessel walls suggesting pulmonary hypertension. The echocardiogram showed the presence of centripetal blood flow in the right pulmonary artery from the periphery of the lung to the heart. A selective right angiography demonstrated the presence of pulmonary venous obstruction at the veno-atrial junction, successfully treated by endovascular stent implantation during cardiac catheterization.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/8. 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 = vessel (Clic here for more details about this article) |
4/8. Sideroelastosis pulmonum: cause or consequence of the pulmonary veno-occlusive disease?In a 66-year-old patient morphologic features of a pulmonary veno-occlusive disease were found. Besides, a striking foreign body giant cell reaction with phagocytosis of altered elastic tissue in totally or partially occluded venous blood vessels resembled the so-called pulmonary sideroelastosis, an entity originally described by Ceelen. In this case report, a possible pathogenetic relation between sideroelastosis pulmonum and pulmonary veno-occlusive disease is discussed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/8. Pulmonary vein stenosis.Pulmonary vein stenosis (PVS) is a rare disorder. Accurate diagnosis often requires anatomical examination. We report four children with pulmonary vein stenosis. autopsy showed bilateral lesions in two patients who were thought clinically to have unilateral disease. A diagnosis of PVS was made at autopsy in the third case. Intimal and medial fibromuscular proliferation was noted in extrapulmonary and intrapulmonary veins. Some of the fibromuscular proliferation were eccentric, resembling organized thrombi. In one case a focal organizing thrombus was found in a clinically unobstructed but anatomically narrowed veno-atrial junction. In another case injection of contrast medium into the stenotic pulmonary vein (PV) showed anastomosis between PV and bronchial vessels as well as small pulmonary arteries. Bilateral hypertensive arteriopathy was observed in unilateral and bilateral PVS. Our histological finding of intrapulmonary venous lesions in the lobes in which PVS was not detected clinically suggests that during surgical correction of unilateral PVS multiple biopsies of the opposite lung may help to evaluate possible bilateral disease. Our study also suggests that thrombosis in a stenotic pulmonary vein may further compromise the lumen and contribute to the progression of pulmonary vein obstruction. The possible pathogenesis of bilateral pulmonary hypertensive arteriopathy in unilateral PVS also is discussed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/8. radiation-induced pulmonary veno-occlusive disease.Late occurrence of radiation-induced pulmonary pneumonitis and fibrosis is well documented. We report an unusual case of radiation induced veno-occlusive disease (VOD) occurring six years following mantle irradiation for Hodgkin's lymphoma. The patient developed severe pulmonary hypertension and cor pulmonale. A left lung transplantation was performed successfully and pathologic examination of the explanted lung showed severe changes compatible with VOD. In the absence of exposure to alternate therapeutic or toxic agents that may cause VOD, it is likely that radiation caused damage to the venular endothelium and caused progressive obliteration of the pulmonary vessels. review of the literature reveals only a few similar reports of VOD mostly following radiation for bone marrow transplantation. We conclude that previous irradiation (even several years earlier) should be considered as a possible cause of pulmonary VOD.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/8. Human alveolar capillaries undergo angiogenesis in pulmonary veno-occlusive disease.The bronchial circulation undergoes angiogenesis in several pathological conditions, such as lung neoplasm and bronchiectasis, but whether the pulmonary circulation can do this has been questioned. A woman treated with mitomycin C and 5-fluorouracil developed progressive, fatal pulmonary hypertension over 5 months. In addition to light and transmission electron microscopic examination of her lung, her pulmonary vasculature was cast and the casts were studied with scanning electron microscopy. light microscopy showed that she had pulmonary veno-occlusive disease and angiomatoid capillary growth in the alveolar walls. Transmission electron microscopy confirmed the presence of pulmonary hypertension and showed thickened endothelial basement membrane. Scanning electron microscopy of the cast blood vessels showed distortion and destruction of alveolar capillaries prohibiting the passage of erythrocytes. Large new capillaries developed on top of, and were connected to, the shrivelled capillaries that made up the alveolar wall. The new capillaries were larger and fewer, which reduced the alveolar-capillary interface. arteries and veins were irregularly narrowed and the veins had broad muscularity. Oedema was present, and the pulmonary lymphatics were extensively cast, especially in the lobular septa, but the lymphatics had a normal appearance. It appears that this patient suffered extensive capillary damage and venous occlusion and that the response was extensive new capillary formation, sometimes in angiomatoid configurations, and hypertrophy of pulmonary veins and arteries. Casting the microvasculature and viewing it with scanning electron microscopy identified new alveolar capillaries in this patient with acquired pulmonary hypertension.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/8. pulmonary veno-occlusive disease accompanied by microangiopathic hemolytic anemia 1 year after a second bone marrow transplantation for acute lymphoblastic leukemia.Although hepatic veno-occlusive disease (HVOD) is a common complication of allogenic bone marrow transplantation (BMT), pulmonary veno-occlusive disease (PVOD) is very rare. Only three cases have been described in the literature. We report the case of a 19-year-old woman who developed PVOD accompanied by microangiopathic hemolytic anemia (MAHA) and hemolytic uremic syndrome (HUS) 1 year after a second BMT for relapsed acute lymphoblastic leukemia (ALL). autopsy examination revealed obstruction of the small pulmonary veins with edematous thickening of the intima. These findings are compatible with PVOD. Pulmonary GVHD and pulmonary aspergillosis were also observed. Various etiologic factors have been implicated in PVOD after BMT. We postulate that pulmonary GVHD and pulmonary infection including aspergillosis played an important role in the occurrence of both PVOD and HUS in our patient. Microangiopathic cytokines released in response to the GVHD and infection may damage the intima of microvessels that were previously injured by the two BMT. Despite appropriate therapy, the microangiopathic process was irreversible and the patient died. Thus, measures must be taken to prevent and treat PVOD after BMT.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |