1/83. A case of truncus arteriosus type II.A case of truncus arteriosus type II is reported. Truncus arterious is an uncommon congenital cardiac defect where a single great vessel exits the heart. truncus arteriosus is usually fatal, if untreated. This defect occurs when the conus arteriosus and the truncus divide erroneously in the embryo. Palliative surgery in truncus arteriosus has been unsuccessful. Pulmonary banding has been tried and was ineffective and usually fatal. We operated on a nine-month-old (6200 g) male infant with a type II (Edwards-Collett) defect and a large ventricular septal defect. The pulmonary artery average pressure was 51 mmHg. We performed a cardiopulmonary bypass in the usual manner. Pulmonary arteries were resected from the truncal root, and primary end-to-end anastomosis of the truncal root to the ascending aorta was performed. Right ventricle to pulmonary artery continuity was provided using a valveless Gore-Tex graft. We lost our patient due to intractable pulmonary hypertension on the first postoperative day.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/83. Pulmonary hypertension secondary to neurofibromatosis: intimal fibrosis versus thromboembolism.Neurofibromatosis has been known to involve blood vessels throughout the body. Pulmonary involvement with interstitial fibrosing alveolitis has been described but no case of pulmonary vascular involvement has been reported to date. A 51 year old patient with cutaneous neurofibromatosis is described who presented with severe pulmonary hypertension and radiographic, scintigraphic, and angiographic evidence of chronic thromboembolic pulmonary hypertension. Severe intimal fibrosis consistent with vascular involvement with neurofibromatosis was found on endarterectomy with no evidence of pulmonary thromboembolism. Neurofibromatosis of pulmonary arteries should be considered as a possible cause of pulmonary hypertension.- - - - - - - - - - ranking = 2.1036461909134keywords = blood vessel, vessel (Clic here for more details about this article) |
3/83. In utero development of hypertensive necrotizing pulmonary arterial lesions: report of a case associated with premature closure of the ductus arteriosus and pulmonary hypoplasia.Premature closure of the ductus arteriosus (PCDA) is an uncommon defect in which pulmonary hypertension (PH) has been documented by echocardiography in patients and by direct measurement after experimental PCDA in animals. The pulmonary vascular histology in human cases has received little attention but in the few recorded observations the vessels were either normal or showed increased muscularity. We report the case of a 31 week hydropic female stillborn monozygotic twin in whom postmortem examination disclosed PCDA and hypoplasia of the lungs. Atypical plexiform lesions with necrotizing pulmonary arteritis were present. These lesions represent vascular consequences of severe pulmonary hypertension produced by greatly enhanced blood flow through a restricted vascular bed resulting from the combined effects of these two abnormalities. The findings in this case of PCDA with presumed severe PH indicate that severe pulmonary vascular changes can develop in utero and that the interval of time needed for development of such chances in secondary PH is relatively short.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/83. Congenital misalignment of pulmonary vessels and alveolar capillary dysplasia: how to manage a neonatal irreversible lung disease?Congenital misalignment of pulmonary vessels (MPV) with alveolar capillary dysplasia is a rare condition consisting of anomalous veins in bronchovascular bundles, a decreased number of alveolar capillaries, and increased muscularization of pulmonary arterioles. In the literature, infants reported as having such a malformation developed respiratory distress with persistent pulmonary hypertension and ultimately died. We report the case of an infant with MPV and alveolar capillary dysplasia who was unresponsive to maximal cardiorespiratory support, including high-frequency oscillatory ventilation and inhaled nitric oxide; the infant died of pulmonary hemorrhage after 19 days, during venoarterial extracorporeal membrane oxygenation bypass. We conclude that the diagnosis of MPV and alveolar capillary dysplasia should be considered during autopsy of infants who have died of irreversible persistent pulmonary hypertension. If a lung biopsy in infants with prolonged refractory hypoxemia confirms such diagnosis before death, expensive and invasive treatments such as extracorporeal membrane oxygenation could be avoided.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
5/83. Stable pulmonary capillary haemangiomatosis without symptomatic pulmonary hypertension.Pulmonary capillary haemangiomatosis is a rare disorder characterised by multiple angiomatous lesions composed of proliferating capillary vessels in the lung parenchyma that usually progress rapidly to establish fatal pulmonary hypertension. The 29 year old man presented here, however, has been stable for 3.5 years since the diagnosis without symptoms of pulmonary hypertension. High resolution computed tomographic findings of the pulmonary lesions seemed specific to the disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/83. Pulmonary capillary hemangiomatosis associated with primary pulmonary hypertension: report of 2 new cases and review of 35 cases from the literature.Pulmonary capillary hemangiomatosis (PCH) is a rare cause of primary pulmonary hypertension characterized by thin-walled microvessels infiltrating the peribronchial and perivascular interstitium, the lung parenchyma, and the pleura. These proliferating microvessels are prone to bleeding, resulting in accumulation of hemosiderin-laden macrophages in alveolar spaces. Here we report 2 cases of PCH with pulmonary hypertension, 1 of them associated with mechanical intravascular hemolysis, a feature previously reported in other hemangiomatous diseases, but not in PCH. Case 2 was diagnosed by pulmonary biopsy; to our knowledge the patient is the second adult to be treated with interferon alpha-2a. review of the literature identified 35 patients with PCH and pulmonary hypertension. The prognosis is poor and median survival was 3 years from the first clinical manifestation. dyspnea and right heart failure are the most common findings of the disease. hemoptysis, pleural effusion, acropachy, and signs of pulmonary capillary hypertension are less common. Chest X-ray or computed tomography scan usually shows evidence of interstitial infiltrates, pulmonary nodules, or pleural effusion. Hemodynamic features include normal wedge pressures. Radiologic and hemodynamic findings are undifferentiated from those of pulmonary veno-occlusive disease but differ from other causes of primary pulmonary hypertension. epoprostenol therapy, considered the treatment of choice in patients with primary pulmonary hypertension, may produce pulmonary edema and is contraindicated in patients with PCH. Regression of lesions was reported in 1 patient treated with interferon therapy and 2 other patients stabilized, including our second patient. PCH was treated successfully by lung transplantation in 5 cases. Early recognition of PCH in patients with suspected primary pulmonary hypertension is possible based on clinical and radiologic characteristics. diagnosis by pulmonary biopsy is essential for allowing appropriate treatment.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
7/83. An infant with pulmonary hypertension due to a congenital porto-caval shunt.A 20-month-old male child died during an episode of acute bronchitis. The autopsy revealed massive hypertrophy and acute dilatation of the right heart which was caused by pulmonary hypertension exhibiting plexogenic arteriopathy and necrotizing arteriitis of small lung vessels. Further examination revealed complete old fibrotic occlusion of the extrahepatic portal vein and a large porto-systemic shunt. Esophageal varices indicating portal hypertension were not present. histology showed enlarged hepatic arteries within the portal tracts while branches of the portal vein were lacking. The placenta had been examined at the time of birth and multiple chorangiomas reported. Furthermore, multiple old and florid occlusions of fetal vessels had been detected together with focal lymphoplasmocytic inflammation, which may indicate that intrauterine infection and vascular compromise were the cause of the complete closure of the portal vein around birth. The association of pulmonary hypertension with liver disease is well known, and portal hypertension has been considered a key factor in the pathogenesis of pulmonary hypertension. However, this case illustrates that portal hypertension is not a requisite for the development of pulmonary hypertension. It rather suggests that plexogenic arteriopathy of the lungs can be caused by porto-caval shunting independent of liver damage and portal hypertension. Toxic metabolites of nutrients or residual activity of pancreatic enzymes reaching the pulmonary vascular bed may be involved in the pathogenesis.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
8/83. Portopulmonary hypertension syndrome in schistosomiasis mansoni.Portopulmonary hypertension syndrome (PPHS) is a complication of portal hypertension where the substrate is micro-vessel lesions which are indicative of plexogenic arteriopathy. PPHS has not been linked to pulmonary schistosomiasis. We report, to the best of our knowledge for the first time, a case of PPHS associated with schistosomiasis mansoni.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/83. herpes simplex type 2 pneumonia.Extensive reviews of pulmonary infections in AIDS have reported few herpetic infections. Generally these infections are due to herpes simplex type 1. pneumonia due to herpes type 2 is extremely rare. We describe a 40 year-old hiv positive woman who complained of fever, cough and dyspnea for seven years. She had signs of heart failure and the appearance of her genital vesicles was highly suggestive of genital herpes. echocardiography showed marked pulmonary hypertension, right ventricular hypertrophy and tricuspid insufficiency. After a few days of hospitalization she was treated with Aciclovir and later with ganciclovir. An open pulmonary biopsy revealed an interstitial inflammation, localized in the alveolar walls. Some pulmonary arteries had widened walls and focal hyaline degeneration. immunohistochemistry indicated that the nuclei had herpes simplex virus type 2 in many endothelial cells (including vessels with widened walls), macrophages in the alveolar septa and pneumocytes. There was clinical improvement after treatment for herpes. We concluded that as a consequence of herpes infection, endothelial involvement and interstitial inflammation supervene, with thickening of vascular walls and partial obliteration of the vessel lumen. A direct consequence of these changes in pulmonary vasculature was pulmonary hypertension followed by heart failure.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
10/83. Uveal effusion and angle-closure glaucoma in primary pulmonary hypertension.PURPOSE: To report a patient with uveal effusion and intermittent angle-closure glaucoma associated with primary pulmonary hypertension. methods: Observational case report. RESULTS: A 78-year-old woman with primary pulmonary hypertension noticed pain and decreased vision in her right eye. Ocular examination disclosed excessive dilation of conjunctival and episcleral veins and uveal effusion with annular ciliochoroidal detachment in both eyes. The right eye revealed corneal edema with folds in the descemet membrane, congested iris vessels, and a markedly shallow anterior chamber with a partially closed angle, corresponding to a recent attack of angle-closure glaucoma. Systemic treatment of her congestive heart failure with an angiotensin-II receptor antagonist resulted in a partial resolution of the uveal effusion and complete normalization of the anterior chamber depth. CONCLUSION: Primary pulmonary hypertension may cause uveal effusion, leading to a forward displacement of the lens-iris diaphragm and intermittent angle-closure glaucoma.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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