1/60. Primary pulmonary hypertension with fatal outcome in a young woman and review of the literature.A 32-year-old female is described, who was admitted with symptoms of severe right heart failure. The most likely diagnosis of pulmonary embolism was excluded. echocardiography and left-right catheterisation confirmed the diagnosis of primary pulmonary hypertension. A possible mediator in the process of PPH could be the appetite suppressants she had taken for some months after her second pregnancy. Before further pharmacologic tests could be performed the patient died in circulatory collapse. Postmortem pathological examination confirmed the diagnosis of PPH by the presence of narrowed pulmonary arterioles, media hypertrophy, thrombotic lesions and normal surrounding pulmonary parenchyma. The literature on primary pulmonary hypertension is revised with special emphasis on diagnosis and treatment algorithms.- - - - - - - - - - ranking = 1keywords = hypertrophy (Clic here for more details about this article) |
2/60. Successful pulmonary thromboendarterectomy in a patient with Klippel-Trenaunay syndrome.Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by a triad of cutaneous vascular nevi, soft tissue or bony hypertrophy, and varicose veins or venous malformations involving one or more extremities. An incidence of venous thromboembolism of up to 22% has been reported in this disorder. Also reported is the development of chronic thromboembolic pulmonary hypertension (CTEPH) and subsequent death from right ventricular failure. We report the first patient with KTS to undergo a successful pulmonary thromboendarterectomy for CTEPH.- - - - - - - - - - ranking = 1keywords = hypertrophy (Clic here for more details about this article) |
3/60. Reversible cardio-pulmonary changes due to adeno-tonsilar hypertrophy.Adeno-tonsillar hypertrophy, with signs of upper airway obstruction is a common presentation in ENT clinics. Recently it is identified as a major cause of sleep apnea syndrome. Several isolated case reports of pulmonary hypertension and corpulmonale appeared in the literature. The authors report two such children aged less than 2 years with cardio-pulmonary changes occurring secondary to chronic adeno-tonsillar hypertrophy that were successfully treated with the surgical removal.- - - - - - - - - - ranking = 6keywords = hypertrophy (Clic here for more details about this article) |
4/60. hiv-Related pulmonary hypertension: analytic review of 131 cases.OBJECTIVE: To report two new cases of hiv-related pulmonary hypertension and to review and analyze the existing reports on the subject. METHOD: Two new cases of hiv-related pulmonary hypertension are described, and the cases, case series, and related articles on the subject in all languages were identified through a comprehensive medline search. RESULTS: Among the 131 reviewed cases, 54% were male, and the age range was 2 to 56 years (mean, 33 years). The interval between the diagnosis of hiv disease and the diagnosis of pulmonary hypertension was 33 months. In 82% of cases, pulmonary hypertension was related solely to hiv infection. Presenting symptoms were progressive shortness of breath (85%), pedal edema (30%), nonproductive cough (19%), fatigue (13%), syncope or near-syncope (12%), and chest pain (7%). The mean ( /- SD) pulmonary arterial systolic BP was 67 /- 18 mm Hg (n = 116), and diastolic BP was 40 /-11 mm Hg (n = 39). Pulmonary vascular resistance was 983 /-420 dyne. s. cm(-5) (n = 29). Chest radiographs demonstrated cardiomegaly (72%) and pulmonary artery prominence (71%). Right ventricular hypertrophy was the most common electrocardiographic finding (67%). dilatation of the right heart chambers was the most common echocardiographic finding (98%). Plexogenic pulmonary arteriopathy was the most common histopathology (78%). Pulmonary function tests demonstrated mild restrictive patterns with variably reduced diffusing capacities. The responses to vasodilator agents and antiretroviral therapy was variable. Sixty-six patients died during a median follow-up period of 8 months. The median length of time from diagnosis to death was 6 months. CONCLUSION: hiv infection is an independent risk factor for the development of pulmonary hypertension. The appearance of unexplained cardiopulmonary symptoms in hiv-infected individuals should suggest pulmonary hypertension.- - - - - - - - - - ranking = 1keywords = hypertrophy (Clic here for more details about this article) |
5/60. Pulmonary hypertension.A clinically useful, treatment-based classification of pulmonary hypertension divides the disease into 5 distinct categories: (1) pulmonary hypertension associated with disorders of the respiratory system and/or hypoxemia; (2) pulmonary venous hypertension; (3) chronic thromboembolic disease; (4) pulmonary arterial hypertension; and (5) pulmonary hypertension due to disorders directly affecting the pulmonary vasculature. Pulmonary arterial hypertension includes individuals with primary pulmonary hypertension, congenital heart disease, connective tissue disease, and liver disease. These heterogeneous diseases have similar characteristic pathological changes, including in situ thrombosis, smooth muscle hypertrophy, and intimal proliferation. Right heart catheterization is essential to confirm diagnosis, determine prognosis, and assign therapy. A minority of patients have a favorable response to an acute vasodilator trial and long-term benefit with calcium channel blocker therapy. Continuous intravenous epoprostenol improves symptoms and survival in patients with advanced primary pulmonary hypertension and has potential benefit in other forms of pulmonary arterial hypertension. lung transplantation remains an important option for individuals in whom maximal medical therapy fails. The recent discovery of the gene for familial primary pulmonary hypertension and the increase in new drugs undergoing clinical trials are encouraging developments.- - - - - - - - - - ranking = 1keywords = hypertrophy (Clic here for more details about this article) |
6/60. An autopsy case of pulmonary capillary hemangiomatosis without evidence of pulmonary hypertension.We report a case of pulmonary capillary hemangiomatosis (PCH) affecting a 37-year-old woman. PCH is a rare disease accompanying intrapulmonary capillary growth and pulmonary hypertension. In the present case, capillaries infiltrated the alveolar and bronchial walls but not the vascular walls. No signs or symptoms of pulmonary hypertension or right heart hypertrophy/dilatation were present. These findings suggest that vascular involvement in PCH is responsible for the induction of pulmonary hypertension.- - - - - - - - - - ranking = 1keywords = hypertrophy (Clic here for more details about this article) |
7/60. 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 = 1keywords = hypertrophy (Clic here for more details about this article) |
8/60. 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 = 1keywords = hypertrophy (Clic here for more details about this article) |
9/60. Pulmonary hypertension in the setting of acquired systemic arteriovenous fistulas.We describe 2 patients who presented with symptoms and clinical evidence of pulmonary hypertension. The first patient, who had severe pulmonary hypertension, underwent correction of an acquired arteriovenous (AV) fistula, which led to improvement according to clinical and noninvasive hemodynamic criteria. The second patient had severely symptomatic pulmonary hypertension associated with a large, previously unrecognized, superior mesenteric AV fistula after intra-abdominal surgery. Surgical correction of the fistula resulted in normalization of pulmonary hemodynamics assessed by right heart catheterization. Truly reversible substrates of pulmonary hypertension are rare. Acquired systemic AV fistulas are a less recognized cause of secondary pulmonary hypertension. It is unknown whether high blood flow peripheral AV shunts ultimately lead to endothelial proliferation, vascular smooth muscle hypertrophy, plexiform lesions, and other histopathologic changes that are seen in patients with left-to-right shunts due to atrial septal defects, ventricular septal defects, and patent ductus arteriosus.- - - - - - - - - - ranking = 1keywords = hypertrophy (Clic here for more details about this article) |
10/60. Severe pulmonary hypertension associated with primary sjogren's syndrome.Severe pulmonary hypertension is one of the fetal complications in various connective tissue diseases. We report a case of severe pulmonary hypertension associated with primary sjogren's syndrome. In a lung biopsy specimen, there were findings of intimal and medial hypertrophy with narrowing vessel lumina and plexiform lesions. Moreover, deposits of immunoglobulin m, immunoglobulin a and complement protein C1q were found in the pulmonary arterial walls. Although pulmonary hypertension was refractory to oral prostacyclin, steroid therapy improved the clinical and hemodynamic conditions. In the present case, the immunological etiology may be related to the mechanisms of pulmonary hypertension associated with sjogren's syndrome.- - - - - - - - - - ranking = 1keywords = hypertrophy (Clic here for more details about this article) |
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