Secondary hypertension: A rare cause

A 13-year-old, previously asymptomatic girl was admitted with features of tuberculous meningitis. She was found to be hypertensive and further investigations revealed an extra-adrenal paraganglioma. Tuberculous meningitis and paraganglioma could be chance associations. Paraganglioma is a very rare and potentially lethal cause of secondary hypertension. We are reporting a very rare disease, which has come to light in a most unexpected manner.     

Papillary fibroelastoma: a rare but treatable cause of cerebral infarction

INTRODUCTION: Papillary fibroelastoma is a benign cardiac tumor which can be associated with serious embolic complications. EXEGESIS: We report on a 42-year-old man admitted for an ischemic stroke in the left middle cerebral artery region. Transesophageal echocardiography revealed a mitral valve tumor. Surgical excision and histological examination showed a papillary fibroelastoma. Clinical course was uneventful. CONCLUSION: We consider the high embolic potential of this tumor, which represents a surgically treatable cause of ischemic stroke.     

Systemic mastocytosis: A rare cause of non-cirrhotic portal hypertension

Mastocytosis is a clonal neoplastic disorder of the mast cells(MC) that can be limited to the skin(cutaneous mastocytosis) or involve one or more extracutaneous organs(systemic mastocytosis). The clinical manifestations of mastocytosis are heterogeneous ranging from indolent disease with a long-term survival to a highly aggressive neoplasm with survival of about 6 mo. Although liver involvement in aggressive systemic mastocytosis(ASM) is relatively common, the development of portal hypertension with or without cirrhosis is rare. We report a case of ASM without skin involvement in a 72-year-old caucasian male who presented with non-cirrhotic portal hypertension based on clinical, analytical, imagiological and endoscopic findings. Given the hematological picture, the correct diagnosis was established based on ancillary tests for MC using bone marrow aspirates and biopsy. Extensive involvement of the liver and gastrointestinal tract was histologically documented. The disease progressed rapidly and severe pancytopenia and recurrent upper gastrointestinal bleeding became the dominant problem. This case illustrates the challenge in establishing a diagnosis of ASM especially when the clinical picture is atypical and without skin involvement. Gastroenterologists should consider infiltrative disease, particularly systemic mastocytosis, as a differential diagnosis in a clinical case of portal hypertension of unknown etiology.     

Congenital extrahepatic portocaval malformation: Rare but potentially treatable cause of pulmonary hypertension

ObjectiveAbernethy malformation (congenital extrahepatic portosystemic shunt) is a rare anomaly of the splanchnic venous system. Though rare, it is an important cause of pulmonary artery hypertension (PAH) which is often missed. All patients with PAH should be carefully evaluated for presence of Abernethy malformation before labelling them as Idiopathic PAH.MethodsThis is a retrospective analysis of prospectively collected data. We reviewed the data of all patients referred to our center for evaluation of PAH. 10 patients were diagnosed to have an extrahepatic portocaval malformation. We reviewed their presentation, diagnosis, catheterization data, intervention and their outcome along with review of literature.Results10/104 patients with pulmonary hypertension and no intra or extracardiac shunt were found to have extrahepatic portocaval shunt (EHPCS). 3 patients had EHPCS type 1 and 7 had type 2 EHPCS. 6/7 patient with EHPCS type 2 underwent closure of the shunt. There was no procedure related complication. There was one death 3 months post procedure and one patient who was advised surgical closure was lost to follow up. Closure of the shunt resulted in normalization of the pulmonary artery pressures in 4/5 patients.ConclusionCongenital portosystemic malformations form an important and potentially treatable cause of pulmonary hypertension.     

Hyperthyroidism: a rare cause of reversible pulmonary hypertension

We describe a case of pulmonary hypertension and high-output heart failure in a 61-year-old woman suffering from relapsing Graves disease. The patient experienced prompt hemodynamic and symptomatic recovery after normal thyroid function was restored. Possible mechanisms for the development of pulmonary arterial hypertension due to hyperthyroidism include damage to pulmonary vascular endothelium due to high cardiac output or an autoimmune process or increased metabolism of intrinsic pulmonary vasodilators. Another possible mechanism is vascular vasoconstriction due to decreased cholinergic output.     

Vascular sarcoidosis: a rare cause of pulmonary hypertension

Pulmonary hypertension is an uncommon complication of sarcoidosis and is usually related to the development of end-stage, interstitial fibrosis or honeycomb lung. We describe a patient with sarcoidosis who developed pulmonary hypertension in the absence of significant parenchymal fibrosis. The pulmonary hypertension in this case was caused by granulomatous destruction of arteries and veins, as documented by an open-lung biopsy and autopsy. Clinical features, including pulmonary function tests, are presented which suggested that end-stage interstitial lung disease was not causing the hypertension. Recognition of this complication of sarcoidosis is important if effective therapy is to be found.     

Aortic coarctation: A rare and unexpected cause of secondary arterial hypertension in the elderly

We present a 72-year-old man with long-standing moderate hypertension, due to an aortic coarctation, who presented with a myocardial infarction. In view of his advanced age the aortic coarctation was treated conservatively. © 1996 Wiley-Liss, Inc.     

Hemitruncus arteriosus - a rare cause of pulmonary hypertension

A case of a rare congenital anomaly--hemitruncus arteriosus, in a two month old infant is presented. Diagnosis and treatment of this condition are discussed.     

Aneurysmal dilatation of the portal vein: A rare cause of portal hypertension

Aneurysmal dilatation of the portal vein (ADPV) is a rare cause of portal hypertension. We described a case of ADPV in a female patient who presented with ascites. Imaging studies revealed tortuosity and dilatation of the main portal vein with turbulent flow. Endoscopy revealed oesophageal varices. A liver biospy showed no abnormalities in liver histology. This is the first case of ascites as a complication of ADPV in the absence of liver cirrhosis, arteriovenous fistula or documented portal vein thrombosis. Hyperdynamic circulation and increased portal vein flow could be implicated in the pathogenesis of ascites in this setting.     

Pulmonary capillary hemangiomatosis: a rare cause of pulmonary hypertension

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension characterized by capillary proliferation infiltrating the structures of the pulmonary parenchyma. Although veins are particularly involved, proliferation also affects bronchiolar, interstitial and other structures. We report a case of PCH in a 70-year-old man. Pulmonary artery hypertension was demonstrated by echocardiogram and angiography. Severe emphysema could be seen in a computed tomographic scan of the thorax, even though spirometric values indicated that airflow obstruction was mild. Dyspnea and respiratory insufficiency progressed with marked shunting until death. Tissue inspection at the autopsy revealed capillary proliferation in the alveolar walls with occasional oviform protrusions into air spaces or around small vessels and bronchioles. Endothelial cells in newly formed vessels were not atypical and mitosis was scarce; p53 expression was negative and Ki67 proliferation slight, indicating that PCH is not a neoplastic process as has sometimes been suggested.