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Chronic Thromboembolic Pulmonary Hypertension (chronic + thromboembolic_pulmonary_hypertension)
Selected AbstractsIsolated congenital spleen agenesis: A rare cause of chronic thromboembolic pulmonary hypertension in an adultRESPIROLOGY, Issue 6 2008Fumiyuki TAKAHASHI Abstract: This report describes a case of isolated congenital spleen agenesis complicated by chronic thromboembolic pulmonary hypertension (CTPH) in a 44-year-old female patient. The patient had increasing exertional dyspnoea and thrombocytosis. An echocardiogram showed severe pulmonary hypertension and right ventricular hypertrophy, and contrast-enhanced chest CT revealed multiple thromboemboli within both pulmonary arteries. A perfusion lung scan demonstrated multiple segmental defects and no spleen was detected by abdominal CT, ultrasonography or scintigraphy. Comprehensive clinical examinations disclosed no evidence of a thrombus elsewhere or of an associated malformation such as a cardiac anomaly. Anticoagulation therapy was started, and a perfusion lung scan revealed partial improvement of the hypoperfusion in the right lower lobe. However, repeat echocardiography showed the pulmonary hypertension persisting for 1 year. The multiple segmental defects in the perfusion lung scans were also persistent. Collectively, a diagnosis of CTPH with isolated congenital spleen agenesis was established. This is the first documented case of CTPH in an adult with isolated congenital asplenia. Although congenital spleen agenesis is a rare condition, this case report suggests that this possibility should be considered when a diagnosis of CTPH and thrombocytosis is made. [source] Polymorphism of the promoter region of prostacyclin synthase gene in chronic thromboembolic pulmonary hypertensionRESPIROLOGY, Issue 2 2004Shinya AMANO Objective: Decreased expression of prostacyclin synthase (PGIS) is observed in the lung vasculature of patients with pulmonary arterial hypertension and the biosynthesis of prostacyclin (PGI2) may be impaired in chronic thromboembolic pulmonary hypertension (CTEPH). Whether it is genetically determined or develops as the disease progresses is unclear. A variable-number tandem repeat (VNTR) polymorphism has been detected in the 5,-upstream promoter region of the PGIS gene. It has been demonstrated that the alleles vary in size from three to seven repeats of nine base pairs, and transcriptional activity increased with the number of repeats. The purpose of the present study was to elucidate the association between the VNTR polymorphisms of the PGIS gene and CTEPH in Japanese subjects. Methodology: Ninety patients with CTEPH and 144 control subjects were investigated for the presence of VNTR polymorphisms. Sixty-two blood samples were obtained from CTEPH patients and the plasma concentrations of prostacyclin and thromboxane A2 metabolites were measured. Results: VNTR polymorphisms in the prostacyclin synthase gene were grouped into L alleles (five, six and seven repeats) and S alleles (three and four repeats). The overall distribution of the alleles and genotypes were not significantly different between CTEPH patients and the control subjects. The patients with the LL genotype had higher plasma concentrations of 6-keto-prostaglandin F1, than patients with the LS and SS genotypes. Conclusions: Our results suggested that the specific VNTR polymorphism in the 5,-upstream promoter region of the PGIS gene regulated prostacyclin production, but did not seem to be associated with the development of CTEPH in this patient population. [source] Right Axillary Vein Cannulation for Percutaneous Cardiopulmonary SupportARTIFICIAL ORGANS, Issue 2 2007Masato Tochii Abstract:, A 34-year-old male with a past history of permanent inferior vena cava (IVC) filter placement was referred to us for chronic thromboembolic pulmonary hypertension. Percutaneous cardiopulmonary support (PCPS) was required for the lung hemorrhage and reperfusion injury, although the thromboendarterectomy was successfully completed. The arterial cannula was inserted into the femoral artery, and the venous cannula was inserted into the right axillary vein. The patient was weaned from PCPS 1 day after the operation and was discharged 35 days after the operation. Axillary vein cannulation is thought to be a feasible method when PCPS is required for a patient with previous IVC filter placement. [source] | ||||||||||