Right-sided endomyocardial fibrosis with recurrent pulmonary emboli leading to irreversible pulmonary hypertension
TL;DR: A 26 year old Saudi man with features of both Loeffer's endocarditis and endomyocardial fibrosis presented with mild symptoms and pulmonary emboli and died of severe pulmonary hypertension and right ventricular failure several days after operation.
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Abstract: A 26 year old Saudi man with features of both Loeffer9s endocarditis and endomyocardial fibrosis presented with mild symptoms and pulmonary emboli. Echocardiographic examination showed obliteration of the right ventricular apex by an attached mass. The results of haemodynamic studies were somewhat abnormal and medical treatment was started. Despite anticoagulation with warfarin the patient9s condition deteriorated rapidly over a four month period after a further episode of pulmonary embolism and the development of pulmonary hypertension. Two haemodynamic studies performed four months apart were typical of pulmonary hypertension and later right ventricular failure; they showed none of the characteristics of restriction. Pulmonary embolectomy was attempted but there was no cleavage plane between the organised thrombi and the endothelium of the pulmonary artery. The patient died of severe pulmonary hypertension and right ventricular failure several days after operation. Surgical intervention in the early stages of right-sided endomyocardial fibrosis might have prevented the development of pulmonary embolism and pulmonary hypertension.
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Citations
Diagnosis and Management of Endomyocardial Fibrosis.
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TL;DR: Increased awareness, advocacy, and research are needed to further understand this neglected tropical cardiomyopathy and to improve survival of those affected.
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Qual o seu diagnóstico
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TL;DR: Machado et al. as mentioned in this paper presented the work of the Departamento de Radiologia da Faculdade de Medicina da Universidade de Sao Paulo (FMUSP) in Brazil.
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Kristina Selthofer-Relatić,Martina Mihalj,Aleksandar Kibel,Ana Stupin,Marko Stupin,Ivana Jukić,Akos Koller,Ines Drenjančević +7 more
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Endomyocardiofibrosis: A Systematic Review.
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Tropical endomyocardial fibrosis: an overview -
TL;DR: A systematic review of the most intriguing aspects of epidemiology, natural history, clinical picture and management of endomyocardial fibrosis, proposing new ways to increase research into this challenging and neglected cardiovascular disease.
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