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Bronchopulmonary-Coronary Arterial Fistulas in Chronic Thromboembolic Pulmonary Hypertension: Series of 3 Cases
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A3709 - Bronchopulmonary-Coronary Arterial Fistulas in Chronic Thromboembolic Pulmonary Hypertension: Series of 3 Cases
Author Block: M. Pessi, E. P. Oliveira, D. Calderaro, C. Fernandes, C. Jardim, R. Souza; Pulmonary Division, Heart Institute (InCor), University of São Paulo Medical School, Sao Paulo, Brazil.
Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a condition in which unresolved pulmonary embolism increases pulmonary vascular resistance, leading to right heart failure. One of the findings of CTEPH is the enlargement of the bronchial arteries, in addition to the enlargement of the pulmonary artery, since there is a network of anastomotic channels connecting the two circulations. The pressure in this system increases and neo-vascular formation may occur. The appearance of arterial fistulas between the bronchopulmonary and coronary systems represents a diagnostic and therapeutic challenge. The objective of this study is to report three cases of CTEPH fistulas and to present considerations about clinical manifestations and methods of diagnosis and treatment.
Case reports: records of patients with CTEPH treated at the Pulmonology outpatient clinic of HCFMUSP Heart Institute were reviewed and three cases of bronchopulmonary-coronary fistula were found, all of them male and with a mean age of 47 years (ranging from 42 to 55 years). The functional class ranged from II to III. All were former smokers and two former users of illicit drugs. All patients were treated with anticoagulation and diuretic, but their clinical condition deteriorated. On echocardiography, systolic pulmonary arterial pressure (PAP) ranged from 82 to 112 mmHg, with signs of moderate to severe right ventricular dysfunction. Chest computed tomography showed signs of CTEPH associated with bronchial artery dilation. In right cardiac catheterization, mean PAP ranged from 44 to 60 mmHg, pulmonary vascular resistance from 8.5 to 14.5 W and cardiac output from 2.2 to 4.8 L / min. Left heart catheterization showed fistulas between the pulmonary artery and the coronary circulation in one patient, between the bronchial arteries and the right coronary artery in the other and the right atrium with the circumflex artery in the third case. These fistulas seemed to contribute to myocardial ischemia, evidenced by nuclear analysis in one of the patients. All patients underwent clinical treatment and one underwent endarterectomy, with functional class improvement, while others await surgical intervention.
Discussion: Recognizing such vascular anormalities is important for an adequate understanding of the pathophysiology of the disease, as well as an appropriate surgical planning, in which pulmonary endarterectomy can be considered along with specific intervention in the coronary circulation.
Author Block: M. Pessi, E. P. Oliveira, D. Calderaro, C. Fernandes, C. Jardim, R. Souza; Pulmonary Division, Heart Institute (InCor), University of São Paulo Medical School, Sao Paulo, Brazil.
Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a condition in which unresolved pulmonary embolism increases pulmonary vascular resistance, leading to right heart failure. One of the findings of CTEPH is the enlargement of the bronchial arteries, in addition to the enlargement of the pulmonary artery, since there is a network of anastomotic channels connecting the two circulations. The pressure in this system increases and neo-vascular formation may occur. The appearance of arterial fistulas between the bronchopulmonary and coronary systems represents a diagnostic and therapeutic challenge. The objective of this study is to report three cases of CTEPH fistulas and to present considerations about clinical manifestations and methods of diagnosis and treatment.
Case reports: records of patients with CTEPH treated at the Pulmonology outpatient clinic of HCFMUSP Heart Institute were reviewed and three cases of bronchopulmonary-coronary fistula were found, all of them male and with a mean age of 47 years (ranging from 42 to 55 years). The functional class ranged from II to III. All were former smokers and two former users of illicit drugs. All patients were treated with anticoagulation and diuretic, but their clinical condition deteriorated. On echocardiography, systolic pulmonary arterial pressure (PAP) ranged from 82 to 112 mmHg, with signs of moderate to severe right ventricular dysfunction. Chest computed tomography showed signs of CTEPH associated with bronchial artery dilation. In right cardiac catheterization, mean PAP ranged from 44 to 60 mmHg, pulmonary vascular resistance from 8.5 to 14.5 W and cardiac output from 2.2 to 4.8 L / min. Left heart catheterization showed fistulas between the pulmonary artery and the coronary circulation in one patient, between the bronchial arteries and the right coronary artery in the other and the right atrium with the circumflex artery in the third case. These fistulas seemed to contribute to myocardial ischemia, evidenced by nuclear analysis in one of the patients. All patients underwent clinical treatment and one underwent endarterectomy, with functional class improvement, while others await surgical intervention.
Discussion: Recognizing such vascular anormalities is important for an adequate understanding of the pathophysiology of the disease, as well as an appropriate surgical planning, in which pulmonary endarterectomy can be considered along with specific intervention in the coronary circulation.
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