P01-047 – PH with right-sided heart failure in FMF
نویسندگان
چکیده
Introduction FMF is associated with pulmonary hypertension(PH) due to amyloidosis. However, clinically overt PH with right-sided heart failure remains a rare event limited to few patients with pulmonary amyloidosis secondary to FMF. We report two cases of FMF patients, with and without amyloidosis, who experienced PH with rightsided heart failure. To our knowledge, this is the first case report on cor pulmonale in FMF patient without amyloidosis.
منابع مشابه
A New Minimum Order Lumped-Parameter Model of Circulatory System for Patients with Suffered Left and Right-Sided Heart Failure
In this study a new minimum lumped electrical model of total circulatory system through numerical solutions to approximate the response of the human circulatory system was presented. Among five existent ventricular elastances as activity function of heart pumps, a more accurate model was chosen as well as the operation of baroreflex system to accommodate variations in heart rate and systemic va...
متن کاملP01-012 – Evaluation of autonomic function in FMF
Introduction Familial Mediterranean fever (FMF) is characterized by acute and recurrent attacks of fever and polyserositis. It is associated with conduction disturbances and rhythm abnormalities. Heart rate variability (HRV) is the term used to indicate the fluctuation in cardiac frequency over time. HRV analysis is used to evaluate the condition of the autonomous nervous system, which regulate...
متن کاملThalassemia Associated Pulmonary Hypertension
Cardiac disease is the main cause of death in both forms of thalassemia; thalassemia major (TM) and thalassemia intermedia (TI). Pulmonary hypertension (PH) is one of the cardiopulmonary morbidities with high mortality that, if not treated, may trigger right-sided heart failure and premature death. PH is defined as a mean pulmonary artery pressure of ≥25 mmHg at rest or ≥30 mmHg during exercise...
متن کاملOral vasopressin receptor antagonist tolvaptan in right heart failure due to pulmonary hypertension.
During the past decade, the prognosis of patients with pulmonary hypertension (PH) has improved due to the development of pulmonary arterial vasodilators [1]. Yet, treatment for right-sided heart failure (RHF) associated with PH has not improved, despite being the most important prognostic factor for chronic RHF [2, 3]. Existing treatment strategies for RHF associated with PH are restricted to ...
متن کاملThe pathophysiology of pulmonary hypertension in left heart disease.
Pulmonary hypertension (PH) is characterized by elevated pulmonary arterial pressure leading to right-sided heart failure and can arise from a wide range of etiologies. The most common cause of PH, termed Group 2 PH, is left-sided heart failure and is commonly known as pulmonary hypertension with left heart disease (PH-LHD). Importantly, while sharing many clinical features with pulmonary arter...
متن کامل