Eisenmenger syndrome and other types of pulmonary arterial hypertension related to adult congenital heart disease
| Autor: | SJ Wort, Andrew Constantine, Konstantinos Dimopoulos, Carla Favoccia |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
Cardiac & Cardiovascular Systems
Heart disease Blood viscosity 030204 cardiovascular system & hematology Pulmonary arterial hypertension THERAPY DOUBLE-BLIND 0302 clinical medicine polycyclic compounds 030212 general & internal medicine ESC GUIDELINES PREDICTORS 1102 Cardiorespiratory Medicine and Haematology LUNG TRANSPLANTATION General Medicine congenital heart disease medicine.anatomical_structure Cardiology Cardiology and Cardiovascular Medicine Life Sciences & Biomedicine medicine.drug Adult Heart Defects Congenital medicine.medical_specialty BLOOD-VISCOSITY BOSENTAN Hypertension Pulmonary DIAGNOSIS 1117 Public Health and Health Services 03 medical and health sciences Afterload Internal medicine Internal Medicine medicine MANAGEMENT Humans cardiovascular diseases Science & Technology business.industry cyanosis Eisenmenger syndrome Eisenmenger Complex medicine.disease Pulmonary hypertension Bosentan Cardiovascular System & Hematology Vascular resistance Cardiovascular System & Cardiology secondary erythrocytosis MACITENTAN Quality of Life business |
| Zdroj: | Expert review of cardiovascular therapy. 17(6) |
| ISSN: | 1744-8344 |
| Popis: | Introduction: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD). It is characterised by a severe rise in pulmonary vascular resistance resulting in shunt reversal and cyanosis. Areas covered: In this paper, an overview of ES and other types of PAH related to CHD (PAH-CHD) in adults is provided. The modern management of PAH-CHD in tertiary centers is described, with an emphasis on co-morbidities and complications. Expert opinion: PAH-CHD describes a wide spectrum of conditions, of which ES is the archetype. The size and location of the shunt, the degree of adaptation of the right ventricle to the increased afterload and other compensatory mechanisms, such as secondary erythrocytosis, define the clinical presentation and natural history of these patients. PAH therapies have improved the quality of life and outcome of many patients with PAH-CHD, but expert multidisciplinary management remains essential in optimising the care of this rare and complex group of patients. |
| Databáze: | OpenAIRE |
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