CLINICAL FEATURES OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL SYSTEMIC-TO-PULMONARYSHUNTS
Pulmonary arterial hypertension (PAH), associated with congenital heart disease (CHD) (PAH-CHD), despite the similarities of histological lesions, is а different from other forms of PAH. PAH-CHD is represented by a heterogeneous patient population with varying defect locations, concomitant diseases,...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | Russian |
| Published: |
InterMedservice
2017-12-01
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| Series: | Евразийский Кардиологический Журнал |
| Subjects: | |
| Online Access: | https://www.heartj.asia/jour/article/view/256 |
| Summary: | Pulmonary arterial hypertension (PAH), associated with congenital heart disease (CHD) (PAH-CHD), despite the similarities of histological lesions, is а different from other forms of PAH. PAH-CHD is represented by a heterogeneous patient population with varying defect locations, concomitant diseases, indications and contraindications to surgical correction; also, some patients have a history of a defect closure. According to the European guidelines for the diagnosis and treatment of pulmonary hypertension 2015, there are four main groups in the clinical classification of PAH-CHD: 1) Eisenmenger’s syndrome, 2) PAH associated with prevalent systemic-to-pulmonary shunts, 3) PAH with small/concidental defects, 4) PAH after defects correction. The evolution of the guidelines for the surgical correction of CHD has seen in recent years. Risk stratification has a great importance for further prognosis, response to treatment and timing of surgical correction. For optimal treatment carefulness and expertise in diagnosis and differential diagnosis should be exercised. A better understanding of clinical features, risk stratification and the assessment of the impact of a genetic background will help to determine the best clinical management, which is associated with better quality of life and improved survival in patients with PAH-CHD. |
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| ISSN: | 2225-1685 2305-0748 |