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,...
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| Format: | Article |
| Language: | Russian |
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InterMedservice
2017-12-01
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| Series: | Евразийский Кардиологический Журнал |
| Subjects: | |
| Online Access: | https://www.heartj.asia/jour/article/view/256 |
| _version_ | 1826571922305974272 |
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| author | S. Ye. Gratsianskaya O. A. Arkhipova A. V. Zorin T. V. Martynyuk I. Ye. Chazova |
| author_facet | S. Ye. Gratsianskaya O. A. Arkhipova A. V. Zorin T. V. Martynyuk I. Ye. Chazova |
| author_sort | S. Ye. Gratsianskaya |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-10T02:44:00Z |
| format | Article |
| id | doaj.art-d59c76a85149497d842ad3ba0ecbee52 |
| institution | Directory Open Access Journal |
| issn | 2225-1685 2305-0748 |
| language | Russian |
| last_indexed | 2025-03-14T12:18:49Z |
| publishDate | 2017-12-01 |
| publisher | InterMedservice |
| record_format | Article |
| series | Евразийский Кардиологический Журнал |
| spelling | doaj.art-d59c76a85149497d842ad3ba0ecbee522025-03-02T09:34:19ZrusInterMedserviceЕвразийский Кардиологический Журнал2225-16852305-07482017-12-0104283910.38109/2225-1685-2017-4-28-39255CLINICAL FEATURES OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL SYSTEMIC-TO-PULMONARYSHUNTSS. Ye. Gratsianskaya0O. A. Arkhipova1A. V. Zorin2T. V. Martynyuk3I. Ye. Chazova4National Medical Research Centre of cardiology of Ministry of HealthNational Medical Research Centre of cardiology of Ministry of HealthNational Medical Research Centre of cardiology of Ministry of HealthNational Medical Research Centre of cardiology of Ministry of HealthNational Medical Research Centre of cardiology of Ministry of HealthPulmonary 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.https://www.heartj.asia/jour/article/view/256pulmonary hypertensioncongenital heart defectsthe syndrome eisenmengerresidual pulmonary hypertension |
| spellingShingle | S. Ye. Gratsianskaya O. A. Arkhipova A. V. Zorin T. V. Martynyuk I. Ye. Chazova CLINICAL FEATURES OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL SYSTEMIC-TO-PULMONARYSHUNTS Евразийский Кардиологический Журнал pulmonary hypertension congenital heart defects the syndrome eisenmenger residual pulmonary hypertension |
| title | CLINICAL FEATURES OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL SYSTEMIC-TO-PULMONARYSHUNTS |
| title_full | CLINICAL FEATURES OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL SYSTEMIC-TO-PULMONARYSHUNTS |
| title_fullStr | CLINICAL FEATURES OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL SYSTEMIC-TO-PULMONARYSHUNTS |
| title_full_unstemmed | CLINICAL FEATURES OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL SYSTEMIC-TO-PULMONARYSHUNTS |
| title_short | CLINICAL FEATURES OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL SYSTEMIC-TO-PULMONARYSHUNTS |
| title_sort | clinical features of pulmonary arterial hypertension associated with congenital systemic to pulmonaryshunts |
| topic | pulmonary hypertension congenital heart defects the syndrome eisenmenger residual pulmonary hypertension |
| url | https://www.heartj.asia/jour/article/view/256 |
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