THE COMPLEXITY OF DIAGNOSING IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION
https://doi.org/10.21508/1027-4065-2018-63-5-167-171
Abstract
Idiopathic pulmonary arterial hypertension is a progressive, incurable disease of unclear etiology; it is characterized by intensive remodeling of the pulmonary vascular bed with a predominant lesion of the distal pulmonary arteries and arterioles. A diagnostic criterion of the disease is an increase in the average pressure in the pulmonary artery more than 25 mm Hg. Arterial hypertension is difficult to diagnose, especially at the early stages due to non-specific clinical symptoms. The article describes the clinical case of diagnostics and treatment of a child with primary pulmonary hypertension of high degree.
About the Authors
D. I. Sadykova
Kazan State Medical Academy – branch of the Russian Medical Academy of Post-Graduate Education.
Russian Federation
Russian Federation
D. R. Sabirova
Kazan State Medical Academy – branch of the Russian Medical Academy of Post-Graduate Education.
Russian Federation
Russian Federation
N. V. Kustova
Kazan State Medical Academy – branch of the Russian Medical Academy of Post-Graduate Education.
Russian Federation
Russian Federation
N. N. Firsova
Children’s Republican Clinical Hospital of Tatarstan Republic.
Russian Federation
Russian Federation
G. A. Khusnullina
Children’s Republican Clinical Hospital of Tatarstan Republic.
Russian Federation
Russian Federation
References
1. Fraisse A., Jais X., Schleich J.M., di Filippo S., Maragnès P., Beghetti M. et al. Characteristics and prospective 2-year follow-up of children with pulmonary arterial hypertension in France. Arch Cardiovasc Dis 2010; 103(2): 66–74. DOI: 10.1016/j.acvd.2009.12.001
2. Galiè N., Hoeper M.M., Humbert M., Torbicki A., Vachiery J.L., Barbera J.A. et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society of Cardiology(ESC), and the European Respiratory Society(ERS), endorsed by International Society of Heart and Lung Transplantation(ISHLT). Eur Heart J2009; 30(20): 2493–537. DOI: 10.1093/eurheartj/ehp297
3. Humbert M., Sitbon O., Chaouat A., Bertocchi M., Habib G., Gressin V. et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 2010; 122(2): 156–163. DOI: 10.1161/CIRCULATIONAHA.109.911818
4. Ivy D. Pulmonary Hypertension in Children. Cardiol Clin 2016; 34(3): 451–472. DOI: 10.1016/j.ccl.2016.04.005
5. D’Alonzo G.E., Barst R.J., Ayres S.M., Bergofsky E.H., Brundage B.H., Detre K.M. et al. Survival in patients with primary pulmonary hypertension(Results from a national prospective registry). Ann Intern Med1991; 115(5): 343–349.
6. Moledina S., Hislop A.A., Foster H., Schulze-Neick I., Haworth S.G. Childhood idiopathic pulmonary arterial hypertension: a national cohort study. Heart2010; 96(17): 1401–1406. DOI: 10.1136/hrt.2009.182378
7. Barst R.J., McGoon M.D., Elliott C.G., Foreman A.J., Miller D.P., Ivy D.D. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation 2012; 125(1): 113–122. DOI: 10.1161/CIRCULATIONAHA.111.026591
8. van Loon R.L., Roofthooft M.T., Delhaas T., van OschGevers M., ten Harkel A.D., Strengers J.L. et al. Outcome of pediatric patients with pulmonary arterial hypertension in the era of new medical therapies. Am J Cardiol 2010; 106(1): 117–124. DOI: 10.1016/j.amjcard.2010.02.023
9. Hansmann G., Hoeper M.M. Registries for paediatric pulmonary hypertension. Eur Respir J2013; 42(3): 580–583. DOI: 10.1183/09031936.00065713
10. Pulmonary hypertension. I.E. Chazova, T.V. Martynyuk(eds). M: Praktika2015; 928. (in Russ)
11. Trisvetova E.L. Diagnosis and treatment of pulmonary hypertension. Meditsinskie novosti2009; 15; 13–18.
12. Janda S., Shahidi N., Gin K. Diagnostic accuracy of echocardiography for pulmonary hypertension: a systematic review and meta-analysis. Heart 2011; 97(8): 612–622. DOI: 10.1136/hrt.2010.212084
Review
For citations:
Sadykova D.I., Sabirova D.R., Kustova N.V., Firsova N.N., Khusnullina G.A. THE COMPLEXITY OF DIAGNOSING IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2018;63(5):167-171. (In Russ.) https://doi.org/10.21508/1027-4065-2018-63-5-167-171
Views:
811
Email this article 