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Pathological physiology of complications of Eisenmenger syndrome

Authors: Shmalts A.A.1,2, 2, Kupryashov A.A.1, Chernogrivov I.E.1

Company: 1 Bakoulev National Medical Research Center for Cardiovascular Surgery, Moscow, Russian Federation
2 Russian State Medical Postgraduate Academy, Moscow, Russian Federation

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Type:  Reviews


DOI: https://doi.org/10.24022/1997-3187-2024-18-1-7-19

For citation: Shmalts A.A., Kupryashov A.A., Chernogrivov I.E. Pathological physiology of complications of Eisenmenger syndrome. Creative Cardiology. 2024; 18 (1): 7–19 (in Russ.). DOI: 10.24022/1997-3187-2024-18-1-7-19

Received / Accepted:  29.01.2024 / 12.02.2024

Keywords: Eisenmenger syndrome congenital heart defects pathological physiology



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Abstract

Eisenmenger syndrome (ES) is systemic pulmonary hypertension with venoarterial or bidirectional shunting of blood between the ventricles, atria or great arteries due to the progression of pulmonary vascular disease in the natural course of “simple” and “complex” congenital heart defects. Central cyanosis and chronic hypoxemia lead to damage to many organs and systems – secondary erythrocytosis (often with iron deficiency), blood clotting disorders with thrombosis and bleeding, paradoxical embolism, renal dysfunction, gout, cholelithiasis and fibrotic changes in the liver, diffuse brain damage, hypertrophic osteoarthropathy with scoliosis and deformation of the distal phalanges of the fingers, increasing the risk of arrhythmias, infectious and other complications. The review focuses on pathological physiology, including cardiac function, and prognosis in patients with ES.

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About Authors

  • Anton A. Shmalts, Dr. Med. Sci., Leading Researcher, Associate Professor at Department of Cardiovascular Surgery; ORCID
  • Aleksey A. Kupryashov, Dr. Med. Sci., Head of Department; ORCID
  • Igor E. Chernogrivov, Dr. Med. Sci., Head of Department; ORCID

Chief Editor

Leo A. Bockeria, MD, PhD, DSc, Professor, Academician of Russian Academy of Sciences, President of Bakoulev National Medical Research Center for Cardiovascular Surgery


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