Surgical Treatment of Congenital Heart Diseases Associated with Pulmonary Hypertension in Adults

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Abstract

The outcome of treatment of adult patients with congenital heart disease (CHD) and pulmonary hypertension (PH) depends on the adequacy of diagnosis with a thorough assessment of hemodynamics and the correctness of the chosen tactics. Enormous success has been achieved, various classifications and algorithms for managing children and adults with this nosology have been created and are widely used. Thanks to the creation and widespread introduction into clinical practice of various types of drugs, the results of surgical treatment of CHD with PH have improved significantly. A new cluster of patients has emerged who can undergo palliative surgery against the background of irreversible pulmonary hypertension, leading to a significant improvement in the quality and life expectancy. At the same time, to this day, it is far from always possible to evaluate each specific case with full confidence in the possibility of performing surgical treatment. Modern advances in CHD cardiac surgery have significantly expanded the indications for the correction of defects with high PH in adults. Operability (implying normalization of pulmonary artery pressure) and indications for palliative surgery (without regression of PH, but with an improvement in the quality and life expectancy of patients) are still the main debatable issues.

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About the authors

Sergey V. Gorbachevsky

A.N. Bakulev National Medical Research Center for Cardiovascular Surgery; Russian State Medical Postgraduate Academy

Author for correspondence.
Email: svgorby59@yandex.ru
ORCID iD: 0000-0002-4193-3320
SPIN-code: 5681-2811

MD, PhD, Professor

Russian Federation, Moscow; Moscow

Irina E. Chazova

National Medical Research Center of Cardiology

Email: chazova@hotmail.com
ORCID iD: 0000-0002-9822-4357
SPIN-code: 6175-6138

MD, PhD, Professor, Academician of the RAS

Russian Federation, Moscow

Tamila V. Martynyuk

National Medical Research Center of Cardiology

Email: trukhiniv@mail.ru
ORCID iD: 0000-0002-9022-8097
SPIN-code: 5665-3903

MD, PhD

Russian Federation, Moscow

Anton A. Shmalts

A.N. Bakulev National Medical Research Center for Cardiovascular Surgery; Russian State Medical Postgraduate Academy

Email: shmaltzanton@inbox.ru
ORCID iD: 0000-0001-8937-1796
SPIN-code: 1054-2150

MD, PhD

Russian Federation, Moscow; Moscow

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Supplementary files

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1. Fig. 1. Algorithm for the surgical treatment of adults with congenital heart disease and pulmonary arterial hypertension. A.N. Bakuleva, 2016 [37]

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2. Fig. 2. Stenting of the trunk of the left coronary artery when it is compressed by the trunk of the pulmonary artery [46]: A — pronounced compression of the trunk of the left coronary artery; B — effective stenting of the trunk of the left coronary artery

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3. Fig. 3. Algorithm for the diagnosis and treatment of congenital heart disease with pulmonary arterial hypertension [1] Note. FK - functional class; PDE-5 - inhibitors of phosphodiesterase type 5; O2 is oxygen.

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