Pulmonary arterial hypertension associated with congenital heart disease in adults (clinical guidelines)

Bockeriа, L.A.; Gorbachevskiy, S.V.; Шмальц, А.А.

doi:10.24022/0236-2791-2017-59-2-135-147

Cited by 8 publications

(9 citation statements)

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“…Прекапиллярная легочная гипертензия (легочная артериальная гипертензия) у пациентов с бивентрикулярной гемодинамикой -состояние, при котором среднее давление в легочной артерии ≥25 мм рт.ст., давление заклинивания легочной артерии ≤ 15 мм рт.ст., а легочное сосудистое сопротивление >3 ед. Вуда [1][2][3][4][5][6][7][8][9][10][11][12]. Посткапиллярная легочная гипертензия при бивентрикулярной гемодинамикесостояние, при котором среднее давление в легочной артерии ≥25 мм рт.ст., а давление заклинивания легочной артерии >15 мм рт.ст.…”

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“…Посткапиллярная легочная гипертензия при бивентрикулярной гемодинамикесостояние, при котором среднее давление в легочной артерии ≥25 мм рт.ст., а давление заклинивания легочной артерии >15 мм рт.ст. [1][2][3][4][5][6][7][8][9][10][11][12].…”

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“…может иметься как при увеличенном, так и при нормальном легочном сосудистом сопротивлении (рис. 1) [5][6][7][8][9][10][11][12][13]. Во втором случае легочная гипертензия не сопровождается легочной сосудистой болезнью и после коррекции порока нормализуется.…”

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“…Во втором случае легочная гипертензия не сопровождается легочной сосудистой болезнью и после коррекции порока нормализуется. Легочная сосудистая болезнь -состояние, проявляющееся патологическим ремоделированием с уменьшением суммарного внутреннего просвета легочных сосудов [4][5][6][7][8][9][10][11][12][13][14][15][16][17]. Критериями легочной сосудистой болезни при врожденных пороках с бивентрикулярной гемодинамикой служат среднее давление в легочной артерии ≥25 мм рт.ст.…”

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“…2) [15,18,19]. При легочной артериальной гипертензии, ассоциированной с врожденными пороками сердца, в зависимости от наличия и тяжести легочной сосудистой болезни на дефектах может быть артериовенозный, двунаправленный или веноартериальный (синдром Эйзенменгера) сброс [1][2][3][4][5][6][7][8][9][10][11][12][13][15][16][17][18][19]. Отдельную подгруппу составляет «легочная артериальная гипертензия при малых/сопутствующих дефектах», характеризующаяся тяжелой легочной сосудистой болезнью при дефектах малого размера и несущественно увеличенном легочном кровотоке.…”

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Diagnosis of pulmonary hypertension associated with congenital heart disease. Part 1. Definition, classification and initial examination of patients

Горбачевский

Шмальц

2021

Ross. vestn. perinatol. pediatr.

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Having discussed current definitions and classification of pulmonary hypertension associated with congenital heart defects, the authors consider characteristic clinical symptoms and data of physical examination separately for every subgroup. An increase in pulmonary vascular resistance after radical correction and with small / concomitant defects leads to insufficient filling of the systemic ventricle and the progression of symptoms of low cardiac output , i.e. shortness of breath, palpitations, fatigue, weakness and decreased exercise tolerance. At late stages, due to transient systemic hypotension the patients feel dizziness, lightheadedness and fainting, as well as signs of congestive right ventricular failure – peripheral edema, liver enlargement, ascites and swelling of the cervical veins. The patients with Eisenmenger syndrome develop cyanosis and signs of systemic complications – polycythemia, deformation of the distal phalanges of the fingers like «drumsticks» and «watch glasses», posture disturbance due to osteoarthropathy and scoliosis, pulmonary and paradoxical systemic thrombosis and embolism, bleeding, symptoms of gout and cholelithiasis, impaired renal function. Even mild pulmonary hypertension after Fontaine’s surgery causes venous hypertension with congestive heart failure, hydrothorax, protein deficiency enteropathy and plastic bronchitis, as well as insufficient blood flow to the systemic ventricle with low cardiac output, shortness of breath, palpitations, fatigue, weakness and decreased exercise tolerance. All forms of pulmonary hypertension cause pain in the region of the heart, hemoptysis, pulmonary hemorrhage, arrhythmias, and developmental delay in children. The patient shall be examined «from head to toe.» The diagnostic value of auscultation depends on the complexity of the congenital malformation leading to pulmonary hypertension Pulse oximetry should be performed separately on the arms and legs at rest and exercise.

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Diagnosis of pulmonary hypertension associated with congenital heart disease. Part 1. Definition, classification and initial examination of patients

Горбачевский

Шмальц

2021

Ross. vestn. perinatol. pediatr.

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

et al. 2023

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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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Evidence base for specific pulmonary vasodilators in adults with congenital heart disease

Шмальц

Алексеевич²,

Горбачевский

et al. 2021

Terapevticheskii arkhiv

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After reviewing the current definitions and classification of pulmonary hypertension (PH) associated with congenital heart disease (CHD), based on an analysis of 59 clinical trials (of which 14 are randomized controlled trials) drugs registered in the Russian Federation, the evidence base for PH therapy in adults with CHD is provided. The presence of a randomized controlled trial of bosentan BREATHE-5 and uncontrolled trials of other drugs became the basis for a higher class and level of evidence of bosentan (IB) compared to other drugs (IIaC) for Eisenmenger syndrome in the current European (ERS/ESC 2015) and updated Russian (2020) guidelines. According to the updated European (ESC 2020) guidelines for congenital heart disease in adults, in Eisenmenger patients with reduced exercise capacity (6MWT distance 450 m), a treatment strategy with initial endothelin receptor antagonist monotherapy should be considered followed by combination therapy if patients fail to improve (IIaB), in low- and intermediate-risk patients with repaired simple lesions and pre-capillary PH, initial oral combination therapy or sequential combination therapy is recommended and high-risk patients should be treated with initial combination therapy including parenteral prostanoids (IA) and endothelin receptor antagonists and phosphodiesterase 5 inhibitors may be considered in selected patients with elevated pulmonary pressure/resistance in the absence of elevated ventricular end diastolic pressure (IIbC). Only three (bosentan, macitentan and selexipag) out of seven specific pulmonary vasodilators registered in the Russian Federation have indications for pulmonary arterial hypertension associated with congenital heart disease and Eisenmenger syndrome or pulmonary arterial hypertension associated with corrected simple congenital heart disease in the instructions for use.

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Pulmonary arterial hypertension associated with congenital heart disease in adults (clinical guidelines)

Cited by 8 publications

References 0 publications

Diagnosis of pulmonary hypertension associated with congenital heart disease. Part 1. Definition, classification and initial examination of patients

Diagnosis of pulmonary hypertension associated with congenital heart disease. Part 1. Definition, classification and initial examination of patients

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

Evidence base for specific pulmonary vasodilators in adults with congenital heart disease

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