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Двустворчатый аортальный клапан у детей: малая аномалия или серьезный порок сердца?
Шарыкин А.С. Двустворчатый аортальный клапан у детей: малая аномалия или серьезный порок сердца? Consilium Medicum. Педиатрия (Прил.). 2016; 3: 99–102.
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Sharykin A.S. Bicuspid aortic valve at children: small anomaly or serious heart disease? Consilium Medicum. Pediatrics (Suppl.). 2016; 3: 99–102.
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Аннотация
С широким внедрением эхокардиографии в повседневную практику стало возможным диагностировать малосимптомные врожденные пороки сердца, в частности двустворчатый аортальный клапан (ДАК). Анализ литературных и собственных данных позволяет оценивать ДАК как серьезную патологию, которая может приводить к стенозу или недостаточности клапана, а также сопровождается дилатацией корня аорты. Возникающие нарушения гемодинамики, гипертрофия левого желудочка и угроза диссекции аорты требуют диспансерного наблюдения за пациентами, начиная с раннего возраста.
Ключевые слова: двустворчатый аортальный клапан, врожденный порок сердца, эхокардиография, дилатация аорты, стеноз аортального клапана.
Ключевые слова: двустворчатый аортальный клапан, врожденный порок сердца, эхокардиография, дилатация аорты, стеноз аортального клапана.
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With broad introduction of an echocardiography in daily practice has become possible to diagnose not symptomatic congenital heart diseases, in particular, the bicuspid aortic valve (BAV). The analysis of publications and own data allows to estimate BAV as serious pathology which can lead to a stenosis or insufficiency of the valve, and also is followed by aortic root dilatation. The disorders of circulation, a hypertrophy of the left ventricle and threat of a dissection of an aorta demand dispensary observation over patients, since early age.
Key words: bicuspid aortic valve, congenital heart disease, echocardiography, aortic dilatation, stenosis of the aortic valve.
Полный текст
Список литературы
1. Warren AE, Boyd ML, O’Connell C, Dodds L. Dilatation of the ascending aorta in paediatric patients with bicuspid aortic valve: frequency, rate of progression and risc factors. Heart 2006; 92: 1496–500.
2. Prakash SK, Bossé Y, Muehlschlegel JD et al. A roadmap to investigate the genetic basis of bicuspid aortic valve and its complications. Insights from the International BAVCon (Bicuspid Aortic Valve Consortium). J Am Coll Cardiol 2014; 64: 832–9.
3. Hinton RBJr, Lincoln J, Deutsch GH et al. Extracellular matrix remodeling and organization in developing and diseased aortic valves. Circ Res 2006; 98: 1431–8.
4. Fernandez B, Duran AC, Fernandez-Gallego T et al. Bicuspid aortic valves with different spatial orientations of the leaflets are distinct etiological entities. J Am Coll Cardiol 2009;54: 2312–8.
5. Conti CA, Della Corte A, Votta E et al. Biomechanical implications of the congenital bicuspid aortic valve: A finite element study of aortic root function from in vivo data. J Thorac Cardiovasc Surg 2010; 140: 890–6.
6. Beppu S, Suzuki S, Matsuda H et al. Rapidity of progression of aortic stenosis in patients with congenital bicuspid aortic valve. Am J Cardiol 1993; 71: 322–7.
7. Siu SC, Silversides CK. Bicuspid Aortic Valve Disease. J Am Coll Cardiol 2010; 55: 2789–800.
8. Шарыкин А.С., Шилыковская Е.В., Колесникова М.А., Попова Н.Е. Особенности динамики эхокардиографических показателей в ответ на дозированную физическую нагрузку у детей с врожденным аортальным стенозом. Рос. вестн. перинатологии и педиатрии. 2010; 6: 55–61. / Sharykin A.S., Shilykovskaia E.V., Kolesnikova M.A., Popova N.E. Osobennosti dinamiki ekhokardiograficheskikh pokazatelei v otvet na dozirovannuiu fizicheskuiu nagruzku u detei s vrozhdennym aortal'nym stenozom. Ros. vestn. perinatologii i pediatrii. 2010; 6: 55–61. [in Russian]
9. Tadros TM, Klein MD, Shapira OM. Ascending aortic dilatation associated with bicuspid aortic valve. Pathophysiology, molecular biology, and clinical implications. Circulation 2009; 119: 880–90.
10. Kutty S, Kaul S, Danford CJ, Danford DA. Main pulmonary artery dilation in association with congenital bicuspid aortic valve in the absence of pulmonary valve abnormality. Heart 2010; 96:1756–61.
11. Schievink WI, Raissi SS, Maya MM, Velebir A. Screening for intracranial aneurisms in patients with bicuspid aortic valve. Neurology 2010; 74: 1430–3.
12. Шарыкин А.С., Попова Н.Е., Шилыковская Е.В. и др. Дилатация восходящей аорты при двустворчатом аортальном клапане у детей и подростков. Рос. вестн. перинатологии. и педиатрии. 2012; 2: 40–5. / Sharykin A.S., Popova N.E., Shilykovskaia E.V. i dr. Dilatatsiia voskhodiashchei aorty pri dvustvorchatom aortal'nom klapane u detei i podrostkov. Ros. vestn. perinatologii. i pediatrii. 2012; 2: 40–5. [in Russian]
13. Nkomo МЕ, Enriquez-Sarano M, Ammash NM et al. Bicuspid aortic valve associated with aortic dilatation: A community-based study. Arterioscler Thromb Vasc Biol 2003; 23: 351–6.
14. Gurvitz M, Chang RK, Drant S et al. Frequency of aortic root dilation in children with a bicuspid aortic valve. Am J Cardiol 2004; 94: 1337–40.
15. Mahle WT, Frias PA. Bicuspid aortic valve in children: are we overestimating the risk? J Am Coll Cardiol 2010; 55 (10s1): A45.E435. doi: 10.1016/S0735-1097(10)60436-6
16. Basso C, Nistri S, Rizzo S et al. Bicuspid aortic valve, aortic dissection and sudden death in the young: Just a matter of ascending aorta diameters? Circulation 2007; 116: II–463.
17. Januzzi JL, Isselbacher EM, Fattori R. Characterizing the young patient with aortic dissection: results from the international registry of aortic dissection (IRAD). J Am Coll Cardiol 2004; 43: 665–69.
18. Ha H. II, Seo JB, Le SH. Imaging of Marfan syndrome: Multisystemic manifestations. Radio Graphics 2007; 27: 989–1004.
19. Shores J, Berger KR, Murphy EA, Pyeritz RE. Progression of aortic dilatation and the benefit of long-term β-adrenergic blockade in Marphan’s syndrome. N Engl J Med 1994; 330: 1335–41.
20. Detaint D, Aegerter P, Tubach F et al. Rationale and design of a randomized clinical trial (Marfan Sartan) of angiotensin II receptor blocker therapy versus placebo in individuals with Marfan syndrome. Arch Cardiovasc Dis 2010; 103: 317–25.
2. Prakash SK, Bossé Y, Muehlschlegel JD et al. A roadmap to investigate the genetic basis of bicuspid aortic valve and its complications. Insights from the International BAVCon (Bicuspid Aortic Valve Consortium). J Am Coll Cardiol 2014; 64: 832–9.
3. Hinton RBJr, Lincoln J, Deutsch GH et al. Extracellular matrix remodeling and organization in developing and diseased aortic valves. Circ Res 2006; 98: 1431–8.
4. Fernandez B, Duran AC, Fernandez-Gallego T et al. Bicuspid aortic valves with different spatial orientations of the leaflets are distinct etiological entities. J Am Coll Cardiol 2009;54: 2312–8.
5. Conti CA, Della Corte A, Votta E et al. Biomechanical implications of the congenital bicuspid aortic valve: A finite element study of aortic root function from in vivo data. J Thorac Cardiovasc Surg 2010; 140: 890–6.
6. Beppu S, Suzuki S, Matsuda H et al. Rapidity of progression of aortic stenosis in patients with congenital bicuspid aortic valve. Am J Cardiol 1993; 71: 322–7.
7. Siu SC, Silversides CK. Bicuspid Aortic Valve Disease. J Am Coll Cardiol 2010; 55: 2789–800.
8. Шарыкин А.С., Шилыковская Е.В., Колесникова М.А., Попова Н.Е. Особенности динамики эхокардиографических показателей в ответ на дозированную физическую нагрузку у детей с врожденным аортальным стенозом. Рос. вестн. перинатологии и педиатрии. 2010; 6: 55–61. / Sharykin A.S., Shilykovskaia E.V., Kolesnikova M.A., Popova N.E. Osobennosti dinamiki ekhokardiograficheskikh pokazatelei v otvet na dozirovannuiu fizicheskuiu nagruzku u detei s vrozhdennym aortal'nym stenozom. Ros. vestn. perinatologii i pediatrii. 2010; 6: 55–61. [in Russian]
9. Tadros TM, Klein MD, Shapira OM. Ascending aortic dilatation associated with bicuspid aortic valve. Pathophysiology, molecular biology, and clinical implications. Circulation 2009; 119: 880–90.
10. Kutty S, Kaul S, Danford CJ, Danford DA. Main pulmonary artery dilation in association with congenital bicuspid aortic valve in the absence of pulmonary valve abnormality. Heart 2010; 96:1756–61.
11. Schievink WI, Raissi SS, Maya MM, Velebir A. Screening for intracranial aneurisms in patients with bicuspid aortic valve. Neurology 2010; 74: 1430–3.
12. Шарыкин А.С., Попова Н.Е., Шилыковская Е.В. и др. Дилатация восходящей аорты при двустворчатом аортальном клапане у детей и подростков. Рос. вестн. перинатологии. и педиатрии. 2012; 2: 40–5. / Sharykin A.S., Popova N.E., Shilykovskaia E.V. i dr. Dilatatsiia voskhodiashchei aorty pri dvustvorchatom aortal'nom klapane u detei i podrostkov. Ros. vestn. perinatologii. i pediatrii. 2012; 2: 40–5. [in Russian]
13. Nkomo МЕ, Enriquez-Sarano M, Ammash NM et al. Bicuspid aortic valve associated with aortic dilatation: A community-based study. Arterioscler Thromb Vasc Biol 2003; 23: 351–6.
14. Gurvitz M, Chang RK, Drant S et al. Frequency of aortic root dilation in children with a bicuspid aortic valve. Am J Cardiol 2004; 94: 1337–40.
15. Mahle WT, Frias PA. Bicuspid aortic valve in children: are we overestimating the risk? J Am Coll Cardiol 2010; 55 (10s1): A45.E435. doi: 10.1016/S0735-1097(10)60436-6
16. Basso C, Nistri S, Rizzo S et al. Bicuspid aortic valve, aortic dissection and sudden death in the young: Just a matter of ascending aorta diameters? Circulation 2007; 116: II–463.
17. Januzzi JL, Isselbacher EM, Fattori R. Characterizing the young patient with aortic dissection: results from the international registry of aortic dissection (IRAD). J Am Coll Cardiol 2004; 43: 665–69.
18. Ha H. II, Seo JB, Le SH. Imaging of Marfan syndrome: Multisystemic manifestations. Radio Graphics 2007; 27: 989–1004.
19. Shores J, Berger KR, Murphy EA, Pyeritz RE. Progression of aortic dilatation and the benefit of long-term β-adrenergic blockade in Marphan’s syndrome. N Engl J Med 1994; 330: 1335–41.
20. Detaint D, Aegerter P, Tubach F et al. Rationale and design of a randomized clinical trial (Marfan Sartan) of angiotensin II receptor blocker therapy versus placebo in individuals with Marfan syndrome. Arch Cardiovasc Dis 2010; 103: 317–25.
________________________________________________
1. Warren AE, Boyd ML, O’Connell C, Dodds L. Dilatation of the ascending aorta in paediatric patients with bicuspid aortic valve: frequency, rate of progression and risc factors. Heart 2006; 92: 1496–500.
2. Prakash SK, Bossé Y, Muehlschlegel JD et al. A roadmap to investigate the genetic basis of bicuspid aortic valve and its complications. Insights from the International BAVCon (Bicuspid Aortic Valve Consortium). J Am Coll Cardiol 2014; 64: 832–9.
3. Hinton RBJr, Lincoln J, Deutsch GH et al. Extracellular matrix remodeling and organization in developing and diseased aortic valves. Circ Res 2006; 98: 1431–8.
4. Fernandez B, Duran AC, Fernandez-Gallego T et al. Bicuspid aortic valves with different spatial orientations of the leaflets are distinct etiological entities. J Am Coll Cardiol 2009;54: 2312–8.
5. Conti CA, Della Corte A, Votta E et al. Biomechanical implications of the congenital bicuspid aortic valve: A finite element study of aortic root function from in vivo data. J Thorac Cardiovasc Surg 2010; 140: 890–6.
6. Beppu S, Suzuki S, Matsuda H et al. Rapidity of progression of aortic stenosis in patients with congenital bicuspid aortic valve. Am J Cardiol 1993; 71: 322–7.
7. Siu SC, Silversides CK. Bicuspid Aortic Valve Disease. J Am Coll Cardiol 2010; 55: 2789–800.
8. Sharykin A.S., Shilykovskaia E.V., Kolesnikova M.A., Popova N.E. Osobennosti dinamiki ekhokardiograficheskikh pokazatelei v otvet na dozirovannuiu fizicheskuiu nagruzku u detei s vrozhdennym aortal'nym stenozom. Ros. vestn. perinatologii i pediatrii. 2010; 6: 55–61. [in Russian]
9. Tadros TM, Klein MD, Shapira OM. Ascending aortic dilatation associated with bicuspid aortic valve. Pathophysiology, molecular biology, and clinical implications. Circulation 2009; 119: 880–90.
10. Kutty S, Kaul S, Danford CJ, Danford DA. Main pulmonary artery dilation in association with congenital bicuspid aortic valve in the absence of pulmonary valve abnormality. Heart 2010; 96:1756–61.
11. Schievink WI, Raissi SS, Maya MM, Velebir A. Screening for intracranial aneurisms in patients with bicuspid aortic valve. Neurology 2010; 74: 1430–3.
12. Sharykin A.S., Popova N.E., Shilykovskaia E.V. i dr. Dilatatsiia voskhodiashchei aorty pri dvustvorchatom aortal'nom klapane u detei i podrostkov. Ros. vestn. perinatologii. i pediatrii. 2012; 2: 40–5. [in Russian]
13. Nkomo МЕ, Enriquez-Sarano M, Ammash NM et al. Bicuspid aortic valve associated with aortic dilatation: A community-based study. Arterioscler Thromb Vasc Biol 2003; 23: 351–6.
14. Gurvitz M, Chang RK, Drant S et al. Frequency of aortic root dilation in children with a bicuspid aortic valve. Am J Cardiol 2004; 94: 1337–40.
15. Mahle WT, Frias PA. Bicuspid aortic valve in children: are we overestimating the risk? J Am Coll Cardiol 2010; 55 (10s1): A45.E435. doi: 10.1016/S0735-1097(10)60436-6
16. Basso C, Nistri S, Rizzo S et al. Bicuspid aortic valve, aortic dissection and sudden death in the young: Just a matter of ascending aorta diameters? Circulation 2007; 116: II–463.
17. Januzzi JL, Isselbacher EM, Fattori R. Characterizing the young patient with aortic dissection: results from the international registry of aortic dissection (IRAD). J Am Coll Cardiol 2004; 43: 665–69.
18. Ha H. II, Seo JB, Le SH. Imaging of Marfan syndrome: Multisystemic manifestations. Radio Graphics 2007; 27: 989–1004.
19. Shores J, Berger KR, Murphy EA, Pyeritz RE. Progression of aortic dilatation and the benefit of long-term β-adrenergic blockade in Marphan’s syndrome. N Engl J Med 1994; 330: 1335–41.
20. Detaint D, Aegerter P, Tubach F et al. Rationale and design of a randomized clinical trial (Marfan Sartan) of angiotensin II receptor blocker therapy versus placebo in individuals with Marfan syndrome. Arch Cardiovasc Dis 2010; 103: 317–25.
Авторы
А.С.Шарыкин*
ФГБОУ ВО Российский национальный исследовательский медицинский университет им. Н.И.Пирогова Минздрава России. 117997, Россия, Москва, ул. Островитянова, д. 1
*sharykin1947@mail.ru
ФГБОУ ВО Российский национальный исследовательский медицинский университет им. Н.И.Пирогова Минздрава России. 117997, Россия, Москва, ул. Островитянова, д. 1
*sharykin1947@mail.ru
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A.S.Sharykin*
N.I.Pirogov Russian National Research Medical University of the Ministry of Health of the Russian Federation. 117997, Russian Federation, Moscow, ul. Ostrovitianova, d. 1
*sharykin1947@mail.ru
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