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Двустворчатый аортальный клапан у детей: малая аномалия или серьезный порок сердца?
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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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.
Key words: bicuspid aortic valve, congenital heart disease, echocardiography, aortic dilatation, stenosis of the aortic valve.
Ключевые слова: двустворчатый аортальный клапан, врожденный порок сердца, эхокардиография, дилатация аорты, стеноз аортального клапана.
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Key words: bicuspid aortic valve, congenital heart disease, echocardiography, aortic dilatation, stenosis of the aortic valve.
Полный текст
Список литературы
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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. 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.
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. 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
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
ФГБОУ ВО Российский национальный исследовательский медицинский университет им. Н.И.Пирогова Минздрава России. 117997, Россия, Москва, ул. Островитянова, д. 1
*sharykin1947@mail.ru
________________________________________________
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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