Aortic Root Dilatation in Children and Adolescents At Al-Hawary General Hospital, & National Benghazi Cardiac Center -Libya

• Rasmia H. Feituri

  Department of Pediatric, Faculty of Medicine, Benghazi University, Benghazi cardiac center, Libya

  Author

• Hanan El Megasbi

  Department of Pediatric, Faculty of Medicine, Benghazi University, Benghazi cardiac center, Libya

  Author

• Mariam M. El maadani

  Department of Pediatric, Faculty of Medicine, Benghazi University, Benghazi cardiac center, Libya

  Author

• Amal Khazm

  Department of Pediatric, Faculty of Medicine, Benghazi University, Benghazi cardiac center, Libya

  Author

Isolated dilatation of the aortic root and/or ascending aorta is a rare but well-known cardiovascular manifestation, can be caused by a variety of congenital or acquired conditions; that lead to the weakening of the aortic wall. The study aimed to detect the cause and the rate of the aortic root dilatation in children and adolescents, and to assess the effect of the Beta-adrenergic blockers in preventing further dilatation in the aortic root. A case series study was perform with five years of follow-up at Al-Hawary General Hospital, National Benghazi Cardiac Center. A total of 91 patients were seen with ascending aortic dilatation and/or root dilatation during the period from 6/2016 - 6/2021 included in the study diagnosed by clinical examination, chest x-ray, and echocardiogram. The diagnosis in 34/91(37%) was Tetralogy of fallout (TOF) and truncus arteriosus, 57/91 (63%) was dilated aortic root, 25/57 (44%) bicuspid aortic valve (BAV), 22/57 (38.5%) Marfan syndrome, 4/57(7%) Noonan syndrome, 2/57(3.5%) Turner syndrome, 3/57(5%) Ehlers-Danlos syndrome, 1/57(2%) idiopathic. Follow-up results of three months – five years: 57/91 patients with aortic root dilatation were followed up, none of the Marfan syndrome and Ehlers-Danlos syndrome patients who received beta-blockers had shown progression in the dilatation of the aortic root, and all patients who had bicuspid aortic valve did not show any progression in the dilatation without using medication. Conclusions: Dilated aortic root is a common finding in Marfan syndrome, bicuspid aortic root, and Ehlers-Danlos syndrome, and its progress could be decreased by using beta-adrenergic blockers in rapidly progressing dilation.

Birks, E. J., Webb, C., Child, A., Radley-Smith, R., & Yacoub, M. H. (1999). Early and long-term results of a valve-sparing operation for Marfan syndrome. Circulation, 100(suppl_2), II-29-Ii-35.

Blais, S., Meloche-Dumas, L., Fournier, A., Dallaire, F., & Dahdah, N. (2020). Long-term risk factors for dilatation of the proximal aorta in a large cohort of children with bicuspid aortic valve. Circulation: Cardiovascular Imaging, 13(3), e009675.

Bonderman, D., Gharehbaghi-Schnell, E., Wollenek, G., Maurer, G., Baumgartner, H., & Lang, I. M. (1999). Mechanisms underlying aortic dilatation in congenital aortic valve malformation. Circulation, 99(16), 2138-2143.

Boudoulas, K. D., Pitsis, A. A., Mazzaferri, E. L., Gumina, R. J., Triposkiadis, F., & Boudoulas, H. (2020). Floppy mitral valve/mitral valve prolapse: a complex entity with multiple genotypes and phenotypes. Progress in cardiovascular diseases.

Cameron, D., Dietz, H., Greene, P., Gillinov, A., Pyeritz, R., Alejo, D., Fleischer, K., Anhalt, G., Stone, C., & McKusick, V. (1996). The Marfan syndrome and the cardiovascular surgeon. Eur J Cardlo-thorac Surg, 10, 149-158.

Coady, M. A., Rizzo, J. A., Hammond, G. L., Mandapati, D., Darr, U., Kopf, G. S., & Elefteriades, J. A. (1997). What is the appropriate size criterion for resection of thoracic aortic aneurysms? The Journal of thoracic and cardiovascular surgery, 113(3), 476-491.

David, T. E., Feindel, C. M., & Bos, J. (1995). Repair of the aortic valve in patients with aortic insufficiency and aortic root aneurysm. The Journal of thoracic and cardiovascular surgery, 109(2), 345-352.

Gao, L., Mao, Q., Wen, D., Zhang, L., Zhou, X., & Hui, R. (2011). The effect of beta‐blocker therapy on progressive aortic dilatation in children and adolescents with Marfan’s syndrome: a meta‐analysis. Acta Paediatrica, 100(9), e101-e105.

Gidding, S. S. (2011). The aortic root in Ehlers-Danlos syndrome. The Journal of pediatrics, 158(5), A3.

Gott, V. L., Greene, P. S., Alejo, D. E., Cameron, D. E., Naftel, D. C., Miller, D. C., Gillinov, A. M., Laschinger, J. C., Borst, H. G., & Cabrol, C. E. (1999). Replacement of the aortic root in patients with Marfan's syndrome. New England Journal of Medicine, 340(17), 1307-1313.

Harringer, W., Pethig, K., Hagl, C., Meyer, G. P., & Haverich, A. (1999). Ascending aortic replacement with aortic valve reimplantation. Circulation, 100(suppl_2), II-24-Ii-28.

Knadler, J. J., LeMaire, S., McKenzie, E. D., Moffett, B., & Morris, S. A. (2019). Thoracic aortic, aortic valve, and mitral valve surgery in pediatric and young adult patients with marfan syndrome: characteristics and outcomes. Seminars in thoracic and cardiovascular surgery,

Kouchoukos, N. T., & Dougenis, D. (1997). Surgery of the thoracic aorta. New England Journal of Medicine, 336(26), 1876-1889.

Lin, A. E., Lippe, B., & Rosenfeld, R. G. (1998). Further delineation of aortic dilation, dissection, and rupture in patients with Turner syndrome. Pediatrics, 102(1), e12-e12.

Massih, T. A., Vouhé, P., Mauriat, P., Mousseaux, E., Sidi, D., & Bonnet, D. (2002). Replacement of the ascending aorta in children: a series of fourteen patients. The Journal of thoracic and cardiovascular surgery, 124(2), 411-413.

Miller, D. C. (2003). Valve-sparing aortic root replacement in patients with the Marfan syndrome. The Journal of thoracic and cardiovascular surgery, 125(4), 773-778.

Pfammatter, J.-P., Pavlovic, M., Berdat, P., & Carrel, T. (2001). Dilation of the ascending aorta in childhood: 4 cases without obvious predisposing disease. Cardiology in the young, 11(2), 169-172.

Roman, M. J., Devereux, R. B., Niles, N. W., Hochreiter, C., Kligfield, P., Sato, N., Spitzer, M. C., & Borer, J. S. (1987). Aortic root dilatation as a cause of isolated, severe aortic regurgitation: prevalence, clinical and echocardiographic patterns, and relation to left ventricular hypertrophy and function. Annals of internal medicine, 106(6), 800-807.

Rybczynski, M., Treede, H., Sheikhzadeh, S., Groene, E. F., Bernhardt, A. M., Hillebrand, M., Mir, T. S., Kühne, K., Koschyk, D., & Robinson, P. N. (2011). Predictors of outcome of mitral valve prolapse in patients with the Marfan syndrome. The American journal of cardiology, 107(2), 268-274.

Shores, J., Berger, K. R., Murphy, E. A., & Pyeritz, R. E. (1994). Progression of aortic dilatation and the benefit of long-term β-adrenergic blockade in Marfan's syndrome. New England Journal of Medicine, 330(19), 1335-1341.

Tierney, E. S. S., Feingold, B., Printz, B. F., Park, S. C., Graham, D., Kleinman, C. S., Mahnke, C. B., Timchak, D. M., Neches, W. H., & Gersony, W. M. (2007). Beta-blocker therapy does not alter the rate of aortic root dilation in pediatric patients with Marfan syndrome. The Journal of pediatrics, 150(1), 77-82.

Wenstrup, R., Lyle, J., Rose, P., Levy, H., Hoechstetter, L., Meyer, R., & Francomano, C. (2000). Aortic root dilatation complicates Ehlers-Danlos syndrome. Genetics in Medicine, 2(1), 65-65.

Wenstrup, R. J., Meyer, R. A., Lyle, J. S., Hoechstetter, L., Rose, P. S., Levy, H. P., & Francomano, C. A. (2002). Prevalence of aortic root dilation in the Ehlers-Danlos syndrome. Genetics in Medicine, 4(3), 112-117.

Yacoub, M. H., Gehle, P., Chandrasekaran, V., Birks, E. J., Child, A., & Radley-Smith, R. (1998). Late results of a valve-preserving operation in patients with aneurysms of the ascending aorta and root. The Journal of thoracic and cardiovascular surgery, 115(5), 1080-1090.

• PDF

Copyright (c) 2021 Rasmia H. Feituri, Hanan El Megasbi, Mariam M. El maadani , Amal Khazm

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

Copyright of the articles Published by Almukhtar Journal of Science (MJSc) is retained by the author(s), who grant MJSc a license to publish the article. Authors also grant any third party the right to use the article freely as long as its integrity is maintained and its original authors and cite MJSc as the original publisher. Also, they accept the article remains published by the MJSc website (except in the occasion of a retraction of the article).