Koarktacija aorte kod djece u 10-godišnjoj kliničkoj epidemiološkoj studiji: dijagnostičko i terapijsko razmatranje [Coarctation of the aorta in children in the 10-year epidemiological study: diagnostic and therapeutic consideration]

Malčić, Ivan and Kniewald, Hrvoje and Jelić, Ana and Šarić, Dalibor and Bartoniček, Dorotea and Dilber, Daniel and Belina, Dražen and Anić, Darko (2015) Koarktacija aorte kod djece u 10-godišnjoj kliničkoj epidemiološkoj studiji: dijagnostičko i terapijsko razmatranje [Coarctation of the aorta in children in the 10-year epidemiological study: diagnostic and therapeutic consideration]. Liječnički vjesnik, 137 (1-2). pp. 9-17. ISSN 0024-3477

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Abstract

Our study is a clinical epidemiological retrospective analysis of coarctation of the aorta in a 10-year follow-up (2001-2011). The study includes 201 children, 72 (35.82%) girls and 129 (64.18%) boys (1:1,6), with an average age of 28.57-49.37 mo (0.1-204 mo). They are categorized in 4 age groups: <1 month, 1 month - 1 year, 1 year - 6 years, >6 years. Isolated coarctation of the aorta was found in 125 (62.19%) patients; 33 (16.42%) preductal and 92 (45.77%) postductal. Coarctation of the aorta with an additional heart defect was found in 76 (37.81%) patients; 32 (15.32%) with ventricular septal defect, 28 (13.93%) within a complex heart defect, 11 (5.47%) within the Shone syndrome, and 5 (2.49%) with a dilated cardiomyopathy. Tiredness, intense tachypneic and dyspneic difficulties are dominant features in lower age groups (newborns and infants), while claudications, headaches and epistaxis are typical in older children. In the case of as many as 61 (30.35%) patients diagnosis was missed on the previous cardiological examination. In 20 (9.95%) patients coarctation is found within the known syndromes (Turner, Noonan, Williams Beuren, Ellis van Creveld, Down, partial trisomy 18, fetal valproate syndrome). Echocardiography was performed in all patients, and in 45 (22.38%) it was the only diagnos tic procedure. Altogether, 123 heart catheterizations, 38 multislice computed tomography and 15 magnetic resonance imagings were performed. The gradient on the place of coarctation before surgery or emergency procedures measured by catheter in 132 (65.67%) patients was 57.99 +/- 18.68 mmHg (20-100 mmHg). In 82 (40.80%) patients a bicuspid aortic valve was found. Average age at the time of surgery was 27.92 +/- 47.98 months (0.1-204 mo.). In 169 (84.07%) patients a cardiosurgical intervention was performed; 109 (54.23%) T-T anastomoses, 30 (14.29%) therapeutic catheterisations (balloon dilatation or stent implantation). Fatal outcome occurred in 4 (1.99%) children, all newborns or infants.

Abstract in Croatian

Naša je studija klinička epidemiološka retrospektivna analiza koarktacije aorte u vremenskom slijedu od 10 godina (2001. – 2011.). U studiju je uključeno 201 dijete, 72 (35,82%) djevojčice i 129 (64,18%) dječaka (1 : 1,6) prosječne dobi kod postavljanja dijagnoze 28,57 ± 49,37 mjeseci (0,1 – 204). Razvrstani su u 4 dobne skupine: novorođenačka dob (< 1 mjeseca), 1 mjesec – 1 godine, 1 – 6 godina i > 6 godina. Izoliranu koarktaciju imalo je 125 (62,19%) bolesnika, od čega 33 (16,42%) preduktalnu i 92 (45,77%) postduktalnu, a njih 76 (37,81%) imalo je koarktaciju s dodatnom srčanom grješkom; s ventrikularnim septalnim defektom 32 (15,92%), u okviru kompleksne srčane grješke 28 (13,93%), u sklopu Shoneova kompleksa 11 (5,47%) i s dilatacijskom kardiomiopatijom 5 (2,49%). U mlađim dobnim skupinama (novorođenačka i dojenačka dob) prevladava zamaranje, pojačano znojenje i dispnoičke tegobe, a kod starije djece klaudikacije, glavobolja i epistaksa. Kod čak 61 (30,35%) ispitanika dogodio se previd dijagnoze na prethodnome kardiološkom pregledu. Kod 20 (9,95%) bolesnika koarktacija se nalazi u okviru poznatih sindromnih bolesti (Turner, Noonan, Williams-Beuren, Ellis-van Creveld, parcijalna trisomija 18, Downov sindrom, fetalni valproatni sindrom). Ehokardiografija je učinjena u svih bolesnika, a kod 45 (22,38%) bila je i jedina slikovna dijagnostička pretraga. Učinjene su ukupno 123 kateterizacije srca, 38 kompjutoriziranih tomografija i 15 magnetskih rezonancija. Gradijent na mjestu koarktacije mjeren prije operacije ili interventnog liječenja izmjeren na kateterizaciji srca kod 132 (65,67%) bolesnika iznosio je 57,99 ± 18,68 mmHg (20 – 100 mmHg). Kod 82 (40,80%) bolesnika nađena je bikuspidalna aortalna valvula. Prosječna dob kod operacije bila je 27,92 ± 47,98 mjeseci (0,1 – 204). Kod 169 (84,07%) izvršena je kardiokirurška korekcija, od toga T-T-anastomoza kod 109 (54,23%), a kod 30 (14,92%) učinjena je terapijska kateterizacija (balonska dilatacija ili implantacija stenta). Smrtni ishod zabilježen je u četvero djece (1,99%), svi u novorođenačkoj ili dojenačkoj dobi.

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