Serra BAŞKAN, Pelin KARACA ÖZER, Kazım ÖZTARHAN

KONJENİTAL İZOLE HAFİF AORT KAPAK VE HAFİF PULMONER KAPAK DARLIĞININ DOĞAL SEYRİ: TEK MERKEZLİ BİR TAKİP ÇALIŞMASI

Amaç: Hafif aort darlığı (AD) ve hafif pulmoner darlığın (PD) doğal seyri hakkındaki bilgilerin birçoğu geçmiş 35-40 yıllık kardiak kateterizasyon kullanılarak yapılmış olan çalışmalara dayandırılmaktadır. Bu çalışmanın amacı, çocuk hastalarda izole hafif valvüler AD ve izole hafif valvüler PD’nin doğal seyrini, seri Doppler ekokardiyografik ölçümler ile ortaya koymaktı. Gereç ve Yöntem: Bu çalışmaya hafif AD tespit edilen 50 ve hafif PD tespit edilen 75, toplam 125 çocuk dahil edildi. Hafif AD ve PD’nin klinik seyirleri cinsiyet, ilk tanı anındaki yaş ve valvüler gradiyente göre karşılaştırıldı. Bulgular: Tanı yaşı ortalaması 26,1±37,6 ay (1 gün-13 yaş) idi. Çocukların ortalama izlem süresi 27,65±21,60 (1-120) aydı. PD grubunun final gradiyentlerinde başlangıca göre belirgin azalma saptandı (19,88±11,21’e karşı 23,58±6,97 mmHg, p=0,001). AD grubunda bir yaş altı hastalarda daha belirgin olmak üzere tüm hastaların final gradiyentlerinde artış saptandı (27,74±14,12’ye karşı 22,42±6,12 mmHg, p=0,002). AD grubunda final gradiyentin ortalaması, PD grubuna göre daha yüksekti (27,74±14,12’ye karşı 19,88±11,21 mmHg, p=0,001). PD grubunda toplamda hepsi erkek ve ≤1 yaş olan %4 hastada orta-ileri darlığa progresyon görüldü. AD grubunun %12’sinde orta-ileri darlığa progresyon görüldü. Hafif AD olanlardan iki hastaya, hafif PD olanlardan ise bir hastaya girişim yapıldı. Sonuç: Çalışmamızda hafif PD’nin hafif AD’ye göre seyrinin daha iyi olduğu ve AD’nin progresyon gösterme riskinin daha yüksek olduğu gösterildi. Bir yaş ve altındaki, özellikle erkek hafif PD olgularında sık olmasa da progresyon saptanabileceğinden dikkatli takip yapılmalıdır. Hafif AD ise, hastalık her yaş grubunda progresif özellik gösterebileceğinden yakın takip edilmelidir.

Anahtar Kelimeler:

Konjenital, aort darlığı, pulmoner darlık, doğal seyir, pediatri, ekokardiyografi

NATURAL HISTORY OF CONGENITAL ISOLATED MILD AORTIC VALVE AND MILD PULMONARY VALVE STENOSIS: A SINGLECENTER FOLLOW-UP STUDY

Objective: Most of the available information on the natural history of aortic stenosis (AS) and pulmonary stenosis (PS) in children is based on studies carried out over the past 35-40 years using cardiac catheterization. This study aimed to reveal the natural history of congenital isolated mild valvular AS and PS in children using serial Doppler echocardiographic examinations. Materials and Methods: A total of 125 children, 50 with mild AS and 75 with mild PS, who underwent Doppler echocardiography were included in this study. The prognoses of mild AS and PS were compared according to age, gender, and valvular gradient at the time of initial diagnosis. Results: The mean age of patients was 26.1±37.6 months at diagnosis. The mean follow-up duration was 27.65±21.60 (1- 120) months. There was a significant decrease in the final gradient of the PS group compared to the baseline (23.58±6.97 vs. 19.88±11.21 mmHg, p=0.001). In the AS group, there was an increase in the final gradient, which was more pronounced in patients ≤1-year-old (22.42±6.12 vs. 27.74±14.12 mmHg, p=0.002). Four percent of patients in the PS group and 12% of patients in the AS group progressed to moderate to severe stenosis. All patients who progressed in the PS group were ≤1-year-old and male. Conclusion: The results showed that mild PS had a better prognosis than mild AS and that the risk of progression in AS was higher. Careful follow-up should be performed in mild PS cases ≤1-year-old, especially in boys, since progression may be detected, even if infrequently. Mild AS should also be followed closely, as the disease may show progressive characteristics in all age groups.

Keywords:

Congenital, aortic stenosis, pulmonary stenosis, natural history, pediatrics, echocardiography,

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