İnfantil Scimitar Sendromu: Tek Merkez Deneyiminden Klinik Özellikler, Girişimsel Stratejiler ve Kısa Dönem Sonuçlar

Abstract

Özet Amaç: İnfantil Skimitar sendromu, anormal pulmoner venöz drenaj, sağ akciğer hipoplazisi ve sıklıkla pulmoner hipertansiyon ile kalp yetersizliği birlikteliği ile karakterize nadir ve ağır bir konjenital anomalidir. Nadir görülmesi nedeniyle, özellikle semptomatik yenidoğan ve infantlarda standart tedavi stratejileri halen tartışmalıdır. Yöntemler: Bu retrospektif, tek merkezli çalışmaya Mart 2015 ile Aralık 2023 tarihleri arasında infantil Skimitar sendromu (<2 yaş) tanısı alan hastalar dahil edildi. Tanı ekokardiyografi ve kontrastlı bilgisayarlı tomografi ve/veya kateter anjiyografi ile doğrulandı. Klinik başvuru özellikleri, eşlik eden anomaliler, uygulanan tedavi stratejileri ve kısa dönem sonuçlar analiz edildi. Pulmoner hipertansiyon, kardiyak kateterizasyon sırasında ölçülen ortalama pulmoner arter basıncının ≥25 mmHg olması olarak tanımlandı. Bulgular: Toplam beş hasta çalışmaya dahil edildi (tanı anındaki ortalama yaş: 103,4 ± 89,57 gün; ortalama doğum ağırlığı: 4606 ± 1314,94 g). Tüm hastalarda sağ akciğer hipoplazisi mevcuttu ve %40’ında aortopulmoner kollateral arterler saptandı. Başvuru semptomları arasında kalp yetersizliği, pulmoner hipertansif kriz, solunum sıkıntısı, siyanoz ve gelişme geriliği yer aldı. Bir hasta (%20) girişim öncesinde yenidoğan döneminde kaybedildi. İki hastaya transkateter girişim uygulandı; bunlardan birinde 8 mm vasküler plug kullanılarak sekestrasyon arteri başarıyla oklüde edildi. Tedavi edilen hastalarda ortalama 12 aylık takip süresince pulmoner hipertansiyonda gerileme ve klinik iyileşme gözlendi. Klinik olarak stabil hastalarda cerrahi onarım ertelendi. Sonuç: Erken yaşam döneminde ortaya çıkan infantil Skimitar sendromu yüksek morbidite ve mortalite riski taşımaktadır. Sağ akciğer hipoplazisinin derecesi, pulmoner hipertansiyonun varlığı ve eşlik eden anomaliler prognozu belirleyen temel faktörlerdir. Transkateter girişimler, yüksek riskli infantlarda klinik stabilizasyonun sağlanmasında ve definitif cerrahi düzeltmenin zamanlamasının optimize edilmesinde önemli bir rol oynayabilir.

Keywords

• Scimitar sendromu
• Pulmoner ven anomalisi
• Sağ akciğer hipoplazisi
• Pulmoner hipertansiyon

Infantile Scimitar Syndrome: Clinical Characteristics, Interventional Strategies, and Short-Term Outcomes from a Single-Center Experience

Abstract

Abstract Background: Infantile Scimitar syndrome is a rare and severe congenital anomaly characterized by anomalous pulmonary venous drainage, right lung hypoplasia, and frequent association with pulmonary hypertension and heart failure. Due to its rarity, standardized management strategies remain controversial, particularly in symptomatic neonates and infants. Methods:This retrospective single-center study included patients diagnosed with infantile Scimitar syndrome (<2 years of age) between March 2015 and December 2023. Diagnosis was confirmed by echocardiography and contrast-enhanced computed tomography and/or catheter angiography. Clinical presentation, associated anomalies, management strategies, and short-term outcomes were analyzed. Pulmonary hypertension was defined as a mean pulmonary artery pressure ≥25 mmHg measured during cardiac catheterization. Results:Five patients were included (mean age at diagnosis: 103.4 ± 89.57 days; mean birth weight: 4606 ± 1314.94 g). All patients had right lung hypoplasia, and 40% had aortopulmonary collateral arteries. Presenting symptoms included heart failure, pulmonary hypertensive crisis, respiratory distress, cyanosis, and failure to thrive. One patient (20%) died in the neonatal period before intervention. Two patients underwent transcatheter procedures, including successful occlusion of a sequestration artery using an 8 mm vascular plug. Regression of pulmonary hypertension and clinical improvement were observed in treated patients during a mean follow-up of 12 months. Surgical repair was deferred in clinically stable patients. Conclusions:Infantile Scimitar syndrome presenting in early life carries substantial morbidity and mortality. The severity of right lung hypoplasia, pulmonary hypertension, and associated anomalies are major determinants of outcome. Transcatheter interventions may play a crucial role in stabilizing high-risk infants and optimizing the timing of definitive surgical correction.

Keywords

• Scimitar syndrome
• Pulmonary vein anomaly
• Right lung hypoplasia
• Pulmonary hypertension

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