Fallot tetralojisi ve onarım sonrası komplikasyonlar

Özet

Siyanozlu çocukta Fallot tetralojisi, malalignment VSD, aortanın septum üzerine ata binmesi, sağ ventrikül çıkış yolu obstrüksiyonu ve sağ ventrikül hipertrofisiyle tanınan konotrunkal bir anomalidir. BT anjiyografi, ekokardiyografinin sınırlı kaldığı pulmoner arter dalları, aortopulmoner kollateraller, koroner seyir ve onarım materyalinin anatomik haritasını sunar. Onarılmış hastada RV-PA konduiti, transannüler yama, pulmoner arter darlığı ve yama psödoanevrizması geç dönemde yeniden değerlendirme gerektirebilir. BT anatomiyi gösterir ancak pulmoner kapak yetersizliği ve ventrikül işlevini tek başına nicelleştirmez; yapısal bulguyu işlevsel sonuçla eşitlemek doğru değildir.

Faz ve pencere

BTA tanısal

Kardiyak hareket azaltılmış kontrastlı hacimde sağ ventrikül çıkış yolunu, ana ve dal pulmoner arterleri, aort kökü/arkı, MAPCA'ları ve ameliyat sonrası konduit-yama hattını opaklaştır; koroner çıkışlar veya kompleks anatomi sorusu varsa EKG eşzamanlaması yararlı olabilir.

Önerilen pencereler: Anjiyo (G 600 / M 150), Mediasten (G 350 / M 50).

BT bulguları

• Malalignment VSD — outlet septumun yer değiştirmesiyle oluşan geniş interventriküler açıklık ve aort köküne uzanım gösterilebilir.
• Aortik overriding — aort kökü ventriküler septumun üzerine yerleşerek her iki ventrikülden kan alır; yalnız çıkış damarının konumu değil septumla ilişkisi tanımlanır.
• Sağ ventrikül çıkış yolu darlığı — infundibüler kas demetleri, pulmoner kapak veya ana pulmoner arter düzeyinde daralma olabilir.
• Sağ ventrikül hipertrofisi — serbest duvar kalınlaşması morfolojik olarak görülür; BT tek başına basınç ya da kasılma işlevini belirlemez.
• Pulmoner arter hipoplazisi veya ayrıklığı — ana, sağ ve sol dalların kalibresi, konfluens ve hiler devamlılığı haritalanır.
• MAPCA — inen aorta veya ark dallarından akciğere uzanan sistemik kaynaklı kollateral arterler ve besledikleri akciğer bölgeleri izlenir.
• Onarım sonrası konduit veya yama — RV-PA bağlantısında daralma, kalsifikasyon, deformasyon ya da psödoanevrizma aranır.
• Pulmoner arter ve koroner komplikasyonu — yama veya stent düzeyinde dal darlığı ve çıkış yolu komşuluğunda seyreden anormal koroner arter tanımlanır.

Normalde

Normal kalpte interventriküler septum kesintisizdir, aort kökü sol ventrikülden çıkar ve sağ ventrikül çıkış yolu pulmoner kapağa doğru daralmadan devam eder; pulmoner arter gövdesi sağ ve sol dallara olağan biçimde ayrılır. Aynı düzeylerde VSD ile aort kökünün septuma göre konumunu, RVOT lümenini ve iki pulmoner arterin sürekliliğini kıyasla; ameliyatlı hastada bu referansa cerrahi yama ve konduit geometrisini ekle.

Ayırıcı tanı

Çift çıkışlı sağ ventrikül

her iki büyük arterin önemli bölümü morfolojik sağ ventrikülden çıkar; yalnız overriding aorta değil, her iki çıkış bağlantısı belirlenmelidir.

Pulmoner atrezi-VSD

RVOT boyunca patent dar çıkış yerine pulmoner kapak veya ana arter düzeyinde devamlı lümen yoktur; akciğer kanlanması MAPCA'lara bağlı olabilir.

Trunkus arteriozus

tek ortak arteriyel gövde pulmoner ve sistemik dolaşıma dallanır; ayrı aort ve pulmoner kökler bulunmaz.

Onarım sonrası pulmoner arter darlığı

konduit kalsifikasyonu veya degenerasyona bağlı fokal daralma, üç boyutlu kesitsel imajing teknikleriyle primer doğumsal merkezi darlıktan ayırt edilmelidir.

Tuzaklar

• Geniş bir aort kökünün septumu örtmesini tek başına overriding kabul etme; çıkış kökünün septumla anatomik ilişkisini farklı düzlemlerde doğrula.
• Postoperatif yama çıkıntısını psödoanevrizma saymadan önce kontrast lümeninin kese ile devamını ve eski görüntülerdeki değişimi ara.
• Pulmoner regürjitasyon şiddetini BT morfolojisinden çıkarsama; akım ve sağ ventrikül hacim işlevi için CMR veya ekokardiyografi gerekir.

Kendini dene

1. Geniş outlet VSD, septum üzerinde ata binen aort ve infundibüler daralma birlikte görülüyor. En olası tanı?

   • Fallot tetralojisi
   • Çift çıkışlı sağ ventrikül
   • Trunkus arteriozus
   • İzole pulmoner stenoz
   Cevabı göster

   Fallot tetralojisi. Malalignment VSD, aortik overriding ve RVOT obstrüksiyonunun birlikteliği klasik Fallot morfolojisidir. Çift çıkışlı sağ ventrikülde iki arteriyel kökün kaynağı sağ ventriküldür; trunkusta tek ortak arter bulunur; izole pulmoner stenoz Fallot çıkış ilişkilerini içermez.

2. Geniş transannüler yama içeren eski bir Fallot onarımından sonra, doğal RVOT duvarından değil yama dokusundan gelişebilen geç komplikasyon hangisidir?

   • RVOT psödoanevrizması
   • Pulmoner kapak yetersizliği
   • Rezidü VSD
   • Aortik overriding
   Cevabı göster

   RVOT psödoanevrizması. RVOT psödoanevrizması, geniş transannüler yama içeren onarımlardan sonra görülebilir; psödoanevrizma doğal RVOT duvarından değil yama dokusundan oluşur.

İlgili konular

• Anormal pulmoner venöz dönüş
• Atriyal septal defekt

Kaynaklar

Bu sayfadaki 34 cümle ve 2 quiz sorusu aşağıdaki kaynaklardan alıntılarla otomatik olarak karşılaştırıldı; 2 cümle bu karşılaştırmada düzeltildi (2026-10-09).

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