Scimitar syndrome with different features in pediatric patients: a single-center experience

Ali Orgun; Birgül Varan; İlkay Erdoğan; Niyazi Tokel; Alper Gürsu; Murat Özkan; Mehmet Sait Aşlamacı

Makale detayları

Scimitar syndrome with different features in pediatric patients: a single-center experience

Yazarlar:

Ali Orgun | Birgül Varan | İlkay Erdoğan | Niyazi Tokel | Alper Gürsu | Murat Özkan | Mehmet Sait Aşlamacı

Yayıncı:

Pamukkale University

Ana Başlıklar:

Kalp ve Damar Cerrahisi | Çocuk Sağlığı ve Hastalıkları

Dergi:

Pamukkale Tıp Dergisi Vol.16 Issue 1, pp.37-42

Belge Tipi:

Makale

Yayın Tarihi:

1/2023

Belge Dili:

İngilizce

Document ID (Org No.):

2001281220

pISSN:

1308-0865

Anahtar kelimeler:

Özet:

 Purpose: Scimitar syndrome is a rare congenital cardiac anomaly in which pulmonary veins are drained to the inferior vena cava IVC instead of the left atrium and it is often associated with additional cardiac and pulmonary anomalies. In this article, the different clinical features of pediatric patients with Scimitar syndrome are reported. Material and methods: All patients with scimitar syndrome were diagnosed in our hospital between 2000 and 2020. The clinical findings, angiographic and surgical procedures, and follow-up of the patients are evaluated. Results: Five pediatric patients aged between 1.5 months and 10 years were diagnosed as Scimitar syndrome. All patients were symptomatic. One patient had cor triatriatum and pulmonary venous stenosis, one patient had left atrial isomerism and absence of the IVC azygous continuity, and another patient had coarctation of the aorta. One patient had dual drainage: the IVC and left atrium with meandering pulmonary veins, which we determined as a Scimitar variant. One infant patient with pulmonary hypertension and two patients with pulmonary to systemic flow ratio QpQs1.5 were treated surgically with the reanastomosis technique. Vascular embolization of the aortopulmonary collaterals was performed in two patients using vascular plugs, coils, and onyx. Conclusion: Treatment should be planned individually in this syndrome due to Scimitar syndrome has a wide range of anatomic and clinical variations.

 Purpose: Scimitar syndrome is a rare congenital cardiac anomaly in which pulmonary veins are drained to the inferior vena cava IVC instead of the left atrium and it is often associated with additional cardiac and pulmonary anomalies. In this article, the different clinical features of pediatric patients with Scimitar syndrome are reported. Material and methods: All patients with scimitar syndrome were diagnosed in our hospital between 2000 and 2020. The clinical findings, angiographic and surgical procedures, and follow-up of the patients are evaluated. Results: Five pediatric patients aged between 1.5 months and 10 years were diagnosed as Scimitar syndrome. All patients were symptomatic. One patient had cor triatriatum and pulmonary venous stenosis, one patient had left atrial isomerism and absence of the IVC azygous continuity, and another patient had coarctation of the aorta. One patient had dual drainage: the IVC and left atrium with meandering pulmonary veins, which we determined as a Scimitar variant. One infant patient with pulmonary hypertension and two patients with pulmonary to systemic flow ratio QpQs1.5 were treated surgically with the reanastomosis technique. Vascular embolization of the aortopulmonary collaterals was performed in two patients using vascular plugs, coils, and onyx. Conclusion: Treatment should be planned individually in this syndrome due to Scimitar syndrome has a wide range of anatomic and clinical variations.

 Purpose: Scimitar syndrome is a rare congenital cardiac anomaly in which pulmonary veins are drained to the inferior vena cava IVC instead of the left atrium and it is often associated with additional cardiac and pulmonary anomalies. In this article, the different clinical features of pediatric patients with Scimitar syndrome are reported. Material and methods: All patients with scimitar syndrome were diagnosed in our hospital between 2000 and 2020. The clinical findings, angiographic and surgical procedures, and follow-up of the patients are evaluated. Results: Five pediatric patients aged between 1.5 months and 10 years were diagnosed as Scimitar syndrome. All patients were symptomatic. One patient had cor triatriatum and pulmonary venous stenosis, one patient had left atrial isomerism and absence of the IVC azygous continuity, and another patient had coarctation of the aorta. One patient had dual drainage: the IVC and left atrium with meandering pulmonary veins, which we determined as a Scimitar variant. One infant patient with pulmonary hypertension and two patients with pulmonary to systemic flow ratio QpQs1.5 were treated surgically with the reanastomosis technique. Vascular embolization of the aortopulmonary collaterals was performed in two patients using vascular plugs, coils, and onyx. Conclusion: Treatment should be planned individually in this syndrome due to Scimitar syndrome has a wide range of anatomic and clinical variations.

 Amaç: Scimitar sendromu, pulmoner venlerin sol atriyum yerine inferior vena kavaya IVC açıldığı ve sıklıkla ek kardiyak ve pulmoner anomalilerle ilişkili olduğu nadir görülen bir doğuştan kalp anomalisidir. Bu yazıda farklı klinik özellikleri olan Scimitar sendromlu pediyatrik hastalar tartışılmıştır. Gereç ve yöntem: Hastanemizde 2000-2020 yılları arasında Scimitar sendromlu tanısı alan olgular çalışmaya alındı. Hastaların klinik bulguları, anjiyografik ve cerrahi işlemler ve takipleri değerlendirildi. Bulgular: Çalışma süresi içinde yaşları 1,5 ay ile 10 yaş arasında Scimitar sendromu tanısıolan beş hasta değerlendirildi. Tüm hastalar semptomatikti. Bir hastada kor triatriatum ve pulmoner venöz stenoz, bir hastada sol atriyal izomerizm ve IVC yokluğu azigos ven devamlılığı ve diğer hastada aort koarktasyonu vardı. Diğer bir hastada ise çift drenaj saptandı. Bu hastada İVCnin meandering pulmoner venler aracılığıyla sol atriyum ile olan bağlantısı Scimitar varyantı olarak değerlendirildi. Pulmoner hipertansiyonlu infantil bir hasta ve pulmoner  sistemik akım oranı QpQs1,5 olan iki hasta reanastomoz tekniği ile cerrahi olarak tedavi edildi. İki hastada ise aortopulmoner kollateraller saptanarak bu olgulara vasküler embolizasyon vasküler plug, koil ve onyx kullanılarak yapıldı. Sonuç: Scimitar sendromunun çok çeşitli anatomik ve klinik varyasyonları olması nedeniyle tedavi ve takip kişiye özel olarak planlanmalıdır.

Makale detayları

Scimitar syndrome with different features in pediatric patients: a single-center experience

Yazarlar:

Ali Orgun | Birgül Varan | İlkay Erdoğan | Niyazi Tokel | Alper Gürsu | Murat Özkan | Mehmet Sait Aşlamacı

Yayıncı:

Pamukkale University

Ana Başlıklar:

Kalp ve Damar Cerrahisi | Çocuk Sağlığı ve Hastalıkları

Dergi:

Pamukkale Tıp Dergisi Vol.16 Issue 1, pp.37-42

Belge Tipi:

Makale

Yayın Tarihi:

1/2023

Belge Dili:

İngilizce

Document ID (Org No.):

2001281220

pISSN:

1308-0865

Aortopulmonary collateral

children

meandering pulmonary veins

Scimitar syndrome

Aortopulmoner kollateral

çocuk

meandering pulmoner venler

Scimitar sendromu

Sayı

Makale detayları

Yazarlar: Ali Orgun | Birgül Varan | İlkay Erdoğan | Niyazi Tokel | Alper Gürsu | Murat Özkan | Mehmet Sait Aşlamacı

Yayıncı: Pamukkale University

Ana Başlıklar: Kalp ve Damar Cerrahisi | Çocuk Sağlığı ve Hastalıkları

Dergi: Pamukkale Tıp Dergisi Vol.16 Issue 1, pp.37-42

Belge Tipi: Makale

Yayın Tarihi: 1/2023

Belge Dili: İngilizce

Document ID (Org No.): 2001281220

pISSN: 1308-0865

Sayı

Yazarlar:

Ali Orgun | Birgül Varan | İlkay Erdoğan | Niyazi Tokel | Alper Gürsu | Murat Özkan | Mehmet Sait Aşlamacı

Yayıncı:

Pamukkale University

Ana Başlıklar:

Kalp ve Damar Cerrahisi | Çocuk Sağlığı ve Hastalıkları

Dergi:

Pamukkale Tıp Dergisi Vol.16 Issue 1, pp.37-42

Belge Tipi:

Makale

Yayın Tarihi:

1/2023

Belge Dili:

İngilizce

Document ID (Org No.):

2001281220

pISSN:

1308-0865