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Scimitar syndrome: experience with 6 patients.

Ranjith Baskar Karthekeyan1, Richard Saldanha, M Ranjith Sahadevan

  • 1Department of Cardiac Anesthesiology, Sri Ramachandra Medical College and Research Institute, No. 1 Ramachandra Nagar, Porur, Chennai - 116, India. ranjithb73@gmail.com

Asian Cardiovascular & Thoracic Annals
|August 1, 2009
PubMed
Summary

Scimitar syndrome, a rare congenital anomaly, involves abnormal pulmonary venous drainage. Diagnosis in six pediatric patients utilized echocardiography and CT, revealing associated lung and heart conditions.

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Area of Science:

  • Cardiology
  • Pediatric Surgery
  • Radiology

Background:

  • Scimitar syndrome is a rare congenital anomaly.
  • It is characterized by anomalous pulmonary venous drainage into the inferior vena cava, creating a left-to-right shunt.
  • Associated anomalies include dextroversion, lung malformations, and pulmonary hypertension.

Purpose of the Study:

  • To describe the clinical characteristics, diagnostic methods, and management outcomes of pediatric patients with scimitar syndrome.
  • To highlight the diagnostic utility of color Doppler echocardiography and computed tomography in identifying scimitar syndrome.
  • To review the spectrum of associated congenital anomalies and their impact on patient management.

Main Methods:

  • Retrospective review of six pediatric patients diagnosed with scimitar syndrome between 2002 and 2008.

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  • Diagnosis confirmed by color Doppler echocardiography and computed tomography.
  • Evaluation of associated cardiac and pulmonary anomalies, including pulmonary arterial hypertension.

    • Main Results:

    • Six patients (4 girls, 2 boys) diagnosed with scimitar syndrome.
    • Dextroversion was a key indicator in five cases.
    • Associated findings included horseshoe lung, unilobar right lung, hypoplastic right lung/lobe, and varying degrees of pulmonary arterial hypertension.
    • All patients presented with lower respiratory tract infections and right lung volume loss.

    Conclusions:

    • Scimitar syndrome requires a high index of suspicion, especially in the presence of dextroversion.
    • Color Doppler echocardiography and CT are crucial for accurate diagnosis.
    • The syndrome is frequently associated with significant pulmonary and cardiac abnormalities requiring tailored management strategies.