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Scimitar Syndrome: Late Presentation and Conservative Management

Mohanad Saleh1, Murad Abdelsalam2, Anupam A Sule1

  • 1Department of Internal Medicine, St. Joseph Mercy Oakland, Pontiac, USA.

Cureus
|August 9, 2020
PubMed

Insights

Partial anomalous pulmonary venous return, a rare congenital defect, can manifest as Scimitar syndrome. This case highlights incomplete Scimitar syndrome in an adult, managed non-surgically due to its unique presentation.

Area of Science:

  • Cardiology
  • Congenital Heart Disease
  • Thoracic Surgery

Background:

  • Partial anomalous pulmonary venous return (PAPVR) is a rare congenital malformation.
  • Scimitar syndrome, an infracardiac PAPVR variant, typically presents in infancy or childhood.
  • Adult presentation of Scimitar syndrome is uncommon.

Observation:

  • A 70-year-old woman presented with worsening dyspnea.
  • Echocardiography indicated severe pulmonary hypertension, confirmed by right heart catheterization.
  • Computed tomography revealed an anomalous vein from the right upper lobe, consistent with Scimitar syndrome.

Findings:

  • The patient had incomplete Scimitar syndrome, lacking other congenital heart defects.
  • Pulmonary hypertension was the primary clinical manifestation.
  • The anomalous venous drainage involved only the right upper lobe.

Implications:

  • Incomplete Scimitar syndrome can present in late adulthood with pulmonary hypertension.
  • Non-surgical management may be appropriate for incomplete Scimitar syndrome, avoiding surgical risks.
  • This case expands the understanding of Scimitar syndrome's varied clinical spectrum and management.

Keywords:
congenital anomalyconservative treatmentpartial anomalous pulmonary venous returnpulmonary hypertensionscimitar syndrome

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