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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.
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.
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