Beyond Scimitar Syndrome: A Unique Combination of Complex Congenital Heart Disease
Author: Kelly Paschke, MD, Additional Authors**
Background:
Scimitar syndrome is a rare entity within congenital heart disease. The clinical presentation may be insidious and associated with a number of cardiopulmonary anomalies.
Case:
The patient was a 47 year-old-male with a past medical history of dextrocardia and migraine headaches.
The patient presented in September 2017 to his primary care physician with progressive dyspnea on exertion and palpitations. Chest X-ray showed dextrocardia and a dilated pulmonary vessel representing anomalous pulmonary venous drainage at the right lung with associated dilation of the pulmonary arteries (Figure 2A).
He was noted to have a 3/6 systolic murmur at the right upper sternal border and was referred for transthoracic echocardiogram (TTE). TTE showed dextrocardia, LVEF 41%, right ventricular (RV) dilation with mild reduction in RV systolic function, right atrial dilation, moderate tricuspid valve (TV) regurgitation, and an estimated RVSP of 37 mmHg.
Decision Making:
Cardiac CTA and MRI (Figure 2B, C) were performed which showed dextrocardia with scimitar syndrome with the right pulmonary vein draining via a curvilinear vein to the junction of the IVC without significant stenosis, with Qp/Qs 1.6. No significant intra-atrial shunt was present. The aortic root was dilated (5 cm) with a single coronary artery originating from the right coronary cusp. No coronary artery disease was noted on his coronary angiogram.
In view of these findings, surgical repair was performed with in-situ pericardial repair of scimitar vein routing (Figure 1) to the left atrium, repair of the TV, and repair of the non-coronary aortic sinus with CardioCel. On follow up, the patient reported improved functional capacity with normalization of his right ventricular size and function.
Conclusion:
This unique case highlights a combination of dextrocardia scimitar syndrome, aortic root dilation, and anomalous single coronary artery. This congenital complex went undiagnosed for nearly five decades with only mild symptom burden.
While scimitar syndrome is a rare entity, it typically is associated with partial or complete drainage of the right lung by anomalous pulmonary veins to the IVC, dextroposition or dextrocardia, anomalous systemic arterial supply from the aorta to the right lung, and intracardiac shunt. As a result, the right lung may be hypoplastic or have abnormalities involving the vasculature and bronchial anatomy (Table 1). It is not uncommon for patients with scimitar syndrome to have accompanying cardiac defects such as in the case of this patient.
In patients presenting with worsening functional capacity who are found to have dextrocardia or dextroposition and evidence of pulmonary vein anomalies on chest x-ray, it would be prudent to request tomographic cardiac imaging (MRI or CT) which would be helpful in diagnosing and directing management. In patients with RV volume overload in the setting of a significant shunt, surgical intervention should be pursued at a tertiary care center.
The patient in this case presented with typical features of scimitar syndrome including an anomalous right pulmonary vein with drainage to the IVC and dextrocardia. The patient additionally had an anomalous single coronary artery and aortic root dilation which makes this case an interesting combination of congenital cardiac abnormalities. Multimodality cardiac imaging played a crucial role in the diagnosis and surgical planning of this case.
**Additional Authors
Mouin Abdallah, MD, Department of Cardiovascular Medicine, Cleveland Clinic
James Gentry, MD, Department of Cardiovascular Medicine, Cleveland Clinic
Serge Harb, MD, Department of Cardiovascular Medicine, Cleveland Clinic
Joanna Ghobrial, MD, Department of Cardiovascular Medicine, Cleveland Clinic
Hani Najm, MD, Department of Cardiovascular Medicine, Cleveland Clinic
David Majdalany, MD, Department of Cardiovascular Medicine, Cleveland Clinic
Program Director: Scott Maffett, MD
Program Coordinator: Monica Rios
