Related Experiment Video
Updated: Mar 19, 2026
Biventricular Assessment of Cardiac Function and Pressure-Volume Loops by Closed-Chest Catheterization in Mice
Published on: June 15, 2020
Not all right-sided hearts are the same-the importance of identifying the correct diagnosis
Faisal Mujib Siddiqui1, Edmundo Raul Rubio1, Vishal M Patel1
11 Department of Pulmonary and Critical Care, 2 Department of Radiology, Virginia Tech-Carilion School of Medicine, Roanoke, VA, USA.
Insights
Scimitar syndrome, a rare congenital anomaly, involves abnormal vein drainage into the inferior vena cava (IVC). Early diagnosis is crucial to prevent severe complications like pulmonary hypertension.
Area of Science:
- Cardiology
- Radiology
- Pediatric Medicine
Background:
- Scimitar syndrome is a rare congenital anomaly characterized by anomalous pulmonary venous return.
- Affected individuals often present with a characteristic chest X-ray (CXR) finding resembling a scimitar.
Observation:
- A case of Scimitar syndrome is presented in a 27-year-old woman with a history of dextrocardia.
- Initial presentation included altered mental status, tachycardia, and right-sided heart sounds.
- Radiographic findings revealed dextrocardia and retrocardiac opacities on CXR, confirmed by CT as anomalous venous drainage into the IVC and an aberrant artery supplying the right lower lobe.
Findings:
- The patient was diagnosed with Scimitar syndrome, a condition affecting 1-3 per 100,000 live births.
- Nearly half of patients with Scimitar syndrome are asymptomatic, leading to potential misdiagnosis, such as dextrocardia in this case.
- Associated findings included an anomalous systemic artery supplying the right lower lobe.
Implications:
- Accurate diagnosis of Scimitar syndrome is critical due to the risk of developing severe pulmonary hypertension and right ventricular failure.
- Regular echocardiographic monitoring is recommended for early detection of complications.
- Timely surgical intervention may be necessary to prevent adverse outcomes in patients with Scimitar syndrome.
Abstract:
Scimitar syndrome is characterized by an anomalous venous return with the characteristic chest roentgenogram (CxR) appearance of the anomalous vein draining into the inferior vena cava (IVC). This appears as a curvilinear opacity paralleling the right border of the heart resembling a curved sword or Scimitar. A 27-year-old white woman with a reported history of dextrocardia was admitted after a drug overdose. Examination demonstrated an obtunded woman with tachycardia and right sided heart sounds. Her CxR revealed a right sided heart image with two curvilinear opacities in the retrocardiac area. Chest computed tomography (CT) demonstrated that these opacities join to represent an anomalous vein draining into IVC. Furthermore, an anomalous systemic artery arising from the abdominal aorta was seen to supply the right lower lobe. The patient was eventually diagnosed with Scimitar syndrome. This syndrome affects 1-3 in 100,000 live births while nearly half of the patients remain asymptomatic with some initially being misdiagnosed as dextrocardia, such as in our case. Correctly diagnosing these patients is of paramount importance as some can develop severe pulmonary hypertension and right ventricular failure. In turn, close ongoing echocardiographic monitoring can help identify those that may benefit from surgical interventions to prevent them from developing these complications.
Related Concept Videos
01:22Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies
01:21Cardiac Catheterization II: Right Heart Catheterization
01:30Dysrhythmias V: Evaluating Dysrhythmias
01:20Imaging Studies for Cardiovascular System III: X-Ray
Definition and Purpose
An X-ray, or radiograph, is a non-invasive method that uses ionizing radiation to take images of internal structures. It is mainly used in cardiac imaging to examine the heart, lungs, and major blood vessels, aiming to identify abnormalities in the heart's size, shape, and position, such as heart failure, congenital defects, and vascular...
01:24Cardiac Catheterization III: Left Heart Catheterization
01:23Mitral Stenosis II: Clinical features and Diagnostic Tests