An Unusual Neonatal Presentation of Scimitar Syndrome

Aziz, Ahmad Abdul; Thomas, Sumesh; Lautner, David; Awad, Essa Hamadan Al

doi:10.1055/s-0038-1656533

Cited by 5 publications

(4 citation statements)

References 14 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…9, 10 The diagnosis should be considered when there are signs of respiratory distress and/or heart failure present in association with radiological findings of cardiac dextroposition and haziness or suspicion of atelectasis of the right lung that does not improve. 11 The presence of “scimitar sign” may not always be seen in chest X-rays.…”

Section: Discussionmentioning

confidence: 99%

Congenital Pulmonary Venolobar Syndrome: Neurodevelopmental Follow-up

Abuzaid

James

Amer³

et al. 2021

Indian Journal of Clinical Cardiology

View full text Add to dashboard Cite

Congenital pulmonary venolobar syndrome or infantile scimitar syndrome is a rare congenital cardiopulmonary anomaly and has variable presentation, especially in the neonatal period. It is characterized by partial or total anomalous pulmonary venous return from the right lung along with pulmonary hypoplasia. The infantile form is associated with significant mortality and has a worse prognosis due to severe respiratory insufficiency, cardiac failure, and pulmonary infections. We report an infantile scimitar syndrome diagnosed within few hours of life, which has been followed up for 4 years for the presence of intact survival and without neurodevelopmental disorder in the domains of motor, cognition, communication, personal-social, vision, and hearing.

show abstract

Section: Discussionmentioning

confidence: 99%

Congenital Pulmonary Venolobar Syndrome: Neurodevelopmental Follow-up

Abuzaid

James

Amer³

et al. 2021

Indian Journal of Clinical Cardiology

View full text Add to dashboard Cite

show abstract

“…An association of the variant of SCS and coarctation of the aorta as described in our patient has never been reported in the literature before. In general, presentation of SCS in infancy with heart failure, pulmonary hypoplasia, and hypertension is associated with a poor prognosis [ 6 ]. A diagnosis of SCS should be considered when pulmonary hyper-tension persists after coarctation repair in a child with dextroposition of the heart and right lung hypoplasia.…”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary hypertension is a common finding and a poor prognostic sign. The combination of SCS and aortic coarctation is extremely rare, and only a few cases have been described in the literature [ 6 ]. With this combination, clinical manifestations at birth vary and can range in severity from no symptoms to respiratory distress and heart failure.…”

Section: Introductionmentioning

confidence: 99%

A Scimitar Syndrome Variant Associated with Critical Aortic Coarctation in a Newborn

et al. 2020

View full text Add to dashboard Cite

Patient: Female, newborn Final Diagnosis: Scimitar syndrome variant associated with critical aortic coarctaition Symptoms: Absence of pedal pulse • apnea • cardiogenic shock • extremely poor clinical condition • hepatomegaly • livedo reticularis • pallor • tachycardia Medication: — Clinical Procedure: — Specialty: Cardiology • Pulmonology Objective: Rare disease Background: Scimitar syndrome (SCS) is a rare congenital cardiopulmonary malformation, characterized by anomalous pulmonary venous drainage from the right lung associated with aortopulmonary collateral arteries and pulmonary hypoplasia. The variant described in the case presented here, with total anomalous right pulmonary venous drainage into the superior and inferior vena cava, can be expected in 2% of patients with scimitar syndrome. To the best of our knowledge, the association between the variant of SCS and coarctation of aorta described in our patient has never been reported before in the literature. Case Report: A female newborn with a gestational age of 35 weeks presented with a rare combination of scimitar syndrome and aortic coarctation. The patient had a variant of SCS that included anomalous drainage of the right upper and lower pulmonary vein into the superior and inferior vena cava, respectively; relative right lung hypoplasia; and right lower lobe sequestration supplied by aortopulmonary collateral arteries that originated from the truncus coeliacus. The diagnosis was confirmed with computed tomography angiography after resection of the aortic coarctation with extended end-to-end anastomosis. Subsequently, interventional closure of the collateral artery supplying the right lower lobe was performed with an AMPLATZER™ Vascular Plug 4. The patient’s clinical course was complicated by suspicious acute endocarditis and chylous pleural effusion. After a prolonged hospitalization, she was discharged in clinically stable condition. Conclusions: The diagnosis of SCS should be considered when pulmonary hypertension persists after coarctation repair in a child with dextroposition of the heart and right lung hypoplasia. Successful treatment of this rare combination of conditions calls for teamwork by highly experienced specialists.

show abstract

“…Surgical intervention involving resection of the right lower lung was carried out for the first time in 1950 by Drake and Lynch. Corrective surgery was achieved for the first time in 1956 by Kirklin et al [ 4 ]. This syndrome is a rare entity in and of itself; in this case, two additional unusual correlations are present.…”

Section: Introductionmentioning

confidence: 99%

Successful Surgical Management of Incidentally Diagnosed Scimitar Syndrome With Anomalous Connection of the Left Hepatic Vein to Coronary Sinus and Persistent Left Superior Vena Cava

Yadav¹,

Sharma²,

Pathak³

2023

Cureus

View full text Add to dashboard Cite

Scimitar syndrome is a rare congenital heart pathology that presents at birth, and it is a type of partial anomalous pulmonary venous return (PAPVR). In one in three people with scimitar syndrome, the right pulmonary vein is shaped like a scimitar (a Turkish sword) and can be easily visualized on radiographic imaging. This syndrome is not a simple and benign disease, and associated cardiac anomalies play a role in long-term outcomes, and the presence of pulmonary hypertension contributes to high mortality. The present case is the rarest of rare entities, scimitar syndrome, anomalous vascular connections between the left hepatic vein and coronary sinus, and persistent left superior vena cava in a single patient with no such case report published before.

show abstract

An Unusual Neonatal Presentation of Scimitar Syndrome

Cited by 5 publications

References 14 publications

Congenital Pulmonary Venolobar Syndrome: Neurodevelopmental Follow-up

Congenital Pulmonary Venolobar Syndrome: Neurodevelopmental Follow-up

A Scimitar Syndrome Variant Associated with Critical Aortic Coarctation in a Newborn

Successful Surgical Management of Incidentally Diagnosed Scimitar Syndrome With Anomalous Connection of the Left Hepatic Vein to Coronary Sinus and Persistent Left Superior Vena Cava

Contact Info

Product

Resources

About