Scimitar Syndrome: Late Presentation and Conservative Management

Saleh, Mohanad; Abdelsalam, Murad; Sule, Anupam A; DeGregorio, Michele

doi:10.7759/cureus.1997

Cited by 2 publications

(2 citation statements)

References 7 publications

(8 reference statements)

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“…None of the medically treated presented the previously mentioned. Factors such as the presence or absence of shunts, pulmonary blood flow, the number of anomalous veins, the presence of concomitant heart or lung disease, and severity of symptoms are some of the variables that may be considered when deciding whether to perform a surgical procedure [ 22 , 23 ].…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics, imaging findings, management, and outcomes of patients with scimitar syndrome at a tertiary referral healthcare center in Colombia

Aristizabal,

Guzmán-Serrano,

Mondol-Villamil

et al. 2024

Int J Cardiovasc Imaging

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Scimitar Syndrome is part of a complex spectrum of congenital cardiovascular anomalies related to anomalous pulmonary venous return. Depending on the extent of involvement, treatment can be either expectant or surgical. Prognosis and survival have been controversial, with some results supporting early surgical management. This research aims to disclose the outcomes and describe the management, clinical and imaging characteristics of patients diagnosed with Scimitar Syndrome treated in a tertiary referral healthcare center. Longitudinal descriptive observational study. The study included all patients diagnosed with scimitar syndrome in our institution between January/2011 and December/2022. A description of the sociodemographic and clinical characteristics, diagnostic tools used, treatment features, and patient outcomes is provided. Eleven patients were included, with a mean age at diagnosis of five years (CI 0–17), six of which were female (54.55%). Nine (81.82%) patients had evidence of a scimitar vein on the chest radiograph, six (54.55%) cardiac dextroposition, six (54.55%) pulmonary hypoplasia, five (45.45%) right pulmonary artery hypoplasia, and three (27.27%) had aortopulmonary collaterals. Four (36.36%) patients had horseshoe lungs, and four (36.36%) had bronchopulmonary sequestration. In the associations, two (18.18%) patients were found to have an atrial septal defect, three (27.27%) ventricular septal defect, and one (9%) had Tetralogy of Fallot. Pulmonary hypertension was demonstrated in two (18.18%) patients. Seven (63.64%) required surgical management to correct the scimitar vein, and two patients died due to unrelated complications. Scimitar syndrome presents diagnostic and treatment challenges, necessitating a multidisciplinary approach for timely care. Chest radiography and CT scans are primary diagnostic tools, with surgical intervention often warranted alongside other heart defects or significant hemodynamic repercussions. Medical management is effective for mild to moderate cases. Long-term patient outcomes remain uncertain due to study limitations, but improved life expectancy is anticipated with ongoing care.

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Section: Discussionmentioning

confidence: 99%

Clinical characteristics, imaging findings, management, and outcomes of patients with scimitar syndrome at a tertiary referral healthcare center in Colombia

Aristizabal,

Guzmán-Serrano,

Mondol-Villamil

et al. 2024

Int J Cardiovasc Imaging

View full text Add to dashboard Cite

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“…ASD was present in 3 of the 16 patients diagnosed in adulthood, and 21% of these patients showed concurrent right PA hypoplasia [ 2 ]. SS can even be present in the seventh or eighth decade of life with symptoms of heart failure [ 3 , 4 ]. In our case, it is, however, possible that syncopes in childhood were manifestations of SS that were overlooked.…”

Section: Discussionmentioning

confidence: 99%

Scimitar Syndrome in Adulthood: Challenges in Management and Individualized Approaches

Luknár,

Hlivák,

Lesný

et al. 2024

Cureus

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Scimitar syndrome is a congenital disorder characterized by partial anomalous pulmonary venous return to the inferior vena cava (IVC). Clinical manifestation in adulthood is infrequent. The management approach has not been universally accepted and may be challenging. Individually tailored and multidisciplinary teambased decisions are often necessary. We present the case of a symptomatic patient diagnosed with complex congenital heart disease, including scimitar syndrome and atrial septal defect at the age of 50 years. Surgical repair, involving scimitar vein implantation in the left atrium using a pericardial patch, was performed. Despite surgical correction, dyspnea persisted, and hemoptysis developed. A diagnostic workup revealed a critical stenosis of the re-inserted vein. This was successfully treated by percutaneous intervention with stent implantation. The patient has remained asymptomatic since the procedure. Scimitar syndrome can be first diagnosed in adulthood, and clinical manifestations can vary. Diagnostic workup necessitates a CT angiogram, magnetic resonance scan, and catheterization in selected cases. Stenoses of re-implanted pulmonary veins (PVs) can develop years after surgical correction, and hemoptysis may serve as a warning symptom prompting further PV imaging. Percutaneous vascular intervention using a stent is warranted in symptomatic cases and can lead to long-term success.

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Scimitar Syndrome: Late Presentation and Conservative Management

Cited by 2 publications

References 7 publications

Clinical characteristics, imaging findings, management, and outcomes of patients with scimitar syndrome at a tertiary referral healthcare center in Colombia

Clinical characteristics, imaging findings, management, and outcomes of patients with scimitar syndrome at a tertiary referral healthcare center in Colombia

Scimitar Syndrome in Adulthood: Challenges in Management and Individualized Approaches

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