Inferior Vena Cava Stenosis in Scimitar Syndrome: A Case Report

Jimenez, Maria; Hery, Eric; Doesburg, Nicolaas H. van; Guérin, Renée; Spier, Sheldon

doi:10.1016/s0894-7317(88)80098-1

Cited by 13 publications

(11 citation statements)

References 5 publications

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“…Das Scimitar-Syndrom, erstmals 1836 von Chassinat autoptisch beschrieben [7], ist eine seltene angeborene Missbildung, die ihren Namen der typischen Verschattungsfigur im Röntgenübersichtsbild, an einen klassischen türkischen Krummsäbel erinnernd, verdankt. Die Häufig-keit des Auftretens wird auf 1 bis 3 Fälle/ 100.000 Geburten geschätzt [2].…”

Section: "Klassisches" Scimitar-syndromunclassified

Auffälliger Röntgenthorax bei jungem Feuerwehranwärter

2007

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An 18-year-old, asymptomatic male underwent a routine chest x-ray examination which showed a pathological result. It revealed a characteristic crescent-like shadow in the right lower lung field, resembling a muslim sword (scimitar), responsible for the name of the underlying disease. Scimitar syndrome is a rare congenital malformation with a wide clinical spectrum ranging from asymptomatic to severe disturbances manifesting in childhood. The typical findings are hypoplasia of the right lung with marked mediastinal shift to the right and dextrocardia, reduced right pulmonary artery perfusion with maintained bronchial connection, anomalous arterial supply to the right lower lobe from the aorta and abnormal draining of the enlarged right pulmonary vein into the pulmonary circulation, producing the characteristic radiographic "scimitar sign". The development of right ventricular failure due to long-standing right ventricular overload and recurrent respiratory infections depend on the severity of anatomical abnormalities and functional disorders, sometimes requiring surgical intervention. Nowadays, sophisticated imaging techniques allow precise and rapid diagnosis of these complex abnormalities and their functional implications. The findings for our patient differed from the classic constellation, with a regular emptying of the enlarged right pulmonary vein into the left atrium, leading to a decreased burden on the central haemodynamics. In our very rare case of a scimitar syndrome variant, the functional disorders were negligible.

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Section: "Klassisches" Scimitar-syndromunclassified

Auffälliger Röntgenthorax bei jungem Feuerwehranwärter

2007

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“…The restricted imaging windows limit the usage of transthoracic echocardiography in assessing the exact site of drainage in the prenatal period, and in adults 26,31,41 . Transesophageal echocardiography is useful in confirming the diagnosis preoperatively, and in postoperative assessment of the patency of the scimitar vein 30,41,48,50–52,54,81 …”

Section: Resultsmentioning

confidence: 99%

“…Analysis was based initially on the individualized review of 91 investigations 1–4,6–92 . These were then incorporated from the results of the retrospective study emanating from the multi‐centric European Registry, which itself had collated the findings from 485 patients from 51 institutions, being carried out under the auspices of the Association for European Pediatric Cardiology and the European Congenital Heart Surgeons Association 5,64 …”

Section: Methodsmentioning

confidence: 99%

“…Due to the limited size of the reported series, and the heterogeneity of the clinical state at the time of surgical intervention, along with the difficulties in the selection of appropriate cardiac quantifiable end‐points, we were unable to perform a meta‐analysis. Our analysis, therefore, is based on a review of case reports and small case series, along with incorporation of data from the more extensive series reported from the Joint Multicentric Registry 1–92 …”

Section: Methodsmentioning

confidence: 99%

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Surgical management of the scimitar syndrome

Chowdhury

Anderson

Sankhyan

et al. 2021

Journal of Cardiac Surgery

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Background and Aim: We sought to address the varied anatomical details, the diagnostic challenges, associated cardiopulmonary anomalies, the techniques, and outcomes of management, including re-interventions of scimitar syndrome.Methods: A total of 92 published investigations of scimitar syndrome were reviewed. Diagnostic information was provided by clinical presentations, radiographic findings, transthoracic and transesophageal echocardiography, computedtomographic angiography, magnetic resonance imaging, angiocardiography, and ventilation/perfusion scans. These investigations served to elucidate the origin, course, and termination of the scimitar vein, the intracardiac anatomy, the presence of associated defects, and the patterns of any accompanying pulmonary lesions prior to surgical intervention.Results: Of the patients described, up to four-fifths presented during infancy, with cardiac failure, increased pulmonary flow, and pulmonary hypertension. Associated cardiac and extracardiac defects, particularly hypoplasia of the right lung, are present in up to three-quarters of cases. Overall operative mortality has been cited between 4.8% and 5.9%. Mortality was highest in patients with preoperative pulmonary hypertension, and those undergoing surgery in infancy. Despite timely surgical intervention, post-repair obstruction of the scimitar vein, intra-atrial baffle obstruction, or stenosis of the inferior caval vein were reported in up to two-thirds of cases. The venous obstruction could not be related to any particular surgical technique. On long term follow-up, one sixth of patients reported persistent dyspnoea and recurrent respiratory infections.Conclusions: Any infants presenting with heart failure, right-sided heart, and hypoplastic right lung should be evaluated to exclude the syndrome. An increased appreciation of variables will contribute to improved surgical management.

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“…[160][161][162]183,[189][190][191][192][193][194][195][196][197][198][199][200][201] The reasons for these features include pulmonary venous obstruction of both right or left-sided pulmonary veins, left-to-right shunting through the anomalous systemic artery or atrial septal defect, or from other coexisting cardiac lesions, or persistent pulmonary arterial hypertension of the newborn. 86,87,[160][161][162]183,[188][189][190][191][192][193][194][195][196][197][198][199][200][201][202] Rarely, the inferior caval vein may be congenitally stenotic, as in the patient reported by Tumbarello et al, 203 and others, 204 this feature also contributing to pulmonary venous obstruction (Fig. 6b).…”

Section: Scimitar Syndromementioning

confidence: 99%