The spectrum of the scimitar syndrome

Farnsworth, Alan; Ankeney, Jay L.

doi:10.1016/s0022-5223(19)41683-8

Cited by 56 publications

(23 citation statements)

References 11 publications

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“…Post repair PV stenosis (8), reoperation for PV stenosis (2) Associated anomalies (20), ASD (21), sequestration (17), hypoplastic right lung (17), dextrocardia (15), PV stenosis (7), LSVC (4), MV prolapse (4), COA SV→IVC ASD (11), VSD (1), ASD+VSD (1), VSD, PDA(1), lung sequestration (1), MAPCA (13), hypoplastic right lung (16) CPB (19), non-CPB (2), sternotomy (18), thoracotomy (3), baffle repair (15), reimplantation to RA (4), to LA (1), pneumonectomy | 3781…”

Section: Demographicsmentioning

confidence: 99%

“…40 Cardiac anomalies included atrial and ventricular septal defects, functionally univentricular hearts, double outlet right ventricle, tetralogy, tricuspid atresia, anomalous origin of the left of the inferior caval vein, obstruction of the scimitar vein at its caval connection, pulmonary arterial hypoplasia, and aortopulmonary collateral arteries. 33,37,83 Hemivertebras, proximal rib fusion, subnumerary ribs, and micromelia of the arms have all been reported at necropsy. 33,37,83 Hypoplasia of the right lung to varying degrees has been reported in cardiac position.…”

Section: Surgical Anatomymentioning

confidence: 99%

“…80 Some have subclassified the syndrome into the groups of isolated presentation in infancy, presentation in infancy with congenital heart diseases, and isolated presentation during adulthood (Table 1). 3,11,12 In this regard, half of adults or adolescents reported in the multicentric European study present as isolated Palliative procedure-infant (12), adult (2) No intervention: infant (7), adult (2) Hypothermic CPB (32), circulatory arrest (8), ACCT 62 min CPBT (25-89 min), CPBT 94 min (42-195 min). Intracardiac baffle→ pericardial patch (14), PTFE patch (3) Perfusion scan (15)→ reduced flow right lung 24% (15) Surgery-mean 8.9 years (1.6-17 years)…”

Section: Demographicsmentioning

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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Section: Demographicsmentioning

confidence: 99%

Section: Surgical Anatomymentioning

confidence: 99%

Section: Demographicsmentioning

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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“…Scimitar syndrome is a complex malformation in which the main feature is a partial anomalous venous drainage of the right side toward the inferior vena cava or the portal system. There are anomalies associated in bronchopulmonary segmentation and vascular lung connections [1]. In 1836 in specimens of autopsy Cooper in London published the first description and later Chassinat in París [2,3].…”

Section: Introductionmentioning

confidence: 99%

Scimitar Syndrome: Infra-Diaphragmatic Form a Novel Surgical Approach

Riera-Kinkel¹,

Bocanegra-Flores²,

Rosas‐Peralta³

et al. 2019

AJCTS

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“…[ 16 ] The right pulmonary artery is usually abnormally hypoplastic, and in some cases, is totally absent; on the other hand, in 40% of cases, its size is almost normal. [ 17 ]…”

mentioning

confidence: 99%