Surgical management of the scimitar syndrome

Abstract

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.

Novel operative technique for Scimitar syndrome: Posterior flap technique

“…The aim of SS repair is to create an unrestricted new pathway for the SV without disturbing the systemic venous return. 1 Our method has several advantages compared to techniques previously reported. [2][3][4] The new pulmonary venous pathway is constructed behind the IVC, namely, outside of the heart.…”

“…Various surgical techniques have been reported for its repair. 1 The most popular technique includes the direct reimplantation of the anomalous vein to the left atrium (LA) or the interatrial rerouting. However, these techniques are not applicable to all of the cases because of the heterogeneous anatomy of the disease.…”

A rare case of scimitar syndrome with pulmonary arterial hypertension in an adult female

“…It warrants a multidisciplinary approach with input from interventional cardiology, pulmonary medicine, and cardiothoracic surgery. Treatment options include surgical repair of the anomalous pulmonary vein, transcatheter device closure of the shunt, medical management of PAH, or lung transplantation in refractory 5,6 The choice depends on anatomy, shunt size, severity of PAH, and presence of other comorbidities in the patient population.…”

Prenatal Diagnosis and Outcome of Scimitar Syndrome: A Case Series of Six Patients

“…As corroborated in our series, scimitar syndrome may belong to the cardiac malformations of the VACTERL association [ 19 ]. In general, surgical correction is recommended in all symptomatic patients and asymptomatic patients with pulmonary-to-systemic flow ratios greater than 1.5:1 or pulmonary-to-systemic flow ratios lesser than 1.5:1 in the settings of clinically treated pulmonary hypertension, stenosis of the scimitar vein, or concomitant cardiac lesions [ 18 , 23 ].…”

“…A large European multi-center registry study showed that respiratory (including respiratory distress, recurrent upper respiratory tract infections, cyanosis, and pneumonia) as well as cardiac symptoms (including failure to thrive and congestive heart failure) occur in about 50% of all clinical cases [ 17 ]. An Indian study focusing on surgical outcomes in scimitar patients even showed that associated cardiac and extracardiac defects, particularly hypoplasia of the right lung, are present in up to three-quarters of cases, whereas operative mortality has been cited between 4.8% and 5.9% [ 18 ]. The aim of this study was to assess the spectrum of extra-cardiac anomalies and chromosomal anomalies, the intrauterine course, and the postnatal outcome of fetuses with scimitar syndrome.…”