Scimitar Syndrome Research Articles

Surgical management of the scimitar syndrome.

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. A total of 92 published investigations of scimitar syndrome were reviewed. Diagnostic information was provided by clinical presentations, radiographic findings, transthoracic and transesophageal echocardiography, computed-tomographic 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. 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. 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.

Scimitar Syndrome; Right Time to Operate and Mid-Term Results

Introduction: Scimitar syndrome is a combination of rare congenital cardiopulmonary anomalies that can occur in 3% to 6% of patients with a partial abnormal venous connection. The presence of accompanying cardiac anomalies in these patients and in cases such as severe hypoplasia of the right lung or accompanying pulmonary artery hypertension necessitate early surgery in early infancy. Patients and Methods: Nine patients with scimitar syndrome operated on in our pediatric cardiac surgery clinic from 2012 to 2020 were retrospectively examined in our study. The ages of the patients ranged from 1 to 47 years, with a mean of 18.1 ± 14.4. Patients’ weight values ranged from 9 to 88, with a mean of 47.0 ± 31.5. One patient died and mortality was 11.1%. Of the patients, 4 (44.4%) were male and 5 (55.6%) were female. Patients’ pulmonary arterial pressure ranged from 0.15 to 94 mmHg, with a mean of 39.2 ± 22.5. Results: Close to 25% scimitar vein stenosis or scimitar vein drainage occlusion has been reported in the post- operative period, mostly in the newborn group in the literature. Two patients had non-critical stenosis during the third year follow-up despite the absence of stenosis or occlusion during the first two years of follow-up of nine patients we followed. Their surgical follow-up is still ongoing since they are asymptomatic. Conclusion: The course of the disease depends on the follow-up of the patient, the timing of the surgery, and the quality of the anastomosis. The follow-up and treatment of these patients will be more accurate in advanced centers experienced in scimitar surgery.

The frequency and efficacy of genetic testing in individuals with scimitar syndrome.

Scimitar syndrome is a rare CHD composed of partial anomalous pulmonary venous connection from the right lung, via a scimitar vein, to the inferior vena cava rather than the left atrium. Genetic conditions associated with scimitar syndrome have not been well investigated at present. Our study included patients with scimitar syndrome diagnosed at Texas Children's Hospital from January 1987 to July 2020. Medical records were evaluated to determine if genetic testing was performed, including chromosomal microarray analysis or whole-exome sequencing. Copy number variants identified as pathogenic/likely pathogenic and variants of unknown significance were collected. Analyses of cardiac and extracardiac findings were performed via chart review. Ninety-eight patients were identified with scimitar syndrome, 89 of which met inclusion criteria. A chromosome analysis or chromosomal microarray analysis was performed in 18 patients (20%). Whole-exome sequencing was performed in six patients following negative chromosomal microarray analysis testing. A molecular genetic diagnosis was made in 7 of 18 cases (39% of those tested). Ninety-six per cent of the cohort had some type of extracardiac finding, with 43% having asthma and 20% having a gastrointestinal pathology. Of the seven patients with positive genetic testing, all had extracardiac anomalies with all but one having gastrointestinal findings and 30% having congenital diaphragmatic hernia. Genetic testing revealed an underlying diagnosis in roughly 40% of those tested. Given the relatively high prevalence of pathogenic variants, we recommend chromosomal microarray analysis and whole-exome sequencing for patients with scimitar syndrome and extracardiac defects.

Scimitar syndrome.

Scimitar syndrome.

Complex Infantile Scimitar Variant: Primary Stenosis of a Meandering Pulmonary Vein

A 5-month-old girl presented with failure to thrive, pulmonary hypertension, and a meandering intraparenchymal right pulmonary vein (PV), which continued as a confluence of the right upper and lower pulmonary veins (Figure 1, yellow), crossing the midline, and draining into the left atrium with a discrete stenosis at the connection. 1 Tsitouridis I. Tsinoglou K. Morichovitou A. et al. scimitar syndrome versus meandering pulmonary vein: evaluation with three-dimensional computed tomography. Acta Radiol. 2006; 47: 927-932 Crossref PubMed Scopus (15) Google Scholar ,2 Dupuis C. Charaf L.A.C. Breviere G.M. et al. Infantile form of the scimitar syndrome with pulmonary hypertension. Am J Cardiol. 1993; 71 (1326-1230) Abstract Full Text PDF Scopus (110) Google Scholar Adjacent was a stenotic left lower PV (Figure 1, red). The left upper PV was normal in caliber and position (Figure 1, blue).

PARTICULARITIES OF SCIMITAR SYNDROME IN ADULT PATIENTS

Scimitar syndrome or Felsons veno-lobar syndrome is a very rare congenital disease characterized by a combination of cardiopulmonary abnormalities, including partial right-sided pulmonary venous drainage to the inferior vena cava, the inferior cavo-atrial junction, or low on the right atrium. We report the case of a 53-year-old female patient who presented with recent gradually worsening dyspnea. The diagnosis was suspected on the chest x-ray and confirmed on Cardiac echography andComputed Tomography scan that showed a wide collector gathering the three right superior pulmonary veins that joins the lower part of the superior vena cava, thus joining the right atrium while the right inferior pulmonary vein is drained into the inferior vena cava. The patient was treated surgically by performing a derivation of the right superior pulmonary venous collector to the left atrium with a tricuspid annuloplasty with a good outcome.

Diaphragmatic eventration as a first sign of Scimitar syndrome.

Diaphragmatic eventration is an anomaly of the diaphragm. In Scimitar syndrome, a curved-shaped anomalous pulmonary venous drainage is seen. Association between these conditions is rare. We present a newborn with diaphragmatic eventration, whose diagnosis of Scimitar syndrome was made after surgical repair. Scimitar syndrome is a congenital disorder often associated with other heart and lungs anomalies. Diagnosis can be fortuitous but with important prognostic features.

Scimitar Syndrome Associated With Arterial Pulmonary Hypertension. Report a Case and Literature Review.

Scimitar Syndrome Associated With Arterial Pulmonary Hypertension. Report a Case and Literature Review.

Special Vascular Intervention in Newborn with Scimitar Syndrome

Special Vascular Intervention in Newborn with Scimitar Syndrome

Recurrent fibrovascular granulation on PhotoFix® bovine pericardium causing systemic and pulmonary venous obstruction after repair of scimitar syndrome - CORRIGENDUM.

Recurrent fibrovascular granulation on PhotoFix® bovine pericardium causing systemic and pulmonary venous obstruction after repair of scimitar syndrome - CORRIGENDUM.

Recurrent fibrovascular granulation on PhotoFix® bovine pericardium causing systemic and pulmonary venous obstruction after repair of scimitar syndrome.

A young adult with late diagnosis of scimitar syndrome underwent infradiaphragmatic baffling of the scimitar vein to left atrium through an intra-atrial tunnel using PhotoFix® bovine pericardium with recurrent extensive fibrovascular granulation of the patch causing pulmonary and systemic venous obstruction leading to eventual explantation of the bovine pericardium.

A Particular Infantile Scimitar Syndrome Variant with Anomalous Systemic Arterial Supply-Inferior Vena Cava Fistula

Abstract Scimitar syndrome is a rare congenital cardiovascular malformation that includes a partially anomalous drainage of the pulmonary veins in the inferior vena cava, right pulmonary hypoplasia, and systemic-pulmonary collaterals originating from various segments of the aorta. We present a case of Scimitar syndrome with associated intracardiac lesions and a large arterial conduct supplying the right lung, originating from the abdominal aorta and draining in the inferior vena cava.

Surgical correction for scimitar syndrome by right thoracotomy and direct anastomosis in children.

ABSTRACTImportanceScimitar syndrome (SS) is a rare type of congenital heart disease characterized by total or partial anomalous venous drainage of the right lung to the inferior vena cava. However, the surgical repair techniques for SS vary according to patients’ anatomical and pathological features.ObjectiveThis study was performed to analyze the mid‐term results of a less invasive surgical correction technique for SS in children.MethodsEleven patients with SS who underwent surgical repair from January 2012 to March 2020 were retrospectively analyzed. The anomalous scimitar vein (SV) was directly reimplanted to the left atrium, and the concomitant atrial septal defect was simultaneously repaired with cardiopulmonary bypass.ResultsThree male and eight female patients were included in the study. Their mean age was 3.1 ± 1.3 years, and their mean body weight was 12.8 ± 3.0 kg. Most patients had symptoms, such as upper respiratory tract infection, dyspnea, and recurrent pneumonia, and two patients had pulmonary hypertension. None of the 11 patients who underwent direct SV reimplantation by right thoracotomy developed bleeding, arrhythmia, heart failure, or perioperative death, and no patients required reoperation during a mean follow‐up period of 36.6 ± 15.2 months. Postoperative echocardiography revealed no restenosis or obstruction of the anastomosis in any patients.InterpretationSurgical repair for SS by right thoracotomy and direct anastomosis of the SV to the posterior wall of the left atrium is safe and effective, with good long‐term patency of the reimplanted SV and a low mortality rate.

The Lugones Procedure for Surgical Repair of Scimitar Syndrome: Preserved Growth in a Young Infant.

Surgical repair of Scimitar syndrome is challenging, especially in small patients. Our images demonstrate that the pericardial tunnel technique is feasible even in low-weight patient and that it provides a good growth potential.

Smaller right pulmonary artery is associated with longer survival time without scimitar vein repair.

The optimal management of scimitar syndrome remains incompletely defined. We (1) evaluated the impact of aortopulmonary collateral (APC) occlusion, (2) compared outcomes according to surgical approach for patients who underwent surgery, and (3) identified anatomic factors associated with longer survival time without scimitar vein repair. We conducted a single center, retrospective study of 61 patients diagnosed with scimitar syndrome between 1995 and 2019. Right pulmonary artery to total pulmonary artery cross-sectional area (RPA:PA CSA) quantitatively assessed right pulmonary artery size. Anatomical features were analyzed for association with longer survival time without scimitar vein repair. Median follow-up time was 6 years (Q1-Q3, 2-12), with 96% 5-year survival. Twenty-three patients underwent APC occlusion, which significantly decreased symptoms of overcirculation (100%-46%; p = .001) and systolic pulmonary artery pressure (median, 34-29 mmHg; p = .004). Twenty-three patients underwent scimitar vein repair; 5-year freedom from scimitar vein stenosis was 90% among patients who underwent a reimplantation compared with 42% in patients with baffle repair (p = .1). Three patients underwent surgery before the first year of age, with lower 5-year freedom from scimitar vein stenosis (0% vs. 84%; p < .001). On multivariate analysis, a lower RPA:PA CSA was associated with longer survival time without scimitar vein repair (p = .003). APC occlusion improves the clinical status of young and hemodynamically unstable patients. Repair at an early age is associated with an increased risk of scimitar vein stenosis. Scimitar vein repair might be avoided in patients with a smaller right pulmonary artery.

Scimitar syndrome and a common origin of the brachiocephalic trunk and left common carotid artery: a unique coexistence.

Scimitar syndrome and a common origin of the brachiocephalic trunk and left common carotid artery: a unique coexistence.

Unusual right pulmonary artery-to-left atrial communication associated with scimitar syndrome.

Right pulmonary artery to left atrial fistula is classified based on the right pulmonary artery branching, individual right pulmonary venous drainage, and presence of an aneurysmal segment. A rare association with scimitar syndrome and right lung devoid of blood supply from right pulmonary artery is described in this report. The anatomical and management differences between the different types are highlighted.

Scimitar syndrome – a case report

Abstract Objectives The aim of this article is to describe a clinical case of Scimitar syndrome, an unusual cause of respiratory distress in the neonatal period. This syndrome is a rare form of partial or total anomalous right lung venous drainage to the inferior caval vein, usually associated with right pulmonary hypoplasia. Case presentation The authors report a case of a 36-week-old newborn admitted to the neonatal intensive care unit with worsening respiratory distress. Chest radiography revealed a characteristic right pulmonary hypoplasia and a tubular structure paralleling the right heart border in the shape of a Turkish sword (“scimitar”). Chest computed tomography angiography confirmed the diagnosis. Conclusions Age of presentation and the severe malformations found, contributed to the patient’s poor outcome in this case.

Autologous vascularised pericardial flap tunneling technique in Scimitar syndrome repair with dextrocardia: lessons from the Senning procedure.

Scimitar syndrome is a rare variant (5%) of partial abnormal pulmonary venous return. Surgery is required when pulmonary overcirculation is present. Following repair, Scimitar vein stenosis occurs in approximately 20%. We applied a variant of the atrial switch technique using autologous pericardial flap in a patient with Scimitar syndrome and dextrocardia. This tunneling technique allowed tension-free anastomosis and minimal Scimitar vein rotation.

Scimitar syndrome in an elderly female without any cardiac anomaly: A case report

Scimitar syndrome or congenital pulmonary venolobar syndrome, is a rare congenital cardiovascular defect charactarized by anomalous venous return from the right lung (to the systemic venous drainage, rather than directly to the left atrium). This anomalous pulmonary venous return can be either partial (PAPVR) or total (TAPVR). The syndrome associated with PAPVR is more commonly known as Scimitar syndrome after the curvilinear pattern created on a chest radiograph by the pulmonary veins that drain to the inferior vena cava. Considering its rareness we are reporting scimitar syndrome in middle aged female patient with long standing dyspnea and recurrent chest infections without any cardiac anomaly.