The Scimitar Syndrome: An Italian Multicenter Study

doi:10.1016/j.athoracsur.2009.04.099

The Annals of Thoracic Surgery

Volume 88, Issue 2, August 2009, Pages 440-444

Presented at the Poster Session of the Forty-fifth Annual Meeting of The Society of Thoracic Surgeons, San Francisco, CA, Jan 26–28, 2009.

https://doi.org/10.1016/j.athoracsur.2009.04.099 Get rights and content

Background

Scimitar syndrome is a rare congenital heart disease. To evaluate the results of managing this malformation surgically, we have embarked on a multicenter Italian study involving seven different centers and reporting the largest published series in the medical literature.

Methods

From January 1997 to December 2007, 26 patients with scimitar syndrome who underwent surgical correction were included. Primary outcomes include hospital mortality and the efficacy of repair at the follow-up.

Results

Median age was 11 years (interquartile range, 1.8 to 19.9 years). Nineteen patients (73%) presented with symptoms including upper respiratory tract infections (n = 13), recurrent pneumonia (n = 10), cardiac failure (n = 4), and cyanosis (n = 2). Associated cardiac anomalies were present in 16 patients (63%). Surgical repair included intraatrial baffle repair in 18 patients (69%; group 1), and reimplantation of the “scimitar vein” onto the left atrium in 8 patients (31%; group 2). One patient died in hospital (3.8%; group 1). Postoperative complications were less frequently reported in group 1 (4 of 18 patients, 22%) compared with group 2 (5 of 8 patients, 62%). Median follow-up time was 4 years (interquartile range, 1.8 to 9.7 years). There was 1 late death (1/25 patients, 4%; group 2). Four patients (16%) showed a complete occlusion of the scimitar drainage (2 in group 1, 12%; 2 in group 2, 25%) and 3 patients (12%) required balloon dilation or stenting for scimitar vein stenosis (1 in group 1, 6%; 2 in group 2, 25%).

Conclusions

The intraatrial baffle repair seems to have a lower incidence of postoperative complications and a better patency rate, at last follow-up, than the reimplantation of the scimitar vein onto the left atrium.

Section snippets

Material and Methods

A review of the medical records and computerized hospital data was approved by the Clinical Investigation Committee from the University Hospital of Padua, and the procedures followed were in accordance to the institutional guidelines for retrospective record review and protection of patient confidentiality. Individual consent was not obtained for patients enrolled in this study. Patients are not identified, and the chairperson of the ethics committee of each institution consented to send their

Results

Twenty-six consecutive patients who underwent surgical treatment for scimitar syndrome in 7 different Italian centers were included in this study. There were 16 female patients (61%) and 10 males (39%). Preoperative diagnosis was achieved by echocardiography in all of them. Additional instrumental examinations included cardiac catheterization in 22 patients (85%) and magnetic resonance in 3 patients (11%). Nineteen patients (73%) presented with symptoms, including upper respiratory tract

Comment

Scimitar syndrome is a rare congenital heart disease characterized by a wide spectrum of lesions linked to the anomalous right pulmonary venous drainage and to the severity of right lung hypoplasia [1, 2, 3, 4, 26]. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to the possibility of this syndrome [5, 6]. In addition the plain chest x-ray film is a very relevant tool in the screening of this disease, because it discloses a

References (37)

A. Cukier et al.
Scimitar sign with normal pulmonary venous drainage and systemic arterial supply: scimitar syndrome or bronchopulmonary sequestration?
Chest
(1994)
C. Dupuis et al.
“Infantile” form of the scimitar syndrome with pulmonary hypertension
Am J Cardiol
(1993)
Y.A. Gao et al.
Scimitar syndrome in infancy
J Am Coll Cardiol
(1993)
B. Alsoufi et al.
Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection
Ann Thorac Surg
(2007)
C. Dupuis et al.
The “adult” form of the scimitar syndrome
Am J Cardiol
(1992)
J.W. Brown et al.
Surgical management of scimitar syndrome: an alternative approach
J Thorac Cardiovasc Surg
(2003)
H.K. Najm et al.
Scimitar syndrome: twenty years' experience and results of repair
J Thorac Cardiovasc Surg
(1996)
D.P. Mason et al.
Extrapleural pneumonectomy for scimitar syndrome
J Thorac Cardiovasc Surg
(2006)
R.F. Calhoun et al.
A novel operative approach to scimitar syndrome
Ann Thorac Surg
(2003)
C. Thibault et al.
Lobectomy in the treatment of the scimitar syndrome
Ann Thorac Surg
(1995)

C.C. Wang et al.

Scimitar syndrome: incidence, treatment, and prognosis

Eur J Pediatr

(2008)

N. Espinosa-Zavaleta et al.

Clinical and echocardiographic characteristics of scimitar syndrome

Rev Esp Cardiol

(2006)

V.N. Vasil'ev et al.

The clinical picture, diagnosis and surgical treatment of the “scimitar syndrome”

Grud Serdechnososudistaia Khir

(1993)

A. Pikwer et al.

Pulmonary sequestration—a review of 8 cases treated with lobectomy

Scand J Surg

(2006)

B.C. Mordue

A case series of five infants with scimitar syndrome

Adv Neonatal Care

(2003)

C.B. Huddleston et al.

Scimitar syndrome presenting in infancy

Ann Thorac Surg

(1999)

Y.S. Tjang et al.

Scimitar syndrome presenting in adults

J Card Surg

(2008)

C. Dupuis et al.

Surgical treatment of the scimitar syndrome in children, adolescents and adultsA cooperative study of 37 cases

Arch Mal Coeur Vaiss

(1993)

Cited by (41)

A sword threatening the heart: The scimitar syndrome
2020, JTCVS Techniques
In this review, we discuss the natural and surgical history of patients with scimitar syndrome. We describe the clinical presentation, the different forms and symptoms that can be identified and the possible surgical approaches used. A discussion is also provided on the outcomes of patients undergoing surgery compared to those undergoing medical treatment. The choice of appropriate treatment is still challenging today, but new data and large multicenter studies have shown interesting new results.
Embryology
2017, The Complete Reference for Scimitar Syndrome: Anatomy, Epidemiology, Diagnosis and Treatment
Scimitar syndrome (SS) encompasses a constellation of malformations with as a most prominent feature the anomalous “scimitar” like drainage of the inferior right pulmonary vein into the inferior caval vein. In addition, the syndrome may encompass systemic-to-pulmonary artery connections, hypoplasia of the right pulmonary artery and lung, as well as airway and diaphragmatic abnormalities. In this chapter it is aimed to approach the anatomical spectrum of malformations observed in SS in the perspective of embryonic development. The splanchnic mesoderm, giving rise to the early splanchnic plexus, with initially both pulmonary venous-to-systemic connections and pulmonary arterial-to-systemic connections, will play a pivotal role. A working model for a developmental approach of SS is provided.
Anatomy and Histology
2017, The Complete Reference for Scimitar Syndrome: Anatomy, Epidemiology, Diagnosis and Treatment
Scimitar syndrome is a rare congenital heart defect characterized by anomalous venous drainage of part or all of the right lung to the inferior vena cava, variable systemic blood supply to part of the right lung, dextroposition of the heart, and variable right lung hypoplasia with abnormal right bronchial distribution. A majority of patients with scimitar syndrome presented in association other congenital heart malformations, which contribute to characterize the complex anatomical features of this rare syndrome.
Surgical Treatment and Outcomes
2017, The Complete Reference for Scimitar Syndrome: Anatomy, Epidemiology, Diagnosis and Treatment
Surgical repair is indicated in symptomatic patients and in patients with associated congenital heart diseases (CHDs) and is beneficial in reducing symptoms. Surgical repair consists in the redirection of the anomalous pulmonary venous drainage into the left atrium. A right lung lobectomy or pneumonectomy (either as primary therapy or if postoperative stenosis is present after failed repair) may be required in patients with severe clinical features. The result of surgical treatment was linked to the age of patients; patients requiring surgical treatment under 1 year of age are usually very ill, and have a relatively high operative mortality and complication rate, whereas older patients have a better outcome, both immediately and in the long term. The incidence of postoperative stenosis/occlusion of the scimitar drainage is significant and a continuous follow-up is indicated.
A chronic cough of unusual cause
2017, Revue de Medecine Interne
Adult scimitar syndrome: A surgical approach
2015, Heart Lung and Circulation
Citation Excerpt :
The main methods for surgical repair of Scimitar Syndrome include the creation of an intra-atrial baffle from the orifice of the scimitar vein within the IVC through an existing or created atrial septal defect (ASD) to the left atrium [4], division and reimplantation of the scimitar vein into the right atrium and then directing the anomalous pulmonary venous flow into the left atrium via a baffle through an ASD [5] and direct anastomosis of the divided scimitar vein to the left atrium [6]. We have decided to use the foremost method of repair as a recent multicentre Italian study in 2009 by Vida et al. has shown that intra-atrial baffle repair seemed to carry a lower incidence of postoperative complications (22% vs. 62%) when compared to the technique of dividing and anastomosing the scimitar vein to the left atrium [7]. There was also a reduced chance of stenosis or occlusion in the former at mid-term (18% vs. 50%) [7].
Scimitar syndrome is a rare congenital condition characterised by partial or total anomalous pulmonary venous drainage of the right lung. We present an adult case of Scimitar syndrome that was managed with an intra-atrial baffle repair.
The patient was a 52 year-old lady who had symptoms of increasing dyspnoea and fatigue with a long-term history of poor exercise tolerance. Several investigations such as a chest X-ray, echocardiogram and coronary angiogram confirmed the diagnosis of Scimitar syndrome with significant left to right shunt. The operation proceeded under cardiopulmonary bypass with a short period of hypothermic circulatory arrest. Postoperative recovery was uneventful and a follow-up echocardiogram confirmed the correction of the condition.
There are many alternative and novel surgical techniques developed which include the reimplantation of the scimitar vein into the right atrium with the creation of a baffle through an ASD, direct anastomosis of the divided scimitar vein to the left atrium and many others. However, none of them have outcomes which are proven to be better than the technique we chose.
We utilised a well-recognised technique for a patient that had indications for surgical repair and this resulted in a good prognosis.

View all citing articles on Scopus

View full text

Original articleGeneral thoracicThe Scimitar Syndrome: An Italian Multicenter Study

Background

Methods

Results

Conclusions

Section snippets

Material and Methods

Results

Comment

Chest

Am J Cardiol

J Am Coll Cardiol

Ann Thorac Surg

Am J Cardiol

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

Ann Thorac Surg

Ann Thorac Surg

Scimitar syndrome: incidence, treatment, and prognosis

Eur J Pediatr

Clinical and echocardiographic characteristics of scimitar syndrome

Rev Esp Cardiol

The clinical picture, diagnosis and surgical treatment of the “scimitar syndrome”

Grud Serdechnososudistaia Khir

Pulmonary sequestration—a review of 8 cases treated with lobectomy

Scand J Surg

A case series of five infants with scimitar syndrome

Adv Neonatal Care

Scimitar syndrome presenting in infancy

Ann Thorac Surg

Scimitar syndrome presenting in adults

J Card Surg

Surgical treatment of the scimitar syndrome in children, adolescents and adultsA cooperative study of 37 cases

Arch Mal Coeur Vaiss

Original article
General thoracic
The Scimitar Syndrome: An Italian Multicenter Study