Scimitar syndrome with late presentation / El síndrome de la cimitarra con presentación tardía

Abstract Introduction: Scimitar syndrome is a congenital malformation with an incidence of 1-3 per 100,000 live births, consisting of abnormal drainage of the right pulmonary veins. The age of diagnosis varies according to the severity of the pathology, which depends on the degree of cardiac and pulmonary malformation. The case of a patient with a late diagnosis of the syndrome due to a silent course is reported despite severe anatomical alterations. Clinical Case: A 76-year-old female was admitted due to a 6-month clinical picture of progressive dyspnea to minimal efforts. A transthoracic echocardiogram was performed showing severe pulmonary hypertension of 96 mmHg with abnormal communication between the right pulmonary veins and the inferior vena cava, confirmed by CT angiography. Discussion: The late clinical course in relation to severe anatomical malformations has not been described in the scientific literature due to its high mortality in childhood.

Resumen Introducción: El síndrome de la cimitarra es una malformación congénita con una incidencia de 1 a 3 por cada 100,000 nacidos vivos, que consiste en un drenaje anormal de las venas pulmonares derechas. La edad en el momento del diagnóstico varía según la severidad de la patología, la cual depende del grado de malformación cardíaca y pulmonar. Se reporta el caso de una paciente con diagnóstico tardío del síndrome dado por un curso silencioso a pesar de tener alteraciones anatómicas severas. Caso clínico: mujer de 76 años de edad quien se hospitaliza por un cuadro clínico de seis meses de evolución consistente en disnea progresiva de mínimos esfuerzos. Se le practica un ecocardiograma transtorácico evidenciando hipertensión pulmonar severa de 96 mmHG con una comunicación anormal entre las venas pulmonares derechas y la vena cava inferior, confirmada por angiografía por TC. Discusión: El curso clínico tardío con relación a las malformaciones anatómicas severas no se ha descrito en la literatura científica dada su alta mortalidad en la infancia.

A Rare Associated Anomaly in Tetralogy of Fallot: Scimitar Syndrome in an Infant

Abstract Pulmonary venous connections may be infrequently abnormal in patients with tetralogy of Fallot (TOF). A special subgroup of partial anomalous pulmonary venous return,"scimitar cyndrome", and its coexistence with TOF is less frequently reported. It may proceed unnoticed, as cyanosis already predominates in the clinical picture. This uncommon association must be kept in mind for patients with TOF who have an accessory flow in the inferior vena cava, especially when all pulmonary venous return to the left atrium is not clearly seen.

Analysis of diagnosis and treatment of scimitar syndrome in 13 cases / 中华胸心血管外科杂志

Objective:To investigate the clinical features, diagnosis, treatment and prognosis of Scimitar syndrome.Methods:A retrospective analysis of clinical data of 13 children with scimitar syndrome from January 2013 to November 2020, including clinical symptoms, chest X-ray, echocardiography, cardiac CT and cardiac catheterization prognosis and follow-up.Results:13 children with scimitar syndrome were diagnosed, including 7 girls and 6 boys with a average age of 17 months(21 days to 10 years).3 cases <5 kg in weight. Ten patients presented with the infantile form and 3 with the adult form of scimitar syndrome. 13 infantile form had lower respiratory tract infections, heart failure, and growth retardation, of which 8 cases were with severe pulmonary arterial hypertension. 3 adult form were diagnosed because of heart murmur. 12 cases had coexisting cardiac lesions, including 12 atrial septal defect, 2 patent ductus arteriosus, 1 right ventricular double outlet/ventricular septal defect, 4 right lung and right pulmonary artery dysplasia, 2 right lung dysplasia, 6 additional systemic arterial supply to the right lung. Ten patients had pulmonary venous drainage correction surgery, one patient only underwent right ventricular double-outlet correction, three patients died of severe pulmonary hypertension; one patient lost the opportunity of surgery due to obstructive pulmonary hypertension, and one patient was complicated by nervous system The disease gave up treatment. One corrected case was stenosed during discharge and 2 corrected children became stenotic during follow-ups.Conclusion:Found with the median or dextrocardial heart, recurrent respiratory infections, or unexplained pulmonary hypertension, the possibility of scimitar syndrome should be considered. The combination of echocardiography and cardiac CTA can confirm the diagnosis as soon as possible. The risk factors for mortality included infantile form and severe preoperative pulmonary hypertension. Long-term follow-up is still required after operation, and surgical intervention is required again if necessary.

Hybrid treatment and prognosis of Scimitar syndrome / 中华胸心血管外科杂志

Objective:To evaluated the clinical optimization of treatment strategies for the disease and risk factors for prognosis.Methods:The records of 34 patients with scimitar syndrome evaluated at our medical center between Jan 2013 and Dec 2018 were reviewed, including clinical data, treatment outcome and follow-up.Results:Scimitar syndrome was identified in 34 patients, 16 males and 18 females, aged (21.16±33.19) months. The mean length of follow-up after diagnosis was (50.30±26.99) months. Compared with adult form patients, patients presenting less than 1 year of age had a higher incidence of pulmonary hypertension and pulmonary veins stenosis. 8 patients didn’t require surgical treatment after interventional coil embolization of aortopulmonary collaterals or other interventional maneuvers in associated congenital heart diseases. 15 patients had scimitar vein surgery, 4 patients had postoperative pulmonary vein obstruction that occurred with similar frequency after baffle or reimplantation procedures( P=0.569), and tended to be more common in patients had preoperative obstruction of scimitar vein( P=0.009). Overall, 7(20.5%) of 34 died. High risk factors for death included age at diagnosis( P=0.000), obvious pulmonary hypertension( P=0.007) and pulmonary vein stenosis( P=0.014). Conclusion:Patients with pulmonary artery systolic pressure near normal and absence of congenital heart disease excluding atrial septal defect do not require surgery. Postoperative pulmonary vein obstruction is related with scimitar vein stenosis before surgery regardless of redirection technique. Age at diagnosis, obvious pulmonary hypertension and pulmonary vein stenosis are risk factors for death in patients with scimitar syndrome.

A Case of Scimitar Syndrome in an Adult with an Atrial Septal Defect / 日本心臓血管外科学会雑誌

The patient was a 34-year-old woman who had been routinely monitored after receiving a childhood diagnosis of partial anomalous pulmonary venous connection, but unilaterally discontinued follow-up examinations after the age of 18. At 33 years of age, she was admitted to our hospital after a physical examination revealed an abnormal shadow on a chest X-ray. Transthoracic echocardiography detected an atrial septal defect (ASD), and contrast-enhanced computed tomography showed that the right lower pulmonary vein drained to the inferior vena cava. The patient was diagnosed with scimitar syndrome with ASD. Cardiac catheterization showed a pulmonary/systemic flow ratio (Qp/Qs) of 2.48 and a left-to-right shunt rate of 59.7%. Surgical treatment was deemed to be indicated. The right lower pulmonary vein was anastomosed to the anterolateral wall of the right atrium, and an intra-atrial baffle repair was performed from the orifice within the right atrium to the left atrium through the existing ASD using untreated fresh autologous pericardium. Two years after the operation, good blood flow was maintained within the baffle with no stenosis at the anastomotic site. This report describes a rare case of scimitar syndrome with ASD in an adult woman, and provides a review of the existing literature.

The Rare Congenital Anomaly of Scimitar Syndrome: An Unusual Presentation in Symptomatic Adult

@#Scimitar syndrome is a rare congenital heart defect occurring in 1 to 3 per 100,000 live births. This is a case of a 26 years old lady presenting with episodic fainting spells since the age of 18 years old. She was initially diagnosed with epilepsy until a referral to our centre found a soft splitting of the second heart sound and multiple premature ventricular complexes on ECG. The computed tomography of the pulmonary artery confirmed the diagnosis if Scimitar syndrome in the presence of anomalous single right pulmonary vein draining into infra-diaphragmatic systemic venous circulation. A corrective open-heart surgery to re-implant the pulmonary vein was performed with excellent clinical outcomes. Therefore, it is crucial for clinicians to embody high index of suspicion of congenital anomaly even in adults presenting with indefinite clinical symptoms. This report also represents the first published case of adult Scimitar syndrome from Malaysia.

A very rare combination: two scimitar veins and a myocardial bridge

Abstract Scimitar syndrome is a rare congenital anomaly characterized by partial or complete anomalous pulmonary venous drainage of the right (rarely left) lung into the inferior vena cava. This anomalous vein resembles the curved Turkish sword "scimitar"[1]. Only few cases were reported with two scimitar veins[2]. "Myocardial bridge" constitutes a portion of the myocardial tissue that bridges a segment of the coronary artery, mostly the left anterior descending coronary artery . For the first time, a combination of double scimitar vein and a myocardial bridge was described in this study.

Síndrome de Cimitarra: revisión a partir de un caso clínico / Scimitar syndrome: a review based on a clinical case

Resumen Así como otros síndromes congénitos considerados raros por su baja incidencia, el diagnóstico del síndrome de la Cimitarra se basa en la presencia de un drenaje venoso anómalo de las venas pulmonares derechas que en muchas ocasiones se asocia con la presencia de otras malformaciones pulmonares y cardíacas. La gran mayoría pasan desapercibido puesto que los síntomas pueden manifestarse hasta en edades adultas y puede pasarse por alto. A propósito de un caso clínico, presentamos una revisión de este tema, que es importante tomar en cuenta para darles a estos pacientes un seguimiento y tratamiento adecuados.

Abstract As other congenital syndromes are considered rare based on their low incidence, the diagnosis of scimitar síndrome is based on the presence of an abnormal right venous pulmonary drainage, accompanied in many cases with other pulmonary and cardiac malformations. The vast majority of them has been undiagnosed, because the symptoms present later in adulthood. With regard to a clinical case, we present a review of this topic, which is important to take into account to give this patients a follow-up and adequate treatment.

Elective Cesarean Section In Scimitar Syndrome: Anaesthesia Management

Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies consisting of a partial anomalous pulmonary venous connection of the right lung (part or the entire right lung) to the inferior vena cava, right lung hypoplasia, dextroposition of the heart, and anomalous systemic arterial supply to the right lung. Anaesthesia management of pregnant patients with Scimitar syndrome is challenging due to coexisting congenital heart disease. The literature on anaesthesia management of patients with Scimitar syndrome undergoing Elective cesarean section is rare and hence authors wish to share their experience of anaesthesia management of a case of Scimitar syndrome posted for elective cesarean in a rural area hospital.

Prenatal ultrasonographic features of fetal total anomalous pulmonary venous connection / 中华围产医学杂志

Objective To investigate the prenatal ultrasonographic features of fetal total anomalous pulmonary venous connection (TAPVC).Methods Forty-one cases who received prenatal ultrasound examination and then were diagnosed with TAPVC by postnatal surgery at the Third Affiliated Hospital of Zhengzhou University from June 2013 to June 2018 were enrolled.Echocardiography findings of all cases were analyzed retrospectively,and the prenatal imaging features were summarized.Results Among all cases,21 were confirmed as supracardiac type,14 as intracardiac type,four as infracardiac type and two as mixed type after surgery.The echocardiographic features of TAPVC were as follows:all 41 cases showed smooth posterior wall of left atrium without visible part of pulmonary venous connected to the left atrium in the-four chamber view;in 33 cases,the space between left atrium and descending aorta was widened and the pulmonary venous confluence was observed in the four-chamber view;ten cases showed a dilated coronary sinus in the four-chamber view and 27 cases showed vertical vein in the three vessels and trachea or the upper abdomen view.None of the 41 cases was complicated by other structural intracardiac abnormalities.However,seven cases were complicated by obstruction of blood flow,and the blood flow velocity measured by Doppler ultrasound was 0.76 m/s to 1.25 m/s.Conclusions Blood flow in pulmonary veins should be carefully observed in prenatal ultrasonography,and anomalous pulmonary venous connection should be considered when pulmonary veins do not connect to the left atrium.

Síndrome de cimitarra, valoración con ecocardiografía transesofágica intraoperatoria. Informe de caso / Scimitar syndrome; intraoperative transesophageal echocardiography assessment. Case report

9-year-old patient diagnosed with scimitar syndrome through transthoracic echocardiogram and cardiac catheterization. Surgical correction is performed by anastomosing the collector of the pulmonary right vein to AI through the interatrial septum, tunneling it with a pericardic patch. The study findings and echocardiographic images of such surgical repair and the spectrum of pulsed Doppler differentiating between right and left pulmonary veins will be shown below. The connection through the septum was evidenced, simulating an interatrial communication. A difference was found among the measured points S, D, A and between the left and right pulmonary veins, without this resulting in a stenosis during the repair.

Paciente de 9 años de edad el cual es diagnosticado con síndrome de cimitarra a través de ecografía transtorácica y cateterismo cardiaco. Se realiza cirugía correctora la cual se anastomosa el colector de vena pulmonar derecha hacia AI a través del septum interauricular, tunelizándolo con parche pericárdico. A continuación se muestran los hallazgos e imágenes ecocardiográficas de tal reparación y el espectro doppler pulsado diferenciado entre las venas pulmonares derechas e izquierdas. Se pudo evidenciar la conexión a través del septum, simulando una comunicación interauricular. Se halló una diferencia entre los puntos medidos S, D, A entre las venas pulmonares izquierdas y derechas, sin que esto significara una estenosis en la reparación.

The diagnosis and different treatment of scimitar syndrome in 14 cases / 中华胸心血管外科杂志

Objective Investigate the clinical features,diagnosis and treatments of the scimitar syndrome,and different forms of treatment to alleviate pulmonary hypertension.Methods A retrospective analysis of clinical data of 14 children with scimitar syndrome from 2013 to 2017,including clinical symptoms and signs,chest X ray,echocardiography,chest CT and cardiac catheterization,treatment outcome and follow-up.Assess embolization of systemic pulmonary collateral and pulmonary venous drainage correction surgery,which is better for lowering pulmonary blood flow.Results 14 patients with scimitar syndrome were diagnosed from 2013 to 2017.There were 5 boys and 9 girls;3 cases ＜7 kg in weight.Scimitar syndrome was suspected because of extroversion,and diagnosed by color Doppler echocardiography and 13 of them confirmed by cadiac CT scan when ascimitar vein was detected entering the inferior vena cava.11 patiens had right lung dysplasia and 4 had horseshoe lung.Three patients had severe pulmonary arterial hypertension,3 had moderate to severe pulmonary arterial hypertension,and 2 had moderate pulmonary arterial hypertension,the left had slight pulmonary arterial hypertension.4 patients had pulmonary venous drainage correction surgery,after that 2 of them had systemic pulmonary collateral embolism.6 patients systemic pulmonary collateral embolism first,then 4 of them had surgical repair,1 case of 13 years old asymptomatic child without surgery.1 patient with heart failure,severe pulmonary hypertension,pulmonary infection,died before surgery,while another died after surgical repair.At last 1 patient was lost for follow-up visits.Systemic pulmonary collateral embolism and pulmonary venous drainage correction surgery could all reduce blood flow of pulmonary.After systemic pulmonary collateral embolism,patients had slight pulmonary arterial hypertension just need follow-up visits.Conclusion Clinically,found children with heart of dextrocardia position,growth retardation,recurrent lung infections,unexplained right heart failure,pulmonary hypertension,should consider the possibility scimitar syndrome.Whether pulmonary vascular embolization or surgical repair,can significantly reduce pulmonary artery's blood flow and alleviate pulmonary hypertension to protect pulmonary,even reduced the incidence of pneumonia and mortality.So we suppose ealy pulmonary hypertension in scimitar syndrome patients maybe dynamic pulmonary hypertension.

Cierre percutáneo de conducto arterioso mediante un asa arteriovenosa en un paciente con síndrome de la cimitarra / Percutaneous closure of ductus arteriosus through an arteriovenous loop in a patient with scimitar syndrome

Resumen: Introducción: El síndrome de la cimitarra consiste en una rara malformación, caracterizada por una conexión anómala parcial de una o ambas venas pulmonares derechas a la vena cava inferior, hipoplasia de pulmón derecho y circulación sistémica desde la aorta descendente. El síndrome de la cimitarra en ocasiones se asocia con otras malformaciones congénitas, entre las que se incluye la persistencia del conducto arterioso (PCA). Caso clínico: Paciente de sexo femenino de cuatro años de edad con síndrome de la cimitarra, variedad ''adulto'', asociado con PCA sintomático. Se realizó exitosamente oclusión del conducto mediante un asa arteriovenosa femoral con un dispositivo Amplatzer® PDA, sin complicaciones. Conclusiones: El manejo del síndrome de la cimitarra es complejo y amerita de un estudio hemodinámico completo para determinar el tratamiento adecuado. La hipertensión arterial pulmonar es un factor de mal pronóstico.

Abstract: Background: Scimitar syndrome consists in a rare malformation characterized by a partial abnormal connection in one or both right pulmonary veins to the inferior vena cava, right lung hypoplasia and systemic circulation from the descending aorta. Scimitar syndrome is occasionally associated to other congenital malformations, such as patent ductus arteriosus (PDA). Case report: We report a 4-year-old patient with ''adult'' variety of scimitar syndrome associated to symptomatic PDA, which was successfully occluded using retrograde guidewire-established femoral arteriovenous loop with an Amplatzer® PDA occluder, without complications. Conclusions: Scimitar syndrome is complex and requires a complete hemodynamic study for the determination of the appropriate treatment. Pulmonary arterial hypertension is a factor associated with poor prognosis.

Progress in clinical diagnosis and treatment of scimitar syndrome / 国际儿科学杂志

Scimitar syndrome is a rare congenital cardiopulmonary anomaly,consisting of partial anomalous pulmonary venous return,right lung hypoplasia,and several associated defects.The condition generally has significant morbidity and mortality,but its etiology is poorly understood.Scimitar syndrome has a wide spectrum of clinical presentations ranging from severe respiratory difficulty and high mortality in the perinatal period to asymptomatic and incidental in adulthood.Its diagnosis is mainly dependent on angiocardiography and imging methods.The adult combined with scimitar syndrome are usually given symptomatic treatment.If severe symptoms appears,its primary treatment is surgery to correct anomalous drainage.In the article,the research progress of the pathological anatomy,familial scimitar ayndrome,the clinical diagnosis,clinical therapy and prognosis are reviewed.

Clinics in diagnostic imaging (173). Scimitar syndrome with horseshoe lung

A 56-year-old Chinese man presented with giddiness and vertigo. Subsequent chest radiography showed the classic scimitar sign of an abnormal pulmonary venous return. Further evaluation with non-contrast computed tomography substantiated the finding of a partial anomalous venous drainage pattern and identified an associated rare lung anomaly, horseshoe lung. The imaging findings of scimitar syndrome and its association with horseshoe lung are reviewed.

Síndrome de la cimitarra. Correlación anatomoembriológica / Scimitar syndrome. Correlation anatomo-embryological

Resumen Objetivos Describir morfológicamente un bloque visceral toracoabdominal de un caso de síndrome de la cimitarra. Proponer una hipótesis patogenética que explique el desarrollo de las conexiones venosas pulmonares de este síndrome. Método El espécimen anatómico se describió con el sistema secuencial segmentario. Se determinó el situs, las conexiones entre los segmentos cardíacos y las anomalías agregadas. Se describió la anatomía de ambos pulmones, incluida la conexión venosa pulmonar. Se elaboró una hipótesis patogenética que explica la conexión venosa pulmonar a través de una correlación entre la patología del síndrome y el desarrollo normal de las venas pulmonares. Resultados El situs fue solitus, las conexiones entre los segmentos cardíacos fueron normales, existió hipoplasia y displasia del pulmón derecho con secuestro del lóbulo inferior; las venas pulmonares derechas drenaron a un colector curvo en la porción suprahepática de la vena cava inferior y las venas pulmonares izquierdas lo hicieron a la aurícula izquierda. El secuestro pulmonar recibió irrigación a través de una colateral aortopulmonar. Existió una comunicación interauricular. Conclusiones La hipótesis patogenética propone que las conexiones venosas pulmonares de este síndrome representan la persistencia del horizonte XIV de Streeter (28-30 días de desarrollo), período en el que el seno de las venas pulmonares presenta conexión venosa doble con la aurícula izquierda y con un colector de la conexión primitiva que desemboca en la vitelina derecha de la que se deriva la porción suprahepática de la vena cava inferior.

Abstract Objectives To describe morphologically a toracoabdominal visceral block of a scimitar's syndrome case. We propose a pathogenetic theory wich explains the development of the pulmonary venous connection in this syndrome. Method The anatomic specimen was described with the segmental sequential system. The situs was solitus, the connections between the cardiac segments and the associated anomalies were determined. The anatomy of both lungs, including the venous pulmonary connection, was described. A pathogenetic hypothesis was made, which explains the pulmonary venous connection throw a correlation between the pathology of this syndrome and the normal development of the pulmonary veins. Results The situs was solitus, the connections of the cardiac chambers were normal; there were hypoplasia and dysplasia of the right lung with sequestration of the inferior lobe; the right pulmonary veins were connected with a curved collector which drainaged into the suprahepatic segment of the inferior vena cava; the left pulmonary veins were open into the left atrium. The sequestered inferior lobe of the right lung received irrigation throw a collateral aortopulmonary vessel. There was an atrial septal defect. Conclusions The pathogenetic hypothesis propose that the pulmonary venous connection in this syndrome represent the persistent of the Streeter's horizon XIV (28-30 days of development), period in which the sinus of the pulmonary veins has double connection, with the left atrium and with a primitive collector into the right viteline vein which forms the suprahepatic segment of the inferior vena cava.

The diagnosis and treatment of scimitar syndrome in 6 cases / 中华胸心血管外科杂志

Objective Investigate the clinical features,diagnosis and treatment of the scimitar sydrome.Methods A retrospective analysis of clinical data of six children with scimitar syndrome from 2008 to 2014,including clinical symptoms and signs,chest X-ray,echocardiography,chest CT and cardiac catheterization,treatment outcome and follow-up and review of the literature.Results Six patients with scimitar syndrome were diagnosed between 2008 and 2014.There were 3 girls and 3 boys;5 cases ＜ 6 kg in weight.Scimitar syndrome was suspected in 5 cases because of extroversion,and diagnosed by color Doppler echocardiography in all 6 when a scimitar vein was detected entering the inferior vena cava.Computed tomography confirmed the diagnosis in all patients.One patient had horseshoe lung,3 had a unilobar right lung,3 had a hypoplastic right lower lobe.Three patients had severe pulmonary arterial hypertension,1 had moderate to severe pulmonary arterial hypertension,and 2 had moderate pulmonary arterial hypertension.All patients had lower respiratory tract infections,volume loss of the right lung,a normal or hyperinflated left lung,dextroversion of the heart,and scimitar arteries from the descending aorta.4 cases of pulmonary venous drainage correction surgery,3 underwent body-pulmonary embolism,1 case of 9-year-old asymptomatic children without surgery,and 1 patient with heart failure,severe pulmonary hypertension,pulmonary infection,septicemia died before surgery.Conclusion Clinically,found children with heart of dextrocardia position,growth retardation,recurrent lung infections,unexplained right heart failure,pulmonary hypertension,should consider the possibility scimitar syndrome.Early diagnosis and early treatment of pulmonary venous drainage and pulmonary vascular embolization,can significantly improve the clinical symptoms in children and reduce mortality.

Multiple Detector Computed Tomography Evaluation of Congenital Pulmonary Venolobar Syndrome: Scimitar Syndrome with Pulmonary Arterio-Venous Fistula.

The term dysmorphic lung has been introduced to describe any complex congenital malformation that involves both abnormal pulmonary vascular morphology and disordered growth of the entire lung. The major group within this definition of the dysmorphic lung is the scimitar syndrome. Scimitar syndrome is a rare anomaly consisting of partial anomalous pulmonary venous return into the supra or infradiaphragmatic portion of the inferior vena cava, right lung hypoplasia, dextroposition of the heart, and anomalous systemic arterial supply from the aorta or one of its branches to the right lung. It can be associated with congenital cardiovascular defects namely dextrocardia, coarctation of the aorta, Tetralogy of Fallot, patent ductus arteriosus, atrial or ventricular septal defect, and right pulmonary artery hypoplasia. Associated pulmonary anomalies are lung hypoplasia, sequestration, and tracheobronchial anomalies. Thus, it can manifest with heart failure and recurrent pneumonia. Other reported associated anomalies include bronchogenic cysts, horseshoe lung, accessory diaphragm, and hernias. We report an 8-year-old boy with complaint of chest pain, who was diagnosed as having scimitar syndrome with rare association of pulmonary arteriovenous fistula within the involved lung and role of newer faster multiple detector computed tomography scanner in its evaluation.

Scimitar syndrome: a rare congenital venolobar anamoly.

Scimitar syndrome is a rare congenital anomaly presenting with partial anomalous pulmonary venous drainage from right lung to inferior vena cava. This is the rarest anomaly seen in 1 to 3 in 100000 births. Embryologically it is thought to be a primary developmental anomaly of lung with secondary anomalous venous drainage. This is seen commonly in right lung and is common in females. Most of the cases are asymptomatic or minimally symptomatised. If symptoms are present patient usually presents with pulmonary hypertension and symptoms of left to right shunt. Clinically this syndrome is diagnosed by chest x- ray by the presence of “Scimitar sign”. Surgical treatment effective in this syndrome.