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What is Scimitar Syndrome
Scimitar Syndrome is a kind of Congenital Heart Defect ( CHD ) which is also referred to as Hypogenetic Lung Syndrome or Pulmonary Venolobar Syndrome . This sort of disease is very rarified and principally characterized by anomalous pulmonary venous drainage to the inferior venacava.(1,2 )
Scimitar Syndrome was first identify in the year 1960 by Catherine Neil . Halasz et . al . first used the full term ‘ Scimitar ’ in orderliness to describe the radiographic appearance of an anomalous pulmonary vein.(3)When it is characterized by the partial anomalous drain it is call Partial Anomalous Pulmonary Venous Return ( PAPVR ) , but if it is characterized by total anomalous drainage then it is call as Total Anomalous Pulmonary Venous Return ( TAPVR ) . The syndrome which is mainly associated with Partial Anomalous Pulmonary Venous Return ( PAPVR ) is more commonly live as Scimitar Syndrome.(1 )
Symptoms of Scimitar Syndrome
A lot of people suffering from scimitar syndrome remains asymptomatic and are able to direct a normal life-time . At times , it is very hard to detect the symptoms of this disease since there are many patients who have Scimitar Syndrome yet were diagnose only in their adolescence or adulthood as they stay symptomless or gently symptomatic for several years of their life . The major characteristic feature of scimitar syndrome includes correct pulmonic hypoplasia resulting in the moving of the heart and soul to the right side ( cardiac dextroposition ) , developing right pulmonic artery.(1,3)At time , some patients may also have an anomalous systemic arterial line of descent supply to the hypoplastic lung from the descending aorta.(4)In decree to name scimitar syndrome , several investigation need to be done over some menstruation of years to detect the hardness of the disease or associated abnormalities .
Diagnosis of Scimitar Syndrome
To diagnose Scimitar Syndrome , doctors mainly demand for the observe test :
Prevalence and Epidemiology of Scimitar Syndrome
The incidence of scimitar syndrome is observe to be 1 to 3 per 1,00,000 springy births having a 2:1 distaff predominance.(5,6)However , the true relative incidence can be higher as many patients are symptomless . A large number of patients are by the bye diagnose while doing test fordyspnea , continuing weariness , bureau shadowgraph or when a individual is evaluated for recurrent chest infections.(7,8,9 )
Treatment for Scimitar Syndrome
When it comes to treatment of scimitar syndrome , operating theater should be considered in the presence of significant left hand to correct shunting as well aspulmonary hypertension . While doing the surgery , the surgeon needs to make an interatrial baffle so that the pulmonary venous blood can retrovert into the unexpended atrium . However , there is another cognitive operation that the surgeon can follow in plow scimitar syndrome and that is re - implanting the anomalous vein directly into the left atrium.(4)In asymptomatic cases , the affected role is commonly observed and only if symptom surface , it is managed through conservative criterion . If anomalous arterial blood supply from the come down aorta to the hypoplastic right lung is confirmed , then embolization of the MAPCA ( Major Aortopulmonary Collateral Artery ) should be done.(2 )
Conclusion
Scimitar Syndrome is a congenital pulmonary malformation of heart , lungs and parentage vessel . This disease is rare make more predominance in female . People who are asymptomatic are able to live a normal life without much complication . When soul suffer from scimitar syndrome has Centennial State - morbidity , the condition can be severe and the prognosis can be poor specially in type of baby .
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