aortopulmonary window ( apw ) or aortopulmonary septal defect , first described by elliotson in third decade of last century , is a cardiac abnormality that results from abnormal communication between the proximal aorta and the main pulmonary artery in the presence of two normally separated aortic and pulmonary valves . apw is a relatively rare cardiac lesion representing approximately 0.2% to 0.6% of all cardiac malformation.[24 ] apw could be isolated , but in half of patients , may be associated with other cardiac malformations . the most common associated malformations are arch abnormalities , specifically coarctation of the aorta , type a interruption of aortic arch , asd , tetralogy of fallot , and aortic origin of the right pulmonary artery . more rarely , it is associated with ventricular septal defect , pulmonary or aortic atresia , transposition of great arteries and tricuspid atresia.[69 ] apw can be classified into 3 types : proximal , distal and total defects . proximal defects are the most common and are located in proximal aorta above the sinus of valsalva . distal types are located in the upper portion of the ascending before the aortic branches . finally , total defects are large and involve the majority of the ascending aorta between valsalva and aortic branches . the clinical features of apw are not specific , but majority of patients have the manifestations of a large left to right shunt . patients with large apw usually have symptoms of pulmonary hypertension and congestive heart failure ( tachypnea , diaphoresis , failure to thrive , and recurrent respiratory difficulty ) in the first weeks of life . two - dimensional and doppler echocardiography could diagnose apw and could describe associated anomalies in majority of patients . closure of the defect is offered in all patients with apw and should be performed as soon as possible . without surgical correction , 40 - 50% of patients will die due to congestive heart failure during the first year of life , and a large number of survivors will suffer from sequels of congestive heart failure or pulmonary vascular disease in their lives . wright et al . described first transaortic closure of an apw after cardiopulmonary bypass use in 1968 . deverall et al . described using a patch to close of an apw . in this report , we have presented an 8-month - old boy with apw who was referred to our center by respiratory symptoms and heart murmurs . an 8-month - old boy , 6 kg , was referred to our hospital with a history of recurrent upper and lower respiratory infections , progressive tachypnea , and obvious chest retraction at rest and heart murmurs . in last examination transtorasic echocardiography showed moderate degree of mitral regurgitation ( mr ) , dilated left atrium and left ventricle , severe turbulence flow in pulmonary artery and large apw ( 7 - 8 mm ) and  t  artifact at the edge of the defect that distinguishes it from normal dropout [ figure 1 ] . high parasternal short - axis echocardiogram is showing the aortopulmonary window between the ascending aorta ( ao ) and main pulmonary artery ( pa ) at cardiac catheterization we found a large apw with pulmonary to aortic flow ratio above 3 and elevated pulmonary pressure ( 70% of systemic arterial pressure ) [ figure 2 ] . aortic angiography is showing filling of the main pulmonary artery through the aortopulmonary window ( arrow ) the patient was operated after diagnosis by transaortic approach using a median sternotomy and cardiopulmonary bypass . echocardiography was performed and showed normal left ventricular function , and no residual aortopulmonary defect . no evidence of narrowing or stenosis of aorta and pulmonary arteries and no distortion of semilunar valve were found by echocardiographic study . at the fetal life , abnormal growth of aorticopulmonary septum , due to one of the rarest congenital heart defects , apw apw is a defect between two main great arteries in the presence of two separate aortic and pulmonary semilunar valves . truncus arteriosus can be differentiated from this anomaly by absence of two separated normally aortic and pulmonary valves . in large defects this lesion often mimics as a large intra or extracardic shut such as ventricular septal defect , atrioventricular septal defect or patent ductus arteriosus ( pda ) and resulting in congestive heart failure , pulmonary hypertension and progressive of pulmonary vascular disease . nearly 50 - 70% of all patients with apw were associated with other cardiac malformations.[1921 ] several cardiac malformation could be seen include coarctation of the aorta , type a interruption of aortic arch , ventricular septal defect , atrial septal defect ( asd ) , pda , transposition of the great arteries , and tetralogy of fallot . one of these classifications separates apw into 3 types , proximal or type a , distal or type b , and total or type c. proximal defect which is the most common type is a small defect located between the semilunar valves and the pulmonary bifurcation . distal defects are located in the upper portion of the ascending aorta before aortic branches near the pulmonary biforcation . the rarest type or total defects consist of the entire aortopulmonary septum . in the past , the defect can be best visualized from a different views by echocardiographic study , including parasternal short and long - axis views , high parasternal short - axis view and subcostal coronal view . echocardiographic study helps us to identify the morphological features and typing of the defect , exact location , sizing of defect , and associated cardiac anomalies . at doppler echocardiography , abnormal continuous forward flow in the pulmonary arteries indicates the presence of an aortopulmonary communication . closure of apw is indicated in all patients and it should be performed as soon as possible after diagnosis . successful repair of an apw was reported from several years ago and many authors have reported their results . transaortic direct closure of an apw was first reported by wright and coworkers in 1968 . in 1969 , deverall et al . currently , several patients have had successful closure using various catheter - delivered devices.[2629 ] transaortic approach using a median sternotomy and cardiopulmonary bypass was performed for our patient . neither pulmonary stenosis or distortion nor coronary artery injury was found . during the follow - up period ( one year ) , most patients with apw develop severe clinical symptoms such as congestive heart failure or pulmonary hypertension during early stages of life . early surgical closure or device employment is indicated as soon as the diagnosis is established to prevent congestive heart failure and pulmonary hypertension . repair of apw have good results and indicated as soon as possible , even in early infancy . the outcome of these patients is excellent if correction is performed before complication of disease such as pulmonary hypertension .
one of the rarest congenital heart diseases that results from a defect between the main pulmonary artery and the proximal aorta is named aortopulmonary window ( apw ) 
 . such abnormality could be isolated , but in fifty percent of patients may be associated with other cardiac abnormalities , including arch abnormalities , specifically coarctation of the aorta , interrupted aortic arch , tetralogy of fallot , and atrial septal defect ( asd ) . surgical closure or catheter - delivered devices 
 is recommended in all patients with apw and should be performed after diagnosis as soon as possible to prevent irreversible pulmonary vascular disease . in the current era , early mortality following repair of simple 
 apw is low and depends on the presence of associated lesions , especially interrupted aortic arch . 
 we report an 8-month - old boy with apw who was referred to our center by respiratory symptoms and heart murmurs .