congenital pulmonary vein stenosis occured due to failed incorporation of common right and/or left pulmonary vein into the left atrium . main pulmonary artery branch or ppas should be suspected when a continuous murmur is heard or chest x - ray shows post stenotic dilatation or area of decreased vascularity . below is a case report of a 13 year old boy who presented with a rare combination of congenital pulmonary vein stenosis along with congenital stenosis of right pulmonary artery . a 13-year - old male patient presented to cardiology outpatient department with a complaint of easy fatigability for last 2 years . , he had blood pressure of 100/70 mmhg , pulse rate of 78/min ( regular ) , grade iii parasternal heave , and loud and palpable second heart sound in left second intercostal space . a systolic ejection murmur of grade 3/6 intensity was audible in left second intercostal space . echocardiography with color doppler revealed moderate pulmonary artery hypertension ( pulmonary artery systolic pressure of 55 mmhg ( using mahan 's formula ) , turbulent , continuous , and high velocity flow consistent with severe stenosis of both left superior and inferior pulmonary vein and also right inferior pulmonary vein as viewed in modified parasternal short axis view [ figure 1 ] . a pressure gradient of 32 mmhg was documented at the insertion of right inferior pulmonary vein in left atrium in apical four - chamber view [ figure 2 ] . a stenosis of the right pulmonary artery was also detected distal to the bifurcation of main pulmonary artery [ figure 3 ] . a pressure gradient of approximately 100 mmhg was observed at the level of the right pulmonary artery in parasternal short axis view [ figure 4 ] . computed tomography ( ct ) angiogram of thorax confirmed the stenosis of three pulmonary veins as well as branch stenosis of the right pulmonary artery . ct scan revealed dilated main pulmonary artery ( diameter = 3.9 cm ) and dilated left pulmonary artery ( diameter = 2.7 cm ) . right pulmonary artery ( diameter of proximal part = 1.7 cm ) had a focal narrowing in distal part ( diameter = 0.49 cm ) . narrowing was seen at junction of bilateral inferior and left superior pulmonary vein with left atrium . the diameter of the right inferior pulmonary vein was 2.2 mm , and left superior and inferior pulmonary vein was 3.9 mm each . turbulent flow detected at the insertion of left upper pulmonary vein and left lower pulmonary vein seen in modified parasternal short axis view . a pressure gradient detected at the level of insertion of right inferior pulmonary vein in apical four - chamber view . it occurs due to failed incorporation of common right and/or left pulmonary vein into the left atrium . usually , the condition is diagnosed in older infants and children , but it may be manifested as severe persistent pulmonary hypertension of the newborn . the most common cause of acquired pulmonary vein stenosis is post - radiofrequency ablation for treatment of atrial fibrillation . the condition is relentlessly progressive causing 50% death in 5 years even after successful surgery . initial experience with cutting balloon angioplasty was successful as palliative procedure though longest interval before repeat intervention was only 6 months . nowadays , ppas can lead to pulmonary hypertension but is likely misdiagnosed as either idiopathic pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension . therefore , a high index of suspicion during the initial evaluation of pulmonary hypertension is essential for its prompt diagnosis and adequate treatment . a combination of predominantly left - sided pulmonary vein stenosis ( as both the left upper and lower pulmonary veins were involved ) with right pulmonary artery branch stenosis is very rare , and in this particular patient , development of pulmonary artery hypertension was delayed because of restricted blood flow through right pulmonary artery .
congenital pulmonary vein stenosis is a rare entity caused due to failed incorporation of common right and/or left pulmonary vein into the left atrium . 
 below is a case report of a combination of predominantly left - sided pulmonary vein stenosis with right pulmonary artery branch stenosis . 
 the patient was an adolescent boy with mild symptoms . 
 clinical examination revealed features of pulmonary artery hypertension . 
 echocardiography and computed tomography scan were done to confirm the disease .