bland - white - garland ( bwg ) syndrome is a congenital heart defect in which the left coronary artery abnormally branches off the pulmonary trunk .
its prevalence is approximately 1 : 300,000 births , which corresponds to approximately 0.4% of all congenital heart defects [ 1 , 2 ] .
it is the most frequent cause of myocardial infarction in children ; mortality among untreated individuals reaches 90% , while the remaining 10% survive due to the existence of collateral circulation from the right coronary artery . depending on the extent of the connections between the coronary arteries , bwg syndrome may present itself at elderly age or remain asymptomatic throughout the patient 's life [ 2 , 3 ] . symptoms of congestive heart failure , myocardial ischemia , left ventricular contractility impairments , or impairments in the functioning of the mitral valve
the syndrome 's subjective manifestations may include dyspnea , fatigability , tearfulness , or paleness [ 2 , 4 ] .
the present study presents cases of two children in whom extracorporeal membrane oxygenation ( ecmo ) was employed after the surgical repair of bwg syndrome and one child in whom ecmo was not required after this procedure .
a 12-year - old boy diagnosed with bwg syndrome and secondary dilated cardiomyopathy was admitted in order to undergo surgical treatment .
physical examination revealed a systolic murmur above the apex of the heart ; no other abnormalities were found . left ventricular contractility was in the lower range of normal levels .
retrograde coronary blood flow was observed from the opening of the left coronary artery in the pulmonary trunk .
the location of the coronary opening on the posterolateral wall of the pulmonary trunk precluded coronary artery transplantation . therefore , using extracorporeal circulation and takeuchi 's procedure , a tunnel was formed inside the pulmonary artery from the outlet of the left coronary artery to the created aortopulmonary window .
a 4-month - old girl diagnosed with a hemodynamically significant defect  bwg syndrome , third - degree mitral valve insufficiency , and left ventricular heart failure was admitted in order to undergo a repair procedure .
preoperative electrocardiography ( ecg ) revealed st - segment elevation in precordial leads v5 and v6 as well as elevation of cardiac markers . the cardiac ejection fraction ( ef )
38% , and left ventricular end - diastolic ( lved ) diameter  at approx .
cardiac catheterization demonstrated that the trunk of the left coronary artery branched off the posterolateral wall of the pulmonary trunk , which , considering the distance , precluded the implantation of a vascular graft into the aorta .
the procedure was performed under extracorporeal circulation ; the pulmonary artery was incised transversely above the origin of the left coronary artery ; subsequently , a strip of the pulmonary trunk was excised together with the outlet of the left coronary artery .
a strip of the aorta , pedicled on the posterior wall , was incised and sutured to the pulmonary trunk strip , creating a tunnel from the outlet of the left coronary artery to the ascending aorta , running outside the pulmonary trunk .
the defect in the wall of the pulmonary trunk was repaired with preserved bovine pericardium .
postoperatively , on the day of the procedure , significant left ventricular failure was observed along with impairment of contractility 
a decision was made to introduce mechanical left ventricular support using venous - arterial extracorporeal circulation .
on the 4 postoperative day , circulatory support was disconnected . due to pneumonitis , the patient remained in the intensive care unit until the 24 postoperative day .
a control cardiac echo performed at discharge from the icu demonstrated ef of 32% , lved diameter of 36 mm , and mitral insufficiency reduced to the second degree . during the following days
, the clinical condition of the child improved as the indices of inflammation subsided systematically .
, the patient was transferred to the pediatric cardiology ward , where she remained for the next four weeks .
she was discharged home on the 51 postoperative day in good general condition and stable circulatory and respiratory status .
although cardiac ultrasonography revealed generalized left ventricular hypokinesia , it also demonstrated a significant improvement of the ejection fraction ( ef = 48% ) in comparison to previous examinations ; the mitral insufficiency was still present , but its gradual regression was observed .
a 6-month - old girl with body mass of 4.6 kg was referred to our center in order to undergo surgical repair of bwg syndrome .
her records from the previous hospital included the diagnosis of third - degree mitral insufficiency and left ventricular heart failure .
the ejection fraction was 18% ; left ventricular dilatation with an akinetic apical aneurysm was observed .
as in the case of the previous patient , cardiac catheterization demonstrated that the whole left coronary artery branched off the posterolateral wall of the pulmonary trunk .
the performed procedure employed the same technique that was used in the case of the previous patient and also involved the closure of asd .
therefore , a decision was made to introduce mechanical circulatory support instead of extracorporeal circulation and to maintain it during the postoperative period .
on the 2 postoperative day , cardiac echo demonstrated ef of 19% , lved diameter of 36 mm , and a reduction of mitral insufficiency from the third to second degree . on the 3 postoperative day
on the 6 day , neurological symptoms appeared in the form of pathological muscle tension and eye movements to the right side . at this point , the patient underwent computed tomography of the head .
the examination revealed the presence of fresh diffuse ischemic lesions in both temporal , frontal , and occipital lobes ( caused by numerous embolisms originating from the akinetic left ventricular apex ) .
on the 30 day , the patient was transferred to the pediatric cardiology ward , where she continued to undergo catecholamine therapy .
a systematic regression of neurological symptoms was noted , but left - sided hemiparesis persisted .
after further seven weeks of treatment , the patient was discharged home in good general condition and stable circulatory and respiratory status .
regular follow - up visits to cardiology and neurology clinics were recommended to the patient .
the following techniques are reported to be feasible in this context : creating a bypass from the aorta to the left coronary artery or the descending branch of the left coronary artery ( lad ) , transplanting the left coronary artery along with its outlet from the pulmonary trunk to the aorta , or forming a tunnel from the wall of the pulmonary trunk , connecting the left coronary artery with the aorta and running inside the pulmonary trunk  using takeuchi 's method    or outside the pulmonary trunk .
the method which appears to offer best results consists in transplanting the left coronary artery to the aorta .
its use is limited by anatomic variants which preclude repairing the defect in this manner [ 5 , 6 ] .
in this situation , takeuchi 's technique may be used , or a connecting tunnel may be created outside the pulmonary trunk [ 5 , 6 ] .
this method of repair was used in patients 2 and 3 , while takeuchi 's procedure was employed in patient 1 .
this technique offers very good early results , but is sometimes followed by long - term complications such as pulmonary artery stenosis or shunt from the tunnel to the lumen of the pulmonary trunk .
although patients 2 and 3 exhibited third - degree mitral insufficiency , no repair procedure was attempted , as the insufficiency was caused by generalized hypokinesia and left ventricular dilatation . in such cases , it is difficult to assess the actual degree of insufficiency . although mechanical circulatory support employing extracorporeal circulation has been used for many years in the treatment of heart and lung diseases , clear qualifying criteria for its use are still lacking .
the primary reasons for its use in children include a low ejection fraction after cardiac surgery procedures [ 7 , 8 ] .
the most significant complications resulting from using this method include bleeding , sepsis , and ischemic or hemorrhagic changes within the central nervous system .
longer duration of therapy entails a lower chance of recovery and a higher risk of complications .
the subject literature features reports of cases similar to ours in that they required the use of mechanical circulatory support after the surgical repair of bwg syndrome [ 1 , 6 ] .
despite the risk of numerous complications , mechanical circulatory support offers a chance of survival to patients with a low left ventricular ejection fraction . as this is a frequent problem after the surgical repair of bwg syndrome , the authors suggest that such repair should only be performed in centers that are able to provide mechanical circulatory support .
 the anomalous origin of the left coronary artery arising from the pulmonary artery ( alcapa ) , also known as bland - white - garland ( bwg ) syndrome , is a rare congenital heart disease . 
 we present cases of three children in whom bwg syndrome was repaired surgically . in two of them , the left coronary artery was transplanted from the pulmonary trunk to the aorta , and in one , the takeuchi procedure was performed . in both cases in which the left coronary artery was transplanted to the aorta , mechanical circulatory support was used after the surgery . 
 this was due to a low ejection fraction ( 10% ) while weaning from cardiopulmonary bypass . 
 although associated with numerous complications , mechanical circulatory support can be a lifesaving therapy in patients with a poor left ventricular function after the correction of bwg syndrome . 
Introduction
Case 1
Case 2
Case 3
Discussion
Conclusions
Disclosure