Posts Tagged ‘mapcos in tof why uncommon’

Before answering the above question ,  there need to be a correction to the question itself . PDA is persistence of ductus arteriosus . In pulmonary atresia ductus itself is not formed .So  the question should  ideally be  Why  ductus  is absent in pulmonary atresia  with VSD  !

Ductus  is formed from the dorsal portion of left 6th arch  .The sixth arch also gives raise to  right and left pulmonary artery.This can happen only if everything from aortic arch and pulmonary artery development is normal

Ductus  has to connect aorta with left pulmonary artery  , when pulmonary artery itself is  poorly developed or not developed   how can the  ductus  connect to  LPA ?

Pulmonary atresia is essentially a defect in the development of pulmonary arterial tree (Please note pulmonary valve and  MPA  is formed  from different  structures in different times and it  would  get  docked  with developing LPA,RPA  and  subsequently to the rest of the  pulmonary tree .)

In fact , one of the terminologies used for pulmonary atresia with VSD  is ,  total anomalous pulmonary arterial connection(TAPAC)  .In fetal    life,   blood flow in ductus is from RV  to  pulmonary artery  and then to  descending aorta  through the  ductus . When RV is disconnected with pulmonary artery( Rather there is no pulmonary artery )  ductus can not be  formed  for  both anatomical and physiological reasons . Some consider the  left 6th arch  in these patients  would become  a poorly   identifiable  minor Aorto pulmonary channel .

A Link to  3D vedio  of aortopulmonary collateral

Embryology of major aorto pulmonary collaterals.

  • The lung perfusion in patients with pulmonary atresia is important only after birth,  as fetal  lung is largely non functional.
  • In patients with pulmonary atresia with  intact IVS this becomes  critical and  usually death ensues unless intervened.
  • If VSD is present it allows the baby to survive as the  lung gets perfused by major or minor aortopulmonary collaterals.
  • These collaterals can range between extensive and   sparse.  Hence the  symptoms can  also vary from volume overload  /cardiac failure to  severe oiligemia  and recurrent  hypoxic spells.*

MAPCOS ,  if present can connect directly the aorta  to  hilar pulmonary artery or indirectly  from the branches of  aorta (subclavian /LIMA/RIMA etc) . These arteries  supply  with or without  a central confluence . It may enter the lung through the hilum or  away from hilum .The MAPCOS can be located anywhere from the arch of aorta to descending thoracic aorta.It is very rare in the ascending Aorta .

There is also  strong argument for MAPCOS are  nothing but dilated bronchial arteries.(Link to Full text )

*The  natural history  directly depends  on  extent of aorto pulmonary collaterals and its anatomical patency .

Final message

Embryologically   both  the  major  arteries of thorax  Aorta and Pulmonary artery have  the  same parent structure namely the dorsal aorta and its six  arches.Hence there  is no surprise  ,  when these embryological  divisions and fusions   goes awry ,   pulmonary artery fails to get  carved out from  the dorsal aorta  in the normal fashion .The randomly formed pulmonary  arteries continue  to have link  with   the parent -dorsal aorta .These are manifested in various ways as major aorto pulmonary collaterals .

(It is to be noted  in pulmonary atresia  , VSD is an offshoot developmental defect  . Embryologically  VSD   is not linked to the primary defect of pulmonary artery development . This is the reason many would consider   PA with VSD as a  distinct entity with that of TOF (Which is a cono truncal anomaly) This also explains the lack of MAPCOS in true TOF .

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