VSD with pulmonary atresia : Management issues

VSD with Pulmonary atresia is a complex form of cyanotic heart disease .Though it’s  a close  companion of Tetrology of Fallot  physiologically, it is a vastly different entity in embryological and anatomic terms. TOF is cono truncal anomaly where abnormal anterior displacement of conal septum result in malalignment VSD, RVOT obstruction ,aortic override and RVH. While ,pulmonary atresia with VSD  is not a primary cono truncal anomaly, the defect occurs much earlier than TOF in fetal life , where the origin of PA fails to materialise,(Fetal arteritis?) and which triggers a series of anatomical disarray in pulmonary arterial circulation.  The PA growth arrests in various levels (Somerveille Types) .It is important to realise while the PA may be patent , pulmonary valve is always atretic and disconnected from RV. In severe forms there is Zero pulmonary artery content .The lung is perfused in chaotic manner. This situation akin to “TAPVC” in arterial side  and result in  total anomalous pulmonary arterial connection. Natural History of PA with VSD . The blood supply of lungs is maintained by  MAPCAS.Since , the  fetus is not dependent on its lung for survival,  life goes on well , till  birth  and face the harsh reality that  it has no independent blood supply for  lungs from RV  and has to depend on collaterals from aorta. Survival depends upon the  the quantum of collateral .( Size , number, arborisation pattern etc). Life is shortened in mos...
Source: Dr.S.Venkatesan MD - Category: Cardiology Authors: Tags: cardiology congenital heart disese aorto pulmonary collaterals hemoptysis in mapcas pulmonary atresia and vsd K S murthy KM cherian madras medical mission Innova children's hospital pulmonary atresia vsd tetrology single vs multiple stage unifocal Source Type: blogs