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: dr s venkatesan 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
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