Congenital cyanotic heart disease 2


#1

Congenital cyanotic heart disease 2

:revolving_hearts:Complete AV Canal Defect (AV Septal Defect, Endocardial Cushion Defect):revolving_hearts:

:sparkling_heart:Complete AV canal defect occurs when the “central" heart fails to develop.
:cupid:The resulting large hole communicates between the atria and ventricles, and the defect also creates a failure of septation of the mitral and tricuspid valve.
:point_right:The anterior and posterior segments of each leaflet join each other through the defect (normally, they are separated), resulting in a common AV valve.
:point_right:The AV valve abnormatiry can cause a significant left- and right-side AV valve regurgitation.
:point_right:In addition, there is left-to-right flow across the atrial and ventricular septal defects.
:point_right:The overall result is alarge left-to-right shunt and valve regurgitation, leading to a cardiac volume overload and HF.
:point_right:This is all due to a defect in the development of the endocardial cushions.
:point_right:This is the most common heart defect in Down syndrome (trisomy 21).
:point_right:About 30:40% of children with down syndrome who have congenital heart disease will have AVSD.
:point_right:About 70% of endocardial cusion desfects is associated with down syndrome.

:broken_heart:The child can present with the murmur of a VSD, as well as a middiastolic rumble that is due to increased pulmonary venous retum and increased diastolic flow across the AV valve.
:point_right:An apical pansystolic murmur of AV valve regurgitation occurs if there is regurgitation through the mitral portion of the AV valve.
:point_right:These infants most often present with heart failure by 2 months of age.
:point_right:Symptoms can start in early infancy, if there is a large left ventricle- to-right atrial shunt or if there is significant valvular dysfunction.

:heartpulse:ECG usually shows severe LAD (northwest axis) and prominent voltage with biventricular hypertrophy.
:point_right:CXR reveal non spesfic generalized cardiomegaly with increased pulmonary blood flow.
:point_right:Echo is diagnostic

:gift_heart:Medical management of HF can be helpful, but early surgery is necessary within the first 6 :12 months to prevent pulmonary vascular disease ( Eisenmenger syndrome).
:point_right:Usually, the best outcomes occur in those with “balanced” defects and similar-size ventricles.
:point_right:Occasionally, 1 ventricle can be small/hypoplastic.
:point_right:Potenial post-operative complications :-
:pushpin:Damage to AV node conduction fibers.
:pushpin:Av valve dysfunction.
:pushpin:Pulmonary hypertension.
:point_right:Children with Down syndrome traditionally have a good chance of having compatible anatomy for correction, but they do require surgical correction early (i.e., before 6 months of age) in order to avoid irreversible pulmonary vascular disease.