05-16-2007, 07:07 AM
principles of congenital heart disease
first determine whether it is CYANOTIC or ACYANOTIC? and
whether patient has TWO VENTRICLES or ONE ventricle.
(most of the cyanotic diseases with two ventricles start with T(Tetralogy{complete repair},TGA{Arterial switch operation}, TAPVC{rerouting of pulm veins to LA},TRUNCUS{ VSD Closure with RV to pA valved conduit},these are usually two ventricles and treatment is surgical correction)
HYPOPLASTIC LEFT heart(this presents with severe heart failure) and Tricuspid atresia are usually cyanotic diseases with ONE ventricle.
treatment is three stage palliation starting with BT Shunt( at birth), Glenn shunt +/- Atrail septectomy( SVC To PA anastamosis @ 6 months) followed by Fontan(@ 2-4 years (connect all systemic venous drainage to PA)
you keep these patients on PROSTAGLANDIN to keep PDA open till some procedure is done(THE PRINCIPLE FOR SINGLE VENTRICLE REAPIR IS TO CONVERT HUMAN HEART TO AMPHIBIAN HEART!)
Some PRENATAL predispositions : Maternal diabetes is associated with transposition of the great arteries ,ventricular septal defect.
Drugs taken in pregnancy may lead to cardiac defects. Examples include pulmonary or aortic stenosis in fetal hydantoin syndrome and Ebstein's anomaly and other cardiac defects with maternal lithium treatment
SOme syndromes associated with cong diseases
Downs: AV canal defects
cong rubella syndrome
DA or peripheral pul stenosis
neoanatl lupus: congenital heart blocks
william Syndrome : supra valvular aortic stenosis
Common acyanotic diseases are
ASD,VSD,PDA, AP window
ASD: examination Fixed second heart sound
diagnosis: echo( cath if there is associated lesions or old patients and
suspected eissemmengers)
Treatment: closure of an atrial septal defect recommended when the ratio of pulmonary blood flow to systemic blood flow (Qp/Qs) is greater than 1.5:1 to 2:1 exception is you do closure if there is history of emboli(closure either by surgery or asd device in cath lab, now a days they do closures just with TEE)
VSD: Surgical treatment (same rules as above)most imp post op complication is heart blocks
PDA: try indomethacin if fails surgical closure or coil in little older patients most imp sur complication injury to recurrent laryngeal nerve
(EISSENMENGER is longterm coplication of this L r Shunts and treatment is heart lung transplantation)
first determine whether it is CYANOTIC or ACYANOTIC? and
whether patient has TWO VENTRICLES or ONE ventricle.
(most of the cyanotic diseases with two ventricles start with T(Tetralogy{complete repair},TGA{Arterial switch operation}, TAPVC{rerouting of pulm veins to LA},TRUNCUS{ VSD Closure with RV to pA valved conduit},these are usually two ventricles and treatment is surgical correction)
HYPOPLASTIC LEFT heart(this presents with severe heart failure) and Tricuspid atresia are usually cyanotic diseases with ONE ventricle.
treatment is three stage palliation starting with BT Shunt( at birth), Glenn shunt +/- Atrail septectomy( SVC To PA anastamosis @ 6 months) followed by Fontan(@ 2-4 years (connect all systemic venous drainage to PA)
you keep these patients on PROSTAGLANDIN to keep PDA open till some procedure is done(THE PRINCIPLE FOR SINGLE VENTRICLE REAPIR IS TO CONVERT HUMAN HEART TO AMPHIBIAN HEART!)
Some PRENATAL predispositions : Maternal diabetes is associated with transposition of the great arteries ,ventricular septal defect.
Drugs taken in pregnancy may lead to cardiac defects. Examples include pulmonary or aortic stenosis in fetal hydantoin syndrome and Ebstein's anomaly and other cardiac defects with maternal lithium treatment
SOme syndromes associated with cong diseases
Downs: AV canal defects
cong rubella syndrome
DA or peripheral pul stenosis neoanatl lupus: congenital heart blocks
william Syndrome : supra valvular aortic stenosis
Common acyanotic diseases are
ASD,VSD,PDA, AP window
ASD: examination Fixed second heart sound
diagnosis: echo( cath if there is associated lesions or old patients and
suspected eissemmengers)
Treatment: closure of an atrial septal defect recommended when the ratio of pulmonary blood flow to systemic blood flow (Qp/Qs) is greater than 1.5:1 to 2:1 exception is you do closure if there is history of emboli(closure either by surgery or asd device in cath lab, now a days they do closures just with TEE)
VSD: Surgical treatment (same rules as above)most imp post op complication is heart blocks
PDA: try indomethacin if fails surgical closure or coil in little older patients most imp sur complication injury to recurrent laryngeal nerve
(EISSENMENGER is longterm coplication of this L r Shunts and treatment is heart lung transplantation)