Congenital Heart Disease Congenital Heart Disease • Most common type of heart disease among children a) ~ 1% of live births b) most causes unknown i) ~ 10% genetic - e.g., trisomy 21 (Down syndrome) - congenital defect in parent or sibling is greatest risk factor
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Congenital Heart DiseaseCongenital Heart Disease
• Most common type of heart disease among children
a) ~ 1% of live birthsb) most causes unknown
i) ~ 10% genetic- e.g., trisomy 21 (Down
syndrome)- congenital defect in
parent or sibling is greatest risk factor
• Types:a) L to R shuntb) R to L shuntc) obstructions
1.- L to R shuntsL to R shuntsa) ASD, VSD, PDA
i) pulmonary blood flow (ASD)
- NO cyanosisb) RV pressures and Vol.
(VSD,PDA)i) hypertrophyii) PVR (vasoconstriction)
- to prevent edema
c) Over time PVR to that of SVRi) reverses shunt (cyanosis)
2.- R to L shuntR to L shunta) pulmonary blood flow
i) Cyanosis “blueness” of skinb) examples:
i) tetralogy of Fallot ii) great vessel transpositioniii) truncus arteriosusiv) tricuspid atresiav) anomalous pulmonary
venous connection
c) long standing cyanosis is associated with “clubbing”
of the tips of the fingers and toes
3.- Obstructions (of flow)Obstructions (of flow)a) coarctation of the aortab) valvular stenosis
i) aorticii) pulmonary
c) complete obstruction is called “Atresia”
Left to Right ShuntsLeft to Right Shunts
• Most common:a) VSD, ASD, PDA and AVSD
i) VSD most common- close spontaneously
(50%) ii) ASD usually not
symptomatic before 30 yrs
iii) DA remains open after birth- ~ 90% occur as isolated anomaly - reversal of flow with PVR causes cyanosis- PGE will maintain DA
cardiac defects such as obstructive disease
iv) complete atrioventricular canal defect
- all 4 chambers freely communicate (Down
syndrome)
Right to Left ShuntsRight to Left Shunts
• Tetralogy of FallotTetralogy of Fallota) Most common form of cyanotic congenital heart disease
• Defects:a) VSDb) Pulmonary artery stenosis
i) determines clinical outcomec) aorta that overrides VSDd) RV hypertrophy
• Clinical Presentation:a) The onset and degree of
cyanosis depends on:i) severity of the pulmonary
obstruction ii) the size of the shunt.
b) Cyanosis is usually not seen until 3- 6 months of age.
c) The cyanosis is due to right ventricle outflow obstruction causing unoxygenated
blood through the VSD.
• Transposition of the Great Transposition of the Great ArteriesArteries(TGA)(TGA)
a) aorta arises from RVi) is anterior and to the right
of the pulmonary arteryb) pulmonary artery arises from
LVc) total separation of pulmonary
and systemic circulationsi) need a shunt to survive following birth
- VSD stable shunt
- PDA, foramen ovale unstable shunt (close
quickly after birth) need surgery• the aorta originates from the right ventricle, so most of the blood returning to the heart from the body is pumped back out without first going to the lungs.
• the pulmonary artery originates from the left ventricle, so that most of the blood returning from the lungs goes back to the lungs again
Truncus ArteriosusTruncus Arteriosus
• Failure of separation into aorta and pulmonary artery
a) results in single great arteryi) receives blood from both ventricles
- early systemic cyanosisb) accompanying VSDc) pulmonary blood flow
i) danger of irreversible pulmonary hypertension
Tricuspid AtresiaTricuspid Atresia
• Clinical Presentation:a) Cyanosis is almost always
present at birth and is progressive.
b) Etiology/Pathophysiology:i) Due to absence of the
tricuspid valve. This leads to an interatrial right to left shunt, usually through a patent foramen ovale.
ii) It is classified by the absenceof tricuspid valve,pulmonary stenosis,and VSD.
iii)The most Common form is also associatedWith a hypoplastic right ventricle
TRICUSPID ATRESIATRICUSPID ATRESIA1 - atrial septal defect2 - absent tricuspid valve3 - ventricular septal defectBlood is shunted through an atrial septal defect to the left atrium and through the ventricular septal defect to the pulmonary artery. The shaded arrows indicate mixing of the blood.
Total Anomalous Pulmonary Total Anomalous Pulmonary Venous Connection (TAPVC)Venous Connection (TAPVC)
• No pulmonary veins directly join LAa) drain into left innominate vein
or coronary sinusb) PV drain into RA
• ASD or foramen ovale always present
a) allows PV blood to enter LAb) R to L shunt
• Volume and pressure hypertrophy of RV
1 - superior vena cava2 - atrial septal defect3 - left innominate vein4 - pulmonary veinsOxygenated blood returning from the lungs is routed back into the superior vena cava, rather than the left atrium. The presence of an atrial septal defect is necessary to allow partially oxygenated blood to reach the left side of the heart.
Total Total Anomalous Anomalous Pulmonary Pulmonary Venous Venous Connection Connection (TAPVC)(TAPVC)
Coarctation of the Aorta• Narrowing• Males 2:1 vs. female
a) females with Turners frequently have coarctation
• 2 types:a) infantile (with PDA; poor
outcome)i) prior to PDA
- symptoms early in life- cyanosis of lower body
b) adult (without PDA)i) most children asymptomatic until late in lifeii) hypertension in upper
extremitiesiii) hypotension in lower
extremities• Murmurs
a) throughout systole• LV hypertrophy
1 - pinched or coarcted aorta flow patterns are normal but are reduced below the coarctation. Blood pressure is increased invessels leaving the aorta above the coarctation.The broken white arrow indicates diminishedblood flow through the aorta.
Pulmonary Stenosis and AtresiaPulmonary Stenosis and Atresia
• Obstruction at pulmonary valve• May occur as isolated defect or with:
a) Tetralogy of Fallot orb) TGA
• RV hypertrophya) usually there is poststenotic dilation
i) “jet” streaming• Relatively frequent defect
• Atresia of valvea) no communication between RV
and lungsb) hypoplastic RV with ASDc) flow enters lung through PDA
Aortic Stenosis and AtresiaAortic Stenosis and Atresia• 3 types:
ascending aortab) PDA MUST be present to allow blood flow to:
i) aortaii) coronary arteriesiii) always fatal in first week of life
- when ductus closes !!
Aortic stenosis (valvular)1 - narrowed aortic valveFlow patterns are normal but blood flow to the aortais reduced as indicated by the broken white arrows
• Subaortic stenosisSubaortic stenosisa) thickened ring below level of