Congenital Heart Disease Most occur during weeks 3 to 8 Incidence 6 to 8 per 1,000 live born births
Some genetic – Trisomies 13, 15, 18, & 21 and Turner syndrome
Environmental – congenital rubella Most cases the cause is not apparent
Congenital Heart Disease Malformation causing a left to right shunt
Malformation causing a right to left shunt
Malformation causing an obstruction
Shunts Right to left – cyanosis
Tetralogy of Fallot Transposition Persistent truncus arteriosus Tricuspid atresia
Clubbing and polycythmia
Left to Right Shunts Cause increase pulmonary blood flow – no cyanosis early on
Leads to pulmonary hypertension Right ventricular hypertrophy
As pulmonary vessels respond – raising pressure Leads to increase right ventricular pressure
Right to left flow and cyanosis – late (tartive)
Atrial Septal Defect Not patent foramen ovale – 25% of normal
3 types: Secundum - most common-90% Primum – associated with cleft mitral valve
Sinus venosus – anomalous connections of right pulmonary veins
ASD
Left to right shunt Pulmonary resistance less Compliance of right ventricle is greater than the left
Pulmonary blood flow 2-4 times normal
Well tolerated until 30’s Less than 10% develop pulmonary hypertension
ASD
ASD
Ventricular Septal Defect Incomplete closure of the ventricular septum
Roger’s disease Most associated with other defects (tetralogy)
30% are isolated Many spontaneous close over time 90% in the membranous septum
VSD Most the size of aortic orifice
Left to right Right ventricular hypertrophy and pulmonary hypertension
Leads to shunt reversal, cyanosis and death
VSD
VSD
Patent Ductus Arteriosus 90% isolated Continuous harsh murmur Left to right Obstructive pulmonary vascular disease gradually reverses shunt
Close as soon as feasible May need to keep open if other abnormalities exist
PDAPDA
PDA
Atrioventricular Septal Defect Partial – 10 ASD and cleft anterior mitral leaflet
Complete – common AV valve 1/3 have Down’s syndrome
Right-to-Left Shunts – Early Cyanosis
“T” – tetralogy, transposition of great arteries, tricuspid atresia, total anomalous pulmonary venous connections, and truncus arteriosus
Tetralogy of Fallot VSD Obstruction to the right ventricular outflow tract (subpulmonary stenosus)
Aorta that overides the VSD Right ventricular hypertrophy Anterosuperior displacement of their infundibular septum
Tetrology
Tetrology of Fallot
Transposition of Great Vessels Abnormal formation of the truncal and aortopulmonary septa
Aorta arises from the right ventricle and lies anterior and to the right of the pulmonary artery
USD in about 35% - stable Patent formen ovale or PDA – unstable shunt
Right ventricular hypertrophy
Transposition
Transposition
Truncus Arteriosus Failure of separation of the embryologic truncus arteriosus into the aorta and pulmonary artery
Tricuspid Atresia Unequal division of the AV canal
Underdevelopment of right ventricle
ADS or VSD
Total Anomalous Pulmonary Venous Connection No pulmonary veins directly joins the lt atrium
Results from when the common pulmonary vein fails to develop
Primative systemic vein persist to drain lungs
ASD or patent foramen ovale
Coarctation of Aorta 2:1 males Infantile – hypoplasia of aorta – proximal to PDA
Adult form – distal to Ductus 50% accompanied by a bicuspid aortic valve
Pulmonary Stenosis and Atresia
Pulmonary valve stenosis
Aortic Stenosis and Atresia Valvular
Hypoplastic, dysplastic, abnormal in number
Subvalvular supravalvular