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