Congenital Cardiovascular Anomalies


Classification of CHDs
1. Structural heart defects —due to abnormal development of the heart during the first 2 months after conception 2. Functional heart defects– ex: congenital heart block 3. Positional heart defects — ex: dextrocardia

Dextrocardia • May occur: – With Situs Inversus: carries a slightly increased risk of heart defects (~ 5 – 10% associated with other CHDs) – Without Situs Inversus: carries a greatly increased risk of associated heart defects (~95% associated with other CHDs) • Both conditions are EXTREMELY rare Situs Inversus .

Classifications of Structural Congenital Heart Defects Increased Pulmonary Blood Flow •PDA •ASD •VSD •AV Canal •Total Anomalous Pulmonary Venous Return (TAVPR) •Truncus Arteriosis Decreased Pulmonary Blood Flow •Tetralogy of Fallot (TOF) •Transposition of the Great Arteries (TGA) •Pulmonary Stenosis (PS) •Pulmonary Atresia (PA) •Tricuspid Atresia (TA) Obstruction to Systemic Blood Flow •Coarctation of the Aorta •Aortic Stenosis (AS) •Hypoplastic Left Heart Syndrome (HLHS) •Mitral Stenosis (MS) .

Shunts Right to Left vs. and then enters the systemic circulation. Left to Right • Right to left shunt: un-oxygenated blood is shunted from the right side of the heart to the left side. increasing the work load for the right heart . • Left to right shunt: a portion of the oxygenated blood is shunted from the left side of the heart to the right side and enters the pulmonary circulation.

Hypoplastic Left Heart – O2 Sat less than 95% – Child may have chronic hypoxia – Caused by: • • Decreased pulmonary blood flow –and/or-Right-to-left shunting: de-oxygenated blood is shunted from the right side of the heart to the left side without traveling though the pulmonary circulation. Acyanotic • Acyanotic (usually left to right shunts): – PDA.Cyanotic vs. VSD • Cyanotic (right to left shunts): – TOF. and blood ejected from the left side of the heart to the systemic circulation is only partly oxygenated . Transposition of the Great Arteries. ASD.

Most Common Congenital Heart Defects These account for 85% of all CHDs: Atrioventricular Septal Defect 9% 44% 12% 15% 10% Coarctation of the Aorta 10% Tetralogy of Fallot Transposition of the Great Arteries Ventricular Septal Defects All other congenital heart defects .

Some Statistics • Most common birth defect – 30% of all congenital birth defects (36.000/yr in the United States) • Most common cause of infant death for children dying as the result of a birth defect • In the US over 130.000 hospitalizations/year are related to CHD .

Etiology of CHD • Unknown in most cases • Incidence of CHD in children is slightly increased if a sibling or parent has CHD • Gender Factors • Environmental Factors • Genetic Factors .

coarctation of the aorta – More common in females: PDAs. ASDs .Gender Factors • Occur equally among males and females. but— – More common in males: aortic stenosis.

hypoplastic left ventricle – SLE: Congenital heart block . pulmonary stenosis. single ventricle. ASD • Maternal Drugs: – – – – Lithium: Tricuspid valve abnormalities. VSD. situs inversus. VSD. Ebstein’s Anomaly Thalidomide Possibly related to CHDs: Dilantin & Cocaine Alcohol abuse: VSD • Maternal Disease: – Diabetes: transportation of the great vessels.Environmental Factors • Maternal Infections: – Rubella: PDA.

VSD • XO (Turner’s Syndrome): coarctation of the aorta. aortic stenosis • Osteogenesis Imperfecta: Aortic incompetence • Marfan Syndrome: Aortic dilatation. aortic & mitral incompetence .Genetic Factors • Trisomy 21 (Down’s Syndrome): A-V canal defects.

The good news is-• From 1991 – 2001 deaths related to CHD declined 28% due to improvements in surgical techniques and medical management .

maintain tight control of blood sugars Folic acid 400 mcg/daily before conception may help to prevent CHD (unproven) – If there is a family history of CHD seek genetic counseling prior to conception .there are actions a woman can take to reduce her risk of having a child with CHD: – – – – Abstain from alcohol during pregnancy Be immunized against rubella before conception If diabetic.Prevention of CHD • Not possible in most cases • But -.

Signs/Symptoms of CHD • • • • Murmurs Cyanosis –worsens with crying or other exertion Respiratory distress Signs of poor perfusion. diminished peripheral pulses • Fatigue – commonly observed during feedings in newborns or during play in children • Failure to thrive . such as slow capillary refill.

Embryonic Heart Development The heart develops in the embryo during post-conception weeks 3 .8 .

Beginning Development • Early week 3 post-conception: heart begins as 2 endothelial tubes • Mid-week 3 : endothelial tubes fuse to form a tubular structure • 28 days following conception: the singlechambered heart begins pumping blood .

the truncus arteriosus (divides to form aorta & pulmonary veins) – Single inflow tract.Week 4 • Heart has: – single outflow tract. the sinus venosus (divides to form the superior and inferior vena cavae) – Single atrium – Single ventricle – AV canal begins to close .

and folds back on itself to form its completed anatomic shape Week 7 • Ventricular septum fully developed • Coronary Sinus forms • Outflow tracts (aorta & pulmonary truck) fully separated .7 Week 5 • AV canal closure complete • Formation of atrial and ventricular septums • Heart growing rapidly.Weeks 5 .

8 Weeks After Conception • By the end of the 8th week after conception the fetus has a fully developed 4-chambered heart .

Fetal Circulation • Before birth the placenta provides the oxygen needed by the developing fetus— the lungs receive only enough blood to perfuse the lung tissues due to high pulmonary vascular resistance & fetal vascular shunts .


through the ductus arteriosus to enter the systemic circulation. bypassing the pulmonary circulation .Fetal Circulation • Arterial blood in the fetus: – enters the fetal circulation via the umbilical vein: – passes through the ductus venosus and enters the inferior vena cava – flows into the right atrium and passes through the foramen ovale into the left side of the heart – passes from the right side of the heart.

Fetal Circulation • Venous blood in the fetus: – returns to the placenta through the 2 umbilical arteries .

Pulmonary blood flow increases • The foramen ovale and ductus venosus usually close during the first day of life • The ductus arteriosus usually closes during the first 24 – 72 hours of life .After Birth • Lungs distend with air and pulmonary vascular resistance falls.

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