Beruflich Dokumente
Kultur Dokumente
SMF / Bagian Kardiologi Pediatrik RSU Dr. Zainal Abidin Fakultas Kedokteran Universitas Syiah Kuala Banda Aceh
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 1. 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)
Shunts
Right to Left vs. Left to Right Right to left shunt: un-oxygenated blood is shunted from the right side of the heart to the left side, and then enters the systemic circulation. 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, increasing the work load for the right heart
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
Gender Factors
Occur equally among males and females, but
More common in males: aortic stenosis, coarctation of the aorta More common in females: PDAs, ASDs
Environmental Factors
Maternal Infections:
Rubella: PDA, pulmonary stenosis, VSD, ASD
Maternal Drugs:
Lithium: Tricuspid valve abnormalities, Ebsteins Anomaly Thalidomide Possibly related to CHDs: Dilantin & Cocaine Alcohol abuse: VSD
Maternal Disease:
Diabetes: transportation of the great vessels, VSD, situs inversus, single ventricle, hypoplastic left ventricle SLE: Congenital heart block
Genetic Factors
Trisomy 21 (Downs Syndrome):
A-V canal defects, VSD
XO (Turners Syndrome):
coarctation of the aorta, aortic stenosis
Osteogenesis Imperfecta:
Aortic incompetence
Marfan Syndrome:
Aortic dilatation, aortic & mitral incompetence
The good news is- From 1991 2001 deaths related to CHD declined 28% due to improvements in surgical techniques and medical management
Prevention of CHD
Not possible in most cases But -- 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, 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
Signs/Symptoms of CHD
Murmurs Cyanosis worsens with crying or other exertion Respiratory distress Signs of poor perfusion, such as slow capillary refill, diminished peripheral pulses Fatigue commonly observed during feedings in newborns or during play in children Failure to thrive
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
Week 4
Heart has:
single outflow tract, the truncus arteriosus (divides to form aorta & pulmonary veins) Single inflow tract, the sinus venosus (divides to form the superior and inferior vena cavae) Single atrium Single ventricle AV canal begins to close
Weeks 5 - 7
Week 5 AV canal closure complete Formation of atrial and ventricular septums Heart growing rapidly, 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
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
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, through the ductus arteriosus to enter the systemic circulation, bypassing the pulmonary circulation
Fetal Circulation
Venous blood in the fetus:
returns to the placenta through the 2 umbilical arteries
After Birth
Lungs distend with air and pulmonary vascular resistance falls. 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
PDA
VSD
Most common congenital heart defect May occur alone, or with other abnormalities About one-third of small VSDs will close spontaneously
Truncus Arteriosus
Truncus fails to divide completely during fetal life, leaving a connection between the aorta and pulmonary arteries Mixed oxygenated and de-oxygenated blood exits the heart and enters the systemic circulation
Tetralogy of Fallot
TOF =
Ventricular septal defect Aorta position is shifted to the right and over-rides the VSD Stenosis of the pulmonary outflow tract, often involving the pulmonary valve Hypertrophy of the right ventricle
TAPVR
The pulmonary veins, instead of being connected to the left ventricle, are connected to the right ventricle or superior vena cava, and return oxygenated blood to the right side of the heart.
AV Canal
Includes:
ASD VSD Abnormalities of the Mitral and/or Tricuspid valves