Congenital Heart Disease Flashcards
What is the incidence of congenital heart disease?
8/1000 live births
25% associated with chromosomal abnormalities
What is an Atrial Septal Defect (ASD)?
Defect in atrial septum allowing blood flow between the left atrium and right atrium
What is the prevalence of Atrial Septal Defects?
8% of all congenital heart defects
What causes Atrial Septal Defects?
Incomplete separation of the common atrium in the 4th-6th week of gestation
What are the types of Atrial Septal Defects?
- Primum (20%)
- Secundum (70%)
- Sinus Venosus (10%)
- Coronary sinus defects (very rare)
What is the pathophysiology of ASD?
Left-to-right shunt causes pulmonary volume overload
What are the symptoms of ASD?
- Asymptomatic in most children
- Soft systolic ejection murmur + split S2
- May predispose to chest infections and failure to thrive
What is the ECG finding associated with ASD?
Right bundle branch block and right axis deviation
What is the treatment for ASD?
Surgical or device closure
What is a Ventricular Septal Defect (VSD)?
Defect in ventricular septum allowing blood flow between the left ventricle and right ventricle
What is the prevalence of Ventricular Septal Defects?
30% of all congenital heart defects
(most common CHD!!!)
What are the types of Ventricular Septal Defects?
Classified according to boundaries and region of RV opening
What are the associations of VSD?
- Fetal alcohol syndrome
- Down syndrome
- Other congenital heart defects
What is the pathophysiology of VSD?
Left to right shunt where oxygenated blood leaks into the right ventricle
What type of murmur is associated with VSD?
Pansystolic murmur - best heard at the left lower sternal border resulting from turbulent blood flow across defect.
What happens with larger VSDs?
More severe symptoms with pulmonary hypertension and potential right-to-left shunt (Eisenmenger syndrome)
What is a Patent Ductus Arteriosus (PDA)?
Patent Blood vessel in fetal circulation connecting the aorta and pulmonary artery
What causes Patent Ductus Arteriosus?
Remnant of the distal portion of the 6th left aortic arch
What are the risks associated with PDA?
- 80% incidence in premature babies
- Associated with diaphragmatic hernia, transposition, Tetralogy of Fallot, pulmonary atresia
What is the treatment for PDA?
- Prostaglandin inhibitor (indomethacin)
- Surgical ligation or device closure
What is the murmur associated with PDA?
Continuous ‘machinery murmur below the clavicle - complete murmur that occurs in systole and diastole
What is coarctation of the aorta?
Narrowing of the aortic isthmus after the left subclavian
What is the prevalence of coarctation of the aorta?
6% of all congenital heart defects
What are the associations of coarctation of the aorta?
- Turner’s syndrome
- Bicuspid aortic valve
- Ventricular septal defects
What are the symptoms of coarctation of the aorta?
SEVERE = Collapse, left ventricular heart failure, loss of femoral pulse
MODERATE = gradual heart failure, murmur + upper limb hypertension usually with lower limb hypotension and weak pulses
What is the treatment for coarctation of the aorta?
- Prostaglandin infusion
- Surgical repair
What is Tetralogy of Fallot (ToF)?
Four heart abnormalities including RV outflow narrowing, right ventricular hypertrophy, large VSD, and overriding aorta
What is the prevalence of Tetralogy of Fallot?
6% of all congenital heart defects
What are the clinical presentations of Tetralogy of Fallot?
- Blue skin
- Lips and fingernail beds cyanosis
- Clubbing of fingers and toes
- TET Spells
What is Transposition of Great Arteries (TGA)?
Pulmonary artery and aorta switch places, creating parallel circulations
What is the prevalence of Transposition of Great Arteries?
5% of all congenital heart defects
What is the treatment for TGA?
- Prostaglandin E to keep the foramen ovale open
- Emergency Rashkind septostomy
- Arterial switch surgery
What is Hypoplastic Left Heart Syndrome (HLHS)?
Underdeveloped left ventricle and ASD causing reversed circulation
What is the prevalence of Hypoplastic Left Heart Syndrome?
1.5% of all congenital heart defects but 40% of neonatal cardiac deaths
What is the initial treatment for HLHS?
Prostaglandin to keep ductus arteriosus open
