6. Congenital Heart Diseases Flashcards
Definition of congenital heart diseases
Abnormalities of the heart or great vessels that are present from birth
Types of congenital heart diseases
- Left-to-right shunt
a. Atrial septal defect
b. Ventricular septal defect
c. Patent ductus arteriosus - Right-to-left shunt
a. Tetralogy of Fallot
b. Transposition of the great vessels
c. Tricuspid atresia
d. Persistent truncus arteriosus - Obstructive congenital anomalies
a. Coarctation of aorta
b. Pulmonary stenosis & atresia
c. Aortic stenosis & atresia
Definition of Left-to-Right Shunts
Typically acyanotic at onset, but may become cyanotic with eventual pulmonary hypertension or shunt reversal
Atrial septal defect
- Abnormal, fixed opening in the atrial septum due to
incomplete tissue formation - Despite right sided volume overload, ASD is usually well totally and asymptomatic until adulthood
- Ejection systolic murmur (due to excessive flow through the pulmonary valve) + permanent splitting of S2
Ventricular septal defect
- Incomplete closure of the ventricular septum (mostly in the membranous portion, minority in the muscular part)
- Functional consequences depend on size of VSD
- If small, asymptomatic until adulthood
- If large, cause right ventricular hypertrophy &
pulmonary hypertension virtually from birth, which may progress to irreversible pulmonary vascular disease and shunt reversal (Eisenmenger syndrome) - Pan-systolic murmur
Patent ductus arteriosus
- Ductus arteriosus remaining open after birth
- Depending on diameter of PDA, may result in progressive
obstructive changes in pulmonary vasculature (due to
pulmonary volume overload) - Continuous/harsh/machinery-like murmur
Definition of right-to-left shunts
Typically cyanotic
Tetralogy of fallot
- Four cardinal features that arises from an anterocephalad deviation of the anterior septum:
- Ventricular septal defect
- Aorta that overrides the VSD
- Pulmonary stenosis
- Right ventricular hypertrophy
PROVe - Pulmonary stenosis, Right ventricular hypertrophy, Overriding aorta, VSD
- Heart is enlarged & ‘boot-shaped’
Transposition of the great vessels
- Ventriculoarterial discordance: aorta originates from RV, pulmonary trunk from LV
- Due to abnormal formation of truncal septa
- Right ventricular hypertrophy & left ventricular atrophy
- Without surgical repair, patient dies within first few
months of life
Tricuspid atresia
- Complete occlusion of the tricuspid valve orifice due to unequal division of the atrioventricular canal
- Concomitant ASD & VSD allows maintenance of
pulmonary & systemic circulation - High mortality within first few weeks or months of life
Persistent Truncus Arteriosus
Single great artery receiving blood from both ventricles
due to a failure of separation of truncus arteriosus into aorta & pulmonary trunk
Coarctation of the aorta
- A narrowing/constriction of the aorta which usually
occurs distal to the branches of the arch of aorta - Results in hypertension of the upper extremities and hypotension in the lower extremities (weak pulses, intermittent claudication, coldness)
- Left ventricular hypertrophy (due to pressure overload)
- Radiographically, notching of the undersurfaces of the ribs evident (due to dilation of intercostals arteries involved in anastomosis to bypass coarcation)
- Ejection systolic murmur
Pulmonary Stenosis & Atresia
- May occur in isolation or as part of a more complex
anomaly (e.g. Tetralogy of Fallot) - Right ventricular hypertrophy (due to pressure overload)
Aortic Stenosis & Atresia results in?
Left ventricular hypertrophy (due to pressure overload)
