Congenital Heart Disease Flashcards
What is most common known genetic cause of CHD?
Which type of CHD?
Down Syndrome. 40% of kids have heart problems (mainly septal defects).
Which CHD is associated w/ Turner Syndrome?
Coarctation of the aorta
Mnemonic for R–>L shunts
Terrible T’s (blue)
Tetrology of Fallot, Truncus arteriosus, Tricuspid atresia, Total anomalous pulmonary venous connection, Transposition of the great vessels.
Tetrology of Fallot How common? 4 main features Timing Clinical features (7) Pink tetrology
- Most common form of cyanotic congenital heart disease.
- 4 main features (PROV): Pulmonary stensosis (below the valve), Right ventricular hypertrophy, Overriding aorta, and Ventricular septal defect.
- Usually presents by 6 months.
- Clinical features
- Boot-shaped heart due to RVH.
- Decreased pulmonary vasculature
- Dyspnea, cyanosis
- Polycythemia from hypoxia may cause cerebral thrombosis.
- Infective endocarditis due to malformation
- Hypertrophic osteoarthropathy (clubbing)
- Pardoxical emboli
- Pink tetrology = no cyanosis b/c the stenosis is not too severe.
Truncus arteriosus
Single blood vessel comes out of the R / L ventricles, instead of the normal 2 vessels.
Total anomalous pulmonary venous connection
Pulmonary veins drain into RA instead of LA. Atrial septum allows for R→L shunt.
Transposition of the great vessels
What is required to survive?
Changes in heart wall
- 1/3 have VSD, which is stable
- 2/3 have PFO/PDA, which are unstable b/c they can close.
- RV hypertrophies and LV gets hypoplastic
How do you keep the ductus arterioles open?
Prostaglandin E1
Mnemonic for L–>R shunts
“The D’s”. Acyanotic.
atrial septal Defect, ventricular septal Defect, patent Ductus arteriosus
How common are atrial septal defects?
Which type is most common?
Make up 10% of CHDs. Most common CHD diagnosed in adults.
90% are from osmium secundum (septum secundum doesn’t come down far enough to cover the hole.
Ventricular septal defect How common? Association Where is the defect? Type of murmur
- Most common CHD (40%)
- Associated w/ Trisomy 21, 13, and 18.
- 90% are membranous septal defects.
- Infundibular VSD is found below the pulmonary valve
- Pansystolic / holosystolic murmur
Patent Ductus Arteriosus Difference from other L-->R shunts Associations Murmur How do you close a PDA?
- 90% are isolated anomalies.
- Higher incidence w/ maternal Rubella infection. Associated w/ polycythemia.
- Continuous machine-like murmur. 2 phases due to systole / diastole.
- Close w/ indomethacin (NSAID that blocks Pg’s)
Coarctation of the aorta Gender Genetic association Other heart / vascular problems 2 types
- Twice as common in males
- Associated w/ Turner Syndrome
- Also associated w/ VSD, ASD, and berry aneurysms in CoW.
- 50% of cases have a bicuspid aortic valve, which increases risk for calcification later in life.
- 2 types are pre ductal (infantile) and post-ductal (adult)
Characteristics of preductal / infantile coarctation of aorta
- PDA
- Congestive heart failure
- Selective cyanosis of lower extremities due to PDA
Characteristics of post-ductal / adult coarcation of aorta
- Closed ductus arteriosus (ligamentum arteriosum) so cyanosis does NOT occur.
- Notching of ribs due to collaterals b/w internal thoracic arteries and intercostal arteries, which are found below the ribs. Internal thoracic arteries are proximal to coarct so form collaterals w/ posterior. Blood flows around the rib and replugs back into the aorta to bypass the coarct.
