Congenital Heart Disease Flashcards
What might cause CHD? What are some cariac malformations?
Genetic and environment
- Maternal rubella- cataracts, deafness, microcephaly + PDA (patent ductus arteriosus) + pumonary stenosis
- Fetal alcohol syndrome: assc w/ cardiac lesions + microcephaly,micrognathia, micropthalmia, growth retardation.
- Maternal SLE- assc w/ fetal complete heart block due to trans placental anti-Ro Abs passing through placenta
What are some genetic associations?
Trisomy 21: endocardial cushon defects, ASD, VSD, tetralogy of Fallot.
Turner syndrome: XO- coarcation of Aorta.
Marfan syndrome: connective tissue diseasea aortic dilatation + aortic and mitral regurg.
Kartagener syndrome: dextacardia + ciliary dysmobility.
What are some complication of cardiac lesions?
Central cyanosis - defined as >5g/L Hb in Arterial blood. -due to shunting of venous straight into arterial circulation bypassing the lungs. So..this type of cyanosis does not respond to giving oxygen.
(R-L shunts)
Congestive HF: occurs due to inability of heart to sufficiently perfuse tissues due to a large lesion- eg VSD in infancy.
Pumonary hypertension: due to L-> R shunts. (ASD, VSD, PDA)
More vol to lungs-> causes pumonary smooth muscle hypertrophy, causing an increased pulmonary vascular resistance- pulm HTN.
Eventually…… Pulmonary pressur exceeds systemic pressure causing reversal of shunt, resul ing in cyanotic heart disease: Eisenmenger syndrome.
Infective endocarditis- prone to Bacterial colonization. Appropriate antibiotic prophylaxis !
Sudden death- due to arryhtmias (more common in these) or outflow tract obstruction eg in aortic stenosis.
Pulmonary stenosis
Others: pulmonary atresia, hypoplastic left heart.
Notes on cyanosis
Central cyanosis: below tongue
Peripheral cyanosis- lips, feet, hands.
Cyanosis caused by pulmonary disease or cardiac failure improves on increasing inspired oxygen.
Cyanosis caused by R-> L shunts do not improve on oxygen
What are the 2 most common locations of ASD defects?
Venous defect - near SA node aka sinus venosus asd- assc with anomalous pulmonary venous drainage.one of pulmonary veins drains in right instead of left.
Septum secundum defect- lio pio kato- aka ostium sucundum
Most common- mid septal location.
Septum primum defect- telefteo punkato, still in atria. (Ostium primum) - adjacent to AV valaves- these are usually abnormal.
Whats eisenmenger syndrome? When can it occur?
Can occur in any condition involving L-> R shunts.
With worsening pulmonary htn the shunt reverses to R->L causing venous blood to bypass the lungs and –> cyanosis that is not resposive to oxygen.
What are the findings O/E of an ASD?
Large: pulmonary hypertension, Check for P2 sound in 2nd ICS and look for a prominent R ventricular heave. (Stronger beat)
P2: if pulm htn developed,pulmonary flow mirmur disappears- loud pulmonary component to S2 cz increased pressure causes pulmonary valve to slqm shunt.
If there is shunt reversal- clubbing, central cyanosis, and signs of congestive cardiac failure. If shunt reveral - Eisenmenger synrome
A2, JVP, P2, S1.
What are some signs of congestive cardiac failure?
Peripheral oedema
Ascites
Bilateral basal crepitations
What does Congenital heart disease mean?
Refers to cardial lesions present from birth.
What are some uncommon causes of congenital cardiac abnormalities?
Only if severe- maternal rubella- CF-> systolic murmur- if R-> L by Foramen ovale- child cyanosed and dyspnoeic. - reocurance or the lesion not suitable for pulmonary angioplasty- obstruction surgically removed.
Tetralogy of Fallot (cyanotic) - 4 - 1. VSD, 2. Pulmonary stenosis
3. Overriding aorta 4. RVH- R-> L shunt . “Spells of cyanotic attacks” -> relieved by squatting- invreases systemic resistamce and therefore reduced R->L shunt. Tx- total surgical correction.
Complete transpositioon of great arteries: (cyanotic)
Aorta from R and pulm artery from L. 2 circulations are parallel- death is quick if no communication, somits also common to see an ASD, VSD, PDA in these infants. CF: eatly cardiac F and cyanosis.
Dx- echo and cardiac catheterisation. Give prostaglandin E1 to prevent postnatal closure of ductus arteriosus. + short term- op to male ASD larger.
Congenital aortic stenosis(acyanotic) M>F-
Hypotensive, dyspnoeic, sweaty, angina, sudden death,
operative correction of stenosis. - ejection systolic murmur esp on exertion- 2nd ICS.
Hypoplastic L heart- cyanotic- underdevelopment of all parts of the L side. HF. Echo dx. - surgical correction- high mortality rates.
Pulmonary artery stenosis- cyanotic-
Assc w/ Fallot tetralogy sometimes. If asx pt may have a left parasternal heave- ie RVH signs. And pulmonary ejection
Dx- confirm by ECHO. Pulm angioplasty cure if obstruction?
Some words about pulmonary hypertension
Causes mild dyspnoea when shunt is from L-R . Signs:
Dominat a wave in JVpulse
Palpable and loud pulmonary component in S2.
Ejection systolic murmur in pulmonary area due to increased flow.
RV heave
Tricuspid regurgitation if r ventricle dilates.
What are the physical findings for an aortic coarcation?
If severe: continuous murmur beneath the L clavicle and signs of LVF (basal bilateral crepitations and audible 3rd sound)
Bp: HTN in both or just R arm. Low BP in legs !
Pulse: radiofemoral delay, weak leg pulses.
Eyes: tortuous retinal vessels
Neck: prominent carotid pulsation
Chest: systolic murmur beneath L clavicle, systolic murmur of colaterals over scapulae.
CXR: rib notching in kids >6 (1+2 arteries)
Aortic knuckle absent
Surgery !
VSD fphysical findings??
If large: apex is displaced and pulm HTN can develop. So loud P2 and RV heave.
Mx- 30% resolve spontaneously till 3Y.
If sign L-R then surgical correction.
