Congenital heart disease Flashcards
holosystolic murmur (HSM) at the lower left sternal border (LSB) that is unchanged with standing or the Valsalva maneuver
VSD
Standing and Valsalva
decreased venous return, increase HCM murmur
easy bruising,” hypertelorism, bifid uvula, cleft palate, pectus excavatum, and aortic root aneurysm
Loeys-Dietz syndrome (LDS), aortic root > 4.2 by echo or >4.4 by CT/MR: intervene. TGFBR1 gene.
Asymptomatic aortic repair
4.5 for Marfan, >5.5 for bicuspid
vascular Ehlers-Danlos syndrome (EDS) vs Marfan
thin, translucent skin with easy bruising, mitral valve prolapse and POTS. Mutation COL3A1
headache, dizziness, slow mentation, a sense of dissociation, tinnitus, paresthesias, myalgias, restless legs, and bleeding or stroke.
hyperviscosity syndrome. Can occur in setting of eisenmenger after dehydration. diagnose with hgb level, treat with fluids, iron if needed->Erythropheresis
eisenmenger management
obtain yearly hematocrit, uric acid, transferrin, iron, and ferritin. Iron deficiency is common and should be treated.
refractory HTN+ diastolic forward flow in the abdominal aorta
coarct. Intervene if: peak-to-peak gradient >20 mm Hg, clinically significant collateral flow, systemic HTN attributable to CoA, or heart failure attributable to CoA. Treat with stent placement.
Complication with greatest risk during Marfan pregnancy
aortic dissection. If > 4cm, replace before preg. Preg CI if >4.5
Post coarct repair surveillance
q3-5 years with CT or MR
TOF with severe PR. Intervene when
Symptomatic.
Asympt + RV/LV systolic dysfunction OR severe RV dilation OR RVOT obstruction
cyanosis and clubbing of the toes and not the fingers
PDA. continuous “machinery”-type murmur-> becomes diastolic murmur with eisenmenger
Secundum ASD with L-R shunting. Close if:
PVR < 1/3 SVR,
PASP <50% systemic,
right heart enlargment, Qp/Qs> 1.5. TEE, then RHC for workup
Ischemic eval for anomalous coronaries
has to be exercise as it dilates aorta.
restrictive asymptomatic VSD
F/u echo in 2 years
Close small VSDs if :
symptoms OR
left heart enlargement with 1.5:1 shunt OR
pulmonary hypertension (as long as [PA] systolic pressure is <50% the systemic and the pulmonary vascular resistance is < 1/3 SVR OR
endocarditis
turbulent flow in the mid RV
double chambered RV due to muscle bundles that separate a proximal high-pressure chamber from the distal, lower-pressure one
ASD closure CI
Eisenmenger
ASD workup
Define anatomy first with TEE/MR. Then RHC for physiology specifics
Positional hypoxemia, platypnea-orthodeoxia syndrome, better when laying down.
PFO
Holt-Oram syndrome. DiGeorge syndrome. Williams syndrome.
secundum ASD.
TOF.
supra-aortic stenosis and hypercalcemia.
Anomalous coronaries
left from right.
right from left.
automatic surgery.
surgery if there is ischemia or arrhythmias.
ALCAPA
right-to-left shunting from the coronary steal into the pulmonary artery, and the LCA would be markedly dilated over time due to chronic shunting.
Trisomy 21
AV canal defect: AV valves in same plane, primum ASD, VSD
18-60 years of age with a nonlacunar stroke and PFO
(MRI) of the brain, MRI or CT of intra- and extracranial vessels, venous doppler, event monitor, echocardiography. Then, consider high risk (atrial septal aneurysm or large (>20 bubbles) right-to-left shunt: if so, close.
Marfan aortic surveillance
annual
Right heart enlargement without ASD on TTE.
Do not repeat TTE with saline. TEE/CMR
ebstein surgical repair
decline in exercise capacity and clinical heart failure.
TOF ICD
LV systolic or diastolic dysfunction, NSVT, QRS duration ≥180 msec, extensive RV scarring, or inducible sustained ventricular tachycardia (VT
Bicuspid aorta surveillance
If > 4.5 cm, annual surveillance
VSD closure CI
severe PH.
*Pregnancy is CI in eisenmenger. *
systolic ejection click that occurs early in systole, left second interspace and the apex, does not change with any maneuvers
bicuspid valve
