Cardiology Flashcards
Inheritance of long QT syndrome
autosomal dominant!
homozygous = Jervell Lange-Neilson (assoc with sensorineural hearing loss)
heterozygous = Romano-Ward
Treatment of long QT
avoid exercise, beta-blockers
What is the axis on ECG of a child with an AVSD?
left-axis deviation
in what congenital heart disease would you see left axis deviation?
AVSD, small RV (tricuspid atresia), Noonan syndrome (no clear reason why…)
what are the signs on ECG of LV hypertrophy
Tall R waves in V6, deep S waves in V1
ECG signs of RV hypertrophy
Tall R waves in V1, deep S waves in V6, Q waves in V1, upright T waves in V1 (after day 5 of life - should be flipped until about age 9)
Palivizumab - what is it
monoclonal IgG antibody against RSV
recurrence rate of congenital heart disease
4% up to 10% (higher if left-sided)
Complications of Fontan
protein-losing enteropathy, plastic bronchitis
What cardiac patients need antibiotic prophylaxis?
those with a high risk lesion (Cyanotic, repaired with prosthetic material in last 6 months, persistent defects - leak) undergoing a high risk procedure (dental procedure, non-infected gut/GU procedure)
ECG findings - pericarditis
diffuse ST elevation, PR depression, T wave flattening eventually T wave inversion
Main cause of CHF - first week of life
- obstructions! hypoplastic left heart (d3-5), severe aortic stenosis, coarctation (d7-10)
- asphyxia
- uncontrolled tachycardia (SVT >24hrs)
- severe mitral/tricuspid regurgitation
Main cause of CHF - week 2-6 of life
Things that cause left to right shunting: VSD, AVSD, PDA
NOT ASD!
Main cause of CHF - older children
pump failure!
dilated cardiomyopathy, myocarditis, tachycardia
3 signs of CHF in infants
tachycardia, tachypnea, hepatomegaly
Risk of recurrence of congenital heart disease (isolated case) in sib?
2-3%
CHD in Turner syndrome (name 3)
- coarctation of the aorta, bicuspid aortic valve, aortic stenosis
CHD in fetal alcohol syndrome (name 3)
VSD, ASD, TOF
CHD in VACTERL
ASD, VSD, PDA (65-85%)
CHD in Trisomy 13 (patau) - name > 3
PDA, VSD, ASD, valvular disease, coarctation of the aorta, complex defects
CHD in Trisomy 18
VSD, polyvalvular disease (~100%)
Duct dependent for pulmonary circulation CHD
- pulmonary atresia, critical pulmonary stenosis, pulmonary atresia with intact septum, TOF
Duct dependent for systemic circulation CHD
Severe aortic stenosis, interrupted aortic arch, hypoplastic left heart syndrome
CHD - duct required for “mixing”
TGA
Most common type of ASD? (primum, sinus venosus, secundum) and ECG finding
secondum, right anterior deviation on ECG
Most common congenital heart disease and CXR finding
VSD, 14-18%
CXR - increased pulmonary vasculature, cardiomegaly
Most common CHD in T21
AVSD
What is bicuspid aortic valve associated with?
5-8% also have coarcation
Noonan syndrome CHD (name 2)
pulmonary stenosis w/ dysplastic pulmonary valve, hypertrophic cardiomyopathy
Alagille syndrome CHD
peripheral pulmonary stenosis
Physical exam and CXR findings of coarctation of aorta
shock, hypertension, murmur, ejection click (assoc with bicuspid valve), weak/delayed femoral pulses, BP gradient (upper/lower extremities), rib notching on cxr (due to collateral vessels going to descending aorta)
AVSD ECG finding
Left-ward and superior axis on ECG
Auscultation of aortic stenosis
Systolic ejection murmur at right upper sternal border, ejection click
Tetralogy of Fallot
- VSD
- overriding aorta
- pulmonary stenosis
- right ventricular hypertrophy
X-ray with “wall to wall” heart
tricuspid regurgitation
What CHD to think about with tricuspid regurgitation
Ebstein’s anomaly!
Rhythm abnormalities with Ebsteins
WPW
Initial management of TGA
- prostaglandin to keep duct open, balloon atrial septostomy
3 types of CHD associated with 22q11
common arterial trunk, tetralogy, interrupted aortic arch
Complications of Fontan
- arrhythmias
- cyanosis
- protein losing enteropathy
- plastic bronchitis
- thromboembolism
TAPVD - clinical findings and x-ray
- cyanosis, respiratory distress
- xray: snowman (only in supracardiac), increased pulmonary vasculature (venous blood stuck in pulmonary circulation)
What CHD should you NOT use PGE in?
TAPVD - increases pulmonary blood flow
Medical management of TAPVD
NONE!
Features of innocent heart murmurs
soft (gr II or less), normal heart sounds, quiet precordium, along sternal border, varies with position
Diastolic murmur heard in…
aortic or pulmonary valve regurgitation, AV valve stenosis
Pathologic systolic ejection murmur heard in…
aortic/pulmonic valve stenosis, ASD, coarctation
pansystolic murmur
VSD, AVVR (mitral, tricuspid)
continuous murmur
runoff lesions (PDA, AVM, aortopulmonary collaterals, BT shunt)
carditis of rheumatic fever
endomyocarditis with valvulitis involving the MITRAL and aortic valves
management of rheumatic fever associated carditis
mild = ASA, severe = prednisone
secondary prevention of rheumatic fever
- Pen G IM Q3-4wks
- Pen V PO 250 mg BID
if no carditis - 5 yrs or 21 yrs old (whichever is longest)
if carditis - 10 yrs or 25 yrs of age
if carditis with valve involvement - at least 40 yrs or life-long
clinical features of pericarditis
sharp, stabbing precordial pain; worse when supine, better leaning forward (pain = referred from diaphragm/pleura); friction rub on auscultation; ECG = diffuse ST elevation
Cardiac findings in Pompe disease
- hypertrophic cardiomyopathy, increased wall thickness, supraventricular tachycardia, short PR interval, very high QRS voltages
What is Jervell and Lange-Nielsen syndrome?
Long QT + sensorineural deafness, autosomal recessive (homozygous)
Unexplained nocturnal death in a structurally normal heart - what syndrome?
Brugada syndrome
Leading cause of acquired heart disease in childhood
Kawasaki disease
Greatest risk of developing aneurysms in KD
- male gender
- young age (<6mos)
- not treated with IVIG
Emergency treatment of VT
synchronized cardioversion, IV lidocaine, IV amiodarone, correct underlying etiology, others - procainamide, magnesium
Definition of pulmonary hypertension
mean pulmonary arterial pressure > 25 mmHg at rest
ECG change in acute rheumatic fever
prolonged PR
A/E of adenosine
atrial fibrillation
A/E of PGE1
apnea
hypotension
fever
edema