Cyanotic and Acyanotic
Lists the cyanotic and Acyanotic hearts diseases
Cyanotic: ToF, TGA, truncus arteriosus, tricuspid atresia, TAPVR (total anomalous pulmonary venous return), Ebstein anomaly, Eisenmenger syndrome, Hypoplastic left heart syndrome (HLHS)
Acyanotic: AVSD, ASD, VSD, PDA, CoA, AS, PS, AR, PR, MS, TS, MR, TR
Anatomy (simple)
Communication between the right and left atria
Anatomy simple (double points to name the 4 locations)
Communication between the right and left ventricles
EXTRA:
Locations: Perimembranous, muscular, outlet, inlet
Pathophysiology of ToF
RVOT obstruction (PS), VSD, overriding aorta, and RVH
Name two innocent murmurs
Still’s murmur, venous hum, peripheral pulm art stenosis,
Which ones causes obstructive lesions
Cyanotic: HLHS
Acyanotic: AS, PS, CoA
Pathophysiology
L to R shunt independent of PVR à right-sided volume overload overtime
Pathophysiology (double points to classify based on pressure differences)
L to R shunt depends on PVR (i.e. shunting increases as PVR decreases overtime), large shunts leads to dilated LA and LV
EXTRA:
>30mmHg pressure difference leads to restrictive defect, <30mmHg is non-restrictive defect
CXR findings for ToF, TGA, TAPVR
ToF: boot-shaped heart
TGA: egg on a string
TAPVR: snowman due to dilated SVC/small heart with ground glass appearance in lungs
List 3 diagnostic tools used in paediatric cardiology
12-lead ECG
Chest x-ray
Echocardiogram
Classify the cyanotic heart diseases based on inc/dec of pulmonary blood flow
Inc pulmonary blood flow: ToF, tricuspid atresia
Dec pulmonary blood flow: TGA, TAPVR, truncus arteriosus
Clinical presentation (double points for Investigation findings)
Asymptomatic mostly, ejection-systolic murmur best heard in the LUSB, S2 wide with a fixed split with no variation with respiration, Mid-diastolic rumble murmur heard at LLSB d/t inc flow across tricuspid valve.
CXR: R atrium and ventricular enlargement, inc pulmonary markings
ECG: RAD, RSR1 pattern in V1
Presentation (double points for investigation findings)
Holosystolic murmur best heard in the LLSB, inc S2/single S2 (if P-HTN present), LV heave w/ hyperdynamic precordium, ejection systolic murmur at LUSB d/t inc flow across pulm valve, mid-diastolic rumble in mitral area heard best at apex d/t inc flow across mitral valve.
CXR: small shunts leads to cardiomegaly (minimum), inc pulm vasculature, large shunts leads to prominent cardiomegaly (LA and LV enlargement)
ECG: LV or biventricular hypertrophy, LA enlargement, T wave inversions in left lateral leads
Clinical presentation of TAPVR
Cyanosis, tachypnea d/t pulm edema from pulm venous congestion, murmur of pulmonary stenosis
Differentiate innocent murmurs from pathological murmurs
Innocent: Grade 2 or less, systolic, musical/vibratory, minimal radiation, dec intensity when sitting, normal S2, no added heart sounds, quiet precordium
Pathological: Grade 3 or above, pansystolic/diastolic/continuous, radiation, harsh in nature, abnormal S2, additional sounds (gallop, click, rhythm), heave/thrill
Clinical presentation and pathophysiology of clinical presentation for cyanotic heart disease
Central cyanosis: desaturated blood entering systemic circulation
Peripheral cyanosis: poor circulation in extremities d/t vasoconstriction
Clubbing: chronic hypoxia
Polycythaemia: chronic hypoxia
Indications for closure
Evidence of volume overload, symptomatic patients, elective closure (3-5 years old), heart failure symptoms
Management (include drugs)
Diuretics, digoxin, ACE inhibitor, nutritional supplementation (higher calorie formula, NG feeding), surgical closure/transcatheter device closure
Management of ToF
Neonates: PGE infusion, knee-chest position
Older children: squat
General: O2, fluid resuscitation, morphine, NaHCO3, Betablockers
Surgical: within 1st year of life, aortopulmonary shunt in infancy/closure of VSD and opening of the pulmonary valve or valve replacement
Location of majority of coarctation, 2 syndromes associated and complications (3) of CoA
98% below left subclavian artery at origin of ductus arteriosus
Syndromes: Turner syndrome, DiGeorge syndrome
Complications: berry aneurysm, HTN, CVA, Interrupted aortic arch, shock, severe heart failure
ALL or Nothing!!!!
Everything ASVD
Anatomy: failure of central portion of heart to develop leading to inlet VSD, primum ASD and a common AV valve.
AKA: atrioventricular canal defect/endocardial cushion defect
Pathophys: L to R shunt with pulmonary congestion and elevation in pulmonary artery pressure
CF: failure to thrive, CHF, tachypnea, cyanosis at birth until pulmonary vasculature resistance drops, associated w/ Down Syndrome
PE: active precordium (if large shunt), low frequency VSD murmur, high freq systolic regurgitation murmur, mid-diastolic rumble (large shunt)
CXR: cardiomegaly w/ prominent LA and LV
ECG: superior QRS axis (negative deflection in aVF)
Management: diuretics, ACE-i, high-calorie formula, surgical: 1st year of life - closure of ASD and VSD, division of common AV valve
Complications: MR, MS, complete heart block
Different types and 1 characteristic about each
Ostium Secundum defect: MC, deficiency of septum primum, located midportion of atrial septum, associated w/ Holt-Orlan syndrome, upper limb anomalies, closure via transcatheter/surgical approach.
Ostium Primum defect: located in lower portion of atrial septum, deficiency of septum secundum, associated with a cleft mitral valve and partial AV canal defect, an associated inlet VSD and common AV valve is known as a complete AV canal (CAVC) defect or endocardial cushion defect, Associated syndrome: Down syndrome, Closure is not amenable to transcatheter closure and is performed surgically
Sinus Venosus defect: located by SVC (at junction of pulmonary veins and post-superior wall of atrium) or by IVC to right atrium junction, Superior type often associated with anomalous drainage of the right pulmonary veins to the SVC • Closure is performed surgically and usually includes baffling of right pulmonary veins to the left atrium
Indications for closure and pathophys of ACEi
Indications: heart failure symptoms with failure to thrive, reactive P-HTN
Pathophys: reduce afterload to promote more systemic flow and less pulmonary flow
Double or Nothing
Everything HLHS
Anatomy: Underdevelopment of the mitral valve, LV, aortic valve
Pathophys: L to R shunt across ASD, depends on R to L shunting across PDA
Clinical Presentation: cyanosis, tachypnea, circulatory shock when PDA closes, heart failure
PE: TR, flow murmur, peripheral cyanosis
CXR: cardiomegaly, inc pulm markings
Management: PGE, palliation (Norwood, bidirectional glenn, fontan procedure)
(double or nothing question):
Ebstein Anomaly: anatomy, presentation (3), CXR, ECG, Indications for valve repair/replacement
Anatomy: abnormal delamination of the septal leaflet of the tricuspid vale resulting in deformity and varying degrees of TR
Presentation: cyanosis, holosystolic murmur, enlarged liver
CXR- cardiomegaly (box-shaped heart) d/t RA and RV enlargement
ECG: WPW, RBBB
Indications: cyanosis limiting activity, heart failure, arrhythmias