Pediatric Cardiology 101 Misty Carlson, M.D.DISCLAIMER: = This lecture is based on generalizations. = In reality, a congenital heart defect (CHD) can act completely different from one patient to the next (eg- classic ToF vs “pink” ToF). = There are many more CHDs than what I've listed and | hope you can use these principles to help you out with those.Fetal Circulation ™ For the fetus the placenta is the oxygenator so the lungs do little work = RV &LV contribute equally to the systemic circulation and pump against similar resistance ™ Shunts are necessary for survival ductus venosus (bypasses liver) foramen ovale (RL atrial level shunt) ductus arteriosus (RL arterial level shunt)Transitional Circulation = With first few breaths lungs expand and serve as the oxygenator (and the placenta is removed from the circuit) = Foramen ovale functionally closes = Ductus arteriosus usually closes within first 1-2 daysEE Neonatal Circulation = RV pumps to pulmonary circulation and LV pumps to systemic circulation = Pulmonary resistance (PVR) is high; so initially RV pressure ~ LV pressure = By 6 weeks pulmonary resistance drops and LV becomes dominantNormal Pediatric Circulation = LV pressure is 4-5 x RV pressure (this is feasible since RV pumps against lower resistance than LV) = RV is more compliant chamber than LVEE NORMAL HEART = No shunts ™ No pressure gradients = Normal AV valves = Normal semilunar valves = If this patient was desaturated what would you think?. If you have a hole in the heart what affects shunt flow? 1. Pressure — easy enough to understand 2. Resistance — impedance to blood flow Remember, the LV has higher pressure and a higher resistive circuit relative to the RV. Now onto the nitty-gritty ...EEL Congenital Heart Disease (CHD) ™ Occurs in 0.5-1% of all live births = Simple way to classify is: LR shunts Cyanotic CHD (RL shunts) Obstructive lesionsL—R Shunts (“Acyanotic” CHD) = Defects VSD PDA ASD AVSD (or complete atrioventricular canal) = May not be apparent in neonate due to high PVR (ie- bidirectional shunt)L—R Shunts — General Points PDA&VSD | ASD ™ Presents in infancy w/ Fizssebie ii Grilehised wi heart failure, murmur, intolerance (AVSD or 1° ASD z poor g presents earlier) Feft heartenlargement ig Right heart enlargement (CHE) (RHE) = Transmits flow and = ‘Transmits flow.onik pressure y [AVSD can present as either depending on size of ASD & VSD component|entricular Septal Defect (perimembranous) i Defect (secundum). oy fentncular Sept fect (perimembranous) lal Septal Defect (secundum) Left Heart Right Heart Overload OverloadPulm vase markings equal in upper and lower zones. Eisenmenger’s Syndrome = A long standing L—R shunt will eventually cause irreversible pulmonary vascular disease = This occurs sooner in unrepaired VSDs and PDAs (vs an ASD) because of the high pressure ™ Once the PVR gets very high the shunt reverses (ie- now R->L) and the patient becomes cyanoticEE R—L Shunts (CCHD) * “Blue blood bypasses the lungs” * Degree of cyanosis varies * Classify based on pulmonary blood flow (PBF) + PBF PBF = Truncus arteriosus = Tetralogy of Fallot = Total anomalous pulm. = Tricuspid atresia venous return (TAPVR) = Ebstein's anomaly = Transposition of the great arteries (TGA) Please note: This is a generalization. In reality most of these defects can present with low or high PBF (eg- ToF with little PS acts more like a VSD with high PBF)Truncus Arteriosus (Type |) Left Aortic Arch There is unimpeded PBF; thus, extreme pulmonary overcirculation. R—L Shunts + PBF = Presents more often with heart failure (except TGA) = Pulmonary congestion worsens as neonatal PVR lowers = Sats can be 93-94% if there is high PBFR—L Shunts Tetralogy of Fallot Left Aortic Arch PBF = Presents more often with cyanosis = See oligemic lung fields = Closure of PDA may worsen cyanosis Dynamic subvalvular obstruction here causes “et spells” ssessWhy are “© pressures equal?[ metelogy of Fait Left Aortic Arch eS— Different amounts of PBF (Truncus vs ToF)Obstructive Lesions Ductal Dependent Critical PS/AS Critical CoA/IAA HLHS Presents in CV shock at 2-3 days of age when PDA closes +/- cyanosis = Needs PGE, Non-Ductal Dependent 1. Mild-moderate AS 2. Mild-moderate CoA 3. Mild-moderate PS a Presents in older child w/ murmur, exercise intolerance, or HTN (in CoA) = Not cyanotic ao N=A manners Ductal-Dependent Lesion Without a PDA there is no blood flow to the abdomen and lower extremities. (Blue blood is better than no blood.)Physical Exam = Inspection and palpation Cardiac cyanosis must be central Differential cyanosis = R-L PDA shunt Differential edema/congestion implies obstruction of SVC or IVC Increased precordial activity Displaced PMI RV heave = RV hypertensionPhysical exam = Lungs Respiratory rate and work of breathing | Oxygen saturations = Abdominal exam ~ Liver size = Extremities Perfusion | Edema — ClubbingPhysical Exam = Pulses (very important) Differential pulses (weak LE) = CoA Bounding pulse = run-off lesions (_+R PDA shunt, Al, BT shunt) Weak pulse = cardiogenic shock or CoA Pulsus paradoxus is an exaggerated SBP drop with inspiration — tamponade or bad asthma Pulsus alternans — altering pulse strength > LV mechanical dysfunctionPhysical Exam = Heart sounds Ejection click = AS or PS Mid-systolic click = MVP Loud S2 = Pulmonary HTN Single S2 = one semilunar valve (truncus), anterior aorta (TGA), pulmonary HTN Fixed, split S2 = ASD, PS Gallop (S3) — may be due to cardiac dysfunction/ volume overload Muffled heart sounds and/or a rub = pericardial effusion + tamponadePhysical Exam = Types of Murmurs Systolic Ejection Murmur (SEM) = turbulence across a semilunar valve Holosystolic murmur = turbulence begins with systole (VSD, MR) Continuous murmur = pressure difference in systole and diastole (PDA, BT shunt)Innocent murmurs = Peripheral pulmonic stenosis (PPS) Heard in newborns — disappears by one year of age (often earlier) Soft SEM at ULSB w/ radiation to axilla and back (often heard best in axilla/back) Need to differentiate b/w PPS and actual pulmonic stenosis. PS often associated with a valvular click and heard best over precordiumInnocent murmurs = Still's murmur Classic innocent murmur Heard most commonly in young children (3-5 yrs of age) but can be heard in all ages “Vibratory” low-frequency murmur often heard along LSB and apex Positional — increases in intensity when pt is in supine position Also louder in high output states (i.e. dehydration, fever) Need to differentate from VSDInnocent murmurs = Pulmonary flow murmur — Often heard in older children and adolscents Soft SEM at ULSB, little radiation; normal second heart sound — Not positional Need to differentiate b/w mild PS and especially an ASD ® Hint: ASD would have a fixed split second heart soundInnocent murmurs = Venous hum | Often heard in toddlers, young children Low pitched continuous murmur often heard best in infraclavicular area, normal heart sounds ~ Positional — diminishes or goes completely away when pt in supine position or with compression of jugular vein Need to differentiate between a PDAEE Syndrome Associations = Down — AV canal and VSD = Turner — CoA, AS = Trisomies 13 and 18 — VSD, PDA = Fetal alcohol - L—R shunts, ToF =™ CHARGE - conotruncal (ToF, truncus)EE Hereditary Diseases = Marfan (AD)- aortic root aneurysm + dissection, MVP, MR, Al HCM (AD) — outflow tract obstruction, arrhythmias Noonan (AD) — HCM, PS DMD/BMD (X-link) —- DCM (>12 y.o.) Williams (AD) — supravalvar AS Tuberous sclerosis — rhabdomyoma Romano-Ward — AD LQTS Jervell & Lange-Nielsen — AR LQTS & deafnessA Kawasaki Disease (KD) = Now the #1 cause of acquired heart disease = A systemic vasculitis (etiology-unknown) = Tests - CBC, CMP, CRP, ESR, EKG, ECHO = Rx -—IVIG at 2g/kg and high-dose ASA = Prognosis — Coronary artery dilatation in 15-25% wio IVIG and 4% wi IVIG (if given within 10 days of fever onset). Risk of coronary thrombosis.Kawasaki — Clinical criteria = Fever for at least 5 days AND 4 of the following 5 criteria: Eyes - conjunctival injection (ie- no exudate) Lips & mouth - erythema, cracked lips, strawberry tongue Hands & feet - edema and/or erythema Skin - polymorphous exanthem (ie- any rash) Unilateral, cervical lymphadenopathyRheumatic Fever A post-infectious connective tissue disease Follows GAS pharyngitis by 3 weeks (vs. nephritogenic strains of GAS) = Injury by GAS antibodies cross-reacting with tissue = Dx —JONES criteria (major and minor) = Tests — Throat Cx, ASO titer, CRP, ESR, EKG, +/- ECHO ™ Rx—PCN x10 days and high-dose ASA or steroids = 2 Prophylaxis — daily po PCN or monthly IM PCN. Rheumatic Fever — organs affected 1. Heart muscle & valves — myocarditis & endocarditis (pericarditis rare w/o the others) 2. Joints — polyarthritis 3. Brain — Sydenham’s Chorea (“milkmaid’s grip” or better yet, “motor impersistance”) 4, Skin — erythema marginatum (serpiginous border) due to vasculitis 5. Subcutaneous nodules — non-tender, mobile and on extensor surfaces: In case you haven’t had enough....Hypoplastic Left Heart Syndrome O A ductal-dependent lesion Q One ventricle pumps both PBF & SBF Q Difficult to balance PBF & SBFNorwood Procedure ™ What is the purpose of the BT shunt? = Is there a murmur? = What is your guess for the arterial saturation?Tricuspid atresia s/p bidirectional Glenn Bidirectional Glenn = What is the purpose of the Glenn? = Is there a murmur? = What is your guess for the arterial saturation?. | Tricuspid atresia s/p Fontan onca0 1 Fontan circuit = What is the path of blood? = Is there a murmur? = What is your guess for the arterial saturation?