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Dr. Pravin Asir Abraham.
This lecture is based on generalizations and common cases presented at the pediatrics dept, AMH. There are many more CHDs than what I’ve listed and I hope you can use these principles to help you out with those.
Cardiac cases at AMH
Newborn referralsCardiac murmurs. Cyanosis. Respiratory symptoms. Pediatric referrals-Cardiac murmurs. Chest pain evaluation. Palpitation evaluation. Syncope evaluation. Evaluation of genetic syndromes. Pre-operative evaluation. Evaluating cardiac involvement in systemic diseases.
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 (R→L atrial level shunt) ductus arteriosus (R→L 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 .
Neonatal Circulation Pulmonary resistance (PVR) is high. so initially RV pressure ~ LV pressure RV pumps to pulmonary circulation and LV pumps to systemic circulation 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 .
Varying degrees of acrocyanosis. . S2 may be single for first few days of life. An ejection click representing pulmonary hypertension heard in first hours of life. Maximal cardiac impulse at LLSBRV hyperactive.Normal cardiac findings in newbornsHR range 100-180.
contd. Peripheral pulses are bounding due to lack of subcutaneous tissue.Normal cardiac findings in newborns. Pulmonary flow murmur-grade 12/6 systolic ejection murmur(innocent murmur)characteristically radiates to sides and back of the chest. . This prevalence and loudness increased in preterms.
5-1% of all live births Simple way to classify is: shunts Cyanotic CHD (R→L shunts) Obstructive lesions L→R .Congenital Heart Disease (CHD) Occurs in 0.
May not be apparent in neonate due to high PVR (ie. AVSD (or complete atrioventricular canal) 1.L→R Shunts (“Acyanotic” CHD) Defects VSD 2. ASD 4.bidirectional shunt) . PDA 3.
and poor growth Left heart enlargement (LHE) Transmits flow and pressure Presents in childhood w/ murmur or exercise intolerance (AVSD or 1o ASD presents earlier) Right heart enlargement (RHE) Transmits flow only AVSD can present as either depending on size of ASD & VSD component .L→R Shunts –General Points PDA /VSD(5-10/15-20) ASD(5-10) Presents in infancy w/ heart failure. murmur.
Increased PBF Left Heart Overload Right Heart Overload .
Pulm vasc markings equal in upper and lower zones Cardiomegaly .
now R→L) and the patient becomes cyanotic .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.
In reality most of these defects can present with low or high PBF (eg.R→L Shunts (CCHD) • “Blue blood bypasses the lungs” • Degree of cyanosis varies • Classify based on pulmonary blood flow (PBF) ↑ PBF Truncus arteriosus Total anomalous pulmvenous return (TAPVR) Transposition of the great arteries (TGA) ↓ PBF Tetralogy of Fallot Tricuspid atresia Ebstein’s anomaly Please note: This is a generalization.ToF with little PS acts more like a VSD with high PBF) .
Equal pressures here too 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 . extreme pulmonary overcirculation. thus.R→L Shunts ↑ PBF There is unimpeded PBF.
R→L Shunts ↓ PBF Presents more often with cyanosis See oligemic lung fields Closure of PDA may worsen cyanosis Dynamic subvalvular obstruction here causes “Tet spells” Why are pressures equal? .
90% Pulmonary overcirculation 70% Too little PBF 70% 99% 60% 99% 99% 99% 70% 60% .
Different amounts of PBF (Truncus vs ToF) .
3. Mild-moderate AS 2.Obstructive Lesions 1. 2. Mild-moderate CoA 3. or HTN (in CoA) Not cyanotic .cyanosis Needs PGE1 Non-Ductal Dependent 1. Mild-moderate PS Presents in older child w/ murmur. Ductal Dependent Critical PS/AS(8-12/3-6) Critical CoA/IAA(8-10/1) HLHS Presents in CV shock at 2-3 days of age when PDA closes +/. exercise intolerance.
) .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 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 .
COMMON PEDIATRIC REFERRALS .
e. fever) Need to differentate from VSD . dehydration.Innocent murmurs Still’s murmur Classic innocent murmur Heard most commonly in young children (35 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.
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 . little radiation.Innocent murmurs Pulmonary flow murmur Often heard in older children and adolscents Soft SEM at ULSB.
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 .
mastalgia Costochondritis(9-22) Musculoskeletal trauma(21) Lung(15-21) Psychogenic(5-9) GI(4-7) Sickle cell crisis(2) Cardiac(0-4) .CHEST PAIN IN CHILDREN Idiopathic(12-45)-precordial catch.
Arrhythmias(WPW syndrome. HOCM.LQT) . CAD(ALCAPA or kawasaki disease sequelae) Cocaine abuse. Pericarditis-pericardiotomy syndrome)/Myocarditis.Cardiac pains Severe AS. Severe PS. Mitral Valve prolapse.
Palpitations in children .
Syncope in children Vasovagal syncope. pallor.SVT. HOCM. nausea. diaphoresis.AS. PS. Orthostatic hypotension.with prodromedizziness. arrhythmias(WPW. hyperventilation followed by LOC. CVOD. palpitation.SSS) . LQTS. CHB.RV dysplasia. Cardiac causes.No prodrome-light headedness. Myocardial dysfunction.VT.
AS Trisomies 13 and 18 – VSD. truncus) .Syndrome Associations Down – AV canal and VSD Turner – CoA. ToF CHARGE – conotruncal (ToF. PDA Fetal alcohol – L→R shunts.
AI HCM (AD) – outflow tract obstruction. MVP.) Williams (AD) – supravalvar AS Tuberous sclerosis – rhabdomyoma Romano-Ward – AD LQTS Jervell & Lange-Nielsen – AR LQTS & deafness . arrhythmias Noonan (AD) – HCM. MR.o.Hereditary Diseases Marfan (AD)– aortic root aneurysm ± dissection. PS DMD/BMD (X-link) – DCM (>12 y.
Acquired Valvular disease.Pre-operative evaluation Indications for prophylaxis . .High risk.prosthetic cardiac valve. previous bacterial endocarditis. CCHD. Surgically constructed systemic to pulmonary artery conduits. HCM. MVP with MR or thickened leaflets. .Moderate risk.Other CHD.
IE prophylaxis for DORE procedures Oral.Amoxicillin 50mg/kg 1 hr before Sx. . Allergic to PCN and unable orallyClindamycin 20mg/kg or Cefazolin 25mg/kg within 30mts. Inj. Allergic to PCN.Cephalexin or Cefadroxil 50mg/kg/ Azithromycin 15mg/kg 1 hr before Sx.Ampicillin 50mg/kg IM or IV within 30 minutes before Sx.
IE prophylaxis for GI and GU High risk-Ampi 50mg/kg IM or IV(2g)+genta 1. Ampi 25mg/kg IM or IV 6hr(OR)Vanco 20mg/kg IV over 1-2 hr+genta 1mg/kg IM or IV.5mg/kg within 30mts. Moderate risk-Amox 50mg/kg orally 1hr or ampi 50mg/kg IM or IV within 30mts(OR)Vanco 20mg/kg IV over 1-2hr. .
CMP. EKG. ESR. ECHO Rx – IVIG at 2g/kg and high-dose ASA Prognosis – Coronary artery dilatation in 15-25% w/o IVIG and 4% w/ IVIG (if given within 10 days of fever onset). Risk of coronary thrombosis. . CRP.Kawasaki Disease (KD) Now the #1 cause of acquired heart disease.A systemic vasculitis (etiology-unknown) Tests – CBC.
polymorphous exanthem (ie.edema and/or erythema Skin . cervical lymphadenopathy .erythema.any rash) Unilateral.Kawasaki – Clinical criteria Fever for at least 5 days AND 4 of the following 5 criteria: Eyes .conjunctival injection (ie.no exudate) Lips & mouth . cracked lips. strawberry tongue Hands & feet .
EKG. CRP.Rheumatic Fever A post-infectious connective tissue disease Follows GAS pharyngitis by 3 weeks (vs. ASO titer. +/. nephritogenic strains of GAS) Injury by GAS antibodies cross-reacting with tissue Dx – JONES criteria (major and minor) Tests – Throat Cx. ESR.ECHO Rx – PCN x10 days and high-dose ASA or steroids 2o Prophylaxis – daily po PCN or monthly IM PCN .
Rheumatic Fever – organs affected 1. 5. “motor impersistance”) Skin – erythema marginatum (serpiginous border) due to vasculitis Subcutaneous nodules – non-tender. 3. Heart muscle & valves – myocarditis & endocarditis (pericarditis rare w/o the others) Joints – polyarthritis Brain – Sydenham’s Chorea (“milkmaid’s grip” or better yet. 4. 2. mobile and on extensor surfaces .
HEART-FELT THANKS .