Heart Diagrams by Dr. James L. Wilkinson MB. ChB.,FRCP, FRACP, FACC, FRCPCH. (Royal Childrens Hospital, Melbourne, Australia)(http://www.cc.umanitoba.ca/~soninr)NORMAL HEART

GENERAL PRINCIPLES Pediatric Cardiology and Adult Cardiology Pediatric Cardiology : 1. Congenital Heart Disease (CHD) Occurs since organogenesis 2. Acquired Heart Disease (AHD) Disturbances occur after birth

INCIDENCECHD : 6-8/1000 live births8 types of CHD (85%) : VSD, ASD, PDA, PS, AS, TF, TGAAHD :Neonatus : virus5 - 15 yrs : RF

ETIOLOGY CHD : 90% genetic environmental factorsenvironment : 1st trimester pregnancy organogenesis of the heart : radiation, smoking, drugs (thalidomide), maternal infection (rubella), mother age (young/old), high geografic location (less O2), metabolic disorders (DM), Down syndrome (50% with CHD)AHD :infection (RF, diphtheriae)neonatus (Coxsackie B virus)

FETAL CIRCULATIONSigns :Parallel systemic and pulmonary circulationsForamen ovale, ductus Botalli, ductus venosus still openRA : enlargement, cross circulation Head, heart and upper extremities are supplied by high O2 content Minimal pulmonary circulation

CIRCULATION AFTER BIRTHAfter birth : Expansion of lung & O2 uptakeSystemic and pulmonary circulation serial typeNo cross circulation in RAForamen ovale, d. Botalli & d. venosus closed

Cyanosis Reduced Hb > 5 gr% (N=2,25 gr%)2 types : Central C : arterial unsaturationPeripheral C : without arterial unsaturation

Distinction between A and B : measurement of arterial O2 content (N=95%)

Central C :Pulmonary C :Lung disorders diffusion, ventilation, perfusionCerebral C:brain disorders center of respiration Cardial C: R-L shunt Hyperoxic (100% O2) test / Crying : pulmonary C less / no C intracardial C C still persist Peripheral C Decreased cardiac output

Heart Diseases in ChildrenClassification CHD Cyanotic Type Non cyanotic typeAHD Rheumatic Heart Disease Rheumatic Fever Myocarditis Endocarditis

Congenital Heart DiseaseNon Cyanotic type VSD ASD PDACyanotic type TOF Double Outlet Right Ventricel Great Artery Transposition

Acyanotic Defect PBFNormal PBFLVH or CVHRVHLVHRVHVSDPDAECDASDPAPVRPVODAS or ARCOAMRPSCOAMSCyanotic PBFPBFLVH or CVHCVHRVHLVHRVHSingle VentricelTGA + VSDTGAHLHSTGA + PSATAP + Hypoplastic RVTOFPVOD

Congenital heart disease (CHD)EtiologyUnknownMothers disease (TORCH) RubellaMedicine : fenitoin, Alcohol, lithiumRadiationGenetics (dominan autosomal)Syndrome (Down, Noonan,Turner)

Congenital heart disease (CHD)Early signs of CHDCyanosisInadequate intakeHeart murmur & soundsUnpalpable femoral & brachial pulseCirculation collapseArrhythmia

Congenital heart disease (CHD)DiagnosisAnamnesis and physical examinationSimple investigationLaboratory, ECG, X RayEchocardiographyCatheterization

ATRIAL SEPTAL DEFECT (ASD)

ATRIAL SEPTAL DEFECT (ASD)Any opening (defect) in the atrial septum shunt Ostium Primum (15%) Ostium Secundum (50%-70%) , Sinus venosus defectHemodynamic : depends on the size compliance of Ventriclesresistance of Pulm. and Syst. circulation

CONTINUEDATRIAL SEPTAL DEFECT (ASD)Signs/Symptoms :Usually asymptomatic, murmur is found by chanceFatigue, dyspnea, recurrent respiratory infection , FTTAusc: ( murmur may be absent in infants) widely split and fixed S2, HS 2nd N , P2 intensity N

CONTINUEDATRIAL SEPTAL DEFECT (ASD)X-ray:increased PBF , RA and Pulmonal Conus protrudeECG : RAD, RVHEcho :position and size of the defectCatheterization :

ManagementSpontaneous closure of ASD, 40% (4 years) or become smallTranscatheter closure (Amplatzer Septal Occluder)Surgical closure :Indication : P / S ratio 1.5 : 1

Ventricular septal defect (vSD)

Defect in the ventricular septum (perimembran, muscular, subarterial)Prevalence : CHD no. 1 (25%) Hemodynamic :Depends on the size and pressure between RV and LVPressure LV > RV L-R shuntR-L, L-R, R-L (Eisenmenger S) Ventricular septal defect (vSD)

Ventricular septal defect (vSD)CONTINUED SIMPLE VSD20 % of CHD, 25 % of VSDSmall 1-5 mm, Moderate 5-10 mm, RVH (-)Asymptomatic : Rogers disease , Ausc ( murmur holosistolik) ECG and X Ray : normal

Ventricular septal defect (vSD)CONTINUED MODERATE VSDfatigue, intolerance activity, dyspnea, recurrent respiratory tract infection, CHFPansystolic (holosystolic) 3-4/6, punctum maximum LSB 3-5, P2 intensity >

Ventricular septal defect (vSD)CONTINUED X-ray : Increased PBF, LAH, LVH ECG : Small VSD : normalModerate VSD : LVH (+LAE)Catheterization : O2 in RV > RAECHO : 2D & Doppler: number, size, location

Ventricular septal defect (vSD)CONTINUEDManagement : Nonsurgical closure : Amplatzer septal occluderSurgical : infant with large VSD + CHFPrognosis : Perimembranous : surgical interventionMuscular defect : spontaneous become small/ prolaps aorta , Infundibulum Stenosis, PH, CHF, Endocarditis

PATENT DUCTUS ARTERIOSUS (PDA)

PATENT DUCTUS ARTERIOSUS (PDA)Incidence : 12 % CHD (no. 2), F > MAnatomy/physiology : diameter mm - 1 cmIntrauterine: AP d.Botalli AortaExtrauterine: d. Botalli 1015 hrs still openL-R shunt (syst-diast) continuous murmur (+ 90% PDA)

TYPICAL PDA (SIMPLE PDA)Clinical Manifestations :Asymptomatic, recurrent respiratory tract infection, tachipneuContinuous murmur at LSB2, middiastolic murmur at apical X-ray : PBF >>, LVH, RVHEcho : direction of shunt & PDAPrognosis : rarely closed spontaneously (1 yr), except in premature babiesCONTINUED

TYPICAL PDA (SIMPLE PDA)

Management : Surgical closure (ligation)Nonsurgical closure : Amplatzer Ductal Occluder CONTINUED

Pulmonary stenosis (PS)

Pulmonary stenosis (PS)

Incidens 5-7% of CHD , Abn pulmonary Noonans syndromeAsymptomaticDifference of systolic pressure between RV and PA > 100 mmHgHemodynamic :RV activity increased RVHPulmonary ejection click (valve opening) Clinical Manifestation Eject. Syst murmur LSB2

Pulmonary stenosis (PS)CONTINUEDX-ray : PBF

COARCTATION OF THE AORTA (CoA)

CoACONTINUEDNarrowing of the aorta.Frequency : 5 8% CHD, M > FLocation : distal of left subclavian artery2 types : Preductal (CoA + Systemic LV/RV)Postductal (CoA + Sytemic LV)

CoACONTINUEDHemodynamic : Adequate O2 to distal of CoA : (Adaptation mechanism)Increased systolic pressure at proximal of CoA Increased diastolic pressure at distal of CoA (arterioles vasoconstriction)Collateral circulation (a subclavian, intercostal, etc)

POSTDUCTAL CoAClinical ManifestationsPain of calves, headaches, epistaxisHypertension (pathognomonic)Brachial Femoral lagReduced / abcent lower extremity pulses

POSTDUCTAL CoACONTINUEDX-ray :Rib notching (collateral vessels)E sign on barium meals ECHO / Doppler : Gradient and pattern of diastolic flowCatheterization : Confirmation of diagnosisManagement :Surgery, balloon angioplasty

TETRALOGY OF FALLOT (TF)

TETRALOGY OF FALLOT (TF)4 Defects : VSD, PS, RVH, Overriding of the AortaFrequency : 10-15% CHD, cyanotic CHD nr.1 (75%)Hemodynamic :PS + VSD R-L shuntCyanosis/ acyanotic pink TOFR-L shunt polycytemia & tromboemboly

TETRALOGY OF FALLOT (TF)CONTINUEDClinical Manifestation : Clubbing fingers, scoliosis, squatting position Ejection systolic murmur LSB3-4, single HS 2nd Lab : Hb, Ht, RBC levels increased

TETRALOGY OF FALLOT (TF)CONTINUEDEcho : VSD, Overriding Ao, RVOT obstructionX-Ray : couer en sabot, RVH, PBF , concav pulmonary segment Complication : Cerebral Infarction (age < 2 yrs) Cerebral Absces (age > 2 yrs)Treatment : Surgery : palliative / total correction (BT Shunt, Glenn, Waterstone)

TRANSPOSITION OF THE GREAT ARTERIES (TGA)

TRANSPOSITION OF THE GREAT ARTERIES (TGA)Ventriculoarterial discordance, Ao RV and PA - LVCyanotic CHD no.2, incidens 3-5%CHD, M > FHemodynamic :parallel pulmonary and systemic circulation (cyanosis)To prolong life : mixing of oxy- and deoxygenated blood (ASD, VSD, PDA)deficient O2 supply to the heart, enlargement of the heart, heart failure

TRANSPOSITION OF THE GREAT ARTERIES (TGA)CONTINUEDX-ray : like an egg on its side bootshaped heart (=TF)Echo : double circle, parallel PA & Ao Management :Balloon atrial septostomySurgery palliative or arterial switch procedure

DEXTROCARDIA

DEXTROCARDIACONTINUEDThe heart is located on the right side of the chest & the apex points to the right. Dextroposition is not a DIAGNOSIS.Anatomy :1. Visceroatrial relationship : S (solitus), I (inversus) or A (ambiguus)2. Ventricular Loop : D (D-loop), L (L-loop) or X (uncertain or undeterminate)3. Great arteries (conotruncal) : S (solitus), I (inversus), D (D-transposition) or L (L-transposition)

DEXTROCARDIACONTINUEDIsolated mirror image dextrocardia (IMID)Kartagener syndrome: Dextrocardia / situs inversusBronkhiectasisParanasal sinusitis

DEXTROCARDIACONTINUEDClinical Manifestations :Loudest heart sound on the right chestIMID 50-80% without CHDX-ray IMID: liver left, stomach bubble- rightEcho : dextrocardiaPrognosis : depends on the lesionsTreatment : overcome the associated lesions

Whats next?

ASD

VSD

PDA

SP

CoA

TF

TGA

DextrocardiaDextrocardiaInversusDextrocardiaSolitus

TOFRARVLALVVCSVC IAPAOVPTMVCSAOAPAKAVKAVKIAKIAKaVKAVKIAKIAPAO

PDARARVLALVVCSVC IAPAOVPDATM

VSDRARVLALVVCSVC IAPAOVPTM

ASDRARVLALVVCSVC IAPAOVPTM