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NORMAL HEART

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

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

INCIDENCE
CHD :
6-8/1000 live births
8 types of CHD (85%) :
VSD, ASD, PDA, PS, AS, TF, TGA
AHD :
Neonatus : virus
5 - 15 yrs : RF

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

FETAL CIRCULATION .

heart and upper extremities are supplied by high O2 content • Minimal pulmonary circulation . cross circulation Head. FETAL CIRCULATION Signs : Parallel systemic and pulmonary circulations Foramen ovale. ductus venosus  still open RA : enlargement. ductus Botalli.

d.CIRCULATION AFTER BIRTH After birth :  Expansion of lung & O2 uptake Systemic and pulmonary circulation  serial type No cross circulation in RA Foramen ovale. venosus  closed . Botalli & d.

Central C :  arterial unsaturation B.25 gr%)  2 types : A. Peripheral C :  without arterial unsaturation Distinction between A and B : measurement of arterial O2 content (N=95%) . Cyanosis  Reduced Hb > 5 gr% (N=2.

Central C : Pulmonary C :  Lung disorders  diffusion. ventilation. perfusion Cerebral 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 Children • Classification • CHD Cyanotic Type Non cyanotic type • AHD Rheumatic Heart Disease Rheumatic Fever Myocarditis Endocarditis .

Congenital Heart Disease • Non Cyanotic type VSD ASD PDA • Cyanotic type TOF Double Outlet Right Ventricel Great Artery Transposition .

Acyanotic Defect ↑ PBF Normal PBF LVH or CVH RVH LVH RVH VSD ASD AS or AR PS PDA PAPVR COA COA ECD PVOD MR MS Cyanotic ↑ PBF ↓PBF LVH or CVH RVH LVH RVH CVH Single Ventricel TGA AT TOF TGA + PS TGA + VSD HLHS AP + Hypoplastic RV PVOD .

Turner) . lithium  Radiation  Genetics (dominan autosomal)  Syndrome (Down. Noonan. Alcohol.Congenital heart disease (CHD) Etiology  Unknown  Mother’s disease (TORCH) Rubella  Medicine : fenitoin.

Congenital heart disease (CHD) Early signs of CHD  Cyanosis  Inadequate intake  Heart murmur & sounds  Unpalpable femoral & brachial pulse  Circulation collapse  Arrhythmia .

X Ray  Echocardiography  Catheterization . ECG.Congenital heart disease (CHD) Diagnosis  Anamnesis and physical examination  Simple investigation  Laboratory.

ATRIAL SEPTAL DEFECT (ASD) .

ATRIAL SEPTAL DEFECT (ASD)  Any opening (defect) in the atrial septum  shunt  Ostium Primum (15%) Ostium Secundum (50%-70%) . and Syst. circulation . Sinus venosus defect  Hemodynamic : depends on the  size  compliance of Ventricles  resistance of Pulm.

recurrent respiratory infection . HS 2nd N . murmur is found by chance  Fatigue. P2 intensity N . dyspnea. FTT  Ausc: ( murmur may be absent in infants) widely split and fixed S2. ATRIAL SEPTAL DEFECT (ASD) CONTINUED Signs/Symptoms :  Usually asymptomatic.

ATRIAL SEPTAL DEFECT (ASD) CONTINUED  X-ray: increased PBF . RVH  Echo : position and size of the defect  Catheterization : . RA and Pulmonal Conus protrude  ECG : RAD.

Management Spontaneous closure of ASD.5 : 1 . 40% (4 years) or become small Transcatheter closure (Amplatzer Septal Occluder) Surgical closure : Indication : P / S ratio ≥ 1.

Ventricular septal defect (vSD) .

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 LV
 Pressure LV > RV  L-R shunt
 R-L, L-R, R-L (Eisenmenger S)

Ventricular septal
defect (vSD) CONTINUED

 SIMPLE VSD

20 % of CHD, 25 % of VSD

Small 1-5 mm, Moderate 5-10 mm, RVH (-)

Asymptomatic : Roger’s disease ,

Ausc ( murmur holosistolik)

ECG and X Ray : normal

Ventricular septal
defect (vSD) CONTINUED

 MODERATE VSD
fatigue, intolerance activity, dyspnea,
recurrent respiratory tract infection, CHF
Pansystolic (holosystolic) 3-4/6, punctum
maximum LSB 3-5, P2 intensity >

LAH. Ventricular septal defect (vSD) CONTINUED  X-ray :  Increased PBF. location . size. LVH  ECG :  Small VSD : normal  Moderate VSD : LVH (+LAE) Catheterization : O2 in RV > RA ECHO : 2D & Doppler: number.

CHF. Endocarditis . Ventricular septal defect (vSD) CONTINUED  Management :  Nonsurgical closure : Amplatzer septal occluder  Surgical : infant with large VSD + CHF  Prognosis :  Perimembranous : surgical intervention  Muscular defect : spontaneous become small/ prolaps aorta . Infundibulum Stenosis. PH.

PATENT DUCTUS ARTERIOSUS (PDA) .

Botalli  Aorta  Extrauterine: d. F > M Anatomy/physiology : diameter mm . Botalli 10–15 hrs still open  L-R shunt (syst-diast)  continuous murmur (+ 90% PDA) .1 cm  Intrauterine: AP  d.PATENT DUCTUS ARTERIOSUS (PDA) Incidence : 12 % CHD (no. 2).

TYPICAL PDA (SIMPLE PDA) CONTINUED Clinical Manifestations : Asymptomatic. recurrent respiratory tract infection. RVH Echo : direction of shunt & Ø PDA Prognosis : rarely closed spontaneously (1 yr). except in premature babies . tachipneu Continuous murmur at LSB2. LVH. middiastolic murmur at apical X-ray : PBF >>.

TYPICAL PDA (SIMPLE PDA) CONTINUED Management : Surgical closure (ligation) Nonsurgical closure : Amplatzer Ductal Occluder .

Pulmonary stenosis (PS) .

Syst murmur LSB2 . Abn pulmonary Noonan’s syndrome Asymptomatic Difference of systolic pressure between RV and PA > 100 mmHg Hemodynamic : RV activity increased  RVH Pulmonary ejection click (valve opening)  Clinical Manifestation Eject.Pulmonary stenosis (PS) Incidens 5-7% of CHD .

dilated PA Cineangio : a jet contrast Management :  Balloon valvuloplasty  Surgery if balloon failt . cardiomegaly ECG : RAD.Pulmonary stenosis (PS) CONTINUED X-ray : PBF <<. RVH Echo : thick pulmonary valve.

COARCTATION OF THE AORTA (CoA) .

CoA CONTINUED Narrowing of the aorta. M > F Location : distal of left subclavian artery 2 types :  Preductal (CoA + Systemic LV/RV)  Postductal (CoA + Sytemic LV) . Frequency : 5 – 8% CHD.

etc) . intercostal. CoA CONTINUED  Hemodynamic : 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.

POSTDUCTAL CoA Clinical Manifestations Pain of calves. headaches. epistaxis Hypertension (pathognomonic) Brachial – Femoral lag Reduced / abcent lower extremity pulses .

balloon angioplasty . POSTDUCTAL CoA CONTINUED X-ray : Rib notching (collateral vessels) E sign on barium meals ECHO / Doppler : Gradient and pattern of diastolic flow Catheterization : Confirmation of diagnosis Management : Surgery.

TETRALOGY OF FALLOT (TF) .

PS. RVH.1 (75%) Hemodynamic :  PS + VSD  R-L shunt  Cyanosis/ acyanotic “pink TOF  R-L shunt  polycytemia & tromboemboly . Overriding of the Aorta Frequency : 10-15% CHD. cyanotic CHD nr. TETRALOGY OF FALLOT (TF) 4 Defects : VSD.

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

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

TRANSPOSITION OF THE GREAT ARTERIES (TGA) .

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

TRANSPOSITION OF THE GREAT ARTERIES (TGA) CONTINUED X-ray : like an egg on its side bootshaped heart (=TF) Echo : double circle. parallel PA & Ao Management : Balloon atrial septostomy Surgery palliative or arterial switch procedure .

DEXTROCARDIA .

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

DEXTROCARDIA CONTINUED Isolated mirror image dextrocardia (IMID) Kartagener syndrome: Dextrocardia / situs inversus Bronkhiectasis Paranasal sinusitis .

stomach bubble- right  Echo : dextrocardia Prognosis : depends on the lesions Treatment : overcome the associated lesions . DEXTROCARDIA CONTINUED Clinical Manifestations :  Loudest heart sound on the right chest  IMID 50-80% without CHD  X-ray IMID: liver – left.

What’s next? .

ASD .

VSD .

PDA .

SP .

CoA .

TF .

TGA .

DextrocardiaSolitus DextrocardiaInversus Dextrocardia .

VCS TOF AO AP AKI VCS VP VC I AKA VKI RA LA VKA T M AO RV LV AKa AP AO AKI AP VKA VKI .

PDA VCS VP VC I RA LA T M RV LV AO AP DA .

VSD VCS VP VC I RA LA T M RV LV AO AP .

ASD VCS VP VC I RA LA T M RV LV AO AP .