CARDIAC DISORDERS
Cardiac Abnormalities
Maternal Disorders
Rubella Infection
Systemic Lupus Erythematous
(SLE)
Diabetes Mellitus
Peripheral Pulmonary Stenosis, PDA
Complete heart block (anti-Ro and anti-La
antibody)
Incidence increased overall
Chromosomal Abnormality
Down Syndrome (T 21)
AVSD, VSD
Edwards Syndrome (T 18)
Complex
Patau Syndrome (T 13)
Complex
Turner Syndrome (45 XO)
Aortic Valve Stenosis, CoA
Chromosome 22q11.2 Del
Aortic arch anomalies, TOF, common
arterial trunk
Noonan Syndrome (PTPN11
mutation and others)
Hypertrophic cardiomyopathy, ASD, PS
Symptoms
• Cyanosis, especially central
• Shortness of Breath
• Easy Fatigability & Failure to Thrive
• Squatting
• Hypoxic spells (TET spell, cyanotic spell)
• Syncope
• Palpitation
• Chest Pain
Cyanosis
 CENTRAL
o Cyanotic CHD
o Lung Disease
o Bluish Discoloration: Lips, Nail beds, Mucosa, Skin
 PERIPHERAL (acrocyanoisis)
o Peripheral Body Part
o Vasoconstriction due to cold weather or poor cardiac
output
Shortness of Breath
• Increase pulmonary blood flow
• Left to Right shunt
• Pulmonary vascular resistance (about 3 Wood units) systemic
vascular resistance (25 Wood units)
• Engorged lungs vasculature, interstitial edema, the excess fluid in
the lungs tissues – barrier for proper gaseous exchange
• Composition increase respiratory rate and effort = Respiratory
Distress
Hypoxic Spell (tet spell, cyanotic spell)
• Young infants 2-4 month TOF
• Paroxysm of hyperpnea
• Irritability and prolonged crying
• Increasing cyanosis
• Decreased intensity of heart murmur
• Severe spell – limpness, convulsions, cerebrovascular accident or
death
• Children with Tetralogy of Fallot exhibit bluish skin during
episodes of crying or feeding = ¨Tet spell¨
• Pathophysiology:
Decreased SVR/Increase respiratory RVOT will Increase R-L shunt
– hyperpnea
Hyperpnea Increase systemic venous return which Increase R-L
shunt through VSD
Squatting
• Tetralogy of Fallot – Before squatting
o Reduced pulmonary flow
o Increased aortic flow
• Tetralogy of Fallot – After squatting
o Increased pulmonary flow
o Reduced Aortic Flow
o Increased venous return(sustained squatting)
Palpitation
• Abnormal heart rhythm:
o Too slow
o Too fast
o Just irregular
Children may complain of chest pain when experiencing arrhythmias.
Syncope
NEUROLOGICAL/CARDIAC
CARDIAC – Significant reduction of cardiac output
Arrhythmia:
– HR too fast to allow for proper filling of ventricles prior
to contraction reduced cardiac output
– HR too slow to generate adequate cardiac output
Obstruction to blood flow:
– LVOT obstruction, severe hypertrophy of the ventricular
septum
– Obstruction of RVOT, such as with TOF
Cardioneurogenic Syncope: Reduced venous return and bradycardia
 drop in cardiac output
Chest Pain
CARDIAC REASONS (rarely)
• Myocardial infarction (ALCAPA)
Coronary arterial wall thickening in Williams Syndrome or
Kawasaki Disease (in the majority of these cases chest pain is not
verbalized)
• Pericarditis
• Arrhythmia
NON-CARDIAC REASONS
• Costochondritis: Viral inflammation of the costochondral joints
(usually viral illness)
• Musculo-skeletal: due to muscle strain such as with exercies,
particularly weight lifting, worsening when using involved
muscles
• Pleural-pericardial pain: due to inflammation
• Skin disease: such as herpes zoster, or other lesions
Heart Defects
Left-to-right shunts (Breathless)
• Ventricular Septal Defect (VSD) 30%
• Persistent Arterial Duct (PDA) 12%
• Atrial Septal Defect (ASD) 7%
Right-to-left shunts (Blue)
• Tetralogy of Fallot (TOF) 5%
• Transposition of the great arteries (TGA) 5%
Common mixing (Breathless and Blue)
• Atrioventricular Septal Defect (Complete) (AVSD) 2%
Outflow obstruction in a wall child (Asymptomatic with a
murmur)
• Pulmonary stenosis (PS) 7%
• Aortic Stenosis (AS) 5%
Outflow obstruction in sick neonate (collapsed with shock)
• Coarctation of the aorta (CoA) 5%
Heart failure
Symptoms
• Breathlessness (particularly on feeding or exertion)
• Sweating
• Poor feeding
• Recurrent chest infection
Signs
• Poor weight gain or ¨Faltering Growth¨
• Tachypnea
• Tachycardia
• Heart murmur, gallop rhythm
• Enlarged Heart
• Hepatomegaly
• Cool Peripheries
Right side Heat Failure
• Signs of right heart failure (ankle edema, sacral edema and
ascites) are in developed counties, but may be seen with longstanding rheumatic fever or pulmonary hypertension, with
tricuspid regurgitation and right atrial dilation
Etiology of Hear Failure
Neonates – obstructed (duct-dependent) systemic circulation
• Hypoplastic left heart syndrome
• Critical aortic valve stenosis
• Severe coarctation of the aorta
• Interruption of the aortic arch
Infants(High pulmonary blood flow)
• Ventricular Septal Defect
• Atrioventricular Septal Defect
• Large persistent Ductus Arteriousus
Older children and adolescents (right or left heart failure)
• Eisenmenger syndrome (right heart failure only)
• Rheumatic heart disease
• Cardiomyopathy
Other Heart Disease
• Kawasaki Disease
o Mainly in young children, may leave the heart muscle or
coronary arteries damaged
• Myocarditis – DCM, arrhythmias
• Cardiomyopathy
o A disease of the heart muscle, caused by a genetic disorder
or after an infection. It leads to poor heart function (HCM,
RCM, DCM, ARV/D)
• Rheumatic Heart Disease
o Caused by rheumatic fever, this disease leads to heart
muscle and valve damage
• Bacterial endocarditis
• Pericarditis
• Arrhythmias
o Abnormal heart rhythm created by a disturbance in the
hearts electrical system
Kawasaki Disease
Small and medium vessel vasculitis - Mnemonic ¨Warm CREAM¨
• Warm = Fever
• C = Conjunctivitis
• R = Rash - Erythematous
• E = Erythema palms and soles – With Swelling
• A = Adenopathy, cervical – 1 Unilateral node
• M = Mucous Membrane – Dry, red, strawberry tongue
Complication:
– Coronary artery aneurysm and Myocarditis
Physical Examination – Inspection
General condition assesment: Happy or cranky, nutritional state,
respiratory status (tachypnea, dyspnea), pallor (vasoconstriction from
CHD or circulatory shock or severe anemia), sweat on the forehead.
• Physical Development
• Dysmorphic features
• Cyanosis
• Edema
• Clubbing of Digits
• Left-sided chest prominence (precordial bulge)
• Visible ventricular impulse
Edema
• Is not a common feature of CHF in children
• Best detected over the sacral region, particularly in infants
• Swelling of the head and distended neck veins is noted in
patients with Glenn shunt and increased pulmonary vascular
resistance
Clubbing of Digits
• Occurs because of hypoxia (peripheral tissues are most
vulnerable to hypoxia, capillaries opening causes swelling of
the digits)
• Clubbing is seen in other lesions with low oxygen supply such
as with lung diseases or chronic anemia
Precordial Bulge
• With or without actively visible cardiac activity
• Caused by chronic cardiac enlargement
• Pectus Carinatum (Pigeon Chest) – usually not a result of heart
enlargement
• Pectus Excavatum (Depression of sternum) may be a cause of
pulmonary systolic murmur
Visible Ventricular Impulse
• RV Impulse
Under the Xiphisternum
• LV Impulse (apex beat)
Frequently visible in children
Hyperdynamic circulation (fever or excitement)
LV enlargement
Physical Examination - Palpation
• Precordium palpation
• Peripheral perfusion
• Femoral and brachial arterial pulses
• Peripheral pulses
• Hepatomegaly
• A palpable thrill
Precordium Palpation
• RV enlargement – fingertips placed between 2nd and 3rd – 4th
ribs along the left sternal edge – Abnormal palpation of RV is
called a tap or a lift.
• The apex beat – 4th intercostal space infants, 4th – 5th
schoolchild midclavicular line – LV hypertrophy – diffuse,
forceful and displaced apex beat – the feeling is described as a
heave.
• If the apical beat is difficult to ascertain, ask the child to roll
over onto their left side and breath out
Peripheral Perfusion
• Capillary refill time
• Normally is 1-2 seconds in duration
• Prolonged indicates poor cardiac output
• A brisk capillary refill is seen, despite poor cardiac output in
cases where the peripheral vasculature are forced to vasodilate
such as with sepsis or the use of pharmacologic agents
Pulses (Examination)
• The rate (Value ex. Rheumatic fever: Fixed tachycardia, loss of sinus
arrhythmia)
• Irregularities (Arrhythmias)
o Sinus arrhythmia increase on inspiration, slowing on
expiration
• Volume
• Localization:
o Radial, brachial and femoral arteries
o Use finger pulps
o Femoral often difficult to palpate
 (If diminished check radio-/brachio-/femoral delay)
o Palpation of the dorsalis pedis pulse excludes coarctation in
infancy
Femoral and Brachial arterial pulse
• Should be felt simultaneously to assess their strength and timing
• CoA femoral is weaker and delayed in timing when compared to
the brachial arterial pulse
• It is important when doing this assessment to use the right
brachial arterial pulse, as the left subclavian may be involved or
distal in its origin to the coarctation and will therefore be as weak
as the femoral arterial pulse
Peripheral pulse
• Give a sense of the cardiac output, systolic and diastolic pressures
• Poor cardiac output result in low systolic and high diastolic blood
pressure = narrow pulse pressure
• Low diastolic BP, such as with PDA or aortic regurgitation will
cause = wide pulse pressure
Pulse Paradoxus – change in pulse volume with respiration 
CARDIAC TAMPONADE
Palpable Thrill
A palpable thrill over the precordium or suprasternal notch indicates
significant murmur.
• Location
• ULSB – PS
• URSB – AS
• LLSB – VSD
• Suprasternal notch – AS, occasionally PS, PDA or COA
• Over the carotid arteries – AS or COA
Physical Examination - Auscultation
• Sounds first, murmurs second
• Try to ensure the child is not crying
• Use both diaphragm and Bell
• Listen to the child in lying and sitting position
• Note any variation with respiration
Auscultation - Sounds First
• First heart sound (S1):
o Best heard at the apex with bell closure of atrio-ventricular
valves
• Second heart sound (S2):
o Best heard at the base with the diaphragm, usually split in
children – widens on inspiration
• A2:
o Closure of aortic valve
• P2:
o Closure of pulmonary valve
• Added sounds:
o Gallop rhythm: (S3, 34)
Auscultation - Murmurs second
• Problems
o Hearing them at all
o Distinguishing between significant and innocent
• Hints
o Majority is systolic until proven otherwise
o Try to wipe out all extraneous noise and listen between S1
and S2 using both diaphragm and bell
Murmur mnemonic
• Grade 1: Barely audible
• Grade 2: Soft, variable, innocent usually
• Grade 3: Easy to hear, intermediate, no thrill
• Grade 4: Loud, audible to anybody, thrill
• Grade 5: Sound like a train, very significant, thrill
• Grade 6: Scarcely required a stethoscope, thrill
Innocent murmurs (physiological, flow murmur)
• 30-50% (80%)
• High output state
o Increased fever
• Mnemonic: 4xS
• S = aSymptomatic
• S = Soft
• S = Left Sternal Edge
• S = Systolic only
Systolic murmur
• Holosystolic murmur:
o Indicate shunting of blood between two structures in which
the pressure in one structure is higher than the other
throughout systole
o Example:
 Harsh: VSD
 Soft: Atrio-ventricular valve regurgitation
• Ejection systolic murmur:
o Increase in blood flow turbulence as systole progresses due
to an increasing amount of blood flow through a restricted
orifice
o Example
 Aortic stenosis
 Pulmonary stenosis
 Small VSD
• Mid-systolic murmur:
o Increase volume of blood flowing through normal valve
o ASD
o Anemia
Diastolic Murmur
• Early diastolic murmur:
o Regurgitate blood flow from aorta or pulmonary artery into
the ventricles
 Aortic insufficiency
 Pulmonary insufficiency
• Late diastolic murmur:
o Austin Flint murmur
o Aortic regurgitation blood flow causes vibration of left
ventricular free wall
• Systolic and diastolic murmur:
o Pressure difference between two structures during systole
and diastole
 PDA
 Shunts and collaterals
 AS and Al
Blood Pressure
• Patience, practice and selection of cuffs
• Right arm
• Seated or standing
• Size – inner bladder encircles arm, width – 40-50% of the
circumference of the arm or leg
• Doppler ultrasound recording – neonates and infants
• Sphygmomanometer – older children
• Arm – heart – sphygmomanometer on the same horizontal plane
Normal blood pressure
Age
Systolic
BP
Diastolic
BP
Upper limit
(+2SD)
Neonates
60 - 70
40
90/52
1 – 4 year
90
62
110/80
6 year
100
66
120/82
10 year
110
70
130/88
14 year
120
74
140/92
Mnemonic hints:
• SBP at the age of 6 year 100 mmHg – than 2,5 mm/year
thereafter
DBP 60 + age in years