Transcript Cardiac Disorders

Cardiac Disorders
Epidemiology
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mostly congenital
8/1000 life born infants with significant c.m.
1/10 stillborn infants
10-15% complex lesions
10-15% non-cardiac abnormality
Cardiac Abnormalities
Frequency %
Maternal Disorders
Rubella Infection
Peripheral Pulmonary Stenosis, PDA
30-35 %
Systemic Lupus
Erythematous (SLE)
Complete heart block (anti-Ro and antiLa antibody)
35%
Diabetes Mellitus
Incidence increased overall
2%
Down Syndrome (T 21)
AVSD, VSD
30%
Edwards Syndrome (T 18)
Complex
60-80%
Patau Syndrome (T 13)
Complex
70%
Turner Syndrome (45 XO)
Aortic Valve Stenosis, CoA
15%
Chromosome 22q11.2 Del
Aortic arch anomalies, TOF, common
arterial trunk
80%
Noonan Syndrome(PTPN11
mutation and others)
Hypertrophic cardiomyopathy, ASD, PS
50%
Chromosomal Abnormality
Symptoms
Pre-natal & Neonatal History
• Circulation change
• The need to stay in the hospital for prolonged
period
• Left to Right shunting lesions, such as ASD,
BVSD and PDA to be silent du to low velocity
shunting
Symptoms
• Cyanosis, especially central
• Shortness of Breath
• Easy Fatigability(sweating during feeding –
infants) & Failure to Thrive
• Squatting
• Hypoxic spells (tet spell, cyanotic spell)
• Syncope
• Palpitation
• Chest Pain
Cyanosis
• CENTRAL
– Cyanotic CHD
– Lung Disease
– Bluish Discoloration: Lips, Nail beds, Mucosa, Skin
• PERIPHERAL (acrocyanoisis)
– Peripheral Body Part
– Vasoconstriction due to cold weather or poor
cardiac output
Cyanosis
Scenario
O2%
Saturation
O2%
Desaturation
Hemoglobin
Concentration
(g/dl)
Deoxygenated
Hemoglobin
Comments
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95%
5%
14 g/dl
0.7 g/dl
Normal, No
Cyanosis
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85%
15%
14 g/dl
2.1 g/dl
Cyanosis
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85%
15%
5 g/dl
0.75 g/dl
Anemia, No
Cyanosis
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95%
5%
25 g/dl
1.25 g/dl
Borderline
Cyanosis
• Increase during crying
• Deep pressure blanched area will not pink up as quickly in central cyanosis
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
Easy Fatigability & Failure to Thrive
• Suckle required considerable effort – easy to
fatigability and failure to thrive
Hypoxic Spell (tet spell, cyanotic spell)
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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
– Reduced pulmonary flow
– Increased aortic flow
• Tetralogy of Fallot – After squatting
– Increased pulmonary flow
– Reduced Aortic Flow
– Increased venous return(sustained squatting)
Palpitation
• Abnormal heart rhythm:
– Too slow
– Too fast
– 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
Left-to-right shunts (Breathless)
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Ventricular Septal Defect (VSD) 30%
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Persistent Arterial Duct (PDA) 12%
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Atrial Septal Defect (ASD) 7%
Right-to-left shunts (Blue)
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Tetralogy of Fallot (TOF) 5%
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Transposition of the great arteries (TGA) 5%
Common mixing (Breathless and Blue)
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Atrioventricular Septal Defect (Complete) (AVSD) 2%
Outflow obstruction in a wall child (Asymptomatic with a murmur)
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Pulmonary stenosis (PS) 7%
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Aortic Stenosis (AS) 5%
Outflow obstruction in sick neonate (collapsed with shock)
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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
• Signs of right heart failure (ankle edema,
sacral edema and ascites) are in developed
counties, but may be seen with long-standing
rheumatic fever or pulmonary hypertension,
with tricuspid regurgitation and right atrial
dilation
Cause of Heart 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 Diseases
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Kawasaki Disease
Mainly in young children, may leave the heart muscle or coronary arteries
damaged
Myocarditis – DCM, arrhythmias
Cardiomyopathy
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
Caused by rheumatic fever, this disease leads to heart muscle and valve damage
Bacterial endocarditis
Pericarditis
Arrhythmias
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
– 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
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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
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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
• Check for:
– The rate (Value ex. Rheumatic fever: Fixed tachycardia, loss
of sinus arrhythmia)
– Irregularities (arrhythmias)
• Sinus arrhythmia increase on inspiration, slowing on expiration
– Volume
– Localization:
• Radial, brachial and femoral arteries
• Use finger pulps
• Femoral often difficult to palpate
– (if diminished check radio-/brachio-/femoral delay)
• Palpation of the dorsalis pedis pulse excludes coarctation in
infancy
Femoral and Brachial arterial pulses
• 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 Pulses
• 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
Hepatomegaly
• Hepatomegaly, rarely hepato-spleenomegaly
is seen in CHF due to elevated central venous
pressure
Palpable thrill
A palpable thrill over the precordium or
suprasternal notch indicates significant murmur.
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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
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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):
– Best heard at the apex with bell closure of atrio-ventricular
valves
• Second heart sound (S2):
– Best heard at the base with the diaphragm, usually split in
children – widens on inspiration
• A2:
– Closure of aortic valve
• P2:
– Closure of pulmonary valve
• Added sounds:
– Gallop rhythm: (S3, 34)
Murmurs second
• Problems
– Hearing them at all
– Distinguishing between significant and innocent
• Hints
– Majority is systolic until proven otherwise
– Try to wipe out all extraneous noise and listen
between S1 and S2 using both diaphgram and bell
Murmur mnemonic
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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 murmurs)
• 30-50% (80%)
• High output state
– increased fever
• Mnemonic: 4xS
• S = aSymptomatic
• S = Soft
• S = Left Sternal Edge
• S = Systolic only
Usual features
Mid-systolic
Soft in intensity (Grade 1-3)
Localized
Poorly conducted
Musical or vibratory in character
Variable with position and respiration
Not associated with other signs of heart disease
Common Innocent Heart Murmurs
Type (Timing)
Description of murmur
Age group
Classic Vibratory
Murmur (Still Murmur)
Systolic
Maximal at MLSB or between LLSB and apex.
Low frequency vibratory, twanging string,
groaning, squeaking or musical.
3-6 year
Occasionally in infancy
Pulmonary ejection
murmur (Systolic)
Maximal at ULSB, Early to mid-systolic, Grade 13/6 in intensity. Blowing in quality
8 – 14 year
Pulmonary flow murmur
Maximal at ULSB, Transmits well to left and right
chest, axillae and back. Grade 1-2/6 in intensity
Premature and full-term
newborns
Usually disappears by 3 – 6
months of age
Venous hum
(Continuous)
Maximal at right (or left) supraclavicular or
3 – 6 year
infraclavicular areas. Grade 1-3/6 in intensity.
Inaudible in supine position. Intensity changes
with rotation of head and compression of jugular
vein.
Carotid bruit (Systolic)
Right supraclavicular area and over carotid.
Grade 2-3/6 in intensity. Occasional thrill over
carotid
Any age
Significant murmurs
Significant murmurs: Usual Features
Pansystolic
Conducted all over precordium
Soft to loud (Grades 4 – 6 ) in intensity
Associated with a thrill
Accompanied by other signs, e.g.. Ventricular enlargment
Any diastolic
Systolic murmur
• Holosystolic murmur:
– Indicate shunting of blood between two structures in which the pressure in
one structure is higher than the other throughout systole
– Example:
• Harsh: VSD
• Soft: Atrio-ventricular valve regurgitation
• Ejection systolic murmur:
– Increase in blood flow turbulence as systole progresses due to an increasing
amount of blood flow through a restricted orifice
– Example
• Aortic stenosis
• Pulmonary stenosis
• Small VSD
• Mid-systolic murmur:
– Increase volume of blood flowing through normal valve
– ASD
– Anemia
Diastolic murmur
• Early diastolic murmur:
– Regurgitate blood flow from aorta or pulmonary artery into the
ventricles
• Aortic insufficiency
• Pulmonary insufficiency
• Late diastolic murmur:
– Austin Flint murmur
– Aortic regurgitation blood flow causes vibration of left ventricular free
wall
• Systolic and diastolic murmur:
– Pressure difference between two structures during systole and
diastole
• PDA
• Shunts and collaterals
– AS and Al
Blood pressure
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Patience, practice and selection of cuffs
Right arm
Seated or standing
Size – inner bladder encircles arm, width – 4050% 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
Mnemoic hints:
• SBP at the age of 6 year 100 mmHg – than 2,5 mm/year thereafter
• DBP 60 + age in years