Cardiac Disorders

Epidemiology

• 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 anti-La antibody)

35%

Diabetes Mellitus Incidence increased overall 2%

Chromosomal Abnormality

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%

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

CyanosisScenario O2%

SaturationO2%

DesaturationHemoglobin

Concentration (g/dl)

Deoxygenated Hemoglobin

Comments

1 95% 5% 14 g/dl 0.7 g/dl Normal, No Cyanosis

2 85% 15% 14 g/dl 2.1 g/dl Cyanosis

3 85% 15% 5 g/dl 0.75 g/dl Anemia, No Cyanosis

4 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)

– 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/CARDIACCARDIAC – Significant reduction of cardiac outputArrhythmia:

– 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 PainCARDIAC 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)• 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 FailureSymptoms• 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 FailureNeonates – 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• 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 DiseaseCaused 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 vasculitisMnemonic ¨Warm CREAM¨

Warm = Fever C = Conjunctivitis R = Rash - ErythematousE = Erythema palms and soles – With SwellingA = Adenopathy, cervical – 1 Unilateral nodeM = 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

• 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

• 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.• 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): – 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

• 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 MurmursType (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 yearOccasionally in infancy

Pulmonary ejection murmur (Systolic)

Maximal at ULSB, Early to mid-systolic, Grade 1-3/6 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 newbornsUsually disappears by 3 – 6 months of age

Venous hum (Continuous)

Maximal at right (or left) supraclavicular or infraclavicular areas. Grade 1-3/6 in intensity. Inaudible in supine position. Intensity changes with rotation of head and compression of jugular vein.

3 – 6 year

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

• 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 PressureAge 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