pediatric cardiology - vfn.cz · atrial septal defect ... to maintain pda patency (tga)...
TRANSCRIPT
HISTORY
• Family history (sudden death, SIDS,
structural cardiac abnormality)
• Pregnancy (gestational diabetes
mellitus, chronic maternal illness,
congenital infections, drug use)
• Perinatal course
• Symptoms
SYMPTOMS AND SIGNS OF
HEART DISEASE
• rate of growth and development
• history of past illnesses
• feeding difficulties-congestive heart
failure (30% infants with CHD)
• tachypnea, dyspnea, grunting, flaring of
the alae nasi
• cyanosis (reduced hemoglobin >3-5gm%)
PHYSICAL EXAMINATION
• height, weight - growth chart
• heart and respiratory values
rate/min birth-6w 6w-2y 2-6y 6-10y over 10y
resp. 45-60 40 30 25 20
heart 125±30 115±25 100±20 90±15 85±15
• blood pressure measurement
appropriately sized cuffs, covers 2/3 of the full length of the arm
PHYSICAL EXAMINATION
• Arterial examination (radial and femoral
pulse - volume, character, delay) - CoA
• Venous examination - generally difficult or
impossible in infants and children
• Liver - position, size, consistency (1-2 cm
below the right costal margin by 1 year of
age)
• Precordial examination (bulge, thrills,
apical impulse)
DIAGNOSIS OF CHD
• PHYSICAL EXAMINATION
• BLOOD PRESSURE MEASUREMENT
• ECG , X-RAY
• ECHOCARDIOGRAPHY
• CARDIAC CATETRIZATION
• HOLTER MONITORING
• EXERCISE TESTING
AUSCULTATION
• under optimal condition
• supine, sitting and standing position
• first heart sound - atrioventricular valves closure
• second heart sound - aortic and pulmonary valve closure ( split during inspiration)
• cardiac murmurs
CARDIAC MURMURS
• systolic, diastolic, continuous
• intensity: graded as 1-6 / 6
1. heard with intense concentration
2. faint, but heard immediately
3. easily heard
4. associated with thrill
5. very loud
6. audible off the chest wall
INNOCENT MURMURS
OF CHILDHOOD
• Intensity grade 3 or less
• Never solely diastolic
• Never associated with thrill
• Diminishes and the pitch changes or
disappears with upright position
Vibratory Still´s, Pulmonary flow, Peripheral
pulmonary stenosis, Supraclavicular or
Brachiocephalic Systolic, Aortic systolic, Venousis hum,
ETIOLOGY OF CHD
• multifactorial
• 3% chromosomal aberrations
• 2% new mutations
• viremia during pregnancy, inflammation,
• treatment (hormones, insulin,
anticonvulsants, cytostatics)
RISK OF CHD IN FAMILY
one member two members
• VSD 3,0%
• PDA 3,0%
• ASD 2,5% 3 times higher
• TOF 2,5%
• PS 2,0%
• TGA 2,0%
• TA 1,0%
• PTA 1,0%
PREVALENCY OF CHD
7-8 / 1 000 newborns
40% of all congenital defects • 35% VSD
• 12% ASD
• 6% PDA, PS, AS, CoA
• 5% TGA
• 4% TOF
• 20% others
•
CLASSIFICATION OF CHD
• ANATOMICAL
• FUNCTIONAL
LEFT-TO-RIGTT SHUNT
RIGHT-TO-LEFT SHUNT
WITHOUT SHUNT
• URGENT - NONURGENT
LEFT-TO-RIGHT SHUNT
LESIONS
• Atrial septal defect
ostium secundum, ostium primum, sinus venosus, inferior vena cava
• Ventricular septal defect
perimembranous, supracristal, atrioventricular, muscular
• Patent ductus arteriosus
• Atrioventricular septal defect
• Aorticopulmonary window
ATRIAL SEPTAL DEFECT
1. sinus venosus
2. ostium secundum
3. ostium primum
4. cleft in the MV
septal leaflet
ATRIAL SEPTAL DEFECT
• Symptoms usually after 1. year of life
• Systolic murmur –excess blood flow through
the pulmonary valve, fixed splitting of second
sound
• ECG: hypertrophy of the RV, IRBB
• X-ray: increase pulmonary vascularity
• Echocardiogram: 2D, Doppler, color mapping
• Treatment: cardiac failure, surgical, catheterization
VENTRICULAR SEPTAL DEFECT
• Holosystolic murmur depends on the pressure (after the decrease of pulmonary vascular resistance, mostly in 2 month)
• ECG: normal or hypertrophy of LV, RV
• X-ray: increase pulmonary vascularity
• Echocardiogram: 2D, Doppler, color mapping
• Treatment: cardiac failure, surgical, catheterization
PATENT DUCTUS ARTERIOSUS
• Continuous murmur (in newborn infants systolic)
• ECG: normal or hypertrophy of LV, RV
• Chest X-ray: increase pulmonary vascularity
• Echocardiogram: 2D, Doppler, color mapping
• Treatment: surgical, catheterization, pharmacological in premature neonates
• In ductus dependent CHD prostaglandins
to maintain PDA patency (TGA)
RIGHT-TO-LEFT SHUNT
LESIONS (CYANOTIC)
MECHANISM
• 1. DECREASED PULMONARY
BLOOD FLOW ( TA, TS, PA, PS, TOF)
• 2. MALPOSITION OF GREAT
ARTERIES (TGA, DORV, DOLV,
Truncus arteriosus)
• 3. SINGLE VENTRICLE
TRANSPOSITION OF GREAT
ARTERIES
• Balloon atrial septostomy - Rashkind
• Surgical atrial switch- atrial baffle in the
atrium redirects deoxygenated blood
Mustard, Sennig procedure
ARTERIAL SWITCH
TETRALOGY OF FALLOT
• Cyanosis
• Polyglobulia
• Hypoxic attacks
• Surgery: close the hole and open
obstruction
TOF repair
• Closure of VSD with pericardial
patch
• Shifting aorta to LV
• Reconstruction of right ventricle
outflow tract
LESIONS WITHOUT SHUNT
• PULMONARY STENOSIS
• AORTIC STENOSIS
subvalvular, valvular, supravalvular
• COARCTATION OF AORTA
PULMONARY STENOSIS
• Symptoms related to aortic or pulmonary
stenosis depend on the degree of valve
stenosis.
• Systolic ejection murmur
• ECG: normal or ventricular hypertrophy
• Echocardiography: pressure gradient
across the valve
COARCTATION OF THE AORTA
• Clinical presentation depends on the age and
severity of the obstruction
• Preductal – critical CoA – cardiac failure in
neonates
• Postductal – elective repair between the ages 3
and 10 years
• Upper extremity hypertension, femoral pulse
• Systolic murmur medial to the left scapula
COARCTATION OF THE AORTA
surgical corrections
• Patch aortoplasty
• Subclavian flap repair
• Resection with end-to-end
anastomosis
Surgery - Kardiocentrum Prague
1977 – 2011
• 12 000 operated children
• 3096 operations before age 1
• Switch TGA 443
• TOF 199
• A-V canal 146
• TAPVD 148
• 97% survive 93% 10 years after surgery, 84% 15 years after surgery