PEDIATRIC CARDIOLOGY - vfn.cz · atrial septal defect ... to maintain pda patency (tga)...

PEDIATRIC CARDIOLOGY

Zuzana Urbanová

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%

Fetální kardiologie

FETAL ECHOCARDIOGRAPHY

FETAL ECHOCARDIOGRAPHY HYPOPLASTIC LEFT HEART SYNDROMA

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

•

PREVALENCY OF CHD

ASDAS CoA

TOF

0

10

20

30

40

50

%

VSD PDA PS TGA 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


• 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

ASD AMPLATZ

ASD – AMPLATZ

VENTRICULAR SEPTAL DEFECT

1. supracristal

2. perimembranous

3. muscular

4. apical


• 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


• Treatment: cardiac failure, surgical, catheterization

PERIMEMBRANOUS VSD

MUSCULAR VSD

SURGICAL REPAIR OF VSD

ATRIOVENTRICULAR CANAL

PATENT DUCTUS ARTERIOSUS

PDA - ECHO


• Continuous murmur (in newborn infants systolic)

• ECG: normal or hypertrophy of LV, RV

• Chest X-ray: increase pulmonary vascularity


• 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

TRICUSPID ATRESIA

TRANSPOSITION OF GREAT

ARTERIES

TGA - echocardiography

PA

A

O

LV

RV

TGA-prenatal ECHO

TRANSPOSITION OF GREAT

ARTERIES

• Balloon atrial septostomy - Rashkind

• Surgical atrial switch- atrial baffle in the

atrium redirects deoxygenated blood

Mustard, Sennig procedure

ARTERIAL SWITCH

SURGICAL REPAIR OF TGA

TETRALOGY OF FALLOT

1. VSD

2. RV HYPERTROFY

3. OVERRIDING OF

AORTA

4. SUBPULMONARY

STENOSIS

TETRALOGY OF FALLOT

TOF - ECHO

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

SURGICAL REPAIR OF TOF

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

PULMONARY STENOSIS

BALLOON DILATATION OF

CRITICAL PS

SURGICAL REPAIR OF PS

COARCTATION OF THE AORTA

INTERRUPTION OF THE

AORTIC ARCH


• 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


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

Surgical mortality rate

Kardiocentrum Prague (1980-2010)

PEDIATRIC CARDIOLOGY - vfn.cz · atrial septal defect ... to maintain pda patency (tga)...

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