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Congenital heart disease 2 (Tetralogy of Fallot (TOF) (Diagnosis…
Congenital heart disease 2
Aortic stenosis (AS)
obstruction at valve level caused by imperfect cusp development with leaflet thickening and fusion
produced pressure gradient between the left ventricle and aorta.
3-8 % of congenital cardiovascular defects
4 :1 male : female ratio
most offen - bicuspid valve
Clinical manifastations
most children are asymptomatic
fatigability (10-30 %), angina pectoris (5-9 %)
syncope, sudden death, endocarditis
Diagnosis
Phisical examination
Auscultation: Systolic murmur over base of heart and over aorta radiated to the carotid vassels
Palpation: precordial thrill
ECG
Hypertrophy of left ventricle
ST segment depressions
T wave inversions
Chest X-ray
Normal in most patients
Left ventricular (and left atrial) enlargement
Poststenotic dilatation of aorta
Echocardiography
aortic valve morphology
left ventricle hypertrophy and function
LV-Ao systolic gradient
associated lesions
Cardiac catheterisation
pressure gradient and left ventricular function
therapeutic catheterisation (valvuloplasty)
Treatment
Indication
LV-Ao pressure gradient
LV systolic and diastolic function
LV hypertropy (ecg, x-ray, echocardiography)
clinical manifestation: fatigability, angina pectoris, syncope
endocarditis
surgical: valvuloplasty, valve implantation, Ross procedure
nonsurgical: balloon aortic valvuloplasty
Aortic valve valvuloplasty is method of choice in congenital aortic stenosis without aortic insufficiency in patients < 21 years old
Tetralogy of Fallot (TOF)
congenital heart malformation characterised by underdevelopment of right ventricular infundibulum producing right ventricle outflow stenosis and large VSD.
10 % of all CHD
Right-to-left shunt leads to early cyanosis
Anatomy
ventricular septal defect
pulmonary stenosis: subvalvar, valvar, supravalvar
aortic override
RV hypertrophy
Physiology
RVOT obstruction with VSD resulting in:
RV to LV shunting / systemic blood desaturation / decreaced pulmonary vascular flow
pO2 and SO2 depending upon the severity of pulmonary stenosis
elevated Hb and Ht levels
symptoms
Clinical symptoms are related on severity of PS (pulmonary flow)
cyanosis – may be present at birth or may appear later
episodes of intense cyanosis with abnormal respirations – cyanotic spells (tet spells)
limited exercise tolerance
poor growth
symptoms of heart failure - very rare
Cyanotic spells:
more common in the morning hours after awakening and may be precipitated after activity
increased cyanosis
Softening or disappearence of systolic murmur
rapid respirations
sometimes child become letharrgic or convulsions appear
Diagnosis
Auscultation:
pulmonary stenosis ejection murmur
single second sound
X-ray
normal size of heart ('Boot-shaped' heart – upturned apex)
decreased pulmonary vascular markings
ECG:
right axis deviation
right ventricular hypertrophy
echocardiography:
RVOT stenosis and RV morphology, VSD
overriding of aorta, additional defects
cardiac catheterisation:
pulmonary artery morphology (hypoplasia, stenosis)
RVOT stenosis and RV morphology
VSD, overriding of aorta
additional defects (coronary arteries pathology)
Treatment
surgical: paliative procedures (shunt), corective operation
nonsurgical: pulmonary valve and pulmonary artery menagement (balonoplasty, stent)
Results of treatment
operation dependent morality –> 2-8 %
residual VSD
residual RVOTO or pulmonary artery stenosis
severe PV insufficiency
dysrhythmias -> sudden death
A-V block III
Transposition of the great vessels
Characterized by pulmonary artery arising from the left ventricle and aorta arising from the right ventricle
Associated with maternal diabetes
Presents with early cyanosis; pulmonary and systemic circuits do not mix
Results in hypertrophy of the right ventricle and atrophy of the left ventricle
TRUNCUS ARTERIOSUS
single large vessel arising from both ventricles (Truncus fails to divide)
Presents with early cyanosis; deoxygenated blood from right ventricle mixes with
oxygenated blood from left ventricle before pulmonary and aortic circulations separate.
TRICUSPID ATRESIA
Tricuspid valve orifice fails to develop; right ventricle is hypoplastic.
Often associated with ASD, resulting in a right-to-left shunt; presents with early cyanosis.