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Persistent Truncus arteriosus (Pathology (Truncal valve (Bicuspid,…
Persistent Truncus arteriosus
Prevalence
<1% of all congenital heart defects
Pathology
Single arterial trunk
Large VSD
Perimembranous
and Infundibular
Truncal valve
Bicuspid
Tricuspid
Quadricuspid
Incompetent
Collet and Edward's Classification (Branching of pulmonary arteries)
Type 1
Main PA arises from truncus
Then divides to RPA and LPA
Increased pulmonary blood flow
Quite prevalent
Type 2
PA arises from back of truncus
Normal pulmonary blood flow
Type 3
PA arises from lateral of truncus
Normal pulmonary blood flow
Type 4
Pseudotruncus arteriosus arises from descending aorta to supply lungs
Decreased pulmonary blood flow
Severe form of TOF
with pulmonary atresia
with aortic collaterals supplying the lung
Associated abnormalities
Coronary artery abnormalities
Abnormal branching
High and low takeoff of coronary arteries
Abnormal courses of coronary artery
Stenotic coronary ostia
Interupted Aortic arch
13% of cases
Type A4 Van Praagh classification
DiGeorge Syndrome with hypocalcemia - 33% patients
Clinical Manifestation
History
Cyanosis immediately after birth
CHF - several days to week age
Dyspnea with feeding
Failure to thrive
Frequent respiratory infection
Physical examination
Cyanosis
CHF + Tachypnea + Dyspnea
Bounding pulse
Wide pulse pressure
Hyperactive precordium
Displaced apical impulse to lateral
Systolic click frequently audible
Apex
Upper left sternal border
Regurgitant systolic murmur (grade 2 4/6)
ECG
QRS Axis normal
Bi-Ventricular Hypertrophy present in 70% of cases
Left atrial hypertrophy occasionally present
X-Ray
Right aortic arch 30% of cases
Cardiomegaly
Increased pulmonary vascularity
Echocardiography
Large VSD under truncal valve
Truncus arteriosus
Truncal valve
Right aortic arch
Natural history
CHF first 2 week
85% die in 1 year if untreated
Clinical improvement occurs
if Develop pulmonary vascular obstructive disease
Occur in 3-4 months
Death occur in 30ies
Truncal valve insuf will be worsened in tme
Management
Medical
Associated with DiGeorge
Chek Mg and Ca level
Prophylaxis
Against
Pneumococcal
Streptococcal
Due to Thymus based-immune deficiency
Immunization with live vaccine avoided
Prophylaxis against SBE
Surgical
Paliative procedures - PA banding
High mortality (30%)
Does not prevent pulmonary vascular obstructive diseases
Definitive procedures
Rastelli procedure
Ideally - 1st week of life
if delayed diagnosis --> after 2-3 days medical stabilization
