truncus arteriosus dr shiva ctvs jipmer
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TRUNCUS ARTERIOSUSDr.S.Sivasankar SR MCh, JIPMER
24.10.2012
April 11, 2023 Dr S.Sivasankar
Persistant truncus arteriosus Truncus Arteriosus communis Common aortico pulmonary trunk
April 11, 2023 Dr S.Sivasankar
• Definition• History• Embryology• Anatomy and classification• Pathophysiology• Presentation• Workup• Treatment• Conclusion
April 11, 2023 Dr S.Sivasankar
Definition
• Congenital cyanotic cardiac defect with a single common arterial trunk giving rise to systemic, pulmonary and coronary circulations proximal to brachiocephalic branches
• Associated with a large perimembranous VSD below the truncus
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Exclusions
• TOF with pulm. Atresia with MAPCA (Collet Edwards type 4)
• Hearts with common arterial trunk but, intact septum
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History
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1798 – Wilson documents 1st case
1942 –Basic morphologic criteria - Lev and Safir
1949 – Collet & Edwards Classification
1962 – Ist ICR with PTFE (non valved) conduit University of Michigan
1965 – Van Praaghs alternative classification
1967 – Ascending aortic allograft and valved conduit - McGoon et al.
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1971 – first conduit repair in infancy by Barratt-Boyes
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Embryology
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• incomplete or failed septation of the embryonic truncus arteriosus
• Or abnormality of conotruncal septation
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Primitive mesoderm & neural crest cells
Heart & great vessels
Give rise to
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Blood islands of cardiogenic plate
Left and right endocardial tubes
Intra embryonic coelom (early pericardial cavity)
Coalesce at 20 days
within
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Left & right endocardial tubes
Bulbous cordis
Fuse at 23
days
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At this period, bulbo ventricular structures rotate anteriorly and
to the rightto form the heart loop
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Trunco-conal swellings
Trunco-conal ridges
Truncal septum
Fuse
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Truncal septum divides aorta fromPulmonary artery andConal septum
Supraventricular crest and subpulmonic infundibulum
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Day 37Fusion of conal septum with endocardial cushions
establishes ventricular separation
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Anatomy and classifications
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• Single aortopulmonary trunk from base of heart and all 3 circulations arising from it
• Large perimembranous VSD (obligatory) below truncus• Truncal valve – bi, tri or quadricuspid and often
incompetent.• Pulmonary artery arise in several patterns• Truncal overriding equally in 60 – 80%, to right in 10-30%, left in 4 –
6%
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Coronary anomalies • Stenotic ostia, • Single ostium• high & low take off,• abnormal branching & course anterior descending from RCA & cross RV circumflex from RCA RCA from LAD intramuscular course
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Other associations
• Right aortic arch – 30%• Interrupted aortic arch – 10% (distal to left common
carotid)• Di George syndrome with hypocalcemia - 33%• PFO• OS-ASD• Tricuspid valve lesions• 22q11 chromosome deletion
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Collett and Edwards
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Collett and Edwards
Type 1 single pulmonary trunk from the left lateral aspect of
the common trunk, with branching of the left and right pulmonary arteries
from the pulmonary trunk
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Collett and Edwards
Type 2 separate but proximate origins of the left and right
pulmonary arterial branches from the posterolateral aspect of the common trunk
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Collett and Edwards
Type 3 branch pulmonary arteries originate independently from
the common trunk
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Collett and Edwards
Type 4 Pseudo-truncus; TOF with pulm. Atresia with MAPCA
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Van Praaghs
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Van Praaghs
Type A1 Identical to the type I of Collett and Edwards
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Van Praaghs
Type A2Collett and Edwards type II and most cases of type III
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Van Praaghs
Type A3 (hemitruncus)• one branch pulmonary artery (usually the right) from the
common trunk• The other branch pulmonary artery from the aortic arch
(a subtype of Collett and Edwards type III) or by systemic to pulmonary arterial collaterals
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Van Praaghs
Type A4coexistence of an interrupted aortic archnot by the pattern of origin of branch pulmonary arteries
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Pathophysiology
Cyanotic congenital heart disease with increased pulmonary blood flow
Fetal pulmonary blood flow less than 10% PVR falls in early infancy improving PBF hence good
oxygen saturation Hypoxia in this period implies pulmonary arterial
narrowing Equilibration of RV LV pressures
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As the PBF increases PAH
Increased pulmonary venous return
CCF
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Clinical Features
History• Cyanosis at birth• Early CCF• Failure to thrive, • Respiratory tract infections
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Clinical Features
Physical examination• Cyanosis• Signs of CCF• Bounding peripheral pulses, wide pulse pressure• Single S2• Harsh systolic regurgitant murmur – VSD• EDM – truncal valve regurgitation
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Natural history
Survival 50% survival in 1 month 18% survival in 6 months 12% survival in 1 year Modes of death . Congestive heart failure in early life . SBE, cerebral abscess → Eisenmenger syndrome (death in 3rd
decade) . Adversely affected by truncal regurgitation, IAA, CoA . Survival is favorably affected by PS
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Workup
Prenatal and early postnatal diagnosis common Proper evaluation of cyanotic infants clinches diagnosis ABG Pulse oximetry ECG CXR TTE, TOE
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Workup
ECG• Normal QRS axis• Bi-ventricular hypertrophy – 70%
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Workup
CXR• Cardiomegaly• Pulmonary plethora• Right aortic arch – 30%
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Workup
Echo – single truncal valve; no pulmonary valve
CARDIAC CATHETERISATION• Delineation of anatomy in complex forms.• Assess PVR in late presentations.
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Treatment
MedicalSurgical• Palliative• Defintive
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Medical
Treat CCF – diuretics, digoxin Ensure oxygen delivery – intubation and mechanical
ventilation Hypocalcemia correction in syndromic Prostaglandin I.V.in Van Praagh Type A4
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Surgical outcome
Improved greatly after early corrective surgery policy Increased mortality is associated with other coexisting
anomolies, truncal valve insufficiency presurgically truncal valve replacement• Survival rates – 92% at 1 year; 60% at 20 years
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Surgical outcome
Significant late deaths due to re-operations truncal valve replacement conduit replacement (now treated more with PCI)
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Surgery
complete primary repair• closure of the ventricular septal defect• committing the common arterial trunk to the left
ventricle• reconstruction of the right ventricular outflow tract.
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Median sternotomy Aortic cannula placed distally at base of innominate Bicaval cannulation RSPV vent Pulmonary arteries snared Full flow, moderate hypothermia for simple forms( I & II) Cold antegrade cardioplegia +/- RCP if there is truncal regurgitation.
April 11, 2023 Dr S.Sivasankar
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BARBERO MARCIAL MODIFICATION
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REPAIR OF TRUNCUS ARTERIOSUS WITH INTERRUPTED AORTIC ARCH
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REPAIR OF TRUNCUS ARTERIOSUS WITH INTERRUPTED AORTIC ARCH
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TRADITIONAL APPROACH
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Thank you