This document discusses persistent truncus arteriosus, a congenital heart defect where a single arterial trunk arises from the heart to provide blood flow to the systemic, pulmonary, and coronary circulations. It describes the embryology, anatomy, classification, presentation, diagnosis, and treatment of the condition. Surgical repair aims to close the ventricular septal defect, commit the common trunk to the left ventricle, and reconstruct the right ventricular outflow tract. Outcomes have improved with early corrective surgery, though additional anomalies increase mortality risk.

1. Dr.S.Sivasankar SR MCh, JIPMER
24.10.2012

2.  Persistant truncus arteriosus
 Truncus Arteriosus communis
 Common aortico pulmonary trunk
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3. • Definition
• History
• Embryology
• Anatomy and classification
• Pathophysiology
• Presentation
• Workup
• Treatment
• Conclusion
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4. • 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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5. • TOF with pulm. Atresia with MAPCA (Collet Edwards type 4)
• Hearts with common arterial trunk but, intact septum
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8. 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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9. 1971 – first conduit repair in infancy by Barratt-Boyes
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11. • incomplete or failed septation of the embryonic truncus
arteriosus
• Or abnormality of conotruncal septation
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12. Primitive mesoderm & neural crest cells
Heart & great vessels
Give
rise
to
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13. 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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14. Left & right endocardial
tubes
Bulbous cordis
Fuse
at 23
days
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16. At this period, bulbo ventricular structures rotate anteriorly and
to the right
to form the heart loop
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18. Trunco-conal swellings
Trunco-conal ridges
Truncal septum
Fuse
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19. Truncal septum divides aorta from
Pulmonary artery and
Conal septum
Supraventricular crest and subpulmonic infundibulum
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20. Day 37
Fusion of conal septum with endocardial cushions establishes
ventricular separation
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23. • 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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24. 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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25. • 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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28. 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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30. 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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32. Type 3
branch pulmonary arteries originate independently from the
common trunk
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34. Type 4
Pseudo-truncus;
TOF with pulm. Atresia with MAPCA
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37. Type A1
Identical to the type I of Collett and Edwards
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39. Type A2
Collett and Edwards type II and most cases of type III
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41. 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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43. Type A4
coexistence of an interrupted aortic arch
not by the pattern of origin of branch pulmonary arteries
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52.  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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53. As the PBF increases PAH
Increased pulmonary venous return
CCF
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54. History
• Cyanosis at birth
• Early CCF
• Failure to thrive,
• Respiratory tract infections
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55. 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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56. 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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57.  Prenatal and early postnatal diagnosis common
 Proper evaluation of cyanotic infants clinches diagnosis
ABG
Pulse oximetry
ECG
CXR
TTE, TOE
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58. ECG
• Normal QRS axis
• Bi-ventricular hypertrophy – 70%
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59. CXR
• Cardiomegaly
• Pulmonary plethora
• Right aortic arch – 30%
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60.  Echo – single truncal valve; no pulmonary valve
 CARDIAC CATHETERISATION
• Delineation of anatomy in complex forms.
• Assess PVR in late presentations.
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61. Medical
Surgical
• Palliative
• Defintive
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62.  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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63.  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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64. Significant late deaths due to re-operations
truncal valve replacement
conduit replacement (now treated more with PCI)
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65. 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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66.  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.
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86. Thank you
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