A brief presentation about Truncus Arteriosus surgical management.

1. Truncus Arteriosus
Clinical Features and Surgical Management
Navy Laksmono
Thoracic, Cardiac and Vascular Surgery Program
Faculty of Medicine Universitas Indonesia

2. Definition
“congenital malformation
which a single arterial trunk
arises from the heart, overrides
the interventricular septum and
supplies systemic,
pulmonary, and coronary
circulations”
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History, Techniques, Results and Indications. 4 ed.
Philadelphia: Elsevier; 2013:1608-30.
Mavroudis C, Backer CL. Truncus Arteriosus. Pediatric Cardiac Surgery. 4 ed. Philadelphia: Blackwell
Publishing; 2013:361-75.
1864
••Buchanan, first autopsy
1942
••Lev and saphir, morphology criteria
1949
••Collet and Edwards classification
1962
••Michigan uni, repair using nonvalved PTFE (11 years
survival)
1965
••Van praagh classification
1967
••Rastelly, aortic allograft with valve
••McGoon aortic allograft and valve conduit
1971
••Barrat-Boyes first infant surgery 6wk
••Binet, xenograft in polyester cylinder
Historical Perspective

3. Anatomy Morphology
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History, Techniques, Results and Indications. 4 ed.
Philadelphia: Elsevier; 2013:1608-30.
Truncal Arteries
• Single vessel from base of
heart
• Larger than normal aorta
• Usually more in RV
• Arise coronary arteries and PA
Pulmonary Arteries
• Classification based on PA
origin
Ascending Aorta and Ductus
Arteriosus
• When truncal and asc aorta in
continuation and well
developed PDA usually not
exist
• When PDA exist, transverse
arch usually absent and asc ao
underdeveloped. (IAA)

4. Coronary Arteries
• Orifices of coronary arteries have
a variable relationship to the
sinuses of valsava above truncal
cusps
• 80% are at the margin of the sinus
or at upper margin of the
commisure
• 2/3 of cases LCA rise form left
posterior aspect of truncal artery
and RCA from right anterior
aspect
• 18% single ostium
• Rare, coronary artery rise from PA
Semilunar Valve
• Single truncal valve, posterior and
inferior in position
• Fibrous continuity between
posterior cusp and anterior mitral
leaflet
• It may have 3 cusp, 4 cusp or
bicuspid
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History, Techniques, Results and Indications. 4 ed.
Philadelphia: Elsevier; 2013:1608-30.

5. Ventricular Septal Defect
• VSD is high, anterior, large and
juxtatruncal in position
• Superior margin by truncal valve (U
shaped)
• Inferior and anterior bounded by two
division of septal band (TSM)
• Posterior by free wall mucle band,
separates TrV from TV
(ventriculoinfundibular fold)
Right Ventricle
• Infundibular (conal) septum is absent
from RVOT
Left Ventricle
• Relatively normal
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History, Techniques, Results and Indications. 4 ed.
Philadelphia: Elsevier; 2013:1608-30.

6. Associated Anomalies
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History, Techniques, Results and Indications. 4 ed.
Philadelphia: Elsevier; 2013:1608-30.

7. Embryology & Pathogenesis
Moore, K.L., Persaud, T.V.N. & Torchia, M.G. (2015). The developing human: clinically oriented embryology (10th ed.). Philadelphia: Saunders

8. Clinical Features and Diagnostic Criteria
• Symptoms
• Tachypneu, tachycardia, irritability, signs of heart failure
• Respiratory distress
• Compression of left upper lobe bronchus between LPA (anterior displaced)
and posterior desc Ao
• Aneurysmal truncal artery in IAA
• compress right main bronchus, total right lung collapse
• Cynanosis usually mild, except in longer survivor with PVOD (eisenmenger)
• Physical exam
• Jerky, collapsing arterial pulse (rapid runoff from truncal artery to PA)
• Overactive heart
• Prominent left parasternal systolic murmur and thrill
• Ejection click of truncal valve
• Aortic early diastolic murmur (truncal regurgitation)
• 2nd heart sound single
• Continous murmur in stenosis of PA
• Truncal valve stenosis -> peripheral pulse decrease
Park MK. Cyanotic Congenital Heart Defects. Pediatric Cardiology for Practitioners. Philadelphia: Elsevier; 2014.
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History, Techniques, Results and Indications. 4 ed.
Philadelphia: Elsevier; 2013:1608-30.

9. Clinical Features and Diagnostic Criteria
• Chest Xray
• Cardiomegaly, increased
pulmonary vasculature, Right Ao
Arch (30%)
• Electrocardiogram
• ECG : bilateral ventricular
hipertrophy (mostly LVH
dominant), Right axis slight
deviation
• Cathetherization
• Define PA anatomy
• Assess hemodynamic state (PA
pressure and PVR
• Response to oxygen test and
operability
Park MK. Cyanotic Congenital Heart Defects. Pediatric Cardiology for Practitioners. Philadelphia: Elsevier; 2014.
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History, Techniques, Results and Indications. 4 ed.
Philadelphia: Elsevier; 2013:1608-30.

10. Clinical Features and Diagnostic Criteria
• Echocardiography
• A large VSD directly under truncal valve
• Large single artery arises from heart, type of truncus arteriosus
• Only one semilunar valve (truncal valve is imaged)
• Presence of TrV regurgitaion
• Right aortic arch, interrupted aortic arch
Park MK. Cyanotic Congenital Heart Defects. Pediatric Cardiology for Practitioners. Philadelphia: Elsevier; 2014.
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History, Techniques, Results and Indications. 4
ed. Philadelphia: Elsevier; 2013:1608-30.

11. Natural History
• Rare cases, 2.8% of CHD
• Survival
• 50% survived 1 mo
• 30% survived 3 mo
• 10% survived 1 y.o
• When PVOD developed can survive another 1-15 years (eisenmenger)
• Concomitant lesion, affects adversely
• AV valve regurgitation
• IAA
• Coarctation Aorta
• LV hipoplasia
• Mitral atresia
• CAVSD
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History, Techniques, Results and Indications. 4 ed.
Philadelphia: Elsevier; 2013:1608-30.

12. Indication of Operation
• Every truncus arteriosus is indicated for operation
• 50% will not survived 1 month without surgery
• Do early as possible
• PVOD
• Most occur onset in 6 months
• Catheterization is prior
• If reactive to oxygen (PVR fall below 7 W.U) surgery is advisable
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History, Techniques, Results and Indications. 4 ed.
Philadelphia: Elsevier; 2013:1608-30.

13. Technique of Operation
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History,
Techniques, Results and Indications. 4 ed. Philadelphia: Elsevier; 2013:1608-30.
Jonas RA. Ventciular Septal Defect with Pulmonary Stenosis. Comprehensive Surgical
Management of Congenital Heart Disease. 2 ed. Washington: Taylor & Francis Group; 2014.
Repair Truncus without IAA, using RV-PA autologous conduit

14. Technique of Operation
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History,
Techniques, Results and Indications. 4 ed. Philadelphia: Elsevier; 2013:1608-30.
Repair Truncus without IAA, using homograft patch
“Barbero-Marcial”

15. Technique of Operation
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History,
Techniques, Results and Indications. 4 ed. Philadelphia: Elsevier; 2013:1608-30.
Repair Truncus with IAA

16. Technique of Operation
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History,
Techniques, Results and Indications. 4 ed. Philadelphia: Elsevier; 2013:1608-30.

17. Postoperative Care and Survival
• Beware of PH crisis
• Follow up every 4 -12 months
• Evaluation of TrV regurgitation
• Conduit may need changed to larger size by 2 or 3 year
• Calcification of the conduit may occur
Park MK. Cyanotic Congenital Heart Defects. Pediatric Cardiology for Practitioners. Philadelphia: Elsevier; 2014.
Kouchoukos NT, Blackstone EH, Hanley FL, Kirklin JK. Truncus Arteriosus. Kirklin/Barrat-Boyes Cardiac Surgery: Morphology, Diagnostic Criteria, Natural History, Techniques, Results and Indications. 4 ed.
Philadelphia: Elsevier; 2013:1608-30.

23. Thank you
And have a nice day