truncus arteriosus dr shiva ctvs jipmer

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

• Definition• History• Embryology• Anatomy and classification• Pathophysiology• Presentation• Workup• Treatment• Conclusion

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

Exclusions

• TOF with pulm. Atresia with MAPCA (Collet Edwards type 4)

• Hearts with common arterial trunk but, intact septum

History

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.

1971 – first conduit repair in infancy by Barratt-Boyes

Embryology

• incomplete or failed septation of the embryonic truncus arteriosus

• Or abnormality of conotruncal septation

Primitive mesoderm & neural crest cells

Heart & great vessels

Give rise to

Blood islands of cardiogenic plate

Left and right endocardial tubes

Intra embryonic coelom (early pericardial cavity)

Coalesce at 20 days

within

Left & right endocardial tubes

Bulbous cordis

Fuse at 23

At this period, bulbo ventricular structures rotate anteriorly and

to the rightto form the heart loop

Trunco-conal swellings

Trunco-conal ridges

Truncal septum

Truncal septum divides aorta fromPulmonary artery andConal septum

Supraventricular crest and subpulmonic infundibulum

Day 37Fusion of conal septum with endocardial cushions

establishes ventricular separation

Anatomy and classifications

• 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 –

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

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

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

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

Collett and Edwards

Type 3 branch pulmonary arteries originate independently from

the common trunk

Collett and Edwards

Type 4 Pseudo-truncus; TOF with pulm. Atresia with MAPCA

Van Praaghs

Type A1 Identical to the type I of Collett and Edwards

Van Praaghs

Type A2Collett and Edwards type II and most cases of type III

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

Van Praaghs

Type A4coexistence of an interrupted aortic archnot by the pattern of origin of branch pulmonary arteries

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

As the PBF increases PAH

Increased pulmonary venous return

Clinical Features

History• Cyanosis at birth• Early CCF• Failure to thrive, • Respiratory tract infections

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

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

Workup

Prenatal and early postnatal diagnosis common Proper evaluation of cyanotic infants clinches diagnosis ABG Pulse oximetry ECG CXR TTE, TOE

Workup

ECG• Normal QRS axis• Bi-ventricular hypertrophy – 70%

Workup

CXR• Cardiomegaly• Pulmonary plethora• Right aortic arch – 30%

Workup

Echo – single truncal valve; no pulmonary valve

CARDIAC CATHETERISATION• Delineation of anatomy in complex forms.• Assess PVR in late presentations.

Treatment

MedicalSurgical• Palliative• Defintive

Medical

Treat CCF – diuretics, digoxin Ensure oxygen delivery – intubation and mechanical

ventilation Hypocalcemia correction in syndromic Prostaglandin I.V.in Van Praagh Type A4

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

Surgical outcome

Significant late deaths due to re-operations truncal valve replacement conduit replacement (now treated more with PCI)

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.

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.

BARBERO MARCIAL MODIFICATION

REPAIR OF TRUNCUS ARTERIOSUS WITH INTERRUPTED AORTIC ARCH

TRADITIONAL APPROACH

Thank you

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