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Truncus Arteriosus. Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery. Truncus Arteriosus. 1. Definition Congenital cardiac malformation in which one great artery, arising from the base of heart by way of a single semilunar(truncal) valve,

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Truncus Arteriosus

Seoul National University Hospital

Department of Thoracic & Cardiovascular Surgery


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Truncus Arteriosus

  • 1. Definition

  • Congenital cardiac malformation in which one great artery, arising

  • from the base of heart by way of a single semilunar(truncal) valve,

  • gives originof coronary, systemic , and one or two pulmonary

  • arteries proximal to the origin of the brachiocephalic branches.

  • Beneath the truncal valve, there is a VSD.

  • 2. History

  • Wilson : 1st description in 1798

  • Buchanan : Clinical & autopsy report in 1864

  • Collett & Edwards : Classification in 1949

  • Van Praagh : Alternative classification in 1965

  • McGoon : 1st repair with homograft in 1967


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Truncus Arteriosus

  • Pathophysiology

  • A single common artery, or truncus , overlying the ventricular septum and a nonrestrictive VSD gives rise to the coronary arteries, pulmonary arteries, and ascending aorta.

  • Complete mixing of systemic and pulmonary venous return at the VSD and truncal valve level results in moderate cyanosis.

  • As the pulmonary vascular resistance decreases after birth, significant left-to-right shunting at the truncal valve level leads to excessive pulmonary blood flow, pulmonary hypertension, and congestive heart failure.


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Truncus Arteriosus

  • Morphogenesis

  • Truncus arteriosus is called also as persistent truncus arteriosus, truncus arteriosus communis, common aorticopulmonary trunk

  • Chromosomal 22q11 deletion is present in a substantial number of patients with conotruncal abnormalities ( about one third with truncus arteriosus)

  • Many of these have additional characteristic features of DiGeorge syndrome, velocardiofacial syndrome, or conotruncal face syndrome

  • As such, their natural history may be complicated by hypocalcemia, palatal abnormalities, learning disability, or other noncardiac problems.


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1. Truncal artery

2. Pulmonary arteries

Type I, II : 80~90%

III, IV : 5~10%

Hemitruncus : 2%

Stenosis of origin : 10%

3. Aorta & ductus arteriosus

Wide PDA : Arch hypoplasia,

IAA or CoA(10~15%)

No PDA : majority

4. Coronary arteries

5. Semilunar valve

Tricuspid (1/2~2/3), quadricuspid,

bicuspid (5%)

Myxomatous thickening (1/3)

Truncal stenosis (20%)

6. VSD (juxtatruncal)

7. RV (absent conal septum)

8. Left ventricle ; normal

9. Associated anomalies

IAA or CoA with PDA : 10~15%

RAA : 25~35%

Anomalous branch : 10%

LSVC : 10%

ASD : 10%

DiGeorge synd. AV discordance,

situs inversus, heterotaxia,

DILV, MS, AV-canal,

tricuspid stenosis

Morphology of Truncus Arteriosus






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Truncus Arteriosus

Truncal valve

VSD


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Clinical Features & Diagnosis

  • 1. Symptoms

  • tachypnea, tachycardia, irritability, mild cyanosis

  • 2. Physical examination

  • signs of CHF, overactive heart

  • truncal insufficiency (systolic & diastolic murmur)

  • stenosis of PA (continuous murmur)

  • 3. Chest radiography

  • marked cardiomegaly as well as plethora

  • 4. EKG

  • RAD, biventricular hypertrophy

  • 5. Echocardiography

  • 6. Cardiac catheterization & cineangiography


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Truncus Arteriosus

  • Natural History

  • 1. Incidence

  • rare, unfavorable natural history

  • 1.7% to 2.8% of CHD

  • 2. Survival

  • 50% survival in 1 month

  • 18% survival in 6 months

  • 12% survival in 1 year

  • Others : Eisenmenger syndrome (death in 3rd decade)

  • 3. Modes of death

  • . Congestive heart failure in early life

  • . SBE, cerebral abscess, pulmonary vascular disease

  • . Survival is favorably affected by PS


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Operative Techniques

  • 1. Repair with allograft valved conduit

  • 2. Repair truncus I,II with autologous tissue

  • Barbero-Marcial technique

  • 3. Repair of hemitruncus

  • Unifocalization of pulmonary artery

  • 4. Repair of truncus arteriosus with IAA


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Operative Indications

  • 1. Diagnosis of truncus is an indication for it’s repair;

  • because about 50% of surgically untreated patients

  • die in the 1st month of life.

  • 2. Repair should be recommended as early in life

  • as possible rather than deferring to some

  • predetermined age

  • 3. Importantly elevated PVR is a contraindication

  • in old infant (more than 6~12 months old).




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Truncal Valve Remodeling Technique

  • Diagrams shows truncal valve repair by leaflet excision

  • and annular remodeling, usually there is one leaflet

  • that is grossly prolapsed


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Surgical Results of Truncus Arteriosus

  • 1. Survival

  • Early death

  • Time-related survival

  • 2. Modes of death

  • 3. Incremental risk factors for premature death

  • 1) Age at repair 2) Functional class

  • 3) Type 4) Size of VSD

  • 5) Predominance of origin of truncal artery

  • 6) Small size of pulmonary arteries

  • 7) Truncal valve abnormalities

  • 8) Hemitruncus

  • 9) Major associated cardiac anomalies

  • 10) Pulmonary vascular disease

  • 4. Progressing truncal valve incompetence

  • 5. Conduit reoperation