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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

Truncus Arteriosus

Seoul National University Hospital

Department of Thoracic & Cardiovascular Surgery

truncus arteriosus2
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
truncus arteriosus3
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.
truncus arteriosus4
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.
morphology of truncus arteriosus
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
truncus arteriosus10
Truncus Arteriosus

Truncal valve

VSD

clinical features diagnosis
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
truncus arteriosus12
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
operative techniques
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
operative indications
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).
truncal valve remodeling technique
Truncal Valve Remodeling Technique
  • Diagrams shows truncal valve repair by leaflet excision
  • and annular remodeling, usually there is one leaflet
  • that is grossly prolapsed
surgical results of truncus arteriosus
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