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  • • A congenital heart lesion that increases pulmonary arterial blood flow

    • Results in left-to-right shunt, results in lung infection, pulmonary vascular congestion, pulmonary artery (PA) hypertension, right heart failure, pulmonary vasoconstriction, pulmonary vascular obstructive disease

    Eisenmenger syndrome: Increased pulmonary hypertension such that left-to-right shunt ceases and shunt becomes right-to-left, requiring heart-lung transplant

    • Inhaled nitric oxide, oxygen, or IV tolazoline reverses PA vasoconstriction

    • PA band is palliative and can reduce PA flow to alleviate RV failure and progression of pulmonary hypertension

    • Single large truncal vessel overrides ventricular septum and distributes all blood ejected from heart

    • Truncal root bifurcates into pulmonary trunk and aorta

    • Ventricular septal defect (VSD) present usually direct beneath truncal valve

    • Atrial septal defect (ASD) (> 40%), interrupted aortic arch (10%), abnormal origins of coronary artery

    • In most cases, pulmonary flow is increased

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Symptoms and Signs

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  • • Heart failure in most

    • Pulmonary vascular disease early

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  • • Evaluate for associated cardiac and extracardiac anomalies

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  • • Echocardiography: Diagnostic

    • Catheterization: Assess coronary arteries or truncal valve, or if age > 3 mos to assess for pulmonary hypertension

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  • • Surgical repair: Closure of VSD, separation pulmonary arteries from truncal root, placing pulmonary allograft valve from RV, correct truncal insufficiency

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Surgery

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Indications

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  • • Warranted once diagnosis made

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Contraindications

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  • • Eisenmenger physiology (irreversible)

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Prognosis

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  • • Operative mortality 5-30% (related to truncal valve insufficiency, interrupted arch, coronary anomalies, and pulmonary hypertension)

    • Survivors often need future repair or replacement of truncal or pulmonary valve

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References

Brown JW et al: Truncus arteriosus repair: outcomes, risk factors, reoperation and management. Eur J Cardiothorac Surg 2001;20:221.  [PubMed: 11463535]
Henaine R et al: Fate of the truncal valve in truncus arteriosus. Ann Thorac Surg 2008;85:172.  [PubMed: 18154803]
Konstantinov IE et al: Truncus arteriosus associated with interrupted aortic arch in 50 neonates: a Congenital Heart Surgeons Society study. Ann Thorac Surg 2006;81:214.  [PubMed: 16368368]
Rodefeld MD, Hanley FL. Neonatal truncus arteriosus repair: surgical techniques and clinical management. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002;5:212.  [PubMed: 11994881]
Thompson LD et al: Neonatal repair of truncus arteriosus: continuing improvement in outcomes. Ann Thorac Surg 2001;72:391.  [PubMed: 11515872]

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