Truncus Arteriosus Clinical Presentation

Updated: Dec 31, 2019
  • Author: Doff B McElhinney, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Presentation

History

Historical presentation of patients with truncus arteriosus (TA) who are not diagnosed before the onset of symptoms typically consists of the following:

  • Poor feeding/inadequate weight gain

  • Diaphoresis

  • Tachypnea

Symptoms vary and may be more or less pronounced, depending on specific anatomic features and age at presentation. For example, patients with significant truncal valve regurgitation tend to present earlier with more profound symptoms of congestive heart failure.

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Physical Examination

Patients with truncus arteriosus rarely present with cyanosis but it may occur in very young neonates in whom pulmonary vascular resistance remains elevated. Even in slightly older neonates and young infants, pulmonary overcirculation and streaming of left and right ventricular outflow into the aorta and pulmonary arteries, respectively, may occasionally result in systemic oxyhemoglobin saturation well above 90%.

Symptoms and signs of congestive heart failure are more common findings than cyanosis in patients presenting early in life. Symptoms of failure typically manifest as pulmonary vascular resistance falls and pulmonary overcirculation increases. With progressively increasing pulmonary blood flow and, consequently, myocardial work, the initial symptoms of congestive heart failure (eg, poor feeding, diaphoresis, mild lethargy) become more evident as failure to thrive ensues.

Patients occasionally present in extremis, with the usual high output failure exacerbated by significant regurgitation of the truncal valve. Patients with associated interruption of the aortic arch may exhibit a shocklike picture of cardiovascular collapse during ductal closure, although the arterial duct frequently remains patent in patients with truncus and interrupted arch, even without pharmacologic therapy.

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