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August 23, 2026Circulation

The natural history of truncus arteriosus.

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Key result

Unoperated truncus arteriosus is linked to ~61% mortality, including universal infant death.

  • n=23

Why the study?

What is the natural history and prognosis of patients with unoperated truncus arteriosus?

Population

23 patients diagnosed with truncus arteriosus, type I or II, seen before corrective operation became feasible

Design

Retrospective case series

Authors

CMCarlo MarcellettiUniversity of PisaDMDwight C. McGoonTwitter (United States)DMD D MairQueen Mary University of London

Discussion

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Implication

Historical mortality data caution against delayed repair in truncus arteriosus; leaves open effects of current neonatal strategies on vascular outcomes.

Key Points

  • To evaluate the natural history, mortality rates, and clinical progression of untreated type I and II truncus arteriosus prior to the availability of corrective surgery.
  • Retrospective review of 23 patients with truncus arteriosus (type I or II) evaluated at the Mayo Clinic in the decade prior to 1967, before corrective surgical repair became feasible.
  • Overall mortality was 61% (14 of 23 died), with 100% mortality (10 of 10) observed among infants presenting at one year of age or younger.
  • Among patients older than seven years (n=5), all had severe pulmonary vascular obstructive disease, and three died.
  • Of the surviving patients across all age groups, all but one had persistent mild to moderate disability.

Study Design

Type

Observational (n=23)

Multicenter

No

Structured PICO

What is the natural history and prognosis of patients with unoperated truncus arteriosus?

P
Population
23 patients with truncus arteriosus type I or II evaluated before corrective surgery became feasible to assess natural history and mortality.
E
Exposure
Natural history (no corrective operation)
O
Outcome
Mortalityhard clinical

The grave prognosis of unoperated truncus arteriosus, particularly in infants, underscores the necessity of early corrective surgery before the development of severe pulmonary vascular obstructive disease.

Cite This Study

Marcelletti et al. (1976) conducted an observational in Truncus arteriosus (n=23). Unoperated truncus arteriosus (natural history) was evaluated on Mortality. The natural history of unoperated truncus arteriosus was associated with high mortality, with 14 of 23 patients dying, including all 10 infants.

synapsesocial.com/papers/6a8b63be4307c6fbfc8042e3https://doi.org/10.1161/01.cir.54.1.108
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Also Consider

Synapse has enriched 3 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Management of Truncal Valvular Regurgitation1974 · 29 citations
  2. 2Pediatric Cardiology, ed 3.1974 · 10 citations
  3. 3Persistent truncus arteriosus with survival to the age of 38 years.1966 · 33 citations