A rare conotruncal anomaly: absent pulmonary valve syndrome, interrupted aortic arch, and severe aortic stenosis

The coexistence of absent pulmonary valve syndrome, Type B interrupted aortic arch, and severe aortic stenosis represents an exceptionally rare and complex congenital cardiac phenotype. The diagnosis was established in a newborn using multimodality imaging. Given the profound airway compression secondary to the massive pulmonary artery dilatation and the associated poor prognosis, palliative care was chosen.

Authors

Publication Details

Journal
Cardiology in the Young
Published
2026-09-30
DOI
https://doi.org/10.1017/s1047951126124172
Primary Topic
Congenital Heart Disease Studies
Type
article
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article

A rare conotruncal anomaly: absent pulmonary valve syndrome, interrupted aortic arch, and severe aortic stenosis

Martin Ngie Liong Wong, Ghoon Hoong Cheh
Cardiology in the Young
Congenital Heart Disease Studies
article

A rare conotruncal anomaly: absent pulmonary valve syndrome, interrupted aortic arch, and severe aortic stenosis

Martin Ngie Liong Wong, Ghoon Hoong Cheh
article en

Abstract

The coexistence of absent pulmonary valve syndrome, Type B interrupted aortic arch, and severe aortic stenosis represents an exceptionally rare and complex congenital cardiac phenotype. The diagnosis was established in a newborn using multimodality imaging. Given the profound airway compression secondary to the massive pulmonary artery dilatation and the associated poor prognosis, palliative care was chosen.

Cardiology in the Young
No poverty
Openalex Percentile: Top 11%
Congenital Heart Disease Studies
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A rare conotruncal anomaly: absent pulmonary valve syndrome, interrupted aortic arch, and severe aortic stenosis — Martin Ngie Liong Wong, Ghoon Hoong Cheh · Cardiology in the Young (2026) | TGRS Research Map | TGRS