Evidence mapPaperPMID 42540458Full record

ArticlePulmonary circulation2026

Survival Into Adulthood With FOXF1-Associated Pulmonary Vascular Disease After Neonatal Onset.

Khalifah A Aldawsari, Steven H Abman, David Badesch, David Dunbar Ivy

Abstract read
In one paragraph

Article in Pulmonary circulation, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

4 authors.

Khalifah A AldawsariDepartment of Pediatrics, Section of Cardiology University of Colorado Anschutz Medical School and Children's Hospital Colorado Aurora Colorado USA.ORCID https://orcid.org/0000-0003-0163-8658
Steven H AbmanDepartment of Pediatrics and Pediatric Heart Lung Center University of Colorado Anschutz Medical School and Children's Hospital Colorado Aurora Colorado USA.ORCID https://orcid.org/0000-0002-7292-2085
David BadeschDivision of Pulmonary Sciences and Critical Care Medicine University of Colorado Anschutz Medical School Aurora Colorado USA.ORCID https://orcid.org/0009-0001-6311-4828
David Dunbar IvyDepartment of Pediatrics, Section of Cardiology University of Colorado Anschutz Medical School and Children's Hospital Colorado Aurora Colorado USA.ORCID https://orcid.org/0000-0003-2541-3402

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Neonates and infants with FOXF1 mutation develop alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV), which is typically considered a uniformly fatal neonatal lung disorder due to marked hypoxemic respiratory failure with severe pulmonary hypertension (PH). Survival beyond infancy is exceedingly rare, with limited reports of FOXF1 genetic abnormalities in older children or adult cases of severe PH. Here, we report the case of a male patient with a pathogenic FOXF1 variant who presented with severe PH in early infancy whose disease stabilized and improved with early and aggressive PH therapy. The patient subsequently died at the age of 30 years, however, due to severe right ventricular failure due to the acute onset of pneumonia. This case highlights that survival into adulthood is feasible in patients with FOXF1-related pulmonary vascular disease and supports the inclusion of FOXF1 in pulmonary hypertension genetic panels for subjects who develop PH across the lifespan.

Indexed as

alveolar capillary dysplasiamisalignment of pulmonary veinspulmonary arterial hypertensionpulmonary vascular disease

Identifiers

PMID42540458
PMCPMC13425634

What Socratic holds

Textmetadata
LicenceCC BY-NC
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.