Evidence map›Paper›PMID 30813450›Full record

ReviewJournal of cardiovascular development and disease2019

Hypoplastic Left Heart Syndrome: A New Paradigm for an Old Disease?

Paul Grossfeld, Shuyi Nie, Lizhu Lin, Lu Wang, Robert H Anderson

Open access · goldAbstract readReview
In one paragraph

Review in Journal of cardiovascular development and disease, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.

0numbers the graph read from it
0cells of the map it votes in
40citing papers in PubMed
5.9field-weighted citation impact, top 3% of its field
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

40 citing papers in PubMed, 68 citations in OpenAlex.

  1. Article
  2. Article
  3. Is There a Unified Etiology of Hypoplastic Left Heart Syndrome? Evaluating Genetic, Structural, and Hemodynamic Models of Disease Initiation.Pathophysiology : the official journal of the International Society for Pathophysiology · 2026
    Review
  4. Current practice and challenges in fetal cardiac intervention.Frontiers in cardiovascular medicine · 2026
    Review
  5. Article
  6. Review
  7. Article
  8. Article
  9. Article
  10. Article
  11. Article
  12. Rare variants inHGG advances · 2023
    Article
  13. Article
  14. Article
  15. Article
  16. Hypoplastic Left Heart Syndrome: About a Postnatal Death.Diagnostics (Basel, Switzerland) · 2023
    Article
  17. Use of Frogs as a Model to Study the Etiology of HLHS.Journal of cardiovascular development and disease · 2023
    Article
  18. Article
  19. Article
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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

5 authors at 3 institutions in 2 countries.

Paul GrossfeldDivision of Cardiology, Department of Pediatrics, UCSD School of Medicine, La Jolla, CA 92093, USA. pgrossfeld@ucsd.edu.
Shuyi NieSchool of Biology, Georgia Institute of Technology, Atlanta, GA 30332, USA. shuyi.nie@biology.gatech.edu.
Lizhu LinDivision of Cardiology, Department of Pediatrics, UCSD School of Medicine, La Jolla, CA 92093, USA. lilin@ucsd.edu.
Lu WangDivision of Cardiology, Department of Pediatrics, UCSD School of Medicine, La Jolla, CA 92093, USA. luw059@ucsd.edu.
Robert H AndersonCardiovascular Research Centre, Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne NE1 7RU, UK. sejjran@ucl.ac.uk.ORCID 0000-0001-5163-9021
University of California San Diego · USGeorgia Institute of Technology · USNewcastle University · GB

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Hypoplastic left heart syndrome occurs in up to 3% of all infants born with congenital heart disease and is a leading cause of death in this population. Although there is strong evidence for a genetic component, a specific genetic cause is only known in a small subset of patients, consistent with a multifactorial etiology for the syndrome. There is controversy surrounding the mechanisms underlying the syndrome, which is likely due, in part, to the phenotypic variability of the disease. The most commonly held view is that the "decreased" growth of the left ventricle is due to a decreased flow during a critical period of ventricular development. Research has also been hindered by what has been, up until now, a lack of genetically engineered animal models that faithfully reproduce the human disease. There is a growing body of evidence, nonetheless, indicating that the hypoplasia of the left ventricle is due to a primary defect in ventricular development. In this review, we discuss the evidence demonstrating that, at least for a subset of cases, the chamber hypoplasia is the consequence of hyperplasia of the contained cardiomyocytes. In this regard, hypoplastic left heart syndrome could be viewed as a neonatal form of cardiomyopathy. We also discuss the role of the endocardium in the development of the ventricular hypoplasia, which may provide a mechanistic basis for how impaired flow to the developing ventricle leads to the anatomical changes seen in the syndrome.

Indexed as

cardiac myocyteendocardiumhyperplasiahypoplastic left heart syndromeJacobsen syndromeneural crest cell

Identifiers

PMID30813450
PMCPMC6462956
OpenAlexW2918095385

What OpenQuestion holds

Textmetadata
LicenceCC BY
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 OpenQuestion graph.