{
  "abstract": "Objective\n\nMarfan syndrome (MFS) and Loeys-Dietz syndrome (LDS) are hereditary connective tissue diseases, both characterized by aortic root and vessel involvement with an increased risk of aneurysms and dissections. Despite advances in surgical techniques and improved postoperative follow-up, the impact of surgery on long-term outcomes in these patient groups is still unknown.Methods\n\nWe performed a systematic review and a meta-analysis to compare the postoperative long-term events. The main outcome was long-term survival. Additional outcomes were freedom from dissection and reoperation. After reconstruction of time-to-event data for the individual survival analysis, pooled Kaplan-Meier curves for the endpoints were generated.Results\n\nA total of 1,794 studies were identified, of which 5 were included in the analysis, encompassing 1,005 patients (MFS: 778; LDS: 227). With MFS as the reference group, there was no significant difference in long-term survival between both groups, based on age (hazard ratio [HR]: 0.919; 95% confidence interval [CI]: 0.58-1.45; p=0.717) or postoperative follow-up (HR: 0.997; 95% CI: 0.45-2.19; p=0.995). However, LDS was associated with higher risk of dissection (HR: 0.331; 95% CI: 0.21-0.53; p<0.005) and reoperation (HR: 0.603; 95% CI: 0.40-0.90; p=0.014), reflecting unadjusted comparisons reconstructed from Kaplan-Meier curves.Conclusion\n\nAlthough long-term survival is similar between MFS and LDS patients, those with LDS face a significantly higher risk of aortic dissection and reoperation. This elevated risk may be partly explained by the intrinsic genotypic heterogeneity of the LDS population. However, our pooled analysis could not fully adjust for this heterogeneity, underscoring the need for vigilant lifelong imaging surveillance and individualized surgical thresholds in LDS.",
  "abstract_shown": true,
  "affiliations": [
    "Friedrich-Schiller-University Jena, Department of Cardiothoracic Surgery, Germany, Jena.",
    "College of Health Sciences, Obafemi Awolowo University, Medical School, Nigeria, Ile-ife.",
    "University College London, Department of Cardiovascular Science, United Kingdom, London.",
    "MaterDei Hospital, Department of Thoracic Surgery, Bahia, Brazil, Salvador.",
    "West German Heart and Vascular Center, University Hospital Essen, Department of Cardiology and Vascular Medicine, Germany, Essen.",
    "UPMC Heart and Vascular Institute, University of Pittsburgh Medical Center, Department of Cardiothoracic Surgery, Pennsylvania, United States, Pittsburgh.",
    "Jena University Hospital, Universitatsklinikum Jena, Department of Cardiothoracic Surgery, Germany, Jena."
  ],
  "article_type": "research article",
  "arxiv": null,
  "author_total": 11,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Johannes Fischer"
    },
    {
      "affs": [
        0
      ],
      "name": "Steve Wehner"
    },
    {
      "affs": [
        1
      ],
      "name": "Ayodeji Arobo"
    },
    {
      "affs": [
        2
      ],
      "name": "Abdullah Almehandi"
    },
    {
      "affs": [
        3
      ],
      "name": "Felipe S Passos",
      "orcid": "0009-0001-7381-2806"
    },
    {
      "affs": [
        4
      ],
      "name": "Christian Eitel"
    },
    {
      "affs": [
        0
      ],
      "name": "Hristo Kirov"
    },
    {
      "affs": [
        5
      ],
      "name": "Xander Jacquemyn"
    },
    {
      "affs": [
        5
      ],
      "name": "Ibrahim Sultan"
    },
    {
      "affs": [
        6
      ],
      "name": "Torsten Doenst"
    },
    {
      "affs": [
        0
      ],
      "name": "Tulio Caldonazo",
      "orcid": "0000-0001-5697-2659"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/7anwmm5qb76r9.bib",
  "cited_by": 0,
  "doi": "10.1055/a-2965-6400",
  "fulltext_url": "https://europepmc.org/articles/PMC13657142",
  "id": "7anwmm5qb76r9",
  "issns": [
    "2325-4637"
  ],
  "landing_url": "https://europepmc.org/article/MED/42849511",
  "language": "eng",
  "licence": "CC-BY",
  "licence_label": "CC BY",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "openalex",
      "1311|Connective tissue disorders research"
    ]
  ],
  "openalex": "W7220909371",
  "pdf_url": "https://europepmc.org/articles/PMC13657142?pdf=render",
  "pmcid": "PMC13657142",
  "pmid": "42849511",
  "published": "2026-10-08",
  "publisher": null,
  "ris": "https://research.draconitic.com/paper/7anwmm5qb76r9.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:18:02Z",
      "native_id": "MED:42849511",
      "source": "europepmc",
      "url": "https://europepmc.org/article/MED/42849511"
    }
  ],
  "subjects": [
    {
      "field": "biology",
      "origin": "openalex:1311|Connective tissue disorders research",
      "subfield": "genetics-genomics"
    }
  ],
  "title": "Marfan Syndrome versus Loeys-Dietz Syndrome after Aortic Surgery: A Systematic Review and Meta-analysis of Reconstructed Time-to-Event Data.",
  "title_text": "Marfan Syndrome versus Loeys-Dietz Syndrome after Aortic Surgery: A Systematic Review and Meta-analysis of Reconstructed Time-to-Event Data.",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T12:18:02Z",
  "url": "https://research.draconitic.com/paper/7anwmm5qb76r9",
  "venue": "Aorta (Stamford, Conn.)",
  "versions": []
}