{"doi":"10.1093/ehjqcco/qcaa090","title":"Tuning and external validation of an adult congenital heart disease risk prediction model","abstract":"AIMS: Adequate risk prediction can optimize the clinical management in adult congenital heart disease (ACHD). We aimed to update and subsequently validate a previously developed ACHD risk prediction model. METHODS AND RESULTS: A prediction model was developed in a prospective cohort study including 602 moderately or severely complex ACHD patients, enrolled as outpatients at a tertiary centre in the Netherlands (2011-2013). Multivariable Cox regression was used to develop a model for predicting the 1-year risks of death, heart failure (HF), or arrhythmia (primary endpoint). The Boston ACHD Biobank study, a prospectively enrolled cohort (n = 749) of outpatients who visited a referral centre in Boston (2012-2017), was used for external validation. The primary endpoint occurred in 153 (26%) and 191 (28%) patients in the derivation and validation cohorts over median follow-up of 5.6 and 2.3 years, respectively. The final model included 5 out of 14 pre-specified predictors with the following hazard ratios; New York Heart Association class ≥II: 1.92 [95% confidence interval (CI) 1.28-2.90], cardiac medication 2.52 (95% CI 1.72-3.69), ≥1 reintervention after initial repair: 1.56 (95% CI 1.09-2.22), body mass index: 1.04 (95% CI 1.01-1.07), log2 N-terminal pro B-type natriuretic peptide (pmol/L): 1.48 (95% CI 1.32-1.65). At external validation, the model showed good discrimination (C-statistic 0.79, 95% CI 0.74-0.83) and excellent calibration (calibration-in-the-large = -0.002; calibration slope = 0.99). CONCLUSION: These data support the validity and applicability of a parsimonious ACHD risk model based on five readily available clinical variables to accurately predict the 1-year risk of death, HF, or arrhythmia. This risk tool may help guide appropriate care for moderately or severely complex ACHD.","journal":"European Heart Journal - Quality of Care and Clinical Outcomes","year":2020,"id":72761,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":15,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9229,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2020-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":361914,"name":"Alexander R. Opotowsky","orcid":"0000-0002-6107-2281","position":1,"is_corresponding":false},{"id":383378,"name":"Cara L. Lachtrupp","orcid":"0000-0002-7047-8398","position":2,"is_corresponding":false},{"id":383379,"name":"Vivan J.M. Baggen","orcid":"0000-0003-0692-9147","position":3,"is_corresponding":false},{"id":383380,"name":"Sarah Brainard","orcid":"0000-0001-6228-8095","position":4,"is_corresponding":false},{"id":384362,"name":"Michael J. Landzberg","orcid":null,"position":5,"is_corresponding":false},{"id":89354,"name":"David van Klaveren","orcid":"0000-0002-2096-606X","position":6,"is_corresponding":false},{"id":85616,"name":"Hester F. Lingsma","orcid":"0000-0003-2063-9533","position":7,"is_corresponding":false},{"id":4886,"name":"Eric Boersma","orcid":"0000-0002-2559-7128","position":8,"is_corresponding":false},{"id":383381,"name":"Jolien W. Roos‐Hesselink","orcid":"0000-0002-6770-3830","position":9,"is_corresponding":false},{"id":383377,"name":"Laurie W. Geenen","orcid":"0000-0002-0113-2801","position":0,"is_corresponding":true}],"reference_count":21,"raw_metadata":null,"created_at":"2026-07-18T21:45:09.279825Z","pmid":"33313813","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}