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Long-Term Outcomes of Cardiovascular Operations in Children With Connective Tissue Disorders.

ABSTRACT: Connective tissue disorders can be associated with significant cardiovascular morbidity needing cardiac surgery during childhood. In this retrospective study, we used the Pediatric Cardiac Care Consortium, a multicenter United States-based registry of pediatric cardiac interventions, to describe the long-term outcomes of patients who underwent their first surgery for connective tissue-related cardiovascular conditions aged <21 years. Between 1982 and 2003, a total of 103 patients were enrolled who underwent cardiac surgery for a connective tissue-related cardiovascular disorder, including 3 severe infantile cases operated on within the first year of life. Most patients underwent aortic site surgery (n = 85) as a composite graft (n = 50), valve-sparing (n = 33), or other aortic surgery (n = 2). The remaining patients underwent atrioventricular valve surgery (mitral 17, tricuspid 1). Of the 99 patients surviving to discharge, 80 (including the 3 infantile) had adequate identifiers for tracking long-term outcomes through 2019 through linkage with the National Death Index and the Organ Procurement. Over a median period of 19.5 years (interquartile range 16.0 to 23.1), 29 deaths and 1 transplant occurred in the noninfantile group, whereas all 3 infantile patients died before the age of 4 years. The postdischarge survival for the noninfantile group was 92.2%, 68.2%, and 56.7% at 10, 20, and 25 years, respectively. Cardiovascular-related pathology contributed to all deaths in the infantile and 89% (n = 27) of deaths for the noninfantile cases after hospital discharge. The significant late attrition from cardiovascular causes emphasizes the need for close monitoring and ongoing management in this population.

SUBMITTER: Thomas AS

PROVIDER: S-EPMC9633117 | biostudies-literature | 2022 Nov

REPOSITORIES: biostudies-literature

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Long-Term Outcomes of Cardiovascular Operations in Children With Connective Tissue Disorders.

Thomas Amanda S AS Falk Erin E EE Mahoney Sarah S Knight Jessica H JH Kochilas Lazaros K LK

The American journal of cardiology 20220919

Connective tissue disorders can be associated with significant cardiovascular morbidity needing cardiac surgery during childhood. In this retrospective study, we used the Pediatric Cardiac Care Consortium, a multicenter United States-based registry of pediatric cardiac interventions, to describe the long-term outcomes of patients who underwent their first surgery for connective tissue-related cardiovascular conditions aged <21 years. Between 1982 and 2003, a total of 103 patients were enrolled w ...[more]

PMID: 36137823

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Project description:PurposeWhile children with hypermobility and/or ligamentous laxity due to coexisting connective tissue disorders might be expected to have worse outcomes after open reduction for hip dislocations, there is minimal prior research on this topic.MethodsAll open reduction surgeries for hip dislocations performed at a single urban, tertiary-care children's hospital from 2009 to 2023 were reviewed retrospectively. Those with connective tissue disorders secondary to a diagnosed syndrome or genetic disorder were included. Patients with <1 year of follow-up or hip instability in the setting of Trisomy 21 were excluded. Clinical and radiographic data was collected. Instances of re-dislocation, proximal femoral growth disturbance, residual acetabular dysplasia, and arthrofibrosis were recorded.ResultsTwenty-three hips (15 patients) were included. Mean age at the time of surgery was 19.6 months (Range: 8.2-36.0 months), and mean follow-up was 4.3 years. The most common connective tissue disorder condition included was Ehlers-Danlos syndrome (13%). A majority of open reductions were performed via an anterior approach (96%). Seven hips (30%) underwent a concomitant pelvic osteotomy without femoral osteotomy and seven hips (30%) underwent both pelvic and femoral osteotomies. Twenty-two hips (96%) were International Hip Dysplasia Institute grade 1 at the final follow-up. Re-dislocation occurred in four hips (17%); eight hips (35%) demonstrated residual acetabular dysplasia, five hips (22%) demonstrated proximal femoral growth disturbance, and nine hips (39%) developed stiffness postoperatively.ConclusionsPatients with connective tissue disorders and ligamentous laxity have comparable rates of residual acetabular dysplasia, proximal femoral growth disturbance, and (surprisingly) stiffness as typical developmental dysplasia of the hip following open hip reduction surgery. Although the re-dislocation rate in the connective tissue disorders group was approximately 2-3 times higher, the difference did not reach statistical significance. Given that the study was limited by a low sample size, however, it is possible that the findings of no difference in residual acetabular dysplasia and proximal femoral growth disturbance were potentially due to a lack of power.Level of evidenceIV.

Project description:ImportanceMultiple cases of autoimmune and autoinflammatory diseases after COVID-19 have been reported. However, their incidences and risks have rarely been quantified.ObjectiveTo investigate the incidences and risks of autoimmune and autoinflammatory connective tissue disorders after COVID-19.Design, setting, and participantsThis was a retrospective population-based study conducted between October 8, 2020, and December 31, 2021, that used nationwide data from the Korea Disease Control and Prevention Agency COVID-19 National Health Insurance Service cohort and included individuals who received a diagnosis of COVID-19 via polymerase chain reaction testing and a control group with no evidence of COVID-19 identified from National Health Insurance Service of Korea cohort. Data analysis was conducted from September 2022 to August 2023.ExposuresReceipt of diagnosis of COVID-19.Main outcomes and measuresThe primary outcomes were the incidence and risk of autoimmune and autoinflammatory connective tissue disorders following COVID-19. A total of 32 covariates, including demographics, socioeconomic statuses, lifestyle factors, and comorbidity profiles, were balanced through inverse probability weighting. The incidences and risks of autoimmune and autoinflammatory connective tissue disorders were compared between the groups using multivariable Cox proportional hazard analyses.ResultsA total of 354 527 individuals with COVID-19 (mean [SD] age, 52.24 [15.55] years; 179 041 women [50.50%]) and 6 134 940 controls (mean [SD] age, 52.05 [15.63] years; 3 074 573 women [50.12%]) were included. The risks of alopecia areata (adjusted hazard ratio [aHR], 1.12; 95% CI, 1.05-1.19), alopecia totalis (aHR, 1.74; 95% CI, 1.39-2.17), antineutrophil cytoplasmic antibody-associated vasculitis (aHR, 2.76; 95% CI, 1.64-4.65), Crohn disease (aHR, 1.68; 95% CI, 1.31-2.15), and sarcoidosis (aHR, 1.59; 95% CI, 1.00-2.52) were higher in the COVID-19 group. The risks of alopecia totalis, psoriasis, vitiligo, vasculitis, Crohn disease, ulcerative colitis, rheumatoid arthritis, adult-onset Still disease, Sjögren syndrome, ankylosing spondylitis, and sarcoidosis were associated with the severity of COVID-19.Conclusions and relevanceIn this retrospective cohort study, COVID-19 was associated with a substantial risk for autoimmune and autoinflammatory connective tissue disorders, indicating that long-term management of patients with COVID-19 should include evaluation for such disorders.

Dataset Information

Long-Term Outcomes of Cardiovascular Operations in Children With Connective Tissue Disorders.

Publications

Long-Term Outcomes of Cardiovascular Operations in Children With Connective Tissue Disorders.

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