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Pericardiectomy for Constrictive Pericarditis in a Young Patient With Erdheim-Chester Disease.

ABSTRACT: Erdheim-Chester Disease (ECD) is an extremely rare non-Langerhans histiocytosis that most often presents in the fifth to seventh decades of life. In this case report, we present a 34-year-old woman who underwent successful pericardiectomy for constrictive pericarditis secondary to ECD, which is the youngest reported patient with ECD to undergo pericardiectomy. (Level of Difficulty: Advanced.).

SUBMITTER: El-Andari R

PROVIDER: S-EPMC9334141 | biostudies-literature | 2022 Jul

REPOSITORIES: biostudies-literature

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Pericardiectomy for Constrictive Pericarditis in a Young Patient With Erdheim-Chester Disease.

El-Andari Ryaan R Baghaffar Abdullah A O'Connell Andrew A Kang Jimmy J H JJH Deschenes Jean J George Anthony A Moon Michael C MC

JACC. Case reports 20220720 14

Erdheim-Chester Disease (ECD) is an extremely rare non-Langerhans histiocytosis that most often presents in the fifth to seventh decades of life. In this case report, we present a 34-year-old woman who underwent successful pericardiectomy for constrictive pericarditis secondary to ECD, which is the youngest reported patient with ECD to undergo pericardiectomy. (<b>Level of Difficulty: Advanced.</b>). ...[more]

PMID: 35912320

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Project description:BackgroundThe extent of pericardiectomy is an important issue in constrictive pericarditis but its impact on long-term outcomes has been rarely reported. We compared long-term results of radical pericardiectomy with conventional phrenic to phrenic pericardiectomy.MethodsNinety patients who underwent pericardiectomies between February 1995 and April 2015 were reviewed retrospectively. They were classified into conventional (n = 37) and radical (n = 53) groups according to pericardiectomy being performed anterior or posterior to the phrenic nerves, respectively. The follow-up duration at outpatient clinic was 37.6 (11.7, 86.6) months and the survival data until 91.6 (54.5, 147.0) months were obtained. The last echocardiographies were done at 22.4 (4.35, 60.85) months.ResultsThe early mortality rate was 4.4% (4/90). They all belonged to the conventional group and died of low cardiac output syndrome. The survival rate was higher in the radical group (P = .032, 74.7 ± 9.2% versus 50.4 ± 11.9% in 20 years). NYHA class of both groups had recovered until the last follow-up but the radical group showed better recovery (P < .001). The conventional pericardiectomy (HR = 6.181; 95% CI (1.042, 36.656)), redosternotomy (HR = 6.441; 95% CI (1.224, 33.889) and preoperative grade of tricuspid regurgitation (HR = 15.003; 95% CI (1.099, 204.894) were associated with late mortality. Right ventricular systolic pressure decreased, and pericardial thickening resolved only in the radical group with significant intergroup differences as time went on. Tricuspid regurgitation worsened after the operation in both groups, but it deteriorated more in the conventional group. However, it improved over time in the radical group.ConclusionsRadical pericardiectomy led to greater improvement in right ventricular systolic pressure and lesser deterioration of tricuspid regurgitation with the passage of time than did the conventional procedure. Conventional pericardiectomy and preoperative higher grade tricuspid regurgitation were associated with long-term mortality.

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Pericardiectomy for Constrictive Pericarditis in a Young Patient With Erdheim-Chester Disease.

Publications

Pericardiectomy for Constrictive Pericarditis in a Young Patient With Erdheim-Chester Disease.

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