%0 Journal Article
%T Treatment and outcomes of 844 cases of pneumothorax in heritable connective tissue disorders.
%A Kutsukake M
%A Konishi T
%A Aso S
%A Fujiogi M
%A Takamoto N
%A Morita K
%A Ohbe H
%A Matsui H
%A Fushimi K
%A Fujishiro J
%A Yasunaga H
%J Ann Thorac Surg
%V 0
%N 0
%D 2024 Jun 13
%M 38878950
%F 5.102
%R 10.1016/j.athoracsur.2024.05.037
%X <B>BACKGROUND: </B>Characteristics, practice patterns, and clinical outcomes of secondary spontaneous pneumothorax (SSP) with heritable connective tissue disorders (Marfan, Ehlers-Danlos, and Birt-Hogg-Dubé syndromes) are unclear.<BR><B>METHODS: </B>A nationwide Japanese inpatient database, which included data on 524 patients with SSP (884 hospitalizations) and 137821 with primary spontaneous pneumothorax (PSP) between July 2010 and March 2020, was used in this study. Hospitalization for SSP (n=884) was categorized into surgical (n=459) and non-surgical (n=425) groups, and the patient characteristics, treatment, and outcomes were compared between the groups. Multivariable analyses were performed to evaluate risk factors for pneumothorax recurrence. We also compared the patient characteristics among those with different underlying heritable connective tissue disorders.<BR><B>RESULTS: </B>Compared to the non-surgical group, the surgical group had less frequent readmission for pneumothorax (26% vs. 44%; hazard ratio, 0.47; 95% confidence interval, 0.38-0.58). Young patients (hazard ratio, 2.46; 95% confidence interval, 1.83-3.32) or those with Birt-Hogg-Dubé syndrome (2.53; 1.77-3.63) had a high risk of recurrence. Pneumothorax occurred frequently in teenagers with Marfan syndrome, in those aged 20-39 years with Ehlers-Danlos syndrome, and in those aged ≥40 years with Birt-Hogg-Dubé syndrome.<BR><B>CONCLUSIONS: </B>Detailed information on the characteristics and clinical course of SSP in heritable connective tissue disorders will aid the clinical decision-making process.