Pulmonary hypertension (PH) is associated with impaired quality of life, worsened functional status and increased mortality in idiopathic pulmonary fibrosis (IPF) [1]. The prevalence and clinical impact in other idiopathic interstitial pneumonias (IIPs) has not previously been reported. Given the prognostic significance of PH in IPF, we sought to determine the prevalence and severity of World Health Organization (WHO) group 3 PH in a cohort of patients with biopsy-proven idiopathic nonspecific interstitial pneumonia (NSIP). We recognised that the prevalence of PH in this population of patients with advanced NSIP would be an overestimation of the NSIP population at large, but felt that mandating surgical lung biopsy and right heart catheterisation (RHC) were the only way to ensure accurate diagnosis of NSIP and PH, respectively. A retrospective review of patients with idiopathic NSIP diagnosed between 2002 and 2016 at our centre was performed. All cases were reviewed at our multidisciplinary pulmonary pathology conference. Connective tissue disease (CTD) was rigorously excluded in all patients and RHC was required for confirmation of PH. RHCs are not performed standardly on all patients in our clinic, and would have been performed as part of lung transplant evaluation or to confirm PH suggested by clinical parameters or echocardiographic data. PH was defined as a resting mean pulmonary artery pressure (mPAP) ⩾25 mmHg and severe PH as mPAP ⩾35 mmHg. Patients with a pulmonary capillary wedge pressure >15 mmHg were excluded from the cohort as they were suspected to have left-heart disease associated (WHO group 2) PH. Chronic thromboembolic disease was excluded in all patients with ventilation/perfusion scanning. The primary endpoints of this study were the prevalence and impact of WHO Group 3 PH on patient outcomes in idiopathic NSIP. Secondary outcomes included the correlation of forced vital capacity (FVC) and mPAP in idiopathic NSIP patients, as well as the association between other demographics and PH in this population. Review of records from our multidisciplinary pulmonary pathology meeting identified 95 potential patients with biopsy proven NSIP. Of these patients, 35 met criteria for inclusion in the study. 11 (31.4%) out of 35 patients had WHO group 3 PH. The mean±SD mPAP for those with and without WHO group 3 PH were 32.0±10.3 mmHg and 18.1±3.1 mmHg, respectively. Of the 35 patients in the study, seven (20%) patients had a mPAP of 25-30 mmHg, two (5.7%) had a mPAP of 31-34 mmHg and two (5.7%) had a mPAP ⩾35 mmHg. Table 1 details the characteristics of the patients with PH versus those without. Per cent predicted FVC was similar between the WHO group 3 PH and no PH cohorts, with r= −0.12 suggesting poor correlation between FVC per cent predicted and mPAP (p=0.48). Median transplant-free survival was significantly lower in patients with WHO group 3 PH at 17.6 months as compared to 47.9 months in the cohort without PH (p=0.05). Severe PH (mPAP ⩾35 mmHg or mPAP ⩾25 mmHg with a low cardiac index) was only seen i...
Practice patterns in the early postoperative care of lung transplant recipients differ considerably among centers. Many of the reported practices do not conform to consensus guidelines on management of critically ill patients.
COVID-19 can cause irreversible lung damage from acute respiratory distress syndrome (ARDS), chronic respiratory failure associated with post COVID-19 de novo fibrosis or worsening of an underlying fibrotic lung disease. Pregnant women are at increased risk for invasive mechanical ventilation, extracorporeal membrane oxygenation, and death. The Centers for Disease Control and Prevention reported more than 22,000 hospitalizations and 161 deaths for COVID-19 in pregnant women. Between August 2020 and September 2021, five patients underwent bilateral lung transplant (LT) for COVID-19 ARDS at the Henry Ford Hospital in Detroit, Michigan. De-identified demographics data, clinical characteristics, perioperative challenges, explanted lung pathology, and post-transplant outcomes are described. In post-hospitalization follow-up (median survival 273 days), we see improving endurance and excellent lung function. One patient did not survive to hospital discharge and succumbed to complications 5 months after LT. We report the first cases of bilateral LT in two postpartum women.
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