Evolution of pulmonary hypertension in interstitial lung disease: a journey through past, present, and future.

Interstitial lung diseases (ILD) are a spectrum of disorders often complicated by pulmonary hypertension (PH) in its course. The pathophysiologic mechanism of WHO group 3 PH is different to other forms of PH. The advent of PH is a harbinger for adverse events like mortality and morbidity, implying that the PH component of disease expedites deteriorated clinical outcomes. In fact, WHO group 3 PH due to ILD has the worse prognosis among all groups of PH. Hence, early detection of PH by a comprehensive screening method is paramount. Given considerable overlap in clinical manifestations between ILD and PH, early detection of PH is often elusive. Despite, the treatment of PH due to ILD has been frustrating until recently. Clinical trials utilizing PAH-specific pulmonary vasodilators have been ongoing for years without desired results. Eventually, the INCREASE study (2018) demonstrated beneficial effect of inhaled Treprostinil to treat PH in ILD. In view of this pioneering development, a paradigm shift in clinical approach to this disease phenotype is happening. There is a renewed vigor to develop a well validated screening tool for early detection and management. Currently inhaled Treprostinil is the only FDA approved therapy to treat this phenotype, but emergence of a therapy has opened a plethora of research toward new drug developments. Regardless of all these recent developments, the overall outlook still remains grim in this condition. This review article dwells on the current state of knowledge of pre-capillary PH due to ILD, especially its diagnosis and management, the recent progresses, and future evolutions in this field.

Recipient Age Impacts Long-Term Survival in Adult Subjects with Cystic Fibrosis after Lung Transplantation.

Rationale: Lung transplant is an effective treatment option providing survival benefit in patients with cystic fibrosis (CF). Several studies have suggested survival benefit in adults compared with pediatric patients with CF undergoing lung transplant. However, it remains unclear whether this age-related disparity persists in adult subjects with CF.Objectives: We investigated the impact of age at transplant on post-transplant outcomes in adult patients with CF.Methods: The United Network of Organ Sharing Registry was queried for all adult patients with CF who underwent lung transplantation between 1992 and 2016. Pertinent baseline characteristics, demographics, clinical parameters, and outcomes were recorded. The patients were divided into two groups based on age at transplant (18-29 yr old and 30 yr or older). The primary endpoint was survival time. Assessment of post-transplant survival was performed using Kaplan-Meier tests and log-rank tests with multivariable Cox proportional hazards analysis to adjust for confounding variables.Results: A total of 3,881 patients with CF underwent lung transplantation between 1992 and 2016; mean age was 31.0 (± 9.3) years. The 18-29-year-old at transplant cohort consisted of 2,002 subjects and the 30 years or older cohort had 1,879 subjects. Survival analysis demonstrated significantly higher survival in subjects in the 30 years or older cohort (9.47 yr; 95% confidence interval [CI], 8.7-10.2) compared with the 18-29-year-old cohort (5.21 yr; 95% CI, 4.6-5.8). After adjusting for confounders, survival remained higher in recipients aged 30 years or older (hazard ratio, 0.44; 95% CI, 0.2-0.9). Mortality due to allograft failure was significantly lower in patients with CF aged 30 years or older (28% vs. 36.5%; odds ratio [OR], 0.7; 95% CI, 0.6-0.8), whereas the incidence of malignancy was higher in the 30 years or older cohort (8% vs. 2.9%; OR, 3.0; 95% CI, 1.9-4.6).Conclusions: Age at transplant influences lung transplant outcomes in recipients with CF. Subjects with CF aged 30 years or older at transplant have superior survival compared with adult subjects with CF transplanted between the ages 18 and 29 years.

Short-term outcomes of pulmonary embolism: A National Perspective.

BACKGROUND: Pulmonary embolism (PE) is associated with significant morbidity and mortality in hospitalized patients. Real time data on 90-day mortality, bleeding, and readmission is sparse. METHODS: The study cohort was derived from the National Readmission Data (NRD) 2013 to 2014. PE was identified using International Classification of Diseases, ninth Revision (ICD-9-CM) code 415.11/3/9 in the primary diagnosis field. Any admission within 90 days of primary admission was considered a 90-day readmission. Readmission etiologies were identified by ICD-9 code in the primary diagnosis field. Co-primary outcomes were 90-day readmission and 90-day mortality. RESULTS: We identified 260 614 patients with primary admission PE, 55 659 (21.36%) patients were readmitted within 90 days. Most of them were of old age (age ≥ 65 years: 49.04%) and females (52.78%). Among the etiologies of readmission pulmonary disorders (22.94%) (Including recurrent PE 7.33%), malignancies (8.31%), and bleeding disorders (6.75%) were the most important causes of 90-day readmissions. On multivariate analysis, higher readmission rates and 90 days mortality were seen in patients with heart failure, chronic pulmonary disease, Anemia, malignancy, and with higher Charlson score. Patients with longer length of stay during primary admission and who discharged to short/long-term facility were more likely get readmitted and die in 90 days. Paradoxically, obese patients showed an inverse relationship with co-primary outcomes. CONCLUSIONS: Older female patients were more likely to have a pulmonary embolism. High-risk groups such as heart failure, chronic pulmonary disease, anemia, and malignancy need to be given extra attention to prevent worse outcomes.