Pulmonary Hypertension Associated With Trastuzumab-Emtansine: An Analysis of French PH Registry and WHO Pharmacovigilance Database.

Background: Trastuzumab emtansine has been recently suspected to be associated with the development of pulmonary arterial hypertension (PAH).

Research Question: Is there an association between trastuzumab, trastuzumab emtansine, or trastuzumab deruxtecan and the development of PAH?.

Study Design And Methods: Characteristics of incident PAH cases treated with trastuzumab, trastuzumab emtansine, or trastuzumab deruxtecan were analyzed from the French PH Registry, the VIGIAPATH program, concurrently with a pharmacovigilance disproportionality analysis using the World Health Organization pharmacovigilance database using a broad definition of pulmonary hypertension (PH) and a narrow definition of PAH.

Characteristics and outcomes of gemcitabine-associated pulmonary hypertension.

Background: Despite its known cardiac and lung toxicities, the chemotherapy drug gemcitabine has only rarely been associated with pulmonary hypertension (PH), and the underlying mechanism remains unclear. The objective of the present study was to assess the association between gemcitabine and PH.

Methods: We identified incident cases of precapillary PH confirmed by right heart catheterisation in patients treated with gemcitabine from the French PH Registry between January 2007 and December 2022.

Therapeutic bronchoscopy for malignant central airway obstruction: Introduction to the EpiGETIF registry.

Background And Objective: EpiGETIF is a web-based, multicentre clinical database created in 2019 aiming for prospective collection of data regarding therapeutic rigid bronchoscopy (TB) for malignant central airway obstruction (MCAO).

Methods: Patients were enrolled into the registry from January 2019 to November 2022. Data were prospectively entered through a web-interface, using standardized definitions for each item.

Endobronchial valves: 1st Multicenter retrospective study on the 2-step approach.

Background: Although the endobronchial valves (EBV) were successfully developed as treatment for severe emphysema, its main complication, pneumothorax, remained an important concern.

Objective: To assess whether the placement of Zephyr© endobronchial valves throughout 2 procedures instead of 1 minor the frequency of pneumothorax without lowering the benefits of such treatment.

Methods: This retrospective study was conducted in 15 pulmonology department in France.

Hemorrhage in a patient with Bronchial Dieulafoy's disease and associated pulmonary fibrosis: A case report.

Background: Bronchial Dieulafoy's disease (BDD) is a rare vascular anomaly that was first described in 1995. The main symptom is recurrent hemoptysis. It can be diagnosed through angiography, bronchoscopy, and sometimes histology and endobronchial ultrasound scan (EBUS).

Balloon pulmonary angioplasty versus riociguat for the treatment of inoperable chronic thromboembolic pulmonary hypertension (RACE): a multicentre, phase 3, open-label, randomised controlled trial and ancillary follow-up study.

Background: Riociguat and balloon pulmonary angioplasty (BPA) are treatment options for inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, randomised controlled trials comparing these treatments are lacking. We aimed to evaluate the efficacy and safety of BPA versus riociguat in patients with inoperable CTEPH.

COVID-19 in Patients with Pulmonary Hypertension: A National Prospective Cohort Study.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is associated with pulmonary endothelial dysfunction. There are limited data available on the outcomes of coronavirus disease (COVID-19) in patients with pulmonary hypertension (PH), a disease characterized by pulmonary endothelial dysfunction. To describe characteristics and outcomes of patients with precapillary PH and COVID-19.

Association between Initial Treatment Strategy and Long-Term Survival in Pulmonary Arterial Hypertension.

The relationship between the initial treatment strategy and survival in pulmonary arterial hypertension (PAH) remains uncertain. To evaluate the long-term survival of patients with PAH categorized according to the initial treatment strategy. A retrospective analysis of incident patients with idiopathic, heritable, or anorexigen-induced PAH enrolled in the French Pulmonary Hypertension Registry (January 2006 to December 2018) was conducted.

Association between Leflunomide and Pulmonary Hypertension.

Pulmonary hypertension (PH) has been described in patients treated with leflunomide. To assess the association between leflunomide and PH. We identified incident cases of PH in patients treated with leflunomide from the French PH Registry and through the pharmacoVIGIlAnce in Pulmonary ArTerial Hypertension (VIGIAPATH) program between September 1999 to December 2019.

Characteristics and Long-term Outcomes of Pulmonary Venoocclusive Disease Induced by Mitomycin C.

Background: Pulmonary venoocclusive disease (PVOD) is an uncommon form of pulmonary hypertension (PH) predominantly characterized by pulmonary vein and capillary involvement. An association between chemotherapy, in particular mitomycin C (MMC), and PVOD has been reported.

Research Question: What are the characteristics of MMC-induced PVOD, and what is the prognosis for patients with MMC-induced PVOD?

Study Design And Methods: We report the clinical, functional, radiologic, and hemodynamic characteristics at diagnosis and outcomes of patients with PVOD from the French PH Registry after exposure to MMC.

Phenotype and Outcomes of Pulmonary Hypertension Associated with Neurofibromatosis Type 1.

Pulmonary hypertension (PH) associated with neurofibromatosis type 1 (NF1) is a rare and largely unknown complication of NF1. To describe characteristics and outcomes of PH-NF1. We reported the clinical, functional, radiologic, histologic, and hemodynamic characteristics, response to pulmonary arterial hypertension (PAH)-approved drugs, and transplant-free survival of patients with PH-NF1 from the French PH registry.

Portopulmonary hypertension in the current era of pulmonary hypertension management.

Background & Aims: Long-term outcomes in portopulmonary hypertension (PoPH) are poorly studied in the current era of pulmonary hypertension management. We analysed the effect of pulmonary arterial hypertension (PAH)-targeted therapies, survival and predictors of death in a large contemporary cohort of patients with PoPH.

Methods: Data from patients with PoPH consecutively enrolled in the French Pulmonary Hypertension Registry between 2007 and 2017 were collected.

Familial pulmonary arterial hypertension by heterozygous loss of function.

Beyond the major gene , several new genes predisposing to PAH have been identified during the last decade. Recently, preliminary evidence of the involvement of the gene was found in a large genetic association study.We prospectively analysed the gene by targeted panel sequencing in a series of 311 PAH patients referred to a clinical molecular laboratory for genetic diagnosis of PAH.

Update on childhood and adult infectious tracheitis.

The trachea is a pivotal organ of the respiratory tract. Rather than a genuine anatomic border, it acts as a crossroad in all respiratory infectious processes. Even though not strictly limited to the trachea, infections such as laryngotracheitis and tracheobronchitis are frequently diagnosed in children, in particular during the winter season.

Endobronchial metastases from melanoma: a survival analysis.

Background: Metastatic spread to the tracheobronchial tree from other than bronchopulmonary tumors is a common clinical problem. However, malignant melanoma, a highly metastatic potential tumor, is rarely metastasing in the airways. Therefore little is known about survival of patients with endobronchial metastasis from melanoma.

[Drug-induced interstitial lung diseases].

Drug-induced infiltrative lung disease may manifest as variable clinical radiological patterns, including subacute or chronic interstitial pneumonia, pulmonary fibrosis, eosinophilic pneumonia, organising pneumonia, pulmonary edema, or sarcoidosis. A large amount of drugs have been incriminated, including those used in cardiovascular diseases (amiodarone, statins and angiotensin converting enzyme inhibitors), antibiotics (minocycline, nitrofurantoin), most of anticancer drugs (and especially chemotherapy and chest radiation), treatment of rheumatoid arthritis, as well as more recent drugs. A high index of suspicion is therefore required in any patient with infiltrative lung disease and the web-based tool www.

[A rare cause of pulmonary opacities: Lung localization of Waldenström's macroglobulinemia].

Introduction: Pulmonary localized forms of Waldenström's macroglobulinemia are rare.

Case Report: We report the observation of a 71-year-old woman with chronic cough and persisting alveolar opacities after several courses of antibiotics. Physical examination was unremarkable.

[Pulmonary embolism by cyanoacrylate following embolisation of an arteriovenous malformation].

Introduction: Symptomatic complications can occur after intravascular injection of cyanoacrylate glue. We report a case of pulmonary embolism following embolisation of an arteriovenous malformation (AVM).

Case Report: A 46-year-old woman was found to have an internal iliac AVM which was obliterated using N-butyl-2 cyanoacrylate (NBCA) mixed with lipiodol.

[Difficult weaning due to tracheal compression by an esophageal bezoar].

Introduction: Bezoars result from the aggregation of ingested materials (food, drugs, hair) that accumulate at a certain anatomic level in the gastrointestinal tract. It is a rare condition, which is favoured by a reduction in intestinal motility, or by a primary abnormality reducing the patency of gastrointestinal tract.

Case Report: We present a case when acute respiratory symptoms revealed an oesophageal bezoar.