Expert management of congenital portosystemic shunts and their complications.

Congenital portosystemic shunts are often associated with systemic complications, the most challenging of which are liver nodules, pulmonary hypertension, endocrine abnormalities, and neurocognitive dysfunction. In the present paper, we offer expert clinical guidance on the management of liver nodules, pulmonary hypertension, and endocrine abnormalities, and we make recommendations regarding shunt closure and follow-up.

[COPD: Guidelines for primary care physicians]. / BPCO: recommandations pour les médecins de premiers recours.

Chronic obstructive pulmonary disease (COPD) is common and should be suspected in any patient with chronic dyspnea, cough, or sputum with a history of exposure to tobacco or harmful particles. Spirometry is used for diagnosis. Full evaluation includes the severity of obstruction and clinical data, following the Global Initiative for Chronic Obstructive Lung Disease guidelines. Although the only treatments that have an impact on mortality are tobacco cessation, pulmonary rehabilitation and, for advanced disease, oxygen therapy, new symptomatic treatment have recently been made available. The duration of antibiotic and corticosteroid treatment for exacerbations has been shortened. The new diagnostic and management recommendations are summarized in this article.

La bronchopneumopathie chronique obstructive (BPCO) est fréquente et doit être suspectée chez tout patient présentant une dyspnée, une toux ou des expectorations chroniques avec une anamnèse d'exposition au tabac ou à des particules nuisibles. La spirométrie permet son diagnostic. Son évaluation se base sur la sévérité de l'obstruction et la clinique et tient compte des recommandations du guide de la Global Initiative for Chronic Obstructive Lung Disease. Bien que les seuls traitements impactant la survie soient l'arrêt du tabac, la réhabilitation respiratoire et tardivement l'oxygénothérapie, des nouveautés sont apparues dans l'arsenal thérapeutique symptomatique. La durée du traitement antibiotique et corticostéroïde des exacerbations est raccourcie. Les nouvelles recommandations diagnostiques et de prise en charge sont résumées dans cet article.

[Pulmonology 2021: year in review]. / Pneumologie.

Among the new therapeutic developments in pulmonology during 2021, we have identified three topics of interest. A new biotherapy is now available for the management of severe uncontrolled non-Th2 asthma. In the field of pulmonary arterial hypertension, upfront triple therapy at the time of diagnosis is associated with a survival benefit in high-risk patients. Riociguat is a therapeutic option for patients that remain at intermediate risk despite treatment with iPDE5. Sotatercept, a promising new class of drug for treatment of group 1 PAH will soon be available. Finally, the use of transbronchial cryobiopsies as a valid alternative to surgical lung biopsy for the diagnosis of diffuse interstitial lung diseases will also be discussed in this review.

Parmi les nouveautés thérapeutiques en pneumologie au cours de l'année 2021, nous aborderons trois sujets. Une nouvelle biothérapie est désormais disponible pour la prise en charge de l'asthme sévère non contrôlé non T-Helper 2. Concernant l'hypertension artérielle pulmonaire (HTAP), une triple thérapie d'emblée au moment du diagnostic est associée à un bénéfice sur la survie chez les patients à haut risque. Le riociguat est une option thérapeutique lors d'HTAP restant à risque intermédiaire malgré un traitement par inhibiteur de la phosphodiestérase de type 5, et le sotatercept vise une nouvelle cible thérapeutique prometteuse pour l'HTAP du groupe 1. Enfin, la place des cryobiopsies transbronchiques comme alternative valable à la biopsie chirurgicale pour le diagnostic des pneumopathies interstitielles diffuses selon des recommandations récentes se précise.

Outcome of paediatric portopulmonary hypertension in the modern management era: A case report of 6 patients.

Portopulmonary hypertension is a rare but serious complication of portal hypertension or portosystemic shunting. Portopulmonary hypertension is an indication for liver transplantation or shunt closure. However, liver transplantation is contraindicated in patients with severe pulmonary arterial hypertension. Reported mortality rates are high in children with portopulmonary hypertension and there are scarce recommendations on its management. Our aim was to report on our real-world experience of managing portopulmonary hypertension in a specialised centre. We describe a series of 6 children with portopulmonary hypertension. Their median age at diagnosis was 13 years (range 10-15). The underlying liver conditions were cirrhosis of unknown origin (1), congenital portocaval shunts (3), biliary atresia (1), and portal vein cavernoma with surgical mesenterico-caval shunt (1). Median mean pulmonary arterial pressure was 47 mmHg (range 32-70), and median pulmonary vascular resistance was 6.6 Wood units (range 4.3-15.4). All patients except one were treated with a combination of pulmonary arterial hypertension-specific therapy (phosphodiesterase type 5 inhibitors and/or endothelin receptor antagonists and/or prostacyclin analogues). Three patients then benefited from shunt closure and the others underwent liver transplantation. Five patients showed improvement or stabilisation of pulmonary arterial hypertension with no deaths after a mean follow-up of 39 months. Based on our limited experience, early and aggressive treatment with a combination of pulmonary arterial hypertension-specific therapy significantly improves patients' haemodynamic profile and enables the performance of liver transplantation and shunt closure with satisfactory outcomes.

[Pulmonary rehabilitation in respiratory disorders other than COPD : an individualized approach]. / Réhabilitation pulmonaire lors de pathologies autres que la BPCO : un programme sur mesure.

Pulmonary rehabilitation is a multimodal intervention which includes an individualized physical training program, patient education, nutritional assessment and counseling, and recommendations for behavioral changes (smoking cessation, physical activity, nutrition...). This approach, validated and widely accepted by international expert societies in COPD, has been explored and evaluated in other chronic respiratory disorders. This overview analyses the impact of pulmonary rehabilitation in interstitial lung diseases, pulmonary hypertension, lung cancer (pre and post surgery), and morbid obesity. The recent medical literature encourages clinicians to consider the possibility of pulmonary rehabilitation in most chronic pulmonary disorders.

La réhabilitation respiratoire est une intervention multimodale comprenant un entraînement physique personnalisé, un enseignement thérapeutique, une prise en charge nutritionnelle, et un encouragement à des modifications comportementales. Cette approche, validée et recommandée par les sociétés savantes lors de BPCO, est de plus en plus étudiée dans d'autres pathologies pulmonaires chroniques. Ce travail analyse l'impact de la réhabilitation respiratoire lors de pneumopathies interstitielles, d'hypertension pulmonaire, d'obésité morbide et lors de cancer pulmonaire (en phase pré ou postopératoire). Les données les plus récentes encouragent les cliniciens à envisager systématiquement la possibilité d'une réhabilitation respiratoire lors de pathologies respiratoires chroniques.

Radiological findings of complications after lung transplantation.

Complications following lung transplantation may impede allograft function and threaten patient survival. The five main complications after lung transplantation are primary graft dysfunction, post-surgical complications, alloimmune responses, infections, and malignancy. Primary graft dysfunction, a transient ischemic/reperfusion injury, appears as a pulmonary edema in almost every patient during the first three days post-surgery. Post-surgical dysfunction could be depicted on computed tomography (CT), such as bronchial anastomosis dehiscence, bronchial stenosis and bronchomalacia, pulmonary artery stenosis, and size mismatch. Alloimmune responses represent acute rejection or chronic lung allograft dysfunction (CLAD). CLAD has three different forms (bronchiolitis obliterans syndrome, restrictive allograft syndrome, acute fibrinoid organizing pneumonia) that could be differentiated on CT. Infections are different depending on their time of occurrence. The first post-operative month is mostly associated with bacterial and fungal pathogens. From the second to sixth months, viral pneumonias and fungal and parasitic opportunistic infections are more frequent. Different patterns according to the type of infection exist on CT. Malignancy should be depicted and corresponded principally to post-transplantation lymphoproliferative disease (PTLD). In this review, we describe specific CT signs of these five main lung transplantation complications and their time of occurrence to improve diagnosis, follow-up, medical management, and to correlate these findings with pathology results. KEY POINTS: • The five main complications are primary graft dysfunction, surgical, alloimmune, infectious, and malignancy complications. • CT identifies anomalies in the setting of unspecific symptoms of lung transplantation complications. • Knowledge of the specific CT signs can allow a prompt diagnosis. • CT signs maximize the yield of bronchoscopy, transbronchial biopsy, and bronchoalveolar lavage. • Radiopathological correlation helps to understand CT signs after lung transplantation complications.

Pulmonary Perfusion Changes as Assessed by Contrast-Enhanced Dual-Energy Computed Tomography after Endoscopic Lung Volume Reduction by Coils.

BACKGROUND: Endoscopic lung volume reduction by coils (LVRC) is a recent treatment approach for severe emphysema. Furthermore, dual-energy computed tomography (DECT) now offers a combined assessment of lung morphology and pulmonary perfusion. OBJECTIVES: The aim of our study was to assess the impact of LVRC on pulmonary perfusion with DECT. METHODS: Seventeen patients (64.8 ± 6.7 years) underwent LVRC. DECT was performed prior to and after LVRC. For each patient, lung volumes and emphysema quantification were automatically calculated. Then, 6 regions of interest (ROIs) on the iodine perfusion map were drawn in the anterior, mid, and posterior right and left lungs at 4 defined levels. The ROI values were averaged to obtain lung perfusion as assessed by the lung's iodine concentration (CLung, µg·cm-3). The CLung values were normalized using the left atrial iodine concentration (CLA) to take into account differences between successive DECT scans. RESULTS: The 6-min walk distance (6MWD) improved significantly after the procedure (p = 0.0002). No lung volume changes were observed between successive DECT scans for any of the patients (p = 0.32), attesting the same suspended inspiration. After LVRC, the emphysema index was significantly reduced in the treated lung (p = 0.0014). Lung perfusion increased significantly adjacent to the treated areas (CLung/CLA from 3.4 ± 1.7 to 5.6 ± 2.2, p < 0.001) and in the ipsilateral untreated areas (from 4.1 ± 1.4 to 6.6 ± 1.7, p < 0.001), corresponding to a mean 65 and 61% increase in perfusion, respectively. No significant difference was observed in the contralateral upper and lower areas (from 4.4 ± 1.9 to 4.8 ± 2.1, p = 0.273, and from 4.9 ± 2.0 to 5.2 ± 1.7, p = 0.412, respectively). A significant correlation between increased 6MWD and increased perfusion was found (p = 0.0027, R2 = 0.3850). CONCLUSIONS: Quantitative analysis based on DECT acquisition revealed that LVRC results in a significant increase in perfusion in the coil-free areas adjacent to the treated ones, as well as in the ipsilateral untreated areas. This suggests a possible role for LVRC in the improvement of the ventilation/perfusion relationship.

Preoperative Peak Oxygen Uptake in Lung Cancer Subjects With Neoadjuvant Chemotherapy: A Cross-Sectional Study.

BACKGROUND: In non-small-cell lung cancer patients, high peak oxygen uptake (peak V̇O2 ) predicts lower rates of postoperative complications and better long-term survival. Neoadjuvant chemotherapy (NAC) may negatively impact peak V̇O2 . METHODS: Cardiopulmonary exercise testing (CPET) was performed in 34 consecutive stage IIIA/IIIB non-small-cell lung cancer subjects scheduled for elective lung surgery. Using multivariate linear regression adjusted for potential confounders, we compared CPET results in subjects receiving or not receiving NAC (NAC+, n = 19; NAC-, n = 15). RESULTS: Adjusted peak V̇O2 was lower in NAC + compared with NAC- subjects (-5.3 mL/min/kg [95% CI -8.3 to -2.2], P = .01). Likewise, oxygen pulse, maximal work load, and ventilatory threshold were also lower in NAC+ subjects, whereas peak heart rate and breathing reserve were similar. NAC+ subjects presented lower values of diffusion capacity for carbon monoxide (DLCO) (P = .035) and hemoglobin concentrations (P < .001). DLCO was strongly correlated with peak V̇O2 (r(2) = 0.56). Adjustment for DLCO reduced the effect of NAC on peak V̇O2 without suppressing it. CONCLUSIONS: NAC was associated with lower preoperative peak V̇O2 in subjects with non-small-cell lung cancer. This lower aerobic fitness may result from NAC-induced reduction in pulmonary gas exchange or heart toxicity. Since lower fitness is linked to poorer outcome, the decision for NAC may have to be balanced with its possible toxicity.

A 28-Year-Old Man Presenting With Intractable Dry Cough and a History of Ulcerative Colitis.

A 28-year-old man of Japanese descent presented to the ED with a 2-month history of dry cough, shortness of breath, and weakness. He did not complain of fever, chest pain, or abdominal symptoms, and had no history of smoking. The patient's medical history was significant for an episode of ulcerative colitis 6 years previously after presenting with bloody diarrhea, stomach pain, fever, weight loss, and bilateral episcleritis. He had been treated consecutively with mesalazine, azathioprine, infliximab, golimumab, and adalimumab. Concomitant respiratory symptoms had been present during 2 flare-ups of severe ulcerative colitis disease activity and were successfully treated with a course of oral prednisone.

[New therapeutic approaches in 2015 in pulmonary medicine]. / Pneumologie.

During the past year, among the many novelties in the field of pulmonary medicine, the authors chose to focus on 4 items: the positive contribution of systemic steroids on clinical improvement and length of stay in patients hospitalized for community-acquired pneumonia; the interesting results obtained with high flow oxygen, heated and humidified, in acute care and in normocapnic respiratory failure, a device which warrants further clinical testing in other indications; the now documented benefits of lung volume reduction procedures by bronchoscopy using coils in severe emphysema with hyperinflation; and the publication of new recommendations regarding pulmonary hypertension, with an emphasis on new molecules and their efficacy, on an early use of combination treatments, and on the importance of expert centres in managing these patients.