Curr Opin Pulm Med [JOURNAL]
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200 papers
Novel interventions on the tricuspid valve: how to consider the pulmonary circulation?
Wensel R, Opitz C
Curr Opin Pulm Med · 2024 Sep · PMID 39114937 · Publisher ↗
PURPOSE OF REVIEW: This review addresses treatment options for moderate to severe tricuspid valve regurgitation and the importance of right ventricular function and the pulmonary circulation. RECENT FINDINGS: Several int...
PURPOSE OF REVIEW: This review addresses treatment options for moderate to severe tricuspid valve regurgitation and the importance of right ventricular function and the pulmonary circulation. RECENT FINDINGS: Several interventional treatment options for severe tricuspid regurgitation have been developed including transcatheter edge-to-edge repair, annuloplasty and valve replacement. So far, transcatheter edge-to-edge repair is most frequently used with procedural success rates of more than 95% and improvements in functional and quality of life parameters for up to 2 years. Right ventricular function as well as pulmonary artery pressure and resistance levels are important outcome predictors. Mean pulmonary artery pressure more than 30 mmHg, transpulmonary gradient more than 17 mmHg and right ventricular to pulmonary artery coupling ratio less than 0.406 indicate poor outcome. SUMMARY: Despite the remarkable safety of interventional treatment of severe tricuspid regurgitation right ventricular dysfunction and abnormal pulmonary hemodynamics are important determinants of procedural success and clinical outcome.Complete hemodynamic work-up should be an integral part of prerepair assessment although validated data predicting outcome are limited.
Current hot topics in pulmonary hypertension.
Kovacs G, Zeder K
Curr Opin Pulm Med · 2024 Sep · PMID 39114936 · Publisher ↗
Abstract loading — click title to view on PubMed.
Elexacaftor-tezacaftor-ivacaftor use after solid organ transplant.
Kapnadak SG, Ramos KJ
Curr Opin Pulm Med · 2024 Nov · PMID 39082904 · Full text
PURPOSE OF REVIEW: In 2019, the United States Food and Drug Administration approved a breakthrough therapeutic for cystic fibrosis, elexacaftor-tezacaftor-ivacaftor (ETI), because of its profound effect on lung function...
PURPOSE OF REVIEW: In 2019, the United States Food and Drug Administration approved a breakthrough therapeutic for cystic fibrosis, elexacaftor-tezacaftor-ivacaftor (ETI), because of its profound effect on lung function in large phase III clinical trials. ETI acts directly on the dysfunctional protein that causes the systemic manifestations of cystic fibrosis and also leads to improvement in nonpulmonary symptoms of cystic fibrosis. Transplant recipients were excluded from the pivotal clinical trials of ETI but may stand to benefit from correction of the underlying protein defect. Drug interactions between the three drugs in ETI and immunosuppression medications are one of the primary concerns about using ETI after transplant. No rigorous studies exist to assess the safety of ETI in transplant recipients. RECENT FINDINGS: Multiple recent publications describe the use of ETI after solid organ transplantation, including lung and nonlung transplants, and the real-world evidence for drug interactions between ETI and immunosuppression medications. In nonlung transplant recipients, the pulmonary benefits of ETI have been confirmed, but adverse events occur and may have implications for their transplanted organ (e.g. liver biopsy in the setting of elevated transaminases). Lung transplant recipients may have higher rates of ETI discontinuation than nontransplant recipients given a lack of direct pulmonary benefit and frequency of side effects. Drug interactions have not been difficult to manage, with most studies reporting variable rates of mild to moderate increased tacrolimus levels after initiation of ETI. SUMMARY: Limited data exist to support the use of ETI after solid organ transplantation and further research is warranted. Given the unknown risks and benefits, case by case consideration of ETI use is indicated when extra-pulmonary manifestations are present in lung transplant recipients with cystic fibrosis. Given the proven benefit in cystic fibrosis lung disease, benefits likely outweigh the risks of ETI for nonlung solid organ transplant recipients.
Epigenetics and aging: relevance for sleep medicine.
Cortese R
Curr Opin Pulm Med · 2024 Nov · PMID 39082896 · Full text
PURPOSE OF REVIEW: Sleep disorders encompass a wide range of conditions with substantial individual variability. Epigenetics, the study of heritable changes beyond DNA sequence, offers a promising avenue for personalized...
PURPOSE OF REVIEW: Sleep disorders encompass a wide range of conditions with substantial individual variability. Epigenetics, the study of heritable changes beyond DNA sequence, offers a promising avenue for personalized medicine in this field. RECENT FINDINGS: There is great potential of epigenetic markers for sleep disorder diagnosis and the development of epigenetic drugs for targeted treatment. Epigenetic age acceleration, a marker of biological aging, is linked to sleep disorders and comorbidities. Very importantly, this acceleration may be reversible with effective treatment. SUMMARY: While the underlying mechanisms and assessment of clinical utility require further investigation, the potential of epigenetics in sleep medicine is recognized. Future research focused on closing knowledge gaps and clinical validation is crucial to translate these findings into practical applications, paving the way for more effective and personalized management of sleep disorders.
Outcomes of lung transplantation in cystic fibrosis.
Avdimiretz N, Halloran K, Benden C
Curr Opin Pulm Med · 2024 Nov · PMID 39082306 · Publisher ↗
PURPOSE OF REVIEW: Lung transplantation (LTX) has transformed care for people with cystic fibrosis (pwCF) suffering from advanced cystic fibrosis lung disease (ACFLD), and it has evolved into an accepted therapy for pati...
PURPOSE OF REVIEW: Lung transplantation (LTX) has transformed care for people with cystic fibrosis (pwCF) suffering from advanced cystic fibrosis lung disease (ACFLD), and it has evolved into an accepted therapy for patients with ACFLD across all ages. We review cystic fibrosis as a major indication for LTX, particularly highlighting outcomes including survival, a changing landscape over time, and factors affecting sequelae following LTX in cystic fibrosis. RECENT FINDINGS: Although some populations such as those undergoing lung retransplantation exhibit inferior posttransplant outcomes, LTX for pwCF provides an excellent long-term survival that has significantly improved over time, likely due to specialized cystic fibrosis center care and recognition of common comorbidities in pwCF post-LTX. There are gaps in post-LTX outcomes for pwCF, including that identified between Canada and the United States, and that seen in adolescents - both of which are likely multifactorial. In particular, the revolution in cystic fibrosis medical therapy with CFTR modulator therapy has resulted in a dramatic decline in programs performing LTX for cystic fibrosis. How durable this effect will remains to be seen. SUMMARY: Overall, LTX remains a well accepted ultimate therapy option in patients with ACFLD if compatible with the individual's goals of care, offering an improved quality of life and maximization of overall survival.
Pulmonary vascular diseases at high altitude - is it safe to live in the mountains?
Titz A, Hoyos R, Ulrich S
Curr Opin Pulm Med · 2024 Sep · PMID 39036990 · Full text
PURPOSE OF REVIEW: This review addresses the concern of the health effects associated with high-altitude living and chronic hypoxia with a focus on pulmonary hypertension. With an increasing global population residing at...
PURPOSE OF REVIEW: This review addresses the concern of the health effects associated with high-altitude living and chronic hypoxia with a focus on pulmonary hypertension. With an increasing global population residing at high altitudes, understanding these effects is crucial for public health interventions and clinical management. RECENT FINDINGS: Recent literature on the long-term effects of high-altitude residence and chronic hypoxia is comprehensively summarized. Key themes include the mechanisms of hypoxic pulmonary vasoconstriction, the development of pulmonary hypertension, and challenges in distinguishing altitude-related pulmonary hypertension and classical pulmonary vascular diseases, as found at a low altitude. SUMMARY: The findings emphasize the need for research in high-altitude communities to unravel the risks of pulmonary hypertension and pulmonary vascular diseases. Clinically, early and tailored management for symptomatic individuals residing at high altitudes are crucial, as well as access to advanced therapies as proposed by guidelines for pulmonary vascular disease. Moreover, identifying gaps in knowledge underscores the necessity for continued research to improve understanding and clinical outcomes in high-altitude pulmonary vascular diseases.
Applications of artificial intelligence in computed tomography imaging for phenotyping pulmonary hypertension.
Sharkey MJ, Checkley EW, Swift AJ
Curr Opin Pulm Med · 2024 Sep · PMID 38989815 · Full text
PURPOSE OF REVIEW: Pulmonary hypertension is a heterogeneous condition with significant morbidity and mortality. Computer tomography (CT) plays a central role in determining the phenotype of pulmonary hypertension, infor...
PURPOSE OF REVIEW: Pulmonary hypertension is a heterogeneous condition with significant morbidity and mortality. Computer tomography (CT) plays a central role in determining the phenotype of pulmonary hypertension, informing treatment strategies. Many artificial intelligence tools have been developed in this modality for the assessment of pulmonary hypertension. This article reviews the latest CT artificial intelligence applications in pulmonary hypertension and related diseases. RECENT FINDINGS: Multistructure segmentation tools have been developed in both pulmonary hypertension and nonpulmonary hypertension cohorts using state-of-the-art UNet architecture. These segmentations correspond well with those of trained radiologists, giving clinically valuable metrics in significantly less time. Artificial intelligence lung parenchymal assessment accurately identifies and quantifies lung disease patterns by integrating multiple radiomic techniques such as texture analysis and classification. This gives valuable information on disease burden and prognosis. There are many accurate artificial intelligence tools to detect acute pulmonary embolism. Detection of chronic pulmonary embolism proves more challenging with further research required. SUMMARY: There are numerous artificial intelligence tools being developed to identify and quantify many clinically relevant parameters in both pulmonary hypertension and related disease cohorts. These potentially provide accurate and efficient clinical information, impacting clinical decision-making.
Optimal long-term anticoagulation after acute pulmonary embolism: current state of the art and a look into the near future.
Kimmerle AR, Noflatscher M, Raggam RB
Curr Opin Pulm Med · 2024 Sep · PMID 38989794 · Publisher ↗
PURPOSE OF REVIEW: This review aims to summarize the current state of the art and future directions in optimal long-term anticoagulation following acute pulmonary embolism (PE). RECENT FINDINGS: Actual studies and guidel...
PURPOSE OF REVIEW: This review aims to summarize the current state of the art and future directions in optimal long-term anticoagulation following acute pulmonary embolism (PE). RECENT FINDINGS: Actual studies and guidelines underscore the preference for direct oral anticoagulants (DOAC) in standard therapeutic doses for maintenance therapy post-PE, while considering patient-specific factors and dose-reduction criteria. Risk stratification should always include the assessment of concomitant trigger- or risk factors regarding their strength and persistence. The use of tools like specific scores can facilitate the identification of optimal candidates for long-term therapy, emphasizing once more personalized approaches and strategies. Special patient groups, such as cancer associated thrombosis, chronic thromboembolic pulmonary hypertension or antiphospholipid syndrome require even more tailored therapy approaches. SUMMARY: Optimal long-term anticoagulation post-PE should be guided by straightforward and individual risk assessment strategies. The array of indications for DOACs has gotten wider in last years, also within special patient groups. Still, chronic thromboembolic pulmonary hypertension and antiphospholipid syndrome remains domain of vitamin K agonists.
Progress for all: addressing disparities in sarcoidosis.
Obi ON, Sharp M, Harper L
Curr Opin Pulm Med · 2024 Sep · PMID 38989780 · Full text
PURPOSE OF REVIEW: The impact of healthcare disparities in the treatment, care, and outcomes of patients with sarcoidosis has been described. There is paucity of literature on ways to address these disparities with a goa...
PURPOSE OF REVIEW: The impact of healthcare disparities in the treatment, care, and outcomes of patients with sarcoidosis has been described. There is paucity of literature on ways to address these disparities with a goal to improving health outcomes for patients with sarcoidosis. RECENT FINDINGS: Recent findings in other respiratory and systemic diseases suggest that multifaceted interventions directed at improving care at various levels including individual, family, and larger societal levels have been successful in dismantling some of the social and structural barriers to care and consequently have resulted in a reduction in disparate disease outcomes. We explore what some of these interventions would look like in sarcoidosis. SUMMARY: The impact of healthcare disparities in the treatment, care, and outcomes of patients with sarcoidosis has been described. We outline various steps and approaches aimed at addressing these health disparities with a goal to improving outcomes for those most impacted by disease.
Leveraging AI technology in sarcoidosis.
Premjee A, Li L, Garikapati S … +2 more , Sarpong KN, Morgenthau AS
Curr Opin Pulm Med · 2024 Sep · PMID 38989774 · Publisher ↗
PURPOSE OF REVIEW: Sarcoidosis is a systemic, granulomatous disease of uncertain cause. Diagnosis may be difficult, prognosis uncertain and response to treatment unpredictable. The application of artificial intelligence...
PURPOSE OF REVIEW: Sarcoidosis is a systemic, granulomatous disease of uncertain cause. Diagnosis may be difficult, prognosis uncertain and response to treatment unpredictable. The application of artificial intelligence to sarcoidosis may provide clinical decision support for these challenges. This review will provide an overview of current and potential future applications of artificial intelligence in sarcoidosis. RECENT FINDINGS: The predominant application of artificial intelligence in sarcoidosis is imaging. Imaging models may differentiate sarcoidosis from other pulmonary disorders. Models, which predict survival and identify key factors relevant to prognosis are also available. The application of cluster analysis to organize sarcoidosis patients into developmental phenotypes is underway. Machine learning algorithms to evaluate the treatment response of sarcoidosis patients do not yet exist but similar models may evaluate patients with other inflammatory disease. The potential applications of artificial intelligence to sarcoidosis is vast, but there are practical limitations that warrant consideration. These include: the accessibility of data, biases in data, cost and privacy. SUMMARY: The application of artificial intelligence in medicine is still in its early stages but models are poised to support the diagnostic and prognostic challenges in sarcoidosis patients. The predictive power of these artificial intelligence is likely to come from combining various models, trained on content-rich datasets from phenotypically heterogeneous sarcoidosis patients.
Big data in sarcoidosis.
Rivera NV
Curr Opin Pulm Med · 2024 Sep · PMID 38967053 · Full text
PURPOSE OF REVIEW: This review provides an overview of recent advancements in sarcoidosis research, focusing on collaborative networks, phenotype characterization, and molecular studies. It highlights the importance of c...
PURPOSE OF REVIEW: This review provides an overview of recent advancements in sarcoidosis research, focusing on collaborative networks, phenotype characterization, and molecular studies. It highlights the importance of collaborative efforts, phenotype characterization, and the integration of multilevel molecular data for advancing sarcoidosis research and paving the way toward personalized medicine. RECENT FINDINGS: Sarcoidosis exhibits heterogeneous clinical manifestations influenced by various factors. Efforts to define sarcoidosis endophenotypes show promise, while technological advancements enable extensive molecular data generation. Collaborative networks and biobanks facilitate large-scale studies, enhancing biomarker discovery and therapeutic protocols. SUMMARY: Sarcoidosis presents a complex challenge due to its unknown cause and heterogeneous clinical manifestations. Collaborative networks, comprehensive phenotype delineation, and the utilization of cutting-edge technologies are essential for advancing our understanding of sarcoidosis biology and developing personalized medicine approaches. Leveraging large-scale epidemiological resources and biobanks and integrating multilevel molecular data offer promising avenues for unraveling the disease's heterogeneity and improving patient outcomes.
Palliative care in advanced pulmonary fibrosis.
Kalluri M
Curr Opin Pulm Med · 2024 Sep · PMID 38963163 · Publisher ↗
PURPOSE OF REVIEW: Updated clinical practice guidelines for pulmonary fibrosis recommend early integrated palliative care using a primary palliative approach. Clinicians need to be aware of the various palliative interve...
PURPOSE OF REVIEW: Updated clinical practice guidelines for pulmonary fibrosis recommend early integrated palliative care using a primary palliative approach. Clinicians need to be aware of the various palliative interventions in order to implement guidelines' recommendations. This review provides an update on evidence-based palliative therapies. RECENT FINDINGS: Literature review indicates early integration of palliative care in pulmonary fibrosis is feasible and meets patient needs. Key components of a primary palliative approach include comprehensive symptoms and needs screening, systematic symptom management using nonpharmacologic interventions, supplemental oxygen and opioids for dyspnea and cough. Patient-centered communication is essential for successful integration of palliative care. Early, iterative advance care planning in clinic to understand patient goals, values and preferences for current and future care, improves patient care and satisfaction. Prioritizing caregiver inclusion in clinics can address their needs. Collaborating with a multidisciplinary allied team facilitates integration of palliative care and supports patients throughout the disease course. Different models of palliative care delivery exist and can be adapted for local use. The use of artificial intelligence algorithms and tools may improve care and continuity. SUMMARY: Clinicians must develop competency in palliative care. Organizational and policy support is essential to enable seamless integration of palliative care throughout the care continuum.
Clinical trials in pulmonary sarcoidosis; what is needed, what is happening and what is next?
Veltkamp M
Curr Opin Pulm Med · 2024 Sep · PMID 38963156 · Publisher ↗
PURPOSE OF REVIEW: There is a clinical unmet need to improve treatment for patients with pulmonary sarcoidosis. Both retrospective and prospective drug trials are hampered by the fact that patients with sarcoidosis are c...
PURPOSE OF REVIEW: There is a clinical unmet need to improve treatment for patients with pulmonary sarcoidosis. Both retrospective and prospective drug trials are hampered by the fact that patients with sarcoidosis are characterized by a heterogeneous presentation and disease course. In this review, an overview is given of different drug trials in pulmonary sarcoidosis with an emphasis on different primary endpoints and the problems related to them. RECENT FINDINGS: In recent years, using significant input from patients with sarcoidosis, different task-forces/studies tried to develop a core set of most important outcomes to measure in future studies on treatment of sarcoidosis. Furthermore, at present, three major clinical trials are being conducted on new drugs for treatment of pulmonary sarcoidosis. SUMMARY: Progress has been made to develop a core set of outcomes measures that can be used in making a combined primary endpoint in future drug trials in sarcoidosis.
The future of clinical trials in idiopathic pulmonary fibrosis.
Spagnolo P, Maher TM
Curr Opin Pulm Med · 2024 Sep · PMID 38963152 · Full text
PURPOSE OF REVIEW: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with a poor prognosis and limited therapeutic options. A multitude of promising compounds are currently being investigated; however, th...
PURPOSE OF REVIEW: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with a poor prognosis and limited therapeutic options. A multitude of promising compounds are currently being investigated; however, the design and conductance of late-phase clinical trials in IPF has proven particularly challenging. RECENT FINDINGS: Despite promising phase 2 data, ziritaxestat, an autotaxin inhibitor, pentraxin-2, an endogenous protein that regulates wound healing and fibrosis, and pamrevlumab, a human monoclonal antibody against connective tissue growth factor, failed to show efficacy in phase 3 trials. Endpoint selection is critical for the design, execution, and success of clinical trials; recently, attention has been paid to the assessment of how patients feel, function, and survive with the aim of aligning scientific objectives and patient needs in IPF. External control arms are control patients that derive from historical randomized controlled trials, registries, or electronic health records. They are increasingly used to assess treatment efficacy in clinical trials owing to their potential to reduce study duration and cost and increase generalizability of findings. SUMMARY: Advances in study design, end point selection and statistical analysis, and innovative strategies for more efficient enrolment of study participants have the potential to increase the likelihood of success of late-phase clinical trials in IPF.
Cardiac sarcoidosis and neurosarcoidosis - multidisciplinary approach for diagnosis.
Kouranos V, Sharma R, Wells A … +1 more , Singh-Curry V
Curr Opin Pulm Med · 2024 Sep · PMID 38958578 · Publisher ↗
PURPOSE OF REVIEW: The current review aims to highlight the role of multidisciplinary approach in the diagnosis of patients with cardiac and neurosarcoidosis. Multidisciplinary approach integrates the available clinical...
PURPOSE OF REVIEW: The current review aims to highlight the role of multidisciplinary approach in the diagnosis of patients with cardiac and neurosarcoidosis. Multidisciplinary approach integrates the available clinical information, imaging and histopathological results aiming to reach a definite or at least provisional diagnosis and allow appropriate management. Multidisciplinary approach is the reference standard for diagnosis of interstitial lung disease and should be strongly considered in complex clinical conditions such as cardiac sarcoidosis (CS) and neurosarcoidosis. RECENT FINDINGS: Histopathological confirmation of noncaseating granulomatous inflammation provides a definite diagnosis of sarcoidosis involving any organ. However, a provisional high confidence or even definite clinical diagnosis can be reached using multidisciplinary evaluation of all available evidence. The diagnosis of cardiac sarcoidosis and neurosarcoidosis requires the integration of different expertise based on the current diagnostic criteria sets. Identifying typical or at least compatible patterns on advanced imaging modalities (CMR and Fluro-Deoxy-Glucose Positron Emission Tomography (FDG-PET)) seems key for the diagnosis of CS, while a confident diagnosis of extra-cardiac disease supports an at least provisional diagnosis. Similarly, in neurosarcoidosis integrating compatible MRI appearances and cerebrospinal fluid results in patients with systemic sarcoidosis allows an at least provisional diagnosis. Exclusion of alternative differential diagnoses is crucial and requires high clinical suspicion, imaging review expertise and appropriate tests performance. SUMMARY: There have been considerable advances in the diagnostic approach of patients with cardiac and neurosarcoidosis. Multidisciplinary approach for both diagnosis and management is required to reach a confident clinical diagnosis and should be applied when possible.
Pulmonary vascular disease in chronic lung diseases: cause or comorbidity?
Blanco I, Torres-Castro R, Barberà JA
Curr Opin Pulm Med · 2024 Sep · PMID 38958570 · Publisher ↗
PURPOSE OF REVIEW: To provide timely and relevant insights into the complex relationship between pulmonary vascular disease (PVD) and chronic lung disease (CLD), focusing on the causative and consequential dynamics betwe...
PURPOSE OF REVIEW: To provide timely and relevant insights into the complex relationship between pulmonary vascular disease (PVD) and chronic lung disease (CLD), focusing on the causative and consequential dynamics between these conditions. RECENT FINDINGS: There are shared pathogenic mechanisms between pulmonary arterial hypertension (PAH) and group 3 pulmonary hypertension, including altered expression of mediators and growth factors implicated in both conditions. Factors such as hypoxia, hypoxemia, and hypercapnia also contribute to pulmonary vascular remodelling and endothelial dysfunction. However, the role of hypoxia as the sole driver of pulmonary hypertension in CLD is being reconsidered, particularly in chronic obstructive pulmonary disease (COPD), with evidence suggesting a potential role for cigarette smoke products in initiating pulmonary vascular impairment. On the other hand, interstitial lung disease (ILD) encompasses a group of heterogeneous lung disorders characterized by inflammation and fibrosis of the interstitium, leading to impaired gas exchange and progressive respiratory decline, which could also play a role as a cause of pulmonary hypertension. SUMMARY: Understanding the intricate interplay between the pulmonary vascular compartment and the parenchymal and airway compartments in respiratory disease is crucial for developing effective diagnostic and therapeutic strategies for patients with PVD and CLD, with implications for both clinical practice and research.
Pulmonary rehabilitation in idiopathic pulmonary fibrosis.
Dowman LM, Holland AE
Curr Opin Pulm Med · 2024 Sep · PMID 38958566 · Publisher ↗
PURPOSE OF REVIEW: This review synthesizes the expanding evidence for pulmonary rehabilitation that has led to its recommended inclusion in the holistic care of people with idiopathic pulmonary fibrosis (IPF), as well as...
PURPOSE OF REVIEW: This review synthesizes the expanding evidence for pulmonary rehabilitation that has led to its recommended inclusion in the holistic care of people with idiopathic pulmonary fibrosis (IPF), as well as discussing strategies that may maximize and sustain benefits. RECENT FINDINGS: Pulmonary rehabilitation is an effective intervention leading to significant improvements in exercise tolerance, symptoms, and quality of life for people with IPF. Improvements in symptoms and quality of life can persist longer term, whereas functional capacity does not; therefore, strategies to preserve functional capacity are an important area of research. Referral early in the disease course is encouraged to promote longer lasting effects. Evidence that high-intensity interval training may optimize benefits of exercise training is emerging. Supplemental oxygen is frequently used to manage exercise-induced desaturation, although its use as an adjunct therapy requires more evidence. SUMMARY: Current evidence strongly supports the inclusion of pulmonary rehabilitation in the standard holistic care of IPF, with early participation encouraged. Further research is needed to establish the optimal exercise strategies, modalities and adjunct therapies that enhance outcomes of pulmonary rehabilitation and promote longer lasting effects.
The prognostic relevance of exercise pulmonary hypertension in cardiac and pulmonary diseases.
Zeder K, Douschan P, Foris V … +4 more , Sassmann T, Maron BA, Olschewski H, Kovacs G
Curr Opin Pulm Med · 2024 Sep · PMID 38958564 · Publisher ↗
PURPOSE OF REVIEW: In this review, we provide an overview of the prognostic implications of exPH in patients with various common cardiac and pulmonary diseases. RECENT FINDINGS: Exercise pulmonary hypertension (exPH) has...
PURPOSE OF REVIEW: In this review, we provide an overview of the prognostic implications of exPH in patients with various common cardiac and pulmonary diseases. RECENT FINDINGS: Exercise pulmonary hypertension (exPH) has been recently re-introduced in the current European Society of Cardiology/European Respiratory Society pulmonary hypertension guidelines. Accordingly, exPH is defined as a mean pulmonary arterial pressure (mPAP)/cardiac output ( CO ) slope greater than 3 mmHg/l/min. Key considerations for this re-introduction included increasing understanding on normal pulmonary hemodynamics during exercise and the broadly available evidence on the association of an abnormal mPAP/ CO slope with poor survival in the general population and in different disease entities. SUMMARY: Exercise (patho-)physiology has opened a new field for clinical research facilitating recognition of cardiovascular and pulmonary vascular diseases in an early stage. Such early recognition with significant prognostic and possibly therapeutic relevance, but being undetectable at rest, makes exercise pulmonary hemodynamics particularly interesting for common diseases, such as valvular heart disease, left heart disease, and chronic pulmonary disease.
Integrating the assessment of quality of life in care and research in pulmonary fibrosis.
Aronson KI, Swigris JJ, Wijsenbeek M
Curr Opin Pulm Med · 2024 Sep · PMID 38946547 · Publisher ↗
PURPOSE OF REVIEW: Pulmonary fibrosis (PF) negatively influences health-related quality of life (HRQOL). Patients living with PF have voiced the desire for a focus on symptoms and HRQOL in both disease monitoring and tre...
PURPOSE OF REVIEW: Pulmonary fibrosis (PF) negatively influences health-related quality of life (HRQOL). Patients living with PF have voiced the desire for a focus on symptoms and HRQOL in both disease monitoring and treatment decisions. RECENT FINDINGS: Currently available disease modifying treatments do little to impact HRQOL. Newer studies evaluating pharmacologic and nonpharmacologic therapies targeting symptoms and HRQOL in PF have been conducted with some promising results. There is increasing recognition of the importance of incorporating HRQOL as a higher tier endpoint in clinical trials. Disease-specific measure of HRQOL have been developed for those living with PF, and there is ongoing work to better understand the validity and reliability characteristics of these tools. In addition to research, there is recognition of the potential benefits of measuring HRQOL and symptoms in clinical practice in facilitate integrating patient perspective into care and allow for more personalized treatment approaches. SUMMARY: There is increased momentum to discover treatments that impact HRQOL in PF. More work is desperately needed to identify better treatment targets, and to incorporate HRQOL and symptoms as higher tier endpoints in clinical trials. Further work is also needed to address the practicalities of integrating HRQOL measurement into clinical care.
Genetic background of pulmonary (vascular) diseases - how much is written in the codes?
Eichstaedt CA, Haas SO, Shaukat M … +1 more , Grünig E
Curr Opin Pulm Med · 2024 Sep · PMID 38913028 · Publisher ↗
PURPOSE OF REVIEW: To provide a comprehensive overview of the underlying genetic defects of pulmonary (vascular) diseases and novel treatment avenues. RECENT FINDINGS: Pulmonary arterial hypertension (PAH) is the prime e...
PURPOSE OF REVIEW: To provide a comprehensive overview of the underlying genetic defects of pulmonary (vascular) diseases and novel treatment avenues. RECENT FINDINGS: Pulmonary arterial hypertension (PAH) is the prime example of a pulmonary vascular disease, which can be caused by genetic mutations in some patients. Germline mutations in the BMPR2 gene and further genes lead to vessel remodelling, increase of pulmonary vascular resistance and onset of heritable PAH. The PAH genes with the highest evidence and strategies for genetic testing and counselling have been assessed and evaluated in 2023 by international expert consortia. Moreover, first treatment options have just arisen targeting the molecular basis of PAH. SUMMARY: Apart from PAH, this review touches on the underlying genetic causes of further lung diseases including alpha 1 antitrypsin deficiency, cystic fibrosis, familial pulmonary fibrosis and lymphangioleiomyomatosis. We point out the main disease genes, the underlying pathomechanisms and novel therapies trying not only to relieve symptoms but to treat the molecular causes of the diseases.
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