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Pulmonary hypertension associated with chronic obstructive lung disease and idiopathic pulmonary fibrosis

Purpose of review : Severe pulmonary hypertension worsens the prognosis of patients with chronic obstructive pulmonary disease (COPD) or idiopathic pulmonary fibrosis (IPF). With the aim of better understanding the pathogenesis of this event and identifying the possible targets for therapeutic intervention, a great deal of clinical and translational research is now focused on this relevant field of medicine.

Recent findings : Some studies that were published last year have helped to better define the clinical and physiological profiles of patients with COPD or IPF and severe pulmonary hypertension. The importance of pulmonary rehabilitation was confirmed, particularly in patients with pulmonary hypertension associated with IPF. Information on the use of drugs approved for the treatment of pulmonary arterial hypertension is still very limited, because of some limitations and selection biases in the studies’ design. New strategies (i.e. the use of fasudil or sepiapterin in pulmonary hypertension associated with IPF) have been evaluated in animal models.

Summary : Pulmonary hypertension in COPD or IPF may range from mild to severe. When pulmonary hypertension is more advanced, it can drive a poor outcome. Therefore, future studies should focus on this subset.

Goal-oriented treatment of pulmonary arterial hypertension

Purpose of review : To understand the rational of establishing a goal-oriented therapy for pulmonary arterial hypertension management.

Recent findings : During the last decades, the development of targeted therapies and the recognition of prognostic markers represented a major step for improving pulmonary arterial hypertension survival. Now, changing the strategy on using these compounds therapies might represent the best way to optimize treatment response.

Summary : Prespecified goals with regular reassessment should be incorporated as the routine practice for pulmonary arterial hypertension management to provide the best available treatment, aiming to improve or maintain every patient in a clinical and functional status that reflects better long-term survival.

Chronic thromboembolic pulmonary hypertension: evolution in management

imagePurpose of review : Chronic thromboembolic pulmonary hypertension (CTEPH) is an important cause of pulmonary hypertension. Although surgery is potentially curative, some patients present with inoperable disease. In these patients, medical therapies for pulmonary arterial hypertension are increasingly being used.

Recent findings : The pathobiology of CTEPH development remains incompletely understood; however, evidence supports both large and small vessel disorder in patients with the disease. Surgical thromboendarterectomy is an increasingly well tolerated and often curative procedure and is the management strategy of choice for most patients. Although excellent outcomes in surgical management have been noted, the role of medical management in selected patients with inoperable or recurrent or persistent disease after surgery is increasing. A recent large, randomized controlled clinical trial of riociguat in CTEPH demonstrated improvements in exercise capacity, functional class, and hemodynamics. A safe, effective angioplasty approach to CTEPH is being pursued in addition.

Summary : The approach to CTEPH management in the operable patient remains surgical, without clear benefit to preoperative pulmonary arterial hypertension-specific therapy at this time. Patients with inoperable disease or pulmonary hypertension following thromboendarterectomy, however, should be considered for medical management, with riociguat currently having the strongest evidence specific to CTEPH.

Role of imaging in the diagnosis of diffuse and interstitial lung diseases

imagePurpose of review : This review aims to provide a literature update focused on the role of imaging in the diagnosis, prognosis and quantification of interstitial lung diseases (ILDs). Special emphasis is given in the management of atypical cases and in the multidisciplinary team approach in reaching the diagnosis of the various ILDs.

Recent findings : Accumulated knowledge on imaging has increased the accuracy of differential diagnosis in atypical cases, in overlap of findings and in secondary as opposed to idiopathic ILDs. There is increasing awareness about interstitial lung abnormalities in smokers’ lungs from lung cancer screening and indirect evidence of linkage of fibrosis and smoking. Improvement in radiologic–pathologic correlation reveals less typical high-resolution computed tomography patterns to be predictive of pulmonary fibrosis. Major diagnostic criteria such as honeycombing may be hampered by the coexistence of emphysema. High-resolution computed tomography may predict clinical outcome and survival of patients in ILDs and is a decision maker in the multidisciplinary approach of diagnosis.

Summary : High-resolution computed tomography plays a crucial role in the diagnosis, prognosis, quantification and monitoring of ILDs. It provides a definite noninvasive diagnosis in typical findings and helps in reaching the most accurate diagnosis in a multidisciplinary discussion in equivocal cases.

Management of massive and submassive pulmonary embolism: focus on recent randomized trials

Purpose of review : Although early pulmonary revascularization is the treatment of choice for patients with high-risk (massive) pulmonary embolism, it remains controversial in patients with intermediate-risk (submassive) pulmonary embolism until recently. Recent published data on the management of high-risk and intermediate-risk pulmonary embolism patients will be the main focus of this review.

Recent findings : The PEITHO trial supports the rationale of risk stratification in normotensive patients with pulmonary embolism. Patients with right ventricular dilation on echocardiography and positive cardiac troponin test have a high intermediate risk of complication and death. Thrombolysis prevents hemodynamic collapse in these patients but with an increased risk of major bleeding particularly in older patients (>75 years). Reduced dose of thrombolysis and catheter-based reperfusion with or without fibrinolysis have shown promising results.

Summary : Thrombolysis is the treatment of choice for patients with high-risk pulmonary embolism. Surgical embolectomy is recommended in case of absolute contra-indication to thrombolysis. In patients with acute right ventricular dysfunction on cardiac imaging and myocardial injury, thrombolysis should be considered if they are 75 years or less of age and are at low risk of bleeding. Full-dose thrombolysis may be excessively risky in patients over 75 years. In patients with either RV dilation or elevated cardiac biomarker, thrombolysis is not recommended.

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