Unusual Inherited Pulmonary Diseases Which Provide Clues to Pulmonary Physiology and Function
Stephen D. Litwin in Genetic Determinants of Pulmonary Disease, 2020
Symptoms usually appear when the pulmonary hypertension is well established. Dyspnea on exertion, fatigue, and weakness are related to a low cardiac output which cannot increase on demand. Similarly, syncope may occur on effort or, when occurring at rest, is likely to be due to cardiac arrhythmias. Chest pain on exertion is probably the result of ischemia of the considerably hypertrophied right ventricle. Hoarseness may result from compression of the left recurrent laryngeal nerve by the enlarged left pulmonary artery. Hemoptysis is not uncommon, usually minor, but may be recurrent. Sudden death is a serious risk and is particularly liable to occur during catheterization, anesthesia, or surgical procedure. The mechanism is either arrhythmias or acute right ventricular failure.
Pulmonary Hypertension in Pregnancy
Afshan B. Hameed, Diana S. Wolfe in Cardio-Obstetrics, 2020
Medical management of pulmonary hypertension in pregnancy should be done carefully by a pulmonary hypertension specialist as part of a multidisciplinary team approach [9]. Management is guided by the etiology of the pulmonary hypertension, severity of disease, and the functional status. Most pregnant women with pulmonary hypertension are treated with a prostanoid, usually epoprostenol. Other medication classes include phosphodiesterase 5-inhibitors and CCBs (Table 15.4). Data on the benefits of each option are primarily limited to the nonpregnant patient. It is important to note that endothelin receptor antagonists (bosentan, macitentan, ambrisentan) and guanylate cyclase stimulants (riociguat) are contraindicated in pregnancy due to concern for teratogenicity [16]. As a result, access to these medications is restricted for female patients and must be through a registry with outlined contraception and sterilization recommendations [16].
Physiological interpretation of pressure waveforms
John Edward Boland, David W. M. Muller in Interventional Cardiology and Cardiac Catheterisation, 2019
Most patients with mitral stenosis present with dyspnoea on effort and these patients have elevated PAW pressures and, more importantly, elevated pulmonary capillary pressure which causes the symptoms. Pulmonary artery pressures are often only mildly elevated (passive pulmonary hypertension). At times these symptoms are replaced by listlessness and fatigue. Haemodynamically the latter patients have much higher PA pressures, higher pulmonary vein resistance and slightly higher PAW and LA pressures, with a much lower cardiac output. Such patients develop what Grossman refers to as ‘second stenosis’ from pulmonary vascular disease affecting distal arterioles and the capillary bed (active pulmonary hypertension).8 The cause of these reactive changes is uncertain. The latter group shows clinically and on the electrocardiogram (ECG) the signs of pulmonary hypertension and right ventricular pressure overload. When the active pulmonary hypertension becomes severe, prognosis is worsened.
Diagnosis and management of pulmonary veno-occlusive disease
Published in Expert Review of Respiratory Medicine, 2023
Sabina Solinas, Athénaïs Boucly, Antoine Beurnier, Mithum Kularatne, Julien Grynblat, Mélanie Eyries, Peter Dorfmüller, Olivier Sitbon, Marc Humbert, David Montani
PVOD and PAH share a common clinical presentation and are characterized by progressive dyspnea, fatigue, and decreased exercise tolerance, even if PVOD patients present a more severe dyspnea than PAH patients having the same hemodynamic parameters [50]. Other symptoms include palpitations, chest pain with exertion, dizziness, syncope, and hemoptysis. Although occult alveolar hemorrhage has been documented in bronchoalveolar lavage of PVOD patients, the occurrence of hemoptysis does not seem to be more frequent than in PAH [8,16]. Physical signs include classical signs of pulmonary hypertension and right heart failure. Cyanosis is more frequently observed due to frequent concomitant hypoxemia. Clubbing and Raynaud’s phenomenon are reported to occur in both PVOD and PAH in a comparable proportion [16]. Other signs suggestive of PVOD may include pulmonary edema and/or pleural effusions [51]. The development of pulmonary edema following initiation of PAH-approved drugs strongly suggests the diagnosis of PVOD.
New promising drugs for the treatment of systemic sclerosis: pathogenic considerations, enhanced classifications, and personalized medicine
Published in Expert Opinion on Investigational Drugs, 2021
Alain Lescoat, John Varga, Marco Matucci-Cerinic, Dinesh Khanna
Recent breakthroughs in the management of pulmonary hypertension, with a positive impact on survival, have been achieved by early association of existing drugs rather than the design of new treatments. This should inform the research on investigational drugs in SSc, especially considering that immunomodulatory drugs are now broadly used in patients with early dcSSc. Therefore, the identification of new therapeutic options has to be considered on top of existing immunomodulators already in use as background therapies. The association of monoclonal antibodies could also constitute a change of paradigm in the management if SSc, if such combinations prove to be well tolerated. The simultaneous inhibition of profibrotic and pro-inflammatory pathways by a single drug (JAK inhibitors) or through combination strategies (pirfenidone and MMF, or romilkimab and immunomodulators) may constitute a promising option for disease-modifying management of SSc. The introduction of anti-fibrotic drugs at the early inflammatory phase of the disease, in addition to immunomodulatory treatments, may also allow clinically meaningful results for patients with dcSSc in the future. Nonetheless, the relevance and safety of such associations are still to be determined in RCT.
Xinmai 'an extract enhances the efficacy of sildenafil in the treatment of pulmonary arterial hypertension via inhibiting MAPK signalling pathway
Published in Pharmaceutical Biology, 2021
Yaolu Zhu, Yabin Sun, Shichang Zhang, Chuyuan Li, Yiwei Zhao, Boxin Zhao, Guofeng Li
Pulmonary hypertension refers to a hemodynamic and pathophysiological state in which the increase of pulmonary artery pressure exceeds a certain threshold, which can lead to right heart failure. It is a common and frequently-occurring disease with a high disability rate and fatality rate, which should arouse people’s great attention (Voelkel et al. 2012; Zangiabadi et al. 2014). The primary treatments for pulmonary arterial hypertension (PAH) include epoprostenol and derivatives, endothelin receptor antagonists, calcium channel blockers, and recently researchers have focussed on phosphodiesterase type 5 enzyme (PDE-5) inhibitors such as sildenafil (SIL), which mainly focus on expanding blood vessels selectively, weakening anti-proliferation ability of PASMCs (Montani et al. 2014). These drugs can improve the quality of life of patients with pulmonary hypertension to a certain extent. However, none of the aforementioned drugs, including SIL, can completely reverse the development of the disease, and there are many adverse reactions (Hao et al. 2020). Therefore, there is an urgent need for more new methods to treat PAH.
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