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Purpose: To evaluate the frequency of sleep-disordered breathing (SDB) and predictors of the presence of nocturnal desaturation in adults with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension., Methods: Outpatients with a hemodynamic diagnosis of precapillary pulmonary hypertension who underwent portable polysomnography were evaluated. Diagnosis and severity of SDB were assessed using three well-established respiratory disturbance index (RDI) thresholds: 5.0/h, 15.0/h, and 30.0/h, while nocturnal hypoxemia was defined by the average oxygen saturation (SpO 2 ) < 90%. Multiple linear regression analysis evaluated the potential relationships among explanatory variables with the dependent variable (average SpO 2 values), with comparisons based on the standardized regression coefficient (β). The R-squared (R 2 ; coefficient of determination) was used to evaluate the goodness-of-fit measure for the linear regression model., Results: Thirty-six adults were evaluated (69.4% females). The majority of the participants (75.0%) had SDB (26 with obstructive sleep apnea [OSA] and one with central sleep apnea [CSA]); while 50% of them had nocturnal hypoxemia. In the linear regression model (R 2  = 0.391), the mean pulmonary artery pressure [mPAP] (β - 0.668; p = 0.030) emerged as the only independent parameter of the average SpO 2 ., Conclusion: Our study found that the majority of the participants had some type of SDB with a marked predominance of OSA over CSA, while half of them had nocturnal desaturation. Neither clinical and hemodynamic parameters nor the RDI was a predictor of nocturnal desaturation, except for mPAP measured during a right heart catheterization, which emerged as the only independent and significant predictor of average SpO 2 ., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious and debilitating disease caused by occlusion of the pulmonary arterial bed by hematic emboli and by the resulting fibrous material. Such occlusion increases vascular resistance and, consequently, the pressure in the region of the pulmonary artery, which is the definition of pulmonary hypertension. The increased load imposed on the right ventricle leads to its progressive dysfunction and, finally, to death. However, CTEPH has a highly significant feature that distinguishes it from other forms of pulmonary hypertension: the fact that it can be cured through treatment with pulmonary thromboendarterectomy. Therefore, the primary objective of the management of CTEPH should be the assessment of patient fitness for surgery at a referral center, given that not all patients are good candidates. For the patients who are not good candidates for pulmonary thromboendarterectomy, the viable therapeutic alternatives include pulmonary artery angioplasty and pharmacological treatment. In these recommendations, the pathophysiological bases for the onset of CTEPH, such as acute pulmonary embolism and the clinical condition of the patient, will be discussed, as will the diagnostic algorithm to be followed and the therapeutic alternatives currently available.

Background: No studies have described and evaluated the association between hemodynamics, physical limitations and quality of life in patients with pulmonary hypertension (PH) without concomitant cardiovascular or respiratory disease., Objective: To describe the hemodynamic profile, quality of life and physical capacity of patients with PH from groups I and IV and to study the association between these outcomes., Methods: Cross-sectional study of patients with PH from clinical groups I and IV and functional classes II and III undergoing the following assessments: hemodynamics, exercise tolerance and quality of life., Results: This study assessed 20 patients with a mean age of 46.8 ± 14.3 years. They had pulmonary capillary wedge pressure of 10.5 ± 3.7 mm Hg, 6-minute walk distance test (6MWDT) of 463 ± 78 m, oxygen consumption at peak exercise of 12.9 ± 4.3 mLO2.kg-1.min-1 and scores of quality of life domains < 60%. There were associations between cardiac index (CI) and ventilatory equivalent for CO2 (r=-0.59, p <0.01), IC and ventilatory equivalent for oxygen (r=-0.49, p<0.05), right atrial pressure (RAP) and 'general health perception' domain (r=-0.61, p<0.01), RAP and 6MWTD (r=-0.49, p<0.05), pulmonary vascular resistance (PVR) and 'physical functioning' domain (r=-0.56, p<0.01), PVR and 6MWTD (r=-0.49, p<0.05) and PVR index and physical capacity (r=-0.51, p<0.01)., Conclusion: Patients with PH from groups I and IV and functional classes II and III exhibit a reduction in physical capacity and in the physical and mental components of quality of life. The hemodynamic variables CI, diastolic pulmonary arterial pressure, RAP, PVR and PVR index are associated with exercise tolerance and quality of life domains.

Background: Pulmonary arterial hypertension is a severe and progressive disease. Its early diagnosis is the greatest clinical challenge., Objective: To evaluate the presence and extension of the delayed myocardial contrast-enhanced cardiovascular magnetic resonance, as well as to verify if the percentage of the myocardial fibrosis mass is a severity predictor., Methods: Cross-sectional study with 30 patients with pulmonary arterial hypertension of groups I and IV, subjected to clinical, functional and hemodynamic evaluation, and to cardiac magnetic resonance., Results: The mean age of patients was 52 years old, with female predominance (77%). Among the patients, 53% had right ventricular failure at diagnosis, and 90% were in functional class II/III. The mean of the 6-minute walk test was 395 m. In hemodynamic study with right catheterism, the mean average pulmonary arterial pressure was 53.3 mmHg, of the cardiac index of 2.1 L/min.m(2), and median right atrial pressure was 13.5 mmHg. Delayed myocardial contrast-enhanced cardiovascular magnetic resonance was found in 28 patients. The mean fibrosis mass was 9.9 g and the median percentage of fibrosis mass was 6.17%. The presence of functional class IV, right ventricular failure at diagnosis, 6-minute walk test < 300 meters and right atrial pressure > 15 mmHg, with cardiac index < 2.0 L/min.m(2), there was a relevant association with the increased percentage of myocardial fibrosis., Conclusion: The percentage of the myocardial fibrosis mass indicates a non-invasive marker with promising perspectives in identifying patients with high risk factors for pulmonary hypertension.