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Cleveland Clinic Journal of Medicine Oct 2023Portopulmonary hypertension, ie, pulmonary arterial hypertension in a patient with portal hypertension, affects transplant eligibility and has a poor prognosis. The... (Review)
Review
Portopulmonary hypertension, ie, pulmonary arterial hypertension in a patient with portal hypertension, affects transplant eligibility and has a poor prognosis. The pathogenesis remains an area of active research, with various mechanisms proposed. Diagnosing it requires a detailed history, physical examination, laboratory tests, and echocardiographic evaluation, followed by a careful hemodynamic assessment.
Topics: Humans; Pulmonary Arterial Hypertension; Hypertension, Pulmonary; Hypertension, Portal; Hemodynamics; Echocardiography
PubMed: 37783493
DOI: 10.3949/ccjm.90a.23023 -
Seminars in Respiratory and Critical... Dec 2023The clinical presentation of pulmonary hypertension (PH) is nonspecific, resulting in significant delays in its detection. In the majority of cases, PH is a marker of...
The clinical presentation of pulmonary hypertension (PH) is nonspecific, resulting in significant delays in its detection. In the majority of cases, PH is a marker of the severity of other cardiopulmonary diseases. Differential diagnosis aimed at the early identification of patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) who do require specific and complex therapies is as important as PH detection itself. Despite all efforts aimed at the noninvasive assessment of pulmonary arterial pressure, the formal confirmation of PH still requires catheterization of the right heart and pulmonary artery. The current document will give an overview of strategies aimed at the early diagnosis of PAH and CTEPH, while avoiding their overdiagnosis. It is not intended to be a replica of the recently published European Society of Cardiology (ESC) and European Respiratory Society (ERS) Guidelines on Diagnosis and Treatment of Pulmonary Hypertension, freely available at the Web sites of both societies. While promoting guidelines' recommendations, including those on new definitions of PH, we will try to bring them closer to everyday clinical practice, benefiting from our personal experience in managing patients with suspected PH.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Artery; Risk Assessment; Chronic Disease
PubMed: 37487526
DOI: 10.1055/s-0043-1770116 -
Clinics in Chest Medicine Sep 2023The current approach for the management of pulmonary arterial hypertension (PAH) relies on data gathered from clinical trials and large registries. However, there is... (Review)
Review
The current approach for the management of pulmonary arterial hypertension (PAH) relies on data gathered from clinical trials and large registries. However, there is concern that minorities including Black, Indigenous, and People of Color are underrepresented in these trials and registries, making current data not generalizable to these groups of patients. Hence, it is important to discuss the significance of race/ethnicity and socioeconomic factors in patients with PAH. Here, we review the current knowledge on health care disparities in PAH. We also propose future steps in the global task of assuring justice and equality in access to pulmonary hypertension health care.
Topics: Humans; Pulmonary Arterial Hypertension; Healthcare Disparities; Hypertension, Pulmonary
PubMed: 37517834
DOI: 10.1016/j.ccm.2023.03.010 -
Clinics in Perinatology Mar 2024Pulmonary hypertension (PH) in preterm neonates has multifactorial pathogenesis with unique characteristics. Premature surfactant-deficient lungs are injured following... (Review)
Review
Pulmonary hypertension (PH) in preterm neonates has multifactorial pathogenesis with unique characteristics. Premature surfactant-deficient lungs are injured following exposure to positive pressure ventilation and high oxygen concentrations resulting in variable phenotypes of PH. The prevalence of early PH is variable and reported to be between 8% and 55% of extremely preterm infants. Disruption of the lung development and vascular signaling pathway could lead to abnormal pulmonary vascular transition. The management of early PH and the off-label use of selective pulmonary vasodilators continue to be controversial.
Topics: Infant; Infant, Newborn; Humans; Hypertension, Pulmonary; Lung; Infant, Extremely Premature; Positive-Pressure Respiration; Pulmonary Surfactants
PubMed: 38325940
DOI: 10.1016/j.clp.2023.11.005 -
Chest Mar 2024Pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with left-sided heart and lung diseases are most commonly easily discriminated and treated... (Review)
Review
Pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with left-sided heart and lung diseases are most commonly easily discriminated and treated accordingly. With the changing epidemiology of PAH, however, a growing proportion of patients at the time of diagnosis present with comorbidities of varying severity. In addition to classical PAH, two distinct phenotypes have emerged: a heart failure with preserved ejection fraction-like phenotype and a lung phenotype. Importantly, the evidence supporting the currently proposed treatment algorithm for PAH has been generated mainly from PAH trials in which patients with cardiopulmonary comorbidities have been underrepresented or excluded. As a consequence, the best therapeutic approach for patients with common PAH with cardiopulmonary comorbidities remains largely unknown and requires further investigation. The present article reviews the relevant literature on the topic and describes the authors' views on the current therapeutic approach for these patients.
Topics: Humans; Pulmonary Arterial Hypertension; Hypertension, Pulmonary; Familial Primary Pulmonary Hypertension; Heart Diseases; Heart Failure
PubMed: 38461018
DOI: 10.1016/j.chest.2023.08.023 -
Pneumologie (Stuttgart, Germany) Nov 2023Chronic thromboembolic pulmonary disease (CTEPD) is an important late complication of acute pulmonary embolism, in which the thrombi transform into fibrous tissue,...
Chronic thromboembolic pulmonary disease (CTEPD) is an important late complication of acute pulmonary embolism, in which the thrombi transform into fibrous tissue, become integrated into the vessel wall, and lead to chronic obstructions. CTEPD is differentiated into cases without pulmonary hypertension (PH), characterized by a mean pulmonary arterial pressure up to 20 mmHg and a form with PH. Then, it is still referred to as chronic thromboembolic pulmonary hypertension (CTEPH).When there is suspicion of CTEPH, initial diagnostic tests should include echocardiography and ventilation/perfusion scan to detect perfusion defects. Subsequently, referral to a CTEPH center is recommended, where further imaging diagnostics and right heart catheterization are performed to determine the appropriate treatment.Currently, three treatment modalities are available. The treatment of choice is pulmonary endarterectomy (PEA). For non-operable patients or patients with residual PH after PEA, PH-targeted medical therapy, and the interventional procedure of balloon pulmonary angioplasty (BPA) are available. Increasingly, PEA, BPA, and pharmacological therapy are combined in multimodal concepts.Patients require post-treatment follow-up, preferably at (CTE)PH centers. These centers are required to perform a minimum number of PEA surgeries (50/year) and BPA interventions (100/year).
Topics: Humans; Hypertension, Pulmonary; Chronic Disease; Pulmonary Embolism; Lung; Pulmonary Artery
PubMed: 37963483
DOI: 10.1055/a-2145-4807 -
Seminars in Perinatology Oct 2023Bronchopulmonary dysplasia (BPD) is the leading cause of chronic lung disease in infants and the commonest complication of prematurity. Advances in respiratory and... (Review)
Review
Bronchopulmonary dysplasia (BPD) is the leading cause of chronic lung disease in infants and the commonest complication of prematurity. Advances in respiratory and overall neonatal care have increased the survival of extremely low gestational age newborns, leading to the continued high incidence of BPD. Pulmonary hypertension (PH) represents the severe form of the pulmonary vascular disease associated with BPD, and affects almost one-third of infants with moderate to severe BPD. PH responds suboptimally to pulmonary vasodilators and increases morbidity and mortality in BPD infants. An up-to-date knowledge of the pathogenesis, pathophysiology, diagnosis, treatment, and outcomes of BPD-PH can be helpful to develop meaningful and novel strategies to improve the outcomes of infants with this disorder. Therefore, our multidisciplinary team has attempted to thoroughly review and summarize the latest advances in BPD-PH in preventing and managing this morbid lung disorder of preterm infants.
Topics: Infant; Infant, Newborn; Humans; Bronchopulmonary Dysplasia; Infant, Premature; Hypertension, Pulmonary; Lung; Gestational Age
PubMed: 37783579
DOI: 10.1016/j.semperi.2023.151817 -
Journal of the American College of... Nov 2023Group 1 pulmonary arterial hypertension (PAH) is a progressive fatal condition characterized by right ventricular (RV) failure with worse outcomes in connective tissue...
BACKGROUND
Group 1 pulmonary arterial hypertension (PAH) is a progressive fatal condition characterized by right ventricular (RV) failure with worse outcomes in connective tissue disease (CTD). Obstructive sleep apnea and sleep-related hypoxia may contribute to RV dysfunction, though the relationship remains unclear.
OBJECTIVES
The aim of this study was to prospectively evaluate the association of the apnea-hypopnea index (AHI) and sleep-related hypoxia with RV function and survival.
METHODS
Pulmonary Vascular Disease Phenomics (National Heart, Lung, and Blood Institute) cohort participants (patients with group 1 PAH, comparators, and healthy control participants) with sleep studies were included. Multimodal RV functional measures were examined in association with AHI and percentage of recording time with oxygen saturation <90% (T90) per 10-unit increment. Linear models, adjusted for demographics, oxygen, diffusing capacity of the lungs for carbon monoxide, pulmonary hypertension medications, assessed AHI and T90, and RV measures. Log-rank test/Cox proportional hazards models adjusted for demographics, oxygen, and positive airway pressure were constructed for transplantation-free survival analyses.
RESULTS
Analysis included 186 participants with group 1 PAH with a mean age of 52.6 ± 14.1 years; 71.5% were women, 80.8% were Caucasian, and there were 43 events (transplantation or death). AHI and T90 were associated with decreased RV ejection fraction (on magnetic resonance imaging), by 2.18% (-2.18; 95% CI: -4.00 to -0.36; P = 0.019) and 0.93% (-0.93; 95% CI: -1.47 to -0.40; P < 0.001), respectively. T90 was associated with increased RV systolic pressure (on echocardiography), by 2.52 mm Hg (2.52; 95% CI: 1.61 to 3.43; P < 0.001); increased mean pulmonary artery pressure (on right heart catheterization), by 0.27 mm Hg (0.27; 95% CI: 0.05 to 0.49; P = 0.019); and RV hypertrophy (on electrocardiography), 1.24 mm (1.24; 95% CI: 1.10 to 1.40; P < 0.001). T90, but not AHI, was associated with a 17% increased 5-year risk for transplantation or death (HR: 1.17; 95% CI: 1.07 to 1.28). In non-CTD-associated PAH, T90 was associated with a 21% increased risk for transplantation or death (HR: 1.21; 95% CI: 1.08 to 1.34). In CTD-associated PAH, T90 was associated with RV dysfunction, but not death or transplantation.
CONCLUSIONS
Sleep-related hypoxia was more strongly associated than AHI with measures of RV dysfunction, death, or transplantation overall and in group 1 non-CTD-associated PAH but only with RV dysfunction in CTD-associated PAH. (Pulmonary Vascular Disease Phenomics Program [PVDOMICS]; NCT02980887).
Topics: Adult; Aged; Female; Humans; Male; Middle Aged; Heart Failure; Hypertension, Pulmonary; Hypoxia; Oxygen; Pulmonary Arterial Hypertension; Sleep; Ventricular Dysfunction, Right; Ventricular Function, Right
PubMed: 37968017
DOI: 10.1016/j.jacc.2023.09.806 -
Current Problems in Cardiology Aug 2023Pulmonary hypertension is one of the difficult situations to treat. Complex pathophysiology, association of the multiple comorbidities make clinical scenario... (Review)
Review
Pulmonary hypertension is one of the difficult situations to treat. Complex pathophysiology, association of the multiple comorbidities make clinical scenario challenging. Recently it is being shown that patients who had recovered from coronavirus disease infection, are at risk of developing pulmonary hypertension. Studies on animals have been going on to find out newer treatment options. There are recent advancements in the treatment of pulmonary hypertension. Role of anticoagulation, recombinant fusion proteins, stem cell therapy are emerging as therapeutic options for affected patients. SGLT2 inhibitors have potential to have beneficial effects on pulmonary hypertension. Apart from the medical managements, advanced interventions are also getting popular. In this review article, the authors have discussed pathophysiology, recent advancement of treatments including coronavirus disease patients, and future aspect of managing pulmonary hypertension. We have highlighted treatment options for patients with sleep apnea, interstitial lung disease to discuss the challenges and possible options to manage those patients.
Topics: Animals; Humans; Hypertension, Pulmonary; Comorbidity
PubMed: 35500734
DOI: 10.1016/j.cpcardiol.2022.101236 -
Genetics of Pulmonary Pressure and Right Ventricle Stress Identify Diabetes as a Causal Risk Factor.Journal of the American Heart... Aug 2023Background Epidemiologic studies have identified risk factors associated with pulmonary hypertension and right heart failure, but causative drivers of pulmonary...
Background Epidemiologic studies have identified risk factors associated with pulmonary hypertension and right heart failure, but causative drivers of pulmonary hypertension and right heart adaptation are not well known. We sought to leverage unbiased genetic approaches to determine clinical conditions that share genetic architecture with pulmonary pressure and right ventricular dysfunction. Methods and Results We leveraged Vanderbilt University's deidentified electronic health records and DNA biobank to identify 14 861 subjects of European ancestry who underwent at least 1 echocardiogram with available estimates of pulmonary pressure and right ventricular function. Analyses of the study were performed between 2020 and 2022. The final analytical sample included 14 861 participants (mean [SD] age, 63 [15] years and mean [SD] body mass index, 29 [7] kg/m). An unbiased phenome-wide association study identified diabetes as the most statistically significant clinical , () code associated with polygenic risk for increased pulmonary pressure. We validated this finding further by finding significant associations between genetic risk for diabetes and a related condition, obesity, with pulmonary pressure estimate. We then used 2-sample univariable Mendelian randomization and multivariable Mendelian randomization to show that diabetes, but not obesity, was independently associated with genetic risk for increased pulmonary pressure and decreased right ventricle load stress. Conclusions Our findings show that genetic risk for diabetes is the only significant independent causative driver of genetic risk for increased pulmonary pressure and decreased right ventricle load stress. These findings suggest that therapies targeting genetic risk for diabetes may also potentially be beneficial in treating pulmonary hypertension and right heart dysfunction.
Topics: Humans; Middle Aged; Diabetes Mellitus, Type 2; Genome-Wide Association Study; Heart Ventricles; Hypertension, Pulmonary; Obesity; Risk Factors; Aged
PubMed: 37522172
DOI: 10.1161/JAHA.122.029190