pulmonary hypertension

Objective: To investigate in an animal model of Pulmonary Hypertension (PH) by monocrotaline whether a lower exercise intensity, which has lower potential to provoke dyspnea symptoms, could prevent the increase the right ventricle pressure and the decrease in respiratory compliance. Setting: A research laboratory. ANIMALS: twenty-one Wistar rats were randomized to the groups: Control (CO; saline solution); PH-sedentary; PH-low and PH-moderate intensity of exercise training (ET). Interventions: They received a single saline or monocrotaline subcutaneous injection (50 mg/kg). The exercise program was performed during 3-weeks. Main Outcome Measures: Rats were evaluated by their morphometric and hemodynamic changes and by the respiratory mechanic responses induced by the exercise protocols. Results: Both protocols of ET significantly (p < 0.05) attenuated the increase in the right ventricular systolic pressure. However, the lower intensity was more effective to prevent the impairment in the respiratory and quasi-static compliance. Conclusion: Collectively, our results showed for the first time the benefits of ET to the respiratory system mechanics. We also demonstrated that intensity is crucial in PH, probably due to the difficulty to match VO2 capacity and O2 demand during exercise. The improvement in quasi-static compliance not only might improve the ability to breathe, and capture oxygen, but also welfare.

Idiopathic Pulmonary Fibrosis (IPF) is a chronic and progressive disease without treatment that leads to death. Therefore, to control its progression to pulmonary hypertension is still a challenge. Moreover, there is no study that has investigated the Renin-Angiotensin System in patients with IPF. Objective: Verify the plasma concentrations of Angiotensin I, Angiotensin II (AngII), Angiotensin-(1-7) [Ang- (1-7)] and Alamandine in patients with IPF. Methods: Ten IPF patients, with or without PH, were included, and ten controls matched by sex and age. Quantitative plasma peptide concentrations (PPC) were expressed as mean and standard deviation or median and interquartile range. The Student Newman-Keuls t test was used for parametric data, Mann-Whitney for nonparametric data and, to compare proportions, the Fisher exact test was performed. The associations between clinical variables and the PPC were evaluated by Pearson or Spearman correlation coefficients. A p ≤ 0.05 was considered statistically significant. Results: The Alamandine plasma concentration was significantly (365%) lower in the IPF group and positively associated (r = 0.876) with pulmonary artery pressure (PAP). In addition, only in control group, the forced expiratory volume (FEV1%) was positively associated (p = 0.758) with Ang-(1-7). Conclusion: This study showed, for the first time, that there is a decrease in Alamandine participation in patients with IPF. The ACE-AngII-AT1 axis may be more active in this disease. In addition, our results suggest that Alamandine might be compensating the increase in PAP, as well as the Ang-(1-7) is improving the forced expiratory volume.

Antiphospholipid syndrome may present in various ways from cutaneous manifestation, obstetric complications, neurological manifestation, and cardiac manifestation to renal involvement. There are many cardiac complication of anti-phospholipid syndrome, among them are valvular dysfunction, pulmonary hypertension, myocardial infarction, intracardiac thrombi, and ventricular dysfunction [1]. The most common cardiac manifestation is valvular abnormalities ranging from 11.6-32% [2-5].

Introduction: Pulmonary hypertension (PH) is prevalent in hemodialysis (HD). In the general population, more women than men have PH due to left ventricular (LV) disease with preserved ejection fraction (EF). Little is known about the gender-specific prevalence of PH and associated LV abnormalities in patients with end stage renal disease (ESRD) on HD. Our aim was to evaluate gender differences and LV structural and functional changes in PH among ESRD patients on HD. Methods: Ninety-four patients (ages 23-77 years) underwent echocardiography after HD. Patients were divided based on estimated pulmonary artery systolic pressure (PASP) (Group A PASP < 40 mm Hg, Group B PASP ≥ 40 mm Hg). LV measurements included LV mass, LV internal dimensions, and LV ejection fraction (EF). LV diastolic function (LVDF) was assessed from mitral inflow deceleration time (DT) and E/A ratio. Results: Fifty-five patients (59%) had PH, including 32 of 49 men (65%) and 23 of 45 women (51%). LVEF was lower in Group B (46.4 ± 17.6 vs. 62.4 ± 14.4%, p < 0.001). Men with PH had higher LVIDd, cm (5.52 ± 0.89 vs 4.78 ± 0.75, p < 0.001), LVIDs, cm (3.75 ± 0.94 vs 3.14 ± 0.91, p = 0.03) LV mass, g (236 ± 74vs 189 ± 56, p = 0.02) and lower LVEF (40.0 ± 16.7 vs 52.0 ± 15.6, p = 0.008) than women. Conclusion: Patients on HD have a high prevalence of PH. PH was not associated with clear LV structural changes. There was a depression in LV systolic function without changes in LVDF. PH patients were more often men with hypertrophied LV with depressed LV systolic function. 

Background: Macitentan significantly improves pulmonary hemodynamics and survival in patients with primary pulmonary hypertension (PPH). Its beneficial effect, however, may be blunted due to the adverse impacts such as anemia and peripheral edema. Pulmonary arterial hypertension (PAH) is a significant consequence of congenital heart disease (CHD). Its presence and severity are associated with increased morbidity and mortality. We tried to evaluate that the effectiveness of the macitentan in patients with late-onset pulmonary hypertension after atrial septal defect operation in our center. Methods: The effect of a single dose of macitentan (10 mg) on pulmonary hemodynamics, functional capacity was examined in four patients with late-onset pulmonary hypertension after atrial septal defect operation. Results: The macitentan significantly improved mean pulmonary artery pressure (MPAP), cardiac output (CO), tricuspid annular plane systolic excursion (TAPSE), right ventricle systolic wave(RVS’), 6-minute walking test and NT-proBNP levels compared with before treatment. Conclusions: Macitentan can be used in patients with late-onset pulmonary hypertension after shunt operation especially atrial septal defect.

The case is that of 83 year-old African American man with hypertension, hepatitis C induced decompensated cirrhosis with ascites, end-stage renal disease (ESRD) on hemodialysis, fluid overload with peripheral edema and chronic hypotension. The patient was referred to the dialysis access center of Pittsburgh, PA for evaluation of his prolonged bleeding from the left upper arm brachial-basilic arterial-venous fistula (BBAVF).

Objectives: We describe the clinical course and management of two patients with post-capillary pulmonary hypertension due to diffuse pulmonary venous baffle calcification decades post-Mustard procedure.Background: From the late 1950s to the early 1990s, the definitive surgical repair for children with D-transposition of the great vessels (D-TGA) was an atrial switch procedure (either Senning or Mustard operation) which utilizes atrial-level baffles to shunt pulmonary venous blood to the morphologic right (systemic) ventricle and caval blood to the morphologic left (sub-pulmonary) ventricle. From a hemodynamic standpoint, baffle leaks and stenoses as well as precapillary pulmonary hypertension have all been described as both early and late complications [1]. Recently, delayed post-capillary pulmonary hypertension (in the absence of discrete baffle obstruction) decades post-atrial switch has also been described [2]. The underlying pathophysiology for this postcapillary pulmonary hypertension is unclear but is theorized to involve impaired diastology referable to the pulmonary venous baffle. Methods/Results: Using hemodynamic and imaging data, we describe two patients with extensive pulmonary venous baffle calcification and resultant pulmonary hypertension from the so-called “stiff left atrial (LA) syndrome.” This problem can be difficult to treat medically and is not amenable to catheter-based interventions. We hypothesize that this is an underlying mechanism for pulmonary hypertension in at least some post-Mustard and Senning patients. Conclusion: We describe the treatments and clinical course for each of these patients, and in particular describe how the surgical revision of the pulmonary venous baffle in one case led to the complete resolution of symptoms.

In pregnancy, the incidence of pulmonary embolism (PE) is increased fivefold when compared to nonpregnant women of the same age, and PE is one of the leading causes of death during pregnancy.However, the diagnosis of PE among pregnant women is complicated by concerns regarding radiation exposure. Systemic lupus erythematosus (SLE) is an autoimmune disorder with a wide array of presentations and a predilection to affect women of certain ethnic backgrounds. The hallmark of the disease is multisystem involvement, dispersed in time and severity. Usual pulmonary involvement includes pleuritis, pleural effusions, pneumonitis, shrinking lung syndrome, pulmonary hypertension, and alveolar hemorrhage. Pulmonary embolism (PE) is a relatively unusual presentation of SLE. We report the case of a 20-year-old primi at 21 weeks gestation with an acute PE with central chest pain and shortness of breath. The absence of overt signs and symptoms and traditional risk factors prompted a fragmentary workup. This led to the detection of antibodies sensitive for SLE, in the absence of overt signs and symptoms. We revive the concept of latent lupus, a condition construed as early lupus. We firmly suspect direct causation between SLE and PE. Further studies are needed to establish pathogenesis to facilitate early diagnosis and prevent morbidity and mortality from PE. Due to persistent hypotension, thrombolytic therapy with streptokinase was administered and the clinical and hemodynamic response was excellent, with no maternal or fetal hemorrhagic complications. The clinical presentation of pulmonary embolism is sometimes camouflaged by the physiological changes that occur in pregnancy and diagnosis is often delayed by a reluctance to expose the fetus to ionizing radiation. 

Background: Heart Failure with Preserved Ejection Fraction (HFPEF) accounts for more than half of the cases of heart failure.Long regarded as an abnormality of left ventricular diastolic function, recent studies using longitudinal strain (two-dimensional speckle tracking mode) have suggested that left ventricular longitudinal systolic function is altered in HFPEF.Despite these interesting pathophysiological perspectives, the data in the literature on the prognostic value of the alteration of longitudinal strain are controversial. Given these conflicting results, it is difficult to confirm the magnitude and prevalence of impaired LV longitudinal systolic function in patients with HFPEF and its prognostic relevance. Purposes: This work aims to study the prognostic value of Global the left ventricle’s Global Longitudinal Strain (GLS) Algerian cohort of patients with HFPEF. Patients and methods: We conducted a monocentric prospective observational study from April 2018 to April 2020, with a minimum follow-up of 1 year for each recruited patient. We included patients over the age of 18 referred to the echocardiography laboratory for chronic or acute HFpEF, defined according to the criteria of ESC 2016. 153 consecutive patients underwent clinical examination, biological tests, and echocardiography with measurement of GLS at rest, in addition to routine management by the attending physicians.Results: 153 patients were collected. The average age of our patients is 73 +/- 11 years ranging from 42 to 91 years old. The female population is predominant with a rate of 67%. Comorbidities are predominant mainly by arterial hypertension (86%) and diabetes (64%), with a history of atrial fibrillation (46%).63% of patients have impaired GLS (< 16%). Contrary to our hypothesis, GLS was not shown to be a powerful predictor of cardiovascular events in HFPEF patients either in dichotomous analysis (OR = 0.79; p = 0.64) or in continuous analysis (OR = 0.97; p = 0.69).We were able to identify that congestive venous signs, anemia, and pulmonary hypertension, are the main independent prognostic factors in our Algerian population study. Conclusion: We were unable to demonstrate the prognostic role of mpaired GLS in our population of patients with HFPEF.

Chronic thromboembolic pulmonary hypertension is a notoriously underdiagnosed cause of severe pulmonary hypertension. It is a form of precapillary pulmonary hypertension (PH) that results from intraluminal thrombus organization and fibrous formation which ultimately results in the complete obliteration of pulmonary arteries, resulting in increased pulmonary vascular resistance which leads to the development of pulmonary hypertension and as a result right heart failure. The mechanism involves the narrowing of the pulmonary artery which increases blood pressure within the lungs and impairs blood flow which increases the workload of the right side of the heart ultimately causing right heart failure. Pulmonary hypertension can also cause arrhythmias, blood clots, and bleeding in the lungs. Even though CTEPH is a deadly condition, among all forms of pulmonary hypertension, CTEPH is the only curable form. Echocardiography is the initial assessment tool for suspected PH. A right heart catheterization may be performed to confirm the presence of pulmonary hypertension. Confirmation of CTEPH requires a V/Q scan. Although ventilation/perfusion scintigraphy has a major role in the evaluation of patients with suspected CTEPH, nowadays CTA chest is being used widely as it produces much better-quality images compared to V/Q scan. Without treatment, the prognosis is very poor. Out of three treatment modalities such as; pulmonary endarterectomy (PEA) surgery, balloon pulmonary angioplasty (BPA), and medical therapy, surgery is the gold standard. The physician must be familiar with the disease entity, early diagnosis, and appropriate treatment to improve survival. Here we present a literature review on this topic.

The prevalence of intra-uterine dysfunction of ductus arteriosus is unknown and the clinical consequences are poorly understood. We report a case of prenatal diagnosis of premature closure of the ductus arteriosus due to maternal intake of metamizole during pregnancy. Fetal echocardiography at 37 weeks of gestation revealed a right ventricular hypertrophy and suspected stenosis of the tricuspid valve. A cesarean section led to an excellent neonatal outcome. The aim of this report is to show echocardiographic abnormalities and outcomes of this rare phenomenom.

Pulmonary hypertension (PH) in sickle cell disease (SCD) is associated with a mortality rate of 37%. There is an upregulation of adhesion molecules which leads to the expression of endothelin-1, a potent vasoconstrictor. A prospective, descriptive study was done to determine the safety and efficacy of macitentan in patients with SCD and PH. Continuous variables were reported as mean ± SEM or percentage where appropriate. We screened 13 patients and recruited five. All five patients were adults. Data were analyzed as appropriate by student t - test. Statistical significance was assumed at p < 0.05. Baseline pulmonary hemodynamics obtained by right heart catheterization and systemic hemodynamics were (± SEM): mean pulmonary artery pressure (MPAP) 32 ± 8 mmHg, right atrial pressure (RAP) 9 ± 4 mmHg, pulmonary vascular resistance (PVR) 257 dynes-sec/cm5 and CI 3·7 ± 0.39 l/m2. Of all parameters, only PVR and 6-min walk distance changed significantly. For the group, MPAP decreased by 15.6%, PVR by 22.5% and RAP by 25.5%. The 6-minute walk distance increased over sixteen weeks except in Patient 4 who had a 3% decrease. The mean walk distance increased in the total distance, from 464 ± 158 meters to 477 ± 190 meters (p .123). In four patients, the adverse events were mild to moderate and did not lead to study drug discontinuation. Significant improvement in pulmonary hemodynamics and exercise capacity in patients with SCD-related pulmonary arterial hypertension. We found that macitentan was safe and well tolerated.

Background: Idiopathic intracranial hypertension (IIH or pseudotumor cerebri) has two major morbidities: papilledema with visual loss and disabling headache. Intracranial Venous Hypertension (IVH) is a fundamental mechanism of IIH. Although traditionally considered limiting to the central nervous system, evidence suggests IIH as a systemic disease associated with cardiorespiratory disorders, which has been far less comprehended. Case Report: A 60-year-old female with Chronic Obstructive Pulmonary Disease (COPD) was admitted for dyspnea and developed a coma with a pH of 7.01 and pCO2 of 158 mmHg. She was intubated and had persistent nuchal rigidity, a brief myoclonus episode with a negative electroencephalogram, and negative CT head studies. A Lumbar Puncture (LP) revealed elevated opening pressure (35 cmH2O) with normal Cerebral Spinal Fluid (CSF) studies. Her nuchal rigidity improved after the removal of 40 mL CSF. The ophthalmology examination the next day after her the large volume LP didn’t show visual loss or papilledema. The patient improved clinically and was extubated two days later. Her echocardiogram showed a dilated right ventricle with pulmonary hypertension. The patient was discharged home.Discussion: IIH is different from hypercapnic encephalopathy and characterized by increased intracranial pressure with papilledema, vision loss, and debilitating headache. Hypercapnia-induced increased intracranial venous flow and pulmonary hypertension-caused elevated central venous pressure with consequent outflow resistance lead to IVH. In hypercapnic encephalopathy, the presentation is mostly cognitive changes. In this case, nuchal rigidity with a negative CT head scan triggered the investigation of IIH. Conclusion: A deep understanding of the relationship between COPD and IIH is vital. There is insufficient evidence to recommend routine eye examinations in COPD patients for papilledema and to conduct a pulmonary function test for a newly diagnosed IIH patient. However, we highly suggest a timely ophthalmology exam prior to performing an LP in COPD patients with suspecting IIH to avoid unnecessary procedures and meanwhile improve clinical outcomes.

Rationale: Pulmonary Arterial Hypertension (PAH) is a progressive vascular remodeling disease with elevated pulmonary vascular resistance that is lethal. While therapeutic progress was recently made with endothelin, nitric oxide, and prostacyclin pathway-based therapy for the treatment of PAH, the disease is currently incurable with a high cost of morbidity and mortality. Sotatercept, a new activin receptor IIA-Fc fusion protein, may prove to be a game-changer as a therapeutic agent for the treatment of PAH by regulating the growth factor signaling aberration of PAH.Methods: It is a narrative review of evidence for the drug Sotatercept for Group 1 PAH from a systematic literature search for clinical trials, mechanism studies, and regulatory data up to 2024. Pivotal clinical trials such as PULSAR, SPECTRA, STELLAR, and ZENITH were evaluated for efficacy, safety, and comparative results.Results: Sotatercept is a TGF-β family member ligand trap that rebalances activin/BMP signaling to target vascular remodeling. Clinically, striking effects were shown with exercise (40.8 m improvement in STELLAR 6MWD), pulmonary hemodynamics (PVR reduction of 146-240 dyn·s·cm-5 in PULSAR), as well as clinical measures (76% reduction of composite morbidity/mortality through ZENITH). On the background with an acceptable drug safety profile of predominantly hematologic effects, as well as injection site reaction, benefits were achieved. Comparison with analyses implies at least similar, if superior in some dimensions, efficacy of current PAH therapies.Conclusion: Sotatercept is a new therapeutic option for PAH as the first drug to act on the activin/BMP pathway. With its strong effect on several clinically relevant end points, it is a “fourth pillar” of PAH therapy. Clinical trials will determine its place in the algorithm, ascertain other combinations, and potentially identify its utility for other types of pulmonary hypertension.