scholarly journals Atrial arrhythmias are associated with increased mortality in pulmonary arterial hypertension

2018 ◽  
Vol 8 (3) ◽  
pp. 204589401879031 ◽  
Author(s):  
Benjamin Smith ◽  
Michael V. Genuardi ◽  
Agnes Koczo ◽  
Richard H. Zou ◽  
Floyd W. Thoma ◽  
...  
Keyword(s):  
Heart Rate ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Left Ventricular ◽  
Sensitivity Analyses ◽  
Right Atrial ◽  
Left Ventricular Ejection ◽  
Atrial Arrhythmias ◽  
Pulmonary Arterial ◽  
Time Varying Covariates

Pulmonary arterial hypertension (PAH) is a deadly vascular disease, characterized by increased pulmonary arterial pressures and right heart failure. Considering prior non-US studies of atrial arrhythmias in PAH, this retrospective, regional multi-center US study sought to define more completely the risk factors and impact of paroxysmal and non-paroxysmal forms of atrial fibrillation and flutter (AF/AFL) on mortality in this disease. We identified patients seen between 2010 and 2014 at UPMC (Pittsburgh) hospitals with hemodynamic and clinical criteria for PAH or chronic thromboembolic pulmonary hypertension (CTEPH) and determined those meeting electrocardiographic criteria for AF/AFL. We used Cox proportional hazards regression with time-varying covariates to analyze the association between AF/AFL occurrence and survival with adjustments for potential cofounders and hemodynamic severity. Of 297 patients with PAH/CTEPH, 79 (26.5%) suffered from AF/AFL at some point. AF/AFL was first identified after PAH diagnosis in 42 (53.2%), identified prior to PAH diagnosis in 27 (34.2%), and had unclear timing in the remainder. AF/AFL patients were older, more often male, had lower left ventricular ejection fractions, and greater left atrial volume indices and right atrial areas than patients without AF/AFL. AF/AFL (whether diagnosed before or after PAH) was associated with a 3.81-fold increase in the hazard of death (95% CI 2.64–5.52, p < 0.001). This finding was consistent with multivariable adjustment of hemodynamic, cardiac structural, and heart rate indices as well as in sensitivity analyses of patients with paroxysmal versus non-paroxysmal arrhythmias. In these PAH/CTEPH patients, presence of AF/AFL significantly increased mortality risk. Mortality remained elevated in the absence of a high burden of uncontrolled or persistent arrhythmias, thus suggesting additional etiologies beyond rapid heart rate as an explanation. Future studies are warranted to confirm this observation and interrogate whether other therapies beyond rate and rhythm control are necessary to mitigate this risk.

Prevalence and Predictors of Pulmonary Arterial Hypertension in Hemoglobin E/ β-Thalassemia Patients.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2015-2015 ◽  
Author(s):  
Suporn Chuncharunee ◽  
Vichai Atichartakarn ◽  
Napaporn Archararit ◽  
Umaporn Udomsubpayakul ◽  
Atiporn Ingsathit ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Univariate Analysis ◽  
Cellular Adhesion ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Low Grade ◽  
Ventricular Ejection Fraction ◽  
Hemoglobin E ◽  
Pulmonary Arterial

Abstract Abstract 2015 Poster Board I-1037 Introduction: Pulmonary arterial hypertension (PAH) associated with thalassemia (Thal) hemoglobinopathy is now an accepted clinical entity. Most of the reports are in sickle cell disease and splenectomized β-Thal patients. Once manifested, it connotes poor prognosis. We herein present its prevalence and predictors in hemoglobin E/β-Thal (E/β-Thal) patients. Patients and Methods: One hundred and ten clinically stable E/β-Thal outpatients, on no medication aside from folic acid and who received no blood transfusion in the preceding 4 weeks were studied. All gave written informed consent, and study protocol was approved by the institution ethics committee on studies in humans (#0774/2548). Echocardiogram was used to estimate systolic PA pressure (SPAP). PAH was defined as an estimated SPAP ≥36 mmHg. Clinical features and laboratory data were stratified according to the presence or absence of PAH, and statistical analysis was done by STATA version 10 (Stata Corp, Texas), considering a P value <0.05 as statistically significant. Predictors of PAH were considered in univariate analysis. Results: There were 110 patients, 56 of whom were female and 61 were asplenic. PAH was present in 41 patients (37.3%), all of whom had normal left ventricular ejection fraction. There was no gender difference between the 2 groups (p=0.055). Selected statistically significant results are shown in the table. Conclusions: Prevalence of PAH in E/β-Thal patients is 37.3% without gender preponderance. Predictors are asplenia, more severe hemolysis, higher number of circulating (activated) platelets and nucleated RBCs, increased chronic low grade inflammation and increased cellular adhesion between blood and endothelial cells. These changes could facilitate development of thrombotic pulmonary arteriopathy, the underlying basis of PAH. Serum NT pro BNP assay can be utilized as a predictor or a screener of PAH in these patients. Disclosures: No relevant conflicts of interest to declare.

Abstract 20086: Influence of Central Apneas and Chemoreflex Activation on Heart Failure Related Pulmonary Arterial Hypertension

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Valentina Raglianti ◽  
Alberto Giannoni ◽  
Annamaria Del Franco ◽  
Gianluca Mirizzi ◽  
Alberto Aimo ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Passive Component ◽  
Ventricular Ejection Fraction ◽  
Pulmonary Vasoconstriction ◽  
Pulmonary Arterial ◽  
Central Apneas

Introduction: Pulmonary arterial hypertension (PAH) is an acknowledged independent prognostic factor in patients with heart failure (HF). Beyond a “passive” component due to the increased left ventricular pressure, an “active” component due to pulmonary vascular reactivity is frequently observed. However, the mechanism behind pulmonary vasoconstriction is not fully understood. Hypothesis: We hypothesized that central apneas (Cheyne-Stokes respiration [[Unable to Display Character: &#8211;]] CSR) through chemoreflex stimulation may contribute to PAH in HF. Methods: we studied 56 HF patients (left ventricular ejection fraction <50%), on stable optimal pharmacological treatment, without increased left ventriculare pressure (excluding patients with severe mitral disease and severe diastolic dysfunction). All patients underwent echocardiographic and neurohormonal assessment, 24-hour cardiorespiratory screening for CSR (patients with obstructive events were excluded) and chemoreflex test for hypoxic (HVR) and hypercapnic (HCVR) ventilatory responses (by rebreathing technique) . Results: Thirteen patients (23%) showed CSR, as defined by a 24 hour AHI > of 15. HF patients with CSR compared with patients with normal breathing, presented higher systolic arterial pulmonary pressure (SPAP: 40.1±7.6 vs 33.1±5.9, p<0.01) at echocardiography, despite similar systolic and diastolic function (data not shown). Furthermore, patients with central apneas also presented with enhanced HVR (median 0.78, interquartile range -IR 0.48-1.22 vs 0.43, IR 0.19-0.69 L/min/%, p<0.05) and HCVR (1.18, IR 0.98-1.35 vs 0.75, IR 0.51-0.95 L/min/mmHg, p<0.01) as well as increased plasma norepinephrine levels (546, IR 371-732 vs 393, IR 229-538 pg/mL, p<0.05). SPAP was indeed correlated with AHI (Spearman’s Rho, R=0.44, p<0.01), HCVR (R=0.48, p<0.001), HVR (R=0.33, p<0.05) and norepinephrine levels (R=0.31, p<0.05). Conclusions: In patients with systolic HF, the presence of diurnal-nocturnal CSR, likely via recurrent hypoxia and hypercapnia cycles, may determine a chemoreflex mediated adrenergic discharge and a consequent pulmonary vasoconstriction, responsible of the undesirable increase in pulmonary arterial pressure.

scholarly journals A Hospital Based Study of Pulmonary Arterial Hypertension in Various Stages of Chronic Kidney Disease and Associated Risk Factors among Patients Attending a Tertiary Care Hospital in Ujjain, Madhya Pradesh

2021 ◽  
Vol 8 (20) ◽  
pp. 1500-1504
Author(s):  
Rajesh Deshpande ◽  
Amit Kumar Yadav ◽  
Vipin Porwal
Keyword(s):  
Chronic Kidney Disease ◽  
Pulmonary Arterial Hypertension ◽  
Kidney Disease ◽  
Arterial Hypertension ◽  
Ventricular Hypertrophy ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Corticomedullary Differentiation ◽  
Pulmonary Arterial

BACKGROUND Multiple mechanisms have been identified contributing to pulmonary arterial hypertension (PAH) in chronic kidney disease (CKD) patients and it is one of the important sequelae of CKD and needs early detection. We wanted to study PAH in various stages of CKD and its association with renal and cardiovascular parameters. METHODS This was an observational study. PAH was diagnosed if mean pulmonary artery pressure (MPAP) was ≥ 25 mmHg using 2D - Doppler echocardiography in 96 CKD patients. Staging of CKD was done as per Kidney Disease Improving Global Outcomes (KDIGO) stages 1 - 5. Age, gender, diabetes, hypertension, stages of CKD, corticomedullary differentiation (CMD), estimated glomerular filtration rate (EGFR), urinary albumin creatinine ratio (UACR), left ventricular ejection fraction (LVEF) and left ventricular hypertrophy (LVH) were included as risk factors. Data was analysed by calculating percentage, mean, standard deviation, chi square and t test. P value < 0.05 was taken as statistically significant. RESULTS PAH was detected in 37 (38.5 %) of CKD patients. Prevalence of PAH increased with stages of CKD being highest (59 %) in stage 5 and this was found to be statistically significant (P = 0.04). PAH was detected earliest in stage 2 (23.5 %). Lower mean eGFR ml / min / 1.733m2 (24.43 ± 17.8 vs 40.98 ± 25.7, P = 0.001) altered corticomedullary differentiation (50.9 % vs 20.5 % p = 0.003), reduced LVEF (81 % vs 26.7 % P = 0.000) and LVH (65 % vs 19.6 %, P = 0.000) were significantly associated with PAH in CKD patients. CONCLUSIONS PAH in CKD patients increases with CKD stages. Onset of PAH in CKD patients may be earlier and significantly associated with left ventricular dysfunction. KEYWORDS Pulmonary Arterial Hypertension, Chronic Kidney Disease, Left Ventricular Ejection Fraction, Left Ventricular Hypertrophy

scholarly journals Cardiac sympathetic dysfunction in pulmonary arterial hypertension: lesson from left-sided heart failure

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401986862 ◽  
Author(s):  
Valentina Mercurio ◽  
Teresa Pellegrino ◽  
Giorgio Bosso ◽  
Giacomo Campi ◽  
Paolo Parrella ◽  
...  
Keyword(s):  
Heart Failure ◽  
Nervous System ◽  
Pulmonary Arterial Hypertension ◽  
Left Ventricular Ejection Fraction ◽  
Arterial Hypertension ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Pulmonary Arterial ◽  
Sympathetic Nervous

Sympathetic nervous system hyperactivity has a well-recognized role in the pathophysiology of heart failure with reduced left ventricular ejection fraction. Alterations in sympathetic nervous system have been related to the pathophysiology of pulmonary arterial hypertension, but it is unclear whether cardiac sympathetic nervous system is impaired and how sympathetic dysfunction correlates with hemodynamics and clinical status in pulmonary arterial hypertension patients. The aim of this study was to evaluate the cardiac sympathetic nervous system activity by means of123Iodine-metaiodobenzylguanidine nuclear imaging in pulmonary arterial hypertension patients and to explore its possible correlation with markers of disease severity. Twelve consecutive pulmonary arterial hypertension patients (nine women, median age 56.5 (17.8), eight idiopathic and four connective tissue-associated pulmonary arterial hypertension) underwent cardiac123Iodine-metaiodobenzylguanidine scintigraphy. The results were compared with those of 12 subjects with a negative history of cardiovascular or pulmonary disease who underwent the same nuclear imaging test because of a suspected paraganglioma or pheochromocytoma, with a negative result (controls), and 12 patients with heart failure with reduced left ventricular ejection fraction. Hemodynamics, echocardiography, six-minute walking distance, cardiopulmonary exercise testing, and N-terminal pro brain natriuretic peptide were collected in pulmonary arterial hypertension patients within one week from123Iodine-metaiodobenzylguanidine scintigraphy. Cardiac123Iodine-metaiodobenzylguanidine uptake, assessed as early and late heart-to-mediastinum ratio, was significantly lower in pulmonary arterial hypertension compared to controls (p = 0.001), but similar to heart failure with reduced left ventricular ejection fraction. Myocardial123Iodine-metaiodobenzylguanidine turnover, expressed as washout rate, was similar in pulmonary arterial hypertension and heart failure with reduced left ventricular ejection fraction and significantly higher compared to controls (p = 0.016). In the pulmonary arterial hypertension group, both early and late heart-to-mediastinum ratios and washout rate correlated with parameters of pulmonary arterial hypertension severity including pulmonary vascular resistance, right atrial pressure, tricuspid annular plane systolic excursion, N-terminal pro brain natriuretic peptide, and peak VO2. Although we evaluated a small number of subjects, our study showed a significant impairment in cardiac sympathetic nervous system in pulmonary arterial hypertension, similarly to that observed in heart failure with reduced left ventricular ejection fraction. This impairment correlated with indices of pulmonary arterial hypertension severity. Cardiac sympathetic dysfunction may be a contributing factor to the development of right-sided heart failure in pulmonary arterial hypertension.

scholarly journals Interventional and Surgical Treatments for Pulmonary Arterial Hypertension

2021 ◽  
Vol 10 (15) ◽  
pp. 3326
Author(s):  
Tomasz Stącel ◽  
Magdalena Latos ◽  
Maciej Urlik ◽  
Mirosław Nęcki ◽  
Remigiusz Antończyk ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Interdisciplinary Approach ◽  
Left Ventricular ◽  
Free Survival ◽  
Atrial Septostomy ◽  
Surgical Options ◽  
Pulmonary Arterial ◽  
Va Ecmo ◽  
Surgical Treatments

Despite significant advancements in pharmacological treatment, interventional and surgical options are still viable treatments for patients with pulmonary arterial hypertension (PAH), particularly idiopathic PAH. Herein, we review the interventional and surgical treatments for PAH. Atrial septostomy and the Potts shunt can be useful bridging tools for lung transplantation (Ltx), which remains the final surgical treatment among patients who are refractory to any other kind of therapy. Veno-arterial extracorporeal membrane oxygenation (V-A ECMO) remains the ultimate bridging therapy for patients with severe PAH. More importantly, VA-ECMO plays a crucial role during Ltx and provides necessary left ventricular conditioning during the initial postoperative period. Pulmonary denervation may potentially be a new way to ensure better transplant-free survival among patients with the aforementioned disease. However, high-quality randomized controlled trials are needed. As established, obtaining the Eisenmenger physiology among patients with severe pulmonary hypertension by creating artificial defects is associated with improved survival. However, right-to-left shunting may be harmful after Ltx. Closure of the artificially created defects may carry some risk associated with cardiac surgery, especially among patients with Potts shunts. In conclusion, PAH requires an interdisciplinary approach using pharmacological, interventional, and surgical modalities.

scholarly journals Study of the effect of Occlutech Atrial Flow Regulator on symptoms, hemodynamics, and echocardiographic parameters in advanced pulmonary arterial hypertension

2021 ◽  
Vol 11 (1) ◽  
pp. 204589402198996
Author(s):  
Kothandam Sivakumar ◽  
Gopalavilasam R. Rohitraj ◽  
Monica Rajendran ◽  
Nithya Thivianathan
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Arterial Hypertension ◽  
Right Heart Failure ◽  
Right Atrial ◽  
Echocardiographic Parameters ◽  
Procedural Complications ◽  
Pulmonary Arterial ◽  
Flow Regulator ◽  
Risk Predictors

Optimal sized balloon atrial septostomy improves hemodynamics in advanced pulmonary arterial hypertension. Occlutech Atrial Flow Regulator is designed to provide an atrial septal fenestration diameter titrated according to the age and right atrial pressures. This observational study analyzed symptoms, exercise distance, oxygen saturations, hemodynamics and echocardiographic parameters after Atrial Flow Regulator implantation in patients with syncope or right-heart failure. Patients with high-risk predictors of mortality during septostomy were scrutinized. Thirty-nine patients (9 children) with syncope (34/39) or right-heart failure (27/39) underwent Atrial Flow Regulator implantation without procedural complications. Six-minute walk distance increased from 310 ± 158.2 to 376.4 ± 182.6 m, none developed syncope. Oxygen saturations reduced from 96.4 ± 6.4% to 92 ± 4.9% at rest and further to 80.3 ± 5.9% on exercise. Right atrial pressures reduced from 9.4 ± 5 (2–27) mmHg to 6.9 ± 2.6 (1–12) mmHg, while cardiac index increased from 2.4 ± 0.8 (0.98–4.3) to 3 ± 1 (1.1–5.3) L/min/m2 and systemic oxygen transport increased from 546.1 ± 157.9 (256.2–910.5) to 637.2 ± 191.1 (301.3–1020.2) ml/min. Echocardiographic improvement included significant reduction of pericardial effusion and inferior caval congestion at a median follow-up of 37 months. Overall survival improved except two early and one late deaths in high-risk patients. Five of seven patients with advanced disease and key hemodynamic predictors of mortality survived. Acute hemodynamic benefits in pulmonary arterial hypertension after Atrial Flow Regulator were improved cardiac output, systemic oxygen transport, and reduced right atrial pressures. Improvement of symptoms especially syncope, exercise duration, and right ventricular systolic function as well as device patency were sustained on mid-term follow-up. Implantation was safe in all including young children without procedural complications. Mortality was noted only in patients who had high-risk predictors and patients at advanced stage of the disease.

Prognostic relevance of sleep apnea syndrome in patients with pulmonary arterial hypertension

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J Precek ◽  
K Vykoupil ◽  
F Kovacik ◽  
M Hutyra
Keyword(s):  
Pulmonary Hypertension ◽  
Sleep Apnea ◽  
Pulmonary Arterial Hypertension ◽  
General Population ◽  
Arterial Hypertension ◽  
Left Ventricular ◽  
Sleep Study ◽  
Prognostic Relevance ◽  
Obstructive Sleep ◽  
Pulmonary Arterial

Abstract Introduction Sleep disordered breathing (SDB) is a group of ventilatory disorders during sleep which includes obstructive sleep apnea (OSA), central sleep apnea (CSA), and sleep related hypoventilation. In patients with SDB, the prevalence of pulmonary hypertension (PH) ranges from 17% to 52%. While SDB is prevalent in the general population with recent estimates of 20% to 30%, in those with cardiovascular disease, particularly left ventricular failure, there is a higher reported prevalence of 47%. Aims The aims of this study were to determine the prevalence and prognostic relevance of sleep apnea in a cohort of patients with newly diagnosed pulmonary arterial hypertension (ESC/WHO Group 1 pulmonary hypertension). Methods We evaluated prospectively 76 patients with the pulmonary arterial hypertension (mean age 54±16 years; 45% male). All patients underwent right heart catheterisation, clinical assessments, sleep study, standard laboratory testing and evaluation of subjective sleepiness by the Epworth Sleepiness Scale. Sleep test was provided with an ApneaLink Plus, consisting of nasal pressure sensor, respiratory effort band, and pulse oximeter worn on the finger. Subjects previously treated for or diagnosed with SDB were excluded from the study. Results Sleep apnea (SA) – defined as apnea-hypopnea index (AHI) ≥5/h – was found in 59 (77.6%) of the pulmonary arterial hypertension (PAH) patients. Mean AHI in the cohort of PAH patients with SA was 26.1±16.6/h. Mean follow-up was 24 months, during which 15 (19.7%) patients died. Characteristics of parameters related to SA in groups of survivors and deceased are in table 1. From the sleep apnea-related parameters, only time with O2Sat &lt;90% – T90 was significantly associated with mortality (AUC 0.856; 95% CI 0.693 – 1.019; p&lt;0.001). Conclusions The presence of sleep apnea in pulmonary arterial hypertension patients is high. The prevalence of sleep apnea is higher in PAH patients than in the general population. The presence of sleep apnea in patients with PAH was not associated with worse prognosis, but noctural hypoxemia (time with O2Sat &lt;90%) was related to poor prognosis. Sleep apnea in patients with PAH should be screened for systematically. Funding Acknowledgement Type of funding source: None

scholarly journals Underfilling decreases left ventricular function in pulmonary arterial hypertension

2021 ◽  
Author(s):  
Hannah Sjögren ◽  
Barbro Kjellström ◽  
Anna Bredfelt ◽  
Katarina Steding-Ehrenborg ◽  
Göran Rådegran ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Stroke Volume ◽  
Arterial Hypertension ◽  
Global Longitudinal Strain ◽  
Left Atrial Volume ◽  
Left Ventricular ◽  
Longitudinal Function ◽  
Right Heart Catheterisation ◽  
Pulmonary Arterial ◽  
Lv Volume

AbstractTo evaluate the association between impaired left ventricular (LV) longitudinal function and LV underfilling in patients with pulmonary arterial hypertension (PAH). Thirty-nine patients with PAH and 18 age and sex-matched healthy controls were included. LV volume and left atrial volume (LAV) were delineated in short-axis cardiac magnetic resonance (CMR) cine images. LV longitudinal function was assessed from atrio-ventricular plane displacement (AVPD) and global longitudinal strain (GLS) was assessed using feature tracking in three long-axis views. LV filling was assessed by LAV and by pulmonary artery wedge pressure (PAWP) using right heart catheterisation. Patients had a smaller LAV, LV volume and stroke volume as well as a lower LV-AVPD and LV-GLS than controls. PAWP was 6 [IQR 5––9] mmHg in patients. LV ejection fraction did not differ between groups. LV stroke volume correlated with LV-AVPD (r = 0.445, p = .001), LV-GLS (r = − 0.549, p < 0.0001) and LAVmax (r = .585, p < 0.0001). Furthermore, LV-AVPD (r = .598) and LV-GLS (r = − 0.675) correlated with LAVmax (p < 0.0001 for both). Neither LV-AVPD, LV-GLS, LAVmax nor stroke volume correlated with PAWP. Impaired LV longitudinal function was associated with low stroke volume, low PAWP and a small LAV in PAH. Small stroke volumes and LAV, together with normal LA pressure, implies that the mechanism causing reduced LV longitudinal function is underfilling rather than an intrinsic LV dysfunction in PAH.

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