Impact of left ventricular late enhancement on pulmonary arterial hypertension in idiopathic dilated cardiomyopathy

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.

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

European Heart Journal ◽

10.1093/ehjci/ehaa946.2243 ◽

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 <90% – T90 was significantly associated with mortality (AUC 0.856; 95% CI 0.693 – 1.019; p<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 <90%) was related to poor prognosis. Sleep apnea in patients with PAH should be screened for systematically. Funding Acknowledgement Type of funding source: None

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Underfilling decreases left ventricular function in pulmonary arterial hypertension

The International Journal of Cardiovascular Imaging ◽

10.1007/s10554-020-02143-6 ◽

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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Pulmonary Capillary Wedge Pressure d15 Mmhg Is A Good Indicator Of Normal Left Ventricular Filling Pressure In Pulmonary Arterial Hypertension Patients

10.1164/ajrccm-conference.2012.185.1_meetingabstracts.a6225 ◽

2012 ◽

Author(s):

Rudolf K.F. Oliveira ◽

Roberta P. Ramos ◽

Eloara V.M. Ferreira ◽

Flávio F. Arbex ◽

Carlos E.B. Kapins ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Left Ventricular ◽

Filling Pressure ◽

Pulmonary Capillary ◽

Normal Left Ventricular ◽

Ventricular Filling Pressure ◽

Pulmonary Arterial ◽

Ventricular Filling ◽

Wedge Pressure

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Why septal motion is a marker of right ventricular failure in pulmonary arterial hypertension: mechanistic analysis using a computer model

AJP Heart and Circulatory Physiology ◽

10.1152/ajpheart.00596.2016 ◽

2017 ◽

Vol 312 (4) ◽

pp. H691-H700 ◽

Cited By ~ 14

Author(s):

Georgina Palau-Caballero ◽

John Walmsley ◽

Vanessa Van Empel ◽

Joost Lumens ◽

Tammo Delhaas

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Pressure Gradient ◽

Right Ventricular ◽

Left Ventricular ◽

Contractile Dysfunction ◽

Ventricular Relaxation ◽

Pulmonary Arterial ◽

Rv Function ◽

Septal Motion

Rapid leftward septal motion (RLSM) during early left ventricular (LV) diastole is observed in patients with pulmonary arterial hypertension (PAH). RLSM exacerbates right ventricular (RV) systolic dysfunction and impairs LV filling. Increased RV wall tension caused by increased RV afterload has been suggested to cause interventricular relaxation dyssynchrony and RLSM in PAH. Simulations using the CircAdapt computational model were used to unravel the mechanism underlying RLSM by mechanistically linking myocardial tissue and pump function. Simulations of healthy circulation and mild, moderate, and severe PAH were performed. We also assessed the effects on RLSM when PAH coexists with RV or LV contractile dysfunction. Our results showed prolonged RV shortening in PAH causing interventricular relaxation dyssynchrony and RLSM. RLSM was observed in both moderate and severe PAH. A negative transseptal pressure gradient only occurred in severe PAH, demonstrating that negative pressure gradient does not entirely explain septal motion abnormalities. PAH coexisting with RV contractile dysfunction exacerbated both interventricular relaxation dyssynchrony and RLSM. LV contractile dysfunction reduced both interventricular relaxation dyssynchrony and RLSM. In conclusion, dyssynchrony in ventricular relaxation causes RLSM in PAH. Onset of RLSM in patients with PAH appears to indicate a worsening in RV function and hence can be used as a sign of RV failure. However, altered RLSM does not necessarily imply an altered RV afterload, but it can also indicate altered interplay of RV and LV contractile function. Reduction of RLSM can result from either improved RV function or a deterioration of LV function. NEW & NOTEWORTHY A novel approach describes the mechanism underlying abnormal septal dynamics in pulmonary arterial hypertension. Change in motion is not uniquely induced by altered right ventricular afterload, but also by altered ventricular relaxation dyssynchrony. Extension or change in motion is a marker reflecting interplay between right and left ventricular contractility.

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Echocardiographic Prognosis Relevance of Attenuated Right Heart Remodeling in Idiopathic Pulmonary Arterial Hypertension

Frontiers in Cardiovascular Medicine ◽

10.3389/fcvm.2021.650848 ◽

2021 ◽

Vol 8 ◽

Author(s):

Qin-Hua Zhao ◽

Su-Gang Gong ◽

Rong Jiang ◽

Chao Li ◽

Ge-Fei Chen ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Survival Rates ◽

Left Ventricular ◽

Functional Improvement ◽

Right Heart ◽

Long Term Follow Up ◽

Pulmonary Arterial ◽

Rv Function

Background: Right ventricular (RV) function is a great determination of the fate in patients with pulmonary arterial hypertension (PAH). Monitoring RV structure back to normal or improvement should be useful for evaluation of RV function. The aims of this study were to assess the prognostic relevance of changed right heart (RH) dimensions by echocardiography and attenuated RH remodeling (ARHR) in idiopathic PAH (IPAH).Methods: We retrospectively analyzed 232 consecutive adult IPAH patients at baseline assessment and included RH catheterization and echocardiography. ARHR at the mean 20 ± 12 months' follow-up was defined by a decreased right atrium area, RV mid-diameter, and left ventricular end-diastolic eccentricity index. The follow-up end point was all-cause mortality.Results: At mean 20 ± 12 months' follow-up, 33 of 232 patients (14.2%) presented with ARHR. The remaining 199 surviving patients were monitored for another 25 ± 20 months. At the end of follow-up, the survival rates at 1, 3, and 5 years were 89, 89, and 68% in patients with ARHR, respectively, and 84, 65 and 41% in patients without ARHR (log-rank p = 0.01). ARHR was an independent prognostic factor for mortality. Besides, ARHR was available to further stratify patients' risk assessment through the French PAH non-invasive-risk criteria.Conclusions: Echocardiographic ARHR is an independent determinant of prognosis in IPAH at long-term follow-up. ARHR might be a useful tool to indicate the RV morphologic and functional improvement associated with better prognostic likelihood.

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