Inhibiting Pyruvate Kinase Muscle Isoform 2 Regresses Group 2 Pulmonary Hypertension Induced by Supra‐coronary Aortic Banding

Introduction: Pregnancy is contraindicated in women with pulmonary hypertension (PH), yet many still decide to pursue pregnancy. Hypothesis: We hypothesized improved maternal mortality with PH at our center’s cardio-obstetrics program and sought to identify factors to estimate the risk of major adverse cardiac events (MACE). Methods: Pregnant women with right ventricular systolic pressure (RVSP) ≥35 mmHg or tricuspid regurgitant velocity > 2.8 m/s on transthoracic echocardiogram (TTE) were identified. Women with intermediate to high probability PH by ESC criteria (TTE or catheterization, n = 70) were classified using the 6 th World Society of PH definitions. Results: In 70 women with PH (30 ± 6 years-old, RVSP 52 ± 16 mmHg) there were 12 (17%) with WHO Group 1 PH, 45 (64%) with Group 2 PH, 4 (6%) with Group 3 PH and 9 (13%) with Group 5 PH (Figure A). Baseline characteristics were similar except: Group 1 PH had 83% on prostacyclin (PC) therapy, higher RVSP (78 ± 20 mmHg vs. Groups 2 (46 ± 9), 3 (44 ± 2 mmHg) and 5 PH (48 ± 10mmHg), p<0.01), and compared to Group 2 PH, more Group 1 PH women were diagnosed pre-pregnancy (9 (75%) vs. 12 (27%), p = 0.01) and had cardio-obstetrics care (10 (83%) vs. 16 (36%), p < 0.01) (Figure B - E). There were no peripartum deaths, however 3 (4.3%) women with Group 2 PH had late mortality (7 ± 4 months post-partum). MACE occurred in 24 (34%) women and was more likely in those with: NYHA FC ≥ 2 (95% CI 4.7-57, p < 0.01), pre-eclampsia (95% CI 1.2-13, p = 0.03), RVSP >50 mmHg (95% CI 1.3-10, p = 0.02) and LVEF <50% (95% CI 1.1-8.8, p = 0.04) (Figure F). Preterm birth occurred in 32 (49%) pregnancies, with no neonatal mortality. Conclusion: To conclude, in a large single center cohort we report 100% 1-year survival in Groups 1, 3, and 5 PH, with most Group 1 PH patients on PC therapy and under cardio-obstetrics care. We identify Group 2 PH as an under-recognized group for adverse outcomes in pregnancy, with NYHA FC, pre-eclampsia, RVSP >50 mmHg and LVEF <50% associated with increased MACE.

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World Health Organization pulmonary hypertension group 2: pulmonary hypertension due to left heart disease

Pulmonary Circulation ◽

10.1201/9781315382753-49 ◽

2016 ◽

pp. 440-455

Keyword(s):

Pulmonary Hypertension ◽

Heart Disease ◽

World Health Organization ◽

World Health ◽

Left Heart ◽

Left Heart Disease ◽

Group 2 ◽

Health Organization

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How is juvenile rheumatic heart disease different: baseline results from a prospective north Indian registry

European Heart Journal ◽

10.1093/ehjci/ehaa946.1898 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

K Mahajan ◽

D.R Prakash Chand Negi

Keyword(s):

Heart Failure ◽

Atrial Fibrillation ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Funding Source ◽

Thromboembolic Events ◽

Group 3 ◽

Group 2 ◽

Rheumatic Heart ◽

Group 1

Abstract Introduction Juvenile rheumatic heart disease (RHD) refers to RHD in patients <20 years of age. There are no contemporary data highlighting the differences between juvenile and older RHD patients. Purpose We aim to report the age related differences in the pattern, and consequencies of valvular dysfunction in patients of RHD. Methods The 2475 consecutive patients of RHD diagnosed using clinical and echocardiographic criteria were registered prospectively from 2011 till December 2019. Patients were divided into 3 groups according to their age: Group 1 (Juvenile RHD), Group 2 (21–50 years), and Group 2 (>51 years).The data concerning the socio-demographic and clinical profile were recorded systematically, and the nature and severity of valvular dysfunction was assessed by echocardiography. The data were analyzed using the Epi-InfoTM Software. Results Out of 2475 RHD patients, Juvenile RHD comprised of 211 (8.5%) patients. Group 2 and 3 comprised of 1691 (68.3%) and 573 (23.2%) patients respectively. Overall, 1767 (71.4%) patients were females, however this female predilection was less pronounced in juvenile RHD (55.5% females vs 44.5% males) as compared to older groups. Past history of acute rheumatic fever was more commonly recorded in Juvenile RHD group (37.9% vs 18.8% in group 2 and 10% in group 3, p=0.0001). At the time of registration, the presence of advanced heart failure symptoms (dyspnea class III and IV) (11.4% group 1 vs 13.9% group 2 vs 20.6% group 3, p<0.0001), right heart failure symptoms (0.9% group 1 vs 2.5% group 2 vs 7.3% group 3, p<0.01), thromboembolic events (0% group 1 vs 4.1% group 2 vs 3.3% group 3, p<0.01), atrial fibrillation (2.8% group 1 vs 24.5% group 2 vs 45.9% group 3, p<0.0001), and pulmonary hypertension (27.1% group 1 vs 40.3% group 2 vs 51.9% group 3, p<0.01), were all more commonly recorded in non-juvenile older RHD groups. Multivalvular involvement was also less common in juvenile RHD (34.6% vs 42.4% and 44.5%, p=0.04). Mitral regurgitation was the most common lesion in Juvenile RHD followed by aortic regurgitation (68.7% and 40.2% respectively). Stenotic lesions (both mitral and aortic) were present more commonly in older age groups. Conclusion RHD is predominantly a disease of females, however the predilection is less common in juvenile patients. Juvenile RHD predominantly affects the mitral valve and mainly leads to regurgitant lesions. As the age advances, the complications of RHD, mainly heart failure symptoms, thromboembolic events, pulmonary hypertension, and atrial fibrillation, become more common. Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Self sponsored registry

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Effect of Group 2 Pulmonary Hypertension Subgroups on Outcomes: Impact of the Updated Definition of Pulmonary Hypertension

Heart Lung and Circulation ◽

10.1016/j.hlc.2021.10.002 ◽

2021 ◽

Author(s):

Zubeyde Bayram ◽

Cem Dogan ◽

Suleyman Cagan Efe ◽

Ali Karagoz ◽

Busra Guvendi ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Group 2 ◽

Definition Of

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Abstract 16078: Pulmonary Vasculopathy Assessed By Intravascular Ultrasound in Persistent Pulmonary Hypertension After Mitral Valve Replacement, Comparation to Idiopathic Pulmonary Arterial Hypertension and Healthy Controls

Circulation ◽

10.1161/circ.132.suppl_3.16078 ◽

2015 ◽

Vol 132 (suppl_3) ◽

Author(s):

Juan Carlos J Grignola ◽

Leticia L Fernandez-Lopez ◽

Enric E Domingo-Ribas ◽

Rio R Aguilar ◽

Cristian Humberto C Arredondo ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Elastic Modulus ◽

Mitral Valve ◽

Valve Replacement ◽

Mitral Valve Replacement ◽

Persistent Pulmonary Hypertension ◽

Pulmonary Arterial ◽

Group 3 ◽

Group 2 ◽

Group 1

Purpose: The aim of the study is to assess pulmonary vasculopathy (wall fibrosis, pulmonary arterial pulsatility and elastic modulus ) in patients with persistent pulmonary hypertension (pulmonary systolic pressure by ECHO > 50 mmhg ) at least 1 year after mitral valve replacement with normal function of the valve. The evaluation was carried out by intravascular ultrasound (IVUS) in medium sized pulmonary arteries. We compared three groups: Group 1 ( persistent pulmonary hypertension after mitral valve replacement), Group 2 (pulmonary hypertension belonging to the group 1 of the Dana Point classification) and Group 3 (healthy controls). Methods: We studied 43 patients, 15 in Group 1 , 18 Group 2 and 10 in Group 3. Group 1: 13 females, the mean age of this group was 74+-7 years; Group 2: 14 females, 53+-14 years and Group 3: 6 females, 51+-5 years. All patients were submitted to left and right heart catheterization, and IVUS in medium sized elastic PA ( 2-3 mm diameter ) of the inferior lobes. Studied variables were: mean pulmonary artery pressure (PAP, mm Hg), pulmonary wedge pressure, aortic pressure, cardiac output (CO,l/min), pulmonary vascular resistance (PVR, Wood Units), IVUS pulsatility and elastic modulus (EM,mm Hg). Local pulsatility was estimated by IVUS: (systolic- diastolic lumen area/ diastolic lumen area) X 100. PA stiffness was assessed by the elastic modulus (EM= pulse pressure/ IVUSp). Results: In Group 3 all variables were statistically different from the other 2 groups (p<0.01). Variables are shown in table. Conclusions: Group 1, even with a lower mean PAP than Group 2 (p<0.05) showed a similar anatomical ( wall fibrosis ) and similar functional wall remodeling ( EM ).

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The incremental shuttle walk test predicts mortality in non-group 1 pulmonary hypertension: results from the ASPIRE Registry

Pulmonary Circulation ◽

10.1177/2045894019848649 ◽

2019 ◽

Vol 9 (2) ◽

pp. 204589401984864 ◽

Cited By ~ 1

Author(s):

Catherine G. Billings ◽

Robert Lewis ◽

Judith A. Hurdman ◽

Robin Condliffe ◽

Charlie A. Elliot ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Ceiling Effect ◽

Walk Test ◽

Limited Information ◽

Pulmonary Hemodynamics ◽

Disease Etiology ◽

Invasive Test ◽

Group 2 ◽

Test Distance ◽

Group 1

Pulmonary hypertension (PH) is classified into five groups based on disease etiology but there is only limited information on the prognostic value of exercise testing in non-group 1 PH. In group 1 PH, the incremental shuttle walking test (ISWT) distance has been shown to correlate with pulmonary hemodynamics and predict survival without a ceiling effect. This study assessed the ISWT in non-group 1 PH. Data were retrieved from the ASPIRE Registry (Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre) for consecutive patients diagnosed with PH. Patients were required to have been systematically assessed as group 2–5 PH and to have a baseline ISWT within three months of cardiac catheterization. Patients were stratified according to incremental shuttle walk test distance (ISWD) and ISWT distance percent predicted (ISWD%pred). A total of 479 patients with non-group 1 PH were identified. ISWD and ISWD%pred correlated significantly with symptoms and hemodynamic severity. ISWD and ISWD%pred predicted survival with no ceiling effect. The test was prognostic in groups 2, 3, and 4. ISWD and ISWD%pred and change in ISWD and ISWD%pred at one year were all significant predictors of outcome. In patients with non-group 1 PH the ISWT is a simple non-invasive test that is easy to perform, is predictive of survival at baseline and follow-up, reflects change, and can be used in the assessment of PH of any etiology.

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Electrophysiological Studies in Patients with Pulmonary Hypertension: A Retrospective Investigation

BioMed Research International ◽

10.1155/2014/617565 ◽

2014 ◽

Vol 2014 ◽

pp. 1-6 ◽

Cited By ~ 7

Author(s):

Dirk Bandorski ◽

Jörn Schmitt ◽

Claudia Kurzlechner ◽

Damir Erkapic ◽

Christian W. Hamm ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Atrial Flutter ◽

Electrophysiological Study ◽

Atrioventricular Nodal Reentrant Tachycardia ◽

Reentrant Tachycardia ◽

Group 4 ◽

Electrophysiological Studies ◽

Group 5 ◽

Group 2 ◽

Ventricular Tachycardias

Few studies have investigated patients with pulmonary hypertension and arrhythmias. Data on electrophysiological studies in these patients are rare. In a retrospective dual-centre design, we analysed data from patients with indications for electrophysiological study. Fifty-five patients with pulmonary hypertension were included (Dana Point Classification: group 1: 14, group 2: 23, group 3: 4, group 4: 8, group 5: 2, and 4 patients with exercised-induced pulmonary hypertension). Clinical data, 6-minute walk distance, laboratory values, and echocardiography were collected/performed. Nonsustained ventricular tachycardia was the most frequent indication (n=15) for an electrophysiological study, followed by atrial flutter (n=14). In summary 36 ablations were performed and 25 of them were successful (atrial flutter 12 of 14 and atrioventricular nodal reentrant tachycardia 4 of 4). Fluoroscopy time was 16±14.4 minutes. Electrophysiological studies in patients with pulmonary hypertension are feasible and safe. Ablation procedures are as effective in these patients as in non-PAH patients with atrial flutter and atrioventricular nodal reentrant tachycardia and should be performed likewise. The prognostic relevance of ventricular stimulations and inducible ventricular tachycardias in these patients is still unclear and requires further investigation.

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