Prevalence of pulmonary hypertension in adults after atrial switch and role of ventricular filling pressures

Heart ◽  
2020 ◽  
pp. heartjnl-2020-317111
Author(s):  
William R Miranda ◽  
C Charles Jain ◽  
Heidi M Connolly ◽  
Hilary M DuBrock ◽  
Frank Cetta ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Diastolic Pressure ◽  
Atrioventricular Valve Regurgitation ◽  
Switch Operation ◽  
Atrial Switch ◽  
Pulmonary Arterial ◽  
Atrial Switch Operation ◽  
Ventricular Filling ◽  
Wedge Pressure

ObjectiveTo assess the prevalence of elevated systemic right ventricular (sRV) end-diastolic pressure and pulmonary arterial hypertension in adults with transposition of the great arteries (TGA) who have undergone atrial switch operation.MethodsForty-two adults (aged ≥18 years) with complete TGA and atrial switch palliation undergoing cardiac catheterisation between 2004 and 2018 at Mayo Clinic, MN, were identified. Clinical, echocardiographic and invasive haemodynamic data were abstracted from the medical charts and procedure logs.ResultsMean age was 37.6±7.9 years; 28 were male (67%). The Mustard operation was performed in 91% of individuals. Mean estimated sRV ejection fraction by echocardiography was 33.3%±10.9% and ≥moderate tricuspid (systemic atrioventricular valve) regurgitation was present in 15 patients (36%). Mean sRV end-diastolic pressure was 13.2±5.4 mm Hg. An sRV end-diastolic pressure >15 mm Hg was present in 35% of individuals whereas a pulmonary artery wedge pressure (PAWP) >15 mm Hg was seen in 59%. Mean pulmonary artery pressure ≥25 mm Hg was seen in 47.5% of patients with PAWP being >15 mm Hg in all but one patient.ConclusionIn adults after atrial switch, elevated sRV end-diastolic pressure was present in only one-third of patients whereas increased PAWP was seen in almost 60%. These findings are most likely related to a combination of decreased pulmonary atrial (functional left atrium) compliance and, in a subset of patients, pulmonary venous baffle obstruction. Elevation in pulmonary pressures was highly prevalent with concomitant elevation in PAWP being present in essentially all patients.

Melody® pulmonary valve implantation in two teenage patients with congenitally corrected transposition of the great arteries status after Senning atrial switch operation

2016 ◽  
Vol 27 (3) ◽  
pp. 600-604
Author(s):  
Rodrigo Rios ◽  
Susan R. Foerster ◽  
Todd M. Gudausky
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricle ◽  
Switch Operation ◽  
Atrial Switch ◽  
Atrial Switch Operation ◽  
The Right ◽  
Valve Implantation ◽  
Congenitally Corrected Transposition ◽  
Artery Conduit ◽  
Corrected Transposition

AbstractThe Melody® transcatheter pulmonary valve system was developed for placement within right ventricle-to-pulmonary artery conduits in patients with CHD for treatment of stenosis or regurgitation, providing an alternative to open-heart surgery. Abnormal systemic venous connections altering the catheter course to the right ventricle-to-pulmonary artery conduit may present a challenge to Melody® valve implantation. We present two such cases, in which the Melody® valve was successfully implanted in teenage patients with congenitally corrected transposition of the great arteries after Senning atrial switch operation. Despite the abnormal catheter course, the right ventricle-to-pulmonary artery was approachable via the right femoral vein allowing for deployment of the Melody® valve in the appropriate position. This suggests that systemic vein-to-left atrium baffles are not prohibitive of Melody® valve implantation. This is an important implication considering the substantial population of ageing patients with CHD who have undergone atrial switch. Melody® valve implantation can be considered as a viable option for treatment of these patients if they develop right ventricle-to-pulmonary artery conduit failure.

scholarly journals Prognostic impact of neurohormonal inhibition in pulmonary arterial hypertension

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
R Scagliola ◽  
I Rota ◽  
M Cheli ◽  
C Brunelli ◽  
M Balbi ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Beta Blocker ◽  
Diastolic Pressure ◽  
Smoking Habit ◽  
Prognostic Impact ◽  
Mineralocorticoid Receptor Antagonist ◽  
Kaplan Meier ◽  
Pulmonary Arterial

Abstract Background Experimental evidence points towards a hyperactivity of the sympathetic nervous system and renin-angiotensin-aldosterone system in the pathobiology of pulmonary arterial hypertension (PAH), raising the hypothesis that blockade of neurohormonal axis may have favorable effects in this context. Purpose To assess the use and prognostic impact of neurohormonal inhibitors (NEUi) in a single centre cohort of subjects with PAH. Methods We analysed retrospectively collected data from our register of right heart catheterizations (RHC) performed consecutively from January 1st 2005 until October 31st 2018. We selected patients with PAH and complete information about demographics, biochemical data and drug therapy at the time of RHC. Patients on beta-blocker, angiotensin-converting enzyme inhibitor (ACEi), angiotensin receptor blocker (ARB) or mineralocorticoid receptor antagonist (MRA) at the time of RHC were classified as NEUi users. Comparisons between NEUi recipients and non-recipients were drawn by chi-square or t-test, as appropriate. Death from any cause was assessed by Kaplan-Meier analysis. Results Complete data were available for 57 PAH patients. Mean pulmonary artery pressure, pulmonary artery wedge pressure, diastolic pressure gradient, pulmonary vascular resistance and cardiac index were 45.0±14.9 mmHg, 10.9±3.5 mmHg, 16.0±10.2 mmHg, 8.8±5.1 Wood units and 2.5±0.8 l/min/m2 respectively. Twenty-seven subjects (47.4%) were taking at least one NEUi when RHC was performed: 12 (21.1%) were on beta-blocker, 15 (26.3%) on ACEi/ARB and 6 (10.5%) on MRA. NEUi users were significantly older (67.6±11.9 vs. 58.3±15.2 years, p=0.039), had a higher body mass index (25.9±4.4 vs. 23.6±3.5, p=0.029), more frequently systemic arterial hypertension (74.1% vs. 40.0%, p=0.020), smoking habit (51.9% vs. 20.0%, p=0.025) and lower estimated glomerular filtration rate (58.7±22.7 vs. 73.7±24.7 ml/min/1.73 m2, p=0.022) than non-users. Moreover, 5 NEUi users (18.5%), but no NEUi non-users, had a history of coronary artery disease. Hemodynamic parameters were similar in NEUi recipients and non-recipients (p=NS). Seven patients (25.9%) died in the NEUi users group vs. 17 (56.7%) in the non-users one (p=0.038). Kaplan-Meier analysis confirmed that subjects not taking NEUi were more likely to die over the course of follow-up (Log-Rank p=0.020) (Figure 1). Conclusions Our data identify a subset of atypical PAH patients, with pre-capillary pulmonary hypertension and a comorbidity profile for left heart disease (LHD), in whom NEUi have shown to improve survival. A prognostic benefit of NEUi, due to their effects on cardiovascular comorbidities in this kind of patients, has been speculated. Future prospective studies are needed to identify the most appropriate treatment strategies for atypical forms of PAH, with subtle and probably covert LHD. Figure 1. Kaplan-Meier survival curves Funding Acknowledgement Type of funding source: None

ID: 123: ROLE OF MICRORNA-1 IN REGULATING PULMONARY VASCULAR REMODELING IN PULMONARY ARTERIAL HYPERTENSION

2016 ◽  
Vol 64 (4) ◽  
pp. 969.1-969 ◽  
Author(s):  
JR Sysol ◽  
J Chen ◽  
S Singla ◽  
V Natarajan ◽  
RF Machado ◽  
...  
Keyword(s):  
Smooth Muscle ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Vascular Remodeling ◽  
Luciferase Reporter ◽  
Pulmonary Vascular Remodeling ◽  
Pulmonary Arterial ◽  
Pulmonary Artery Smooth Muscle

RationalePulmonary arterial hypertension (PAH) is a severe, progressive disease characterized by increased pulmonary arterial pressure and resistance due in part to uncontrolled vascular remodeling. The mechanisms contributing to vascular remodeling in PAH are poorly understood and involve rampant pulmonary artery smooth muscle cell (PASMC) proliferation. We recently demonstrated the important role of sphingosine kinase 1 (SphK1), a lipid kinase producing pro-proliferative sphingosine-1-phosphate (S1P), in the development of pulmonary vascular remodeling in PAH. However, the regulatory processes involved in upregulation of SphK1 in this disease are unknown.ObjectiveIn this study, we aimed to identify novel molecular mechanisms governing the regulation of SphK1 expression, with a focus on microRNA (miR). Using both in vitro studies in pulmonary artery smooth muscle cells (PASMCs) and an in vivo mouse model of experimental hypoxia-mediated pulmonary hypertension (HPH), we explored the role of miR in controlling SphK1 expression in the development of pulmonary vascular remodeling.Methods and ResultsIn silico analysis identified hsa-miR-1-3p (miR-1) as a candidate targeting SphK1. We demonstrate miR-1 is down-regulated by hypoxia in human PASMCs and in lung tissues of mice with HPH, coinciding with upregulation of SphK1 expression. PASMCs isolated from patients with PAH had significantly reduced expression of miR-1. Transfection of human PASMCs with miR-1 mimics significantly attenuated activity of a SphK1-3'-UTR luciferase reporter construct and SphK1 protein expression. miR-1 overexpression in human PASMCs also inhibited proliferation and migration under normoxic and hypoxic conditions, both important in pathogenic vascular remodeling in PAH. Finally, we demonstrated that intravenous administration of miR-1 mimics prevents the development of experimental HPH in mice and attenuates induction of SphK1 in PASMCs.ConclusionThese data demonstrate that miR-1 expression in reduced in PASMCs from PAH patients, is modulated by hypoxia, and regulates the expression of SphK1. Key phenotypic aspects of vascular remodeling are influenced by miR-1 and its overexpression can prevent the development of HPH in mice. These studies further our understanding of the mechanisms underlying pathogenic pulmonary vascular remodeling in PAH and could lead to novel therapeutic targets.Supported by grants NIH/NHLBI R01 HL127342 and R01 HL111656 to RFM, NIH/NHLBI P01 HL98050 and R01 HL127342 to VN, American Heart Association Predoctoral Fellowship (15PRE2190004) to JRS, and NIH/NLHBI NRSA F30 Fellowship (FHL128034A) to JRS.

scholarly journals ACCURACY OF IMAGING MODALITIES IN DETECTION OF BAFFLE LEAKS IN PATIENTS FOLLOWING ATRIAL SWITCH OPERATION

2015 ◽  
Vol 65 (10) ◽  
pp. A497
Author(s):  
Carolyn Wilhelm ◽  
Tracey Sisk ◽  
Sharon Roble ◽  
Joanne Chisolm ◽  
John Cheatham ◽  
...  
Keyword(s):  
Imaging Modalities ◽  
Switch Operation ◽  
Atrial Switch ◽  
Atrial Switch Operation

P4689How is the impact of updated hemodynamic definitions on frequencies of overall pulmonary hypertension and pre-capillary pulmonary hypertension as compared to those with previous criteria

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Tanyeri ◽  
B Keskin ◽  
O Y Akbal ◽  
A Hakgor ◽  
A Karagoz ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Critical Role ◽  
Right Heart ◽  
Right Heart Catheterisation ◽  
Mean Pressure ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology ◽  
The Impact ◽  
Wedge Pressure

Abstract Background and aim In this study we evaluated the impact of the updated pulmonary hypertension (PH) definitive criteria proposed in 6th World PH Symposium (WSPH) on numbers and frequencies of and pre- versus post-capillary PH as compared to those in European Society of Cardiology (ESC) 2015 PH Guidelines. Methods Study group comprised the retrospectively evaluated 1299 patients (pts) (age 53.1±18.8 years, female 807, 62.1%) who underwent right heart catheterisation (RHC) with different indications between 2006 and 2018. For ESC and WSPH PH definitions, pulmonary arterial mean pressure (PAMP) ≥25 mmHg (definition-A) and PAMP >20 mmHg (definition-B) RHC criteria were used, respectively. For pre-capillary PH definitions, pulmonary artery wedge pressure (PAWP) ≤15 mmHg and pulmonary vascular resistance (PVR) ≥3 Wood units criteria were included in the both definitions. Results In RHC assessments, PAMP ≥25 mmHg and >20 mmHg were noted in 891 (68.6%) and 1051 (80.9%) of overall pts, respectively. Moreover, pre-capillary PH was diagnosed in 284 (21.8%) and 298 (22.9%) with definition-A and B, respectively. Although updated WSPH definition was associated with a net 12.3% and a relative 18% increase in the overall PH diagnosis, net and relative changes in the frequency of the pre-capillary PH were only 1% and 4.9%. Increase in the overall PH with updated WSPH criterias compared to previous ESC definitions was associated with increase in the number of pre-capillary PH (n=298, 22.9%) but not in the overall frequency of post-capillary PH (688, 52.9%). Because PVR was the product of the transpulmonary gradient (PAMP minus PAWP) divided by cardiac output, this measure was found to keep specificity for distinction between pre- versus post-capillary PH even after lowering thetreshold diagnostic for PAMP from 25 to 20 mmHg. Conclusions Although updated WSPH definition was associated with net 12.3% and relative 18% increase in the overall PH diagnosis, its impact on frequencies of pre- versus post-capillary PH within overall PH population was negligible.These seem to be due to critical role of PVR ensuring specificity in pre-capillary PH diagnosis even after lowering the definitive PAMP treshold to 20 mmHg.

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