Atrial flow regulator for severe drug resistant pulmonary arterial hypertension after congenital heart defect correction.
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ABSTRACT:
SUBMITTER: Dabrowska-Kugacka A
PROVIDER: S-EPMC8086679 | biostudies-literature | 2019
REPOSITORIES: biostudies-literature
Publications
Atrial flow regulator for severe drug resistant pulmonary arterial hypertension after congenital heart defect correction.
Dąbrowska-Kugacka Alicja A Ciećwierz Dariusz D Żuk Grzegorz G Fijałkowski Marcin M Ottowicz Antoni A Kwiatkowska Joanna J Lewicka Ewa E Sabiniewicz Robert R
Cardiology journal 20190101 1
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Project description:AimsPotts shunt has been proposed as a bridge or alternative to lung transplantation for children with severe and drug-refractory suprasystemic pulmonary arterial hypertension (PAH). We describe the management of the atrial shunt when a Potts shunt is planned in refractory PAH.Methods and resultsWe report a case series of children in whom a Potts shunt was done for severe PAH associated with an atrial septal defect to illustrate the different clinical and haemodynamic scenarios. Five children (2 to 13 years) underwent a Potts shunt: three surgical, one percutaneous Potts shunt, and one percutaneous stenting of a restrictive arterial duct. All had associated atrial septal defect. Those who had generalized cyanosis before the procedure had a complicated postoperative course and required longer ventilatory and inotropic support, except the one who had atrial septal defect closure before the Potts shunt. One of the three cyanotic patients died. Two patients with left-to-right shunt before the Potts shunt had an uncomplicated postoperative course.ConclusionsShunt physiology is only partially predictable after the Potts shunt in children with PAH and atrial septal defect. Abrupt drop in left ventricle preload while the right ventricle is decompressed can potentially be prevented by atrial septal defect closure prior to the Potts shunt.
| S-EPMC7835613 | biostudies-literature
Project description:Although multiple gene and protein expression have been extensively profiled in human pulmonary arterial hypertension (PAH), the mechanism for the development and progression of pulmonary hypertension remains elusive. Analysis of the global metabolomic heterogeneity within the pulmonary vascular system leads to a better understanding of disease progression. Using a combination of high-throughput liquid-and-gas-chromatography-based mass spectrometry, we showed unbiased metabolomic profiles of disrupted glycolysis, increased TCA cycle, and fatty acid metabolites with altered oxidation pathways in the severe human PAH lung. The results suggest that PAH has specific metabolic pathways contributing to increased ATP synthesis for the vascular remodeling process in severe pulmonary hypertension. These identified metabolites may serve as potential biomarkers for the diagnosis of severe PAH. By profiling metabolomic alterations of the PAH lung, we reveal new pathogenic mechanisms of PAH in its later stage, which may differ from the earlier stage of PAH, opening an avenue of exploration for therapeutics that target metabolic pathway alterations in the progression of PAH. Global profiles were determined in human lung tissue and compared across 11 normal and 12 severe pulmonary arterial hypertension patients. Using a combination of microarray and high-throughput liquid-and-gas-chromatography-based mass spectrometry, we showed unbiased metabolomic profiles of disrupted glycolysis, increased TCA cycle, and fatty acid metabolites with altered oxidation pathways in the severe human PAH lung.
2014-11-14 | E-GEOD-53408 | biostudies-arrayexpress
Project description:A Gerbode-type defect is a ventricular septal defect communicating directly between the left ventricle and right atrium. It is usually congenital, but rarely is acquired, as a complication of endocarditis. This can be anatomically possible because the normal tricuspid valve is more apically displaced than the mitral valve. However, identification of an actual communication is often extremely difficult, so a careful and meticulous echocardiogram should be done in order to prevent echocardiographic misinterpretation of this defect as pulmonary arterial hypertension. The large systolic pressure gradient between the left ventricle and the right atrium would expectedly result in a high velocity systolic Doppler flow signal in right atrium and it can be sometimes mistakably diagnosed as tricuspid regurgitant jet simulating pulmonary arterial hypertension. We present a rare case of young woman, with endocarditis who presented with severe pulmonary arterial hypertension. The preoperative diagnosis of left ventricle to right atrial communication (acquired Gerbode defect) was suspected initially by echocardiogram and confirmed at the time of the surgery. A point of interest, apart from the diagnostic problem, was the explanation for its mechanism and presentation. The probability of a bacterial etiology of the defect is high in this case.
| S-EPMC2958911 | biostudies-literature
Project description:We report a case of a 61-year-old woman with a large atrial septal defect (ASD) that was detected incidentally on chest radiography and computed tomography when she presented with sepsis. Echocardiography confirmed a large secundum ASD with left-to-right shunt flow, right heart dilatation and severe pulmonary hypertension. The patient had a poor clinical outcome despite intensive care and eventually passed away. Haemodynamically significant ASDs have a known association with increased morbidity and mortality, and their early detection and closure cannot be understated. This article aimed to highlight the imaging features of ASD, with special emphasis on the routine chest radiograph. The pathophysiology and clinical manifestations of ASD are also briefly discussed.
| S-EPMC5966638 | biostudies-literature
Project description:AimsPatients with pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH) after defect correction have a poor prognosis compared with other CHD-PAH patients. Therefore, it is important that these patients are treated as early and effectively as possible. Evidence supporting the use of PAH therapies in patients with corrected CHD-PAH from randomised controlled trials is limited. The purpose of these analyses was to characterise the corrected CHD-PAH patients from the GRIPHON study and examine the response to selexipag.Methods and resultsOut of the 110 patients diagnosed with corrected CHD-PAH, 55 had atrial septal defects, 38 had ventricular septal defects, 14 had persistent ducti arteriosus, and 3 had defects not further specified. Hazard ratios (HR) and 95% confidence intervals (CI) for the primary composite endpoint were calculated using Cox proportional hazard models. Compared with the non-CHD patients from GRIPHON, patients with corrected CHD-PAH were slightly younger, with a greater proportion being treatment-naive and in World Health Organization functional class I/II. The rate of the primary composite endpoint of morbidity/mortality was lower in patients with corrected CHD-PAH who were treated with selexipag compared with those treated with placebo (HR 0.58; 95% CI 0.25, 1.37). The most common adverse events were those known to be related to selexipag.ConclusionsThese post-hoc analyses of GRIPHON provide valuable information about a large population of patients with corrected CHD-PAH, and suggest that selexipag may delay disease progression and was well-tolerated in patients with corrected CHD-PAH.
| S-EPMC6607487 | biostudies-literature
Project description:Although multiple gene and protein expression have been extensively profiled in human pulmonary arterial hypertension (PAH), the mechanism for the development and progression of pulmonary hypertension remains elusive. Analysis of the global metabolomic heterogeneity within the pulmonary vascular system leads to a better understanding of disease progression. Using a combination of high-throughput liquid-and-gas-chromatography-based mass spectrometry, we showed unbiased metabolomic profiles of disrupted glycolysis, increased TCA cycle, and fatty acid metabolites with altered oxidation pathways in the severe human PAH lung. The results suggest that PAH has specific metabolic pathways contributing to increased ATP synthesis for the vascular remodeling process in severe pulmonary hypertension. These identified metabolites may serve as potential biomarkers for the diagnosis of severe PAH. By profiling metabolomic alterations of the PAH lung, we reveal new pathogenic mechanisms of PAH in its later stage, which may differ from the earlier stage of PAH, opening an avenue of exploration for therapeutics that target metabolic pathway alterations in the progression of PAH.
2014-11-14 | GSE53408 | GEO
Project description:Pulmonary arterial hypertension (PAH) related to an atrial septal defect (ASD) poses a challenge to transcatheter closure of an ASD (tcASD). We aimed to determine the predictors for remaining PAH (rPAH) post-tcASD. This retrospective study was conducted at a single tertiary university hospital. Adult patients with an ASD and PAH were divided into three groups according to pulmonary vascular resistance (PVR). Normalization of pulmonary atrial systolic pressure (PASP) was defined as an estimated right ventricular systolic pressure < 40 mmHg and was determined using transthoracic echocardiography. Among 119 patients, 80% showed PAH normalization post-tcASD. Normalization of PAH post-tcASD was observed in 100%, 56.2%, and 28.6% of patients in mild, moderate, and severe PVR groups, respectively. The patients' New York Heart Association functional class improved. Multivariate logistic regression analysis showed that age and high PVR were significant risk factors for rPAH. A receiving operator curve analysis showed a PASP cutoff value > 67.5 mmHg to be predictive of rPAH post-tcASD, with an area under the curve value of 0.944 (sensitivity, 0.922; specificity 0.933). Most patients, including moderate-to-severe PAH patients, improved hemodynamically and clinically with tcASD. Since patients with severe PAH are at a risk of rPAH, tcASD should be performed by selecting the patient carefully based on pre-procedure medication, a vasoreactivity test, and a balloon occlusion test.
| S-EPMC10095540 | biostudies-literature
Project description:BackgroundElevated right atrial (RA) pressure is an established prognostic measure in pulmonary arterial hypertension (PAH). However, little is known about perturbations in RA function in PAH.Methods and resultsReservoir (RA longitudinal strain [RA LS]), conduit (RA early LS rate), and active (RA late LS rate) phases were assessed by 2D speckle tracking in 65 patients with PAH, 6-minute walk distance ≤450 m, and a pulmonary vascular resistance >800 dynes·s/cm(5), despite therapy with at least 2 PAH-specific medications enrolled in the Imatinib in Pulmonary Arterial Hypertension, a Randomized Efficacy Study (IMPRES) trial and were compared with 30 healthy controls of similar age and sex. We studied the association of RA functional measures with invasive pulmonary hemodynamics, cardiac structure and function, and N-terminal pro brain natriuretic peptide. RA LS and early LS rate were reduced in PAH patients compared with controls (27.1±11.6 versus 56.9±12.7, adjusted P<0.001 and -0.6±0.5 versus -1.5±0.5, adjusted P<0.001, respectively) even after adjusting for RA area and invasive RA pressure, whereas RA late LS rate was similar between groups (-1.4±0.7 versus -1.5±0.4, P=0.42). Among PAH patients, worse RA LS correlated with greater RA size (r=-0.50, P<0.0001) and pressure (r=-0.37, P=0.002), but not pulmonary artery pressure (r=-0.07, P=0.58). Worse RA LS was also associated with right ventricular enlargement and dysfunction and higher N-terminal pro brain natriuretic peptide independent of RA size and pressure.ConclusionsRA reservoir and passive conduit functions are impaired in PAH, independent of RA size and pressure, and likely reflect right ventricular failure and overload.Clinical trial registrationURL: http://www.clinicaltrials.gov. Unique identifier: NCT00902174.
| S-EPMC4629509 | biostudies-literature
Project description:Irreversible pulmonary arterial hypertension is considered a contraindication for surgical or percutaneous closure of atrial septal defects (ASD) due to risk of right heart failure. We present a case of 37 years-old woman who was referred to our center due to progressive worsening fatigue and high probability of pulmonary hypertension on a transthoracic echocardiogram. The diagnostic work-up revealed the presence of an ostium secundum atrial septal defect and severe pre-capillary pulmonary hypertension on right heart cathetherization (RHC). The patient was considered inoperable and started medical therapy with sildenafil and bosentan. After one year of treatment, she repeated RHC that showed a significant reduction in pulmonary vascular resistance making her eligible for closure. Surgical closure of ASD with a fenestra was performed with success. Our case emphasizes the importance of individual assessment even if cases where initial evaluation is unfavorable to closure in accordance with the guidelines.
| S-EPMC9749546 | biostudies-literature
Project description:Restrictive cardiomyopathy (RCM) has a poor prognosis and limited treatment options apart from heart transplantation (HTx). We report on the first-in-human interventional atrial flow regulator (AFR) implantations in 3 children with RCM, leading to marked clinical and hemodynamic improvement. We propose the AFR as bridge to HTx or destination therapy in RCM. (Level of Difficulty: Advanced.).
| S-EPMC9334142 | biostudies-literature