Abstract 13374: Larger Atria and Increased Atrial Filling Pressures in Corrected Atrial Septal Defect Patients

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Zarmiga Karunanithi ◽  
Mads Andersen ◽  
Søren Mellemkjær ◽  
Mathias Alstrup ◽  
Farhad Waziri ◽  
...  
Keyword(s):  
Atrial Septal Defect ◽  
Septal Defect ◽  
Holter Monitoring ◽  
Cardiopulmonary Exercise Test ◽  
Right Heart Catheterization ◽  
Morbidity And Mortality ◽  
Volume Index ◽  
Heart Catheterization ◽  
Pulmonary Arterial ◽  
The Right

Introduction: After atrial septal defect (ASD) correction, pulmonary arterial pressures drop and the right-sided chambers start to remodel. Full normalization may not occur, which can explain the increased morbidity and mortality observed later in life. We described cardiac physiology in adults with a corrected ASD in order to understand the long-term morbidity and mortality. Hypothesis: ASD patients have enlarged right atria and increased pulmonary arterial pressures despite correction. Methods: Participants (percutaneously (n=19) and surgically (n=19) corrected ASD patients and 11 controls of similar age) underwent echocardiography, International Physical Activity Questionnaire, right heart catheterization, cardiopulmonary exercise test, and Holter-monitoring 15-20 years after ASD correction as part of a larger study. Echocardiographic measurements and invasive pressures obtained at rest are reported. Results: Right and left atrial end-systolic volumes, volume index, and filling pressures are higher in corrected ASD patients (particularly the surgically corrected) when compared with controls. Conclusion: ASD patients (particularly surgically corrected) have higher atrial volumes compared with healthy matched controls 15-20 years after correction, while still within the normal range. During systole, the peak atrial pressures are increased in ASD corrected patients, which correlate well with the atrial volumes seen on echocardiography. These findings show that cardiac remodeling has occurred post-correction, but full normalization is not reached. The differences in atrial size and filling pressure in corrected ASD compared to that in controls may be a marker of ASDs leading to myocardial disease and explain why ASD patients experience increased morbidity and mortality later in life.

Metalloproteinases and Their Inhibitors Are Associated with Pulmonary Arterial Stiffness and Ventricular Function in Pediatric Pulmonary Hypertension

2021 ◽  
Author(s):  
Michal Schafer ◽  
D. Dunbar Ivy ◽  
Kathleen Nguyen ◽  
Katie L Boncella ◽  
Benjamin S Frank ◽  
...  
Keyword(s):  
Arterial Stiffness ◽  
Cardiac Mri ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Sample Collection ◽  
Protein Levels ◽  
End Diastolic Volume ◽  
Pathologic Features ◽  
Pulmonary Arterial ◽  
The Right

Disturbed balance between matrix metalloproteinases (MMPs) and their respective tissue inhibitors (TIMPs) is a well-recognized pathophysiologic component of pulmonary arterial hypertension (PAH). Both classes of proteases have been associated with clinical outcomes as well as with specific pathologic features of ventricular dysfunction and pulmonary arterial remodeling. The purpose of this study was to evaluate the circulating levels of MMPs and TIMPs in children with PAH undergoing the same day cardiac MRI and right heart catheterization. Children with PAH (n=21) underwent a same-day catheterization, comprehensive cardiac MRI evaluation, and blood sample collection for proteomic analysis. Correlative analysis was performed between protein levels and 1) standard PAH indices from catheterization, 2) cardiac MRI hemodynamics, and 3) pulmonary arterial stiffness. MMP-8 was significantly associated with the right ventricular end-diastolic volume (R = 0.45, P = 0.04). MMP-9 levels were significantly associated with stroke volume (R = -0.49, P = 0.03) and pulmonary vascular resistance (R = 0.49, P = 0.03). MMP-9 was further associated with main pulmonary arterial stiffness evaluated by relative area change (R = -0.79, P < 0.01).TIMP-2 and TIMP-4 levels were further associated with the right pulmonary artery pulse wave velocity (R = 0.51, P = 0.03) and backward compression wave (R = 0.52, P = 0.02), respectively. MMPs and TIMPs warrant further clinically prognostic evaluation in conjunction with the conventional cardiac MRI hemodynamic indices.

scholarly journals Effects of Pulmonary Vascular Resistance Index on Oxygen Saturation in Patients with Atrial Septal Defect

2020 ◽  
Vol 31 (1) ◽  
pp. 66
Author(s):  
Supomo Supomo
Keyword(s):  
Oxygen Saturation ◽  
Atrial Septal Defect ◽  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Medical Records ◽  
Septal Defect ◽  
Resistance Index ◽  
Heart Catheterization ◽  
Pulmonary Vascular Resistance Index ◽  
The Right

<p>Atrial septal defect (ASD) is a congenital lesion in atrium septum. The lesion may cause pulmonary hypertension due to the high pressure in the right ventricle. This condition leads to cyanosis in ASD patient, but the pathophysiology of cyanosis in ASD patient is still unknown. This study aimed to identify the pathophysiology of cyanosis in ASD patients using the Pulmonary Vascular Resistance index (PVRi). The design of this study was retrospective cohort study. The data used in this study were the results of right heart catheterization procedure taken from forty ASD patient medical records at Dr. Sardjito general hospital. The exclusions criteria were the history of previous vasodilator administration and incomplete medical records. The median age of the patients was 30 (18-55) years old. The mean of the Qp/Qs ratio was 1.210 (0.57-6.33). Optimum oxygen saturation was found in vessel leaving the heart. The PVRi median is 61.98 (-15.58-676.64). The PVRi has a significant correlation with oxygen saturation, except in the right atrium. There is a significant correlation between PVRi and oxygen saturation in various heart chambers. Pathophysiology of cyanosis in ASD patients is central cyanosis.</p>

scholarly journals The Accuracy of Combined Electrocardiogram Criteria to Diagnose Right Atrial Enlargement in Adults With Uncorrected Secundum Atrial Septal Defect

2019 ◽  
Vol 13 ◽  
pp. 117954681986994
Author(s):  
Purwati Pole Rio ◽  
Hariadi Hariawan ◽  
Dyah Wulan Anggrahini ◽  
Anggoro Budi Hartopo ◽  
Lucia Kris Dinarti
Keyword(s):  
Atrial Septal Defect ◽  
Wave Height ◽  
Transthoracic Echocardiography ◽  
Predictive Value ◽  
Septal Defect ◽  
P Wave ◽  
Right Atrial ◽  
Volume Index ◽  
Heart Catheterization ◽  
Ecg Criteria

Background: Right atrium (RA) enlargement in uncorrected atrial septal defect (ASD) is due to chronic volume overload. Several electrocardiogram (ECG) criteria had been proposed for screening RA enlargement. This study aimed to compare the accuracy of ECG criteria in detecting RA enlargement in adults with uncorrected ASD. Methods: This was a cross-sectional study involving 120 adults with uncorrected secundum ASD. The subjects underwent ECG examination, transthoracic echocardiography, and right heart catheterization. An RA enlargement was determined with RA volume index by transthoracic echocardiography. Various ECG and combined ECG criteria were evaluated. Statistical analysis was performed to analyze the sensitivity, specificity, accuracy, positive predictive value (PPV), and negative predictive value (NPV). Results: An RA enlargement was detected in 64.2% subjects. The P wave height > 2.5 mm in lead II criterion had the best specificity (100%) and PPV (100%), but low sensitivity (19%) and accuracy (48%). The combined 2 ECG criteria (QRS axis > 90°, R/S ratio > 1 in V1) had 82% sensitivity, 56% specificity, 73% accuracy, 77% PPV, and 63% NPV. The combined 3 ECG criteria (QRS axis > 90°, R/S ratio > 1 in V1, and P wave height > 1.5 mm in V2) had 35% sensitivity, 86% specificity, 53% accuracy, 82% PPV, and 43% NPV. Conclusions: The combined 2 ECG criteria (QRS axis > 90° and R/S ratio > 1 in V1) had increased sensitivity, better accuracy, and more balance of PPV and NPV as compared with P wave > 2.5 mm in II criterion and combined 3 ECG criteria to diagnose RA enlargement in adults with uncorrected ASD.

scholarly journals Effects of oxygen therapy in a pediatric normoxemic patient with pulmonary arterial hypertension and congenital heart disease

2019 ◽  
Vol 9 (2) ◽  
pp. 1177_2045894019 ◽  
Author(s):  
Simon Green ◽  
Deidre Stuart
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Oxygen Therapy ◽  
Septal Defect ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Mean Pulmonary Arterial Pressure ◽  
Rapid Fall ◽  
Pulmonary Arterial ◽  
Observation Period

To shed light on the efficacy of oxygen therapy in pulmonary arterial hypertension (PAH) when hypoxemia is not present, we report seven years of observational data for a female patient recorded between February 2012 and February 2019 when she was aged 7.8–14.8 years. The patient was born with an atrial septal defect (closed spontaneously at 2.3 years) and ventricular septal defect (surgically repaired at 8.3 years) and then diagnosed with PAH at 8.9 years . The patient was prescribed bosentan soon after diagnosis and for the next 4.8 years, during which a first phase of oxygen therapy (nocturnal) was trialed for 2.8 years. Mean pulmonary arterial pressure (mPAP) and systolic PAP (sPAP) remained stable and at mild levels when oxygen was administered, but then increased progressively to severe levels over two years without oxygen. This coincided with worsening right ventricular pathology during the later part of this period without oxygen. Re-initiation of more intensive oxygen therapy while the patient was still on bosentan and before pharmocotherapy was changed coincided with a large and rapid fall in sPAP, confirmed by right heart catheterization measurements of mPAP. During this entire observation period, the patient remained normoxemic. These observations challenge the notion that oxygen therapy should be restricted to patients with hypoxemia and strengthen calls for further study of oxygen therapy in PAH.

scholarly journals A Zero-Dimensional Model and Protocol for Simulating Patient-Specific Pulmonary Hemodynamics From Limited Clinical Data

2016 ◽  
Vol 138 (12) ◽  
Author(s):  
Vitaly O. Kheyfets ◽  
Jamie Dunning ◽  
Uyen Truong ◽  
Dunbar Ivy ◽  
Kendall Hunter ◽  
...  
Keyword(s):  
Clinical Data ◽  
Right Heart Catheterization ◽  
Patient Specific ◽  
Heart Catheterization ◽  
Functional Markers ◽  
Clinical Implementation ◽  
Pulmonary Hemodynamics ◽  
Pulmonary Arterial ◽  
The Right ◽  
Initial Results

In pulmonary hypertension (PH) diagnosis and management, many useful functional markers have been proposed that are unfeasible for clinical implementation. For example, assessing right ventricular (RV) contractile response to a gradual increase in pulmonary arterial (PA) impedance requires simultaneously recording RV pressure and volume, and under different afterload/preload conditions. In addition to clinical applications, many research projects are hampered by limited retrospective clinical data and could greatly benefit from simulations that extrapolate unavailable hemodynamics. The objective of this study was to develop and validate a 0D computational model, along with a numerical implementation protocol, of the RV–PA axis. Model results are qualitatively compared with published clinical data and quantitatively validated against right heart catheterization (RHC) for 115 pediatric PH patients. The RV–PA circuit is represented using a general elastance function for the RV and a three-element Windkessel initial value problem for the PA. The circuit mathematically sits between two reservoirs of constant pressure, which represent the right and left atriums. We compared Pmax, Pmin, mPAP, cardiac output (CO), and stroke volume (SV) between the model and RHC. The model predicted between 96% and 98% of the variability in pressure and 98–99% in volumetric characteristics (CO and SV). However, Bland Altman plots showed the model to have a consistent bias for most pressure and volumetric parameters, and differences between model and RHC to have considerable error. Future studies will address this issue and compare specific waveforms, but these initial results are extremely promising as preliminary proof of concept of the modeling approach.

COMPARATIVE EVALUATION OF IMMEDIATE RESULTS OF PLASTIC ATRIAL SEPTAL DEFECT IN ADULT PATIENTS, LIVING IN HIGH ALTITUDE CONDITIONS

2021 ◽  
pp. 16-21
Author(s):  
Kaldarbek Abdramanov ◽  
◽  
Emilbek Kokoev ◽  
Emir Alimbekov ◽  
Parida Arzibaeva ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Atrial Septal Defect ◽  
High Altitude ◽  
Septal Defect ◽  
Adult Patients ◽  
Control Group ◽  
Observation Data ◽  
Clinical Methods ◽  
Pulmonary Arterial ◽  
The Right

The article gives a comparative assessment of the results of surgery for atrial septal defect (ASD) surgery in patients living in low and high altitude conditions. sizes of the right ventricle were practically equal, but at the same time higher than the norm. Objective. To conduct a comparative analysis of the direct results of ASD plastic in patients living in low, medium and high mountains Material and methods. The study material included 30 patients with ASD living in highlands; the control group consisted of 30 patients living in flat areas. Using clinical methods and instrumental studies, an analysis of operated patients, ASD plastic in adult patients with complicated pulmonary hypertension, heart failure, cardiac arrhythmias and functional tricuspid insufficiency was performed. Results. The authors studied pulmonary arterial pressure (PAP) indices between the groups both in the preoperative and postoperative periods, the analysis showed significant differences. The parameters of the right ventricle (RV) in the preoperative period had significant differences, however, in the postoperative period, the sizes of the right ventricle were practically equal, but at the same time higher than the norm. Conclusion. Our observation data show a significant decrease in PAP in patients living in high altitude conditions. However, the decrease in the PAP indicators remained slightly higher than the standard indicators.

A novel right-heart catheterization technique for in vivo measurement of vascular responses in lungs of intact mice

2000 ◽  
Vol 278 (1) ◽  
pp. H8-H15 ◽  
Author(s):  
Hunter C. Champion ◽  
Douglas J. Villnave ◽  
Allen Tower ◽  
Philip J. Kadowitz ◽  
Albert L. Hyman
Keyword(s):  
Arterial Pressure ◽  
Pulmonary Arterial Pressure ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Catheterization Technique ◽  
Pulmonary Arterial ◽  
The Right ◽  
Spontaneously Breathing

The present study employed a new right-heart catheterization technique to measure pulmonary arterial pressure, pulmonary arterial wedge pressure, and pulmonary vascular resistance in anesthetized intact-chest, spontaneously breathing mice. Under fluoroscopic guidance, a specially designed catheter was inserted via the right jugular vein and advanced to the main pulmonary artery. Cardiac output was determined by the thermodilution technique, and measured parameters were stable for periods of ≤3 h. Pressure-flow curves in vivo were curvilinear, with mean pulmonary arterial pressure increasing more rapidly at low pulmonary blood flows of 5–10 ml/min and less rapidly at higher blood flow rates. The pressure-flow relationship was shifted to the left by the nitric oxide synthase inhibitor nitro-l-arginine methyl ester (l-NAME) at higher blood flow levels, whereas the cyclooxygenase inhibitor sodium meclofenamate was without effect. The increase in pulmonary arterial pressure in response to acute hypoxia (fractional inspired O2 10%) was augmented byl-NAME but unaltered by sodium meclofenamate. The present results demonstrate that the right-heart catheterization technique can be used to measure pulmonary vascular pressures and responses in the mouse. This is, to our knowledge, the first report of a right-heart catheterization technique to measure pulmonary vascular pressures and responses in the intact-chest, spontaneously breathing mouse and should prove useful for the investigation of pulmonary vascular responses in transgenic mice.

scholarly journals Sinus Venosus Atrial Septal Defect Complicated by Eisenmenger Syndrome and the Role of Vasodilator Therapy

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Amornpol Anuwatworn ◽  
Maheedhar Gedela ◽  
Edgard Bendaly ◽  
Julia A. Prescott-Focht ◽  
Jimmy Yee ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Primary Treatment ◽  
Sinus Venosus ◽  
Eisenmenger Syndrome ◽  
Vasodilator Therapy ◽  
Septal Defects ◽  
Pulmonary Arterial ◽  
The Right

Sinus venosus atrial septal defect is a rare congenital, interatrial communication defect at the junction of the right atrium and the vena cava. It accounts for 5–10% of cases of all atrial septal defects. Due to the rare prevalence and anatomical complexity, diagnosing sinus venous atrial septal defects poses clinical challenges which may delay diagnosis and treatment. Advanced cardiac imaging studies are useful tools to diagnose this clinical entity and to delineate the anatomy and any associated communications. Surgical correction of the anomaly is the primary treatment. We discuss a 43-year-old Hispanic female patient who presented with dyspnea and hypoxia following a laparoscopic myomectomy. She had been diagnosed with peripartum cardiomyopathy nine years ago at another hospital. Transesophageal echocardiography and computed tomographic angiography of the chest confirmed a diagnosis of sinus venosus atrial septal defect. She was also found to have pulmonary arterial hypertension and Eisenmenger syndrome. During a hemodynamic study, she responded to vasodilator and she was treated with Ambrisentan and Tadalafil. After six months, her symptoms improved and her pulmonary arterial hypertension decreased. We also observed progressive reversal of the right-to-left shunt. This case illustrates the potential benefit of vasodilator therapy in reversing Eisenmenger physiology, which may lead to surgical repair of the atrial septal defect as the primary treatment.

Nerve growth factor receptor is involved in maintaining homeostasis of pulmonary arterial hypertension

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
C Goten ◽  
S Usui ◽  
O Inoue ◽  
H Okada ◽  
S Takashima ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Chronic Hypoxia ◽  
Systolic Pressure ◽  
Growth Factor Receptor ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Ventricular Systolic Pressure ◽  
Ventricular Systolic Pressure ◽  
Pulmonary Arterial ◽  
The Right

Abstract Introduction Pulmonary arterial hypertension (PAH), characterized by vascular remodeling, is still disease with poor prognosis although many pulmonary vasodilators have been developed, and new mechanism of treatment for PAH is desired. Nerve growth factor receptor (Ngfr) is known to relate to inflammatory reaction and repair process in the damaged tissue. We have reported that Ngfr is associated to vascular remodeling in patients with acute coronary syndrome. However, it is unclear how Ngfr is involved in the pathogenesis of PAH. Purpose In this study, we investigated whether Ngfr relate to pathophysiology in PAH. Methods We estimated the frequency of Ngfr positive cells (% Ngfr+) in peripheral blood mononuclear cells obtained from PAH and non-PAH patients using flowcytometric analysis. In PAH patients, the hemodynamic parameters such as mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance (PVR), and cardiac index (CI) were obtained by right heart catheterization, and evaluated for correlation with the % Ngfr+. Next, adult 8-week-old C57BL/6 (WT) mice and Ngfr knock out (KO) mice were exposed to chronic hypoxia (10% O2) or normoxia for 6 weeks. Then, mice were anesthetized and performed echocardiography and right heart catheterization. Then, mice were exsanguinated and blood sample was collected to evaluate the % Ngfr+ by flow cytometry. Right ventricular weight was measured and lung tissue was also collected for histological assessment and molecular pathway profiling. Results PAH (n=24) patients and non-PAH patients (n=17) were enrolled. The % Ngfr+ was significantly higher in PAH patients than that in non-PAH patients (0.056% versus 0.019%, p&lt;0.0001). In PAH patients, the % Ngfr+ was correlated with severity of hemodynamic parameters such as mPAP (R=0.64 p&lt;0.001), PVR (R=0.62 p&lt;0.005), and CI (R=−0.48 p&lt;0.05). In WT mice, chronic hypoxia significantly increased the right ventricular systolic pressure and induced vascular medial thickness and fibrosis around the pulmonary artery. Flow cytometry analysis revealed that the % Ngfr+ was significantly increased in the hypoxia compared to that in the normoxia. Under hypoxic conditions, the right ventricular systolic pressure was significantly increased in Ngfr KO mice compared to that in WT mice. In histological analysis, hypoxia-induced peripheral vascular fibrosis and medial thickness was more severe in Ngfr KO than that in WT mice. Conclusion Circulating Ngfr-positive cells are associated with severity of PAH in patients. In the hypoxia-induced PH model, gene deletion of Ngfr shows the progression of the pathogenesis of PAH. These results suggest that circulating Ngfr-positive cells have an important role in the pathogenesis of PAH and may be a novel target for PAH therapy. Funding Acknowledgement Type of funding source: None

scholarly journals A rare case of quadrileaflet mitral valve and ostium primum atrial septal defect

2021 ◽  
Author(s):  
Corrado Fiore ◽  
Tugba Kemaloglu Oz ◽  
Luigi Lombardi ◽  
Rebani Sinani ◽  
Renato Gregorini ◽  
...  
Keyword(s):  
Mitral Valve ◽  
Atrial Septal Defect ◽  
Rare Case ◽  
Septal Defect ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Progressive Dyspnea ◽  
History Of ◽  
Asd Closure

A 60-year-old female was referred to our clinic for evaluation of her rapidly progressive dyspnea, she had no previuos history of heart disease. A murmur was noted on her examination and transthoracic echocardiography was so difficult to be performed due to poor acoustic windows so she was referred to do a transesophageal echocardiography that showed an ostium primum atrial septal defect (ASD) with left to right shunt and a quadrileaflet mitral valve with severe regurgitation. Later on, she underwent surgery with Ostium Primum ASD closure by a patch and double cleft repair by suture after right heart catheterization.

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