scholarly journals Early right ventricular fibrosis and reduction in biventricular cardiac reserve in the dystrophin-deficient mdx heart

2015 ◽  
Vol 308 (4) ◽  
pp. H303-H315 ◽  
Author(s):  
Tatyana A. Meyers ◽  
DeWayne Townsend
Keyword(s):  
Right Ventricle ◽  
Right Ventricular ◽  
Cardiac Fibrosis ◽  
Striated Muscle ◽  
Mdx Mice ◽  
Catheterization Technique ◽  
Dystrophic Cardiomyopathy ◽  
Systolic And Diastolic Function ◽  
Ventricular Fibrosis ◽  
The Right

Duchenne muscular dystrophy (DMD) is a progressive disease of striated muscle deterioration. Respiratory and cardiac muscle dysfunction are particularly clinically relevant because they result in the leading causes of death in DMD patients. Despite the clinical and physiological significance of these systems, little has been done to understand the cardiorespiratory interaction in DMD. We show here that prior to the onset of global cardiac dysfunction, dystrophin-deficient mdx mice have increased cardiac fibrosis with the right ventricle being particularly affected. Using a novel biventricular cardiac catheterization technique coupled with cardiac stress testing, we demonstrate that both the right and left ventricles have significant reductions in both systolic and diastolic function in response to dobutamine. Unstimulated cardiac function is relatively normal except for a significant reduction in the ventricular pressure transient duration compared with controls. These biventricular analyses also reveal the absence of a dobutamine-induced increase in isovolumic relaxation in the right ventricle of control hearts. Simultaneous assessment of biventricular pressure demonstrates a dobutamine-dependent enhancement of coupling between the ventricles in control mice, which is absent in mdx mice. Furthermore, studies probing the passive-extension properties of the left ventricle demonstrate that the mdx heart is significantly more compliant compared with age-matched C57BL/10 hearts, which have an age-dependent stiffening that is completely absent from dystrophic hearts. These new results indicate that right ventricular fibrosis is an early indicator of the development of dystrophic cardiomyopathy, suggesting a mechanism by which respiratory insufficiency may accelerate the development of heart failure in DMD.

Pulmonary Vascular Versus Right Ventricular Function Changes During Targeted Therapies of Pulmonary Hypertension – An Argument for Upfront Combination Therapy?

2012 ◽  
Vol 8 (3) ◽  
pp. 209
Author(s):  
Wouter Jacobs ◽  
Anton Vonk-Noordegraaf ◽  
◽  
Keyword(s):  
Combination Therapy ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Meta Analysis ◽  
Right Heart Failure ◽  
Functional Class ◽  
Pulmonary Vasculature ◽  
The Right

Pulmonary arterial hypertension is a progressive disease of the pulmonary vasculature, ultimately leading to right heart failure and death. Current treatment is aimed at targeting three different pathways: the prostacyclin, endothelin and nitric oxide pathways. These therapies improve functional class, increase exercise capacity and improve haemodynamics. In addition, data from a meta-analysis provide compelling evidence of improved survival. Despite these treatments, the outcome is still grim and the cause of death is inevitable – right ventricular failure. One explanation for this paradox of haemodynamic benefit and still worse outcome is that the right ventricle does not benefit from a modest reduction in pulmonary vascular resistance. This article describes the physiological concepts that might underlie this paradox. Based on these concepts, we argue that not only a significant reduction in pulmonary vascular resistance, but also a significant reduction in pulmonary artery pressure is required to save the right ventricle. Haemodynamic data from clinical trials hold the promise that these haemodynamic requirements might be met if upfront combination therapy is used.

CMR markers for early right ventricular dysfunction in precapillary pulmonary hypertension

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J Vos ◽  
T Leiner ◽  
A.P.J Van Dijk ◽  
F.J Meijboom ◽  
G.T Sieswerda ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Circumferential Strain ◽  
Peak Strain ◽  
Healthy Controls ◽  
Free Wall ◽  
Global Circumferential Strain ◽  
The Right ◽  
Rv Function

Abstract Introduction Precapillary pulmonary hypertension (pPH) causes right ventricular (RV) pressure overload inducing RV remodeling, often resulting in dysfunction and dilatation, heart failure, and ultimately death. The ability of the right ventricle to adequately adapt to increased pressure loading is key for patients' prognosis. RV ejection fraction (RVEF) by cardiac magnetic resonance (CMR) is related to outcome in pPH patients, but this global measurement is not ideal for detecting early changes in RV function. Strain analysis on CMR using feature tracking (FT) software provides a more detailed assessment, and might therefore detect early changes in RV function. Aim 1) To compare RV strain parameters in pPH patients and healthy controls, and 2) to compare strain parameters in a subgroup of pPH patients with preserved RVEF (pRVEF) and healthy controls. Methods In this prospective study, a CMR was performed in pPH patients and healthy controls. Using FT-software on standard cine images, the following RV strain parameters were analyzed: global, septal, and free wall longitudinal strain (GLS, sept-LS, free wall-LS), time to peak strain (TTP, as a % of the whole cardiac cycle), the fractional area change (FAC), global circumferential strain (GCS), global longitudinal and global circumferential strain rate (GLSR and GCSR, respectively). A pRVEF is defined as a RVEF >50%. To compare RV strain parameters in pPH patients to healthy controls, the Mann-Whitney U test was used. Results 33 pPH-patients (55 [45–63] yrs; 10 (30%) male) and 22 healthy controls (40 [36–48] yrs; 15 (68%) male) were included. All RV strain parameters were significantly reduced in pPH patients compared to healthy controls (see table), except for GCS and GCSR. Most importantly, in pPH patients with pRVEF (n=8) GLS (−26.6% [−22.6 to −27.3] vs. −28.1% [−26.2 to −30.6], p=0.04), sept-LS (−21.2% [−19.8 to −23.2] vs. −26.0% [−24.0 to −27.9], p=0.005), and FAC (39% [35–44] vs. 44% [42–47], p=0.02) were still significantly impaired compared to healthy controls. The RV TTP was significantly increased in pPH patients compared to healthy controls (47% [44–57] vs. 40% [33–43], p≤0.001). Conclusions Several CMR-FT strain parameters of the right ventricle are impaired in pPH patients when compared to healthy controls. Moreover, even in pPH patients with a preserved RVEF multiple RV strain parameters (GLS, sept-LS, and FAC) remained significantly impaired, and TTP significantly prolonged, in comparison to healthy controls. This suggests that RV strain parameters may be used as an early marker of RV dysfunction in pPH patients. Funding Acknowledgement Type of funding source: None

scholarly journals Repair of double-chambered right ventricle using right ventricular outflow chamber ventriculotomy via left intercostal thoracotomy under beating heart in two dogs

2014 ◽  
Vol 4 (1) ◽  
Author(s):  
Keiichi Sato ◽  
Isamu Kanemoto ◽  
Kippei Mihara ◽  
Koudai Kawase ◽  
Takuya Mori ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Right Ventricular ◽  
Continuous Wave ◽  
Clinical Signs ◽  
Major Complication ◽  
Beating Heart ◽  
Moderate Hypothermia ◽  
Continuous Wave Doppler ◽  
Novel Approach ◽  
The Right

Double-chambered right ventricle was diagnosed in two dogs, one of them a pup and the other full grown. Both dogs underwent surgery using the novel approach of right ventricular outflow chamber ventriculotomy via left intercostal thoracotomy with moderate hypothermia and moderate pump flow cardiopulmonary bypass under beating heart. No major complication occurred during and after the operation. On continuous wave Doppler echocardiography, the pressure gradient across the stenosis in the right ventricle decreased from 130 mmHg pre-operatively to 40 mmHg post-operatively at 1 year 5 months in the adult dog, and from 209 mmHg pre-operatively to 47 mmHg post-operatively at 1 year in the pup. Both dogs are active without clinical signs.

scholarly journals Metastatic adenocarcinoma involving the right ventricle and pulmonary artery leading to right heart failure: case report

2017 ◽  
Vol 136 (3) ◽  
pp. 262-265 ◽  
Author(s):  
Turgut Karabag ◽  
Caner Arslan ◽  
Turab Yakisan ◽  
Aziz Vatan ◽  
Duygu Sak
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Right Ventricular Outflow Tract ◽  
Right Heart Failure ◽  
Ventricular Outflow Tract ◽  
Right Heart ◽  
The Right

ABSTRACT CONTEXT: Obstruction of the right ventricular outflow tract due to metastatic disease is rare. Clinical recognition of cardiac metastatic tumors is rare and continues to present a diagnostic and therapeutic challenge. CASE REPORT: We present the case of a patient who had severe respiratory insufficiency and whose clinical examinations revealed a giant tumor mass extending from the right ventricle to the pulmonary artery. We discuss the diagnostic and therapeutic options. CONCLUSION: In patients presenting with acute right heart failure, right ventricular masses should be kept in mind. Transthoracic echocardiography appears to be the most easily available, noninvasive, cost-effective and useful technique in making the differential diagnosis.

P3527Echocardiographic assessment of the right ventricle in chronic heart failure with focus on patients with atrial fibrillation

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
E Majos ◽  
A Kraska ◽  
I Kowalik ◽  
E Smolis-Bak ◽  
H Szwed ◽  
...  
Keyword(s):  
Heart Failure ◽  
Atrial Fibrillation ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Systolic Function ◽  
Pressure Overload ◽  
Ventricular Ejection Fraction ◽  
Rv Pacing ◽  
The Right ◽  
Rv Function

Abstract Background Assessment of the right ventricle (RV) in heart failure (HF) is challenging and requires applicable methods and parameters. Atrial fibrillation (AF) is a common and clinically significant arrhythmia in 30–50% of HF patients. Assessment of the RV function in patients with AF is problematic. Still little is known about RV function in HF and AF patients. The aim of the study was to assess RV function in HF with focus on AF patients. Methods Patients with HF of ischemic etiology, NYHA II-III, LVEF ≤40%, with AF and sinus rhythm (SR), underwent two- and three- dimensional echocardiography (2DE and 3DE) for assessment of the RV with use of multiple parameters. The RV was examined for: linear dimensions, end-diastolic and end-systolic areas adjusted to body surface area (RV EDA and RV ESA/BSA) and end-diastolic and end-systolic volumes adjusted to lean body mass (RV EDV and RV ESV/LBM) to reflect volume overload and in terms of right ventricular pressure (RVSP) as an index of pressure overload. RV systolic function was assessed with 2DE: tricuspid annular plane systolic excursion (TAPSE), right ventricular fractional area change (RV FAC), tricuspid lateral annular systolic velocity (s') and 3DE parameters: right ventricular ejection fraction (RVEF) and free wall right ventricular longitudinal strain (FW RVLS). Also, TAPSE/RVSP parameter was included. Results The study included 126 patients: 94 with AF and 32 with SR. Within the AF group 28 patients were treated medically, 41 had RV pacing (pacemaker or an implantable cardioverter-defibrillator, ICD) and 25 had cardiac resynchronisation therapy (CRT). In comparison with SR group AF patients had: larger RV inflow tract dimension (4.49±0.85 vs. 3.95±0.72 cm; p=0.0017), RV EDA/BSA (12.7±3.9 vs. 11.1±3.0 cm2/m2; p=0.0358) and RV ESA/BSA (8.0±3.0 vs. 6.7±2.4 cm2/m2; p=0.0226). Similarly, patients with AF had greater RV volumes in 3DE than patients with SR: RV EDV/LBM (1.82±0.60 vs. 1.61±0.38ml/kg, p=0.0267) and RV ESV/LBM (1.11±0.40 ml/kg vs. 0.81±0.28, p<0,0001). Also, in patients with AF right ventricular systolic pressure (RVSP) was higher (40.8±10.2 vs. 34.0±8.1 mmHg, p=0,0010). No differences in TAPSE and RVFAC were found but the relation TAPSE/RVSP was higher in AF than in SR group (0.51±0.21 vs. 0.65±0.24 cm/mmHg; p=0.0046). Also, in AF patients in comparison to SR group some parameters had worse values: s' (9.7±2.31 vs. 12.1±3.83, p=0.014), RVEF (37.2±7.3 vs. 48.2±7.5, p<0.0001 and FW RVLS (−18.3±4.6 vs. −23.9±4.23%, p<0,0001). Within the AF group no significant differences in studied variables depending on RV pacing or CRT were found. Conclusions Larger volumes and higher pressure overload of the RV were observed in patients with AF in comparison to SR. Systolic function of the RV seems to be more depressed in AF compared to SR patients with systolic heart failure. Further research in larger groups is required to identify the most applicable and valuable methods of RV evaluation.

scholarly journals Late follow-up of Persistent Truncus Arteriosus, after one-stage Repair, with Right Ventricular Remodeling.

2021 ◽  
Vol 4 (5) ◽  
pp. 01-04
Author(s):  
Miguel Maluf
Keyword(s):  
Right Ventricle ◽  
Right Ventricular ◽  
Ventricular Remodeling ◽  
Surgical Repair ◽  
Pulmonary Valve ◽  
Truncus Arteriosus ◽  
Persistent Truncus Arteriosus ◽  
The Right ◽  
Right Ventricular Remodeling

We report long-term outcome after one-stage, surgical repair, in a two months-old girl with persistent truncus arteriosus type I, II. The operation was carried out with the remodeling of the right ventricle, using a swine bicuspid pulmonary prosthesis. Twenty-six years later, the patient is in excellent clinical condition, CF I (NYHA), with normal peripheral oxygen saturation. Recent invasive and not invasive imaging show: absence of intracardiac shunt and growing of the right ventricle outlet tract and discrete double lesion of the pulmonary valve. The pulmonary flow directed uniformly for both lungs. In selected cases, the long-term prognosis of patients with persistent truncus arteriosus, undergoing early surgical repair, avoiding the use of valved conduit, makes for an excellent evolution, without new interventions. Endovascular procedures, now well standardized, for the implantation of a pulmonary valve stent, through a catheter, will allow an effective approach, in the presence of late obstructions, in patients who have undergone right ventricular remodeling, without the use of valved conduits.

Levels of brain natriuretic peptide in children with right ventricular overload due to congenital cardiac disease

2005 ◽  
Vol 15 (4) ◽  
pp. 396-401 ◽  
Author(s):  
Thomas S. Mir ◽  
Jan Falkenberg ◽  
Bernd Friedrich ◽  
Urda Gottschalk ◽  
Throng Phi Lê ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Brain Natriuretic Peptide ◽  
Right Ventricular ◽  
Natriuretic Peptide ◽  
Cardiac Disease ◽  
Left Ventricular Pressure ◽  
Left Ventricular ◽  
Ventricular Pressure ◽  
Congenital Cardiac Disease ◽  
The Right

Objective:To evaluate the role of the concentration of brain natriuretic peptide in the plasma, and its correlation with haemodynamic right ventricular parameters, in children with overload of the right ventricle due to congenital cardiac disease.Methods:We studied 31 children, with a mean age of 4.8 years, with volume or pressure overload of the right ventricle caused by congenital cardiac disease. Of the patients, 19 had undergone surgical biventricular correction of tetralogy of Fallot, 11 with pulmonary stenosis and 8 with pulmonary atresia, and 12 patients were studied prior to operations, 7 with atrial septal defects and 5 with anomalous pulmonary venous connections. We measured brain natriuretic peptide using Triage®, from Biosite, United States of America. We determined end-diastolic pressures of the right ventricle, and the peak ratio of right to left ventricular pressures, by cardiac catheterization and correlated them with concentrations of brain natriuretic peptide in the plasma.Results:The mean concentrations of brain natriuretic peptide were 87.7, with a range from 5 to 316, picograms per millilitre. Mean end-diastolic pressure in the right ventricle was 5.6, with a range from 2 to 10, millimetres of mercury, and the mean ratio of right to left ventricular pressure was 0.56, with a range from 0.24 to 1.03. There was a positive correlation between the concentrations of brain natriuretic peptide and the ratio of right to left ventricular pressure (r equal to 0.7844, p less than 0.0001) in all patients. These positive correlations remained when the children with tetralogy of Fallot, and those with atrial septal defects or anomalous pulmonary venous connection, were analysed as separate groups. We also found a weak correlation was shown between end-diastolic right ventricular pressure and concentrations of brain natriuretic peptide in the plasma (r equal to 0.5947, p equal to 0.0004).Conclusion:There is a significant correlation between right ventricular haemodynamic parameters and concentrations of brain natriuretic peptide in the plasma of children with right ventricular overload due to different types of congenital cardiac disease. The monitoring of brain natriuretic peptide may provide a non-invasive and safe quantitative follow up of the right ventricular pressure and volume overload in these patients.

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