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Norbert F. Voelkel - One of the best experts on this subject based on the ideXlab platform.
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The Right Ventricle and pulmonary hypertension.
Heart failure reviews, 2016Co-Authors: Mariëlle C. Van De Veerdonk, Harm Jan Bogaard, Norbert F. VoelkelAbstract:In patients with pulmonary hypertension (PH), the primary cause of death is Right ventricular (RV) failure. Improvement in RV function is therefore one of the most important treatment goals. In order to be able to reverse RV dysfunction and also prevent RV failure, a detailed understanding of the pathobiology of RV failure and the underlying mechanisms concerning the transition from a pressure-overloaded adapted Right Ventricle to a dilated and failing Right Ventricle is required. Here, we propose that insufficient RV contractility, myocardial fibrosis, capillary rarefaction, and a disturbed metabolism are important features of a failing Right Ventricle. Furthermore, an overview is provided about the potential direct RV effects of PH-targeted therapies and the effects of RV-directed medical treatments.
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Comprehensive Physiology - Right Ventricle in pulmonary hypertension
Comprehensive Physiology, 2011Co-Authors: Norbert F. Voelkel, Ramesh Natarajan, Jennifer I. Drake, Herman J. BogaardAbstract:During heart development chamber specification is controlled and directed by a number of genes and a fetal heart gene expression pattern is revisited during heart failure. In the setting of chronic pulmonary hypertension the Right Ventricle undergoes hypertrophy, which is likely initially adaptive, but often followed by decompensation, dilatation and failure. Here we discuss differences between the Right Ventricle and the left Ventricle of the heart and begin to describe the cellular and molecular changes which characterize Right heart failure. A prevention and treatment of Right Ventricle failure becomes a treatment goal for patients with severe pulmonary hypertension it follows that we need to understand the pathobiology of Right heart hypertrophy and the transition to Right heart failure.
Daniel Bernstein - One of the best experts on this subject based on the ideXlab platform.
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The vulnerable Right Ventricle.
Current opinion in pediatrics, 2015Co-Authors: Sushma Reddy, Daniel BernsteinAbstract:Purpose of reviewThe Right Ventricle (RV) is uniquely at risk in many patients with repaired or palliated congenital heart disease (CHD) such as tetralogy of Fallot, corrected transposition, single Right Ventricle, and in those with pulmonary hypertension. These patients live with abnormal cardiac l
Robert H. Anderson - One of the best experts on this subject based on the ideXlab platform.
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Double-outlet Right Ventricle revisited.
The Journal of thoracic and cardiovascular surgery, 2017Co-Authors: Ameneh Ebadi, Diane E. Spicer, Carl L. Backer, F. Jay Fricker, Robert H. AndersonAbstract:Abstract Objectives Double-outlet Right Ventricle is a form of ventriculoarterial connection. The definition formulated by the International Society for Nomenclature of Paediatric and Congenital Heart Disease is based on hearts with both arterial trunks supported in their greater part by a morphologically Right Ventricle. Bilateral infundibula and ventricular septal defects are highly debated criteria. This study examines the anatomic controversies surrounding double-outlet Right Ventricle. We show that hearts with double-outlet Right Ventricle can have atrioventricular-to-arterial valvular continuity. We emphasize the difference between the interventricular communication and the zone of deficient ventricular septation. Methods The hearts examined were from the University of Florida in Gainesville; Johns Hopkins All Children's Hospital, St Petersburg, Fla; and Lurie Children's Hospital, Chicago, Ill. Each specimen had at least 75% of both arterial roots supported by the morphologically Right Ventricle, with a total of 100 hearts examined. The morphologic method was used to assess anatomic features, including arterial-atrioventricular valvular continuity, subarterial infundibular musculature, and the location of the hole between the Ventricles. Results Most hearts had fibrous continuity between one of the arterial valves and an atrioventricular valve, with bilateral infundibula in 23%, and intact ventricular septum in 5%. Conclusions Bilateral infundibula are not a defining feature of double-outlet Right Ventricle, representing only 23% of the specimens in our sample. The interventricular communication can have a posteroinferior muscular rim or extend to become perimembranous (58%). Double-outlet Right Ventricle can exist with an intact ventricular septum.
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Double-chambered Right Ventricle: a review.
Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology, 2013Co-Authors: Marios Loukas, Brian Housman, Christa Blaak, Sarah Kralovic, R. Shane Tubbs, Robert H. AndersonAbstract:A double-chambered Right Ventricle is a rare heart defect in which the Right Ventricle is separated into a high-pressure proximal and low-pressure distal chamber. This defect is considered to be congenital and typically presents in infancy or childhood but has been reported to present rarely in adults. It can be caused by the presence of anomalous muscle tissue, hypertrophy of the endogenous trabecular bands, or an aberrant moderator band; all of which will typically result in progressive obstruction of the outflow tract. In this paper, we will discuss the general anatomy of the Right Ventricle, the relevant embryology of the heart, and the presentation, diagnosis, and treatment of a double-chambered Right Ventricle.
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Unnatural history of the Right Ventricle in patients with congenitally malformed hearts.
Cardiology in the Young, 2010Co-Authors: Leo Lopez, Robert H. Anderson, Meryl S. Cohen, Andrew N. Redington, David Nykanen, Daniel J. Penny, John E. Deanfield, Benjamin W. EidemAbstract:The long-term outcome of patients with congenitally malformed hearts involving abnormal Right ventricular morphology and haemodynamics is variable. In most instances, the patients are at risk for Right ventricular failure, in part due to morphological differences between the Right and left Ventricles and their response to chronic volume and pressure overload. In patients after repair of tetralogy of Fallot, and after balloon valvotomy for valvar pulmonary stenosis, pulmonary regurgitation is the most significant risk factor for Right ventricular dysfunction. In patients with a dominant Right Ventricle after Fontan palliation, and in those with systemic Right Ventricles in association with surgically or congenitally corrected transposition, the Right Ventricle is not morphologically capable of dealing with chronic exposure to the high afterload of the systemic circulation. In patients with Ebstein's malformation of the tricuspid valve, the degree of atrialisation of the Right Ventricle determines how well the Right Ventricle will function as the pump for the pulmonary vascular bed.
Herman J. Bogaard - One of the best experts on this subject based on the ideXlab platform.
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Comprehensive Physiology - Right Ventricle in pulmonary hypertension
Comprehensive Physiology, 2011Co-Authors: Norbert F. Voelkel, Ramesh Natarajan, Jennifer I. Drake, Herman J. BogaardAbstract:During heart development chamber specification is controlled and directed by a number of genes and a fetal heart gene expression pattern is revisited during heart failure. In the setting of chronic pulmonary hypertension the Right Ventricle undergoes hypertrophy, which is likely initially adaptive, but often followed by decompensation, dilatation and failure. Here we discuss differences between the Right Ventricle and the left Ventricle of the heart and begin to describe the cellular and molecular changes which characterize Right heart failure. A prevention and treatment of Right Ventricle failure becomes a treatment goal for patients with severe pulmonary hypertension it follows that we need to understand the pathobiology of Right heart hypertrophy and the transition to Right heart failure.
Mohan V Reddy - One of the best experts on this subject based on the ideXlab platform.
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double chambered Right Ventricle presenting in adulthood
The Annals of Thoracic Surgery, 2000Co-Authors: Doff B Mcelhinney, Kanu Chatterjee, Mohan V ReddyAbstract:Abstract Background . Double-chambered Right Ventricle is a form of Right ventricular outflow tract obstruction that develops over time, often in patients with an abnormally short distance between the moderator band and pulmonary valve. This lesion typically presents in childhood or adolescence and is often accompanied by a ventricular septal defect. Only a handful of previous cases have been described in which double-chambered Right Ventricle occurred in adulthood. Methods . Since 1992, three patients more than 30 years old (38, 43, and 66 years of age) have presented at our institution with unusual symptoms or a previous incorrect diagnosis. We reviewed the clinical data in these patients. Results . Presenting symptoms included syncope, angina, and severe dyspnea resembling pulmonary hypertension. In 1 patient, disease was categorized as New York Heart Association class IV, and in the other 2 as class III. Coexisting anomalies included a patent foramen ovale or secundum atrial septal defect in 2 patients, a small ventricular septal defect in 1 (with a probable history of ventricular septal defect in another), and mild aortic regurgitation in 1. All patients required urgent or emergent operations, with peak pressures in the proximal Right ventricular chamber of 135 to 180 mm Hg and severely depressed left ventricular function in 1 patient. Resection of the anomalous Right venticular muscle bundles was achieved through a Right atrial approach in all patients. All patients were alive with improved functional status at follow-up, which was between 15 and 40 months. Conclusions . Right ventricular outflow tract obstruction resulting from a double-chambered Right Ventricle is rare in adults, but when it does occur it can present with unusual symptoms. When evaluating the patient with signs or symptoms of primary Right heart failure, cardiologists should make an effort to image the entire Right heart complex. Subcostal echocardiography can facilitate adequate visualization of the Right Ventricle when it is difficult to distinguish the subpulmonary outflow tract from the parasternal and apical windows.
