Circulation Topic Review Recent Progress in the Understanding and Management of Postoperative Right Ventricular Outflow Tract Dysfunction in Patients With Congenital Heart Disease
نویسنده
چکیده
Palliative surgery for conotruncal congenital cardiovascular defects such as tetralogy of Fallot and truncus arteriosus includes reconstruction of the right ventricular (RV) outflow tract (RVOT). Depending on patient age, anatomy, and other factors, a variety of techniques, prosthetic materials, and valves are used to reconstruct the RVOT, but essentially all become dysfunctional over time, with obstruction, pulmonary regurgitation (PR), or both. One of the challenges of managing patients with dysfunction of a surgically reconstructed RVOT has been balancing the risks of ongoing RVOT dysfunction against the risks and benefits of open heart surgery to replace the pulmonary valve. This calculus is complicated by the fact that the replacement valve will have a finite lifespan that is substantially shorter than the life expectancy of the patient, so the “wait-or-intervene” dilemma will recur again and again. In general, the information used to populate these risk-benefit calculations has been limited and confounded by a variety of factors, so clinicians caring for these patients have often been tasked with making recommendations that are based on inadequate or conflicting data. Moreover, there is such wide and often unexplained variability between patients in how the RV remodels in response to dysfunction of the RVOT that it is difficult to systematize practice and to serve all patients optimally. Given the expanding population of adults with tetralogy of Fallot and other disorders associated with RVOT dysfunction, who constitute a large proportion of adults with significant congenital heart disease,1 this clinical problem will only continue to grow. In recent years, there have been numerous advances in our understanding of and methods for evaluating the physiological and clinical sequelae of RVOT dysfunction, as well as in the conceptual and technical aspects of managing such dysfunction and in therapeutic technologies. Although these advances have not resolved the difficulties that clinicians encounter in managing these patients, they have helped clarify and address some of the uncertainty. The purpose of this review is to summarize some of the fundamental dimensions of this evolution. Clinical and Diagnostic Advances Our understanding of the ramifications of prolonged RVOT dysfunction has been advanced dramatically in recent years, thanks in large part to the dissemination of imaging technologies that allow reliable quantification of RV and RVOT performance and pathophysiology, and to increasingly sophisticated investigations using these and other tools. Many of the important insights have resulted from a more incisive characterization of the extent to which RV dysfunction, electrophysiological abnormalities, and exercise dysfunction are associated with PR and abnormalities of RVOT contraction.2–6 With the emergence of such data, practices have begun to shift toward a more conservative approach to the management of RVOT dysfunction. Namely, pulmonary valve replacement (PVR) in patients with significant PR and consequent RV volume overload is being performed more often, and apparently at a younger age, than was the case even a decade ago, and data on the differential clinical benefits depending on age at PVR appear to support this trend.5 It has long been known that surgical PVR reduces RV volume.7 With the introduction of volumetric cardiac MRI and MRI techniques for measuring flow,8,9 precise quantification of RV volume, RV function, and PR severity became possible and increasingly routine. One of the most confounding questions that arise in virtually every patient with RVOT dysfunction is when to implant or replace the pulmonary valve. The emergence of symptoms does not always correlate with the severity of obstruction, PR, or RV dilation or dysfunction. With the incorporation of MRI into the evaluation of these patients, it became clear that, although PVR reduces RV volume, all ventricles do not respond equally, and normalization of volume depends to some extent on the severity of enlargement before PVR.10 Even when the volume is reduced to normal, however, RV function does not necessarily improve, and PVR at advanced stages of RV dilation does not engender a survival benefit or improve electrophysiological parameters or outcomes.11 These disappointing realizations, along with evidence suggesting that younger age at PVR may lead to more consistent improvement in RV function,5 support the notion that chronic RV volume and/or pressure overload lead to changes that precede or are otherwise uncoupled from severe dilation or manifest dysfunction. Deeper insight into the tissue-level biology will be necessary to elucidate this problem, but other emerging imaging techniques, such as the assessment of global ventricular fibrosis,12 may aid in the understanding of the mechanisms and time frame in which RVOT dysfunction leads to adverse RV remodeling. From the Department of Cardiology, Children’s Hospital Boston, Boston, MA. Correspondence to Doff B. McElhinney, MD, Department of Cardiology, Children’s Hospital, 300 Longwood Ave, Boston, MA 02115. E-mail [email protected] (Circulation. 2012;125:e595-e599.) © 2012 American Heart Association, Inc.
منابع مشابه
Residual problems with repaired tetralogy of fallot.
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Palliative surgery for conotruncal congenital cardiovascular defects such as tetralogy of Fallot and truncus arteriosus includes reconstruction of the right ventricular (RV) outflow tract (RVOT). Depending on patient age, anatomy, and other factors, a variety of techniques, prosthetic materials, and valves are used to reconstruct the RVOT, but essentially all become dysfunctional over time, wit...
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Background Pulmonary artery banding (PAB) is a technique of palliative surgical therapy used by congenital heart surgeons as a staged approach to operative correction of congenital heart defects. Materials and Methods We report 5- year experiences from January 2011 to January 2016 of Imam Reza Hospital center (a tertiary referral hospital in Mashhad city, North East of Iran) that consist of 50 ...
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تاریخ انتشار 2012