Recent Progress in the Understanding and Management of Postoperative Right Ventricular Outflow Tract Dysfunction in Patients With Congenital Heart Disease

Abstract

alliative 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 AdvancesOur 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][3][4][5][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...

Expansion and Characterization of Neonatal Cardiac Pericytes Provides a Novel Cellular Option for Tissue Engineering in Congenital Heart Disease

“…Despite a constant decline in mortality rate, CHD still represents the primary cause of death among infants in industrialized countries. The definitive therapeutic option for these patients is corrective surgery . However, prostheses currently used to reconstruct complex cardiac defects, including synthetic Gore‐tex material, xenografts and homografts, are unable to match the growth of an infant's heart and they become dysfunctional over time .…”

Experiência Inicial com o Implante Percutâneo da Válvula Melody® no Brasil

“…However, there are studies published in the literature pointing to percutaneous treatment as the best option in selected cases. [26][27][28][29]…”

Adult Congenital Heart Disease

“…11,75 This almost inevitably leaves the patient with significant pulmonary regurgitation, which leads to chronic volume overload, dilatation, and failure of the right ventricle over decades. 76 As the population of patients with repaired TOF lives longer, MRI has become a primary method for follow-up. 11 The benefits of these methods include relative preservation of pulmonic valve function and prevention of a right ventricular surgical scar that may cause dysrhythmia.…”

“…76,79,80 Traditional surgical valve replacement has been the treatment of choice; however, prosthetic valves have finite life spans, and multiple repeat thoracotomies are undesirable. 76,79,80 Traditional surgical valve replacement has been the treatment of choice; however, prosthetic valves have finite life spans, and multiple repeat thoracotomies are undesirable.…”