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Why would you ever operate on an asymptomatic person? Of course, it must be that you believe you are providing a prognostic benefit, that which outweighs any procedural risk.
Severe pulmonary regurgitation (PR) following repair of congenital right ventricular outflow tract obstruction is a common occurrence, and though well tolerated in childhood and adolescence, it eventually leads to right ventricular (RV) dilatation and dysfunction, conferring an increased risk of arrhythmia, heart failure and death.1 Pulmonary valve replacement (PVR) is indicated at the onset of symptoms, but often patients are asymptomatic even in the presence of significant RV enlargement. Intervention before irreversible myocardial dysfunction manifests is crucial, but the optimal timing remains unclear. It is a stressful decision for clinicians; wait too long and risk irreversible ventricular dysfunction or operate too soon and expose patients to unnecessary risk.
Consensus guidelines recommend PVR in asymptomatic patients with severe PR based primarily on RV volume cut-offs (see table 1).2 These recommendations are based on studies predicting near-normalisation of RV volumes following PVR.3 Operating at these stipulated volumes is believed to improve late prognosis, and since the publication of these putative markers of increased risk, PVR numbers have increased and are being performed at younger ages.4 This is a potential concern, given the existing knowledge gaps.
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PR and the chronic volume load on the RV are thought to be central in the pathophysiology of late cardiovascular complications in this situation, including heart failure, ventricular arrhythmia and sudden cardiac death (see figure …
Contributors DWB and DSC both contributed to the planning and writing of the editorial. DSC is guarantor.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Disclaimer These authors take responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation. The views expressed in the submitted article are his or her own and not an official position of the institution or funder.
Competing interests None declared.
Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.
Patient consent for publication Not required.
Provenance and peer review Commissioned; internally peer reviewed.
- Congenital heart disease