A narrative review of modern approach and outcomes evaluation in congenital heart defects

Abstract

Objective: The purpose of this review is to highlight critical advances and innovative approaches to the most challenging clinical situations in congenital heart surgery, to establish a new perspective from which to evaluate current clinical practice patterns and contemporary United States program ranking systems. Background:The past decades have witnessed substantial advances in the treatment of congenital heart defects. New strategies are deeply rooted in calculated risk-taking innovations. Pioneer surgeons developed, improved and refined critical operative skills and techniques to optimize cardiovascular physiology, decrease operative mortality and improve clinical outcomes.Methods: Unfortunately, in the modern surgical era, supportive environments to allow surgeons to make similar gains and innovative contributions remain scarce. In the current practice, overall procedure volume is prioritized to safeguard quality metrics, including hospital survival and length of stay, surgical complications, and neurocognitive outcomes. As a result, exceptional surgical results have become translated and defined by public ranking systems such as the US News and World Report Best Children's Hospital National Ranking (USNWR) and the Congenital Heart Surgery Database of the Society for Thoracic Surgeons (CHSD-STS), primary based upon early post-operative mortality. This reality places surgeons in a vulnerable position where pressure to achieve a high clinical ranking contrasts with a surgeon definition of "acceptable" surgical risk. Currently, the most frequently used risk stratification tools do not factor in important differences in strategies, such as staged palliation versus complete repair, or bi-ventricular versus uni-ventricular physiology. This favors hospitals pursuing multistage surgical approaches, even if the result is worse long-term morbidity, mortality and increased resource utilization. This economy of ranking-based decision-making causes surgeons either avoid operating altogether or accept less advantageous multi-staged treatment strategies for patients with elevated expected mortality. Such an environment also might present much farther-reaching negative impacts on the growth and development of junior surgeons and trainees, as well as on the pursuit of new surgical innovations to aid future generations of patients.Conclusions: Risk aversive surgical behavior is creating an environment not favorable for the children born with truly complex congenital heart defects.

The pendulum of Fontan fenestration

“…Largely related to better post-operative management, the option of Fontan completion has been extended to different patient populations, including patients undergoing completion at younger age, and with complex congenital heart defects, such as hypoplastic left heart syndrome ( 21 ), heterotaxy syndrome ( 22 ), and single ventricle physiology with associated total anomalous pulmonary venous connection ( 23 ). Because of these changes in the risk stratification of the patients accepted for surgery ( 24 ), the cohort of patients presented for Fontan completion are frequently at high risk for a complicated post-operative course. With the increasing complexity of patients undergoing a Fontan, surgical centers have begun to reconsider the use of fenestration and in some centers, especially those accepting the highest risk cases, it is used nearly universally.…”