Surgical management of Ebstein anomaly: impact of the adult congenital heart disease anatomical and physiological classifications

Surgical management of Ebstein anomaly: impact of the adult congenital heart disease anatomical and physiological classifications

Homzova L, Photiadis J, Sinzobahamvya N, Ovroutski S, Cho M-Y, Schulz A. Interact CardioVasc Thorac Surg 2021;32:593–600.

Take home message:

  • Physiological assessment of patients with Ebstein anomaly is important when referring patients for surgery
  • Survival free from complications is lower for patients with a preoperative severe physiological condition (compared to a moderate one)
  • Patients with Ebstein anomaly should undergo surgery before they reach a severe clinical condition

Commentary from Dr. Frederic Jacques (Quebec City, QC, Canada), chief section editor of Congenital Heart Surgery Journal Watch: 

Homzova L. et al. report on a cohort of 33 patients operated for Ebstein anomaly from 2000 to 2017. They divided the small group in two according to clinical preoperative presentation as defined by the adult congenital heart disease anatomical and physiological classification. As the reader knows, this classification comprises elements such as the NYHA classification, the exercise capacity, the presence of arrhythmias and pulmonary hypertension, and even end-organ dysfunction. It gives a physiological portrait of the patient rather than simply relying on the name of the diagnosis and its subtypes. They compared the 2-year survival of patients having a moderate or a severe pre-operative condition. They also compared postoperative major complications. Their main finding is that survival free from major adverse events was lower among patients with the worse preoperative condition, particularly in women. The survival free from major adverse events was 60% in the moderate group, compared to 38% in the severe group. In fact, only two patients died in the immediate postoperative period and both were female. Another female patient died 8 months after surgery. Seven patients required re-operations, and 5 required a pacemaker. Early tricuspid re-operation was required in 1 patient. One patient had a stroke and 1 required mechanical support. All of these patients were in the severe group. One patient of the severe group was listed for heart transplant. Survival free from re-operation was 72% in the moderate and 46% in the severe group. Of the survivors, 18% had improved to mild clinical condition, and the remaining 82% were in a moderate condition. Overall, the authors propose to consider operating patients before reaching a severe clinical condition.

This study is small and limited in its ability to exert authority in our clinical decision making regarding Ebstein anomaly patients. Nevertheless, the authors should be commended in their efforts to improve our understanding of the physiological repercussions of a long-standing tricuspid valve anomaly. In fact, their study supports the assumption that most clinicians have: patients should be brought to the operating room before they reach a condition were the outcome will be significantly affected.

For Ebstein anomaly, the indication and the appropriate timing for surgery are difficult to define. In fact, indication and the timing for surgery are difficult for the tricuspid valve in general. When patients are clinically well-enough, most cardiologists will defer referring to surgeons in the fear of potential postoperative complications. The problem is that when patients become “ripe enough” for surgery, the risk of complications increases in a steep manner. It is almost as if patients were traveling on a flat ground for a long period before falling off a cliff. Ideally, we should be able to catch them just before they do. The tipping point being hard to predict, this study is a reminder to seize the opportunity before reaching the cliff.

In order to improve our clinical judgement when assessing these patients, the authors missed some opportunity. In truth, some clinically important elements are missing. For example, what was the liver function of these patients? Obviously, one of the organs affected the most by right heart dysfunction and tricuspid regurgitation is certainly the liver. In turns, its function affects significantly the outcome of any cardiovascular operation: vasoplegia, bleeding, volume management, etc. In order to improve care of these patients, and especially to better determine the best timing for surgery, we need to look at preoperative clinically relevant variables.