Choosing an appropriate treatment strategy is a difficult endeavor when no existing prediction criteria are available and postnatal outcomes are unclear. According to a case report published in Pediatrics, the best approach in these cases is to facilitate a group discussion that reviews the pros and cons of all considered strategies.
This ethics round involved a 20-week old fetus diagnosed with critical pulmonary valve stenosis with intact ventricular septum. The postnatal prognosis was difficult to predict, given that the condition implicates a range of congenital heart diseases with variable outcomes, and evidence of coronary fistulae via fetal imaging was associated with a lower prospect of postnatal biventricular repair. The referred fetal cardiologists discussed with the parents a pulmonary valvuloplasty.
Cardiologists who were experienced with the procedure thought the potential for a good postnatal prognosis in this case outweighed the risks of the procedure. The parents understood the moderate risk of fetal demise, yet clearly voiced their preference for a fetal intervention that would prevent progression of heart disease, which would likely lead to ventricle palliation or transplant. Four different prediction models were applied to the case with conflicting outcomes: 2 predicted nonbiventricular repair, 1 predicted probable nonbiventricular repair, and 1 predicted biventricular repair restoring whole heart function.
The treatment strategy — either fetal intervention or conventional postnatal therapy — was difficult to agree on in this case, as no widely accepted criteria predicting outcomes for a small, nonviable right ventricle currently exist. A final fetal echocardiogram revealed multiple large coronary fistulae, causing clinicians to question the safety of performing a pulmonary valvuloplasty in utero. Furthermore, conclusive evidence of ventricular and valve growth over the remainder of the gestation period improved the postnatal prognosis. These factors ultimately led the cardiology team to decide not to proceed with the fetal intervention.
An echocardiogram performed after the baby was born showed no evidence of heart block, and the coronary fistulae appeared smaller. Confirming coronary circulation was not dependent on the right ventricle, the cardiologists proceeded with a postnatal pulmonary valvuloplasty and repeat catheterizations hoping to augment right ventricular filling volumes and optimizing his chances for biventricular circulation. At the conclusion of this case report, it is still unclear if the infant’s right ventricle will grow adequately to allow for biventricular repair.
Without widely accepted criteria for predicting postnatal outcomes based on fetal findings, non-clinical factors often influence decision makers in cases like this. To resolve conflicting attitudes toward a treatment strategy, discuss pros and cons of each strategy together using a qualified facilitator.
Edwards LA, Justino H, Morris SA, et al. Controversy about a high-risk and innovative fetal cardiac intervention [published online August 10, 2018]. Pediatrics. doi: 10.1542/peds.2017-3595