Percutaneous transient occlusion of the transtricuspid flow: a new method to evaluate the right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.

ABSTRACT

Pulmonary atresia with an intact ventricular septum is characterised by heterogeneity in right ventricle morphology and coronary anatomy. In some cases, the presence of ventriculocoronary connections may promote coronary artery stenosis or interruption, and aortic diastolic pressure may not be sufficient to drive coronary blood flow. This requires a correct evaluation (currently done by angiography) which depends on whether the patient can be offered decompression of the right ventricle. To date, there is no objective method to do so, so we designed a percutaneous, transitory technique with the purpose of occluding the transtricuspid anterograde flow. The manoeuverer was performed in a 25-day-old female with pulmonary atresia with intact ventricular septum, right ventricle at suprasystemic level, and selective coronarography was not conclusive, the anterior descendant with stenosis in its middle third and from this point, thinner with to-fro flow. Occlusion was performed with a balloon catheter. We re-evaluated the coronary flow and the normalised anterior descendant flow. We hope that with this new method, we can give a more accurate diagnosis and determine the cases in which the coronary circulation is truly not right ventricle dependent to offer a greater number of patients biventricular or 1.5 ventricular repairs and thereby improve their quality of life and survival, the ones that turn out to be right ventricular dependant; offer them an early reference for cardiac transplant or in case it is not available to consider univentricular palliation knowing that this probably would not reduce the risk of ischaemia and/or death over time.