How to analyze complex single ventricle cases?

  I recently saw a patient with an 11 month old child with bruised lips and an ultrasound diagnosis of single atrium, single ventricle (type A), pulmonary artery stenosis, somatic pulmonary collateral, and permanent left superior vena cava. He had a common atrioventricular valve with massive regurgitation and poorly developed pulmonary arteries with 4.6 mm main pulmonary artery, 4.5 mm left pulmonary artery and 3.8 mm right pulmonary artery. First of all, this patient had a single ventricle and the general direction was determined to perform a single ventricle correction with the ultimate goal of performing a total vena cava pulmonary artery anastomosis, where the heart is used only as a left ventricle, allowing blood to return directly from the vena cava to the pulmonary artery.  The first step is a Glenn procedure (superior vena cava pulmonary artery anastomosis). Can this child have a Glenn?  This single ventricle correction, whether it is a total vena cava pulmonary artery anastomosis or a Glenn procedure, requires a well developed pulmonary artery, not high pulmonary artery pressure and low pulmonary resistance, otherwise the right ventricular pump function is lost and it is difficult for blood to flow back into the pulmonary artery. If the pulmonary pressure is high need to perform pulmonary artery circumferential surgery to lower the pulmonary artery pressure, and so on down to the appropriate level in the Glenn procedure. If the pulmonary artery pressure is low, it depends on the development of the pulmonary artery. This child has very poor pulmonary artery development and needs a B-T shunt (aortic to pulmonary artery shunt) to increase pulmonary blood, stimulate pulmonary artery development and increase oxygen saturation.  B-T shunt surgery, blood from the aorta to the pulmonary artery, then back to the heart, and then to the aorta, forming a small circulation, this small circulation will all increase the burden, the aortic burden increases thickening, pulmonary artery burden increases is what we need in order to develop, the heart increases the burden, have to strengthen to do work, so the main problem of B-T shunt is to increase the preload of the heart, the return blood volume increases, the heart burden increases. This child has a large amount of common atrioventricular regurgitation, and the heart itself is heavily burdened, so if we do B-T shunt alone, the heart will be overburdened, and it will be difficult to recover after surgery.  Therefore, the surgical steps for this child are: Stage I: B-T shunt + common atrioventricular valvuloplasty; Stage II: bilateral bidirectional Glenn; Stage III: total vena cava pulmonary artery anastomosis.