The early management

The management, the early management, is recognizing the congestive heart failure and treating it. Usually these babies go on to surgery once we make the diagnosis, at whatever age, of total anomalous pulmonary venous return.

The fifth of these cyanotic congenital heart diseases would be truncus arteriosus. Again, to remind us that we are dealing with a single trunk arising from the base of the heart which then subsequently divides into an aorta and into a pulmonary artery. There are actually four different types of truncus arteriosus depending on whether that pulmonary artery comes off the single trunk or as separate vessels.
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The presentation, like that of the total anomalous super-cardiac, is usually not in the immediate newborn period so our case presentation would not likely be either of these last two diagnoses. But these babies often get out for a week or two and then begin to look a little cyanotic and to have some signs of congestive heart failure. Some of the distinguishing features, physical features, of truncus would be the bounding pulses as there is a run off to the lower resistant pulmonary bed from the aorta. A single second heart sound – there is a single semilunar valve, a click related to this single vessel and its dilated nature, or the valve mechanics. And often a systolic murmur as well.

The treatment initially is that of managing the congestive heart failure, but early surgical intervention and early surgical correction is appropriate as well. In fact we worry about waiting too late in these babies because of the high flow, high pressure dynamics to the pulmonary bed that may cause some progressive and irreversible pulmonary vascular changes. So these babies go to surgery for their conduit. Placed in their right ventricles to the pulmonary arteries and closure of the ventricle septal defect in such a way that the left ventricle then connects to the ascending aorta.

We turn our attention to a little different kind of setting, of the newborn who presents in shock. A typical case presentation may be this; the baby who gets out of the newborn nursery and goes home and appears to be doing well for the first few days of life, but at about a week of age is noted to be fussy, not eating very well, diaphoretic and to have signs of respiratory distress. These babies will often present in the ER in shock, appearing quite pale and clammy and lethargic. The differential that comes to mind with a young infant presenting in shock should include sepsis, perhaps some metabolic disorder, but of the cardiac lesions we should consider, we should think of hypoplastic left heart and coarctation of the aorta. Coarctation of the aorta … here’s a picture, another drawing of the aorta and the coarctation being an obstruction usually at the distal part of the aortic arch. We can see here internally what that shelf may look like. Hypoplastic left heart on the other hand – meaning that there is usually a very small left ventricle in association with either aortic atresia or severe stenosis. Here’s a small, small ascending aorta compared to the pulmonary annulus here. And/or mitral stenosis or atresia. The things that can help to distinguish these two diagnoses would include looking at color, pulses and blood pressure and looking for a discrepancy. In which case if there are pink upper extremities and blue lower extremities, or if there are good pulses in the upper extremities and poor in the low, good blood pressure in the upper extremities, etc., then we are more likely dealing with coarctation of the aorta. If the pulses, color, blood pressure uniformly decrease then we are likely dealing with hypoplastic left heart syndrome.

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