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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. Canadian pharmacy viagra and generic viagra.

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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If our patient turns out to have total anomalous pulmonary venous return, let’s remember what that means. First of all, there are four different types of total anomalous pulmonary venous return. The majority of which are super-cardiac type. The intracardiac type accounts for the second category and the infradiaphragmatic - which is usually the most problematic - accounts for 10-20%. And then there is a form in which you may have a mixture of these different forms of return of individual veins. The super-cardiac type is one in which the pulmonary is … looking at the back of the heart, so that the pulmonary veins are here collected into a common vessel, which drains superiorally. A vertical vein, or sometimes we call it a left superior vena cava, then joins the innominate vein to go over to the right superior vena cava. So the super-cardiac from pulmonary venous drainage. Some information about viagra for women.

The infradiaphragmatic is one in which the pulmonary veins collect again behind the heart but drain inferiorally through the diaphragm and the porta cava system to return then via the inferior vena cava. The presentation of these babies is quite variable, again. If they present within the first few hours of life then it’s usually with severe cyanosis with significant pulmonary edema. This is usually the infradiaphragmatic form and these babies are very difficult to manage. The more common forms of total anomalous are the super-cardiac or the cardiac form which drains to the coronary sinus. Another form in fact is when the pulmonary veins collect behind the heart again and actually drain into a dilated coronary sinus and therefore into the right atrium.

So the super-cardiac, the intracardiac forms usually don’t present in the immediate newborn period but actually get out a week or two before they are recognized, usually having mild - mild almost always - as much as moderate cyanosis and usually with some degree of congestive heart failure. They are usually impressive in their examination too, in having these multiple sounds. Multiple murmurs which represent the increased blood flow through the right heart and out the pulmonary outflow tract. So often we will have systolic and diastolic murmurs as well as wide fixed splitting of the second heart sound. Now in the first type, the ones that present within the first few hours of life, the x-ray is likely to look like this. And if you think you are not seeing much, you are right. It’s hard to distinguish the cardiac border here because there is a lot of pulmonary edema. If we saw the heart it would actually be fairly normal in size, but this is an x-ray that we often see with the infradiaphragmatic type of total anomalous pulmonary venous return. The problem being that the long tract of venous return presents resistance to this low velocity flow and therefore results in significant pulmonary edema.
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On the other hand, the baby who presents a few weeks out with mild cyanosis and a low congestive heart failure may have an x-ray that looks like this. And this is a “snowman” that we talk about in blue babies, associated with total anomalous pulmonary venous return. This leftward shadow represents the dilated venous channel, the vertical vein or the left superior vena cava which has collected the pulmonary veins here, drains the return in this way across the innominate vein to a now dilated superior vena cava, because of the pulmonary venous return is systemic venous return as well. With some increased pulmonary vascular markings. So this x-ray can be helpful in distinguishing the total anomalous super-cardiac type.