What is the management? Congenital Heart Disease .

What is the management? Again, to support the systemic circulation, be it to only the lower body as in coarctation or to all the systemic circulation, by giving prostaglandin to maintain the ductus allowing then the pulmonary artery to be the source of supply to the systemic circulation. Often we need to include inotropic support, usually intravenous dopamine or dobutamine and more often than not, ventilatory assistance as well.
Switching gears again to talk about an older patient with cyanotic congenital heart disease and what might be a typical presentation is a toddler who is playing vigorously on a hot day in the Valley, who becomes progressively cyanotic and may even collapse on the playground. Usually gets rushed off to the ER via the paramedics and if you have the opportunity to get some history there may be history that the child has appeared to be mildly cyanotic for some time now and has episodes in which he appeared to have increased cyanosis. Often associated with hyperpnea. On examination, the child is severely cyanotic. The precordium is active but there is a very short, short murmur. And in the 2 ½-year-old you may see that there is mild clubbing. So here’s a child with a typical presentation of tetralogy spell. And what usually happens with a child playing is it decreases his systemic resistance so that there is a tendency for more blood from the right ventricle out the aorta and at the same time can increase the obstruction dynamically of the right ventricle outflow tract, increase sympathetic tone with play activity etc. to obstruct even further the pulmonary flow.
The pathology we talked about, increased outflow obstruction, decreased peripheral resistance, this may be triggered by acidemia or anemia. Spells often occur in the early morning. It’s thought to be associated with a sort of relative acidemia. And certainly has the tendency to occur in children who are anemic with tetralogy of Fallot.
The management; we teach the parents, once we make this diagnosis, knee-chest position to effect an increase in the systemic vascular resistance acutely. Under medical management, they will often receive oxygen, morphine, propanolol and phenylephrine that do certain different kinds of things. Phenylephrine increasing the systemic resistance, the propanolol decreasing the inotropic tone if you will, including the outflow tract to relieve that dynamic obstruction. With severe spells often if you need general anesthesia, then cadamine (?) would be a good choice because it does tend to increase systemic venous resistance. This child who has been out playing on a hot day, certainly think about volume expanding. If the child is anemic, certainly to think about packed red blood cells and if necessary, to treat the secondary acidemia.

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