Important Complications of Eisenmenger Syndrome

Transcript of the video: Eisenmenger syndrome is an important complication of large left to right shunts which develop later due to development of pulmonary vascular obstructive disease and severe pulmonary hypertension. The first report of Eisenmenger was by Victor Eisenmenger in 1897 and that was in a thirty year old person who later succumbed to massive hemoptysis. This highlights one of the most important complications of Eisenmenger syndrome, that is airway hemorrhage. Airway hemorrhage can occur in those who ascend to high altitude and during air travel. But it commonly occurs at low level itself. It is one of the most important causes of mortality in Eisenmenger syndrome.

Diagrammatic representation of VSD Eisenmenger. It is also called Eisenmenger complex. The other Eisenmenger syndromes are not called Eisenmenger complex, only VSD Eisenmenger is called Eisenmenger complex. There is a large ventricular septal defect with severe pulmonary hypertension and reversal of shunt across the ventricular septal defect, producing cyanosis, clubbing, exertional dyspnea, and as already mentioned, hemoptysis is an important complication of Eisenmenger syndrome. Airway hemorrhage, which is more likely to occur when the person ascends to high altitude and in air travel because of the lower atmospheric pressure. But if you go by the statistics, majority of the hemorrhages occur at sea level or ordinary level of height. That is because most of these persons do not go for mountain climbing or air travel.

Diagrammatic representation of PDA with Eisenmenger syndrome. Importance of PDA with Eisenmenger syndrome is that shunting is going into the descending aorta. So there will be differential cyanosis and clubbing. Clubbing and cyanosis will be more in the lower limbs. Sometimes, as the left subclavian is near the shunt, left upper limb can also be having cyanosis. Right upper limb cyanosis is less likely. That is PDA Eisenmenger syndrome with differential cyanosis and clubbing.

Shunt reversal across ASD occurring in severe pulmonary hypertension secondary to large left to right shunt, ASD Eisenmenger syndrome. There are some important features differentiating ASD Eisenmenger syndrome from other two varieties. In ASD, shunt is established only when right ventricular muscle regresses after birth. So it is a delayed onset of shunt and larger shunts are common in large ASD. As the pulmonary vascular regression occurs by the time ASD shunt is established, usually Eisenmenger syndrome is a very delayed complication of large ASD. Some even say that those with Eisenmenger in ASD are really those who are destined for primary pulmonary hypertension. But, leave that alone. Large ASDs usually develop Eisenmenger syndrome, may be after decades, not like early development of Eisenmenger syndrome in VSD and PDA. Another important feature is that, there is no connection between the ventricles or the pulmonary artery and the aorta. So, pressures here can go suprasystemic. That is, right ventricular pressure and pulmonary artery pressure can go suprasystemic in ASD Eisenmenger, while in VSD Eisenmenger, it does not go beyond systemic level because the large VSD equalizes pressures in two ventricles. Similarly, in PDA Eisenmenger also, the systemic and pulmonary circulations are connected by the PDA, so that in PDA Eisenmenger also, maximum pressure possible is only systemic level in the pulmonary arteries. While in ASD, there is an isolation. So in ASD Eisemenger, suprasystemic pulmonary hypertension is possible. So they do badly, but it develops only very late and in very few also. There are several persons in sixth and seventh decade, having ASD, with still left to right shunt. They have heart failure sometimes. Another difference is that, right atrium is grossly enlarged in ASD. It produces cardiomegaly on X-ray in ASD Eisenmenger. In the other two Eisenmenger syndromes, even though they may have cardiomegaly when there is a large left to right shunt, in infancy, the cardiac size regresses when pulmonary hypertensison develops and shunt decreases. So, if you see cardiomegaly on X-ray in an Eisenmenger syndrome, most likely it is ASD Eisenmenger, mostly contributed to (by) large right atrium which enlarges towards the right and produces cardiomegaly in ASD Eisenmenger. So two features, one is cardiomegaly, second is suprasystemic pulmonary pressures in ASD Eisenmenger. Then, in PDA Eisenmenger, you can have aortic enlargement because the shunt is received in the aortopulmonary region, aorta can be enlarged in PDA Eisenmenger. In VSD Eisenmenger, there is no aortic enlargement, there is no cardiomegaly. And in PDA Eisenmenger, sometimes you can see the ductal calcification, inverted Y shaped ductal calcification may be seen sometimes in persons with PDA Eisenmenger.

Moving on to important complications in Eisenmenger syndrome. Airway hemorrhage, that is even massive airway hemorrhages, it can also be mild hemoptysis, or massive hemorrhage which can cause choking and mortality. Mortality is usually due to airway obstruction due to clots rather than due to the amount of bleed. It is a very catastrophic event in Eisenmenger syndrome. And it was described from the very first report of Eisenmenger syndrome. That has already been discussed. Decompensated erythrocytosis is something which we see very often. That is, there is renal ischemia due to clogging of capillaries, which leads to increased erythropoietin secretion and this causes decompensated erythrocytosis. Another feature is that if you do venesection, for removing the excess RBCs, to reduce the symptoms of hyperviscosity, there is a temporary improvement. But then erythropoietin secretion increases further and you can have a decompensated erythrocytosis. So venesection for Eisenmenger syndrome is a double edged situation. Unless it is carefully taken care of, it can cause decompensated erythrocytosis which is a vicious cycle. Iron deficiency cause microcytes and microcytes are less deformable and when they go through the capillaries, they can cause renal ischemia and increase in erythropoietin secretion, further rising the hemoglobin levels. And, thrombotic strokes are also an important complication due to hyperviscosity and they are more likely to occur if there is iron deficiency. So one has to be very careful about avoiding iron deficiency in those with Eisenmenger syndrome. Thrombotic stroke prevention, in that case also, avoiding iron defiency is important. Coagulation abnormalities can be seen because there are platelet function abnormalities in Eisenmenger syndrome. Then, brain abscess is an important complication, which can occur in Eisenmenger syndrome. And, because of the large cell turnover, there is also a possibility of hyperuricemia and related symptoms in persons with Eisenmenger syndrome. And, regarding thrombotic stroke, one important aspect is that if the persons undergo contrast angiography, they are likely to have higher risk of thrombotic stroke. This is because of hemoconcentration. So adequate hydration is very important, especially, in all cyanotic persons undergoing contrast angiography, to avoid periprocedural thrombotic stroke.