Year : 2012 | Volume
: 15 | Issue : 4 | Page : 257--258
Eisenmenger syndrome: An unsolved malady
Praveen Kumar Neema
Additional Professor Anaesthesiology, SCTIMST, Trivandrum, India
Praveen Kumar Neema
Additional Professor Anaesthesiology, SCTIMST, Trivandrum
|How to cite this article:|
Neema PK. Eisenmenger syndrome: An unsolved malady.Ann Card Anaesth 2012;15:257-258
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Neema PK. Eisenmenger syndrome: An unsolved malady. Ann Card Anaesth [serial online] 2012 [cited 2022 May 21 ];15:257-258
Available from: https://www.annals.in/text.asp?2012/15/4/257/101844
Eisenmenger syndrome was initially described as Eisenmenger complex by Victor Eisenmenger in 1897 when he reported on a patient with symptoms of dyspnea and cyanosis from infancy; the patient later developed heart failure and succumbed to massive hemoptysis.  A large ventricular septal defect (VSD) and an overriding aorta were found on autopsy. This was the first description of a link between a large congenital cardiac shunt defect and the development of pulmonary hypertension (PH). The intracardiac defects like atrial septal defect (ASD), VSD, single ventricle with unobstructed pulmonary blood flow, and extracardiac shunts like truncus arteriosus and patent ductus arteriosus result in left to right shunt and an increase in pulmonary blood flow. During fetal life and neonatal period, the shunt is usually small or absent because of high pulmonary vascular resistance (PVR), but as the PVR deceases the shunt increases. All the intracardiac and extracardiac shunts except ASD are shunts with increased pressure. Pulmonary vasculature being highly compliant accommodates increased blood flow without significant increase in pulmonary artery pressure. However, because of continuous exposure of pulmonary vasculature to the stress of increased blood flow and pressure, the pulmonary vasculature remodels and gradually over years (usually before puberty) develops increased muscularity, obliterative changes, and finally, a severe increase in PVR sets in. The pulmonary artery pressure equals or exceeds systemic arterial pressure and the shunt reverses right to left, initially phasic and finally through whole of the cardiac cycle. The reversal of flow represents a point of irreversibility of PH (Eisenmenger syndrome) and indicates inoperability of the cardiac lesion. At this point, the PH is unresponsive to inhalation of 100% oxygen or nitric oxide and it usually correlates to a PVR of more than 12 Woods units.  Concurrently, the patient gradually develops clinical features of Eisenmenger syndrome such as increasing arterial desaturation, cyanosis, dyspnea on exertion, syncope, chest pain, right ventricular hypertrophy, congestive heart failure, dysrhythmia, complications of hyperviscosity, pulmonary hemorrhage/hemoptysis, stroke, brain abscess, endocarditis, etc. The incidence of Eisenmenger syndrome is constantly decreasing as all types of cardiac defects are now repaired in infancy or childhood. The usual life expectancy of a patient with Eisenmenger syndrome is 20-50 years if the syndrome is diagnosed promptly and treated with vigilance; however, some patients survive into the sixth decade of life. The onset of pulmonary hemorrhage is usually the hallmark of a rapid progression of the disease. The most frequent terminal event in this syndrome is a combination of hypoxemia and arrhythmia in the setting of rapid increases in PVR or decreases in systemic vascular resistance (SVR). Death commonly results from congestive heart failure, massive hemoptysis, or thromboembolism. 
A few of these patients with Eisenmenger syndrome may present for incidental surgery. The perioperative mortality of these patients is extremely high. The mortality rate in pregnant patients with Eisenmenger syndrome is reported to be approximately 30-50%.  In this issue of Annals of Cardiac Anaesthesia, Minicucci et al.  describe safe conduct of pregnancy and elective cesarean section in a 29-year-old woman with Eisenmenger syndrome. A thorough analysis of the pathophysiological changes that cause high mortality in patients of Eisenmenger syndrome during pregnancy may perhaps provide answers for the safe perioperative management of these patients.
The cardiovascular changes during pregnancy are directed to increase the cardiac output to ensure supply of O2 and nutrients to the growing fetus. The major changes include blood volume expansion, hemodilution, and addition of a low vascular resistance placenta in the systemic circulation. Blood volume begins to increase in the 6th week of gestation, and by the end of pregnancy, it reaches approximately 50% more than in the pre-pregnant state. , Red cell mass increases as much as 40% above pre-pregnancy levels;  the plasma volume increase is proportionally greater than the increase in red blood cell mass, and the resulting hemodilution explains the so-called "physiological anemia of pregnancy." These changes - hemodilution, relative anemia, decrease in viscosity, and addition of placental circulation to the systemic circulation - lead to a significant decrease in SVR. These hemodynamic changes in pregnant patients of Eisenmenger syndrome lead to cardiac decompensation and are potentially catastrophic. In patients of Eisenmenger syndrome, a decrease in SVR results in an increase in right to left shunt, which worsens arterial desaturation and hypoxemia, and is likely to precipitate myocardial dysfunction in an already stressed right ventricle. Anemia by compromising the myocardial O2 supply and hypoxemia by increasing PVR and right ventricular stress can further compound the devastating effects of decreases in SVR and increased right to left shunt. Arguably, anemia, decrease in SVR, increase in PVR, hypoxemia, and right ventricular stress are the basic causes of right ventricular dysfunction and failure and adverse outcome in patients of Eisenmenger syndrome during pregnancy. Apparently, the physiological cardiovascular changes of pregnancy are disadvantageous for patients with Eisenmenger syndrome and are the possible reasons of high mortality of these patients during pregnancy.
Onset of labor is a stressful period for the pregnant patients with Eisenmenger syndrome. Uterine contractions and bearing down cause tremendous increase in SVR and mobilization of large amount of blood volume in the circulation, which can severely increase the cardiac preload, right ventricular stress, left ventricular afterload, and acute failure of both the ventricles. The authors, Minicucci et al.,  planned a cesarean delivery when the fetus was reasonably grown, avoided labor, and intelligently manipulated the SVR after administration of spinal anesthesia, and thus achieved a successful outcome.
Conceivably, during management of the patients with Eisenmenger syndrome undergoing incidental surgery, prevention of acute decreases in SVR and hemodilution might prevent acute cardiovascular collapse and improve their perioperative outcome. However, it is important to appreciate that uncontrolled increase in SVR can cause both right as well as left ventricular failure and should be avoided. Replacement of lost blood by clear fluids can precipitate acute right ventricular failure; perhaps, Eisenmenger syndrome is a disease state where blood conservation does not have a place and the lost blood should be replaced by red blood cells.
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