Re: Eisenmeinger's Syndrome

Posted By CCF Cardio MD-SGM on November 19, 1997 at 09:59:46:

In Reply to: Eisenmeinger's Syndrome  posted by Rhonda Bell on November 08, 1997 at 22:41:47:







: Is there a general life expectancy limitation for eisenmeinger (VSD)
patients? If so, what is it? Also, would the higher O2 ratio at sea
level be of any appreciable long-term benefit?



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Dear Ms. Bell,
As you know, a ventricular septal defect(VSD) is an abnormal communication between the right and left ventricles of the heart, allowing blood flow to pass from one chamber to the other.  VSDs come in multiple sizes and locations, but the natural history of the disease(when not corrected surgically during childhood) follows one of two principle courses.  Adult patients with VSDs either experienced spontaneous closure of the defect(or the defect is very small with limited flow across it), or they develop elevated pulmonary resistance/pulmonary hypertension which reduces the left to right flow across the VSD, sometimes resulting in reverse flow and cyanosis(blue discoloration of the extremities) due to deoxygenated blood from the right heart mixing with the oxygenated blood of the left heart.   This Increased pulmonary resistance and elevated pulmonary blood pressure due to heart disease(in this case the VSD) defines Eisenmengers syndrome, and represents a reaction of the pulmonary circulation to the severely increased blood flow to which it is exposed.  
This is a serious problem, Eisenmengers syndrome, because although rising pulmonary pressures serve to limit the flow from left to right across the VSD, they also place an excessive burden on the right ventricle of the heart, inevitably leading to heart failure.  In answer to your question regarding life expectancy in the setting of Eisenmengers syndrome with VSD, I would reply that this depends greatly on the right ventricular size and function, the left ventricular function, as well as hemodynamic indices such as cardiac index, right atrial pressure, and the severity heart failure symptoms (lower extremity swelling, abdominal distention, breathlessness, etc.).   Any attempt to give you a reasonable estimate of survival without performing a physical examination, a thorough medical history, and reviewing data that I mentioned would be certainly flawed.  In general, patients with Eisenmengers syndrome do reach adulthood, but survival is reduced.  One physician described a patient suriviving to age 69.
Regarding the issue of sea-level living to help with oxygenation, I would say that ambient oxygen at most reasonable elevations (less than approx. 4000 feet above sea level) provides more than enough oxygen.  If you have hypoxia with rest or minimal physical exertion(low oxygen level with finger probe or arterial blood testing) then oxygen is recommended, typically via a nasal canula, in order to prevent further increases in pulmonary vascular resistancea common response to low oxygen saturation in the blood.  If however, your oxygen saturation is in the normal range, then no supplemental oxygen is necessary, and I know of no data that shows increased survival at sea level.  Thus, if a patient with Eisenmengers but normal oxygen saturations wished to move to a sea-side location that would be fine, but I would not recommend relocation for the purpose of increasing survival.
I hope this wordy answer has been of help.  Information provided in the Heart Forum is for general medical informational purposes only. Specific diagnoses and therapeutic recommendations can be provided only by your personal physician.