Large, uncorrected cardiac shunt or palliative, surgically created systemic-to-pulmonary shunt for congenital heart disease. Large, nonrestrictive VSD, Nonrestrictive PDA, Atrioventricular septal defect, including a large ostium primum ASD without a ventricular component, Aortopulmonary window, Palliative, surgically created systemic-to-pulmonary anastomosis for treatment of congenital heart disease. Treatment: Right heart failure: digoxin and diuretics have been used. Diuretics, specifically loop diuretics, are used for symptomatic relief of congestion. Surgical palliation or repair should be performed early in patients with congenital heart disease to prevent progression to Eisenmenger syndrome.
Statistics: A study by Diller et al indicated that survival rates for untreated patients with Eisenmenger syndrome may have been overestimated in previous studies and that these rates have not improved since the 1970s. The report involved a literature review of 12 studies published between 1971 and 2013 (1131 patients total), along with an analysis of 219 contemporary, treatment-na?ve patients at the investigators? own institution.
Major research The investigators stated that almost none of the studies appropriately accounted for immortal time bias and therefore overestimated patient?s survival chances by as much as 20 years. When Diller and colleagues took immortal time bias into account, they determined that the 10-year mortality rate among untreated patients approached 30-40%. They also found no indication that the chances of survival for these patients were better than they would have been in the 1970s, 1980s, or 1990s, although survival prospects were found to be better than for patients in the 1950s and 1960s.