Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study

Abstract

Despite good short-term prognosis, life expectancy is markedly reduced in Eisenmenger patients. Markers of heart failure and parameters associated with arrhythmia are of prognostic value in terms of mortality and may guide clinicians caring for Eisenmenger patients.

The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

“…In ES patients, higher functional class, a history of arrhythmias, patient age, right ventricular dysfunction, syncope, elevated uric acid levels and the presence of Down's syndrome have been found to be of value in predicting a poor outcome [26,[30][31][32][33][34]. In a very recent study based on data of 181 ES patients, a composite echocardiographic score was also highly predictive of clinical outcome [35].…”

Congenital Heart Disease and Pulmonary Hypertension

“…Most left-toright congenital shunts do not initially have cyanosis, but its presence is often the harbinger of increasing pulmonary pressures and the development of Eisenmenger syndrome. 13,20,60 Complex congenital lesions may have some degree of chronic cyanosis, making the identification of worsening cyanosis difficult. In advanced stages, patients develop progressive right ventricular dysfunction, which may result in sudden death.…”

“…It is important to inquire into the onset, severity, and progression of symptoms, because it may help to assess the severity of the condition. 20,60 In addition, associated symptoms may suggest a particular cause of PH.…”

Pulmonary Hypertension in Congenital Shunts

“…Symptoms may not arise until childhood or early adulthood. In general, patients with ES have reduced life expectancy, although many survive into their third or fourth decade, 32 with some even surviving into their seventh decade with appropriate management. 32,33 Of all patients with CHD, those with ES are the most severely compromised in terms of exercise intolerance.…”

“…In general, patients with ES have reduced life expectancy, although many survive into their third or fourth decade, 32 with some even surviving into their seventh decade with appropriate management. 32,33 Of all patients with CHD, those with ES are the most severely compromised in terms of exercise intolerance. 34 Exercise intolerance in these patients has been identified as a predictor of hospitalisation or death independent of age, gender, functional class or underlying cardiac defect.…”