Improving Survival in Patients With Eisenmenger Syndrome
Are We Any Closer?
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Article, see p 1432
The population with Eisenmenger syndrome is only 1 of several congenital heart disease (CHD) cohorts that has pulmonary arterial hypertension. Other groups include those with hemodynamically significant left-to-right shunting (both correctable and noncorrectable) where cyanosis is not a feature, those with small cardiac defects (eg, coincidental CHD), those with pulmonary hypertension after successful congenital heart defect repair, and those with left-sided CHD (including left heart obstructive lesions and congenital cardiomyopathies). Even within the simple class of patients with Eisenmenger syndrome, a variety of underlying cardiac defects exist, including simple atrial septal defect, ventricular septal defect, patent ductus arteriosus, and more complex anatomy resulting in a fairly heterogeneous group of patients. Although the underlying pathology in the pulmonary vascular bed may be similar, the pathophysiology is clearly different.
Eisenmenger syndrome is a multisystem disorder associated with a multitude of complications and reduced survival. It has generally been accepted that survival prospects of patients with Eisenmenger syndrome are substantially better than other forms of pulmonary arterial hypertension (PAH). Although some patients with Eisenmenger syndrome expire during childhood or young adulthood, a sizable proportion survives to middle and old age despite the degree of pulmonary hypertension, detrimental long-term effects of cyanosis, polycythemia, accompanying renal dysfunction, and poor exercise tolerance. Compared with patients who develop PAH with a structurally normal heart, the population with Eisenmenger syndrome has had their heart defect throughout life, thus potentially facilitating adaptation of the right ventricle to the pressure overload at an early age. Nevertheless, estimating the survival prospects of patients with Eisenmenger syndrome and identifying those at high risk for imminent death is considered important to inform when therapy should be initiation or intensified.
The study by Kempny et al1 in this issue …