Reduced left ventricular size and endocardial fibroelastosis as correlates of mortality in newborns and young infants with severe aortic valve stenosis

Abstract

30 patients with severe aortic valve stenosis presented in severe congestive heart failure within the first 2 months of life. In 25 of them, left ventricular volume and contractility were assessed; five of them had a left ventricle of normal size, in 11 left ventricular size was diminished, and in nine patients it was enlarged. Eleven of the infants had extensive endocardial fibroelastosis (EFE) evidenced angiographically by myocardial sinusoids in ten of them and established at autopsy in six. The presence of EFE correlated with the size of the left ventricle; eight of 11 with a small left ventricle, two of five with a normal-sized left ventricle, and one of nine with an enlarged left ventricle displayed EFE. The severe depression of left ventricular function associated with EFE was documented by left ventricular volume determinations on exclusion of the myocardial sinusoids. Of 30 patients, 12 (including eight of 26 who underwent surgery) did not survive. Mortality, severity, and early onset of symptoms were associated mainly with small size of the left ventricle and with the severe left ventricular dysfunction associated with EFE.

Clinical characteristics and outcomes in patients with endocardial fibroelastosis associated with critical aortic stenosis with biventricular circulation

“…Furthermore, several studies have found an association between the higher mortality rates in these patients and the small size of the left ventricles as well as the presence of EFE. [1,5,10] Aortic balloon valvuloplasties and surgical valvotomies offer similar short-and mid-term palliation. However, balloon valvuloplasty patients have higher reintervention rates but shorter hospital and ICU stays and reduced immediate morbidity.…”

“…[1,10] In fact, Friedman et al [10] determined that the more extensive the EFE in a patient, the worse the diastolic dysfunction.…”

“…[1][2][3] The disease can be primary (i.e., not associated with any structural heart defect) or secondary to various congenital heart disease, with the latter being far more common. Secondary EFE occurs in association with underlying cardiovascular anomalies that lead to persistent, increased ventricular wall tension along with an inability to meet the increased myocardial oxygen demands.…”

“…[3] In addition, the presence of EFE in patients with congenital aortic stenosis is known to be associated with increased mortality and left ventricular dysfunction. [1,4,5] In this report, we describe the characteristics and results of our patients with EFE associated with neonatal critical aortic stenosis.…”

Hypoplastic Left Heart Syndrome

“…Fibrotic thickening of the endocardium (endocardial fibroelastosis) can occur in either side of the heart and contribute to poor cardiac function (16,18). Additional complicating factors include right ventricular failure and tricuspid valve or common atrioventricular valve regurgitation (3).…”