Circulation, Vol 86, 1753-1761, Copyright © 1992 by American Heart Association
T Sluysmans, SP Sanders, M van der Velde, A Matitiau, IA Parness, PJ Spevak, JE Mayer Jr and SD Colan
BACKGROUND. Before the era of the Fontan procedure, the typical course of
patients with single left ventricle (LV) consisted of heart failure and
death during the second or third decade of life. Despite the advent of
effective palliative therapy, ventricular dysfunction remains a significant
clinical problem for these patients. METHODS AND RESULTS. To investigate
the causes of ventricular dysfunction in these patients and to determine
whether Fontan-type repair reverses deterioration of LV function, the
ventricular dimensions, volume, shape, wall stress, and systolic function
were determined by echocardiography in 84 patients 0.2-35 years old with
double-inlet single LV or tricuspid atresia. Measurements were obtained in
67 patients after palliation (arterial shunt or pulmonary artery band) and
in 47 patients a median of 4.4 years after a Glenn (n = 9) or a Fontan
operation (n = 38). Before a Fontan procedure, ventricular volumes were 2
to 3 times normal. Ventricular afterload, assessed as circumferential and
meridional end-systolic wall stress, became abnormal after 2 years of age.
With age, LV shape changed progressively from ellipsoidal to spherical, as
indicated by the decrease in long axis:short axis ratio from normal (1.9)
toward unity. Concomitantly, the ratio of circumferential to meridional
end-systolic wall stress fell from 1.3 to unity, the ratio of a sphere at
equilibrium. This age-related change in shape and load occurred in concert
with progressive deterioration of LV systolic function and contractility.
Aortic oxygen saturation, an indicator of pulmonary blood flow and
therefore volume work in single- ventricle physiology, was inversely and
independently correlated with contractility. In the group of patients in
whom a Glenn or a Fontan operation was performed at < 10 years of age,
ventricular dimensions, volumes, and wall stress diminished and LV function
and contractility improved after surgery (p < 0.001). In patients
undergoing surgery after 10 years of age, few had improvement of LV
function after surgery. Postoperative ventricular function and
contractility were inversely related to age at surgery and to aortic oxygen
saturation measured before surgery. CONCLUSIONS. Although Fontan-type
repair of single ventricle early in life is associated with reversal of the
abnormal contractile mechanics associated with age and volume load, this
capacity for recovery diminishes with age at surgery.
ARTICLES
Natural history and patterns of recovery of contractile function in single left ventricle after Fontan operation
Department of Cardiology, Children's Hospital, Boston, MA 02115.
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