Effects of persiting myocardial sinusoids on left ventricular performance in pulmonary atresia with intact ventricular septum

Abstract

The effects of persisting right ventricular myocardial sinusoids on left ventricular global and regional function were studied in 13 patients with pulmonary atresia and intact ventricular septum. Persisting myocardial sinusoids allow the inflow of undersaturated blood into the coronary circulation with subsequent myocardial ischaemia. 7 patients had myocardial sinusoids (group 1). 6 patients had normal coronary perfusion (group 2) The measures of global left ventricular function (ejection fraction, left ventricular enddiastolic volume, left ventricular enddiastolic pressure and shape index) showed no differences between the groups. All patients in group 1 showed disturbances of regional wall motion (P less than or equal to 0.005). There was a high degree of coincidence between the disturbances of regional wall motion and the topography of myocardial perfusion from persisting myocardial sinusoids. In group 1 apical hypokinesia was a regular finding, reflected by a significantly reduced shortening of the left ventricular long axis (P less than or equal to 0.01). As all of these patients had communications between the myocardial sinusoids and the left anterior descending coronary artery, this probably resulted from apical left ventricular ischaemia.

Restrictive Right Ventricular Physiology and Right Ventricular Fibrosis as Assessed by Cardiac Magnetic Resonance and Exercise Capacity After Biventricular Repair of Pulmonary Atresia and Intact Ventricular Septum

“…The influence of associated coronary abnormalities on LV function has been reported. 28,29 Even in the absence of abnormal coronary sinusoidal communications, medial thickening of intramyocardial coronary arteries, discrete areas of fibrosis and calcification, and high levels of endomysial collagen suggestive of chronic ischemia have been documented. 30,31 Our finding of scatteredareas of LGE in the left ventricle is perhaps a reflection of these discrete pathological changes.…”

Exclusion of the non-functioning right ventricle in children with pulmonary atresia and intact ventricular septum☆

“…It has been shown that patients with RV-to-coronary connections and coronary abnormalities have a higher incidence of wall motion abnormalities (which might reflect ongoing ischemia), and that such patients are at risk of late death [5]. Hausdorf and associates [6] found a high degree of coincidence between regional wall motion abnormalities and myocardial perfusion topography resulting from persisting myocardial sinusoids. Fenton and associates [20] attributed the most common cause of interim mortality to abnormal myocardial perfusion.…”

“…Commonly, these patients have a small, hypertrophied right ventricle (RV) with supra-systemic pressure and sinusoidal communication between the RV and the coronary artery [1,2]. Many authors have noticed the possible deleterious effects associated with this situation, i.e., the progression of RV-to-coronary connections and subsequent coronary artery stenosis [3][4][5][6], potential RV 'steal' and coronary ischemia after RV decompression [4,7], impaired left ventricular compliance [8], left ventricular hypertrophy with a prominent subaortic septal bulge [9,10], fibroelastosis of the left ventricle (LV) [10], and an impaired LV function after a Fontan procedure [11] or even after biventricular repair [12]. A surgical trial to prevent these deleterious effects was first described by Waldman and associates [13].…”

Assessment of right and left ventricular function by tissue Doppler echocardiography in patients after biventricular repair of pulmonary atresia with intact ventricular septum

“…Two studies have previously assessed the influence of PAIVS-associated coronary abnormalities on LV function [23,24]. Hausdorf et al demonstrated apical hypokinesia in patients with communications between myocardial sinusoid and left anterior descending coronary artery before RV decompression [23].…”

“…Hausdorf et al demonstrated apical hypokinesia in patients with communications between myocardial sinusoid and left anterior descending coronary artery before RV decompression [23]. After surgical RV decompression, Gentles et al have similarly found regional LV dysfunction in patients with coronary abnormalities studied at a median of 20 months after operation [24].…”