Cardiovascular magnetic resonance assessment of ventricular function and myocardial scarring before and early after repair of anomalous left coronary artery from the pulmonary artery.

Heiner Latus,Christian Apitz,Kerstin Gummel,Hakan Akintuerk,Stefan Rupp,Dietmar Schranz,Juergen Bauer,Matthias Mueller,Gunter Kerst,Christian Jux

doi:10.1186/1532-429x-16-3

Abstract

BackgroundIn patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) left ventricular (LV) dilatation and dysfunction evolves due to diminished myocardial perfusion caused by coronary steal phenomenon. Using late gadolinium enhanced cardiovascular magnetic resonance (LGE-CMR) imaging, myocardial scarring has been shown in ALCAPA patients late after repair, however the incidence of scarring before surgery and its impact on postoperative course after surgical repair remained unknown.Methods8 ALCAPA-patients (mean age 10.0 ± 5.8 months) underwent CMR before and early after (mean 4.9 ± 2.5 months) coronary reimplantation procedures. CMR included functional analysis and LGE for detection of myocardial scars.ResultsLV dilatation (mean LVEDVI 171 ± 94 ml/m2) and dysfunction (mean LV-EF 22 ± 10 %) was present in all patients and improved significantly after surgery (mean LVEDV 68 ± 42 ml/m2, p = 0.02; mean LV-EF 58 ± 19 %, p < 0.001). Preoperative CMR revealed myocardial scarring in 2 of the 8 patients and did not predict postoperative course. At follow-up CMR, one LGE-positive patient showed delayed recovery of LV function while myocardial scarring was still present in both patients. In two patients new-onset transmural scarring was found, although functional recovery after operation was sufficient. One of them showed a stenosis of the left coronary artery and required resurgery.ConclusionsDespite diminished myocardial perfusion and severely compromised LV function, myocardial scarring was preoperatively only infrequently present. Improvement of myocardial function was independent of new-onset scarring while the impact of preoperative scarring still needs to be defined.

Highlights

In patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) left ventricular (LV) dilatation and dysfunction evolves due to diminished myocardial perfusion caused by coronary steal phenomenon
Myocardial scarring assessed by Late gadolinium enhancement (LGE) was found in 2 of the 8 patients (25%) (Figure 1) and was characterised as transmural in thin apical LV myocardium in one patient while the other LGE-positive patient showed transmural scarring at the basal and midventricular level in the anteroand inferolateral segments (Figure 2)
Right ventricular function was preserved with a mean ejection fractions (EF) of 59 ± 11%

Summary

Introduction

In patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) left ventricular (LV) dilatation and dysfunction evolves due to diminished myocardial perfusion caused by coronary steal phenomenon. The knowledge about the existence of myocardial scarring prior to surgery is inasmuch relevant as Shivalkar and colleagues have hypothesized the classic concept of a hibernating myocardium in ALCAPA-syndrome in which chronic hypoperfusion results, in part, in viable but dysfunctional myocardium with a variable degree of fibrosis. This unique condition, which is characterized by severely depressed cardiac function with ischemic but yet viable myocardium, in turn, has been accounted for the dramatic improvement in LV function after successful coronary reimplantation. Thereby, LGE-CMR has the potential to identify abnormal hibernating myocardium, defined as absence of scar in areas with hypokinesia at rest, and distinguishing it from irreversibly damaged necrotic tissue [13]

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