Coronary to left ventricle blood drainage in isolated myocardial non-compaction: novel findings from intracoronary echo-
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CASE IMAGE IN CARDIOVASCULAR ULTRASOUND
Coronary to left ventricle blood drainage in isolated myocardial non‑compaction: novel findings from intracoronary echo‑contrast myocardial imaging Edoardo Verna1,2 · Sergio Ghiringhelli1 · Stefano Provasoli1 · Fabrizio Morandi1 · Mariangela Lattanzio1 · Jorge Saleno‑Uriarte1 Received: 27 February 2019 / Revised: 8 April 2019 / Accepted: 30 April 2019 © Japanese Society of Echocardiography 2019
Left ventricular (LV) non-compaction (NC) is a rare disease due to the interruption of the process of organization of the myocardial fibres into a compact tissue that takes place at 6–18 weeks of the intrauterine growth, in strict relation to the period of formation of the coronary vascular and capillary network [1, 2]. In spite of the apparent normality of the epicardial coronary arteries by angiography, impaired wall motion and resting myocardial perfusion by single-photonemission computed tomography (SPECT) has been reported in LVNC [3]. This has been ascribed to chronic ischemic changes or microvascular dysfunction; whereas, it is still debated whether inter-trabecular recesses of the LV cavity may communicate with the coronary circulation [4]. To assess the integrity of coronary microcirculation, we used myocardial contrast echocardiography (MCE) by selective intracoronary injection of second-generation microsphere echo-contrast agent in four adult patients with isolated LVNC and abnormal CT-SPECT imaging but normal coronary angiography. A mixed saline suspension of 2–4 ml Sono-Vue microbubbles (Bracco Imaging Spa, Milan, Italy) was injected into the left (all cases) and in the right (2 cases) coronary artery in a concentration of 1–5 × 108 per ml. Simultaneous echocardiographic images were taken from transthoracic apical views using a VIVID I system (GE
Healthcare, Horten, Norway) equipped with a S3 transducer probe in the second harmonic contrast-imaging mode. In all subjects, the SPECT perfusion defects correlated with the echocardiographic location of the non-compacted (NC) myocardium, which showed reduced contrast enhancement, opposite to the normal opacification of the adjacent compacted (C) myocardium. More interestingly, we found that in all patients the ultrasound contrast agent did not entrap into the coronary microcirculation after left coronary injection but showed a premature drainage through NC segments into the LV cavity. Opacification of the LV cavity occurred within 1–2 cardiac cycles, before the opacification of the right atrium from the coronary sinus or of the right ventricle. Images from a representative patient are provided in Fig. 1. This novel observation using a new in vivo imaging technique is consistent with the hypothesis that the interruption of the process of myocardial compaction may also involve abnormal deployment of coronary arteriolar or capillary network resulting in the persistence of embryonic blood supply with coronary to LV communications. If confirmed by further studies, this new observation would help understanding the abnormal anatom
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