Vascular involvement in neurofibromatosis type 1 has been described, although coronary artery disease is rare. Data about clinical presentation and natural history are anecdotal. This is the first case of myocardial infarction due to coronary aneurysms in a 13-year-old boy with neurofibromatosis type 1. We discuss pathophysiology, diagnostic images, and therapeutic management of this rare association.

Transoesophageal and epicardial echocardiography are indispensible intraoperative imaging modalities to guide paediatric heart disease surgeries and influence surgical decision-making. A less well-described role of intraoperative imaging is its utility in evaluating coronary artery patency and flow. Focused two-dimensional, colour, and spectral Doppler imaging of the coronary arteries should be performed during surgeries involving coronary manipulation or re-implantation, or in cases where there is unexpected ventricular dysfunction or electrographic signs concerning for ischaemia. Intraoperative imaging allows for any anatomical issues to be detected and addressed promptly in the operating room. Imaging of the coronary arteries should identify unobstructed coronary ostia and proximal course without kinking, angulation, narrowing, or significant calibre change to suggest stenosis or extrinsic compression from neighbouring structures. The aim of this review is to highlight the usefulness of transoesophageal and epicardial echocardiography in evaluating coronary artery patency and flow, provide a how-to guide for optimal imaging, and to introduce a practical guideline to achieve best clinical practice.

Coronary arteriovenous fistula is an uncommon clinical entity. The right coronary artery is the most common site of origin, and the fistula commonly drains into the right-sided cardiac chambers. Very rarely it can arise from the left main coronary artery, and fistulas draining into the superior vena cavity are extremely rare. We report a 12-year-old asymptomatic boy with a large coronary arteriovenous fistula between the left main coronary artery and superior vena cava, with aneurysmal dilatation of the left main coronary artery. As the fistula was very large and to prevent its complications, it was planned to close the fistula percutaneously.

Currently there are no methods available for staining rat and human myocardial microvasculature on thick sections that would allow for specific staining and differentiation of arterioles, venules, and capillaries. A non-injection technique is described that allows for labeling of the microvascular bed (MVB) in formalin-fixed pieces of the myocardium from humans and the white rat Rattus norvegicus, as well as human full-mount pericardium. Vessel staining is based on the activity of phosphatases (ATPases) and the precipitation of the released phosphate with calcium ions at high pH (pH 10.5–11.5). The resulting precipitate subsequently is converted to black or brown lead sulfide. The specificity of this reaction to vessels of the MVB allows arterioles, venules, capillaries, and pre- and postcapillaries to be clearly visualized in thick (60–100 µm) and ultra-thick (300–500 µm) sections against an unstained background of muscle and connective tissue. In addition, smooth muscle cells of arterioles are also stained allowing for differentiation between arteriolar and venular beds. These observations have not been reported in rat or human myocardium using other methods. This procedure should benefit studies of coronary microcirculation in experimental and pathological conditions, as well as in pharmacological investigations.

Acute myocardial infarction is rarely reported in children. Most of the cases are secondary to congenital anomalies or Kawasaki disease. Coronary artery total occlusion caused by fibromuscular dysplasia has never been reported in young children. Here we report a case of a 12-year-old boy with fibromuscular dysplasia, who underwent successful coronary intervention for acute myocardial infarction.

Background: Vasodilator stress perfusion cardiovascular magnetic resonance imaging is a clinically useful tool for detection of clinically significant myocardial ischaemia in adults. We report our 5-year retrospective experience with perfusion cardiovascular magnetic resonance in a large, quarternary adult congenital heart disease centre. Methods: We reviewed all cases of perfusion cardiovascular magnetic resonance in patients referred from the adult congenital heart disease service. Dipyridamole stress perfusion cardiovascular magnetic resonance was undertaken on commercially available 1.5 and 3 T cardiovascular magnetic resonance scanners. Late gadolinium enhancement imaging was performed 8–10 minutes after completion of the rest perfusion sequence. Navigator whole-heart coronary magnetic resonance angiography was also performed where feasible. Results of stress cardiovascular magnetic resonance were correlated with complementary imaging studies, surgery, and clinical outcomes. Results: Over 5 years, we performed 34 stress perfusion cardiovascular magnetic resonance examinations (11 positive). In all, 84% of patients had further investigations for ischaemia in addition to cardiovascular magnetic resonance. Within a subgroup of 19 patients who had definitive alternative assessment of their coronary arteries, stress perfusion cardiovascular magnetic resonance demonstrated a sensitivity of 82% and specificity of 100%. Of the 34 studies, two were false negatives, in which the aetiology of ischaemia was extrinsic arterial compression rather than intrinsic coronary luminal narrowing. Coronary abnormalities were identified in 71% of cases who had coronary magnetic resonance angiography. Conclusion: Stress perfusion cardiovascular magnetic resonance is a useful and accurate tool for investigation of myocardial ischaemia in an adult congenital heart disease population with suspected non-atherosclerotic coronary abnormalities.

A 38-year-old man who had a history of percutaneous coronary artery coil occlusion was admitted to our hospital with chest pain and shortness of breath. His complaint was chest pain, which is typical. ST depressions were observed during the treadmill exercise stress test. Coronary angiography demonstrated the persistence of a coronary arteriovenous fistula and coils in the fistula. Primarily, additional coil placement inside the arteriovenous fistula was decided as the mode of treatment. The coil was first placed inside the arteriovenous fistula and then an attempt was made to detach it. However, it was unsuccessful after four trials and electrical detachment of more than 3 minutes. Finally, a 2.5 × 18-millimetre graft stent was deployed at 20 atmospheric pressure. Electrocardiographic recordings showed bizarre ST segment changes during the electrical detachment of the coil. In this report, we discuss the concealed bizarre electrocardiographic changes that were seen during coronary arteriovenous fistula occlusion.

Thrombotic coronary arterial occlusion, and myocardial infarction, are rare in the newborn. We report such a happening presenting shortly after birth with cardiogenic shock, no left ventricular output and a systemic circulation dependent on flow from a patent arterial duct.

The present study was designed to determine the direct effects of halothane, isoflurane and sevoflurane on the bovine epicardial coronary artery as well as their mode of action. We chose serotonin as the vasoconstrictor because it also causes endothelium-dependent relaxation of coronary arteries. Isolated spiral strips of bovine epicardial coronary artery with and without endothelium were suspended for isotonic contraction recordings in Tyrode's solution. KCl (80.4 mm) solution induced maximal contraction, regarded as the reference value (100%). The muscle strips were then exposed to increasing concentrations of serotonin from 10−8 to 10−4 m in the presence and absence of 1.5 MAC halothane, isoflurane or sevoflurane. All three drugs attenuated serotonin-evoked contraction in the coronary artery strips both those strips with and without endothelium (P < 0.05−0.001). However, there were no significant differences in the attenuation of serotonin-induced contraction of the strips, both with and without endothelium, in each drug group. The attenuation potency of halothane was more than that of isoflurane and sevoflurane. The results demonstrate that halothane, isoflurane and sevoflurane attenuate contractile responses evoked by serotonin in bovine epicardial coronary artery both with and without endothelium.