Hypoplastic left heart syndrome has the greatest mortality rate among all CHDs and without palliation is uniformly fatal. Despite noble efforts, the aetiology of this syndrome is unknown and a cure remains elusive. The genetic and anatomic heterogeneity of hypoplastic left heart syndrome supports a rethinking of old hypotheses and warrants further investigation into the histological and vascular variations recognised with this syndrome. In an effort to elucidate the pathogenesis of hypoplastic left heart syndrome, this review will focus on its unique myocardial and coronary pathology as well as evaluate the association of hypoplastic left heart syndrome with the endocardial fibroelastosis reaction.

With the ever-increasing sophistication of the Internet, and the wealth of information that can now be mined through this source, it may be thought that the age of the book has finished, and hence there is no further need for reviews of newly published books. There is much to be said, nonetheless, for the greater facility of acquiring information from the pages of a book compared to the computer screen. In this review, therefore, I discuss the advantages of obtaining information from the printed page when presented in such splendid form as shown by new books devoted, on the one hand, to the clinical anatomy of the coronary arteries, and on the other hand, to paediatric congenital cardiac surgery. At the same time, I also attempt to provide some insights into the reasons why the authors and editors produced these excellent volumes, which deserve to be on the bookshelves of all those concerned with the diagnosis and treatment of children with congenitally malformed hearts.

Kawasaki disease is a systemic vasculitis occurring in children of all ages. Coronary arterial aneurysms are one of the main fatal complications of the disease, and are usually observed with the onset of coronary arterial disease in adults. We report a young male presenting with myocardial infarction due to coronary arterial aneurysms, but in the absence of previous symptoms of Kawasaki disease.

Surgical intervention for hearts with transposition, defined as concordant atrioventricular and discordant ventriculo-arterial connections, has been one of the landmark achievements in the field of paediatric cardiac surgery. In the early 1950s, pioneer surgeons attempted to palliate patients with transposed arterial trunks with an early form of the arterial switch operation. As a result of initially dismal outcomes secondary to difficulties with coronary arterial transfer, the unprepared nature of the morphologically left ventricle, and primitive methods for cardiopulmonary bypass, the arterial switch was abandoned in favour of several procedures achieving correction at atrial and venous levels, culminating in the Mustard and Senning operations.1,2 These innovative procedures produced the earliest surviving children with transposition. Although the atrial switch procedures achieved widespread acceptance and success during the mid-1960s through the mid-1980s, the search for an operation to return the great arteries to their normal anatomic positions continued. This pursuit was stimulated primarily by the accumulating observations in mid-to-late term follow up studies of: an increasing frequency of important arrhythmic complications, including sinus nodal dysfunction, atrial arrhythmias, and sudden, unexplained death, by the development of late right ventricular dysfunction and significant tricuspid regurgitation in a ventricle potentially unsuited for a lifetime of systemic function by a small but important prevalence of obstruction of the systemic and/or pulmonary venous pathways, and by dissatisfaction with the operative mortality in the subgroup of infants complicated by additional presence of a large ventricular septal defect.3–6 As we have already discussed, a number of novel procedures to achieve anatomic correction had been described as early as 1954, but clinical success was not accomplished until 1975, when Jatene and co-workers7 astounded the world of paediatric cardiology with their initial description.

A 3-month-old infant developed signs of cardiac failure, which was initially attributed to cardiomyopathy. At 8 months, further investigations showed evidence of myocardial ischaemia with reversal of the flow of blood in the left coronary artery, which received no demonstrable inflow from the aorta. An anomalous connection of this artery with the pulmonary trunk was diagnosed but, at surgery, it was found that the arterial orifice was completely atretic, although the main stem was of normal size. A left internal thoracic arterial graft to the anterior descending coronary artery was performed, but he died on the third day after the operation. Postmortem examination showed a small dimple within the aorta at the site of the orificial atresia, extensive myocardial infarction, and two zones of myocardial bridging of the anterior descending coronary artery. We discuss the relationship of coronary orificial atresia with single coronary artery. Although they are related, they typically have different and contrasting clinical presentations. The possible role of the myocardial bridging is also considered.

A young woman with hypertrophic cardiomyopathy presented with intractable chest pain due to a myocardial bridge over the left anterior descending artery, producing severe compression during systole. Percutaneous intracoronary stenting was performed. The patient, however, developed severe and diffuse restenosis within 30 days of the procedure. Myotomy may provide a more effective treatment option for such patients with symptomatic myocardial bridging.

An acute injury to a coronary artery was recognized during the surgical construction of the Fontan circulation. Surgical manipulation of the site of injury was not successful in restoring normal myocardial blood flow. A stent was therefore placed intraoperatively under direct vision, with restoration of normal coronary arterial flow acutely and at short-term follow-up.

The aim of this study was to evaluate the effect of dietary oxysterols on coronary atherosclerosis and vasospasm. Golden Syrian hamsters were fed three diets with different lipid contents for 3 months: (1) a normolipidaemic diet containing 25 g corn oil–fish oil (4:1, w/w)/kg (group Low L); (2) a hyperlipidaemic diet composed of the normolipidaemic diet supplemented with 150 g lard+30 g cholesterol/kg (group High L); (3) a third diet, similar to the hyperlipidaemic diet, in which 4 g cholesterol/kg was replaced by a mixture of oxysterols (group High L+OS). The oxysterol mixture contained (g/kg): 5,6α-epoxycholesterol 211, 5,6β-epoxycholesterol 179, 7α-hydroxycholesterol 67, 7β-hydroxycholesterol (7βOH) 185, 7-ketocholesterol (7 K) 235; and trace amounts of 7-hydroperoxycholesterols (approximately 30 g/kg). Atherosclerosis was evaluated by measuring myocardial Ca, oxysterols and acyl-CoA cholesterol acyl transferase (ACAT) activity; furthermore, coronary reactivity to sodium nitroprusside (5×10-6 m) was measured and the morphology of coronary arteries was visualized by transmission electron microscopy. Coronary spasm was determined by evaluating reactivity to serotonin (5×10-6 m). Feeding the high-lipid diet (group High L) increased the plasma level of 7βOH, 7 K and cholestanetriol. The presence of oxysterols in the diet (group High L+OS) further increased the concentrations of 7βOH and 7 K in the plasma. However, as evidenced by myocardial Ca, ACAT activity and coronary reactivity to sodium nitroprusside, severe atherosclerosis did not develop during the 3-month diet. 7 K was increased in myocardial lipids of groups High L and High L+OS. Electron microscopy did not show the development of atherosclerosis in group High L, whereas vascular wall thickening, endothelial damage and smooth muscle cell proliferation and migration occurred when oxysterols were present in the food. Serotonin (5×10-6 m) induced exacerbated coronary vasoconstriction in group High L that was completely reversed by dietary oxysterols. In conclusion, dietary oxysterols exhibit anti-spasmodic properties, but they cannot be used as agents against excess dietary lipid-induced coronary spasm because of their atherogenic properties.

This paper reports on the origin and distribution of the coronary arteries in normal mice and in mice of the iv/iv strain, which show situs inversus and heterotaxia. The coronary arteries were studied by direct observation of the aortic sinuses with the scanning electron microscope, and by examination of vascular corrosion casts. In the normal mouse, the left and right coronaries (LC, RC) arise from the respective Valsalva sinus and course along the ventricular borders to reach the heart apex. Along this course the coronary arteries give off small branches at perpendicular or acute angles to supply the ventricles. The ventricular septum is supplied by the septal artery, which arises as a main branch from the right coronary. Conus arteries arise from the main coronary trunks, from the septal artery and/or directly from the Valsalva sinus. The vascular casts demonstrate the presence of intercoronary anastomoses. The origin of the coronary arteries was found to be abnormal in 84% of the iv/iv mice. These anomalies included double origin, high take-off, slit-like openings and the presence of a single coronary orifice. These anomalies occurred singly or in any combination, and were independent of heart situs. The septal artery originated from RC in most cases of situs solitus but originated predominantly from LC in situs inversus hearts. Except for this anomalous origin no statistical correlation was found between the coronary anomalies and heart situs or a particular mode of heterotaxia. The coronary anomalies observed in the iv/iv mice are similar to those found in human hearts. Most coronary anomalies appear to be due to defective connections between the aortic root and the developing coronaries. iv/iv mice may therefore constitute a good model to study the development of similar anomalies in the human heart.