Neonates born with hypoplastic left heart syndrome now have a remarkably improved prognosis compared with the situation existing before the development of the Norwood sequence of operative procedures. Some of those born with hypoplastic right ventricles in the setting of pulmonary atresia with an intact ventricular septum, however, still have a relatively poor prognosis. In part this reflects the presence of fistulous communication between the cavity of the right hypoplastic right ventricle and the coronary arterial tree. Such fistulous communications are now increasingly recognised as being important in the setting of hypoplastic left heart syndrome. In this brief review, we describe the anatomy of the communications. Those found with hypoplastic right ventricles are seen most frequently when the cavity of the ventricle effectively represents only the inlet, this in turn reflecting mural overgrowth of the apical trabecular and outlet components during foetal development. This almost certainly reflects an earlier appearance of the pulmonary valvar lesion that promotes the cavitary hypoplasia. In those with hypoplastic left ventricles, the key feature differentiating those with fistulous communications is the presence of a patent mitral valve, since the left ventricle is typically no more than a virtual slit in postero-inferior ventricular wall in the setting of mitral valvar atresia or absence of the left atrioventricular connection.

In the normal heart, the right and left coronary arteries arise from the aortic valvar sinuses adjacent to the pulmonary trunk. The right coronary artery then directly enters the right atrioventricular groove, whereas the main stem of the left coronary artery runs a short course before dividing to become the anterior interventricular and circumflex arteries. These arteries can have an anomalous origin from either the aorta or pulmonary trunk; their branches can have various anomalous origins relative to arterial pedicles. Other abnormal situations include myocardial bridging, abnormal communications, solitary coronary arteries, and duplicated arteries. Understanding of these variations is key to determining those anomalous patterns associated with sudden cardiac death. In the most common variant of an anomalous origin from the pulmonary trunk, the main stem of the left coronary artery arises from the sinus of the pulmonary trunk adjacent to the anticipated left coronary arterial aortic sinus. The artery can, however, arise from a pulmonary artery, or the right coronary artery can have an anomalous pulmonary origin. The key feature in the anomalous aortic origin is the potential for squeezing of the artery, produced by either the so-called intramural origin from the aorta, or the passage of the abnormal artery between the aortic root and the subpulmonary infundibulum.

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.

Over a period of 12 years, we encountered 30 cases of pulmonary atresia with intact ventricular septum. The overall mortality was 53% (16 patients). Coronary arterial abnormalities were detected in 16 patients and, in 10 of these, the coronary arterial circulation was partially or totally dependent on the right ventricle. The mortality in this group was 80%. The strategies for management are reviewed in 6 cases to try to define the best approach to diagnosis, and to assess the benefits of the various modesof treatment used for specific coronary arterial anomalies. Two cases (2 and 3) underwent occlusion of a fistula from the right ventricle to a coronary artery using embolization coils. This modality is considered to be appropriate and useful when the coronary circulation supplied by the fistula is not dependent on flow from the right ventricle. The dependency of the coronary arterial circulation on the right ventricle was extensive in two cases (Case 4 and 6), and complete in one because of atresia of the orifices of the coronary arteries (Case 5). These three patients died. Cardiac transplantation is suggested as the most appropriate treatment in this group. In two cases (1 and 6), there was a supernumerary vessel connecting the right ventricle to the pulmonary trunk. These connections do not appear to be of therapeutic concern, as they do not seem to contribute to myocardial blood supply. The selection of the most appropriate protocol for management of patients with coronary arterial anomalies depends upon a thorough and aggressive approach to investigation.

We report a patient in whom the circumflex coronary artery arose anomalously from the right aortic sinus. The patient also had atrial and ventricular septal defects in the setting of Eisenmenger physiology. We recommended transplantation of the heart and lungs.