SUMMARY Hemodynamically significant primary anomalies of the coronary arteries are those which alter myocardial perfusion. There are four major types: coronary artery fistulae, origin of the left coronary artery from the pulmonary artery, congenital coronary stenosis or atresia, and origin of the left coronary artery from the right sinus of Valsalva, with subsequent passage of the vessel between the aorta and right ventricular infundibulum. Tbc-angiographic features of these lesions are discussed.PRIMARY CONGENITAL ANOMALIES of the coronary arteries (that is, those which can occur as isolated anomalies and are not necessarily associated with other types of congenital heart disease) can be divided into hemodynamically significant and insignificant lesions.The hemodynamically insignificant anomalies are principally those of abnormal aortic origin or distribution of the coronary arteries. They occur in slightly less than 1% of adult patients undergoing coronary arteriography and in children with certain forms of congenital heart disease, such as d-and 1-transposition, tetralogy of Fallot, double outlet right ventricle and single ventricle. Myocardial perfusion is not altered in these individuals.The hemodynamically significant anomalies of the coronary arteries are characterized by abnormalities of myocardial perfusion. There are four major types: 1) coronary fistulae, 2) origin of the left coronary artery (LCA) from the pulmonary artery (PA), 3) congenital coronary stenosis or atresia and 4) origin of the LCA from the right sinus of Valsalva, with subsequent passage of the vessel between the aorta and right ventricular infundibulum. Although angiography provides the key to diagnosis, there have been no previous comprehensive discussions of the radiologic aspects of these lesions. In this paper we review their angiographic appearance, based upon a survey of previously published reports and our own experience with 34 cases. Twenty-one of our patients had coronary artery fistulae, 12 had anomalous origin of the LCA from the PA and one had atresia of the origin of the LCA. Coronary Artery Fistulae A pre-capillary fistula connecting a major coronary artery directly with a cardiac chamber, coronary sinus, superior vena cava or PA, is the most common form of hemodynamically significant coronary anomaly. Physiologically, a left-to-right shunt exists in such cases, except in the rare instances when the termination is in a left heart chamber. Approximately half of these patients are asymptomatic and undergo cardiac catheterization because of a continuous precordial murmur.' The rest may develop congestive heart failure, subacute bacterial endocarditis, myocardial ischemia or infarction resulting from a "steal" phenomenon, or rupture of an aneurysmal fistula.2 The symptoms, murmur, plain radiographic findings and electrocardiographic changes of coronary fistulae may closely resemble those of other lesions, such as patent ductus arteriosus, aorticopulmonary window, ventricular septal defect with aortic regurgitation, rupture ...

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Gianturco coils were used to embolize 77 vessels in 54 patients: 58 aortopulmonary collateral vessels, 14 Blalock-Taussig shunts, 3 arteries involved in pulmonary sequestrations and 2 venae cavae. Embolization resulted in total occlusion in 53 (69%), subtotal occlusion in 19 (25%) and partial occlusion in 3 (1 intentional). Two embolizations failed to reduce flow. Thus, 72 (95%) of 76 embolizations in which complete occlusion was the intended result resulted in total or subtotal occlusion. Analysis of the results demonstrates that completely occluded collateral vessels were longer and had a smaller diameter than did incompletely occluded vessels. Complications included six cases of inadvertent embolization to the pulmonary arteries (n = 5) or the aorta (n = 1); three were retrieved and three were left without symptoms. In addition, there was a case of severe hemolysis after intentional partial occlusion of a Blalock-Taussig shunt. The results demonstrate that coil embolization can be an effective procedure for managing a wide variety of aorto-pulmonary collateral vessels and shunts in children with congenital heart disease.

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We prospectively examined the usefulness of electrocardiography, chest radiography, and M-mode echocardiography in discriminating between the presence and absence of heart disease in 280 children older than one month and newly referred for evaluation of a heart murmur. After taking a history and performing a physical examination but before reviewing diagnostic tests, we categorized the children as having "no heart disease" (142), "possible heart disease" (34), or "definite heart disease" (104). Among the children initially thought to have no heart disease, the diagnosis was changed after a review of diagnostic tests in eight--three with mitral-valve prolapse, two with possible cardiomyopathy, and three with no heart disease on follow-up. Among those initially thought to have possible heart disease, the tests changed the diagnosis to definite heart disease in four, of whom only one had heart disease (mitral-valve prolapse) on follow-up. In no case did a review of tests change the diagnosis of definite heart disease. We conclude that the results of diagnostic tests are unlikely to change the clinical diagnosis of no heart disease or definite heart disease, when made by a qualified pediatric cardiologist in children newly referred for evaluation of a heart murmur.

No geometric and performance changes from the volume-loaded stage are noted 6 to 9 months after the hemiFontan procedure; however, major changes occur 1 to 2 years after the Fontan procedure. The dramatic changes in the mass/volume ratio seen early after the hemiFontan procedure were not detected at 6 to 9 months. Furthermore diminution of mass, volume and ventricular performance are present at least 2 years after the Fontan procedure.

Percutaneous balloon valvotomy was attempted in 27 patients (aged 6 days to 19 years, median 2 years, 11 months) with unoperated typical valvular pulmonary stenosis using a balloon 7 to 60% (mean 30%) larger than the valve anulus. One patient had undergone a previous balloon valvotomy elsewhere. To achieve an oversized dilation diameter in three larger patients, two balloons were inflated side by side. Their "effective dilation diameter" was determined by the diameter of the circle with the same area as that of the oval enveloping the two balloons. A significant reduction of the transvalvular gradient occurred in all patients (mean +/- SD = 74.3 +/- 14.7%, range 33 to 100%). The average gradient of 65.0 +/- 19.0 mm Hg (mean +/- SD) fell to 15.9 +/- 7.6 mm Hg (0 to 30 mm Hg). Twenty-five of 27 patients had a residual transvalvular gradient of less than 25 mm Hg. The calculated valve orifice area increased by an average of 183 +/- 80%. No significant complications occurred. It is concluded that percutaneous balloon valvotomy with a balloon 20 to 40% larger than the valve anulus is the treatment of choice for typical congenital valvular pulmonary stenosis.