L. C. Blieden

Unifying link between Noonan's and Leopard syndromes?

1 . Bedford DE (1960) The anatomic types of atrial septal defect . Ann J Card 6:568-574 2. Gotsman MS, Astley R, Parsons CG (1965) Partial anomalous pulmonary venous drainage in association with atrial septal defect. Br Heart J 27:566-571 3. Chartrand C, Payot M, Davignon A, Guerin R, Stanley P (1976) A new surgical approach for correction of partial anomalous pulmonary venous drainage into the superior vena cava . J Thorac Cardiovasc Surg 71:29-34 4. Lewin AN, Zavanella C, Subramanian S (1978) Sinus venosus atrial septal defect associated with partial anomalous pulmonary venous drainage : surgical repair. Ann Thorac Surg 26:185-191 5. Kyger ER, Frazier OH, Cooley DA, Gillette PC, Reul Sandiford FM, Wukasch DC (1978) Sinus venosus atrial septal defect : early and late results following closure in 109 patients . Ann Thorac Surg 25 :44-50 6. Nasser FN, Tajik AJ, Seward JB, Hagler DJ (1981) Diagnosis of sinus venosus atrial septal defect by two-dimensional echocardiography . Mayo Clin Proc 56 :568-572

Criss-cross heart—a case with horizontal septum, complete transposition, pulmonary atresia and ventricular septal defect

SummaryCriss-cross heart is a recently described anomaly in which the systemic and pulmonary blood streams cross at the atrioventricular (AV) level, without mixing. A case of criss-cross heart is described in which the right atrium, in a solitus position, communicated with a left-superior positioned, morphologically right ventricle, and the left atrium communicated with a normally located, morphologically left ventricle. The interventricular septum occupied a horizontal plane. Associated defects were complete d-transposition of the great arteries with l-positioned aorta, pulmonary atresia, venticular septal defect, atrial septal defect, and patent ductus arteriosus. To the best of our knowledge this is the first angiocardiographic demonstration of this rare combination of lesions.The literature on criss-cross heart and horizontal septum is reviewed. It is stressed that regardless of whether the criss-cross phenomenon is an anatomical fact or an angiocardiographic illusion, it is an established angiocardiographic entity and should be recognized as such.

Right aortic arch with left descending aorta (circumflex aorta) Roentgenographic diagnosis

Right aortic arch with retroesophageal segment is a rare anomaly with distinctive roentgenographic features. We present 2 patients, who on the barium oesophogram had an additional finding which has not previously been described. The typical features include an indentation on the right upper barium filled oesophagus (in the antero-posterior view), an oblique indentation descending from right to left (caused by the retroesophageal segment) and the left descending aorta. The oblique indentation has not previously been described. The embryology and incidence of the lesion are briefly discussed.

Discrete subaortic stenosis associated with congenital valvular aortic stenosis—A diagnostic challenge

Discrete subaortic stenosis has only rarely been reported in association with congenital valvular aortic stenosis. The valvular lesion may obscure the subaortic stenosis or may be obscured by it. Our experience with seven such cases (10% of our 71 patients with discrete subaortic stenosis) is reported and the diagnostic problems are discussed. In six cases the discrete subaortic stenosis was membranous and in one it was a fibromuscular tunnel. Two patients had associated coarctation of the aorta, two had aortic insufficiency, and one had a ventricular septal defect. An important clinical clue to the diagnosis was an ejection systolic click, which was found in four patients. Four patients were accurately diagnosed at cardiac catheterization. Two patients, in whom cross-sectional echocardiography (2DE) was performed, were accurately diagnosed by it.

Cerebral arteriovenous fistula causing congestive heart failure in infancy.

The case of a newborn female in congestive heart failure due to a cerebral A-V fistula is presented. The cardiac manifestations in the presence of this condition are described, and the diagnostic importance of auscultation of the skull is stressed. Because of the precarious condition of most of these neonates, diagnostic procedures should be minimized if the clinical picture is compatible with this diagnosis, and cerebral angiography should be performed before any heart studies are undertaken.

“Complete-transposition-like” coronary arterial pattern in single ventricle with inverted infundibulum and transposition of the great arteries

In 19 out of 28 cases of single ventricle with inverted infundibulum and 1-transposition of the great arteries the right coronary artery arose from the posterior aortic sinus, the right anterior aortic sinus being the noncoronary one. This coronary arterial pattern was described before only in association with d-transposition of the great arteries. We suggest that angiocardiographic demonstration of this coronary arterial pattern in association with an 1-positioned aorta favors the diagnosis of single ventricle.

Interruption of the Aortic Arch With Complete Transposition of the Great Arteries Clinical and Angiocardiographic Diagnosis At the Age of One Day

The association of complete trans portation of the great arteries and com plete interruption of the aortic arch is very rare. This combined lesion was diagnosed clinically in a 1-day-old in fant in whom it caused cyanosis of the upper half of the body. The diagnosis was confirmed angiocardiographically. We believe this is the youngest patient in whom this diagnosis was made in vivo.