Abraham Shem-Tov

Corrected transposition of the great arteries without associated defects — study of 10 patients

Abstract In about 80 percent of the cases of corrected transposition of the great arteries reported in the literature, other primary lesions, mainly ventricular septal defect and/or pulmonic stenosis were present, and the isolated lesion is only rarely found [1–3]. We studied 10 cases of corrected transposition of the great arteries without shunt or obstructive lesions.

Subaortic stenosis: An unrecognized problem in transposition of the great arteries

Right ventricular (subaortic) obstruction has only rarely been described in complete transposition of the great arteries. five patients with complete transposition of the great arteries in whom subaortic stenosis was angiocardiographically demonstrated were studied. All had a pressure gradient of 30 to 55 mm Hg across the aortic outflow tract. Two of the patients manifested mild tricuspid insufficiency, and another two had coarctation of the aorta. The etiologic, anatomic, hemodynamic and prognostic aspects of this unusual anomaly are discussed. An angiocardiographically demonstrated series of this anomaly has not been reported on before.

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

Juxtaposition of atrial appendages: Angiocardiographic observations

Abstract The angiographic features of four cases of left-sided juxtaposition and one case of right-sided juxtaposition of the atrial appendages are described. The anomalous appendage was cephalad and dorsal to the normal position of the left atrial appendage and was larger than normal during atrial diastole; the appendage located on the anatomically correct side was caudad and its diastolic size was less than normal. The four cases of left-sided juxtaposition were all associated with transposition of the great vessels. Right-sided juxtaposition was detected inadvertently in a patient with a congenital obstructive lesion of the mitral valve. Left-sided juxtaposition can be demonstrated angiocardiographically by opacification of the right atrium, whereas right-sided juxtaposition can be seen by contrast injection into any cardiac chamber but the left ventricle, with certain exceptions.

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.

Retroesophageal right aortic arch

SummaryRight aortic arch with left descending aorta and a retroesophageal aortic segment is an uncommon congenital vascular malformation which may cause symptoms of compression from a vascular “ring.” The presence of this malformation may be suspected by the findings on barium swallow and the diagnosis confirmed by angiocardiography. Surgical intervention may be necessary in patients who have severe symptoms.

Diagnosis of pulmonic regurgitation by contrast echocardiography

In order to determine whether pulmonic regurgitation (PR) can be reliably diagnosed using contrast echocardiography, we studied 24 subjects using echocardiography during intravenous injections of 5% dextrose solution. Twelve were without PR, and twelve had PR (10 after intracardiac repair of tetralogy of Fallot, one after a Brock procedure for pulmonic stenosis, and one after insertion of a right ventricle-pulmonary artery conduit for pseudo truncus arteriosus). Two blinded independent observers correctly diagnosed PR in all patients when it was present, and correctly excluded it in 11 of 12 of the patients without PR. They disagreed in one patient who had an unusual contrast pattern during diastole. Four postoperative patients had PR by contrast echocardiography despite the absence of a cardiac murmur at the time of their study. We conclude that contrast echocardiography is a safe, simple, sensitive, and specific method for diagnosing PR. Some methodologic suggestions and limitations of the method are 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.

“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.