11/214. Aortic septal defect and coronary-systemic micro-fistulae. This report concerns a 32-year-old man, who at the age of 11, had an aortic septal defect with severe pulmonary hypertension. The defect was partially closed and the patient was left with a continuous murmur, an a-v shunt and marked diminution of pulmonary hypertension. Five years later he was asymptomatic, auscultation was normal and no shunt was found at cardiac catheterization. At 32 years of age, although asymptomatic he had abnormal "T" waves, and a selective coronary angiography demonstrated micro-fistulae involving the anterior descending coronary artery. It is suggested that these fistulae may be responsible for the abnormality of ventricular repolarization. ( info) |
12/214. The angiocardiographic appearance of the endocardial cushion defect in selected transposition and malposition complexes. Although the angiocardiographic features of the endocardial cushion defect (ECD) have been well described in the patient with two normally related ventricles (D-ventricular loop in situs solitus) and normally related great arteries, little attention has been focused on the angiocardiographic appearance of the ECD in patients with complex cardiovascular disease. Because of recent surgical advances in the treatment of single ventricle and double outlet right ventricle complexes, it has become increasingly important to document the status of the atrioventricular valves in these patients. The angiocardiographic features of the ECD are therefore described in two patients, one with D-loop, single left ventricle and outlet chamber, D-transposition of the great arteries, bilateral conus and pulmonary stenosis and the other with dextrocardia, situs inversus, double outlet right ventricle, common atrioventricular canal and pulmonary stenosis. In both patients, the left ventricular outflow tract, despite aortic-mitral discontinuity, is characterized by elongation, scalloping and serration, and the classic appearance of the "gooseneck" deformity is observed on left ventricular angiography. It thus appears that aortic-mitral continuity and two well-formed, normally related ventricles and great arteries are not invariable necessary to the manifestation of the "gooseneck" deformity in the patient with complex cardiovascular disease and associated endocardial cushion defect. ( info) |
13/214. Transient right-to-left shunt in massive pulmonary embolism. A 56-yr-old man, two months after an operation for an acoustic neurinoma, gradually developed dyspnea. Massive pulmonary embolism (MPE), with a significant right-to-left (R-L) shunt, was seen in a perfusion scan of the lungs with Tc-99m MAA. radioactivity was noted in the thyroid, spleen, kidneys and brain. A cardiac ultrasound study did not reveal intracardiac shunting. A few days later, when the patient's condition improved, another perfusion scan of the lungs did not show the shunt, whereas a subsequent digital subtraction angiographic study confirmed the diagnosis of MPE but failed to reveal the cause of the shunt. In the absence of any possible pathophysiological mechanism, to explain the observed R-L shunt, we deduce that the particles of Tc-99m MAA might have passed through the precapillary pulmonary arteriovenous anastomoses and/or through dilated pulmonary capillaries, as a result of highly increased pulmonary vascular pressure due to MPE. ( info) |
14/214. Two-dimensional color doppler echocardiographic imaging of a Gerbode defect: a case report. A Gerbode defect is a ventricular septal defect that communicates directly between the left ventricle and the right atrium. The pathology may be due to a congenital defect, can result from trauma, or can occur after endocarditis or aortic valve replacement. We report the case of a 20-year-old man who has a defect between the left ventricle and the right atrium (Gerbode defect) that was diagnosed with two-dimensional color Doppler echocardiography. ( info) |
15/214. Unbalanced atrioventricular septal defect with parachute valve. A 5-month-old male patient presented with right-dominant unbalanced atrioventricular septal defect and left-sided parachute valve, and underwent successful biventricular repair. Because of the presence of a small left ventricle, left atrium, and a single left papillary muscle, an additional orifice was created in the left-sided atrioventricular valve with artificial partitioning of the right-sided atrioventricular valve. There was no evidence of mitral stenosis or regurgitation on follow-up echocardiography. ( info) |
16/214. An unusual fetus with complete absence of thoracic, lumbar and sacral vertebrae, bilateral renal agenesis, VSD, meningomyelocele, imperforate anus, and teratoma. We present a 30 week old male fetus who had a very interesting malformation complex which can not be explained by teratogenic or hereditary diseases. The aim of this paper is to discuss this complicated entity and compare it with other reported cases. ( info) |
17/214. Complete surgical repair of aortic atresia with a normal left ventricle. Aortic atresia with a normal left ventricle and normal mitral valve is an uncommon congenital lesion. We present two such cases and describe two different approaches of achieving biventricular surgical repair. ( info) |
18/214. pulmonary atresia with intact ventricular septum, right-sided aortic arch and ventriculocoronary connection--prenatal echocardiographic diagnosis. The authors describe the prenatal echocardiographic diagnosis of a rare case of pulmonary atresia with intact ventricular septum, right-sided aortic arch, and ventriculocoronary connection in a fetus at 21 weeks gestation. The diagnosis was confirmed at autopsy. ( info) |
19/214. Trigonomicrocephaly, severe micrognathia, large ears, atrioventricular septal defect, symmetrical cutaneous syndactyly of hands and feet, and multiple cafe-au-lait spots: new acrocraniofacial dysostosis syndrome? We report on a patient with a unique constellation of anomalies comprising trigonomicrocephaly, asymmetric severe micrognathia, large ears, atrioventricular septal defect, vertebral anomalies, bilateral cutaneous syndactyly of fingers and toes, unilateral cryptorchidism and multiple cafe-au-lait spots. The mother of the propositus has multiple cafe-au-lait spots. Search of POSSUM and the london Dysmorphology database (LDDB) uncovered no similar case. We think that this patient represents a new acrocraniofacial dysostosis syndrome. ( info) |
| esophageal perforation caused by transesophageal echocardiography in an infant is believed to be extremely rare. If unrecognized, serious morbidity can result. We report a case of pharyngeal perforation in a neonate undergoing an interrupted aortic arch repair. ( info) |