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The clinical and electrocardiographic features of anomalous left coronary artery from the pulmonary trunk, ALCAPA, a frequently fatal congenital cardiac malformation, are described in an 8 1/2-month-old female.

There has been a marked increase in device closure of secundum atrial septal defects (ASDs) in the last decade. No clinical reports of late results have been forthcoming until now. The purpose of this report was to provide very long-term follow-up of nonoperative closure of secundum ADSs using the King-Mills Umbrella device. ASD closure using this technique did not appear to protect against the known secundum ASD complication of atrial arrhythmia. Twenty-seven-year follow-up of the umbrella ASD closure via the femoral vein shows effective occlusion, absence of device complications, and no reinterventions.

Case histories of 8 consecutive patients who underwent surgical correction of Type C complete atrioventricular (A-V) canal at the Ochsner Medical Center over the past 2 years were analyzed. Ages ranged from 1 ⅓ to 14 years. Before bypass, the level to which the common leaflets ascend and the arc that they inscribe are determined by palpation to ensure their proper reconstitution on the canal patch. The major area of closure is usually allocated to the ventricular portion. Mapping the conduction system and tying sutures with the heart beating in high-risk conduction areas prevent heart block. Precise marking and splitting of the valve leaflets and reattachment with interrupted felt-buttressed sutures avoid disruption. All patients survived the operation and have shown clinical improvement. At recatheterization, 7 of 8 patients have shown a definite decrease in mitral insufficiency. Right ventricular pressures returned toward normal and there were no significant residual shunts. Associated anomalies or previous operations have not been a contraindication, and since we have begun using the present technique, we have achieved consistently good results without prosthetic valves.

Efforts to close left-to-right shunts at Ochsner Medical Institutions have been directed toward atrial septal defects (ASD) and patent ductus arteriosus (PDA). PDA's were constructed in dogs by interposing a segment of jugular vein between the aorta and main pulmonary artery. Five dogs in which the PDA was closed by a plug device inserted through the femoral vessels were put to death at 6 to 12 months. Histologic sections showed good fibrous ingrowth with endothelial covering on the aorta and pulmonary artery sides. There were no migrations or residual shunts. At cardiac catheterization, 18 patients had ASD's sized and located as to position in the septum. The sizes ranged from 13 to greater than 30 mm. in diameter. The ASD sizes in patients who underwent standard operative closure were compared to the measurements at catheterization, and the variation was insignificant. In 5 patients, centrally positioned secundum ASD's were closed with double umbrella devices, 25 to 35 mm. in diameter. Anatomic contraindications for umbrella closure included ASD's greater than 30 mm. in diameter, anomalous pulmonary venous connection, common atrium, inferiorly or superiorly located secundum ASD, and sinus venosus ASD. Follow-up studies from 6 to 12 months on 5 patients with umbrella closure have revealed no hemolysis, arrhythmias, thromboembolism, migration, or other untoward effects.

A case of double mitral valve orifice associated with atrioventricular canal was found at operation. These two anomalies are frequently associated. Double mitral valve is usually subclinical and does not necessarily need to be corrected, depending on the associated anomalies, the extent of the defect, and the personal experience of the surgeon. The embryology of double mitral valve is complicated and still needs to be clarified.

The size of experimental and clinical secundum atrial septal defects may be accurately measured during cardiac catheterization. The position of the defects in the septum and their distance from the aortic and mitral valves may also be ascertained. A balloon catheter inflated with radiopaque dye is used for the measurement. The volume of dye in the balloon has been correlated with its maximal diameter. Thus, a no. 8-10 French catheter filled with 10 ml of diatrizoate (50 percent) has a diameter of approximately 25 mm and a no. 8-22 French catheter filled with 40 ml of diatrizoate a diameter of approximately 43 mm. Persistent arrhythmias or other adverse effects of the procedure were not observed. There was a high correlation between balloon-measured atrial septal defects and those measured at necropsy in the animals or at operation in the patients. These measurements aid in determining whether an ostium secundum defect is of proper size and location to be closed transvenously and may also prove valuable in physiologic studies of atrial septal defects or other cardiac anomalies.

The origin of body surface P waves was evaluated in the intact dog by recording body surface isopotential maps and atrial epicardial potential maps. P waves were recorded from 150 points on the body surface, and 80 atrial unipolar electrograms were recorded by means of permanently implanted atrial electrodes during normal sinus rhythm and during atrial pacing. When the atrial pacing sites were changed, the associated shape changes in body surface P waves were highly dependent on the position of the recording sites. Throughout most of the P wave, multiple maxima were present on the body surface, and these were caused by widely separated right and left atrial excitation waves; however, the presence of several simultaneous atrial excitation waves could not be appreciated from the body surface maps when the atrial excitation waves were close to one another. In the last third of atrial excitation there were two maxima on the body surface, one due to atrial excitation waves and the other due to early atrial repolarization. In contrast to the more complicated patterns during excitation, during the P-R segment a single maximum and a single minimum were present on the body surface. The sequence of potential changes on both body surface and atrial epicardium during repolarization was similar to the sequence of excitation except that there was reversed polarity.

Atrial septal defects (ASDs) were successfully created by punch biopsy technique in 67 dogs. The technique is simple, effective and provides an ideal model for the study of ASDs, either for testing closure devices or physiological experiments. The position and size of the defects may be varied according to preference. Of 45 animals subjected to cardiac catheterization, 39 (87 percent) has a patent atrial septal defect.

Laryngotracheal esophageal cleft is a rare congenital anomaly that should be considered in the differential diagnosis of any neonatal respiratory distress aggravated by feeding. Diagnosis is difficult and repeat endoscopic examinations may be necessary. In complete clefts, immediate surgical repair is indicated with combined approach through the chest and neck and incorporation of part of the esophageal wall into the repair. Because the cleft may be associated with esophageal atresia and/or tracheal esophageal fistula, it should be suspected in patients who have persistent symptoms after successful repair.

A 5-year-old boy had clinical, cardiac catheterization, and operative findings of a thoracic arteriovenous fistula arising from the right subclavian artery. The venous connection was to the paravertebral plexus with eventual drainage into the inferior vena cava through the right common iliac vein. To our knowledge, this type of fistula has not been reported previously and is believed to represent embryological defects of the first or second posterior intercostal arteries as well as maldevelopment of the venous connections of azygos vein to the superior vena cava, which resulted in the observed caudal venous pathways becoming operative.

The occurrence of aneurysmal dilatation and endocardial fibroelastosis of the left atrial appendage in an 11-year-old boy who experienced chest pain is described. During exploratory thoracotomy the pericardium was found to be intact. Only one previous report of aneurysmal dilatation and endocardial fibroelastosis of the left atrial appendage was found.

The potential distribution at the atrial surface during depolarization and repolarization was studied in intact dogs. A preparation was developed by implanting 30 to 40 miniature electrodes permanently on each atrium to record unipolar electrograms in the intact animal. Heart block was created to dissociate atrial and ventricular activity. The electrograms were recorded on magnetic tape and atrial isopotential heart maps produced with the use of a digital computer. The changing potential distribution during excitation indicated the early presence of multiple wave fronts which were related primarily to the crista terminalis, Bachmann's bundle, and a special bundle to the base of the right appendage. The interatrial septum provided a conducting bridge which had an important influence of global atrial excitation, depending on the site of impulse formation. Colliding excitation wave fronts were quite prominent. During terminal atrial excitation, repolarization maxima were present simultaneously with depolarization maxima. Repolarization was characterized by a changing potential distribution which followed the same general pattern as excitation spread; and, furthermore, the earliest areas of excitation were associated with a repolarization maximum and terminal areas of excitation were associated with repolarization minima.

A 17-year-old girl had clinical and cardiac catheterization findings compatible with a secundum atrial septal defect. During cardiac catheterization, the atrial septal defect was sized and closed using a transvenous umbrella technique. (JAMA235:2506-2509, 1976)