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This report describes the use of the Amplatzer patent ductus arteriosus occluder to close a left ventricle to descending aorta conduit. The patient was a 10-year-old male who was born with critical aortic stenosis and left ventricular outflow tract obstruction. After initial valvotomy, he underwent left ventricular to descending aorta conduit placement. At the age of 10, he had a Konno procedure to enlarge the left ventricular outflow tract and 21-mm St. Jude aortic valve placement. Closure of the conduit was not addressed because it was inaccessable from median sternotomy. Postoperatively, echocardiogram revealed significant flow through the conduit with a wide pulse pressure. Cardiac catheterization was performed with the premise to close the conduit with an Amplatzer patent ductus arteriosus occluder device.

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Patients with double aortic arch may require lengthy intubation for ventilatory support. The need for endotracheal and nasogastric intubation may be prolonged in such patients because of associated tracheomalacia. Iatrogenic tracheal or esophageal erosion with subsequent aortic fistulization is an unusual but catastrophic complication that may result from such intubation. We report the cases of 2 infants with double aortic arch and tracheomalacia who developed iatrogenic esophageal-aortic erosion. This complication was successfully managed in 1 of the infants. We conclude from our experience that the important steps in preventing this complication include 1) expediting the exclusion of upper-airway compromise in intubated infants who have a presentation characteristic of bronchospastic airway disease (hyperinflation and hypercapnia) that seems unresponsive to usual therapeutic measures; and 2) expediting the diagnosis of vascular ring in order to minimize the duration of dual tracheal and esophageal intubation. Effective management of this problem, once established, requires primary closure of the esophageal perforation, removal of the nasogastric tube, interposition of thick viable tissue between the esophagus and the aorta, and decompressive gastrostomy and feeding jejunostomy. Concomitant aortopexy may be appropriate.

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Pulmonary atresia with an intact ventricular septum and a small right ventricle is associated with high mortality and lacks a consensus surgical approach. The results of operations in eight of eleven patients with either pulmonary atresia and an intact ventricular septum or critical pulmonary stenosis, hypoplastic right ventricle, and intact ventricular septum, who were operated on between 1983 and 1989, are presented. Definitive correction was performed via a right ventricular transannular patch with prolonged postoperative prostaglandin E1 infusion. Limiting conditions in using this approach were (1) severely hypoplastic right ventricle, (2) massive tricuspid regurgitation, or (3) right ventricle-dependent coronary artery blood supply. Eleven neonates had the aforementioned diagnoses; eight underwent definitive repair, five successfully. Successful outcome, up to 5 postoperative years, was achieved if the tricuspid valve diameter was greater than or equal to 0.75 cm, or if the tricuspid/mitral valve ratio was greater than or equal to 0.70. Other significant predictors of success were a tripartite right ventricle (p less than 0.006), lack of sinusoids (p less than 0.05), the ratio of the right ventricular internal and external diameters greater than or equal to 0.73 (p less than 0.05), and some contractility (p less than 0.04). Thus we choose a right ventricular transannular patch with long-term prostaglandin E1 infusion for patients with pulmonary atresia and intact ventricular septum if (1) they have a tricuspid valve diameter of 0.75 cm or 70% of the mitral valve size, (2) they have a tripartite right ventricle, (3) they exhibit some right ventricular contractility, (4) they do not have marked tricuspid valve insufficiency, and (5) the coronary arteries do not fill primarily from sinusoids.

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We present our 12-year experience with surgical treatment of aneurysms of the thoracic aorta in a high-risk patient population. Of 52 patients with aneurysms, 36 patients, aged 19 to 80 years, had operation. In 24 of the 36, there were three or more associated major disease processes, such as chronic bronchitis, hypertensive cardiovascular disease, aortic valve disease, cerebrovascular disease, abdominal aortic aneurysm, and coronary artery disease. Eight patients with acute type A (ascending aorta) and seven with acute type B (descending aorta) aneurysms had emergency repair, with survival in four and seven, respectively. All patients with chronic type A or B aneurysms had elective repair, and all patients with acute or chronic type A aneurysms had surgical treatment. Four patients with acute type A and two with acute type B aneurysms had elective operation, with survival in three and one, respectively. Nine patients with chronic type A and six with chronic type B aneurysms had elective operation, and all survived. Twelve patients with chronic type B aneurysms did not have operation, and all were alive at early follow-up. Mortality for patients having acute type A aneurysms requiring emergency operation was greater than that after elective repair of acute or chronic type A aneurysms. Short-term survival for chronic type B aneurysms was similar whether the patient was treated medically or surgically.

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During the last fourteen years, 377 unileaflet tilting-disc prosthetic valves (Björk-Shiley and Medtronic Hall) have been used for single or multiple valve replacements with and without concomitant coronary artery by-pass grafting. In the past five years, five instances of disc immobilization (three in the mitral and two in the aortic position) occurred either at the time of weaning from cardiopulmonary bypass or immediately thereafter. When the implanted site of the prosthetic valve was the mitral position, reexploration in 2 patients revealed chordal remnants in the subannular area stuck between the disc occluder and the valve ring, thereby immobilizing the disc. In the third instance, the free movement of the disc was impeded by the left ventricular myocardium. In the aortic position, an unraveled suture impacted between the disc occluder and the valve ring immobilized the disc in 1 patient. In the other patient, the cause of the malfunction could not be determined at the time of reexploration. The 1 death among these 5 patients was directly related to the malfunction of the prosthesis. The mechanism, recognition, treatment, and prevention of this catastrophic malfunction of tilting-disc valves are discussed.

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Mucocele of the bypassed esophagus is an unusual complication of esophageal replacement and has been described only in isolated references. This report is based on our experience with 6 patients in whom a mucocele developed following esophageal replacement. Esophageal replacement was performed on 37 patients over a 10-year period at the Medical College of Georgia Hospital. A symptomatic mucocele requiring excision developed in 3 patients with achalasia, 1 with congenital tracheoesophageal fistula, 1 with esophageal atresia, and 1 with inflammatory stricture. Conduits used included stomach (4), reversed gastric tube (1), and colon (1). Our experience indicates that conversion of a closed-loop esophagus into a symptomatic mucocele is more likely in the presence of functioning, chronically irritated mucosa. The clinical features were referable to the mucocele itself or respiratory embarrassment therefrom. Thoracic roentgenograms and computed tomographic scans were diagnostic in verifying the presence of the esophageal mucocele. All five mucoceles arose from squamous epithelium. One of 3 patients with achalasia in whom a mucocele developed following esophageal replacement had premalignant changes in the mucosa. Based on this experience, our treatment of choice is early, complete excision of the mucocele.

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The case of a newborn with "absent pulmonary valve," atrial septal defect, and patent ductus arteriosus is reported. He underwent surgical repair at five weeks of age, with closure of the patent ductus arteriosus and porcine pulmonary valve insertion. The clinical course leads us to suggest that reduction of pulmonary artery pressure and pulmonary valve competence will lead to prompt improvement in tracheobronchial compression and respiratory symptoms; no pulmonary arterioplasty is needed.

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We reviewed the hospital records of 36 patients who underwent modified Heller's myotomy for achalasia between January, 1961, and December, 1982. There were 18 male and 18 female patients ranging between 17 months and 75 years old. The most frequent symptom was dysphagia, followed by regurgitation of ingested food and weight loss. Modified Heller's myotomy was performed through a transthoracic incision in 35 patients and a transabdominal incision in 1. An antireflux procedure in addition to esophagomyotomy was performed in 20 patients. There was 1 postoperative death. Thirty-three patients were followed up for periods ranging from 9 months to 21 years. The results were considered good in 27, fair in 2, and poor in 4. One of the 4 underwent repeat esophagomyotomy 71/2 years after the initial operation with a good result. The remaining 3 had an antireflux procedure at the time of esophagomyotomy. Because of recurrence of symptoms, esophagogastrostomy was performed in 1 and colon interposition in 2. These results suggest that an antireflux procedure should not be added to modified Heller's operation in the treatment of achalasia.

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Ventricular ejection fractions, calculated from radionuclide studies, and inulin clearance, were determined in 33 infants and children immediately after surgical repair of their congenital or rheumatic heart defects. Of these children, the seven whose surgery did not require a period of ischemic arrest served as controls. The immediate postoperative ejection fractions in the 26 children who did undergo a period of ischemic arrest were significantly less than their preoperative values (P less than or equal to 0.001), but this decline was not observed in the control group. Ejection fraction tended to be depressed, transiently, in children 3-5 h after open-heart surgery. Early postoperative ejection fraction was significantly related to aortic cross-clamp time even when controlling for preoperative ejection fraction (r = 0.74, n = 25, P less than or equal to 0.001). Some striking declines in ejection fraction were observed among children whose aortic cross-clamp time exceeded 42 min. The decline in ejection fraction was transient; late postoperative (greater than 1 week) ejection fraction was not significantly different from preoperative values. Though glomerular filtration rates (GFRs) were often above normal, they were significantly correlated with ejection fraction (r = 0.74, n = 19, P less than or equal to 0.01). The lower GFRs were associated with the lower early postoperative ejection fractions.

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We have reported a case of traumatic chylothorax occurring as a rare complication of fracture dislocation of the dorsal spine. When conservative management by drainage, low-fat diet, and parenteral alimentation proved unsuccessful, thoracic duct ligation effected a cure.

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Case histories of four infants with single ventricle and left atrioventricular (AV) valve atresia, with interatrial obstruction but without pulmonic stenosis, are presented. Relief of interatrial obstruction by surgical or balloon atrial septostomy resulted in a marked fall in the left atrial pressure and the pulmonary artery mean pressure and resistance as well as a marked increase in the left-to-right interatrial shunt, pulmonary blood flow, and systemic arterial oxygen saturation. Because of this predictable fall in pulmonary vascular resistance, concomitant pulmonary artery banding was performed in each case with good results. There are only a few previously reported cases of single ventricle with left AV valve atresia. Interatrial obstruction is quite common in this lesion. The concept that relief of interatrial obstruction produces a rapid and predictable fall in the pulmonary vascular resistance and that concomitant pulmonary artery banding is necessary in these infants with complex congenital heart disease is emphasized.

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The case history of an infant with tetralogy of Fallot who had a Waterston anastomosis performed at the age of 1 year is presented. Subsequent chest roentgenograms revealed a ringlike calcification which was shown at angiography and operation to be due to a false aneurysm of the right pulmonary artery at the site of the Waterston anastomosis. The aneurysm also produced unidirectional obstruction of the right pulmonary artery. A review of the literature suggested that a false aneurysm of the right pulmonary artery after a Waterson anatomosis had not been reported previously.

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A patient is presented who had cor triatriatum documented with angiography and surgery. On echocardiographic study, an abnormal, dense linear echo on "sector scan" was consistently seen to traverse the left atrial cavity obliquely; the ends of the echo were attached to the posterior aortic and lateral walls of the left atrium. In addition, high frequency oscillatory movements were evident in the echo of the posterior mitral cusp. Both echocardiographic features disappeared promptly after successful resection of the intraatrial fibrinous membrane. This experience indicates that in the presence of strong evidence of mitral stenosis, an unexpectedly normal mitral valve echogram should initiate a thorough echographic search for cor triatriatum, a treatable cardiac malformation whose diagnosis can easily be missed on "routine" echocardiographic studies.

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