Use of the new IntraStent for treatment of transverse arch hypoplasia/coarctation of the aorta.

We report the use of a new biliary stent (IntraStent Double Strut LD) adapted for use in a 16-year-old young man with moderate-severe transverse arch hypoplasia/coarctation of the aorta following two surgical attempts at correction/relief of the coarctation. The stent implantation procedure resulted in complete relief of the coarctation.

Transcatheter coil occlusion of a thoracic arteriovenous fistula in an infant with congestive heart failure.

An 8-week-old baby boy presented at our institution with a continuous murmur and congestive heart failure. Echocardiography showed normal cardiac anatomy. Catheterization revealed the presence of a large thoracic arteriovenous fistula between the descending thoracic aorta and the hemiazygous system, with eventual drainage into the azygous vein and the innominate vein. Coil occlusion was performed successfully with a Gianturco coil.

Transcatheter closure of large persistent left superior vena cava causing cyanosis in two patients post-Fontan operation utilizing the Gianturco Grifka vascular occlusion device.

We report the successful transcatheter closure of a large persistent left superior vena cava draining into the pulmonary venous circulation causing cyanosis in two patients who had previously undergone the Fontan operation utilizing the Gianturco Grifka vascular occlusion device. Cathet Cardiovasc Intervent 2001;53:398-404.

Successful double bypass grafting in a child with coronary artery obstruction due to Kawasaki disease.

We describe the clinical evaluation and surgical treatment of a 7-year-old child who had severe coronary artery obstructions that occurred as a sequela of previously diagnosed Kawasaki disease.

Transtelephonic echocardiography: successful use in a tertiary pediatric referral center.

Recent advances in computers and telecommunications have made transtelephonic echocardiography practical. Our institution is developing a network of transmissional echocardiographic sites at several hospitals in our referral region. We reviewed our initial experience to determine whether transmissional echocardiographic studies (1) gave an appropriate diagnostic impression compared with subsequent videotape review and (2) led to appropriate clinical management (i.e., transfer to a tertiary center or continuation of local care and follow-up). From Aug. 1, 1991, to May 31, 1992, we evaluated 47 transmissional studies (diagnoses: 24 normal, 8 patent ductus arteriosus, 6 ventricular septal defect, 2 pulmonary stenosis, 1 ventricular septal defect with interrupted aortic arch, and 6 miscellaneous). Of 47 studies, 39 (83%) were thought to give accurate diagnostic impressions compared with videotape review. Most inaccuracies were due to the selection and transmission of inconclusive information in an otherwise accurate diagnostic study. Only 1 (2%) of 47 studies resulted in an inappropriate clinical decision; a patient's transfer for treatment of a potent ductus arteriosus was delayed 1 day because of an inconclusive transmissional study. We conclude that transmissional echocardiography is useful in the management of pediatric patients with suspected heart disease in a regional referral setting.

Echocardiography for evaluating anatomic repair of transposition of the great arteries.

Fifteen survivors of a single-stage anatomic correction of complete transposition of the great arteries performed at a mean age of 1 month were studied by cross-sectional echocardiography, conventional Doppler echocardiography, Doppler color flow mapping, and catheterization at mean of 10 months after surgery. Small aortic regurgitant jets were documented by angiography and Doppler color flow mapping in seven patients. A trivial supravalvular pulmonary gradient was present in seven patients and a mild to moderate gradient in three. Pulmonary artery flow velocities correlated well with those measured at cardiac catheterization (r = 0.96, standard error of the estimate = 2.6 mm Hg). The present study suggests that combined cross-sectional echocardiography, conventional Doppler echocardiography, and Doppler color flow mapping can be used in the follow-up of patients who have had anatomic repair of transposition of the great arteries, reducing the need for repeated postoperative cardiac catheterizations.

Two-dimensional echocardiographic assessment of intracardiac masses in infants and children.

We studied six infants and children with intracardiac masses of different causes. The diagnosis was made noninvasively using two-dimensional echocardiography in all six patients. In five patients, surgical excision of the mass was undertaken with good results. Two-dimensional echocardiography is an excellent noninvasive diagnostic tool that allows the clinician to determine the size and location of intracardiac masses in infants and children.

An electrocardiographic midterm follow-up study after anatomic repair of transposition of the great arteries.

Prospective studies of rhythm and conduction, before and after 1-stage anatomic repair of simple transposition of the great arteries, were performed on 24 survivors. Pre- and postsurgical serial standard electrocardiograms were obtained on each patient. Fourteen patients underwent perioperative 24-hour electrocardiograms; all had follow-up 24-hour electrocardiograms. Rare atrial or occasional ventricular premature complexes were detected in 3 (11%) patients before operation. After surgery, 1 patient developed right bundle branch block. Two patients developed a left bundle branch block. One patient had a QS pattern in V6, which disappeared on follow-up electrocardiogram. Transient second-degree atrioventricular block was detected in 1 patient. A normal P-R interval and P-wave axis were present in all but 1 patient. Mild sinus bradycardia or rare atrial or ventricular premature complexes were detected in 4 of twenty-nine 24-hour electrocardiograms performed in the first 2 years after surgery. At 3 years after repair, 5 patients had a normal 24-hour electrocardiogram and 1 had low-grade ectopy (rare atrial and ventricular premature complexes). At 4 years, all 4 patients studied had normal 24-hour electrocardiograms. During a mean follow-up of 3 years, we have yet to document any symptomatic arrhythmias.

Factor VIII related antigen (von Willebrand's factor) in Kawasaki disease.

Kawasaki disease is a systemic vasculitis in which secondary development of coronary artery aneurysms can occur. Because Factor VIII related antigen has been found increased in other vasculiditides, VIII R:Ag was measured serially in patients with Kawasaki disease. Factor VIII related antigen was prospectively evaluated in the acute phase of ten patients with Kawasaki disease, all of whom showed increased values at this stage (p greater than 0001). In six children a second sample was drawn at the convalescent phase, and all were normal. Of the original ten patients, two developed coronary artery aneurysms. Acute Factor VIII related antigen levels were not higher nor did Factor VIII related antigen fail to return to baseline in these two patients. Based on our findings, Factor VIII related antigen is elevated in the acute phase of Kawasaki disease and returns to normal levels in the convalescent phase.

Surgical repair of atypical coarctation of the abdominal aorta in an infant.

A seven-week-old infant with multiple areas of coarctation of the abdominal aorta and renal artery pathology underwent staged surgical repair of his malformations in our medical center. The clinical and routine echocardiographic evaluations suggested the diagnosis of atypical coarctation of the aorta. The exact anatomical characteristics of his vascular malformations were established by repeated angiocardiographic studies, which should be performed in any patient in which the diagnosis of atypical coarctation of the aorta is suspected.

Infant orthotopic cardiac transplantation.

Infant orthotopic cardiac transplantation has been recently applied to various forms of congenital heart disease with encouraging short-term results. Between June 1986 and September 1987 we evaluated 16 infants for orthotopic cardiac transplantation. Fourteen had hypoplastic left heart syndrome, one had endocardial fibroelastosis with aortic atresia, and one had anomalous pulmonary arterial origin of the left main coronary. Eight families accepted the treatment program and eight families refused (two because of associated anomalies and six on philosophical grounds). Of the eight patients who were candidates for orthotopic cardiac transplantation, one died 6 hours after diagnosis, one was allowed to die after 60 days because of acquired neurologic complications, and another had congenital cytomegalic virus infection. The remaining five patients (four with hypoplastic left heart syndrome, one with anomalous pulmonary arterial origin of the left main coronary) had orthotopic cardiac transplantation. The operation was performed with absorbable polydioxanone suture with deep hypothermia and circulatory arrest in four neonates for hypoplastic left heart syndrome (average time 47 minutes) and bicaval cannulation and continuous bypass in one 11-month-old infant for anomalous origin of the left main coronary. In-house retrieval was used in all. One neonate died of complications as a result of pretransplant donor heart dysfunction and size discrepancy, whereas the remaining three neonates and one infant survived and are home 23 months, 12 months, and 8 months (the patients with hypoplastic left heart syndrome) and 17 months (the patient with anomalous origin of the left main coronary) postoperatively. Triple-drug immunosuppression included cyclosporine, azathioprine, and prednisone. Rejection was diagnosed by clinical evaluation of child activity and monocyte cell cycle analysis from peripheral blood samples without myocardial biopsies. Routine echocardiograms, electrocardiograms, and chest x-ray films were not helpful. Six episodes of rejection were successfully treated in four patients. Twelve-month postoperative catherization in one patient (hypoplastic left heart syndrome) showed appropriate graft growth, no aortic or pulmonary anastomotic strictures, normal right and left ventricular function, and no coronary artery disease. We conclude that infant orthotopic cardiac transplantation is an acceptable procedure for severe forms of untreatable congenital heart disease. The excellent short-term results warrant continued application of orthotopic cardiac transplantation.

Neonatal refractory supraventricular tachycardia: successful treatment with amiodarone.

A 3-week-old neonate with supraventricular tachycardia unresponsive to traditional therapy was treated successfully with amiodarone. An electrophysiologic study suggested the presence of a concealed left-sided accessory atrioventricular pathway. Because of its significant side effects, amiodarone should be used only as a last resort in the treatment of neonatal supraventricular tachycardia.

The prognostic value of intraoperative pressure gradients with congenital aortic stenosis.

Patients with congenital aortic stenosis are subject to sudden death without prior symptoms. Indications for operation are based on pressure gradients, but intraoperative evaluation of results after relief of obstruction can be subjective and arbitrary. Between September, 1981, and October, 1983, 21 patients underwent operation for relief of congenital aortic stenosis. Fifteen had valvular, 4 had supravalvular, and 2 had subvalvular stenosis. Seven patients were 1 year of age or younger, 2 being 3 days old. Preoperative inotropic support and emergency operation were necessary in 2 patients. Intraoperative pressure gradients were measured in all but the 2 patients who were 3 days old. All patients survived without complications. Intraoperative and postoperative pressure gradients were available in 16 patients. The average preoperative pressure gradient was 76 mm Hg. It was significantly lowered to 20 mm Hg (p less than 0.001) intraoperatively, which compared with 15 mm Hg postoperatively. Three patients had an unsatisfactorily high intraoperative pressure gradient after initial intervention. Immediate reexploration and incision of the rudimentary commissure in 2 and excision of redundant valvular tissue in the other resulted in a more satisfactory pressure gradient without substantial aortic insufficiency. We suggest that measurement of intraoperative pressure gradients can accurately assess the results of therapy and allow the surgeon the option of reexploration for further intervention. Moreover, the high correlation between intraoperative and postoperative pressure gradients can help in planning postoperative management and future cardiac catheterizations.

Modified aortoseptoplasty for anular abscess and erosion of the membranous septum.

Surgery for bacterial endocarditis may require extensive debridement and appropriate reconstruction for optimal results. Excision of the aortic valve and eroded membranous septum due to pneumococcal endocarditis with modified aortoseptoplasty was successfully accomplished in a six-year-old child.

Left atrial-left ventricular bypass for congenital mitral stenosis.

The case of a patient with congenital mitral stenosis successfully treated by insertion of a left atrial-left ventricular valved conduit is presented. After insertion of the conduit, pulmonary artery pressure was normal, persistent pulmonary edema and ascites disappeared, and the child's rate of growth and level of activities returned to normal.

Detecting ductal shunting in premature infants by range-gated Doppler echocardiography.

Range-gated pulsed Doppler echocardiography has been reported to be a useful noninvasive bedside technic for detecting ductal left-to-right shunting in premature infants. We studied 30 premature infants with a birth weight of less than 1,500 gm, using a 5 MHz system developed by Advanced Technology Laboratories. An umbilical artery catheter was used to obtain a contrast aortogram. Twenty-three infants had both a positive flush aortogram and a continuous turbulence documented by Doppler echocardiography. A clinically audible murmur was present in 17 of these patients. In six infants there was no Doppler evidence of PDA. In five of this last group there was also a negative flush aortogram. The excellent correlation found between the Doppler and aortographic studies suggests that this noninvasive technic is very sensitive for detecting ductal left-to-right shunting, even in the absence of an audible murmur.