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A moderate to large secundum atrial septal defect associated with isolated coarctation in infancy is likely to close spontaneously. Nine of 12 defects, including 4 defects > or =8 mm in at least 1 dimension, had either closed or were believed to be hemodynamically insignificant at follow-up, supporting the strategy of a lateral thoracotomy approach to coarctation and deferral of atrial surgery. [ABSTRACT FROM AUTHOR]

There are very few therapeutic options for severely symptomatic Fontan patients after spontaneous complete or virtual fenestration closure. Its reopening in 14 such patients led to dramatic hemodynamic improvement in most. The clinical experience with transcatheter fenestration creation and/or dilation in symptomatic Fontan patients is reported demonstrating feasibility, safety, and a novel management option for these patients.

Although balloon aortic valvuloplasty (BAV) is effective in the acute management of valvar aortic stenosis (AS), sustained benefit of this technique has been shown to vary in differing age groups. The role of BAV in the young adult with congenital, nondegenerative AS is poorly defined. The catheterization results and follow-up echocardiographic data were reviewed for all patients (n = 18) between the ages of 17 and 40 years (mean 23 +/- 7) undergoing BAV at our institution between March 1986 and January 1992. Peak-to-peak systolic ejection gradient was reduced by 55%, from 85 +/- 29 to 38 +/- 17 mm Hg (p < 0.001). Aortic valve area increased from 0.9 +/- 0.2 to 1.1 +/- 0.3 cm2 (p = 0.003). Results of dilation were inadequate in 2 patients. There were no deaths, myocardial infarction, or embolic events. The 16 "effectively" dilated patients were followed with serial echocardiography for a period of 1 to 82 months, demonstrating persistent gradient relief in most patients (maximal instantaneous gradient at follow-up, 55 +/- 17 vs 79 +/- 22 mm Hg before dilation, p < 0.001). Aortic valve replacements were performed in 5 patients, 2 with unsuccessful initial dilations. At the time of the most recent echocardiogram, 8 of 16 patients remained "incident free," with no subsequent catheterization or surgical interventions, a maximal instantaneous gradient of < or = 55 mm Hg, no more than moderate aortic regurgitation, and preserved ventricular function.(ABSTRACT TRUNCATED AT 250 WORDS)

We attempted balloon angioplasty in 9 children (ages 0.1 to 10 years) with congenital mitral stenosis. All were symptomatic with severe congestive heart failure and failure to thrive. Effective reduction in mitral gradient was initially achieved in 7 patients. For the entire group, mean valve gradient decreased from 14.8 +/- 5.0 to 8.1 +/- 6.7 mm (p = 0.0007) and mean valve area increased from 1.1 +/- 0.5 to 1.8 +/- 0.9 cm2/m2 (p = 0.003). More than mild mitral regurgitation developed in 2 patients but none required surgery for mitral regurgitation. Poor gradient relief followed dilation of valves with unbalanced chordal attachments, with restriction to the valve apparatus as in mitral arcade, and where the obstruction was not purely valvar as it is with a supramitral ring. No strokes, infection or deaths were due to the procedure. Based on these data, balloon angioplasty of congenital mitral stenosis should be considered before mitral valve replacement in younger patients and in those in whom mitral valve replacement would be problematic.

From 1946 to March 1989, 92 patients (33 women and 59 men) were seen with ventricular septal defect (VSD) and audible aortic regurgitation (AR). The VSD was subcristal in 62 patients, subpulmonary in 21 and unknown in the remaining 9. The median age of onset of AR was 5.3 years. The risk of developing AR was 2.5 times greater in those with a subpulmonary VSD. The aortic valve was tricuspid in 90% and bicuspid in 10%. Prolapse was seen in 90% of those with subcristal VSD and in all with subpulmonary VSD. Pulmonary stenosis was seen in 46% of the patients with gradients ranging from 10 to 55 mm Hg. The incidence of infective endocarditis was 15 episodes/1,000 patient years. Among 20 patients followed medically, for 297 patient years, 1 died (1959) and most have been stable, including 2 followed for greater than 30 years. In the 72 patients operated on, there were 15 perioperative and 5 late deaths. Operations consisted of VSD closure alone in 7, VSD closure and valvuloplasty in 50 and VSD closure and aortic valve replacement in the other 15. Valvuloplasty was more effective in those operated on under age 10 compared to those older than 15 years (46 vs 14%). The durability of the valvuloplasty was 76% at 12 years and 51% at 18 years.

Angiocardiograms in 36 infants with tetrad of Fallot were reviewed to determine which projections best displayed the anatomy. The occurrence of associated cardiovascular anomalies was also recorded. An additional ventricular septal defect was found in 14 percent (5 of 36), peripheral pulmonary stenoses in 30 percent (10 of 36) and surgically important coronary arterial abnormalities in 8 percent (3 of 36). This retrospective study indicated that optimal biplane angiocardiography should include: (1) right ventriculography in a sitting or hepatoclavicular view for demonstration of the right ventricular, infundibular, and pulmonary artery anatomy, (2) left ventriculography in the long axial oblique (cranially angled oblique) view for display of ventricular septal defects and coronary arteries, and (3) and aortogram at the valve level (oblique view) if coronary arteries are not well seen in the left ventricular study.

His bundle electrograms were recorded in 59 patients after intracardiac repair of tetralogy of Fallot and were correlated with the postoperative electrocardiogram. Except for five patients with first degree atrioventricular block postoperatively all patients in Group A (those with either a normal electrocardiogram or solitary right bundle branch block) had a normal A-H interval (77.7 +/- 21.6 msec) (mean +/- standard deviation); all had a normal H-V interval (39.5 +/- 7.2 msec). Patients in Group B (bifascicular block) tended to have a normal A-H interval (97.2 +/- 26.2 msec) with a prolonged H-V interval (48.8 +/- 10.7 msec). Patients in Group C (trifascicular block) had prolongation of both the A-H (160.0 +/- 32.4 msec) and the H-V interval (58.8 +/- 10.6 msec) by comparison with control values. Patients in Group D (transient complete heart block) had a normal A-H interval (79.5 +/- 28.2 msec) but a prolonged H-V interval (57.8 +/- 16.4 msec), similar to that in Group C. A good hemodynamic result was associated with a normal H-V interval; a prolonged interval accompanied a poor result.

Although therapeutic catheter procedures for congenital heart disease are still developing, the number of procedures being performed allows statistically relevant review of the acute complications. Between January 1, 1984, and February 1, 1987, 417 such procedures were performed at The Children's Hospital, Boston; the age range of the patients was 1 day to 51 years (median 4 years, 6 months). Catheter procedures consisted predominantly of vascular dilations (peripheral pulmonic stenosis, 97; valvular pulmonic stenosis, 67; valvular aortic stenosis, 62; recurrent coarctation, 49) and embolizations (double umbrella device, 36; steel coils, 45). Overall, there were 50 acute complications (12%); 24 (6%) were major and 26 (6%) were minor. The mortality rate was 3 of 417 (0.7%). Complication rates varied between 4% for dilation of recurrent coarctation and 40% for dilation of aortic stenosis. The age of the patients was a factor in complications of vascular access (11 patients), 8 of which occurred in patients younger than 6 months (median 5), and in cardiac arrest and ventricular fibrillation (4 patients, 3 of whom were younger than 6 months [median 3]). No statistically significant trend toward diminishing overall complication rates was discerned over the 37 months of this study.

Atrioventricular (AV) discordance with ventriculoarterial (VA) concordance is a rare form of congenital heart disease that consists of 5 different anatomic types. The salient therapeutic consideration uniting these 5 different anatomic entities is that anatomic correction can be achieved by an atrial switch procedure with closure of any associated septal defect. Three patients who had AV discordance with VA concordance are presented, with emphasis upon 2-dimensional echocardiographic diagnosis and surgical management. One of these patients had ventricular inversion with inverted normally related great arteries in situs solitus (i.e., [S,L,I]), thus representing a form of congenital heart disease that until now has not been documented anatomically. Although these are complex defects, multiple-plane imaging with 2-dimensional echocardiography was found to be extremely reliable in ascertaining the anatomy when a comprehensive, segmental analysis is applied. This approach includes determination of visceroatrial situs, ventricular loop and great arterial position and alignment. A surgical approach to closure of the ventricular septal defect through the left-sided infundibulum in isolated ventricular inversion afforded excellent exposure of the defect and avoided AV conduction block.

Patients with left atrioventricular (AV) valve atresia or stenosis were studied retrospectively to determine the incidence of early and late failures of procedures to enlarge an interatrial communication. The 61 patients underwent 80 procedures: 5 balloon atrial septostomies, 12 blade atrial septostomies and 63 surgical septectomies. No balloon septostomy provided adequate long-term palliation. Of 12 blade septostomies, 4 resulted in gradients across the atrial septum of 5 to 8 mm Hg and 8 in gradients 3 mm Hg or less. Results from blade septostomy were unrelated to underlying diagnosis, age, gradient before the procedure, number of previous procedures, pulmonary blood flow or size of the postprocedure defect by balloon sizing, but were related to size of the postoperative defect estimated by echocardiography. Among 8 patients with gradients of 3 mm Hg or less after blade septostomy, 7 were followed 9 +/- 7 months and showed no evidence of restenosis. Of 63 surgical septectomies, 11 (17.5%) were inadequate, and in at least 7 cases the failure was due to restenosis of the defect as documented by serial catheterizations or echocardiograms. Outcome after surgical septectomy was unrelated to underlying diagnosis, age or number of previous procedures, but was related to size of the defect created. Our results reveal improved results in terms of residual gradient for blade septostomy compared with previous studies and the need to follow these patients carefully, even those undergoing surgical septectomy.

The charts of 79 patients who required femoral arterial (FA) thrombectomy after cardiac catheterization were reviewed. Fifteen patients (19%) had poor pulses after thrombectomy and 2 had an extremity amputated. One thousand consecutive patients undergoing cardiac catheterization were also studied to prospectively determine the safety and efficacy of systemic fibrinolytic therapy for treatment of FA thrombosis. Among these, 771 patients underwent retrograde arterial catheterization, including 31 patients with left-sided obstructive lesions who had undergone transarterial balloon dilation procedures with large catheters. All patients were given heparin at the time of arterial cannulation. Patients who had a pulseless extremity 4 hours after catheterization continued to receive heparin therapy for 24 to 48 hours. If the extremity continued to have no palpable pulse and the systolic blood pressure was less than 50% of that in the contralateral leg, intravenous streptokinase infusion was begun. The overall incidence of FA thrombosis was 3.6% (28 of 771), including 39% (12 of 31) of all patients undergoing transarterial balloon dilation procedures; 97% (27 of 28) of patients weighed less than 14 kg and the majority weighed less than 10 kg. After an average treatment period of 33 hours, 16 patients continued to have a pulseless extremity and were treated with streptokinase for an average duration of 13 hours. Normal pulses and systolic blood pressure returned in 14 (88%) and were nearly normal in 1 other patient (6%). The incidence of bleeding at the arterial puncture site was 25% and was highest in the patients who had a transarterial balloon dilation procedure. No serious complications occurred.

The ability of first pass radionuclide angiocardiography to detect and quantitate residual intracardiac shunts and systemic venous obstruction after repair of transposition of the great arteries was evaluated in 29 children. Information from radionuclide scans was compared with data obtained during cardiac catheterization. Three children had a residual right to left shunt detected with both methods. There was good agreement between radionuclide and catheterization quantitation of left to right shunt in the nine patients with a residual defect, five of whom had significant shunting. Nineteen patients with signs suggesting superior vena caval obstruction were evaluated with both radionuclide and catheterization methods. In eight, complete obstruction was detected with both techniques; in one additional patient, partial obstruction was found on catheterization only. One of six patients evaluated for possible inferior vena caval obstruction was identified with both techniques. In the group as a whole, information obtained with radionuclide angiography correlated well with cardiac catheterization data in evaluation of residual shunts and obstruction to systemic venous return.

Cineurography is often performed after cineangiocardiography to look for occult congenital urinary tract disease. The accuracy of cineurography was investigated in 171 patients by comparing cineurograms with renal sonograms. One hundred fifteen cineurograms (67%) showed both kidneys well enough to allow assessment of renal structure and function and the results were confirmed in 112 by ultrasonography; 3 cineurograms yielded false-positive results. Limited or no information was obtained from cineurograms of 56 patients (33%) because of nonvisualization or poor visualization of 1 or both kidneys. Of the 11 patients (6%) with urinary tract disease, only 3 were correctly assessed by cineurography. Ultrasonography discovered all 11 renal abnormalities and produced only 1 false-positive result. These data indicate that cineurography is a poor screening test and should be abandoned. When uroradiologic screening is necessary for high-risk patients, sonography is recommended.

To assess the natural history of ventricular tachycardia (VT) in children without heart disease, the clinical course of 26 children, aged 1 day to 15 years at initial detection, was reviewed. Symptoms related to the rhythm were present in 8 children at some time during their course of follow-up. Evaluation included echocardiogram (19 patients), Holter monitoring (22 patients), graded treadmill exercise testing (16 patients) and invasive electrophysiology (4 patients). Exercise induced or exacerbated VT in 9 patients, but suppressed rhythm in 7. Ten patients were never treated. Of the remaining 16 treated patients, therapy was discontinued in 10 on the basis of the electrocardiographic or Holter monitor recordings. Six continued to receive therapy without complications. There were no known deaths over a period of 1 month to 34 years (mean 59 months, median 42 months). Although 2 patients have been lost to follow-up, based on our findings and a review of the reported cases of VT in children to date, after undergoing complete noninvasive cardiac evaluation symptomatic patients should be treated and studied with invasive electrophysiology if antiarrhythmic control is inadequate. The rate of VT or age at onset are not predictive of outcome in asymptomatic patients. As a group, these patients do not appear to benefit from therapy, but warrant follow-up since deaths have been reported in untreated asymptomatic patients.

Eighty-three patients aged 11 months to 25 years were followed up a median of 6.1 years (range 8 days to 24 years) after diagnosis of fixed subaortic stenosis (SAS). Fourteen (17%) had significant noncardiac defects and 47 (57%) had additional cardiac malformations. The left ventricular (LV) outflow gradient increased in 25 of 26 patients catheterized more than once before surgery. Of 15 patients less than 12 years old with gradients less than or equal to 40 mm Hg, 10 ultimately underwent operation after developing severe obstruction; another has progressed to a gradient of 45 mm Hg at 6 years of age. Before surgery (at a median age of 12 years), 55% had aortic regurgitation (AR), which was usually mild. Infective endocarditis occurred in 12% of the group, with a frequency of 14.3 cases per 1,000 patient-years. Seventy-four patients were operated on, with 6 early (8%) and 7 late (9%) deaths. Twelve underwent reoperation to relieve residual obstruction. Surgery reduced gradients in patients with discrete SAS from 83 +/- 33 to 29 +/- 30 mm Hg, but in 6 patients with tunnel SAS the reduction was less satisfactory. AR was absent or mild in most patients postoperatively. When the gradient was reduced to less than 80 mm Hg, infective endocarditis did not occur unless there were other residual lesions. These data suggest that it is reasonable to resect discrete SAS in children less than 10 to 12 years old with LV outflow gradients greater than or equal to 30 mm Hg.

Radionuclide angiocardiography was used to assess pulmonary vascular reactivity in eight patients (nine studies) with a large, relatively unrestrictive intracardiac defect and pulmonary arterial hypertension. Radionuclide angiocardiograms, using technetium-99m pertechnetate, were performed first with the patient breathing room air and then after 10 minutes of breathing a mixture containing 90 percent or more of oxygen. The pulmonary to systemic flow ratios obtained by gamma variate analysis of the radionuclide time-activity curves were compared with those calculated with the Fick principle at the time of cardiac catheterization. There was a good correlation between the two methods both in room air studies (r = 0.88) and in those obtained with 90 percent or more of oxygen (r = 0.94). All six studies (in five patients) with a reactive pulmonary vasculature (judged by a pulmonary vascular resistance at cardiac catheterization of less than 6 units/m2 with oxygen or after tolazoline) had a radionuclide pulmonary to systemic flow ratio of 3.0 or greater with oxygen. The three patients with a nonreactive pulmonary vasculature had a radionuclide pulmonary to systemic flow ratio of 2.3 or less with oxygen, a value that was unchanged from the room air value. These data suggest that radionuclide angiocardiography may be a useful, relatively noninvasive method of assessing pulmonary vascular reactivity in patients with a large, relatively unrestrictive intracardiac defect.

The clinical course of 59 patients who underwent valvotomy for aortic stenosis before 1968 was reviewed. All were older than 1 year at the time of operation. Mean follow-up period was 17.7 years. Forty-six patients are alive; 26 (57%) are 30 to 40 years and 6 (13%) are older. Actuarial analysis indicated that the probability of survival was 94% at 5 years and 77% at 22 years. Thirteen patients died, 7 suddenly. Among the latter, significant obstruction or regurgitation was present in the 4 who underwent catheterization 0.9 to 7.2 years before death, 2 of whom were symptomatic and 2 with progression of a strain pattern on electrocardiogram. Surgery was recommended but declined by the latter 2 patients. Reoperation was carried out in 21 patients (36%), 3 (12%) of whom died. Actuarial analysis revealed the probability of reoperation to increase from 2% at 5 years to 44% at 22 years. Bacterial endocarditis occurred on 4 occasions in 3 patients, 1 of whom died suddenly during treatment. The incidence of endocarditis was 3.8 episodes/1,000 patient-years. Actuarial analysis of serious events, defined as death, reoperation and endocarditis, with the most serious of these and each patient being represented only once, indicated the probability of being free of such an episode to be 92% at 5 years, decreasing to 39% at 22 years. These data emphasize the palliative nature of valvotomy and the meticulous follow-up so necessary in these patients.

The present status of catheter-directed therapy in pediatric congenital and acquired heart disease is reviewed. Balloon pulmonary valvotomy, established as the procedure of choice for children and adults with isolated pulmonary stenosis, has now been extended to neonates with critical pulmonary stenosis. Balloon mitral and aortic valvotomy are being performed for congenital and acquired stenoses, although indications and optimal techniques remain to be established. Balloon angioplasty of coarctation effectively relieves obstruction in native and post-operative aortic arch stenoses, but remains controversial due to late aneurysm formation. Balloon angioplasty of branch pulmonary artery hypoplasia and stenosis, despite a relatively low success rate and significant complications, remains the procedure of choice due to lack of better therapeutic options. Transcatheter closure of congenital and acquired vessels and defects is being performed with increasing frequency. Embolization of aortopulmonary collaterals and shunts using Gianturco coils is safe and effective in selected patients. Although Rash-kind umbrellas are most commonly used for closure of a patent ductus arteriosus, their use, especially in closing intracardiac defects, remains investigational.

Balloon dilation angioplasty (BDA) was attempted 29 times in 27 patients, aged 3 months to 22 years, with postoperative aortic obstructions. Previous operations included end-to-end anastomosis (n = 10), subclavian flap angioplasty (n = 7) and patch angioplasty (n = 3) for aortic coarctation, end-to-end anastomosis for interrupted aortic arch type B (n = 4) and aortic arch reconstruction for hypoplastic left heart syndrome (n = 3). Two of the patients with interrupted arch had multiple areas of obstruction. Balloon sizes were between 2 and 6 times the diameter of the lesion and up to twice the diameter of the proximal transverse arch (mean 1.1). BDA was at least partially successful (more than 50% decrease in gradient and more than 30% increase in diameter) in 26 of the 29 procedures (90%). BDA failed in the 2 lesions with an initial diameter of more than 8 mm. No differences were apparent in the success rate among any of the clinical groups. Peak systolic gradient decreased from 42 +/- 14 to 14 +/- 15 mm Hg (p less than 0.01) and mean diameter increased from 4.1 +/- 2.6 to 6.8 +/- 3.2 mm (p less than 0.01). There was no mortality or significant acute morbidity associated with the procedure. After 1 to 24 months of follow-up, restenosis has occurred in only 1 patient. Aneurysm formation was found in 2 of 5 patients who had undergone repeat catheterization; both aneurysms occurred in patients with repaired interruption of the aortic arch.(ABSTRACT TRUNCATED AT 250 WORDS)