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Objective Evaluate long-term outcomes following balloon pulmonary valvuloplasty (BPV) for pulmonary stenosis (PS). Background Long-term data following BPV is limited to small, single center studies. Methods BPV from April 12, 1985 to January 7, 2015 from three centers were included. Outcomes studied were ≥ moderate PI by echocardiogram and residual PS ≥ 40 mm Hg. Risk factors for ≥ moderate PI, residual PS, and repeat intervention were assessed by univariate and multivariate analysis. Results Among 254 patients, mean age at BPV was 3.8 years (range 1 day-67 years), initial PS catheter gradient was 56 mm Hg (IQR 40-70), 19% had critical PS, and 9% had genetic syndromes. Mean follow-up duration was 7.5 years (maximum 25 years). Sixty-nine (29%) had ≥ moderate PI, 41 patients (17%) had residual PS > 40 mm Hg, and 31 (13%) had re-intervention. In univariate analysis, younger age, lower weight, greater initial PS gradient, greater initial RV/systemic pressure ratio, critical PS, and longer follow-up duration were associated with ≥ moderate PI. Greater initial PS gradient was associated with long-term residual PS or repeat intervention. In multivariate analysis, greater initial gradient and lower weight were independently associated with > moderate PI and greater initial PS gradient and genetic abnormality were independently associated with residual PS and repeat intervention. Conclusion Smaller patients with greater initial PS were more likely to develop significant long-term PI. Patients with greater initial PS and genetic abnormalities were more likely to have residual PS or require repeat intervention following BPV.

A multicenter prospective study was performed to assess the results and risks associated with radiofrequency ablation in children. This report focuses on recurrences following initially successful ablation.Patients recruited for the study were aged 0 to 16 years and had supraventricular tachycardia due to accessory pathways or atrioventricular nodal reentrant tachycardia (AVNRT), excluding patients with more than trivial congenital heart disease. A total of 481 patients were recruited into the prospective cohort and were followed at 2, 6, and 12 months following ablation.There were 517 successfully ablated substrates out of 540 attempted (95.7%). Loss to follow-up for individual substrates was 3.3%, 10.6%, and 21.2% at 2, 6, and 12 months, respectively. Recurrence was observed in 7.0%, 9.2%, and 10.7% of these substrates at 2, 6, and 12 months, respectively (adjusted for loss to follow-up as an independent source of data censoring). Recurrence rate varied by substrate location (24.6% for right septal, 15.8% for right free wall, 9.3% for left free wall, and 4.8% for left septal), as well as for AVNRT versus all others (4.8% vs 12.9%) at 12 months. The recurrence rate was higher for substrates ablated using power control but was not a function of whether isoproterenol was used for postablation testing.Recurrence after initially successful ablation occurs commonly in children. It is least common after AVNRT ablation and most common following ablation of right-sided pathways. These results serve as a benchmark for the time course of recurrence following initially successful ablation of supraventricular tachycardia in children.

A multicenter prospective study was designed and implemented as an activity of the Pediatric Electrophysiology Society to assess the risks associated with radiofrequency ablation in children. Patients (age 0-15 years) with supraventricular tachycardia due to accessory pathways or atrioventricular nodal reentry were enrolled and studied prior to ablation and periodically by clinical evaluation, electrocardiogram (ECG), Holter monitor, and echocardiogram. In addition, a national registry was established, to which the contributing centers report all pediatric patients undergoing ablation at their center. Initial electrophysiological study tracings and all noninvasive studies undergo blinded outside review for quality control. Clinical endpoints were death, recurrence, proarrhythmia, and echocardiographic abnormality. A pilot study demonstrated excellent agreement concerning diagnoses of previously reported ablation patients between the reporting center and the blinded reviewer (kappa = 0.938 +/- 0.062). A total of 317 patients were enrolled in the ongoing study from April 1, 1999 to December 31, 2000. The success rate of ablations was 96% with a complication rate of 4.3% for electrophysiological study and 2.9% for the ablation procedure. Comparison of the registry group versus the study group shows that the groups are comparable in terms of patient characteristics, diagnoses, and the results of ablation making it less likely that the sample of prospectively enrolled patients is biased.

Background Total cavopulmonary connection (TCPC) to repair functional single ventricle involves the sinus node area, in contrast to the Fontan procedure. We compared ECG findings after TCPC and Fontan to evaluate the impact of the cavopulmonary connection on sinus rhythm postoperatively. Methods and Results The Fontan group consisted of 17 patients repaired at 7.8±3.1 years of age (mean±SD): 11 for tricuspid or pulmonary atresia (TA/PA) and 6 for single ventricle. The TCPC group consisted of 19 patients repaired at 5.1±3.2 years of age (mean±SD) ( P P P 2 test). By 2 years postoperatively, only 6 of 15 TCPC patients available for follow-up (40%) were in SR, with 7 of 15 in JR and 2 of 15 in NSAR. By contrast, 13 of 17 Fontan patients (76%) remained in SR, with 1 in NSAR and 3 in JR ( P 2 test). TCPC patients with loss of SR did not differ from other patients in the group in age at repair, preoperative diagnosis, or surgeon performing the procedure. Conclusions This significant incidence of loss of SR temporally related to surgery suggests that operative compromise of the sinus node area is common with TCPC.

The development of pulmonary arteriovenous malformations after cavopulmonary bypass in patients with congenital heart disease is well documented. We report successful management of pulmonary arteriovenous malformations after cavopulmonary bypass in a patient with an interrupted inferior vena cava (IVC) and multiple hepatic veins utilizing an extracardiac conduit from the hepatic veins to the hemiazygous continuation of the interrupted IVC. This technique, performed without circulatory arrest or an atriotomy, may limit morbidity associated with intracardiac procedures in patients with single ventricle morphology. Furthermore, this case suggests an alternative technique for completion Fontan in patients with an interrupted IVC and multiple hepatic venous drainage.

Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) can result in left-ventricular distension and the development of pulmonary edema. We present the case of a 13-year-old girl who presented with cardiogenic shock. VA-ECMO was initiated, but after 6 days, severe left-ventricular distension resulted in decreased VA-ECMO flows. With guidance by bedside transesophageal echocardiography, a percutaneous atrial transseptal cannula was placed and connected to the venous circuit, thus decompressing the left ventricle. The patient improved, was weaned from VA-ECMO 5 days later, and was discharged from the hospital. Bedside transseptal catheter insertion is an effective method of left-ventricular decompression.

Forty-eight cases of infective endocarditis (IE) that occurred in 42 patients with congenital heart disease were reviewed from 1970 through 1990 and were compared with a 20-year review of 108 cases diagnosed between 1953 and 1972. The review demonstrates that the natural history of IE in children has changed over the last 2 decades, with half of the cases occurring after surgery for congenital heart disease. In the postoperative group, 46% of patients had undergone. valve replacement and 7 of these (29%) had a right ventricular to pulmonary artery valved conduit as the site for IE, suggesting significant additional risk in this setting. Among patients with nonsurgically treated congenital heart disease and IE, mitral valve prolapse has emerged as an important underlying heart lesion occurring in 29% of patients. The bacterial spectrum has shifted, with a significant increase in the incidence of uncommon causative organisms. Mortality has continued to decline with survivorship. of 90% in this series.

A 4-year-old child with hypoplastic left heart syndrome and a 6-year-old child with tricuspid atresia had both undergone staged reconstructive operations culminating in a Fontan operation. Peripheral edema, ascites, and hypoalbuminemia refractory to dietary manipulation and steroid therapy developed in both patients. After hemodynamic assessment, each child underwent surgical creation of a 4.8-mm fenestration in the previously placed baffle that separated the systemic venous pathway from the pulmonary venous atrium. Peripheral edema and ascites promptly resolved and serum protein levels normalized within 2 weeks after operation. Systemic arterial saturation is 86% in each child, and both children remain clinically well with no evidence of protein-losing enteropathy on normal diets and without specific medical therapy.

Hydrops fetalis is rarely associated with congestive heart failure caused by obstructive left-sided heart lesions. There are rare cases of live born neonates with critical congenital valvar aortic stenosis and hydrops reported in the literature, all with fatal outcomes. This report describes, to the best of our knowledge, the first two newborns who were diagnosed prenatally to have hydrops fetalis caused by critical valvar aortic stenosis, who were treated prenatally with digoxin and who postnatally had successful percutaneous balloon aortic valvuloplasty. Both patients had not only left but right ventricular dysfunction. We speculate that right ventricular dysfunction was a contributing factor in the development of hydrops in these patients and in utero medical therapy with digoxin is associated with resolution of the hydrops before delivery.

Objectives. We report our experience in eight consecutive neonates who underwent attempted balloon dilation as an initial therapy far critical valvular pulmonary stenosis, and we review in detail technical modifications that improved the success rate.Background. Balloon dilation of the pulmonary valve has become the treatment of choice for valvular pulmonary stenosis in children and adults. There are few reports of its effectiveness in critical pulmonary stenosis in the newborn. In this setting, application of the tecnique of baloon dilation has been limited by the ability to advance the necessary guide wires and catheters across the stenotic, often near-atretic, pulmonary valve.Methods. The pulmonary valve was crossed in all patients. When this could not be accomplished with an end-hole catheter, a soft guide wire was advanced directly across the pulmonary valve through the end-hole catheter positioned in the right ventricular outflow tract below the valve. Initial prediction was achieved in all patients by using a coronary dilation catheter in an effort to facilitate introduction of the definitive balloon dilation catheter. Definitive dilation with a balloon diameter of ≥110% of the diameter of the pulmonary valve annulus was possible in six patients.Results. Right ventricular pressure declined from a mean value of 108 ± 32 mm Hg to a mean value of 49 ± 11 mm Hg after balloon dilation, with no change in heart rate or aortic pressure in these six patients after definitive balloon dilation.Conclusions. The results of this small series suggest that critical valvular pulmonary stenosis in the newborn can be successfully treated by transluminal balloon valvuloplasty.

Of 24 patients, aged 6 days to 24 months, undergoing the Senning procedure for transposition of the great arteries, 2 patients died perioperatively (8% operative mortality): 1 patient, a neonate, from sepsis and 1 patient, born prematurely and with multiple anomalies, from congestive heart failure. One patient died late postoperatively from noncardiac causes. The 21 survivors are clinically well, and in 20 complete hemodynamic and electrophysiologic data were obtained by cardiac catheterization a mean of 13 months after repair. All patients have normal systemic arterial oxygen saturation. Left ventricular function and pulmonary artery pressures are normal in all. None had pulmonary venous obstruction. Narrowing at the junction of the superior vena cava and systemic venous atrium with mean pressure differences of 5 mm Hg or more was found in 4 of the 20 patients, but was clinically manifest in only 1 patient. No abnormality of atrioventricular conduction was seen in response to programmed electrical stimulation. Sinus node dysfunction was present in 6 patients, with abnormalities of both automaticity and sinoatrial conduction. Among these 6 patients were the 5 who were younger than 5 months at operation. The Senning procedure generally results in excellent hemodynamic and electrophysiologic status in patients who undergo operation after the newborn period. Identification of sinus node dysfunction, seen in patients in whom the procedure is performed in the first few months of life, is of concern and identifies a need for close follow-up of sinus node function in this cohort of patients.

Long-standing pulmonary insufficiency after repair of tetralogy of Fallot may adversely affect ventricular function. We evaluated 20 patients at a mean of 9 years after repair by radionuclide ventriculography, 24 hour Holter monitoring, and M-mode echocardiography. The mean age at complete repair was 7.1 ± 2.6 years. Patients were divided into groups as follows: Group I (eight patients), no clinical pulmonary insufficiency; Group II (12 patients), moderate to severe pulmonary insufficiency. Group II was further divided: Group IIa, transannular patch (six patients): Group IIb, no transannular patch (six patients). There was no difference between groups for age at operation, duration of follow-up, right ventricular pressure, or right ventricular-pulmonary arterial gradient. No patient had a residual shunt and all were in New York Heart Association Class I. Serious ventricular dysrhythmias occurred in 38% of Group I patients and 50% of Group II (p = NS). The echocardiographic ratio of right to left ventricular end-diastolic dimension was greater in patients with pulmonary insufficiency than in those without pulmonary insufficiency: 0.83 ± 0.17 versus 0.55 ± 0.15, p

From May, 1982, to September, 1983, 9 patients underwent repair of complete AV septal defect. They ranged in age from 11 months to 48 months and in weight from 5.3 kg to 16.5 kg. Seven patients were 24 months old or less. Previous operations included pulmonary artery banding in 1 patient and ligation of a patent ductus arteriosus with repair of coarctation in another. All patients had large left-to-right shunts (mean pulmonary to systemic flow ratio, 3.1), and the 7 young infants had marked pulmonary hypertension. Mitral regurgitation was absent in 2 patients, mild in 3, moderate in 2, and severe in 2. One patient had the right ventricular dominant form of complete AV septal defect. In all instances, repair was done using separate ventricular and atrial patches. Leaflet tissue was not divided, and a trileaflet mitral valve was left in each patient. Eight patients survived operation and are well 3 to 17 months after repair. The single operative death occurred in the patient with right ventricular dominance. Only 1 patient has mild residual heart failure 4 months after operation. Clinically, mitral regurgitation is absent in 4 patients and, at most, mild in the other 4. No patient has a conduction disturbance. Repair of complete AV septal defect is facilitated by using separate patches for the ventricular and atrial components of the defect. Less distortion is created, and a more accurate reconstruction of a competent trileaflet mitral valve can be done.

Left ventricular (LV) apical aneurysms were observed in 16 of 50 (32%) children (average age 8 years) consecutively catheterized after surgical repair of congenital heart disease. The LV apex was vented by a sump during cardiopulmonary bypass in each. The aneurysms varied in size, but were generally small. Average dimensions were 7.5 X 6.8 mm in the anteroposterior projection and 8.9 X 5.7 mm in the left anterior oblique projection. The LV apex wall was thinner in patients with aneurysms than in age- and lesion-matched controls. All of the LV aneurysm patients were asymptomatic during average follow-up of 4 years. Nevertheless, such aneurysms are anticipated to represent a potential source of cardiovascular complications and, when possible, alternate methods for venting the left ventricle are recommended.

Residual severe pulmonary insufficiency or stenosis may result in significant myocardial dysfunction late after repair of tetralogy of Fallot. Although pulmonary valve replacement has been advocated for selected patients, objective improvement in right ventricular function has been difficult to demonstrate. We undertook pulmonary valve replacement in 11 patients to treat residual insufficiency (n = 8) or stenosis (n = 3) and evaluated them before and after operation by radionuclide ventriculography and M-mode echocardiography. Patients' age at the original repair was 6.6 +/- 0.6 years (range 2 to 8 years) and at subsequent valve replacement was 14.6 +/- 1.5 years (range 5 to 20 years). Indications for pulmonary valve replacement were conduit stenosis indicated by a gradient greater than or equal to 75 mm Hg (n = 3), symptoms (n = 2), progressive cardiomegaly (n = 3), and new onset of tricuspid insufficiency (n = 3). Prior to pulmonary valve replacement, right ventricular ejection fraction was 0.29 +/- 0.12 (range 0.12 to 0.48) and rose to 0.35 +/- 0.10 (range 0.19 to 0.48) at a mean of 10.5 +/- 2.3 months after operation (p less than 0.05). Improvement (defined as an increase in ejection fraction greater than 0.05) was noted in seven patients whereas four demonstrated no change. Left ventricular ejection fraction before operation (0.55 +/- 0.12) was unchanged after pulmonary valve replacement (0.54 +/- 0.06). M-mode echocardiography demonstrated significant reduction in right ventricular dilatation. Right ventricular/left ventricular end-diastolic dimension fell from 1.03 +/- 0.30 to 0.73 +/- 0.13 after operation (p less than 0.01). Cardiothoracic ratio fell from 0.59 +/- 0.02 to 0.55 +/- 0.02 at a mean of 12 months after pulmonary valve replacement (p less than 0.01). Subjective improvement in exercise tolerance was noted in all seven patients who showed an increase in right ventricular ejection fraction. Of the remaining four patients, two had no improvement, one felt symptomatically improved, and one was too young for evaluation. These data demonstrate objective improvement in right ventricular function following pulmonary valve replacement and confirm the usefulness of this procedure in patients with significant right ventricular dysfunction secondary to residual pulmonary insufficiency and stenosis.

Since February 1982, we have used a modified technique to repair tetralogy of Fallot with transatrial closure of the ventricular septal defect, a short infundibular incision with avoidance of muscle resection, and anterior expansion of the right ventricular outflow tract using a patch in every case. The pulmonary anulus is approached in the usual manner. Twenty-six patients have had repair with this technique without mortality, and 20 patients (Group 1) have been evaluated 1 to 3 years postoperatively. Their results are compared with those of all patients followed up in our division who had had repair by the traditional technique in the 5 years before February 1982, with the same evaluation within 4 years of operation (Group 2, n = 22). Ten of 20 patients in Group 1 and 14 of 22 in Group 2 required a transannular patch. At cardiac catheterization, right ventricular outflow tract obstruction was effectively relieved in both groups (right ventricular systolic pressure [mean +/- standard error], Group 1 versus Group 2, 45 +/- 5 versus 49 +/- 4 mm Hg). No patient with the modified technique had a residual ventricular septal defect. By M-mode echocardiography, right ventricular-left ventricular end-diastolic dimension ratio was significantly lower for Group 1 patients (0.58 +/- 0.03 versus 0.78 +/- 0.04; normal less than 0.45; p less than 0.001). With radionuclide ventriculography, right ventricular ejection fractions were significantly higher for Group 1 patients with the modified repair (38% +/- 2.2% versus 33% +/- 1.8%; normal greater than or equal to 45%; p less than 0.05). Because the incidence of obligatory pulmonary insufficiency caused by transannular patch is similar in both groups, these results cannot be directly attributed to the presence of pulmonary insufficiency alone. With ambulatory electrocardiographic monitoring, no patient in Group 1 had any ventricular premature contractions whatsoever, whereas six of 22 patients in Group 2 had demonstrable ventricular premature contractions. These results suggest that in children with tetralogy of Fallot, right ventricular outflow tract obstruction can be effectively relieved with this modified approach with improved early preservation of right ventricular function.

To investigate the nature of the dominant intrinsic cardiac pacemaker activity after the Senning procedure, endocardial mapping of the systemic venous atrium was accomplished a mean of 13 months after operation in 10 patients, aged 22 ± 6 months. Multiple endocardial sites were measured to find the earliest atrial electrical activity timed back from the QRS complex. These data were compared with data from endocardial mapping performed in 6 patients late after the Mustard procedure. In 8 of 10 patients who had undergone the Senning procedure, the earliest activation time, corresponding by definition to the origin of the intrinsic pacemaker, was located in the inferior medial portion of the superior limb of the systemic venous atrium. In the other 2 patients who had undergone the Senning procedure, the earliest activity was in the high superior limb of the baffle at its junction with the superior vena cava. In contrast, the earliest activity in all patients who underwent the Mustard operation was at the junction of the superior vena cava and the superior limb of the systemic venous atrium. In response to programmed extrastimulation, the electrophysiologic behavior of the intrinsic pacemaker in the Senning group was abnormal compared with known normal sinus node (SN) data in only 3 of 10 patients, whereas all patients in the Mustard group had SN dysfunction. Abnormal SN function was noted in both patients in the Senning group, in whom a shift in the position of the earliest endocardial activation point was not seen. The anatomic shift in the position of the earliest pacemaker activity observed after the Senning procedure, but not after the Mustard operation, results from a physical shift in the position of the SN inherent to the atrial free-wall infolding aspect of the Senning operation.

Systemic-pulmonary artery shunts remain an important treatment in cyanotic patients. Central shunts continue to pose early and late problems when standard Blalock-Taussig shunts are not possible. Twenty patients underwent subclavian-pulmonary artery shunt procedures with polytetrafluoroethylene (PTFE) prostheses between October, 1980, and August, 1982. Their ages ranged from 1 day to 15 years; 11 patients were less than 14 days old. The arterial oxygen tension rose from 30.7 +/- 11.9 mm Hg to 51.3 +/- 9.1 mm Hg (standard deviation; p less than 0.001) and from 26.4 +/- 7.5 mm Hg to 50.5 +/- 9.3 mm Hg (p less than 0.001) among the 11 neonates. There were no hospital deaths and only 2 late deaths (not shunt related). All patients have patent shunts and excellent relief of cyanosis. The 18 survivors have been followed for an average of 19 months (range, 7 to 29 months). No patient has required reoperation for shunt inadequacy or thrombosis. Recatheterization in 11 patients has demonstrated normal pulmonary pressures and good pulmonary artery growth without vessel distortion. Subclavian-pulmonary shunts using PTFE provide long-term palliation in cyanotic patients. This type of shunt appears to offer important advantages over other shunt procedures, including the classic Blalock-Taussig operation, in newborns.

A 6 1/2-month-old infant was seen with right-arm monoparesis, right upper extremity hypertension, and findings of a coarctation located in the midaortic arch between the innominate and left common carotid arteries. Associated left cerebral atrophy (Dyke-Davidoff syndrome) was documented by computed tomography. Excision of the coarctation segment with primary repair of the arch and reanastomosis of the left carotid to the innominate artery was accomplished.

Subclavian-to-pulmonary artery anastomoses with interposition polytetrafluoroethylene (PTFE) conduits provide excellent early palliation for many forms of cyanotic heart disease. It is important to assess whether patients with this condition maintain adequate arterial oxygenation without developing pulmonary artery distortion or hypertension. From October, 1980, to December, 1982, 29 PTFE shunts were performed. There were no hospital deaths or shunt failures. Catheterization was performed in 14 patients from 2 months to 2.5 years (mean 13.4 months) following operation. All shunts were patent. Arterial Po2 at the late study ranged from 33 to 96 torr (mean 57.1 +/- 17.3 torr) and was not significantly different from values obtained before hospital discharge at the time of the shunt procedure (mean 47.6 +/- 5.9 torr). Only one patient demonstrated moderate pulmonary hypertension (44/25 mm Hg), believed to be secondary to ventricular dysfunction. No patient demonstrated pulmonary artery distortion or kinking, and none required a second shunt because of inadequacy of the original procedure. The results in these patients, as well as the continued excellent clinical course of the patients not as yet recatheterized, have prompted us to use this shunt as our procedure of choice in neonates.

• We examined 11 children with infective endocarditis initially and serially by two-dimensional echocardiography. Nine (82%) of the 11 patients had echocardiographic findings at initial examination compatible with infective endocarditis. These results provided strong evidence in support of the diagnosis before bacteriologic confirmation was available. Congestive heart failure, major emboli, and/or the need for surgical intervention occurred in seven of the nine patients with positive two-dimensional echocardiograms. Echocardiographic evidence of vegetations persisted during antibiotic therapy and resolved slowly during many months. Serial echocardiograms were useful in cases in which obvious valve destruction or marked increase in vegetation size imaged echocardiographically could be combined with clinical evidence of progressive heart failure to support a decision for early surgical intervention. Two-dimensional echocardiography can make important contributions to the diagnosis and management of children with infective endocarditis. ( Am J Dis Child 1983;137:851-856)