Inducibility of intra-atrial reentrant tachycardia after the first two stages of the Fontan sequence.

OBJECTIVES: We sought to examine the incidence and possible factors for inducible intra-atrial reentrant tachycardia (IART) in a group of patients after two stages of the Fontan sequence but before the operation. BACKGROUND: Intra-atrial reentrant tachycardia occurs in 10% to 40% of patients after the Fontan operation. No data are available regarding the potential for IART after the first two stages of the Fontan sequence but before the operation. METHODS: The IART induction protocol included programmed extrastimulation and rapid atrial pacing, with and without isoproterenol. RESULTS: The median age of the study group (n = 44, 27 males) was 1.7 years (range 1.2 to 5.2). Forty patients were in sinus rhythm. Twelve patients (27%) had inducible, sustained (>1 min) IART. Three patients (8%) had inducible, nonsustained IART. Bivariate analysis revealed that patients with sustained IART were significantly older at their second operation (median 0.54 vs. 0.40 years, p = 0.05). Multivariate logistic modeling revealed that older age (> or =0.55 years) at the second palliative operation (p = 0.04), older age (> or =1.95 years) at evaluation before the Fontan sequence (p = 0.04) and female gender (p = 0.03) were independently associated with sustained IART. A trend toward a greater frequency of sustained IART was seen in those patients with moderate or severe atrioventricular valve regurgitation (p = 0.07) and in those with resection of the atrial septum (p = 0.06). CONCLUSIONS: The rate of inducible, sustained IART in a group of patients before the Fontan operation is 27% and is associated with older age at the time of second-stage palliation, older age at pre-Fontan evaluation and female gender.

Transcatheter closure of fontan fenestrations using the Amplatzer septal occluder: initial experience and follow-up.

We have recently used the Amplatzer septal occluder to close Fontan fenestrations. Between June 1998 and December 1999, 13 patients underwent transcatheter occlusion of their Fontan fenestrations. Systemic blood flow decreased significantly without a concomitant decrease in pulmonary blood flow. All residual shunts detectable by oximetry were at sites separate from those into which occlusion devices were implanted. One patient developed severe tricuspid regurgitation following the procedure requiring surgical removal of the device. At the last follow-up, all patients were doing well clinically. There were no shunts detectable through or around the devices by echocardiography. Our experience indicates that the location of the fenestration within the Fontan baffle is critical to avoiding device interference with other intracardiac structures. The Amplatzer septal occluder offers an effective means of transcatheter closure of Fontan baffle fenestrations. Although more experience is needed, our current follow-up data suggest that long-term outcomes will be favorable. Cathet. Cardiovasc. Intervent. 51:301-304, 2000.

Congenital Heart Surgery Nomenclature and Database Project: therapeutic cardiac catheter interventions.

The extant nomenclature for therapeutic cardiac catheter interventions is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Left ventricular growth in selected hypoplastic left ventricles: outcome after repair of coarctation of aorta.

BACKGROUND: Models that predict survival in neonates with left ventricular hypoplasia and critical aortic stenosis may not be applicable to neonates with left ventricular hypoplasia and coarctation. METHODS AND RESULTS: We report 8 infants with severe aortic coarctation and left ventricular hypoplasia. Mean age was 18 days (range 1-48 days), and mean weight was 3.5 kg (range 2.7-4.3 kg). Associated diagnoses included mild aortic stenosis (4), ventricular septal defect (2), and venous anomalies (2). All had coarctation repair as a primary procedure (3 of these had concomitant intracardiac procedures); 7 had subsequent operations. All are alive and well 1.1-6.7 years (mean 3.1 years) after the first surgery. Progressive increases were observed in aortic and mitral diameters, and in left ventricular dimensions, areas, and volumes when the preoperative, earliest postoperative, and most recent echocardiograms were compared. CONCLUSIONS: Despite severe left ventricular hypoplasia, a two-ventricle repair is possible in selected cases. The prognostic criteria for left ventricular hypoplasia in critical aortic stenosis may not be applicable to infant coarctation. Relief of coarctation may result in the growth of the very small left ventricle, especially when the aortic root and mitral diameters are satisfactory.

Perventricular [correction of Periventricular] closure of ventricular septal defects without cardiopulmonary bypass.

BACKGROUND: Minimally invasive techniques are currently in use to close atrial and ventricular septal defects (VSD). Cardiopulmonary bypass (CPB) is instituted via the femoral vessels, which may cause injury to these vessels, especially in younger patients. The objectives of this study were to demonstrate the feasibility of perventricular [corrected] closure of muscular VSD (MVSD) and paramembranous VSD (PVSD) without CPB, using the Amplatz VSD device. METHODS: Five Yucatan pigs with naturally occurring PVSD (3- to 7-mm diameter) and 5 dogs with surgically created MVSD (6- to 14-mm diameter) were subjects of this study. The VSDs were closed intraoperatively with a 7-French delivery sheath inserted through the free wall of the right (n = 5) or left ventricle (n = 5), under epicardial echocardiogram guidance. The animals were followed for 3 months. RESULTS: There was no operative mortality. All MVSD closed after placement of the device. Closure rate of PVSD was 4 of 5 after placement and 3 of 5 after 3 months. One pig developed aortic incompetence at the last follow-up. CONCLUSIONS: Perventricular closure of MVSD and PVSD is feasible. Avoidance of CPB can decrease recovery time, its complications, and trauma to the femoral vessels.

Impact of arrhythmia circuit cryoablation during Fontan conversion for refractory atrial tachycardia.

Refractory atrial arrhythmias in late postoperative Fontan patients are usually associated with residual hemodynamic abnormalities and result in significant morbidity and mortality. Surgical revision of the Fontan anastomosis may improve hemodynamics without eliminating tachycardia. This study sought to assess the impact of surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion on the clinical recurrence of tachycardia. Sixteen consecutive atriopulmonary Fontan patients with refractory atrial arrhythmias underwent surgical conversion to lateral tunnel total cavopulmonary anastomosis (15) or Fontan revision (1 patient). The initial 4 patients underwent Fontan conversions alone, without specific arrhythmia surgery. The subsequent 12 patients underwent electrophysiologically guided cryoablation of the tachycardia circuits at the time of surgical conversion. The mean age at Fontan revision was 15.6 +/- 3.8 years. Cryoablation was directed to 3 identified major tachycardia circuits: the inferomedial right atrium, the superior rim of the prior atrial septal defect patch, and along the lateral right atrial wall. Transmural antitachycardia pacemakers were implanted in 11 of the 16 patients. There was no surgical mortality in either group, and all patients improved in functional classification. All patients not undergoing cryoablation experienced recurrent symptomatic tachycardia requiring antiarrhythmic therapy (median follow-up, 54 months) versus 2 of 12 patients receiving cryoablation (median follow-up, 25 months; p <0.02). Thus, surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion is highly effective in the management of refractory atrial arrhythmias, and is superior to Fontan conversion alone.

Raising heart-healthy children.

The current article will describe how the cardiovascular risk factors of obesity, increased blood pressure, hyperlipidemia, cigarette smoking, poor physical fitness and prenatal environment impact the development of cardiovascular disease and what types of therapy can be used in the pediatric patient to modify them.

Repair of mitral valve and subaortic mycotic aneurysm in a child with endocarditis.

Endocarditis requiring surgical intervention in children is uncommon. Individualized operative therapy must be fashioned to the particular pathology of each case. In this case we describe mitral anterior leaflet homograft patch augmentation valvuloplasty, subaortic homograft patch closure of a large mycotic aneurysm, and homograft aortic root replacement in a 3-year-old patient with endocarditis after remote repair of complete atrioventricular canal.

Surgical repair of the congenitally malformed mitral valve in infants and children.

BACKGROUND: Mitral valve remodeling techniques were applied to 26 infants and children (mean age, 6.0 years, range, 0.4 to 15.9 years) with various forms of congenital mitral valve disease over a 7-year period. Patients with atrioventricular canal, L-transposition and single ventricle were excluded. Intraoperative transesophageal echocardiography (TEE) was utilized to assess the repair and guide the need for immediate intervention. METHODS: Twenty-one patients had mitral regurgitation: 10 with cleft anterior mitral leaflet, 7 with annular dilatation, 1 with normal leaflets with an obstructing cord, 2 with prolapsed leaflets and elongated cords, and 1 with restricted leaflet motion, normal papillary muscles, and shortened cords. Of the 5 mitral stenosis patients, 3 had supravalvular mitral ring, 1 had midvalvular mitral ring, and 1 had a parachute valve. Three of the mitral stenosis patients had additional stenotic lesions. Concurrent repair of associated lesions was performed in 21 patients (78%). RESULTS: Operative mortality was 3.8% (n = 1). There were no late deaths. Immediate rerepair in 4 patients resulted in improved function. All mitral stenosis patients improved. A total of 20 mitral regurgitation patients (95%) improved; 1 required mitral valve replacement. Mean follow-up is 31 months (range, 2 to 81 months). All patients are in New York Heart Association functional class I or II. CONCLUSIONS: Mitral valve repair can be successfully performed in infants and children with excellent short- and midterm results. Assessment using transesophageal echocardiography can guide the necessity for immediate rerepair to achieve improved function.

Coronary artery fistulas in infants and children: a surgical review and discussion of coil embolization.

BACKGROUND: Coronary artery fistula (CAF) is a rare congenital anomaly that can be complicated by intracardiac shunts, endocarditis, myocardial infarction, or coronary aneurysms. Recent reports have emphasized the efficacy of percutaneous transcatheter techniques. The purpose of this article is to review a 28-year surgical experience with CAF as a standard for comparison and to discuss the emergence and efficacy of transcutaneous catheter coil embolization as an alternative form of therapy. METHODS: From 1968 to 1996, 17 patients (age, 6 weeks to 16.5 years; mean age, 5.5 years) were diagnosed with CAF: 8 of 12 by echocardiography and 17 of 17 by cardiac catheterization. All patients with isolated CAF (n = 13) were asymptomatic despite significant clinical, electrocardiographic, and chest roentgenographic findings in 10. Sixteen had congenital CAF and 1 had acquired CAF after tetralogy of Fallot repair with injury of the anomalous left anterior descending coronary artery. Associated anomalies included tetralogy of Fallot (2), atrial septal defect (1), and patent ductus arteriosus (1). Nine fistulas originated from the right coronary artery and eight from the left. Drainage was to the right ventricle (9), right atrium (4), pulmonary artery (3), and left atrium (1). RESULTS: All patients had a median sternotomy with epicardial or endocardial ligation. Cardiopulmonary bypass was used in 8; 1 of these (iatrogenic CAF) required distal internal mammary artery bypass graft. There were no operative or late deaths. Follow-up evaluation by physical examination (17), echocardiography (8), and catheterization (2) showed no evidence of recurrent or residual CAF. A retrospective review of the 16 available cine cardioangiograms showed that coil embolization was possible in, at most, 6 patients. CONCLUSIONS: Early surgical management of CAF is a safe and effective treatment resulting in 100% survival and 100% closure rate. Transcatheter embolization is a reasonable alternative to standard surgical closure in only a very small, select group of patients. These surgical results should be considered the standard against which transcatheter techniques are compared.

Taussig-Bing anomaly: arterial switch versus Kawashima intraventricular repair.

BACKGROUND: Current corrective surgical approaches for the Taussig-Bing heart include arterial switch with ventricular septal defect (VSD) closure and intraventricular repair as described by Kawashima. METHODS: Between 1983 and 1994, 20 children underwent intracardiac repair of Taussig-Bing anomaly. Mean age at operation was 17 months (range, 1 week to 9 years). Prior palliation included pulmonary artery band (15) with coarctation repair (8) and atrial septectomy (1). Arterial switch with VSD closure was performed in 16 patients, 10 with anteroposterior great arteries. Kawashima repair was performed in 4 patients, all with side-by-side great arteries. RESULTS: After arterial switch, there was one operative death (6.2%) due to myocardial ischemia and three late deaths (18.7%) due to pulmonary hypertension, gastrointestinal bleeding, and acute lymphocytic leukemia. In the Kawashima repair group there have been no deaths. After arterial switch, 9 patients underwent 11 reoperations for residual coarctation (3), residual pulmonary artery stenosis (2), aortic valve replacement, aortic valvuloplasty, unrecognized VSD, mitral valvuloplasty, mediastinitis, and pacemaker insertion. After Kawashima repair, 1 patient underwent reoperation for baffle stenosis and 1 for an unrecognized VSD. CONCLUSIONS: For children with Taussig-Bing anomaly, the Kawashima intraventricular repair (for side-by-side great arteries) preserves the native aortic valve and avoids coronary dissection. The arterial switch operation with VSD closure can be applied without ventriculotomy to all great artery relationships and allows neonatal repair with or without concomitant coarctation repair. Both techniques yield excellent early and intermediate-term results despite the high rates of prerepair palliation and postrepair reoperation for both groups.

Fetal and pediatric origins of adult cardiovascular disease.

Adult-onset cardiovascular disease has its origin in childhood. During the past year there have been a number of articles that have dealt with the effects of fetal nutrition on the development of cardiovascular disease in adult life, identification of the child at high risk for the development of essential hypertension, the effects of passive smoking as a risk factor for coronary artery disease, and strategies for diagnosis and treatment for familial hypercholesterolemia. Two of the most important observations over the past year are that there appears to be a significant relationship between fetal and early childhood nutrition and the risk of developing many of the cardiovascular diseases seen in adult individuals and that environmental tobacco smoking, including smoke at work, results in an increased risk of developing coronary heart disease.

Comparison of risks and short- and long-term results of balloon dilatation versus surgical treatment for pulmonary and aortic valve stenosis and restenosis and coarctation and recoarctation of the aorta.

Balloon valvuloplasty and angioplasty have become accepted alternatives to surgery for valvar stenosis and coarctation of the aorta. Balloon dilation avoids a potentially painful operation, the long postoperative recovery, and at the same time offers substantial cost savings. However, such advantages are meaningless if the safety of the interventional procedure does not match or surpass the results of conventional surgery. This review summarizes a number of studies that compare the natural history of surgical therapy with that of balloon pulmonary and aortic valvuloplasty and balloon coarctation angioplasty. It appears that, regardless of age, balloon valvuloplasty is preferable to surgical valvotomy. For both aortic stenosis and coarctation of the aorta, balloon valvuloplasty and surgical valvotomy produce comparable relief of the hemodynamic obstruction. However, because the length of follow-up after the balloon angioplasties has been short, the actual risk of developing severe aortic regurgitation (postaortic valvuloplasty) and aortic aneurysms (postcoarctation angioplasty) has not been trivial, a large prospective follow-up of both of these interventional procedures will be mandatory before either can be judged superior to surgical therapy.

Outcome after referral for pediatric transplantation.

Although heart, heart-lung, or lung transplantation is performed in more than 200 children annually, the number of patients referred for such procedures is considerably greater, and little is known about the outcome of those referrals. To determine the outcome of pediatric transplant referrals we reviewed the follow-up of 31 patients evaluated at our institution for heart (24), heart-lung (three), or lung (four) transplantations between January 1991 and September 1992. Indications included hypoplastic left heart syndrome (seven patients), cardiomyopathy or myocarditis (seven patients), and postoperative congenital heart disease (10 patients) for heart transplantation; Eisenmenger's syndrome (three patients) for heart-lung transplantation; and primary pulmonary hypertension (two patients), broncho pulmonary dysplasia, and cystic fibrosis for lung transplantation. Only 14 of 31 referred patients were listed for transplantation; the remaining 17 patients either improved when medical therapy was maximized (nine patients), died within days of referral (three patients), refused (two patients), chose alternate surgery (one patient), were medically unacceptable (one patient), or are currently undecided (one patient). To date 7 of 14 patients listed have undergone successful heart transplantation; the remaining seven patients either improved and did not require transplantation (two patients), refused (one patient), died waiting (one patient), are currently awaiting a donor (one patient), or underwent Norwood procedure because of donor unavailability (two patients). Thus including patients who died before listing, only 14 of 31 referrals (45%) were deemed in need of a transplant.(ABSTRACT TRUNCATED AT 250 WORDS)

Results of a right ventricular outflow patch for pulmonary atresia with intact ventricular septum.

BACKGROUND: Although overall outcome has improved, pulmonary atresia with intact septum remains a difficult surgical and clinical problem. To determine whether an early right ventricular outflow patch will result in biventricular repair for this lesion, we reviewed the long-term follow-up (5.8 +/- 0.8 years) of 19 newborns who underwent repair between 1979 and 1990. METHODS AND RESULTS: An early right ventricular outflow patch was placed in 15 of 19 newborns; in the remaining four, this was preceded by an aortopulmonary shunt. Prostaglandin E1 infusion postoperatively eliminated the need for shunt in 14 of 15. Coronary sinusoids were ligated in three newborns. Based on right ventricular morphology, the newborns were divided into two groups: group 1 (tripartite, n = 9) and group 2 (bipartite and monopartite, n = 10). Before surgery, group 1 had significantly larger right ventricular volumes (23.6 +/- 3.7 versus 5.2 +/- 1.1 ml/m2, p < 0.002). Five-year survival was 79% for the entire series. Four infants, all group 2, died within 12 months of their initial surgery. Fourteen of 15 survivors (nine group 1 and five group 2) currently are acyanotic and New York Heart Association functional class I. A biventricular repair was achieved in 12 of 15, and three other children are awaiting evaluation. All 15 survivors had significant right ventricular and tricuspid annulus growth. CONCLUSIONS: Our data suggest that early placement of a right ventricular outflow patch in infants with pulmonary atresia and intact ventricular septum, regardless of right ventricular anatomy, results in an excellent chance for biventricular repair.

Surgical repair is safe and effective after unsuccessful balloon angioplasty of native coarctation of the aorta.

Since 1985 balloon angioplasty, followed by surgical repair if angioplasty is unsuccessful, has been used as a treatment strategy for eligible children with discrete native coarctation of the aorta. Although balloon angioplasty has been successful in most patients, this strategy is appropriate only if surgery is safe and effective in children in whom angioplasty does not succeed. To address this issue, the surgical procedure and clinical outcome in 11 children who underwent surgery after unsuccessful balloon angioplasty (defined as a residual systolic gradient greater than 20 mm Hg in 10 and a saccular aneurysm in 1) were evaluated. Data for subjects were compared with data for a control group of seven children who had surgical repair of a discrete coarctation without prior angioplasty during the same time period. In the study group, balloon angioplasty was performed at 4.3 +/- 1.2 years of age, resulting in a balloon/isthmus ratio of 0.98 +/- 0.05 and decreasing mean peak systolic gradient from 54 +/- 3 to 27 +/- 2 mm Hg (p less than 0.001). Follow-up angiography (n = 7) or nuclear magnetic resonance imaging (n = 4) documented a discrete residual stenosis in 10 patients and a small saccular aneurysm in 1. Collateral circulation decreased in three patients. The subsequent surgical procedure and its outcome were similar in the study and control groups. Chylothorax was the only complication, occurring in one child from each group. No paraplegia or mortality occurred. Pathologic examination revealed irregular intimal surfaces with small flaps of intima in 5 of 10 resected specimens from the study group and in 2 of 6 from the control group.(ABSTRACT TRUNCATED AT 250 WORDS)