Pericardial patch augmentation of anterior tricuspid leaflet in Ebstein's anomaly

Although refinement of tricuspid valvuloplasty and plication techniques has opened the way to a satisfactory outlook for the majority of patients with Ebstein's anomaly, paucity of tissue in the anterior tricuspid leaflet may preclude successful valve repair. In this particular circumstance, a modified Carpentier repair of the tricuspid valve with additional pericardial patch augmentation of the anterior leaflet may result in a well-functioning monocusp tricuspid valve.     

Ebstein's anomaly of the tricuspid valve: a case report

A case of Ebstein's malformation of the tricuspid valve with a complicating ventricular septal defect in a 3-year-old Black child is presented. A pre-operative diagnosis of an atrioventricular canal was made, and only at operation was it discovered to be an Ebstein's anomaly of the tricuspid valve. The clinical picture and diagnoses are reviewed, and the problem of a correct pre-operative diagnosis is discussed. The operative treatment and postoperative result in our patient is described.     

A modified repair technique for tricuspid incompetence in Ebstein's anomaly

OBJECTIVE: A modified technique for tricuspid valve repair in Ebstein's anomaly restructures the valve mechanism at the level of the true tricuspid anulus by using the most mobile leaflet for valve closure without plication of the atrialized chamber. Midterm results of this therapeutic approach for patients with Ebstein's anomaly and tricuspid valve incompetence are reported. METHODS: Between October 1988 and April 1997, the incompetent tricuspid valve was repaired with our technique in 19 patients (12 female, 7 male; 2 to 54 years, mean 21 years). The indication for operation was congestive heart failure of various degrees in all patients. Tricuspid incompetence was grade II in two patients, grade III in 14, and grade IV in three. Associated congenital malformations were simultaneously repaired (interatrial communication in 18, ventricular septal defect in two, pulmonary stenosis in two, mitral valve prolapse in one). Follow-up ranged between 10 and 103 months (median 28 months) and was complete for all patients. RESULTS: There were no operative deaths. One patient with active endocarditis and pulmonary abscess died 2 months after the operation of recurrent sepsis; there were no late deaths. During follow-up, New York Heart Association functional class improved from 2.8 before the operation to 1.9 without recurrent cyanosis, and tricuspid incompetence decreased from a mean grade of 3.1 to one of 0.9, without any echocardiographic deterioration of the tricuspid valve function or right ventricular dilation. CONCLUSIONS: Our technique allows tricuspid valve repair in patients with Ebstein's anomaly, even in cases usually reserved for primary valve replacement, without late functional deterioration.     

Mimics of Ebstein's anomaly

The purpose of this study was to determine the most discriminating clinical and echocardiographic features that are most helpful in correctly identifying Ebstein's anomaly of the tricuspid valve from other causes of tricuspid regurgitation. Ebstein's anomaly is an uncommon malformation of the tricuspid valve with diagnostic echocardiographic features. Other cardiac disorders associated with tricuspid valve regurgitation and predominate right-sided heart chamber enlargement can be misdiagnosed as Ebstein's anomaly. All patients who were referred to our institution between 1982 and 1995 with the diagnosis of Ebstein's anomaly but were found to have other abnormalities of the tricuspid value or right ventricle were identified. Their clinical, echocardiographic, and surgical records were reviewed retrospectively. Twenty-two patients (12 males and 10 females), aged 7 to 68 years (mean 33 years), were referred to our institution with the diagnosis of Ebstein's anomaly but were found to have another abnormality that mimicked clinical and diagnostic features of Ebstein's anomaly. The most common initial symptom was exercise intolerance (13 [59%] patients) followed by atrial arrhythmia (seven [32%] patients). Two patients had cyanosis. Three patients had paroxysmal and six had chronic atrial fibrillation/flutter. Cardiomegaly on chest x-ray film was noted in 18 (82%) patients. Referral diagnosis of Ebstein's anomaly had been made by echocardiography (12 patients), cardiac catheterization (four patients), both techniques (five patients), and echocardiography and magnetic resonance imaging (one patient). All 22 patients had predominate right atrial and right ventricular enlargement, and 18 (82%) of 22 patients also had right ventricular dysfunction. However, Ebstein's anomaly was confidently ruled out with repeat comprehensive echocardiography at our institution by establishing (1) absence of significant apical displacement of the septal tricuspid valve leaflet (> or = 8 mm/m2) and (2) lack of a redundant, elongated, anterior tricuspid valve leaflet in all 22 patients (100%). All had significant tricuspid regurgitation caused by tricuspid valve dysplasia (nine patients), tricuspid valve prolapse (four patients), trauma (four patients), right ventricular dysplasia (three patients), endocarditis (one patient), and annular dilation caused by free pulmonary regurgitation (one patient). In all 15 patients who subsequently underwent surgery (tricuspid valve repair [seven patients] or replacement [eight patients]), the absence of Ebstein's anomaly was confirmed. Echocardiographic absence of the characteristic degree of displacement of the septal leaflet of the tricuspid valve (> or = 8 mm/m2) and the presence of a nonelongated, nonredundant anterior tricuspid valve leaflet consistently excluded the diagnosis of Ebstein's anomaly. Under such circumstances, other anomalies of the tricuspid valve or right ventricle were consistently identified. Recognition of the mimics of Ebstein's anomaly had important surgical implications.     

Successful radiofrequency current catheter ablation of accessory atrioventricular pathway after tricuspid replacement in Ebstein's anomaly

A 15-year-old female with Ebstein's anomaly was referred to hospital for radiofrequency (RF) current catheter ablation of her refractory paroxysmal supraventricular tachycardia (PSVT) after tricuspid valve replacement. A surface ECG showed ventricular preexcitation of type B Wolff-Parkinson-White (WPW) syndrome. In a baseline electrophysiological study, two types of PSVT with left and right bundle branch block (LBBB and RBBB) configurations were induced. The LBBB type was antidromic and the RBBB type was orthodromic atrioventricular reciprocating tachycardia (AVRT) with a right posterolateral accessory pathway. RF current was successfully delivered at the posterolateral site above the prosthetic valve (V-delta interval = -30 msec). The patient has been free from arrhythmias during a follow-up period of 9 months. RF current ablation seems to be useful for AVRT patients with corrected Ebstein's anomaly.     

Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebstein's anomaly

BACKGROUND: In patients with Ebstein's anomaly, localization of accessory pathways (APs) may be impeded by abnormal local electrograms recorded along the atrialized right ventricle and by the presence of multiple APs. The impact of these factors on radiofrequency (RF) current catheter ablation of APs has not been evaluated yet. METHODS AND RESULTS: Twenty-one patients with Ebstein's anomaly and reentrant atrioventricular tachycardias underwent electrophysiological evaluation and subsequent attempts at RF catheter ablation. Thirty-four right-sided APs were found, with 30 located along the atrialized ventricle. Local electrograms in this region were normal in 10 patients but fragmented in 11. Fragmented electrograms prevented the clear distinction between atrial and ventricular activation potentials as well as the identification of AP potentials. Right coronary artery mapping was performed in 7 patients. Abolition of all 26 APs was achieved in the 10 patients with normal local electrograms and in 6 of 11 patients with abnormal electrograms. Right coronary artery mapping allowed AP localization and ablation in 5 patients. In the 5 patients with abnormal electrograms and a total of 8 APs, 6 APs could not be ablated. Unsuccessfully treated patients received antiarrhythmic drugs. During 22 +/- 12 months of follow-up, 5 patients had clinical recurrences, including 4 who had undergone a successful RF procedure. CONCLUSIONS: In patients with Ebstein's anomaly and reentrant atrioventricular tachycardias, factors likely to account for failure of RF catheter ablation include an AP located along the atrialized right ventricle and the abnormal morphology of endocardial activation potentials generated in this region.     

Surgical treatment of patients with Wolff-Parkinson-White syndrome and associated Ebstein's anomaly

Ebstein"s anomaly is the most common congenital heart disease associated with the Wolf-Parkinson-White syndrome. Between November 1973 and March 1993, we surgically treated 42 patients with Wolff-Parkinson-White syndrome and Ebstein's anomaly. The patient's ages ranged from 5 months to 59 years (mean 35.3 +/- 14.0 years). There were a total of 52 accessory pathways, 48 of which were located in the right (65%) or posteroseptal (29%) area. A left-sided accessory pathway was seen in only two patients (3.8%). Division of all right-sided accessory pathways was done during normothermic cardiopulmonary bypass with the heart beating; cryocoagulation was applied together with scalpel dissection of the atrioventricular groove. Division of the left-sided accessory pathways was done with the use cold potassium cardioplegic arrest. Thirty-five of these patients underwent tricuspid valve operation for Ebstein's anomaly and 11 of them underwent tricuspid valve replacement with a bioprosthesis. All 52 accessory pathways were successfully divided, although two patients required reoperation because of tachycardia caused by accessory pathways in different positions. Three hospital deaths (7.1%) occurred. There were no late deaths during the follow-up period (mean 94.3 +/- 52.4 months), but two patients required repeat tricuspid operation because of progression of the tricuspid regurgitation. Because no repeat operations were required during long-term follow-up patients who underwent valve repair or valve replacement, correction should be indicated in some patients.     

Surgical management of tricuspid atresia and anomalous left brachiocephalic vein

An anomalous left brachiocephalic vein is an uncommon systemic venous anomaly, which usually has no clinical significance. We describe a case of tricuspid atresia with such an anomalous left brachiocephalic vein. The presence of this unusual venous anomaly had a number of implications in the surgical management of the tricuspid atresia.     

Exercise capacity and mid-term survival in patients with tricuspid atresia and complex congenital cardiac malformations after modified Fontan-operation

A 68-year-old man experienced systemic pruritus since he was 63 years old, and systemic sclerosis and skin pigmentation were observed when he was 64. When he developed dyspnea the same year, he was admitted and SSc was diagnosed on the basis of the clinical and skin biopsy findings, lung fibrosis on X-P and TBLB findings. At 65, his dyspnea reappeared along with elevated blood pressure, acute renal failure and lung congestion, and he was diagnosed as having a scleroderma renal crisis (SRC) from the clinical and renal biopsy findings. Hemodialysis was started because he showed mental disturbance, and this and other acute symptoms were subsequently reduced. As he showed no recovery from his renal failure, the patient has been maintained on hemodialysis for over four years now. In the meantime, his sclerosis has improved and antinuclear antibody almost disappeared. Hemodialysis appears to be the most likely reason for his improvement, although spontaneous remission, D-penicillamine and angiotensin converting enzyme (ACE) inhibitor therapy may also have contributed, considering the short period and the small amount of drugs given until improvement.     

Determinants of successful balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum

OBJECTIVES: This study reviewed our experience with percutaneous balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum and defined the anatomic and hemodynamic characteristics of infants in whom this procedure is successful and provides definitive therapy. BACKGROUND: Unlike children with valvular pulmonary stenosis, the follow-up of infants with critical pulmonary stenosis undergoing percutaneous balloon valvotomy is limited. METHODS: Between December 1987 and August 1992, percutaneous balloon valvotomy was attempted in 12 infants with critical pulmonary stenosis (n = 10) or pulmonary atresia with intact ventricular septum (n = 2). Two outcome groups were identified: Group A patients are acyanotic, have mild residual pulmonary stenosis and have not required operation; Group B patients have required operation. RESULTS: Of the 12 infants, 11 had a successful balloon valvotomy procedure. Group A patients (n = 7) have a residual gradient of 22 +/- 18.7 mm Hg (mean +/- SD) at follow-up of 3.2 years (range 1.2 to 5.0). In Group B (n = 5), operation was required for inability to cross the pulmonary valve (n = 1) or persistent severe hypoxemia for > or = 2 weeks after valvotomy (n = 4). Significant differences (p < or = 0.01) between the two groups (Group A vs. Group B) were identified in pulmonary valve annulus (Z value) 8.1 mm (-1.1) versus 5.5 mm (-3.4); tricuspid valve annulus (Z value) 14.0 mm (0.8) versus 8.8 mm (-1.8); right ventricular volume 65 versus 29 ml/m2; and Lewis index 10.9 versus 8.9. CONCLUSIONS: Percutaneous balloon valvotomy is effective and likely to provide definitive therapy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum who have a tricuspid valve annulus > 11 mm, pulmonary valve annulus > or = 7 mm and right ventricular volume > 30 ml/m2.     

Anastomosis of the left juxtaposed atrial appendages in a patient with tricuspid atresia

A 9-month-old boy with left juxtaposition of the atrial appendages, tricuspid atresia, pulmonary atresia, and ventriculoarterial discordance underwent anastomosis between the atrial appendages after failure of balloon/blade atrial septostomy because of restrictive atrial septal defect. For surgical creation of atrial communication in patients with juxtaposed atrial appendages, anastomosis between the atrial appendages seemed to be safer, more effective, and less invasive than septectomy by Blalock-Hanlon technique or inflow occlusion technique.     

Ebstein's malformation of the tricuspid valve: genetic and environmental factors. The Baltimore-Washington Infant Study Group

Ebstein's anomaly is a specific structural deformity of the tricuspid valve, and its rarity has hampered etiologic evaluation. Cases of Ebstein's anomaly registered in the Baltimore Washington Infant Study (BWIS), a regional case-control study of cardiovascular malformations (CVM) in infancy, are reviewed. Between 1981 and 1989 a total of 4,390 CVM cases, including 47 Ebstein cases, and 3,572 controls were registered. The prevalence of Ebstein's anomaly was 5.2 per 100,000 livebirths. Additional cardiac anomalies were present in 38.3% of Ebstein cases. Non-cardiac malformations were present in 19.1% of Ebstein cases vs. 25.5% of other CVM, and 1.7% of controls. Case-fatality by 1 year of age was 23.4% in Ebstein vs. 18.1% in other CVM. Interviews of parents of Ebstein cases, other CVM, and controls (n = 44, 3,335, and 3,572, respectively) elicited information on family history of malformations, maternal illnesses, reproductive history, therapeutic drugs, parental lifestyle, and environmental exposures during the periconceptional period. Case-control analyses suggest genetic, reproductive, and environmental risk factors: twins [odds ratio (OR) 8.2, 95% confidence interval (CI) 2.6-25.3]; family history of CVM (OR 6.4, 95% CI 1.8-22.2); white race (OR 2.9 with non-whites as reference, 95% CI 1.2-7.0); previous miscarriages (OR 2.0, 95% CI 1.2-3.3); maternal exposure to benzodiazepines (OR 5.4, 95% CI 1.5-19.1); and varnishing (OR 3.4, 95% CI 1.3-9.1). Additional multicenter investigations are warranted to elucidate the role of genetic, reproductive, and environmental factors in the etiology of this anomaly.     

Congenital diaphragmatic hernia. I. Simple defect of the diaphragm or anomaly of the pulmonary mesenchyme?

Described for the first time in 1848 by Bochdalek, congenital diaphragmatic hernia is still a hot topic. How can it be that a simple defect of the diaphragm still has a mortality rate reaching 50% in 1997, and this despite continuous progress in neonatal intensive care? If some problems remain unsolved, experimental studies over the past 30 years have raised some questions concerning the pathogenesis, and have shed some light into the pathophysiology of congenital diaphragmatic hernia. This article reviews the recent knowledge about the aetiology, pathogenesis and pathophysiology of this complex malformation.     

Surgical treatment of pulmonary atresia with intact ventricular septum

Fifty-nine children with pulmonary atresia and intact ventricular septum underwent various forms of surgical treatment at the Hospital for Sick Children, Toronto, during 1950 to mid 1975. Twenty-three patients had pulmonary valvotomy, 15 direct, 2 indirect, and 6 both direct valvotomy and infundibulectomy. All died, 19 early and 4 late. Of 13 patients who received a systemic-pulmonary artery shunt, 4 combined with surgical atrial septectomy, there are only 2 long-term survivors both of whom were children who had had a Waterston anastomosis. Recently we have been treating infants with small right ventricles with balloon atrial septostomy at cardiac catherterization followed by a Potts anastomosis and pulmonary valvotomy. If the Potts anastomosis appears satisfactory the persistent ductus arteriosus is ligated. This scheme was used in 23 infants, with 4 early deaths and 2 late deaths. Of 17 survivors, further shunts were required in 4 children. One child has had a formal repair, with insertion of valves in both tricuspid and pulmonary areas. We believe that this operative combination of Potts anastomosis and pulmonary valvotomy offers the infant with pulmonary atresia and a small right ventricle a relatively low initial mortality and the possibility of right ventricular enlargement and subsequent repair.     

Suspension of straddling tricuspid valve chordae into the appropriate ventricle

In 2 children with an inlet ventricular septal defect and straddling chordae tendineae of the septal leaflet of the tricuspid valve to the posteromedial papillary muscle of the mitral valve and to an accessory papillary muscle in the left ventricle, the straddling chordae were excised with a wedge of posteromedial papillary muscle and with the top segment of the accessory papillary muscle, respectively. After patch closure of the ventricular septal defect, the papillary muscle segment with its group of chordae was anchored to the right ventricular septum with resulting competence of the tricuspid valve. In contrast to the traditional repair technique, the reported modification is applicable when the straddling chordae insert into a papillary muscle of the mitral valve. In addition, various disadvantages related to the construction of a complex baffle in the inappropriate ventricle are avoided.     

Intraoperative discovery of neuroblastoma in an infant with pulmonary atresia

There have been 28 previously reported cases of neuroblastoma associated with congenital heart disease. Because many of these have been defects of the conotruncal region, it has been proposed that abnormal neural crest cell migration or maturation may be a factor that links these normally disparate pathologic conditions. Most neuroblastomas in these cases have been detected at autopsy or by radiologic studies conducted in the evaluation of the cardiac anomalies. Recently, we discovered an occult posterior mediastinal neuroblastoma in a patient undergoing a unifocalization procedure for tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals. The tumor was resected, and the patient has demonstrated no evidence of residual or metastatic neuroblastoma.     

Evaluation and management of pulmonary atresia with intact ventricular septum

The Consensus Conference of the American College of Medical Genetics has established guidelines regarding the evaluation of patients with mental retardation (MR) [Curry et al., Am. J. Med. Genet. 72:468-477, 1997]. They emphasized the high diagnostic utility of cytogenetic studies and of neuroimaging in certain clinical settings. However, data on the diagnostic yield of these studies in well-characterized populations of individuals with MR are scant. Majnemer and Shevell [J. Pediatr. 127:193-199, 1995] attained a diagnostic yield of 63%. However, this study included only 60 patients and the classification included pathogenetic and causal groups. The Stella Maris Institute has evaluated systematically patients with developmental delay (DD)/MR and performed various laboratory studies and neuroimaging in almost all patients. We report a retrospective analysis of the diagnostic yield of 120 consecutive patients observed at our Institute during the first 6 months of 1996. There were 77 males and 43 females; 47 were mildly delayed (IQ 70-50), 31 were moderately delayed (IQ 50-35), and 42 were severely delayed (IQ 35-20). Diagnostic studies (history, physical examination, standard cytogenetics, fragile X testing, molecular studies, electroencephalography, electromyography, nerve conduction studies, neuroimaging, and metabolic screening tests) yielded a causal diagnosis in 50 (41.6%) and a pathogenetic diagnosis in 47 (39.2%) of the 120 patients. Causal categories included chromosomal abnormalities (14), Fra(X) syndromes (4), known MCA/MR syndromes (19), fetal environmental syndromes (1), neurometabolic (3) disorders, neurocutaneous (3) disorders, hypoxic-ischemic encephalopathy (3), other encephalopathies (1), and congenital bilateral perisylvian syndrome (2). Pathogenetic categories included idiopathic MCA/MR syndromes (35), epileptic syndromes (10), and isolated lissencephaly sequence (2). Diagnostic yield did not differ across categories and degree of DD. Our results, while confirming the diagnostic utility of cytogenetic/molecular genetic, and neuroimaging studies, suggest the usefulness of accurate electroencephalogram recordings, and stress the importance of a thorough physical examination. Referral to a university child neurology and psychiatry service, where a comprehensive assessment with a selected battery of investigations is possible, yields etiologic findings in a high percentage of DD/MR patients, with important implications for management, prognosis and recurrence risk estimate.     

Effectiveness of an umbilical artery "snare assisted" approach for critical pulmonary valve stenosis or atresia in the neonate

Thirteen neonates with critical pulmonary valve stenosis/atresia underwent successful transcatheter balloon valvuloplasty using an umbilical artery "snare assisted" approach. This technique simplifies the procedure and avoids femoral artery injury by using the umbilical artery, reduces fluoroscopy exposure, and eliminates the need for a gradational approach which reduces costs.