Symptomatic atrial arrhythmias and transcatheter closure of atrial septal defects in adult patients.

OBJECTIVE: To determine whether transcatheter device closure of a secundum atrial septal defect (ASD) will reduce the risk of developing subsequent atrial arrhythmias. DESIGN: The incidence and predictors of symptomatic atrial tachyarrhythmias (AT) were examined in adults undergoing transcatheter closure of ASDs. SETTING: Toronto Congenital Cardiac Centre for Adults. PATIENTS: 132 consecutive patients, mean (SD) age 44 (16) years; 74% female. MAIN OUTCOME MEASURE: Sustained or symptomatic atrial arrhythmias at early follow up (six weeks; n = 115) and intermediate follow up (last clinic visit 17 (11) months post surgery; n = 121). RESULTS: 15% of the patients (20 of 132) had AT before the procedure (14 paroxysmal, six persistent). Patients without a history of arrhythmia had a low incidence of AT during early follow up (6%) and intermediate follow up (1%/year), while all patients with persistent AT before closure remained in atrial fibrillation or flutter. Of patients in sinus rhythm but with a previous history of AT, two thirds remained arrhythmia-free at follow up, with overall incidences of paroxysmal and persistent AT of 17%/year and 11%/year. A history of AT before closure (risk ratio (RR) 35.0, 95% confidence interval (CI) 7.2 to 169.0) and age > or = 55 years at the time of device insertion (RR 5.6, 95% CI 1.2 to 25.0) predicted AT after closure. CONCLUSIONS: Device closure of an ASD before the onset of atrial arrhythmias may protect against the subsequent development of arrhythmia, in particular in patients less than 55 years of age.

Acute myocardial infarction late after Mustard procedure for dextrotransposition of the great arteries.

Systemic right ventricular dysfunction has been closely linked to late mortality and sudden cardiac death in patients with Mustard procedure for dextrotransposition of the great arteries. Two young patients with dextrotransposition of the great arteries late after Mustard procedure who presented with acute transmural myocardial infarction and sudden cardiac death (one patient) without prior exertional angina or causative coronary abnormalities are reported. It is surmised that acute coronary emboli originating from a severely dilated, hypocontractile systemic ventricle were the cause of transmural myocardial infarction. This phenomenon may be an important and as yet unrecognized factor in late morbidity and mortality in such patients.

The Fontan procedure in adults.

SETTING: Tertiary adult congenital cardiac referral centre. DESIGN: Retrospective cross sectional analysis. OBJECTIVES: To report our 20 year experience with adult Fontan operations, and to compare late outcome in patients with single ventricle with definitive aortopulmonary or cavopulmonary shunt palliation. PATIENTS AND MAIN OUTCOME MEASURES: Patients older than 18 years undergoing Fontan operation between 1 January 1982 and 31 December 1998 were identified. Mortality and late outcome were derived from hospital records. These patients were compared with a cohort of 50 adults with single ventricle who had not undergone a Fontan operation. RESULTS: 61 adults, median age 36 years (range 18-47 years), with a median follow up of 10 years (range 0-21 years) were identified. Actuarial survival was 80% at one year, 76% at five years, 72% at 10 years, and 67% at 15 years. Compared with before the Fontan operation, more patients were in New York Heart Association (NYHA) functional class I or II at the latest follow up (80% v 58%, p < 0.001). Systolic ventricular function deteriorated during follow up such that 34% had moderate to severe ventricular dysfunction at the latest follow up compared with 5% before Fontan (p < 0.001). Arrhythmia increased with time (10% before Fontan v 57% after 10 years, p < 0.001). Fontan patients had improved NYHA functional class, ventricular function, atrioventricular regurgitation, and fewer arrhythmias than the non-Fontan group at the latest follow up. CONCLUSION: The Fontan operation in adults has acceptable early and late mortality. Functional class, systolic ventricular function, atrioventricular regurgitation, and arrhythmia deteriorate late after surgery but to a lesser degree than in non-Fontan patients with a single ventricle.

Right ventricular form and function after percutaneous atrial septal defect device closure.

OBJECTIVES: We sought to assess the right heart's response to percutaneous device closure of moderate sized atrial septal defects (ASDs) in adults over a one-year follow-up period. BACKGROUND: Percutaneous ASD device closure is a safe and effective means of reducing or eliminating interatrial shunting. The response of the adult's right heart to device closure is incompletely understood. METHODS: Forty consecutive patients had 40 device implantations (32 with the CardioSeal implant and 8 with the Amplatzer device). The patients were assessed with echocardiography, chest radiography and electrocardiography before the procedure and at 1, 6 and 12 months. RESULTS: The mean ASD size was 13+/-4 mm, and the device size ranged from 33 to 40 mm for CardioSeal and 12 to 36 mm for Amplatzer. At one month, heart size (49% vs. 46%), four-chamber right ventricular (RV) size (45 vs. 41 mm), paradoxical septal motion (60% vs. 5%), QRS duration (125 vs. 119 ms), PR interval (181 vs. 155 ms) and echocardiographically determined pulmonary artery systolic pressure decreased significantly and was maintained at 12-month follow-up. At six months, right atrial length decreased from 50 to 47 mm. At one year, 29% of patients had persistent RV enlargement. CONCLUSIONS: Right heart morphology undergoes rapid improvement within one month of defect closure, with associated mechanoelectrical benefit. A small number of patients had persistent RV enlargement or pulmonary hypertension, or both, at one year. Our data support the application of transcatheter methods in achieving excellent hemodynamic and anatomic outcomes.

Sustained atrial arrhythmias in adults late after repair of tetralogy of fallot.

We determined the prevalence of sustained atrial tachyarrhythmia (AT) in adults late after repair of tetralogy of Fallot (ToF) and examined its impact on subsequent heart failure, reoperation, and mortality. Ventricular arrhythmias are associated with increased morbidity and mortality in patients with repair of ToF. The clinical impact of AT in this population has not been established. A retrospective cohort study of 242 patients with repaired ToF identified 29 patients (prevalence of 12%) with sustained episodes of AT. Patients with repaired ToF but without sustained arrhythmia (n = 213) constituted a comparison group. Baseline characteristics and clinical outcomes in the 2 groups were compared. An echocardiographic analysis compared 15 patients with AT and 15 matched for age at operation and timing of echocardiography. The development of AT was associated with substantial morbidity including congestive heart failure, reoperation, subsequent ventricular tachycardia, stroke, and death (combined events, 20 of 29 patients [69%]). The rate of combined events (congestive heart failure, stroke, and deaths) in the 213 "arrhythmia-free" patients was 30% (64 of 213 patients). Event-free survival after repair was 18 +/- 2 years for the AT group and 28 +/- 1 years for the arrhythmia-free group (p < 0.001). Patients with AT were older at surgical repair (25 +/- 16 vs 10 +/- 9 years, p = 0.001), and at most recent assessment were aged 48 +/- 12 vs 32 +/- 10 years (p = 0.001). The AT group had a higher mean right atrial volume and proportion of significant pulmonary regurgitation than matched controls. The development of AT in the adult late after ToF repair identifies patients at risk and is associated with older age at repair, a higher frequency of hemodynamic abnormalities, and increased morbidity.

Care of adults with congenital heart disease--a challenge for the new millennium.

The care of complex congenital heart defect (CHD) patients should be continuous through life. Medium- and high-risk patients should be seen in special facilities for grown-up congenital heart (GUCH) patients, and followed for life. CHD in the adult is different than CHD in the child. Transitional programs should be available to prepare the adolescent patient to take charge of his/her own health. The patient should be transferred smoothly from pediatric to adult care. GUCH care should be regionally anchored in special facilities (groups or sometimes individuals) willing to make a commitment to their care. Ideally, pediatric and adult cardiologists will collaborate. GUCH care should be multidisciplinary wherever possible--and certainly in supraregional centers. Surgery, diagnostic catheterization, interventional cath procedures, EP management, and even MRI should be done in selected centers with high quality services for all cases other than the most simple lesions. Care should be available at all times. Surgeons who operate on children with similar conditions should perform the most GUCH surgery. GUCH surgery should be performed in centers with adequate institutional and individual surgeon's volumes. In determining which surgeons and units have adequate volumes, both pediatric and adult volumes should be combined.

Outcomes of late atrial tachyarrhythmias in adults after the Fontan operation.

OBJECTIVES: The purpose of this study was to compare the clinical and echocardiographic features of adults who developed atrial tachyarrhythmias (ATs) late after a Fontan procedure with those who have remained free of arrhythmias. BACKGROUND: Atrial tachyarrhythmias are a frequent complication of the Fontan operation. However, the outcomes in adult patients with AT who have had the Fontan operation have not been well defined. METHODS: We reviewed the outcomes of 94 consecutive patients who underwent the Fontan operation between 1977 and 1994 and were followed as adults at the University of Toronto Congenital Cardiac Centre for Adults. Sixty patients had an atriopulmonary connection, 21 patients had an atrioventricular connection, and 13 patients received a lateral tunnel connection. RESULTS: Thirty-nine patients (41%) had sustained AT (atrial fibrillation, atrial flutter or supraventricular tachycardia) after their Fontan procedure. Compared with patients who did not develop AT, those who did were more likely to develop heart failure (46% vs. 13%, p = 0.003) and right atrial thrombus (31% vs. 4%, p = 0.006), exhibit left atrial enlargement (mean [+/-SD] diameter: 44 +/- 10 vs. 37 +/- 9 mm, p = 0.002), exhibit right atrial enlargement (mean [+/-SD] volume: 139 +/- 149 vs. 76 +/- 54 ml, p = 0.040) and have moderate-to-severe systemic valve regurgitation (31% vs. 7%, p = 0.010). The mean survival time was not significantly different between the arrhythmia group and the arrhythmia-free group (21.2 +/- 1.3 and 18.0 +/- 0.7 years, respectively; p = 0.900). CONCLUSIONS: Systemic atrioventricular valvular regurgitation and biatrial enlargement are commonly observed in patients who develop AT after the Fontan procedure. These patients are more likely to develop right atrial thrombus and heart failure.

Right and left ventricular systolic function late after repair of tetralogy of Fallot.

Right ventricular (RV) dysfunction has adverse effects on long-term outcome in patients with repaired tetralogy of Fallot (TOF). We employed serial radionuclide angiography (RNA) to examine RV and left ventricular (LV) systolic function in adults late after TOF repair and its relation to clinical outcome. We reviewed 10-year records of 95 patients (53 men) with TOF followed in our clinic (mean age at repair 12.6 +/- 10.5 years, mean age at last follow-up 37.7 +/- 9.8 years) who underwent at least 2 RNAs between 1987 and 1997. Most patients were well by the end of the study (80% were New York Heart Association class I, 17% were class II, and 3% were in class III). Sixteen patients experienced sustained tachyarrhythmias (8 had atrial; 8 patients had ventricular). One patient died suddenly. Fifteen patients underwent RV outflow reoperations (15 underwent pulmonary valve replacement; 7 had relief of RV outflow obstruction); RV systolic function during exercise in these 15 patients was significantly impaired before and returned to similar levels after surgery, compared with the rest of the patients. Overall, RV and LV function remained stable in the whole group at a mean interval of 5.7 +/- 2.2 years between first and last RNA. This group of closely followed adults with TOF remained well over 10 years with a low incidence of sudden death and stable RV and LV systolic function, despite a relatively large number of RV outflow reoperations. Aggressive intervention for right-sided hemodynamic abnormalities may have contributed to this outcome. Preserved ventricular function may herald a favorable long-term outlook in this group.