Atrial tachyarhthmias complicating Fontan correction may have a 'malignant' clinical presentation seriously impairing the patient haemodynamic. Current strategies are surgical total cavopulmonary connection with or without antiarrhythmic surgery or transcatheter ablation. We describe the case of a patient who previously underwent atriocaval Fontan correction and later presented with refractory atrial tachycardia responsible for relapsing syncope. After a failed attempt at surgical conversion, and while waiting for heart transplantation, he was submitted to ablation of the atrioventricular node through an aortic retrograde approach and ventricular pacing through the coronary sinus tree. One year later, the patient is doing well, displaying a stable functional recovery and excellent pacing lead performances. An ablate and pace approach may deserve consideration in selected Fontan patients experiencing life-threatening atrial arrhythmias.

AIMS The aim of this study was to evaluate the feasibility and reliability of selective-site pacing by means of a new lead system in a paediatric population. This lead system is composed of a 4.1 Fr, active-fixation lead and a steerable catheter that allows easy positioning in selective sites. METHODS AND RESULTS Thirty young patients (mean age 9.0 +/- 4.5 years, range 2-16 years) received a single- (10) or dual- (20) chamber pacemaker. The 3830 lead was implanted successfully in the targeted chambers in all patients. The selective RV sites of pacing in 26 of the patients were: 18 mid-septum, 5 outflow tract, 1 low-septum, and 2 LEVO-RV-Apex. In all patients, an intracardiac loop was left in order to avoid stretching of the lead with growth. Mean follow-up duration was 11 +/- 10 months. Atrial sensing and pacing thresholds were 3.2 +/- 1.7 mV and 0.8 +/- 0.6 V at 0.5 ms at implantation and 3.4 +/- 2.1 mV and 0.6 +/- 0.3 V at 0.5 ms at follow-up. Ventricular sensing and pacing thresholds were 12.1 +/- 4.9 and 0.7 +/- 0.4 V at 0.5 ms on implantation and 12.7 +/- 6.1 mV and 0.8 +/- 0.5 V at 0.5 ms at follow-up (P = NS). No adverse events were reported. CONCLUSION Select Secure is a promising system for selective-site pacing in children.

OBJECTIVE:To study anti-Ro/La-negative congenital heart block (CHB). METHODS:Forty-five fetuses with CHB were evaluated by analysis of anti-Ro/La antibodies using sensitive laboratory methods. RESULTS:There were 9 cases of anti-Ro/La-negative CHB; 3 died (33.3%). Only 3 (33.3%) were complete in utero and 5 (55.5%) were unstable. No specific etiology was diagnosed. Six infants (66.6%) were given pacemakers. There were 36 cases of anti-Ro/La-positive CHB. All except 2 infants (94.4%) had complete atrioventricular block in utero. Ten died (27.8%), one (2.7%) developed severe dilated cardiomyopathy, and 26 (72.2%) were given pacemakers. CONCLUSION:Nine of the 45 consecutive CHB cases (20%) were anti-Ro/La-negative with no known cause. They were less stable and complete than the anti-Ro/La positive cases.

A 49-year-old woman affected by the Turner syndrome with a history of repaired congenital cardiomyopathy and persistent atrial tachycardia was referred to our center for radiofrequency ablation. At 11 years of age, she had undergone a surgical closure of an ostium secundum atrial septal defect (direct suture) combined with ligation of a persistent left superior vena cava, the embryological precursor of the ligament of Marshall, at the level of its connection to the coronary sinus. At hospitalization, her electrocardiogram showed an atrial tachycardia (cycle length 375 ms) with long phases of 1:1 atrioventricular conduction. Before the procedure, a computed tomography (CT) scan was performed to merge the anatomic data with the electrophysiological findings during the procedure (CARTO-MERGE, Biosense Webster, Inc, Diamond Bar, Calif). The preacquired CT image, processed with CARTO-MERGE system, showed the presence of a giant left superior vena cava (isolated from the coronary sinus) and a sinus venosus defect (SVD) associated with a right-sided anomalous pulmonary venous connection. In detail, both right superior pulmonary vein and right inferior pulmonary vein drained into the superior …

Long-term survival of patients with congenital heart disease has dramatically improved during the last 50 years and the number of adults with congenital heart disease is therefore increasing in all developed countries. Grown-up patients with congenital heart disease (GUCH) often present difficult and challenging problems. Patients with both native unoperated and operated malformations contribute to the GUCH population. Survivors without surgical treatment mainly have simple malformations; but a few have complex diseases, and some have survived with secondary pulmonary hypertension. Among operated malformations there are patients with a 'complete' repair (anatomical and physiological), others with a definitive palliation (physiological repair) and some with a simple palliation. The clinical spectrum is obviously diversified, depending on the underlying anomaly, surgical outcome, presence of residua, sequelae and/or complications, length of follow-up, comorbidities. Arrhythmias, bacterial endocarditis, cyanosis, polycythemia, heart failure, anomalies of pulmonary circulation, deterioration or malfunction of devices, need of cardiac and non-cardiac surgery, intercurrent non-cardiac diseases, and a need for cardiac and non-cardiac diagnostic procedures are common problems of adults with congenital heart disease. Physiological events such as pregnancy and childbearing deserve a careful multidisciplinary approach. While most simple native and well corrected GUCH do not require very specialized treatments throughout their life, the abnormalities and complexities of postoperative anatomy are often beyond the expertise of the adult cardiologist, requiring multidisciplinary competence in specialized tertiary centers. The problem is still unresolved and involves cultural, medical, technological and economically relevant issues.

OBJECTIVE:Primary repair is the treatment of choice in patients with tetralogy of Fallot. The timing of repair, however, remains controversial, and an initial palliative procedure might be considered a valuable option in the early management of symptomatic young infants and in those with either unfavourable anatomy, major associated lesions or chromosomal abnormalities with a poor life expectancy. METHODS:We reviewed the management of 100 consecutive patients with tetralogy of Fallot who were operated upon at our department during an 8-year period from June 1995 to March 2003. The rationale for the choice of the initial management and the outcome in terms of morbidity and mortality in patients who underwent primary repair was compared to that observed in patients who had had a two-stage repair. RESULTS:Age less than 3 months, the presence of either an unfavourable anatomy or major associated defects and genetic disorders with poor life expectancy were the indications for an initial palliation, which was carried out in 31 patients. There were no hospital deaths, and 28 of these patients underwent later repair with one hospital death (3.5%). Two patients with severe chromosomal abnormalities died at home and the remainder required a further palliation because of severely hypoplastic pulmonary arteries. Primary repair was carried out in 69 patients with one hospital death (1.4%). A transannular patch, which was used in 80% of our patients, was not an incremental risk factor for death regardless of the type of repair. Eight patients were reoperated on because of either residual right ventricular outflow tract obstruction with (four patients) or without (one patient) residual ventricular septal defect or isolated residual ventricular septal defect (three patients). All reoperations occurred in patients undergoing primary repair. CONCLUSIONS:The outcome of patients undergoing repair of tetralogy of Fallot is not influenced in terms of either mortality or morbidity by an initial palliative procedure.

Cervical aortic arch (CAA) is a rare congenital abnormality in which the aortic arch is situated cranially to its usual position, often associated with complex developmental alteration of aortic laterality and branching. More recently, some authors reported deletion of chromosome 22q11 (del22q11) in few patients with CAA. The aim of this study was to describe the clinical, anatomopathological and genetic pattern of 10 new cases of CAA. From 1975 to 2003, 10 patients with CAA (4 female, mean age 12.4+11.1 years) underwent complete cardiovascular evaluation and screening for del22q11 using fluorescence in situ hybridization. Six patients underwent CAA surgical repair with histological analysis of aorta samples. Six patients were asymptomatic; one patient had syncope on effort, 2 patients had dyspnea on effort and 1 had cyanosis and dyspnea (tricuspid atresia). A murmur and/or a pulsatile mass of the neck was present in all patients. Femoral pulses were weak or absent in 6 patients. Four patients had right and 6 left CAA; kinking or true obstruction of the aortic arch was present in 9/10. Abnormal aortic arch branching pattern was found in 8/10 (mainly aberrant subclavian artery). Two patients had del22q11 with dysmorphic features, multiorgan anomalies and degenerative mediocystic necrosis of aortic wall. Our data confirm clinical polymorphism and anatomic complexity of CAA as well as the association between del22q11 and CCA in 2 syndromic patients.

BACKGROUND:Supraventricular arrhythmias complicate operated and unoperated congenital heart disease, especially when atrial dilatation coexists. METHODS:We evaluated the results of intraoperative ablation in a group of 23 patients with chronic supraventricular tachyarrhythmias (mostly intraatrial reentry) that were unresponsive to conventional medical therapy. All procedures were performed consecutively between September 1999 and November 2001. Ablation was done during redo operations (Fontan conversion to total cavopulmonary connection in 16 patients) in 18 patients and during primary surgical correction in 5 patients. The mean age at operation was 25 +/- 12 years (2 to 50 years). Cryoablation was done in 10 patients and radiofrequency ablation in 13 patients. Nineteen patients had ablation in one atrium and 4 had ablation in both atria. A generous atrial reduction was always performed at the end of the operation. RESULTS:The operative mortality rate was 13% (3 patients) from causes unrelated to ablation. In 20 survivors, the ablation was effective immediately. Eight patients required a permanent pacemaker. During a mean follow-up of 22 +/- 4 months, atrial arrhythmias recurred in 25% (5 patients) and were controlled with medical therapy, whereas 1 patient required pacemaker implantation. CONCLUSIONS:Intraoperative treatment of unresponsive atrial tachyarrhythmias associated with operated or unoperated congenital heart disease is feasible and the midterm results are encouraging.

Aortopulmonary window coexisting with tetralogy of Fallot (TF) was prospectively diagnosed by two-dimensional (2D) echocardiography and Doppler in an 18-month-old boy; the diagnosis was confirmed by cardiac catheterization and angiocardiography. Surgical correction was performed, but the patient died in the operating room from right ventricular dysfunction. The autopsy showed an adequate surgical repair, but the histologic examination of the lungs demonstrated severe pulmonary vascular disease, which was presumed to be the cause of death.

From January 1981 to June 1984, 80 patients with double-inlet ventricle with two atrioventricular (AV) valves were studied by cross-sectional echocardiography at Ospedali Riuniti (Bergamo, Italy) and Brompton Hospital (London, United Kingdom). According to Anderson’s classification [1], 69 patients had double-inlet left ventricle, six had double-inlet right ventricle, and five had double-inlet solitary ventricle of indeterminate morphology; identification of the rudimentary ventricle and its relationships with the dominant ventricle allowed the echocardiographic categorization into these three groups [2–4]. Anomalies of the atrioventricular valves were found in 34 cases (42.5%) (Table 1).