[Radiofrequency transcatheter ablation of the left lateral Kent bundle in a premature newborn after 28 weeks of pregnancy with prenatal diagnosis of incessant tachycardia: 28-year follow-up]. / Ablazione transcatetere di fascio accessorio tipo Kent sinistro laterale in nato prematuro a 7 mesi di gestazione con diagnosi prenatale di tachicardia incessante: follow-up a distanza di 28 anni.

A case of incessant tachycardia diagnosed at the seventh month of pregnancy conditioning hydrops fetalis is described. Delivery was induced by caesarean section. Atrioventricular reciprocating tachycardia was due to the involvement of a left lateral accessory pathway refractory to multiple antiarrhythmic drugs and was often associated with ipsilateral left bundle branch block at a slower rate. To the best of our knowledge, this is the smallest premature infant in whom radiofrequency catheter ablation was necessary. There were no complaints of complications with recognizable clinical involvement but only a pericardial effusion diagnosed by echocardiography that reabsorbed within a few days. At 28 years old, the psycho-somatic development of this young man is optimal, with normal findings at cardiac imaging. This treatment modality has proven feasible but remains a procedure to be performed only in exceptional cases by very experienced operators.

Cardiomyopathy in a male patient with neutropenia and growth delay.

Neutropenia encompasses a family of neutropenic disorders, both permanent and intermittent, ranging from severe (<500 neutrophils/mm(3)) to mild (500-1500 neutrophils/mm(3)), which may also affect other organ systems such as the pancreas, central nervous system, heart, muscle and skin. Neutropenia can lead to life-threatening pyogenic infections whose severity is roughly inversely proportional to the circulating neutrophil counts.When neutropenia is detected, an attempt should be made to establish the etiology, and to distinguish acquired forms (the most frequent, including post viral neutropenia and autoimmune neutropenia) and congenital forms (rare disorders) that may be either isolated or part of a complex rare genetic disease. We report on a male patient initially diagnosed with isolated neutropenia who later turned out to be affected with Barth syndrome, a rare complex inherited disorder.

Congenital heart block not associated with anti-Ro/La antibodies: comparison with anti-Ro/La-positive cases.

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.

Ablate and pace as bail-out therapy in a patient with Fontan correction and malignant atrial tachycardia.

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.

Selective-site pacing in paediatric patients: a new application of the Select Secure system.

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.

Previous palliation in patients with tetralogy of Fallot does not influence the outcome of later repair.

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.

The grown-up congenital cardiac patient.

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.

Clinical, anatomopathological and genetic pattern of 10 patients with cervical aortic arch.

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.

Surgical treatment of arrhythmias related to congenital heart diseases.

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.

Age-related aspects of balloon angioplasty for postsurgical aortic recoarctation.

Balloon angioplasty is now the elective technique for relief of aortic recoarctation, since it has low mortality, low morbidity, and good results at follow-up. Some concern exists concerning the possible increased risk in young children. To investigate such age-related aspects, we examined 58 children undergoing consecutive balloon angioplasty for postsurgical recoarctation. Of the children, 30 were younger and 28 older than 1 year. Recoarctation was more frequent with ventricular septal defect or other cardiac anomalies (p < 0.001). Systolic hypertension was present in 70% of children aged less than 1 year, but in only 32% of those older than 1 year (p < 0.001). The initial gradient was inversely related with the age at dilation (r = -0.28, p < 0.05), and correlated with systolic blood pressure (r = 0.81, p < 0.01). The procedure was successful in 87% of children older than, and 86% of those under 1 year. Age was not related with residual gradient, nor with the percentage increase of diameter of the site of stenosis. After balloon angioplasty, systolic hypertension was still present in 37% of children younger than 1 year, and in 25% of those older than 1 year (p < 0.05). Three complications occurred in children younger than 1 year, and 6 in those older (p < 0.05). Our results show, first, that recoarctation occurs earlier in the setting of complex disease, second that children suffering recoarctation at a younger age are more likely to be hypertensive, either before or after balloon angioplasty, third, that balloon angioplasty has the same rate of success in children below or above the age of one year, although the procedure still carries a not negligible risk. Finally, the procedure does not carry a higher risk for those children below the age of 1 year.