Paroxysmal atrioventricular block in young patients.

We describe 17 patients (8 girls, and 9 boys), aged 9.6 +/- 5.7 years, with paroxysmal atrioventricular block (PAVB), a condition rarely described in children. Holter monitoring documented the PAVB in 15 patients, and tilt test was performed in 4 patients (positive in 1). The electrocardiograph (ECG) was normal in 7 patients. Two patients had acquired and 11 patients had congenital heart disease (CHD). Syncope or presyncope were present in 7 patients. A normal ECG was significantly more frequent in symptomatic patients. Pauses were significantly longer in girls and in children <5 years. PAVB was recorded only during nocturnal hours in 6 patients and throughout the day in the others. The sinus rate decreased during PAVB in 6 patients and increased in 4 (generally younger girls with symptoms). Permanent pacemakers were implanted in 13 patients, including 7 asymptomatic patients with CHD and severe bradycardia. During follow-up (3.7 +/- 2.5 years), 1 patient developed complete AVB. Although PAVB was still present in 91% of paced patients, symptoms did not recur because pacing prevented the pauses. In conclusion, PAVB is a rare arrhythmia. Autonomic nervous system dysfunction seems to play an etiological role and permanent pacing was an effective treatment.

Permanent junctional reciprocating tachycardia in infants and children: effectiveness of medical and non-medical treatment.

BACKGROUND: The aim of this study was to identify, in children affected by permanent junctional reciprocating tachycardia (PJRT), the effective treatment. METHODS: Seventeen children (9 males, 8 females, mean age 59 +/- 62 months, median 24) affected by PJRT were referred to our Institute between the years 1987 and 2000. RESULTS: Pharmacological therapy was successfully used in 14 patients: flecainide and propranolol in 5 of them, amiodarone alone in 5 and associated with propranolol in 2, propafenone alone in 1 and in association with sotalol in 1. These drugs were given for a mean period of 54.5 +/- 49.8 months with resolution of the cardiomyopathy in 7/7 patients. Treatment had been continued for 3-6 months and there were no side effects. Nine patients were treated with radiofrequency transcatheter ablation, after 78 +/- 53.5 months of medical treatment, at a mean age of 150 +/- 16 months. The shortest endocardial ventriculo-atrial (VA) interval during tachycardia was recorded in all cases at the coronary sinus ostium (mean value of local VA-surface RP' interval -38 ms, range -24/-55 ms). Successful ablation of the anomalous pathway was obtained at this site in all patients (mean watts delivered 26 +/- 3 W, mean T degrees 64 +/- 5 degrees C). During the follow-up period (mean 21 +/- 17 months) 2 patients with recurrences of PJRT underwent a second successful procedure. CONCLUSIONS: PJRT in pediatric patients can be successfully treated with antiarrhythmic drugs, this may allow delay of the highly effective radiofrequency ablation treatment until the children have reached an adequate growth.

[Left ventricular aneurysm in a patient with mildly dilated cardiomyopathy]. / Aneurisma ventricolare sinistro in un paziente con cardiomiopatia "mildly dilated".

We describe the case of a patient with mildly dilated idiopathic cardiomyopathy and left ventricular aneurysm, diagnosed in absence of a prior clinical history and anatomo-pathological features of myocardial infarction. To ascertain the diagnosis of idiopathic cardiomyopathy, the patient underwent cardiac catheterization with coronary angiography, that showed the lack of epicardial artery stenosis and a slow run-off of the contrast. An endomyocardial biopsy showed the presence of hypertrophic myocytes and interstitial fibrosis. Moreover, a thoracic high resolution computed tomography showed the features of pulmonary bilateral basal emphysema, interstitial thickening and bronchiectasis. Alfa1-anti-trypsin plasma levels were reduced. The patient, because of worsening of clinical and hemodynamic conditions, underwent at age of 36 a combined heart-lung transplantation. The pathological examination of the native organs confirmed the previous diagnosis. At the moment, this is the second report in the literature concerning the presence of left ventricular aneurysm in a patient with idiopathic cardiomyopathy without an underlying coronary artery disease or prior history of myocardial infarction.

[The psychological evaluation of patients with severe heart failure awaiting a heart transplant]. / Valutazione psicologica in pazienti con insufficienza cardiaca grave in attesa di trapianto cardiaco.

Because of the limitations resulting from the severity of heart failure, patients in the waiting list for cardiac transplantation often show a worsening in their social life. They usually present with psychological problems caused by symptoms, medical or surgical therapies and consequences on their own work. We have devised and utilized an experimental psychodiagnostic protocol to study the psychological characteristics of a sample of patients with heart failure (NYHA functional class III and IV) candidate to heart transplantation. Our study was particularly aimed at investigating the quality of life and the psychological mechanisms of adaptation. Findings show a strong attempt of denying the discomfort deriving from the disease and the expectation for transplant. Patients seem to have an adequate reaction to the illness and, otherwise, they feel anxiety and trouble that make very fragile their psychological adaptation. This behaviour is probably correlated with symptoms and the clinical history of heart failure, and does not match with a visible state of well-being that can be detected with a superficial analysis. Nevertheless patients have a good availability in receiving a psychological support from the whole medical staff (physicians, nurses): this suggests that the benefits deriving from a contemporary medical and psychological therapy are able to prevent the appearance of clear psychiatric symptoms. Additional studies involving larger samples of selected populations are needed to confirm the results of the present investigation.