Pulmonary atresia or critical pulmonary stenosis with intact interventricular septum diagnosed in utero: echocardiographic findings and post-natal outcome.

BACKGROUND: Survival of neonates with critical pulmonary obstruction depends on cardiac morphology and function but also on proper perinatal management. METHODS: Postnatal outcome of five neonates with critical pulmonary stenosis or pulmonary atresia detected in utero is reported. RESULTS: Right ventricular morphology and ratio between tricuspid and mitral annulus at Fetal Echocardiography were the most important prognostic parameters. Increasing severity of obstruction and of right ventricular hypoplasia was documented in two fetuses. CONCLUSION: Prenatal diagnosis was useful for planning delivery; we hypothesize that it can be of benefit on postnatal outcome. In selected cases, earlier delivery and relief of obstruction could have beneficial effect on prognosis.

[Generalized lymphangiomatosis with chylopericardium]. / Linfoangiomatosi generalizzata con chilopericardio.

A case of a child with lymphangiomatosis and chylopericardium is reported. Diagnosis of chylopericardium was performed at 11 months with ascendant lymphography which evidenced thoracic duct agenesia. At the age of 3 years he underwent operation of pleuro pericardial window. He was also treated with low fat medium chain triglyceride diet, and temporary clinical improvement occurred. The patient died at 13 years of age because of extensive lymphangiomatosis to abdomen, lungs and bones.

[Kawasaki disease: clinical characteristics and cardiovascular involvement in a cohort of 121 patients]. / Malattia di Kawasaki: presentazione clinica, evoluzione e interessamento cardiocircolatorio in una coorte di 121 pazienti.

Kawasaki disease is an acute generalized self-limiting vasculitis of small and medium size arteries of unknown ethiology, mostly affecting children younger then 5 years of age. Coronary arteries may be involved with aneurysms lesions, thrombotic occlusion, acute coronary syndrome and sudden death. The mortality rate has been significantly reduced by combined therapy of intravenous immunoglobuline (IVIG) and acetil salicylic acid (ASA) from 3% to 0.2%. From February 1986 to November 2001, 121 children, 76 male and 45 females with Kawasaki disease, have been observed at the Paediatric Department, University of Florence. Coronary changes (i.e. coronary ectasia and coronary aneurysms) have been observed in 22.3%. In only one 3-months old child, giant aneurysms of coronary arteries and myocardial necrosis have been detected. In 26 of 27 children at 2D echo a remodelling of the coronary changes within 12 months from the onset of the illness was observed. The mean follow-up has been five years (range 6-138 months) and in no patient further cardiac manifestations have been observed; however, as little is known about the progression of vascular damage, a more protracted follow-up is needed.

[An echocardiographic study of the left ventricular functional indices in pediatric patients on hemodialysis and in treatment with recombinant human erythropoietin (r-HuEPO)]. / Studio ecocardiografico degli indici di funzione ventricolare sinistra in pazienti pediatrici emodializzati e in trattamento con eritropoietina umana ricombinante (r-HuEPO).

Recombinant human erythropoietin (r-HuEPO) is efficient in the treatment of anaemia in chronic renal failure on hemodialysis. We investigated the changes in cardiac function under r-HuEPO therapy using echocardiography. Seven patients with severe renal anaemia (Ht 19%) were treated with 50 U/kg r-HuEPO EV three times/week. Echocardiographic studies were performed in the anemic state and when hematocrit values were stable at levels (Ht 30%). Left ventricular function as showed by fractional shortening (D%) improved, the thickness of the interventricular septum and left ventricular hypertrophy were reduced. Our data indicate that the correction of renal anaemia by r-HuEPO can improve myocardial function in patients on maintenance hemodialysis.

[The usefulness of Doppler echocardiography in the diagnosis of neonatal Marfan's syndrome. A clinical case report]. / Utilità dell'ecocardiografia Doppler nella diagnosi di sindrome di Marfan neonatale. Descrizione di un caso clinico.

Marfan syndrome is very rarely diagnosed in the neonate, and specific pathological and clinical findings are described in this age; cardiac involvement occurs very often, carrying severe prognosis in most patients. In the presented case the diagnosis was suspected according to clinical findings; bidimensional echocardiography showed that the most important lesion was tricuspid dysplasia and prolapse; Doppler echocardiography has contributed to explain the clinical severity, showing regurgitation of all cardiac valves and a pattern of functional tricuspid atresia. Autoptic examination has later confirmed all these findings.

[Long-term follow-up on ventricular pre-excitation in pediatric age]. / Follow-up a lungo termine della preeccitazione ventricolare in età pediatrica.

AIM: Little is known yet about the clinical aspects of ventricular pre-excitation in pediatric age. In order to define clinical course, assess the risk of malignant arrhythmia and evaluate indications for ablative therapy, we studied 82 consecutive pediatric patients (pts) with this affection (61 males-21 females; mean age at diagnosis 5.1 years, range 1 day-15 years). RESULTS: Mean follow-up time was 66 months (range 3-252). Fifty-six pts (68.3%) were asymptomatic. One patient, who was affected with hypertrophic cardiomyopathy and died suddenly when she was 2 while her cardiac rhythm was being monitored, had an EKG pattern of asystolia. In 28 pts (19 m - 9 f, 34.1%), episodes of reentrant atrio-ventricular tachycardia, ranging from 160 to 320 beats/minute, were documented. Five pts, four of whom were less than 1 year old, had syncope or cardiac failure due to tachyarrhythmia. As far as maximum incidence of symptoms is concerned, two peaks were observed: within the first year and between 14 and 16 years of age. A transesophageal electrophysiologic study was performed in 22 pts, tachyarrhythmias were induced in 15, and risk criteria were satisfied in 3/22 (13.6%). Five pts underwent catheter ablation of an accessory pathway and this was successful in three of them. CONCLUSIONS: The clinical course in this series of patients has been benign. Nevertheless, during the first year of life cardiac failure or syncope may occur because of high heart rates. The transesophageal electrophysiologic study, which is also easy to perform in pediatric patients, has been sensitive in assessing the risk of malignant arrhythmia, but it shows low predictive accuracy. Lastly, ablative therapy has shown lower efficacy in children than in adult patients.

[Verapamil-responsive ventricular tachycardia in small children: a case report and review of the literature]. / Tachicardia ventricolare verapamil-sensibile nel piccolo bambino: descrizione di un caso e revisione della letteratura.

A case of verapamil-responsive incessant ventricular tachycardia in a 4-year-old girl is reported. Oral verapamil alone failed in maintaining stable sinus rhythm. With association of oral verapamil and betablockers (nadolol) the patient remained asymptomatic without recurrence of ventricular tachycardia over a follow-up of 1 year. In case of failure of monotherapy with verapamil or betablockers alone, an association of both can be effective and safe also in pediatric age. Radiofrequency catheter ablation should be reserved, especially in pediatric age, only to patients with impaired ventricular function who are not responsive to medical therapy.

[Syncope at pediatric ages: evaluation with head-up tilt]. / La sincope in etá pediatrica: valutazione mediante head-up tilt test.

Syncope is a common medical problem with multiple potential causes and it is very frequent in pediatric population . Neurocardiogenic syncope has been increasingly recognized with the introduction of head-up tilt test (HUTT). The study investigates the clinical utility of HUTT in the evaluation and management of children with recurrent syncope and structurally normal heart. Two-hundred-forty-three consecutive young patients with recurrent unexplained syncope, 100 males and 143 females (mean age 11.4 years, range 5 to 20) underwent HUTT using a 60 degree tilt for 45 min. The test was considered positive when it provoked symptoms of syncope with hypotension and/or bradycardia. Twenty-six patients (10.7%) were positive for neurocardiogenic syncope. Of the 26 patients with the positive tilt, 5 (19.2%) had cardioinhibitory response, 5 (19.2%) mixed response and 16(61.6%) vasodepressive response. Nineteen of 143 females (13.3%) and 7 of 100 males (7%) resulted positive (NS). Among patients < 10 years of age 3/41 (9.8%) were positive and among > 10 years 22/202 patients (10.9%) resulted positive (NS). The cardioinhibitory response is more frequent in males (p = 0.01), and the vasodepressive in females (p = 0.05). In our study, concerning a non selected pediatric population a positive test resulted in a lower percentage than previously reported; moreover, the tilt test has appeared a promising method of identifying patients requiring pharmacotherapy. Additional randomized controlled studies are necessary to better define the prognosis and treatment of neurocardiogenic syncope in children and adolescents with positive tilt test. Finally, an assessment of the outcome of young patients with syncope and a negative tilt test is needed.