Congenital heart defects: 15 years of experience of the Emilia-Romagna Registry (Italy).

OBJECTIVES: Collection and assessment of data from the Emilia-Romagna Region on the occurrence of congenital heart defects in order to identify an homogeneous group of patients for further aetiologic and genetic studies. MATERIALS AND METHODS: The present study is based on 1549 stillborn and live born babies affected by congenital heart defect out of 330,017 consecutive births (4.7 per 1000). RESULTS: The frequency and type of congenital heart defects have been identified together with the sex ratio, associated extracardiac anomalies, chromosomal anomalies and the risk of precurrence in relatives. The impact of prenatal diagnosis on prevalence was low during the study period. CONCLUSIONS: The study has provided epidemiological data for public health surveillance of congenital heart defects in the Emilia-Romagna region. The creation of a system for the nationwide recording of congenital heart defects designed with regard to the sources of ascertainment, the diagnostic criteria, and the system of classification is emphasised.

The Italian Multicentric Study on Epidemiology of Congenital Heart Disease: first step of the analysis. Working Party of the Italian Society of Pediatric Cardiology.

We present the aims, methodology and initial results from the Italian Multicentric Study for the registration and follow-up of congenital heart disease. The general aims are to measure the prevalence of congenital heart disease in different geographic areas of Italy, and to assess the survival and outcome of affected babies. During the years 1992 and 1993, eighteen centers for Pediatric Cardiology spread all over the Country enrolled 1445 new babies with congenital cardiac malformations from a population of 341,647 surveyed livebirths. The new cases were registered using the same methodologic criteria of the EUROCAT study in order to evaluate differences and/or similarities between the studies. The prevalence varied between 1.8% and 8.1%; the average being 4.6%. The large range in prevalence is presumed to be related to different customs and hierarchies in flow and referral of patients. We provide total prevalence of individual lesions, and distribution of sentinel cardiac anomalies, in the Italian study and compare them with EUROCAT. Isolated ventricular septal defect is the most common lesion (39%); followed by atrial septal defect (7.5%); pulmonary valvar stenosis (7.3%); atrioventricular septal defects (5.4%); patency of the arterial duct (3.8%); complete transposition (3.7%); tetralogy of Fallot (3.3%); aortic coarctation (2.4%); aortic valvar stenosis (2.2%); and left heart hypoplasia (1.8%). The echographic stratification of ventricular and atrial septal defects, by location and size, was in keeping with the findings of the EUROCAT study. Because of the recent widespread availability of color-Doppler techniques, the stratification of aortic and pulmonary valvar stenosis was an innovative approach in our study. Among the complex cardiovascular anomalies, double inlet ventricle and pulmonary atresia had a proportion of about 2% each; with double outlet right ventricle, common arterial trunk, Ebstein's malformation, tricuspid atresia, interrupted aortic arch and totally anomalous pulmonary venous connection having a proportion ranging from 0.5 to 0.8%. We discuss clinical features, such as frequency of extracardiac anomalies and familial aggregation of congenital heart disease, in comparison with the EUROCAT data.

[The prevention of DVT after general surgical interventions for cancer pathology]. / Profilassi delle TVP dopo interventi di chirurgia generale per patologie oncologiche.

The efficacy of postoperative DVT prophylaxis of defibrotide and heparin calcium was evaluated in a group of 47 patients undergoing general surgery for oncological pathologies. A sub-group (24 patients) received defibrotide treatment, 400 mg b.i.d. i.m.; the second subgroup (23 patients) was treated with heparin calcium, 5000 IU b.i.d. s.c., from the day before operation until the 7th postoperative day. No postoperative thrombotic symptoms were observed in either group of patients.

Doppler and 2D echocardiographic diagnosis of congenital coronary artery fistulae to the right cardiac chambers: report of 3 cases.

We present three cases of coronary artery fistulae to the right cardiac chambers. The first was a 2-day-old neonate in congestive heart failure: 2D and Doppler echocardiography revealed a dilated proximal left coronary artery and a fistulous connection to the right atrium. The other two patients, respectively 4 and 3 years old, were asymptomatic and presented with a continuous heart murmur: a left coronary artery fistula into the right ventricle was detected by ultrasound in one, and a dilated proximal right coronary artery in the other. The diagnosis was confirmed in all three patients. The first patient was operated upon at 18 months of age; the second patient is awaiting surgery, and in the third patient the fistula was ligated at the age of 3 years. The possibility of ultrasound diagnosis without invasive procedures is suggested.

Echocardiographic assessment of radiation-induced mitral valve disease in a child.

A young girl, previously treated with mediastinum irradiation because of a pulmonary fibrosarcoma, developed mitral valve disease with valvular insufficiency. In consideration of the clinical course and the echocardiographic findings, authors hypothesize a relation between the radiation therapy and the valvular damage.

[Treatment with indobufen of platelet hyperaggregation in a group of patients with vascular diseases]. / Il trattamento con indobufene dell'iperaggregazione piastrinica in un gruppo di pazienti affetti da patologie vascolari.

Twenty-five patients with enhanced spontaneous platelet aggregation and impaired arterial or venous circulation syndromes have been treated by indobufen. Each of them received, by oral administration, 200 mg of the drug twice a day for periods of time from fifty one to hundred eleven days. This treatment normalized, in all the cases, the platelet aggregation. In eight patients of the group, three hours after the last drug administration, the average of the maximum platelet aggregation curves deflections, induced by ADP, epinephrine, ristocetin and collagen, resulted significantly reduced, while, in seven other patients, twelve hours after the last drug administration, only the aggregation curves induced by ADP and epinephrine resulted significantly inhibited. Most on the patients with arterial impairment syndromes noticed some improvements of their symptoms. Also the patients with light subjective post-phlebitic symptoms declared to feel better. The drug has been well tolerated. Therefore, its use is recommendable whenever it is advisable to get a full antiplatelet effect whose the prompt reversion might be required.

Behaviorally augmented versus other components in organophosphate tolerance: the role of reinforcement and response factors.

Male Wistar-derived rats were used to assess the behaviorally augmented component of tolerance to paraoxon depression of a feeding response. Separate groups of animals were treated daily by 0.125 mg/kg of the compound given sc either 1 hr before the start or 45 min after the end of a 90-min feeding session. However, the dose was reduced to 0.0625 mg/kg from Day 9 to Day 12 of the treatment series if animals showed too severe a reduction in food consumption. After development of tolerance by the presession treatment group, the animals treated after feeding were shifted to treatment before feeding. This shift produced a marked depression in food consumption. This confirms similar data previously obtained by a different test (two-way avoidance), and indicates that behaviorally augmented tolerance to paraoxon related to practice factors may be a fairly general phenomenon. Other experiments were to assess the effects of paraoxon in the conditioned taste aversion (CTA) paradigm. These showed the development of an aversion only at high dosage levels (two pairings between the flavor cue and 0.25 mg/kg sc, or four pairings with a 0.17-mg/kg dose). However, the failure of lower doses to produce CTA may have depended on the relatively slow onset of the intoxication, producing an extended interval between the end of cue exposure and the development of malaise or illness. Two pretreatments given 6 and 3 days before the first conditioning session in an experiment using the 2 X 0.25-mg/kg schedule did not affect the development of CTA as measured by a conventional double-bottle test. However, a typical interference effect produced by prior exposure was shown by a substantial acceleration of subsequent CTA extinction in pretreated animals.

Estimate of the daily urinary output based on creatininuria values.

The daily urinary output was calculated on the basis of creatininuria, starting from an accepted formula that evaluates ClCr, employing the subject's height and creatininemia. Results are satisfactory. The new formula estimates the daily urinary volume on the basis of the mean of the three creatininuria values of the day.

[Action of the basic trypsin inhibitor on lymphocyte blastic transformation in vitro]. / Azione dell'inibitore basico della tripsina (bpti) sulla blastizzazione linfocitaria in vitro.

The effect of Basic-trypsin-inhibitor (BPTI) on the in vitro PHA-induced blastic transformation of lymphocytes is reported. Cultures of lymphocytes from healthy subjects were added with PHA, BPTI and PHA + BPTI; simple control-cultures were also performed. The blastic transformation was evaluated by morphologic method after 72 h incubation at 37 degrees C. The results are compared with Wilcoxon's test; it was observed that BPTI reduces the PHA-induced blastic transformation, but it has no effect on cultures without PHA. The hypothesis that BPTI inhibits only PHA-stimulated lymphocytes is suggested.

[Contribution to the study of some plasma protein clearances in children]. / Contributo alla conoscenza della clearance di alcune plasmaproteine in eta' pediatrica.

Renal clearances of transeferrin, IgG and C'3 were determined by the immunodiffusion method in 51 healthy children. Statistical elaboration shows the great dispersion around the mean values. The results of other Authors in healthy adults are confirmed.