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Due to their rare prevalence and marked heterogeneity, pediatric cardiomyopathies (CMPs) are little known and scarcely reported. We report the etiology, clinical profile and outcome of a consecutive cohort of children diagnosed with CMP and followed at Meyer Children's Hospital over a decade.We retrospectively reviewed patients consecutively referred from May 2008 to May 2019 for pediatric onset CMP (18 years). Heart disease caused by arrhythmic disorders, toxic agents, rheumatic conditions and maternal disease were excluded.We enrolled 110 patients (65 males), diagnosed at a median age of 27 [4-134] months; 35% had an infant onset (1 year of age). A positive family history was more often associated with childhood-onset (38.8%). Hypertrophic cardiomyopathy (HCM; 48 patients) was the most frequent phenotype, followed by dilated cardiomyopathy (DCM; 35 patients). While metabolic and idiopathic etiologies were preponderant in infants, metabolic and sarcomeric diseases were most frequent in the childhood-onset group. Major adverse cardiac events (MACE) occurred in 31.8% of patients, including hospitalization for acute heart failure in 25.5% of patients, most commonly due to DCM. Overall, the most severe outcomes were documented in patients with metabolic diseases.In a consecutive cohort of pediatric patients with CMP, those with infantile onset and with a metabolic etiology had the worst prognosis. Overall, MACE occurred in 41% of the entire population, most commonly associated with DCM, inborn errors of metabolism and genetic syndromes. Systematic NGS genetic testing was critical for etiological diagnosis and management.

COVID-19 pandemic continues to strike across the world with increasing number of infected patients, severe morbidity and mortality, social life and economy disruption. Universal access to vaccine prophylaxis will be pivotal in controlling this infection and providing individual level protection. However, mismatch between vaccine request and vaccine availability, as well as constraints in logistics of vaccine campaign is creating a transition phase of progressive but still incomplete inclusion of group of individuals in the vaccination process. Selected patients living with chronic and multisystemic disease may present increased propensity of adverse outcome, should Sars-Cov-2 infection develop. In these patients, expedite access to COVID-19 vaccination may be considered. The Italian Society of Pediatric Cardiology National Board is providing a viewpoint to inform policy makers and public authorities during vaccine allocation strategies development, to consider higher priority and expedite access for selected groups of patients living with congenital heart disease. © 2021, Tech Science Press. All rights reserved.

Despite the recent advances, the clinical approach to persistent pulmonary hypertension of the newborn (PPHN) still represents an important challenge for neonatologists. The care of newborns with PPHN requires meticulous therapeutic and ventilation strategies including, besides the stabilization of the newborn, the use of nitric oxide and high-frequency ventilation. However, not all the neonates with PPHH are responsive to this clinical approach. Recent studies have proposed the use of sildenafil, a phosphodiesterase 5 inhibitor, in refractory forms of PPHN. The aim of this study is to review the cases and the clinical approach of PPHN in the Neonatal Intensive Care Unit of Meyer Children Hospital in the year 2009 and to discuss the possible role of sildenafil in the management of PPHN.

Survival of neonates with critical pulmonary obstruction depends on cardiac morphology and function but also on proper perinatal management.Postnatal outcome of five neonates with critical pulmonary stenosis or pulmonary atresia detected in utero is reported.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.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.

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.

The role of cardiac troponin I (cTnI) is well established in acute myocardial ischemia. However, its role in myocardial contusion remains to be clarified. Since transesophageal echocardiography (TEE) appears, at present, to be the best method for the diagnosis of myocardial contusion, the aim of this study was to measure the concentration of cTnI in patients with blunt chest trauma studied using TEE.Thirty-two patients (27 males, 5 females, mean age 44+/-20 years), admitted to the Trauma Center of our Institution with clinical and/or radiological signs of acute blunt chest trauma, underwent biplane TEE within 24 hours of injury; serial blood samples were taken to measure cTnI levels (normal values0.4 ng/ml), using fluorimetric enzyme immunoassay.Abnormal levels of cTnI were found in 17 patients (53%): 7 patients had levels of cTnI between 0.4 and 1 ng/ml, whereas 10 patients had levels1 ng/ml. Segmental wall motion abnormalities consistent with myocardial contusion could be identified by echocardiography in 6/10 patients with cTnI levels1 ng/ml (60%) but in no patients with normal cTnI levels or with titers between 0.4 and 1 ng/ml; mean cTnI levels showed a significant difference between the two groups of patients with and without echocardiographic signs of myocardial contusion (2.6+/-1.6 vs 0.6+/-1.4 ng/ml, p0.001).Abnormal titers of cTnI suggesting myocardial contusion may be found in more than half of patients with blunt chest trauma; however, myocardial injury can be detected by TEE only for cTnI levels1 ng/ml; cTnI concentrations ranging between 0.4 and 1 ng/ml might be indicative of myocardial microlesions, not detectable by echocardiography, even if TEE is used; cTnI assay could therefore be suggested as a screening test before performing TEE after blunt chest trauma.

In previous studies the reported incidence of cardiovascular events among mitral valve prolapse patients has differed more than 10 fold. We endeavored to determine the relation between the clinical features and mode of ascertainment of mitral valve prolapse and the resulting event rate.Between January 1979 and August 1996, 275 patients (129-47% men, 146-53% women, mean age 43 +/- 19 years), were followed for a mean of 98 months after evaluation in a referral center for valvular heart disease. Comparative data were obtained from a separate, less selected population consisting of 316 patients.A total of 65 events occurred (2.9/100 patient-years): 46 (2.0/100 patient-years) mitral surgery, 12 cardiac deaths (0.5/100 patient-years), 6 neurologic ischemia (0.26/100 patient-years), and 1 infective endocarditis (0.04/100 patient-years). The overall event rate varied significantly according to demographic, clinical and echocardiographic variables (all p0.0001). It was higher among males (odds ratio-OR 2.1), subjectsor = 45 years of age (OR 14.7), those with a holosystolic murmur (OR 25.9), an enlarged left ventricle (OR 13.5) or left atrium (OR 34.9) and those with 3-4+ mitral regurgitation at color Doppler echocardiography (OR 40.0). It was lower in those with an audible mid-systolic click (OR 0.05). These ORs closely resembled those we reported previously in a less selected population. At multivariate analysis, male gender (p = 0.013), severe Doppler mitral regurgitation (p = 0.0048), and left atrial enlargement (p = 0.046) were all independent predictors of events.In a population of mitral valve prolapse patients, including many with significant mitral regurgitation at baseline, we identified similar predictors of events but an overall event rate nearly 3 times higher than that we previously reported for relatively unselected patients or family members in New York City (1/100 patient-years). Therefore, the impact of patient selection on the prevalence of mitral regurgitation, older age and male gender strongly affects the adversity of the "natural history" of mitral valve prolapse.

Despite the recent advances, the clinical approach to persistent pulmonary hypertension of the newborn (PPHN) still represents an important challenge for neonatologists. The care of newborns with PPHN requires meticulous therapeutic and ventilation strategies including, besides the stabilization of the newborn, the use of nitric oxide and high-frequency ventilation. However, not all the neonates with PPHH are responsive to this clinical approach. Recent studies have proposed the use of sildenafil, a phosphodiesterase 5 inhibitor, in refractory forms of PPHN. The aim of this study is to review the cases and the clinical approach of PPHN in the Neonatal Intensive Care Unit of Meyer Children Hospital in the year 2009 and to discuss the possible role of sildenafil in the management of PPHN.

Atrial septal defect (ASD) can be recognized in adult age, mostly in asymptomatic or scarcely symptomatic patients. These patients differ from patients in "historical" clinical series, in whom diagnosis was done on the basis of clinical evidence, and their natural history is probably different.Our aim was to verify retrospectively results of surgery versus medical follow-up in an adult population with ASD with age at first diagnosisor = 30 years.Seventy-two patients with ASD, 52 females (72%), observed at our Institution since 1978, were considered. Mean age at diagnosis was 48 +/- 12 years (range 30-79); 36 patients (50%, group A) are still on medical therapy, 36 patients (group B) were operated. As groups A and B did not differ significantly in any demographic, clinical or echocardiographic parameter, they were compared for the incidence of complications.During follow-up (100 +/- 70 months, range 12-240), the incidence of major clinical events showed no significant differences in the two groups, as cardiac death or cardiovascular complications (cerebral ischemic events, severe mitral insufficiency, reoperation) occurred in 4 patients in group A (11%) and in 4 patients in group B (11%). Worsening of NYHA class was observed in 3 patients from group A (8%) and 2 patients from group B (5.5%; p = ns). New onset of supraventricular arrhythmias occurred more frequently in group B (14 patients, 39%) than in group A (5 patients, 14%) (p = 0.01; OR = 3.9; CI 95%: 1.2-12.6).In an adult population affected with asymptomatic or mildly symptomatic ASD and age at first diagnosisor = 30 years, surgical closure of the defect did not modify morbidity and mortality at a mid-term follow-up. We suggest that, mostly in older asymptomatic patients, surgery should not be a routine choice and clinical decision-making should be individualized in each case.

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 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 patients10 years of age 3/41 (9.8%) were positive and among10 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.

Persistent pulmonary hypertension of the newborn (PPHN) is a rare syndrome with a severe prognosis, in which a prompt diagnosis can be life-saving. The aim of our study was to verify its prevalence in a neonatal population, define clinical and echocardiographic criteria for the diagnosis of PPHN and discuss therapeutic choices.The following clinical and echocardiographic criteria for the diagnosis of PPHN were defined: 1. cyanosis and hypoxemia non-responsive to O2 therapy; 2. right to left shunt at an atrial or ductal level. All neonates fulfilling these criteria referred to the neonatal units of seven pediatric or general hospitals over a two-year period were enrolled.From January 1995 to December 1996, thirty neonates with PPHN (8%) were observed. Birth was pre-term in 5 out of 30. Ten (33%) had experienced chronic and/or fetal asphyxia (FA). Death occurred in 7 (22%), four of whom with FA. Echocardiography showed tricuspid insufficiency in 18 (60%); mean pulmonary systolic pressure was 67 +/- 16 mmHg (range 41-95). In 23 surviving neonates, normalization of clinical and echocardiographic parameters occurred in 8 +/- 5 days.In most neonates, vasodilators (tolazoline, prostacyclin) and/or nitric oxide were employed.PPHN is confirmed to be a rare pathological condition; prognosis is severe, particularly in neonates with FA. Echocardiography is a reliable non-invasive method for a prompt diagnosis and follow-up. Subsequent studies are needed to assess therapeutic choices.

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).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.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.

The aim of this study was to evaluate renal Doppler resistive index in patients with mild to moderate essential hypertension (EH) and to correlate its changes with the presence of left ventricular hypertrophy assessed by echocardiography. Twenty-eight EH patients (19 males, 9 females, mean age 56.2 +/- 8.6 years) and 13 normotensive subjects (7 males, 6 females, mean age 57.6 +/- 7.9 years) were studied; all patients underwent a complete echocardiographic study (M-mode, two-dimensional and Doppler) and a color Doppler echography of renal and intrarenal arteries. After the renal Doppler waveform was obtained, resistive index was calculated by peak systolic velocity (S) and lowest diastolic velocity (D) with the formula S-D/S. EH patients were divided into two subgroups on the basis of left ventricular mass (LVM): Group EH1 with normal LVM (15 patients) and Group EH2 with increased LVM (13 patients). All patients evidenced normal renal morphology and function and received no therapy throughout the entire observation period. Renal resistive index was significantly higher in EH patients than in controls; however, the maximum difference was observed between normotensive subjects and the EH patients with increased LVM (p0.00001). At univariate analysis, significant correlations were found between renal resistive index and age, body mass index, left ventricular relative wall thickness and LVM. However, when multiple regression analysis was used, only age (p0.01) and LVM (p0.05) remained significant predictors of resistive index. In conclusion, our data show that in EH patients resistive index, which is considered an expression of arterial impedance, is well correlated with the presence of left ventricular hypertrophy, presently considered the best index of the severity of hypertensive disease. This correlation may be the expression of the involvement of two target organs in hypertension.

Multiple hepatic hemangioendothelioma are vascular lesions of the liver that generally appear in the infancy with hepatomegaly, high output congestive heart failure and cutaneous hemangiomas. Many plans for management (steroid, radiation, hepatic artery ligation, embolization, cyclophosphamide) have been proposed. We report a case in two months old boy of hepatic hemangioendothelioma with arteriovenous shunts and heart congestive failure, successfully treated with steroid.

Mitral valve prolapse (MVP) is generally regarded as a benign condition, but serious complications (including severe mitral insufficiency, cerebral ischemia, infective endocarditis, complex arrhythmias and sudden death) have been described in a minority of patients and have been correlated to demographic, clinical and echocardiographic characteristics. Both a lack of standardized definition of MVP in earlier studies and the different ways of recruitment of MVP patients may explain the variability in reported complication rates.As an offspring of a larger prospective study this paper focuses on the profile of patients who were found to have MVP by M-Mode and two-dimensional echocardiography in several outpatient hospital departments. A total of 8252 consecutive subjects, examined since March 1990 to February 1991 in the Echo laboratories of the Florence area are considered; according to the presence or absence of structural changes (anterior mitral leaflet thickness5 mm, leaflet redundancy and/or anulus dilatation) two groups of patients with MVP (A and B) were identified.A MVP was diagnosed in 288 subjects (3.5%), 170 females (59%) and 118 males (41%), mean age 41 +/- 18 years (range 7-84). 110 (38%) were in Group A, 178 (62%) in Group B. The following parameters differed significantly in the two groups: age (45 +/- 17 vs 39 +/- 17 years; p0.003); male gender (50% vs 35%; p0.01); auscultatory findings (midsystolic click: 31% vs 68%; p0.00001; holosystolic murmur: 22% vs 3%; p0.00001); left ventricular diameter (53 +/- 7 vs 48 +/- 5 mm; p0.00001) and left atrial diameter (38 +/- 8 vs 33 +/- 5 mm; p0.00001). Among patients with mitral regurgitation detected by Color Doppler Echocardiography 65% were in Group A (p0.00001).These patients with MVP are obviously selected by the modality of recruitment; hence there is a higher prevalence of subjects with morphologic abnormalities and mitral regurgitation who are older and more likely to be male if compared to individuals with MVP who are found in the general population. A long-term follow-up of these patients is ongoing: owing to the data of the literature about prognostic predictors, a higher incidence of complications with a different prognosis between the two groups (with or without structural changes of the mitral valve) is expected.

Congenital arteriovenous malformations represent a rare cause of heart failure in neonates.Four neonates with arteriovenous malformations (cerebral in one, hepatic in two and pulmonary in one) are reported.In the first three cases Color Doppler echography was able to image the arteriovenous malformations, to identify the afferent and efferent vessels and to quantify the arteriovenous shunt. In the neonate with pulmonary malformation, a cardiac cause of heart failure was excluded by echocardiography, and the diagnosis was stated by selective angiography. The neonate with cerebral great arteriovenous malformation died at 4th day of life. The two neonates with hepatic malformations improved both with medical therapy; in the last neonate, with multiple arteriovenous pulmonary fistulae, selective embolization was performed, but the child died a few months later.Congenital arteriovenous malformations are rare, and an early diagnosis is the base for a correct therapy; Color Doppler echography represents the gold standard for cerebral and hepatic malformations, while selective angiography should be reserved to patients who might profit of embolization or surgical resection.

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 term mitral valve prolapse is used to describe either one of the pathophysiological mechanisms of mitral regurgitation or a distinct biological condition with a defined inherited basis, with or without mitral incompetence. In the former case, association with rheumatic fever is implied by the definition itself; in the latter it's questionable (rheumatic fever could act as an environmental factor in the expression of a genetically determined mitral valve prolapse). Three hundred and twenty patients with mitral valve prolapse, diagnosed as a primary one, were studied in order to assess the prevalence of a well-documented history of rheumatic fever: this resulted higher versus a control population (5.6% vs 0.9%-p0.01). The retrospective nature of the study does not allow a definite conclusion. Familial as well as long-term follow-up studies are necessary to better define the association between rheumatic fever and mitral valve prolapse; a potentially different prognosis, mainly accounting to the development of a clinically relevant mitral regurgitation in patients with mitral valve prolapse and previous rheumatic fever, has to be searched.

The aim of this study was to assess the utility of Doppler echocardiography both at rest and during isotonic exercise in evaluating competition eligibility of patients with repaired coarctation of the aorta (CoAo).Seventeen young patients (11 male, 6 female; mean age 17.1 +/- 7.9 years) with previous surgical repair of CoAo were examined. Mean follow-up after repair was 10.3 +/- 3.5 years. All patients underwent complete Echocardiographic examination (M-mode, 2D and Doppler) and an exercise test on an ergometric bicycle, with continuous wave Doppler monitoring of flow velocity in descending aorta, with a transducer positioned in the suprasternal notch. Peak and mean Doppler gradients in descending aorta were measured both at rest and during exercise, using the simplified Bernoulli equation. According to peak Doppler gradient at rest, patients were divided into two subgroups: Group IA = patients with peak gradient lower than 25 mmHg; Group IB = patients with peak gradient greater than 25 mmHg. Finally, 17 healthy subjects (Control Group), matched for age and body surface area, were examined.Systolic and diastolic blood pressure both at rest and during exercise were not significantly different in the 3 groups. Patients of Group IB showed a significant increase of left ventricular mass (124.0 +/- 24.4 vs 85.8 +/- 24.1 g/m2, p0.01), and during exercise, a significant increase of peak gradient (68.3 +/- 27.2 vs 23.5 +/- 9.0 mmHg, p0.0001) and mean gradient (34.8 +/- 11.5 vs 11.9 +/- 5.0 mmHg, p0.0001) at the level of the descending aorta. In patients of Group IA, echocardiographic parameters were not different in comparison with the Control Group, whereas Doppler gradients during exercise were only slightly greater than those observed in the Control Group (peak gradient 36.9 +/- 13.0 vs 23.5 +/- 9.0 mmHg, p0.05; mean gradient 19.6 +/- 6.0 vs 11.9 +/- 5.0 mmHg, p0.05). However, 4 patients of Group IA showed a peak gradient during exercise greater than 40 mmHg (this value was equivalent to the mean value plus 2 Standard Deviations, observed in the Control Group) with the presence of diastolic flow, whereas exercise systolic blood pressure was lower than 200 mmHg.Thus, as a result of this study aimed at evaluating competition eligibility in patients with repaired CoAo, two subgroups of patients have to be distinguished according to Doppler echocardiography results: a) patients with peak Doppler gradient at rest greater than 25 mmHg, for whom competition is forbidden; b) Patients with peak gradient lower than 25 mmHg who must be investigated with exercise Doppler echocardiography to exclude an abnormal increase of Doppler gradients, even if exercise blood pressure is within normal limits.

A multicenter trial was conducted in 267 centers in Italy to evaluate the efficacy, acceptability and safety of a monophasic oral contraceptive containing 30 micrograms ethinyl estradiol and 150 micrograms desogestrel (Marvelon). 13,290 women were followed up for a total of 74,967 cycles. No pregnancies due to method failure were reported and only three because of patient failure. Cycle control was good. There was a decrease in the occurrence of irregular cycles and in the duration and amount of menstrual bleeding; the incidence of breakthrough bleeding and spotting was low. No severe side effects occurred and the incidence of minor complaints was generally lower during treatment than in the pretreatment cycle. Body weight and blood pressure were not significantly altered.

The Doppler characteristics of Björk-Shiley Monostrut prostheses in mitral position were studied in 53 patients (35 women; 18 men; mean age 53.8 yrs). Valvular function was considered normal on the basis of clinical and echocardiographic evaluation. Mean follow-up after surgery was 23.9 +/- 12.0 months (range 9-53). M-mode, two-dimensional and colour flow mapping echo were performed in each patient. Transvalvular blood flow characteristics were examined by colour flow imaging whereas peak and mean gradient through the valve, pressure half-time and prosthetic area were calculated using continuous wave Doppler. In 84% of patients, colour flow mapping showed a transprosthetic flow with 2 jets; in 78% the jets were different: the main one was directed towards the free wall of left ventricle in 52% and towards the interventricular septum in 48%. Thus, the main jet direction was dependent on the spatial position of the prostheses and the orientation of the disc. In 16% a single jet flow was present during the whole diastole. CW Doppler showed the following parameters: peak velocity 1.6 +/- 0.3 m/s; peak gradient 10.7 +/- 3.9 mmHg; mean gradient 3.8 +/- 2.3 mmHg; pressure half-time 83.3 +/- 16.6 msec; prosthetic area 2.7 +/- 0.51 cm2. No statistically significant difference was found between different size prostheses. Our data show the excellent long term hemodynamic parameters of Björk-Shiley Monostrut mitral prosthesis and confirm the value of colour flow mapping in identifying normal transprosthetic flow profile.

A family with 4 cases of pulmonary valvular stenosis with under-developed or normal right ventricle, is reported. The father and his three daughters were affected. In two of the girls, who were twins, a prenatal diagnosis was made using 2D-echo. The importance of genetic counseling and prenatal diagnosis in the families at risk are underlined.

In a series of 75 consecutive patients with transmural acute myocardial infarction (AMI) a right-to-left ventricular filling pressure ratio equal to or greater than 0.65 (RVFP/LVFP greater than or equal to 0.65) was assumed to be indicative of associated right ventricular infarction (RVI). Out of 45 patients with inferoposterior myocardial infarction, 11 (24%) had such hemodynamic evidence of right ventricular infarction (group A). The remaining 34 patients with inferoposterior myocardial infarction (group B) and the 30 patients with anterior myocardial infarction did not. Time-motion and two-dimensional echocardiographic examinations were performed 7-10 days after admission in the 62 patients who survived. Right ventricular wall asynergy was found in six of eight group A patients. In three of these, right ventricular dilatation was also present. No patient in group B with inferior infarction or with anterior infarction showed abnormal right ventricular wall motion. While hemodynamic monitoring seems presently the most specific diagnostic method and it is of invaluable help in the choice of the best pharmacological therapy of right ventricular failure due to RVI, two-dimensional echocardiography is probably highly sensitive and specific for the diagnosis of RVI, by detecting RV wall motion and thickening abnormalities. Due to advantages, such as noninvasivity and repeatibility, two-dimensional echocardiography can be used in the selection of patients who deserve hemodynamic monitoring and in follow-up studies.

In order to study the prevalence of cardiac rhabdomyoma in tuberous sclerosis using non invasive methods, 11 consecutive patients affected by tuberous sclerosis (age: 3 months-22 years, mean 6 years) were examined between January 1984 and April 1987. In each patient clinical examination, 12 lead electrocardiogram, M-mode and two-dimensional echocardiogram were performed: on 7 of them a 24-hour ambulatory electrocardiogram was also performed. Clinical examination revealed a systolic murmur (grade 3/6) in two cases. In one of them it was associated with cyanosis and cardiac failure. None of other patients showed signs or symptoms related to the heart during clinical examination. ECG was abnormal in 3 cases (ventricular pre-excitation in 2 cases and left ventricular strain in 1). Two-dimensional echocardiogram showed single or multiple intracardiac masses suggestive of cardiac rhabdomyoma in 8 cases; in 3 of them masses were isolated, intramural, and in the interventricular septum, from 5 to 16 mm in diameter; in the other 5 cases they were multiple, intramural and endoluminal, in the interventricular septum, in the right or left ventricle, from 7 to 20 mm in diameter. Ambulatory electrocardiogram revealed only isolated ectopic supraventricular and ventricular beats in 2/7 cases. Each patient was clinically controlled every six months using ECG standard and two-dimensional echocardiogram. The mean follow-up period was 32 months (range 9-53). This study confirms the usefulness of the two-dimensional echocardiography to visualize intramural or intracavitary masses due to cardiac rhabdomyoma in patients with tuberous sclerosis, especially when they appear clinically asymptomatic. Two-dimensional echocardiography is probably the ideal method to use in order to evaluate potential increase in cardiac rhabdomyoma, when prolonged follow-up studies are performed.

A study was carried out in 13 patients with stabilized occlusive arterial disease to assess the effect of treatment with creatine phosphate (800 mg/day for 12 days) on walking distance to onset of disabling pain and on haemodynamic parameters (Winsor Index and calf arterial blood flow after ischaemia). Before treatment, patients had daily exercise for 12 days on a treadmill to plateau walking distance increase due to exercise. The results showed a further significant increase in walking distance after treatment, and a significant increase in peak reactive hyperaemia in all but 2 patients. The possible mechanisms of the effects of creatine phosphate are discussed.

A multicenter trial was conducted in 267 centers in Italy to evaluate the efficacy, acceptability and safety of a monophasic oral contraceptive containing 30 micrograms ethinyl estradiol and 150 micrograms desogestrel (Marvelon) 13,290 women were followed up for a total of 74,967 cycles. No pregnancies due to method failure were reported and only three because of patient failure. Cycle control was good. There was a decrease in the occurrence of irregular cycles and in the duration and amount of menstrual bleeding; the incidence of breakthrough bleeding and spotting was low. No severe side-effects occurred and the incidence of minor complaints was generally lower during treatment than in the pre-treatment cycle. Body weight and blood pressure were not significantly altered.

A descriptive epidemiological research, within the Piemonte Region was carried out, to consider whether there is an association between concentrations of nitrates in drinking water and the incidence of gastric carcinomas. The results indicate that an association does exist, but also that concentrations of nitrates higher than 20 mg/l in drinking water are a risk factor for people exposed.

In this paper it has been studied the prevalence of subject positives for the markers of infection of hepatitis B virus in operators of two hospitals. The authors have examined the reports between profession and endemic level of infection in the afferent population.

The biological availability of flufenamic acid after oral administration of the drug in both hard and soft gelatin capsules was studied in dogs and humans. The soft gelatin capsules produced consistently higher plasma concentration-time curves.