Supraventricular tachycardia in neonates: antiarrhythmic drug choice dilemma.

Supraventricular tachycardia (SVT), being atrioventricular reentry the underlying mechanism, is the most frequently tachyarrhythmia requiring a medical treatment in infants with no cardiac disease. The acute treatment of a single episode of SVT has generally an excellent prognosis. An antiarrhythmic prophylaxis of SVT recurrences is usually recommended during the first year of life. Although many efficient drugs are available for the SVT treatment, a careful risk-benefit analysis of each single case should suggest the correct drug choice.

Counseling and informed consent: the experience in a pediatric cardiology unit. How to communicate a pathological diagnosis.

The aim of our study was to create an operative scheme for conveying a pathological diagnosis with emphasis on communication strategies. In order to check the validity of the scheme, parents of 122 of our patients were contacted by an outside observer and were asked to answer anonymously to a questionnaire concerning their experience in our unit. The questionnaire has been structured in three parts in order to verify (1) the quality of the communication of the diagnosis, (2) the team-patient relationship and (3) the comparison between our unit and the others. 87% of parents declared the language used in the communication clear and comprehensible and the time dedicated to the explanations sufficient (p<.01). They judged the quality of the information received as excellent (50%) or good (50%) (p<.01). Eightyfour % of them thought that the information given was sufficient and there was no need for any more details (p<.01). 92% perceived a comfortable atmosphere, they felt involved in the communication and encouraged to ask questions (p<.01). 91% described the behaviour of the team as helpful and warm without differences between the different figures of the unit (p<.01). In regard to the psychological support 70% of parents found it essential, 30% found it very useful (p<.05). These results show that our operative scheme is functional to the communication and to the team-patient relationship. It permits an "informed consent" as an aware choice of the parents and consequently a better way of management of the congenital heart disease.

Idiopathic infantile arterial calcification: case report.

Idiopathic infantile arterial calcificationI (IIAC) is a rare disorder characterized by calcium deposition in the internal elastica lamina of medium and large arteries and it has been defined in term of molecular genetics. It is usually fatal, approximately 85% of all patients die within the first months of life owing to ischemia of vital organs. Death from myocardial infarction usually occurs in the first 6 months. Calcification in a peripheral artery with electrocardiogram (ECG) changes of occlusive coronary artery disease suggests the diagnosis. It is caused by mutations in the ENPP1 gene localized on chromosome 6q22 and has OMIM number 208000. A case with fatal evolution at the end of the first month is presented.

The usefulness of holding in the management of congenital heart disease.

BACKGROUND: In our Pediatric Cardiology Unit we have implemented a number of specific interventions in order to support parents and their children who have congenital heart disease. We paid particular attention about how communicating the diagnosis and how supporting psychologically the parents. METHODS: In order to check the validity of these interventions we used the State-Trait Anxiety Inventory to analyze and compare the anxiety levels of 380 parents of already established patients here in the hospital to the anxiety levels of 240 parents of children seen in our unit for the first time (for screening or evaluating anomalous symptoms). RESULTS: Our analysis shows that during daily life the seriousness of the disease affects parents adversely (the "anxiety trait" in parents of children with cyanotic congenital heart disease has a higher rate than other parents, p < .05). While waiting for medical assessment, the parents of children with a previous diagnosis of congenital heart disease or those with symptomatic children live an increment of the level of anxiety (the "anxiety state" has a higher score than the "anxiety trait", p < .001), but the "anxiety state" of parents of children with congenital heart disease has a lower rate in respect to parents of symptomatic children (p < .001). CONCLUSIONS: Thus adequate interventions, specifically an effective communication in a comfortable environment, can reduce anxiety of parents while waiting for medical assessment. It is very important as the child's psychological wellbeing is very directly related to the emotional state of the parents.

[Timely detection of premature closure of the ductus arteriosus in a full-term fetus. Important role of fetal echocardiography]. / Tempestivo riconoscimento della chiusura prematura del dotto di Botallo in feto a termine. Ruolo cruciale dell'ecocardiografia fetale.

Patency of the ductus arteriosus (DA) is maintained during gestation by locally produced and circulating prostaglandins (PGE's). As gestation proceeds, the ductus becomes less sensitive to dilating prostaglandins and more sensitive to constricting factors such as PGE's synthetase inhibitors. This case report describes a fetus at term (38 weeks) with signs of severe right ventricular failure due to constriction of DA. Maternal history documented 5 day assumption of a non-steroid antiinflammatory agent to relieve skeletal-muscle pain. Careful echocardiogram ruled out a structural heart disease, such as coarctation of the aorta. A gradient of 41 mmHg across the ductus was recorded. A cesarean section delivery was immediately undertaken. The 3.5 kg newborn delivered appeared to be in good health, with Apgar score of 8/9 at 1 and 5'. There were no signs of congestive heart failure and mild respiratory distress. An echocardiogram showed a dilated, well contractile right ventricle, with a pressure of 50 mmHg. DA was already closed. The fetal echocardiogram was the most relevant investigation in the decision-making process of this case treatment. Any different evaluation of this fetal heart, delaying the delivery would have very seriously compromised the survival of the fetus. Fetal echocardiography is the most important diagnostic tool in the evaluation of the fetal heart; non steroid antiinflammatory drugs to mother at term should be avoided or given with close echocardiographic assessment of DA patency.

From functional pulmonary atresia to right ventricular restriction. Long term follow up of Uhl's anomaly.

We report a 14 year old boy who presented as a neonate with functional pulmonary atresia due to Uhl's disease with emphasis on the later detection of restrictive right ventricular physiology.

[Omniplane transesophageal echocardiography: an improvement in the monitoring of percutaneous pulmonary valvuloplasty]. / L'ecocardiografia transesofagea omniplana: un'evoluzione nel monitoraggio della valvuloplastica polmonare percutanea.

A 16 year old girl underwent percutaneous balloon dilation of the pulmonary valve. The procedure was carried out in the catheterization laboratory under transesophageal echocardiographic guidance with the patient anesthetized (ketamine) without endotracheal intubation. A 64 elements phased array "omniplane" TEE probe connected to a Sonos 1500 Hewlett-Packard system was used. The transducer assembly encased within the tip of the endoscope can be rotated through a 180 degrees arc counterclockwise and clockwise, thus obtaining a multitude of imaging planes. The right ventricular outflow tract and pulmonary valve could be visualized very well and therefore monitored accurately during all phases of the procedure. In the future this technique will be able to reduce the need for X-ray exposure and dye injection during percutaneous pulmonary valvuloplasty especially in cases in which their use could be contraindicated.

[A transesophageal Doppler echo-color study of pulmonary venous flow before and after the correction of valvular defects]. / Studio mediante eco-color Doppler transesofageo del flusso venoso polmonare prima e dopo correzione di vizi valvolari.

AIM OF THE STUDY: To verify changes of pulmonary venous flow pattern before and after surgical or percutaneous correction of valvular heart disease. METHODS: The pulmonary venous flow pattern was studied by transesophageal echocardiography in 27 patients affected with heart valve disease (11 mitral insufficiency, 10 mitral stenosis, 2 aortic stenosis and 4 pulmonary stenosis), before and after surgical or percutaneous correction. Pulmonary venous flow velocity variables measured included peak systolic and diastolic flow velocities (VmaxS and VmaxD), systolic and diastolic velocity time integrals (IS and ID) and their respective ratios (VmaxS/VmaxD and IS/ID). Paired Student's t-test was used for analysis of data; a p value < 0.05 was considered statistically significant. RESULTS: In mitral stenosis and insufficiency, as well as in pulmonary stenosis, the VmaxS/VmaxD and IS/ID ratios were constantly < 1. Aortic stenosis, on the contrary, showed a normal preoperative pattern of pulmonary venous flow, which did not change after correction. All other successful corrections (17 surgeries, 4 angioplasties) were characterised by an increase of VmaxS/VmaxD and IS/ID ratios. (Mitral stenosis: VmaxS/VmaxD 0.80 +/- 0.31 vs 1.4 +/- 0.5, p = 0.006; IS/ID 0.86 +/- 0.77 vs 1.62 +/- 0.62, p = 0.016. Severe mitral insufficiency: VmaxS/VmaxD -0.71 +/- 0.32 vs 1.19 +/- 0.32, p < 0.0001; IS/ID 0.41 +/- 0.19 vs 1.04 +/- 0.31, p = 0.006. Moderate mitral insufficiency: VmaxS/Vmax D 0.38 +/- 0.04 vs 0.95 +/- 0.06, p = 0.001; IS/ID 0.32 +/- 0.05 vs 0.95 +/- 0.07, p = 0.02. Pulmonary stenosis: VmaxS/VmaxD 0.43 +/- 0.23 vs 1.09 +/- 0.35, n.s. e IS/ID 0.49 +/- 0.34 vs 0.92 +/- 0.65, n.s.). Failure to return to a normal pulmonary venous pattern was observed in the 2 cases of partially successful mitral valvuloplasty (one of which was subsequently transformed into a mitral valve replacement with immediate normalisation of the pattern) and in the 2 cases of incomplete relief of a pulmonary stenosis after pulmonary valvuloplasty. CONCLUSIONS: Though preliminary, these observations suggest a high sensitivity of this method and, therefore, a possible role of pulmonary venous pattern studies in the assessment of the efficacy of treatment in mitral and pulmonary valve disease.

Usefulness of transesophageal echocardiography in the pediatric catheterization laboratory.

Transesophageal echocardiography was performed in 51 children (aged 2 to 14 years, mean 4; weight 9 to 50 kg, mean 21) undergoing elective diagnostic or therapeutic cardiac catheterization. The interventional procedures were percutaneous balloon dilation of pulmonary (n = 8) and aortic (n = 2) valve stenosis, percutaneous closure of patent ductus arteriosus (n = 8), and attempted occlusion of Pott's anastomosis by the double umbrella device (n = 1). The diagnostic catheterizations were performed on preoperative children of whom 5 had undergone previous palliative procedures. Precise placement of the balloon across the valve, timing of balloon inflation and deflation according to real-time monitoring of ventricular function and immediate evaluation of results and complications were accomplished with transesophageal monitoring. The exact position of distal and proximal umbrellas of patent ductus occlusive devices was checked on transesophageal imaging and completeness of occlusion controlled on color Doppler. The only relevant information in the preoperative cases was the detection of a septic thrombus in a severely ill patient. With more experience and smaller probes, transesophageal echocardiography may become a new method of monitoring cardiac catheterization also in smaller children where it may reduce duration of the procedure and amount of contrast material.

[Echocardiography-guided endomyocardial biopsy]. / La biopsia endomiocardica ecoguidata.

We have performed 20 echocardiography-guided endomyocardial biopsies in 16 patients, totalling 98 bioptic samples. In each case fluoroscopy was available to supplement the echocardiographic findings. The right ventricle was biopsied in 17 cases, the left ventricle in 3. All 3 left ventricular biopsies and 14 out of 17 right ventricular biopsies were satisfactorily guided by echocardiography. An adequate echocardiographic window was not obtained in 3 cases of right ventricular biopsies and the procedures were carried out under fluoroscopy. In 5 cases (25%), totalling 10 samples, echocardiography showed a somewhat different position of the bioptome from that suggested by fluoroscopy, thus guiding a significant repositioning of it. Finally, in one patient, echocardiography promptly visualized a severe pericardial effusion, due to cardiac perforation, thus allowing its immediate drainage.

[Coronary artery dissection secondary to selective coronary angiography]. / Dissezione delle arterie coronarie secondaria ad angiografia coronarica selettiva.

Coronary artery dissection during coronary arteriography is a rare complication. We describe three cases occurred in our laboratory (0.18% of all the procedures). All the invasive procedures were performed with a femoral approach with Judkins catheters by three different surgeons with limited experience; all dissections involved the right coronary artery. Two patients were men with coronary artery disease and the third was a woman who underwent catheterization for mitral valve disease. An inferior acute myocardial infarction occurred in both men and one of them was surgically treated. No ischemia was observed in the female patient with mitral disease. According to previous reports, we believe that the patients at risk for such a complication (adults with diffuse coronary artery disease or young women with valvular disease and normal coronary arteries) must be more carefully considered. Moreover, in these patients soft-tip Judkins catheters should be used during femoral procedures in the right coronary artery.