Chronicle of a death foretold. It is time for echocardiographic screening in young athletes

Abstract Background The novel "Chronicle of a death foretold" by Gabriel Garcia Marquez is a story of a sudden death which could have been prevented. In 1976, within the University of Maryland basketball program and only 8 weeks apart, two athletes died suddenly during physical exertion. They were affected by hypertrophic cardiomyopathy and Marfan syndrome and in both cases an echocardiogram would have prevented the tragic epilogue. This coincidence drew everyone's attention and experts' interest on sudden death in sports. Methods and results Even in recent Italian history, unexpected deaths continue to affect athletes but surprisingly any real knowledge regarding the numbers and the impact of those tragedies must take medical literature and non-medical press into consideration. Herein we report the clinical case of a 13-year-old patient with a bicuspid aortic valve, whose mother was alarmed by the news of a young boy who died because of an anomalous origin of coronary artery (AOCA) which had not been diagnosed at transthoracic echocardiography (TTE). Her obstinacy induced the physicians to repeat TTE and led to the same diagnosis in her son: actually, his right coronary artery originated from the opposite sinus of Valsalva. The suspicion was confirmed by coronary CT scan and, thanks to appropriate therapy, the boy now fares well. Conclusions AOCA is the second most common cause of sudden death in young athletes. Although AOCA is often undetectable at ECG, TTE increases sensitivity of preparticipation screening. It could therefore allow us to avoid such coincidences and prevent sudden juvenile death

Delayed onset of tricuspid valve flow in repaired tetralogy of Fallot: an additional mechanism of diastolic dysfunction and interventricular dyssynchrony

<p>Abstract</p> <p>Background</p> <p>Diastolic dysfunction of the right ventricle (RV) is common after repair of tetralogy of Fallot. While restrictive physiology in late diastole has been well known, dysfunction in early diastole has not been described. The present study sought to assess the prevalence and mechanism of early diastolic dysfunction of the RV defined as delayed onset of the tricuspid valve (TV) flow after TOF repair.</p> <p>Methods</p> <p>The study population consisted of 31 children with repaired TOF (mean age ± SD, 12.3 ± 4.1 years) who underwent postoperative cardiovascular magnetic resonance (CMR). The CMR protocol included simultaneous phase-contrast velocity mapping of the atrioventricular valves, which enabled direct comparison of the timing and patterns of tricuspid (TV) and mitral (MV) valve flow. The TV flow was defined to have delayed onset when its onset was > 20 ms later than the onset of the MV flow. The TV and MV flow from 14 normal children was used for comparison. The CMR results were correlated with the findings on echocardiography and electrocardiography.</p> <p>Result</p> <p>Delayed onset of the TV flow was observed in 16/31 patients and in none of the controls. The mean delay time was 64.81 ± 27.07 ms (8.7 ± 3.2% of R-R interval). The delay time correlated with the differences in duration of the TV and MV flow (55.94 ± 32.88 ms) (r = 0.90, <it>p </it>< 0.001). Delayed onset was associated with prolongation of the RV ejection time in 9 and delayed onset and cessation of the pulmonary arterial flow in 4. Delayed onset was not associated with timing changes in the pulmonary artery in 3. The patients with delayed onset showed reduced RV ejection fraction (p = 0.01). However, the two groups did not show significant differences in TV E/A ratio, ventricular end-diastolic volumes, left ventricular ejection fraction, pulmonary regurgitant fraction, heart rate, PR interval and QRS duration.</p> <p>Conclusions</p> <p>Early diastolic dysfunction with delayed onset of TV flow is common after TOF repair, and is associated with reduced RV ejection fraction. It is a further manifestation of interventricular dyssynchrony and represent an additional mechanism of ventricular diastolic dysfunction.</p

The effects of gender on electrical therapies for the heart: physiology, epidemiology, and access to therapies: A report from the XII Congress of the Italian Association on Arrhythmology and Cardiostimulation (AIAC)

The difference between men and women is clear even just by looking at an electrocardiogram: females present higher resting heart rate, a shorter QRS complex length and greater corrected QT interval. The development of these differences from pubertal age onward suggests that sexual hormones play a key role, although their effect is far from being completely understood. Different incidences between sexes have been reported for many arrhythmias, both ventricular and supraventricular, and also for sudden cardiac death. Moreover, arrhythmias are an important issue during pregnancy, both for diagnosis and treatment. Interestingly, cardiovascular structural and electrophysiological remodelling promoted by exercise training enhances this 'gender effect'. Despite all these relevant issues, we lack gender specific recommendations in the current guidelines for electrical therapies for heart rhythm disorders and heart failure. Even more, we continue to see that fewer women are included in clinical trials and are less referred than men for these treatments

ECG pediatrico: dai limiti alla necessità

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Sempre oscura mi fu quest'aVR. L'insospettabile ricchezza di una derivazione dimenticata

No abstract availabl

Sensory phenotype assessment in a young girl affected by congenital insensitivity to pain (CIPA)

Sensory phenotype was assessed in a young girl affected by congenital insensitivity to pain (CIPA) scheduled for an open surgical drainage. The sensory profile showed that only the Abeta fibers were functioning normally, whereas Adelta and C fibers did not respond to nociceptive stimuli. On the basis of these findings and the results of cardiovascular reflexes, she was submitted to abscess incision and debridement under midazolam sedation alone. She did not report pain or other discomfort during surgery. The sensory (and sympathetic) assessment may have a high potential value in planning anesthesia and analgesia in children with CIPA. This psychophysical procedure could be introduced as standard component of clinical evaluation before surgery

Rasmussen's Aneurysm in Childhood: A Case Report

In medical literature, few cases of Rasmussen's aneurysm have been reported until now, and to the best of our knowledge, there has been no description of a case of Rasmussen's aneurysm in childhood. The child described in the case report had a restrictive cardiomyopathy, complicated by pulmonary hypertension, in association with miliary tuberculosi

Valvuloplastica percutanea della stenosi aortica congenita oltre il primo mese di vita: una valida alternativa alla chirurgia [Percutaneous aortic valvuloplasty in congenital aortic valve stenosis performed in patients older than one month: a good alternative to surgery]

Razionale. La valvuloplastica aortica percutanea è una procedura efficace nel trattamento delle stenosi valvolari aortiche congenite. Il nostro studio si è proposto di valutare sia i risultati immediati della procedura sia l’evoluzione nel medio-lungo termine per stabilire l’efficacia del trattamento nell’evitare o nel posticipare una nuova valvuloplastica aortica o la chirurgia valvolare aortica. Materiali e metodi. Lo studio retrospettivo ha valutato 37 pazienti di età >1 mese (età media 6.3 anni) che sono stati sottoposti a valvuloplastica aortica per stenosi aortica severa. Nel 16% dei casi erano presenti difetti cardiaci congeniti associati. Il follow-up medio è stato di 5.07 anni. È stata posta particolare attenzione all’incidenza e alla progressione dell’insufficienza aortica. Risultati. Il gradiente emodinamico dopo valvuloplastica aortica percutanea è sceso da 58.5 a 22.5 mmHg, con decremento percentuale del 61.5%. Ecograficamente il gradiente massimo è passato da 93.0 a 40.5 mmHg, con riduzione percentuale del 56.5%; il gradiente medio è sceso da 52.0 a 20.5 mmHg, con diminuzione percentuale del 60.6%. All’ultimo follow-up il gradiente massimo e quello medio erano rispettivamente 50.0 e 27.0 mmHg. È stato necessario eseguire reintervento nel 21.6% dei casi: 8.1% sono stati sottoposti a seconda valvuloplastica aortica e 13.5% a chirurgia aortica. A 14 anni dalla valvuloplastica aortica la sopravvivenza è stata del 97.2%, la libertà da chirurgia è stata dell’85.5%, quella da valvuloplastica dell’89.5% e la libertà da qualsiasi tipo di procedura è stata del 76.1%. Conclusioni. La valvuloplastica aortica percutanea è un trattamento sicuro ed efficace nelle stenosi aortiche congenite in pazienti >1 mese di vita. L’insufficienza aortica è la principale complicanza nel follow-up. Nonostante ciò, il 75% dei pazienti è libero da qualsiasi tipo di reintervento valvolare aortico a 14 anni dalla valvuloplastica aortica percutanea

Upside-down position for the out of hospital management of children with supraventricular tachycardia

Background The upside-down position is a little known modified Valsalva manoeuvre (VM). The aim of this study was to investigate the safety and the efficacy of the upside-down position for the treatment of paroxysmal SVT in children. Methods Twenty-four paediatric patients followed for SVT were enrolled. The patients were assigned (1:1) to a standard VM or to an upside-down position at the first episode of SVT at home. If no cardioversion occurred, a second attempt was undertaken with the other VM. At the patient's first relapse, the intervention protocol was applied in the opposite order at home. Results The upside-down position compared to standard VM reached 67% vs 33% rate of cardioversion at a first attempt, followed by 50% vs 0% rate of cardioversion in patients who had failed the first attempt. After having reversed the order of intervention in case of SVT recurrence, we recorded 67% vs 25% and 71% vs 42% success rates in favour of the upside-down position. There were no adverse events. Conclusion The upside-down position was safe and tended to be more effective than standard VM for out of hospital SVT treatment. Doctors and parents should be more aware of this effective but overlooked manoeuvre