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ECOCARDIOGRAFIA
IMPACT OF HETEROZYGOUS FAMILIAL HYPERCHOLESTEROLEMIA ON LEFT VENTRICULAR
MORPHOLOGY AND FUNCTIONS IN PEDRIATIC POPULATION: A SPECKLE TRACKING STUDY
A. D'Aiel o 1, G. Di Salvo 2, A. Rea 1, R. Sorrentino 1, I.J. Romano 1, M. Carrozza 1, C. Iacono 1, G. Santoro 1,M.G. Russo 11 Seconda Università degli Studi di Napoli, Napoli,ITALY, 2 King Faisal Specialist Hospital, Riyadh, SAUDIARABIA Background. So far there are few data on cardiac morphology and function in familial hypercholesterolemia (FH)children. Bidimensional strain (S) imaging, has been added to our capabilities and has been proposed as strongindex of myocardial contractility. FH children represent a unique clinical model to assess the effect of the purehypercholesterolemia on cardiac morphology and functions, excluding the effect of co-morbidities. Aim. Thus, wesought to define in FH children the preclinical effects of isolated hypercholesterolemia on the cardiovascularsystem, by examining left ventricular (LV) function using the more sensitive bidimensional-derived S imaging.
Methods. In al we studied 90 children (45 FH children and 45 controls, mean age 11±3 years). Results. FHchildren showed thicker LV wal s and significantly increased LV mass indexed for height 2.7 (p=0.0008) and forbody surface area (p<0.0001). LV ejection fraction was similar between groups. The diastolic evaluationdemonstrated a prolonged deceleration time(p<0.0001), a reduced early diastolic mitral annular velocity(p<0.0001), and higher transmitral early early diastolic mitral annular velocity ratio (p=0.0003) in FH children.
Longitudinal and circumferential myocardial deformations of the LV were significantly reduced in FH children(p<0.0001). Radial deformation was increased in FH children (p=0.039). Conclusions. This study demonstratedthat already during childhood hypercholesterolemia is responsible of significant LV morphological and functionaldifferences when compared to healthy children. Of interest, a decreased longitudinal and circumferentialdeformations are compensated by increased radial strain in FH children with normal LV ejection fraction. Ourdata could support the need to initiate cholesterol lowering treatment at a young age in children with FH.
EFFECTS OF ARIPIPRAZOLE AND RISPERIDONE ON VENTRICULAR REPOLARIZATION IN CHILDREN
AND ADOLESCENTS
C. Privitera 1, G. D'amico 1, F.L. De Luca 1, L. Bruno 1, L. Oreto 1, E. Germano' 2, D. Italiano 3, M. Lamberti 2, L.
Guerriero 2, E. Spina 3, A. Gagliano 2, M.P. Calabro' 11 University of Messina, Department of Pediatrics, Division of Pediatric Cardiology, Messina,2 Department ofPediatrics, Division of Child Neurology and Psychiatry, Messina,3 Department of Clinical and ExperimentalMedicine, Messina, ITALY Background. Atypical antipsychotics (AP) are increasingly being used in children and adolescents for thetreatment of psychiatric disorders such as psychoses, autism, attention deficit and hyperactivity disorder (ADHD)and aggressive behavior. Atypical AP may cause QT prolongation on the electrocardiogram (ECG), whichpredisposes patients to an increased risk of developing threatening ventricular arrhythmias. Although thisphenomenon has been exhaustively reported in adults, few studies investigated the safety of these drugs inpediatric patients. We performed an open-label, prospective study to assess the arrhythmic risk of aripiprazoleand risperidone in pediatrics. Methods. Subjects consecutively admitted to the Unit of Child Neurology and Psychiatry of the University ofMessina, between January 2009 and December 2012, and starting a treatment of aripiprazole or risperidone,were considered for the study. None of the included patients had a history, signs, or symptoms of cardiovascular,pulmonary or endocrine disorders. A total of 60 patients (55M/5F, mean age 10,2 + 2,6 years, range 4–15 years),receiving a new prescription of aripiprazole or risperidone in monotherapy underwent a standard ECG beforeand after two months from the beginning of antipsychotic treatment. Twenty-nine patients were treated witharipiprazole (mean dosage 7.4+3,1 mg/day) and 31 with risperidone (mean dosage 1,5 + 1 mg/day). A standard12-lead ECG was obtained for each patient with the same instrument (Cardioline delta 3 plus). The duration ofQT intervals was measured manual y in al leads in which the onset of the QRS complex and the return of Twave to baseline were clearly identified. The QT duration was corrected for heart rate according to the Bazett’sformula to produce QTc and averaged for al assessed leads (mean QTc). If respiratory sinus arrhytmia waspresent the QT interval was measured in al leads where RR intervals were almost equal. Heart rate, RR, PRand QRS intervals were also measured. QTd was calculated as the difference between the longest and shortestindividual lead QTc. QTc intervals> 450 msec or 60 ms longer then at baseline, and QTd> 100 ms wereconsidered abnormal. Basal and post-treatment ECG parameters, including QTc and QTd interval duration, werecompared within treatment groups. Mann–Whitney U-test was used for unpaired group comparison andWilcoxon paired rank test was used for comparisons across time. Two sided tests were used and statisticalsignificance was accepted at a p level <0.05.
Results. Although none of the patients exhibited pathological values of QTc or QTd, treatment with risperidonewas associated with a significant increase of both QTc and QTd values (407,4 + 11,9 ms vs 412,4 + 10,3 ms,p<0.05; and 40,0 + 4,4 ms vs 44,7 + 5,5 ms, p<0.01, respectively). Conversely, treatment with aripiprazole wasassociated with a statistical y significant increase of QTd, (40,6 + 6,5 ms vs 46,3 + 7,2 ms, p<0.01) in presenceof an unmodified QTc. Moreover in patients treated with aripiprazole a significant decrease in heart rate wasobserved after treatment (90,0 + 21,2 bpm vs 79,8+19,3 bpm; p<0.01). Conclusions. This study underlines the relative cardiac safety of aripiprazole and risperidone in childhood, evenif these drugs may exert an effect on ventricular repolarization. However, a particular caution in prescribing thesedrugs should be exercised by physicians in patients with a genetic predisposition to arrhythmias. It might beuseful to execute an ECG examination in al patients undergoing AP therapy. Additional y, both QTc and QTdshould be measured in order to warranty a reliable assessment of drug-induced QT prolongation.
RHABDOMYOMAS AND TUBEROUS SCLEROSIS: OUR EXPERIENCE IN 27 CASES
V. Giacchi 1, C. Grasso 1, C. Mattia 2, G. Schiavone Giacco 1, P. Smilari 1, F. Greco 1, P. Sciacca 31 Clinica Pediatrica, Dipartimento Materno - Infantile, Università di Catania, Catania, 2 UTIN, Neonatologia,Dipartimento Materno - Infantile, Università di Catania, Catania, 3 Ambulatorio Cardiovascolare Neonatale ePediatrico Dipartimento Materno-Infantile, Università di Catania, Catania, ITALY IntroductionRhabdomyomas are the most frequent cardiac tumors in children and are often associated to tuberous sclerosiscomplex (TSC) (1). This is an autosomal dominant neurocutaneous disorder that can affect most commonly thebrain, kidneys, heart, and lungs (2). We report our experience with regard to 27 patients with multiple cardiacmasses.
Patients and MethodsFrom January 1989 to December 2012 we diagnosed Rhabdomyomas in 27 babies. In six cases (22 %) themasses were detected antenatal y, in seven cases (26 %) during first three days of life, in others seven cases(26 %) the patients were less than two months old and the last seven children (26 %) were subjected toechocardiography between third and twelfth months of life. In 17 patients (62%) we detected tuberous sclerosisconfirmed by genetic test for TSC. Postnatal y (78 %) the indication for echocardiography was in 2 cases (7,5 %)an arrhythmia, in 12 cases (44,5 %) the presence of heart murmur, in 3 cases (11 %) the appearance of seizureslike infantile spasms, whereas in 4 cases (15 %) the echocardiografic examination had been performed as ascreening. In 24 (89 %) patients we detected multiple mass that led us to define these masses asrhabdomyomas. In three patients al with TSC (11 %) we observed only a single cardiac mass. Subsequently tothe diagnosis, al babies were subjected to EEG and MRI of brain. The family history was investigated. Al babieswere monitored through the execution of ECG and echocardiography-colordoppler every six months and ofHolter-ECG every twelve months. Results and ConsiderationsOnly in 3 babies (11 %) the mass localized in right or left ventricular outflow tract caused a significantobstruction. One newborn (4 %) with diagnosis of cardiac mass in fetal period, as she showed signs of heartfailure and cyanosis, at birth was subjected to surgery to remove the mass. In 1 (4 %) patient echocardiographyexamination revealed only slight hypokinesia without need medical of surgical therapy. Whereas one (4 %)antenatal y diagnosed died soon after birth because of severe hypokinesia The other patients (77 %) weretotal y asymptomatic. In 6 cases (22 %) we found atrial and/or ventricular ectopic beats and in 2 (7,5 %) of thesecases it was revealed random and constituted an indication to echocardiography. For al patients we have notconsidered necessary a drug treatment and the arrhythmia is spontaneously total y disappeared or has beengradual y reduced. Only in 1 (4 %) Wolf-Parkinson-White syndrome was diagnosed. Familial history of diseaseresulted positive in six cases (22 %). With regarding to peculiar skin features in neonatal age, 9 (33 %) showedhypomelanotic macules. Al infants with MRI tipical STC 17 (62%) lesions developed seizures during fol ow-up and we could not rule outalso the other patients will develop subsequently. During fol ow-up we observed in al 26 survived a reduction of rhabdomyomas in terms of both number and size.
Despite the potential y favorable cardiac evolution of patients with cardiac rhabdomyomas, their presencesuggest a tuberous sclerosis with a neurological prognosis that is not related to the number or the dimensions ofrhabdomyomas. In our series only patients with severe symptoms at diagnosis had a poorer cardiac prognosis.
Benyounes N et al. Cardiac Rhabdomyomas in Tuberous Sclerosis patients: a case report and review of literature. Archives of Cardiovascular Disease 2012; 105:442-445.
2) Adriaensen et al. Echocardiographic screening results in patients with Tuberous Sclerosis Complex.
Clinical Investigation 2010; 37:280-283.
VALVOLA AORTICA BICUSPIDE E PERSISTENZA DELLA VENA CAVA SUPERIORE SINISTRA: UNA
DIAGNOSI ELETTROCARDIOGRAFICA?
M. Urru 1, A.B. Cutri' 2, G. Bronzetti 3, G. Di Fazzio 4, M. Brighenti 3, A. Balducci 3, G.D. Gargiulo 3, M. Bonvicini 31 Struttura Complessa di Cardiologia Pediatrica Azienda Ospedaliera G. Brotzu, Cagliari,2 Centro CardiologicoPediatrico Mediterraneo IRCCS OPBG presso P.O. San Vincenzo, Taormina,3 Unità Operativa di Cardiologia eCardiochirurgia Pediatrica e del 'Età Evolutiva, Policlinico Sant Orsola-Malpighi, Bologna,4 Pediatria, OspedaleSanta Maria Nuova, Reggio Emilia, ITALY PREMESSE E SCOPI La vena cava superiore sinistra persistente (VCSSP) si può ritrovare in circa lo 0,4% del e persone. Spesso èisolata ma in circa il 10% dei casi può associarsi ad anomalie congenite come canale atrioventricolare, tetralogiadi Fal ot, DIA, DIV, ritorno venoso anomalo polmonare, coartazione aortica e valvola aortica bicuspide (VAB).
Inoltre, la VCSSP può essere associata ad anomalie strutturali del tessuto di eccito-conduzione con relativealterazioni del ritmo tra cui ritmo del seno coronarico, sindrome di Wolff-Parkinson-White e malattia del nodo delseno. I disturbi del ritmo sono legati anche al a dilatazione del seno coronarico, che determina uno stiramentodel miocardio atriale e del tessuto di conduzione. La VCSSP è di solito l’unica vena cava superiore (assenza divena anonima) ma talora è associata a una vena cava superiore destra, con cui comunica tramite una venaanonima ipoplasica. La VCSSP drena quasi sempre nel ’atrio destro tramite un seno coronarico (SC), dilatatoper il flusso indebito; solo raramente drena nel ’atrio sinistro a causa di un SC unroofed. Scopo del lavoro è statovalutare l’associazione tra la VCSSP, cardiopatie congenite e anomalie del ritmo.
MATERIALI E METODISi sono presi in esame 712 pazienti visionati da un singolo operatore presso l’Ambulatorio diCardiologia-Cardiochirurgia Pediatrica di Bologna nel periodo tra ottobre 2011 e dicembre 2012, sottoposti adesame ecocardiografico ed elettrocardiogramma. Si sono quindi estratti quel i con VCSSP: tutti questi pazienticosì selezionati erano giunti per screening o fol ow-up di cardiopatie congenite, tranne uno inviato dopo riscontrodi ritmo atriale ectopico. RISULTATISi sono individuati 17 pazienti con VCSSP (10 maschi, 7 femmine), di età compresa tra 1 e 30 anni (età media 7anni). L’anomalia vascolare era così associata: 1 pz con VAB, coartazione aortica e ritmo del seno coronarico; 2pz con VAB e ritmo del seno coronarico; 1 pz con VAB e coartazione in sindrome di Turner; 2 pz con VAB ecoartazione aortica; 1 pz con VAB, coartazione aortica e anomalia coronarica; 1 pz con VAB, interruzionedel ’arco e DIV subaortico; 1 pz con VAB, DIV muscolari e displasia polmonare; 1 pz con valvola di Eustachiogigante; 1 pz con DIV subaortico; 1 pz con DIA ostium II; 1 pz con tetralogia di Fal ot; 4 VCSSP isolate.
CONCLUSIONI In questo studio si riscontra una frequente associazione tra persistenza del a vena cava superiore sinistra e VAB(53%); inoltre, il 33 % dei pazienti con bicuspidia aortica presenta un ritmo atriale ectopico (del seno coronarico).
Nonostante il bias di selezione questi dati sono interessanti. La valvola aortica bicuspide è un’anomalia non rara(prevalenza 1%), e non sempre minore. Conoscerne l’esistenza è importante, ma esistono forme che possonoeludere un primo ecocardiogramma; quindi un seno coronarico dilatato o la visualizzazione diretta di unaVCSSP possono richiamare maggiore attenzione sul ’anatomia valvolare aortica e sul e altre condizionipotenzialmente associate. Il ritmo atriale basso, o del seno coronarico, viene solitamente considerato benigno efisiologico. In questa casistica tale ritmo presenta una considerevole associazione con VCSSP e valvola aorticabicuspide. Il dato non appare casuale ed è comprensibile al a luce degli aspetti anatomici sopraddetti. Si puòdunque affermare che il riscontro di un ritmo atriale basso debba essere seguito da un ecocardiogramma.
DETECTION OF LEFT VENTRICULAR DYSFUNCTION BY TISSUE DOPPLER IN ASYMPTOMATIC
PATIENTS WITH HISTORY OF SURGERY FOR CONGENITAL HEART DISEASE
V. Fibbi 1, P. Bal o 2, G.B. Calabri 1, S. Favil i 1, G. Spaziani 1, A. Zuppiroli 2, E. Chiappa 11 Azienda Ospedaliera Universitaria A. Meyer, Firenze,2 Ospedale S. Maria Annunziata, Firenze, ITALY Background: Patients with history of surgery for congenital heart disease often show apparently normal leftventricular (LV) function when assessed by standard systolic and diastolic indexes such as ejection fraction andtransmitral flow pattern. Assessment of long-axis LV dynamics by tissue Doppler provides early and sensitiveindexes of LV performance that can unmask LV systolic and diastolic dysfunction despite normality of standardindexes. However, the existence of subtle abnormalities in LV function in these patients has never beenexplored. Methods: Thirty-seven patients with previous surgical correction of congenital heart disease and no clinicalevidence of heart failure (status post [s/p] group), and 37 age-, gender- and body surface area-matched healthycontrols were recruited. Most common congenital lesions leading to surgery in the s/p group were tetralogy ofFal ot (n=13, 35.1%), transposition of the great vessels (n=5, 13.5%), aortic coarctation (n=5, 13.5%), ventricularseptum defect (n=4, 10.8%), and atrial septum defect (n=4, 10.8%). Al subjects underwent echocardiography toassess standard indexes of LV function. Tissue Doppler was used to assess LV peak longitudinal velocity atsystole (s’) and early diastole (e’). Values were calculated by averaging measurements taken at the septal andlateral site of the mitral annulus. The ratio of peak early diastolic LV fil ing velocity to e’ was also calculated andused as an index of LV fil ing pressure. Results: As a result of 1:1 matching procedure, mean age was 8.5 ± 4.8 in both groups, with equal genderdistribution (45.9% female) and similar body surface area (1.0 ± 0.4 m2 vs 1.1 ± 0.4 m2, p=0.74). The s/p groupand the controls showed no differences in indexed LV mass (63.2 ± 19.9 g/m² vs 58.4 ± 21.6 g/m², p=0.32), LVejection fraction (63.0 ± 7.0% vs 63.8 ± 4.5%, p=0.53), mitral E/A ratio (2.2 ± 0.9 vs 2.1 ± 0.6, p=0.46), and Ewave deceleration time (143 ± 37 ms vs 146 ± 35 ms, p=0.66). However, s/p patients showed depressed s’ (8.0± 1.5 cm/s vs 9.4 ± 1.8 cm/s, p=0.0004) and e’ (13.7 ± 2.1 cm/s vs 15.6 ± 2.0 cm/s, p<0.0001) and higher E/e’ratio (6.9 ± 2.3 vs 6.1 ± 1.1, p=0.039) in comparison with the controls. Thirty-six (97.3%) patients in the controlgroup, but only 29 (78.4%) in the s/p group, showed an E/e’ ratio in the normal range (<8). Similar findings wereobtained when the analysis was focused to the subgroup with history of corrected tetralogy of Fal ot. Conclusions: Despite normality of LV mass, ejection fraction, and transmitral flow pattern, patients with previoussurgical correction of congenital heart disease show significant LV systolic and diastolic dysfunction compared tomatched controls. This impairment seems to be present even when the underlying congenital disease isexpected to predominantly involve right-sided heart chambers. Considering the established prognosticimportance of LV long-axis impairment, careful clinical and echocardiographic fol ow-up, including assessment oflongitudinal LV function by tissue Doppler, should be considered mandatory in these patients. SCREENING AND PREVENTION PROGRAM FOR RHEUMATIC HEART DISEASE IN ERITREAN SCHOOL
CHILDREN
N. Assanta 1, M. Gaeta 2, O. Milanesi 3, R. Biffanti 3, M. Cantinotti 1, T. Gebrehiwot 4, B. MurzI 11 Ospedale del Cuore Fondazione Toscana G. Monasterio, Massa,2 Casa di Cura Vil a d'Abruzzo, Chieti,3Department of Woman and Children's Healt, Padova, ITALY, 4 Pedriatic Department Orotta Hospital, Asmara -ERITREA Background: Rheumatic heart disease (RHD) affect 15.6–19.6 million people worldwide and represent acommon cause of mortality and morbidity . RHD leads to disability and premature death, with importanteconomic and social consequences . The few studies performed in Africa showed a prevalence of RHD inschool children varying between 1,4-6.2 % and demonstrated that the minor rheumatic valve lesions could notbe detected by the only clinical criteria. Patients and methods: From January 2012 to April 2013, four groups of operators, each consisting of acardiologist and a nurse, performed 5 missions in Eritrea for screening and prevention of RHD. Each group hada portable echocardiography devices, a power generator, a laptop computer, light bulb for throat examination,stethoscope, rapid tests for diagnosis of streptococcal infection in progress, oral antibiotics. According toEritrean Ministry of Health and Education, we worked together with local doctors and nurses in order to formthem . We randomly selected students from 51 different schools of Asmara ( elementary and junior) for a total of 3765student ( M 1872, F 1893) with mean age 10 years ( range 5-18 years).
Students received a simple lesson with few slides on prevention, explaining what is RHD and how to prevent it,which are the symptoms of streptococcal tonsil itis and how to treat it. Next step was to col ect medical historyregard to pharyngeal tonsillitis, tonsillectomy or other major diseases, and final y we made complete medicalexamination.
Ultrasound heart study was performed in each student. At present no clear echocardiography criteria exists forthe early diagnosis of RHD. According to latest recommendations, moderate thickening of valve leaflets wasconsidered an indicator of established rheumatic heart disease. Results: We found red pharynx in 43 students (1,1%). Al of them underwent a rapid test for group Astreptococcal research with 7 positive results. Oral antibiotics were administered to children with infection onprogress. In 119 students (3,1%) we found signs of valvular damage as detailed in table 1. Table 1: Incidence and severity of rheumatic heart disease among Eritrean school children.
AO= aorta; MV=mitral valve; RHD=rheumatic heart disease.
<FILE IMAGE='377_20130626142615.JPG'>Students with normal heart, have been educated to primary prevention, while students with ultrasound exam suggestive for RHD were referred to the local pediatric cardiologist, to start anadequate fol ow-up and secondary prophylaxis.
The local staff was been properly instructed to perform the screening autonomously in order to continue theprogram when we will complete our work.
Of interest, as an additional data, in 41 students (1%) various congenital heart defects were also diagnosed.
The most significant ones have been reported to the local pediatric cardiologist.
Conclusion: Data on RHD in Africa are few and the incidence of this disease in some areas like Eritrea is notknown. The African population is exposed to the effects of streptococcal pharyngitis for poor hygiene, geneticpredisposition and poor prevention. The data we have col ected so far are related to the urban area of Asmara,therefore we expect to find a higher incidence of RHD outside the town. Early detection of subclinical RHD is vital, because prophylactic penicil in can prevent progression to clinicalvalve disease and heart failure. Secondary prophylaxis, have been shown to be inexpensive and efficient andhas been recommended by the World Health Organization and the World Heart Federation since the 1980s.
Children education to primary prevention and accurate instruction of local staff are essential for long termsuccess of a prevention program for RHD.
IL FLUSSO IN VENA CAVA SUPERIORE COME MISURA DELLA PERFUSIONE SISTEMICA NEL NEONATO
CRITICO: PROPOSTA DI UNA NUOVA TECNICA ECOGRAFICA
B. Ficial 1, A.M. Groves 2, E. Bonafiglia 1, E.M. Padovani 11 Patologia e Terapia Intensiva Neonatale, Azienda Ospedaliera Universitaria Integrata, Verona, ITALY, 2 Centrefor the Developing Brain, Perinatal Imaging Department, Kings Col ege, London, UNITED KINGDOM IntroduzioneUna promettente metodica di monitoraggio del a perfusione sistemica nel neonato prematuro è la misurazioneecografica del flusso in vena cava superiore (VCS), che rappresenta il flusso di sangue refluo dal a partesuperiore del corpo, l'80% circa dal cervel o. Un ridotto flusso in VCS è associato infatti al o sviluppo diemorragie intraventricolari.
La tecnica tradizionale prevede la misurazione del diametro del a vena da una proiezione parasternale asselungo e la misurazione del a velocità del flusso (VTI) da una proiezione sottocostale. Dal diametro si calcolal’area, assumendo la vena perfettamente circolare. Moltiplicando l’area per il VTI e la frequenza cardiaca, siottiene il flusso.
Tale metodica presenta però una grande variabilità tra osservatori, che ne limita la diffusione nel a pratica clinica.
Per tale motivo proponiamo una nuova tecnica che prevede di misurare direttamente l'area del a VCS da unaproiezione trasversale, a livel o del a biforcazione del ’arteria polmonare (v. Figura1). <FILE IMAGE='661_20130626142636.jpg'>In tal modo si evita di introdurre un errore sistematico calcolando l'area dal diametro, data la forma non circolaredel a vena. Inoltre proponiamo di misurare il VTI da una proiezione sovrasternale. In tal modo i movimentirespiratori e il meteorismo addominale non ostacolano l'acquisizione del e immagini.
Obiettivi del o studioGli obiettivi del o studio sono:- confrontare in 10 neonati le tecniche ecografiche nuova e tradizionale con la Risonanza Magnetica a contrastodi fase (PCMRI)- verificare in 20 neonati la variabilità intra-osservatore tra due misurazioni del flusso in VCS secondo le duetecnica ecografiche MetodiI pazienti studiati con ecografia e PCMRI sono stati reclutati da giugno a novembre 2012 presso le PatologieNeonatali del ’Hammersmith Hospital, Imperial Col ege e del St. Thomas’ Hospital, Kings Col ege, London.
È stato utilizzato un ecografo Vivid 7 (GE Healthcare). L’ecocardiografia è stata eseguita entro ventiquattro oredal a PCMRI, nel e stesse condizioni di sonno o veglia tranquil a. La PCMRI è stata eseguita con scanner 3T(Philips), senza sedazione, durante il sonno naturale, previo posizionamento di protezioni acustiche. I pazienti studiati solo con ecografia sono stati reclutati da dicembre 2012 a marzo 2013 presso la PatologiaNeonatale del ’Azienda Ospedaliera Universitaria Integrata di Verona. È stato utilizzato un ecografo Vivid 7 conle stesse modalità di analisi del e immagini.
Risultati e conclusioniI dieci pazienti sottoposti a ecografia e PCMRI presentavano un’età media di 36+3 settimane e un peso di2123g.
L’area del a VCS calcolata dal diametro, secondo la tecnica tradizionale, sottostima l’area del a vena misurata inPCMRI (13 vs 28 mm2) con un bias medio di -15 mm2 e una scarsa correlazione (r2 0,101).
L’area misurata con la tecnica nuova mostra invece una migliore correlazione con l’area in PCMRI (r2 0,603) eun bias medio di solo -3 mm2.
Entrambe le tecniche ecografiche sovrastimano la velocità di flusso in VCS rispetto al a PCMRI, per un limiteintrinseco del ’ecografia nel determinare la velocità di un fluido in moto turbolento. La misurazione da unaproiezione sovrasternale offre però una stima più accurata rispetto a quel a sottocostale (bias medio + 6 vs + 11cm). Di conseguenza, il flusso in VCS con la tecnica tradizionale mostra una scarsa associazione con la PCMRI (r20,254), mentre la tecnica nuova risulta più accurata (r2 0,657). La coorte di 20 pazienti sottoposta a due misurazioni ecografiche ripetute del flusso in VCS secondo le duetecniche presentava un’età media di 32 settimane e un peso di 1557g. Secondo l’analisi di Bland Altman latecnica nuova mostra una ridotta variabilità intra-osservatore, con coefficiente di ripetibilità che, da 50 ml/kg/minnel a tecnica tradizionale, viene dimezzato a 25ml/kg/min. In conclusione la nuova tecnica ecografica sembra essere uno strumento più affidabile e accurato peridentificare i neonati a rischio di ipoperfusione e di sequele neurologiche severe.
VALUTAZIONE DELLA FUNZIONE VENTRICOLARE NEI NEONATI CON STRAIN E STRAIN RATE :
INDIVIDUAZIONE DEI VALORI DI NORMALITA' COME PRIMO PASSO VERSO L'APPLICAZIONE CLINICA
S. Marcora 1, A. Foresti 2, M. Ciuffreda 1, L. Preda 1, C. Marrone 1, A. Gavazzi 1, A. Borghi 2, L. Gal etti 11 Ospedale Papa Giovanni XXIII, Bergamo, 2 Spedali Civili, Brescia, ITALY SCOPO DELLA RICERCA. 2D speckle tracking (2DSTE) strain and strain rate (SR) sono tecniche poco usatenei neonati per quantificare la funzione ventricolare e a tutt’oggi mancano valori normali di riferimento in questapopolazione. Scopo di questo studio è individuare valori di 2DSTE strain and SR in neonati sani.
METODO IMPIEGATO. Abbiamo arruolato in modo prospettico 68 neonati sani a termine , con età < 72 ore.
Strain e SR longitudinale sono stati ottenuti dal e proiezioni 4-,2-,3-camere apicale per il ventricolo di sinistra(VS) e dal a 4-camere apicale per il ventricolo di destra (VD). Strain and SR radiale del VS sono stati ottenutidal a parasternale asse corto a livel o apicale, medio-ventricolare e basale. Sono stati archiviati 3 cicli cardiaciper l’analisi off-line ( EchoPAC version 7.0 GE system). Tutte le misurazioni sono state fatte da un operatorediverso da chi ha fatto le acquisizioni . Un secondo osservatore cieco ha analizzato 15 casi per valutare lavariabilità interosservatore. I valori sono espressi come media ± DS. Il confronto dei valori di strain and SR neidiversi segmenti del VS e del VD è stato ottenuto con l’analisi di varianza con la correzione di Bonferroni pertest multipli (p <0.05)RISULTATI E CONCLUSIONI. I valori di riferimento di strain and SR sono riportati nel e tabel e 1 e 2. Lariproducibiltà tra gli osservatori è stata più elevata per lo strain e SR radiale piuttosto che quel o longitudinale. Il nostro studio dimostra prospetticamente in una numerosa popolazione di neonati sani che i valori di strain eSR longitudinali non sono omogenei e sono caratterizzati da valori più bassi per il VS che per il VD. Al contrario ivalori di strain e SR radiale sono più omogenei e più alti per entrambi i ventricoli rispetto ai valori longitudinali.
Questo studio ha individuato valori di riferimento per neonati, uti zzabili per ulteriori studi di funzione cardiaca inquesta particolare popolazione usando le nuove tecniche di 2DSTE strain and SR. <FILE IMAGE='162_20130626142706.jpg'> CLINICAL OUTCOME, VALVE DYSFUNCTION AND PROGRESSIVE AORTIC ENLARGEMENT IN A
PEDIATRIC POPULATION WITH ISOLATED BICUSPID AORTIC VALVE
G. Spaziani 1, P. Bal o 2, S. Favil i 1, V. Fibbi 1, I. Pol ini 1, E. Chiappa 11 Azienda Ospedaliera Universitaria A. Meyer, Firenze,2 Ospedale S. Maria Annunziata, Firenze, ITALY Aim of study: Bicuspid aortic valve (BAV) is the most common valvular congenital anomaly, affecting 0.5-2% ofthe general population. Although it’s general y considered a benign condition, concern has been raised in the lastdecades about its association with progression of valve dysfunction and progressive aortic enlargement. Sincethe large majority of studies concerning natural history and complications related to BAV have been conducted inadults, the clinical outcome and the risk of progression of valve dysfunction and aortic dilation in pediatricpatients (pts) have stil to be defined. The aim of this study was to explore the long-term clinical outcome andthe risk of progression of aortic valve disease and aortic dilation in a pediatric population with isolated BAVobserved in Cardiology Unit at Pediatric Hospital A. Meyer in Florence.
Methods: A total of 179 pediatric pts (median [95% IQ] age at enrolment 7.8 [2.7-12.0] years, 76.5% male) withisolated BAV were prospectively fol owed. Pts with severe valve stenosis or regurgitation at baseline wereexcluded. The clinical endpoint included cardiac death, aortic complications and need for surgery orpercutaneous valvuloplasty. The echocardiographic endpoints were: 1) progression of aortic stenosis (AS) orregurgitation (AR) from baseline to fol ow-up, defined as an increase by 1 grade according to a three-level scaleseverity; 2) significant progressive aortic enlargement at different levels of the aortic root, defined asdevelopment of a Z-score >2 not present at baseline.
Results and conclusions: At the initial observation, AS and AR were present in 25 (14.0%) and 87 (48.6%) pts,respectively. After a median fol ow-up of 5.4 [2.3-9.2] years, al pts were alive. The clinical endpoint occurred in 4(2.2%) pts (0.41 events/100 patient-years): elective valvuloplasty was performed in 3 pts for severe AS and 1patient underwent aortic valve replacement for severe valve dysfunction (AS and AR). AS was present in 28(15.6%) pts, whereas AR was present in 102 (57.0%). A progression in AS and AR was observed in 9 (5.0%) and29 (16.2%) pts, respectively. The Z-scores at the end of fol ow-up were not significantly different from thoseobserved at baseline at the annulus, Valsalva sinuses and sinotubular junction, whereas a slight increase wasobserved at the level of the ascending aorta (1.9 vs 1.5, p=0.046). Significant progressive aortic enlargementoccurred in a minority of pts (10.6%, 5.6%, 9.5%, and 19.0% respectively). No baseline variable was able tosignificantly predict the clinical endpoint, the progression of AR, or progressive aortic enlargement at the level ofannulus, sinotubular junction, or ascending aorta. A low Z-score at the aortic annulus was the only variablepredicting progression of AS (odds ratio 0.59, p=0.014), whereas fusion of the right and left coronary cusps wasthe only predictor of progressive enlargement of Valsalva sinuses (odds ratio 7.18, p=0.046). In conclusion, theclinical outcome in paediatric pts with isolated BAV is favourable and the progression of aortic valve dysfunctionand aortic enlargement is relatively slow during childhood and adolescence. On the other hand, considering thata significant progressive enlargement occurred in the ascending aorta, albeit in a minority of pts, our data mightjustify the need for a careful echocardiographic fol ow-up in al these pts, even in those with normal aorticdiameters at first diagnosis. TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION: IS ECHOCARDIOGRAPHIC DIAGNOSIS
SUFFICIENT IN ALL CASES? OUR 24 MONTHS EXPERIENCE
A. Del Pasqua 1, A. Secinaro 1, G. Antonel i 2, E. Cetrano 1, M. Iacomino 1, A. Toscano 1, M. Chinali 1, C. Esposito1, R. Iacobel i 1, A. Franceschini 3, A. Carotti 1, G. Pongiglione 1, G. Rinel i 11 Ospedale Pediatrico Bambin Gesù, Roma,2 Azienda Ospedaliera Universitaria Senese, Siena,3 AziendaOspedaliera Universitaria di Perugia, Perugia, ITALY BACKGROUND: Total anomalous pulmonary venous connection (TAPVC) can be isolated or complex type-when associated to other intra-cardiac lesions besides atrial septal defect and patent ductus arteriosus-. It isfrequently misdiagnosed and without operative correction early death is common. It is important to achieve acorrect and precise preoperative diagnosis which is usual y accomplished by echocardiogram and sometimes byangiography, this last even if invasive, been considered the gold standard until the advent of cardiac magneticresonance angiography (MRA) and computed tomography angiography ( CTA). AIM: Aim of our study was to review our 24 moths experience with diagnosis of TAPVC both isolated andcomplex type and to demonstrate that even in complex cases echocardiographic data are sufficient to achieve acorrect diagnosis of the cardiac disease also in complex and mixed type of TAPVC. In a few, more complexcases, CTA can be very helpful to directly demonstrate some connections in order to precisely plan surgicalcorrection.
METHODS AND MATERIALS: We retrospectively reviewed TAPVC cases treated in our center in 24 months,from April 2011 to April 2013. We col ected data from 10 patients. Mean age at diagnosis was 7 days (1-35days), mean weight was 2 Kg (0.8-3 kg). CTA was performed in 5 patients. Cardiac catheterization was done in0 cases. Surgical correction was achieved in al cases.
RESULTS : TAPVC was isolated in 3 cases , part of asplenia syndrome in 4 cases, associated to other cardiaclesions in 2 cases (ventricular septal defect, aortic arch hypoplasia) and 1 associated to tracheo-oesophagealfistula. TAPVC was mixed type in 2 case, total y infracardiac in 7 cases and total y supracardiac in 1 case.
Echocardiographic diagnosis were confirmed in 5 patients by surgery, in 3 patients by CTA and later on bysurgery. In two cases of mixed type CTA added some information which were useful for surgery.
Echocardiographic and CTA diagnosis –when obtained- were confirmed in 100% of cases by surgicalinspection. Mortality at surgical time was 0%. Mortality at one month fol ow up was 10% (1 asplenic syndromewith mixed type TAPVC). No added mortality were recorded ad 6 months fol ow up where done.
CONCLUSION : Though CTA can facilitate or better demonstrate TAPVC diagnosis especial y in more complexmixed type cases, echocardiography alone is in most cases sufficient to accomplish a precise diagnosis even incomplex subgroups and can correctly guide surgical correction and fol ow up in this congenital heart disease.
Cardiac catheterization in this setting of patients is no more indicated.
STUDIO ECOCARDIOGRAFICO DELLA VALVOLA AORTICA BICUSPIDE IN UNA POPOLAZIONE
PEDIATRICA
L. Filippucci 1, A. Russo 2, B. Stafisso 2, G.R. Ciotti 31 Ambulatorio Cardiologia Pediatrica, Perugia, 2 Servizio Medicina Del o Sport, Perugia, ITALY, 3 ManchesterChildren Hospital CMTF, Manchester, UNITED KINGDOM BACKGROUND: la valvola aortica bicuspide (BAV) rappresenta la più comune cardiopatia congenita conprevalenza pari al 0.5-2%; colpisce il sesso maschile più del femminile con rapporto 3:1; è associata a rischiosignificativo di sviluppare aneurisma del ' aorta toracica e dissezione aortica.
SCOPO DEL LAVORO: studiare la relazione tra morfologia e funzione del a BAV in una popolazione pediatricanon selezionata.
MATERIALI E METODI: da settembre 2010 ad aprile 2013, sono stati sottoposti ad ecocardiografia 1716soggetti di età compresa tra 4 e 18 anni (mediana 12 anni) riferiti al 'ambulatorio di ecocardiografia da pediatri dilibera scelta (52%), medici del o sport (37%) e cardiologi del o sport (11%) per approfondimento diagnosticodopo visita di routine o visita medico-sportiva per idoneità agonistica. Tutti gli esami sotto stati effettuati da dueoperatori, un cardiologo ed un medico del o sport, con ecografo Siemens Acuson CV70 munito di sondapediatrica p9-4 e da adulto p2-4. Per lo studio del a valvola aortica sono state utilizzate proiezioni parasternaleasse lungo e asse corto, particolarmente favorevoli per la ricerca e quantificazione dei lembi valvolari.
Secondo le Linee Guida ASE 2005 le dimensioni aortiche sono state misurate a livel o del ' Anulus, dei Seni diValsalva, del a Giunzione Sino-Tubulare e del 'Aorta Ascendente. Per lo studio del a funzione aortica, i difettivalvolari (stenosi ed insufficienza) sono stati quantificati in accordo con le Linee Guida SIEC 2011.
RISULTATI: La BAV è stata diagnosticata in 39 pazienti (22%), con rapporto M:F pari a 2,7:1 (28:11). La rimadi chiusura valvolare è risultata orizzontale (BAV tipo A) in 28 casi (71%) e verticale (BAV tipo B) in 11 casi(29%). Nel 69% dei casi il rafe si trovava tra la cuspide coronarica destra e la coronarica sinistra. Il 62% dipazienti (n. 24) presentava insufficienza valvolare isolata e di grado lieve; di questi il 67% (n.16) presentavaBAV tipo A. Nel 5% dei soggetti (n.2) è stata riscontrata dilatazione del 'aorta ascendente associata adinsufficienza valvolare di grado moderato; anche in questo caso la BAV era di tipo A. Il 13% dei pazienti (n. 5)presentava steno-insufficienza valvolare e di questi il 60% (n .3) era affetto da BAV tipo B con rafe tra la cuspidecoronarica destra e la non coronarica. Il 20% (n.8) dei pazienti non presentava disfunzione valvolare aortica.
Particolare attenzione è stata dedicata a 2 fratel i, riferiti al 'ambulatorio per screening ecocardiografico inconsiderazione del a forte familiarità per BAV (sia madre che padre): solo il minore, maschio di anni 5 è risultatopositivo per BAV tipo B, il maggiore, maschio di anni 9 è risultato negativo. CONCLUSIONI: nel a valutazione ecocardiografica di una popolazione pediatrica merita particolare attenzionelo studio morfologico e funzionale del a valvola aortica. Il nostro studio afferma che la più frequente disfunzionevalvolare associata a BAV tipo A è l'insufficienza isolata, mentre il tipo B è con maggior prevalenza associato asteno-insufficienza aortica.
PERCUTANEOUS TREATEMENT OF AORTIC COARCTATION: THE VALUE OF PHYSICAL ECHO STRESS
IN THE FOLLOW UP
G. Gaio, G. Santoro, G. Di Salvo, E. Romeo, F. Fratta, E. Campagnano, M. Carrozza, A. Rea, M.G. Russo Cardiology, Monaldi Hospital, 2nd University of Naples, Naples, ITALY Background: An important functional parameter in the assessment of the effects of percutaneus repair of CoA isthe maximal pressure gradient in the descending aorta with the presence of diastolic run off. Increasing of theseparameters after exercise stress test is a clinical parameter that need to be evaluate. Aim of the study was toevaluate the echocardiographyc maximum gradient after physical stress as indicator of recoartaction in patientsunderwent to stent implantation. Methods: In the last 2 years 30 patients underwent to stent implantation for primary aortic coartaction. Al ofthem were evaluated at 6 months from the percutaneous procedure with ecocardiograhy at rest and afterphysical stress. Of these, 10/30 (33%. (8 males, 2 females; mean age 21.8+ 5.4 years) showed a significativeincrease of the maximum gradient. Therefore in this subgroup a second percutaneous procedure was indicated.
Echocardiographic maximum gradient in the descending aorta at rest and after physical stress were evaluatedand then compared with cathlab data. Results: At the rest echo the mean value of maximal pressure gradient was 45mmHg +/- 4 mm Hg withoutdiastolic run off while at the end of the physical stress was 83mmHg +/- 13mmHg (P <0.02 versus rest) witholodiastolic run off. In the cath lab we found a mean peak to peak gradient of 25 mmHg +/- 5mmHg . In 5/10patients (50%) we perfomed a stent redilatation. Conclusions: Physical stress echocardiogrophy could be a predective exam for recoartation even if in about 50%of the cases it overexitmate the gradient DETECTION OF RIGHT VENTRICULAR DYSFUNCTION BY TISSUE DOPPLER IN ASYMPTOMATIC
PATIENTS WITH HISTORY OF SURGERY FOR CONGENITAL HEART DISEASE
V. Fibbi 1, P. Bal o 2, G.B. Calabri 1, S. Favil i 1, G. Spaziani 1, A. Zuppiroli 2, E. Chiappa 11 Azienda Ospedaliera Universitaria A. Meyer, Firenze,2 Ospedale S. Maria Annunziata, Firenze, ITALY Background: Poor data exist about the long-term effect of surgery on right ventricular (RV) dysfunction inasymptomatic patients with congenital heart disease submitted to surgical correction. Methods: Thirty-seven patients with previous surgical correction of congenital heart disease and no clinicalevidence of heart failure (status post [s/p] group), and 37 age-, gender- and body surface area-matched healthycontrols were studied by echocardiography. Most common congenital lesions leading to surgery in the s/p groupwere tetralogy of Fal ot (n=13, 35.1%), transposition of the great vessels (n=5, 13.5%), aortic coarctation (n=5,13.5%), ventricular septum defect (n=4, 10.8%), and atrial septum defect (n=4, 10.8%). M-mode was used toassess tricuspid annulus plane systolic excursion (TAPSE), and pulsed tissue Doppler was used RV peaklongitudinal velocity of tricuspid annulus at systole (s’) and early diastole (e’). The ratio of peak early diastolic RVfil ing velocity to e’ was also calculated and used as an index of RV fil ing pressure. Results: Mean age was 8.5 ± 4.8 in both groups, with equal gender distribution (45.9% female) and similar bodysurface area (1.0 ± 0.4 m2 vs 1.1 ± 0.4 m2, p=0.74). S/p patients showed depressed TAPSE (17.2 ± 5.0 mm vs21.1 ± 3.7 mm, p<0.0001) and tricuspid s’ (9.1 ± 2.0 cm/s vs 12.5 ± 2.1 cm/s, p<0.0001) in comparison with thecontrols. RV diastolic inflow pattern in the s/p group was characterized by lower tricuspid E wave (61.3 ± 14.4cm/s vs 54.5 ± 8.7, p=0.020) and A wave (41.2 ± 14.0 cm/s vs 34.6 ± 7.9, p=0.019) as compared to controls,resulting in similar E/A ratio in the two groups (1.6 ± 0.5 vs 1.7 ± 0.5, p=0.69). However, patients in the s/p groupshowed lower tricuspid e’ (12.2 ± 2.6 cm/s vs 15.3 ± 3.1 cm/s, p<0.0001) and higher tricuspid E/e’ ratio (5.3 ± 1.9vs 3.7 ± 0.7, p<0.0001) than the controls. An abnormal TAPSE (<16 mm) was present in 16 patients in the s/pgroup and in only one subject in the control group (43.2% vs 2.7%, p<0.0001). Similarly, reduced tricuspid s’(<10 cm/s) was present in 26 patients in the s/p group and in 2 subjects in the control group (70.3% vs 5.4%,p<0.0001). A tricuspid E/e’ ratio >6, currently recommended as suggestive of pseudonormal RV fil ing and raisedright atrial pressures, was found in 28 (75.7%) patients in the s/p group, and in none of the controls (p<0.0001).
Conclusions: Patients with previous surgical correction of congenital heart disease show significant RV systolicand diastolic dysfunction compared to matched controls. Considering the established prognostic importance ofRV impairment, careful clinical and echocardiographic fol ow-up should be considered of clinical importance inthese patients.
ROLE OF ECHOCARDIOGRAFI DURING NEONATAL PERIODE .FREQUENCIES AND TIME OF
DIAGNOSIS OF CONGENITAL HEART SEPTAL DEFECTS(CHSD) IN NEONATAL PERIOD
A. Koja 1, N. Kolici 2, N. Kuneshka 1, E. Brahiml ari 31 General Pediatric Service UHC Mother Teresa, TIRANA,2 Neonatology Service, HUOG Koco Gliozheni,TIRANA, 3 Pediatrics Service, TIRANA, ALBANIA Septal defect (DIA, DIV, A-V defect ) are the most frequent congenital heart defect , near 35-40 % of al of them.
Prevalence is 1-4 / 1000 live birth, and often are associated with other congenital heart defects. Large and nottreated defects in the right moment are associated with serious complication like: heart fail ure, arrythmia,pulmonary hypertention etc.
A systolic murmur can be the first sign of the congenital heart defect in a newborn babies, and in the same timethe most habitual reason of for a cardiopediatric consultation. Prevalence of presence of a systolic murmurneonatal period is different in different articles and is published from 0.9 to77.4%. This difference in prevalenceseems to be related to with the size of the study.
Aim. To assess the frequency of congenital heart septal defect in neonate referred to our pediatric cardiologyservice, the single pediatric cardiac tertiary center in Albania. To asses the role of echocardiografi duringneonatal period in our reality, when fetal echocardiografi is stil imperfect. Methods.Were analysed retrospectively the data-base of the outpatient clinic of our service, al neonates duringjune 2009-june 2012, referred for evaluation by the Pediatric community. Referral reasons was a systolicmurmur. Al patients were evaluated with physical examination and echocardiografi. Results: A systolic murmur often is not associated with clinical manifestations, but is strongly related with a DIV .
More affected are male neonates. The high incidence of these defects draws attention to a careful examinationof newborns in terms of time to capture the heart defects that would later complicate the future of healthychildren. Diagnosis and timely correction of congenital heart diseases, cures or improves quality of life. Keywords: congenital heart diseases,septal defekt, DIA, DIV CARDIAC FIBRO-FATTY RHABDOMYOMA IN AN ADULT PATIENT WITH TUBEROUS SCLEROSIS
COMPLEX
M. Tavera 1, A. Marini 2, M. Urru 1, A. Atzei 1, S. Palmas 2, G. Sanna 3, G. Matta 4, R. Tumbarel o 11 SCD Cardiologia Pediatrica, AO Brotzu, Cagliari,2 A.O.U. Cagliari, 3 A.O.U. Sassari, 4 SCD Radiologia, AOBrotzu, Cagliari, ITALY Introduction Cardiac rhabdomyoma is the most common cardiac tumor in childhood (70-86%) frequently associated withTuberous Sclerosis Complex (40-50% of cases), an autosomical dominat neurocutaneous disorder withmultiorgan involvement.
Cardiac rhabdomyoma is usual y the earliest hamartoma detected in TSC patients, with a peak of incidence inthe first two years of life and tendency to spontaneous regression.
Cardiac involvement can range from smal nodular lesions up to larger masses with potential y dangeroussequelae, such as obstruction of ventricular outflow tracts, impairment of ventricular fil ing or rhythm disorders.
Cardiac rhabdomyoma ultrasound features are round, homogeneous and hyperechoic intramyocardial masses,sometimes appearing as multiple foci. Case reportWe decribe a fibro-fatty rhabdomyoma in a 23-years old boy with Tuberous Sclerosis Complex, characterized bymultiorgan involvement: bilateral renal angiomyolipomas, multiple liver angiomyolipomas, subependymalastrocytoma, multiple benign brain tumors, cortical tubers, neck and shoulder angiofibromas, sacral osteolyticlesion. He is asymptomatic for chest pain, syncope or palpitations, referring good exercise tolerance. Theelectrocardiogram is normal. The echocardiogram shows three smal hyperechogenic intraparietal masses in theinterventricular septum, resembling rhabdomyomas. No impairment of ventricular fil ing or outflow tractobstruction is observed. No rhythm abnormalities has been found on 24-hours EKG Holter and stress test.
Cardiac MRI confirms the presence of three smal rhabdomyomas in the interventricular muscular septum withthe signal intensity of fat and absence of enhancement.
DiscussionCardiac rhanbdomyoma is usual y the earliest cardiac detectable hamartoma in TSC and tends to regress in thefirst years of life. Hystopathological y, cardiac rhabdomyoma is composed of vacuolated myocytes. Althoughtrhabdomyoma is wel described in childhood, the pattern of the disease in adulthood is less known.
In this adult patient with TSC and multiorgan involvement, cardiac MRI has evidenced multiple ovoidal intramuralomogeneous masses with imaging features compatible with fibro-fatty tissue. This finding, recently reported onlyin few case reports of adult patients with TSC, may suggest that cardiac rabdomyoma can evolve to fibro-fattytissue in the long term. In comparison with known causes of fat in the myocardium (epicardial fat deposit,arrhythmogenic right ventricular displasia, right ventricular fat, liposarcomas, etc) fibro-fatty masses in TSC seemto be characterized by wel -circumscibed form, intramural location, pure fat density, absence of enhancementand of infiltrative features on surrunding tissue.Therefore, fibro-fatty mass in adult TSC patients may arisechal enging diagnostic issues and need longer fol ow-up to define prognostic implications.

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