R. Gravino 1, G. Limongelli, A. Rea, A. Petraio, F. Ursomando, D. Masarone, T. Palladino, G. Caianiello, M.G. Russo, R. Calabrň, G. Pacileo 1 A.O.R.N. dei Colli - Monaldi Hospital - Second University of Naples, Naples, ITALY

OBJECTIVE: Berlin Heart EXCOR Pediatric ventricular assist device (VAD) is currently used as mechanical circulatory support device for adolescents, infants and small children with advanced heart failure. Here, we report the preliminary experience of a third level centre of Paediatric Cardiology and Cardiac Surgery with VAD METHODS: Between April 2009 and December 2011, 5 children (3 male and 2 female) followed in our Department of Paediatric Cardiology underwent Berlin Heart EXCOR pediatric VAD implantation at the Cardiac Surgery Division of Monaldi Hospital. Two cycles of i.v. inotropic therapy were undertaken before implantation (dobutamine and levosimendan), and all the patients underwent imaging evaluation (including cardiac MRI in 2 patients) and cardiac catheterization. RESULTS: The median age at implant was 8 years (2 to 15 years), the mean weight was 32 Kg (13 Kg to 64 Kg), and the median support time was 90 days (5-210 days). The diagnosis was myocarditis in 4 patients, and heart failure secondary to valve disease in one of them. Three patients received a BIVAD and 2 patients a LVAD. Two patients underwent cardiac Magnetic Resonance Imaging (MRI), showing diffuse subepicardial enhancement without signs of intracellular and interstitial edema, capillary leakage and hyperemia. Endomyocardial biopsy and viral genome PCR were performed in all patients at VAD implantation. Diffuse fibrotic replacement without signs of active inflammation were evidenced in all the patients, and only in one case a viral etiology (CMV) was revealed. Out of the 5 paediatric patients, 4 were bridged to heart transplantation and 1 died on support. After heart transplant, an Extra Corporeal Membrane Oxygenation (ECMO) was needed in 2 patients, 2 patients had chest wound infection and one had hyperacute rejection and bleeding problems. These complications occurred in patients that underwent implantation in I-II INTERMACS level, while patients implanted at an early stage showed no complications. Patients receiving biventricular support had a more favorable clinical course compared with children implanted with left ventricular assist device. CONCLUSIONS: In agreement with literature, our experience confirmed that Berlin Heart EXCOR paediatric VAD could provide satisfactory and safe circulatory support for children with end-stage heart diseases. Timing of device implantation and the careful choice between univentricular/biventricular support represent important prognostic factors in patients with end stage disease.

R. CREPAZ 1, J. Stuefer 2, L. Donazzan 1, C. Romeo 1, C. Pedron 3 1Ospedale di Bolzano dipartimento di Cardiologia, Bolzano, ITALY, 2 Ospedale di Bolzano servizio di Radiologia, Bolzano, ITALY, 3 Ospedale di Bolzano dipartimento di Pediatria, Bolzano, ITALY

BACKGROUND: Systemic hypertension (HT) is a major long-term complication even after successful repair of aortic coarctation (AoCo) and many factors are involved in his pathophysiology. OBJECTIVE: To investigate the role of abnormalities of the aortic arch shape, central aortic flow dynamics and distensibility in developing HT after successful repair of AoCo. METHODS: We selected a group of 27 normotensive patients (pts) among 140 pts regularly followed for AoCo repair, mean age 17,1±7,4 yrs (9-32), with anatomically proven successful repair of AoCo. We analyzed their last clinical and echocardiographic data. Magnetic resonance imaging (MRI) with flow mapping in the ascending and descending aorta was performed in order to study the arch morphology, measure the systolic waveforms and the distensibility of the ascending aorta. Bycicle exercise test (ET) and ambulatory blood pressure monitoring (ABPM) were also obtained. Mean age at surgical repair was 3,4±3,9 yrs (10 days-15 yrs), 12 (44%) pts underwent surgical correction during the first year of life. AoCo repair was performed by end to end-anastomosis (TT) in 24 (extended TT in 6 with arch hypoplasia), patch aortoplasty in 2 and subcalvian flap aortoplasty in 1. The post-surgical follow-up was 13,7±7,1 yrs (3,5-29,4). The shape of the aortic arch was defined by MRI on his global geometry (normal-gothic-crenel) and on the height/transverse diameters ratio (A/T). We enrolled twenty normal age-matched subjects as controls. RESULTS: Six pts were found to have a gothic and 21 a normal aortic arch shape. Pts with a gothic aortic arch shape had an increased A/T ratio (0,80±0,07 vs 0,57±0,06, p <0,001), a greater loss of systolic wave amplitude across the aortic arch (42,8±2,5% vs 33,9±5,6%; p < 0,005) and a lower distensibility of the ascending aorta compared with those with normal shape and controls (4,9±1,1 10-3/mmHg vs 7,2±1,8; p< 0,01). The maximal systolic blood pressure (SBP) on exercise as well as mean SBP, DBP, DP and % > cut off values on ABPM were higher in the gothic than in the normal arch group. There was a correlation between A/T ratio, distensibility of the ascending aorta and % loss of systolic wave amplitude across the aortic arch with maximal SBP on exercise test as well as diurnal and nocturnal SBP on ABPM in the whole group (p < 0,05). CONCLUSIONS: In the long-term follow-up after successful AoCo repair a gothic arch shape is associated with a decreased ascending aorta distensibility and an increased loss of systolic wave amplitude across the aortic arch. This findings explain at least in part the association between this abnormal arch geometry and late HT at rest and on exercise and adverse cardiovascular outcome in this group of young adults with successful AoCo repair.

F. SEDDIO 1, A. IACOVONI 1, R. SEBASTIANI 1, A. FONTANA 1, D. FEDERICI 1, C. MARRONE 1, S. MARCORA 1, D. CUGOLA 1, L. GALLETTI 1, A. TERZI 1, P. FERRAZZI 1 1 OSPEDALI RIUNITI DI BERGAMO, BERGAMO, ITALY

Objectives: Heart transplantation (HTx) in patients with complex congenital heart disease (CHD) is a challenge because of structural anomalies and multiple previous procedures. We analysed our results in adult and paediatric patients to evaluate outcome and assess risk factors of mortality. Methods: Between 1985 and 2011 among 839 patients who underwent HTx, 85 received transplantation for end-stage CHD. Patients were divided in 4 age subgroups: 8 pts <1 year (group I), 20 pts 1-10 years (group II), 24 pts 10-18 years (group III) and 33 pts >18 years (group IV ) and in 2 different era: 1985-2000 (47 pts) and 2001-2011 (38 pts). Anatomic diagnosis were single ventricle in 37 (44%) patients and two ventricles in 48 (56%) patients. Seventy-three (86%) patients had before HTx one or more previous cardiac surgical procedure (mean 2.4±0.9). Twenty-two of them had a failing Fontan.. Mean pulmonary artery pressure was 25,2±14,2 mmHg. Mean transpulmonary gradient 9,4±6,9 mmHg. Results: Mean follow-up after HTx was 7,8 ±6,8 years. Survival at 1 months was 37,7% in group I, 85,8% in group II, 96.8% in group II and 98.4 in group IV and was significantly worse in younger recipients. Overall 30-days mortality was 17,6%. Currently 56 patients (65,8%) are alive. Overall survival at 1,5,10 and 15 years is 83%,73%,67% and 58% respectively. Late deaths were 14. Univariate analysis found risk factors for early and late death were factors reflecting recipient illness such as pre-transplant creatinine, intravenous inotropic drugs, intravenous diuretics, mechanical ventilation and presence of protein-loosing enteropathy (PLE). Multivariate analysis for all events (early and late deaths) identified preoperative mechanical ventilation as independent risk factor for mortality. Number of previous procedures didn't influence survival. Previous Fontan procedure didn't increase mortality. We documented reversibility of PLE in survivors. Conclusions: We demonstrated that heart transplantation for patients with CHD can be performed with expectation of excellent results. Previous procedures and previous Fontan don't reduce survival. Mortality is related to preoperative patient conditions. We encourage to refer complex congenital patients early for transplant assessment and for inclusion in waiting list before detrimental effects of end-stage failure become manifest.

L. DEORSOLA 1, P.A. ABBRUZZESE 2 1 Azienda Ospedaliera O.I.R.M. - S. Anna, Torino, ITALY, 2 Azienda Ospedaliera O.I.R.M. - S. Anna, Torino, ITALY

Introduzione: Fin dalle origini la cardiochirurgia pediatrica ha affrontato le patologie dell' efflusso destro con un semplice patch transanulare, ipotizzando che un' insufficienza polmonare postoperatoria fosse ben tollerata. La storia della Tetralogia di Fallot, con Follow-Up sempre maggiori, ha dimostrato che l' insufficienza polomare causa spesso un danno ventricolare destro progressivo. Pertanto, la chirurgia primaria dell' efflusso destro si č attualmente indirizzata a conservare la valvola polmonare o, se non possibile, ad utilizzare dispositivi che ripristinino la funzione valvolare (monocuspide, condotti valvolati, protesi valvolari). La durata di tali soluzioni č limitata, soprattutto se si considera il rapido mismatch che si sviluppa tra il bambino che cresce ed il dispositivo che non cresce e che condanna il paziente a ripetuti interventi durante la propria vita. Obiettivi: Le Protesi Iniettabili Autoespandibili sono entrate recentemente in commercio per la valvolazione secondaria off-pump di pazienti adulti. A tale scopo sono giŕ state collaudate in centinaia di casi, con risultati molto promettenti. Nel nostro Centro abbiamo iniziato ad utilizzarle anche per la chirurgia primaria dell' efflusso destro nei bambini, impiegando una tecnica basata sul sovradimensionamento. Lo studio esamina i risultati preliminari ad 1 anno, soprattutto per quanto riguarda l' ipotesi che le caratteristiche di queste protesi possano aiutare a ridurre, se non addirittura eliminare, il mismatch legato alla crescita. Materiali e metodi: Da Settembre 2010 ad oggi nel nostro Centro abbiamo impiantato nell' efflusso destro 9 Protesi Iniettabili Autoespandibili, con diametro medio 17,3 mm (range 15 - 19). I pazienti erano 4 maschi e 5 femmine, con etŕ media di 26,4 ± 22,1 mesi (range 2 - 60). Le patologie trattate erano 6 Fallot, 2 Atresie Polmonari ed 1 Truncus. La strategia chirurgica si č basata sul sovradimensionamento. L' efflusso destro č stato allargato il pů possibile con un ampio patch, compatibilmente con le dimensioni del torace. Prima di completare la sutura sulla porzione infundibolare č stata iniettata una Protesi Autoespandibile ulteriormente sovradimensionata, in modo che da non potersi espanderecompletamente. Abbiamo ipotizzato che un efflusso destro sovradimensionato possa precedere la crescita e che la protesi possa seguirla completando il suo processo di espansione fino al diamtro originale. Tutti i pazienti sono stati regolarmente controllati con Ecocardiogramma. Inoltre 6 pazienti sono stati sottoposti anche a TC e 6 a Cateterismo. Le misure del sovradimensionamento sono state confrontate con i diametri attesi per il peso e la superficie corporea dei pazienti. Risultati: Non si sono verificati inconvenienti durante la procedura chirurgica. La pressione ventricolare destra alla fine dell' intervento era sempre il 50% della sistemica. Il funzionamento della protesi, anche se non completamente espansa, č risultato ottimale: non č mai stata osservata insufficienza intraprotesica o periprotesica importante. Al Follow-Up 3 protesi risultano giŕ completamente riespanse ed il gradiente medio č di 19,2 ± 10,3 mmHg. Il sovradimensionamento medio dell' efflusso destro č stato di 3,7 mm e quello della protesi di 2,4 mm, per un totale di 6,1 mm. Il diametro finale della protesi č risultato compatibile con quello di un individuo adulto. Conclusioni: Il sovradimensionamento dell' efflusso destro e l' uso di una Protesi Autoespandibile ulteriormente sovradimensionata permette di impiantare valvole di diametro maggiore di quanto sarebbe anatomicamente possibile. La procedura č di semplice esecuzione e non compromette il funzionamento della protesi. Col tempo la protesi completa il suo processo di espansione fino al raggiungimento del diametro nominale, seguendo la crescita del paziente fino all' etŕ adulta o quasi. In tal modo si dovrebbe ridurre od eliminare il mismatch legato alla crescita e ridurre cosě il numero di interventi nella vita del paziente.

G. AGNOLETTI 1, R. Bordese 1, A. Corleto 1, M. Tibaldi 1, C. Riggi 1 1 Ospedale Regina Margherita, Torino, ITALY

Objective: To identify predictors of poor outcome in neonates with Ebstein's anomaly (EB). Background. EB is a rare congenital heart disease with variable neonatal presentation and outcome. Patients and Methods. From January 2004 to January 2011, 16 newborns, 11 with a prenatal diagnosis, were referred to our centre. Results: Among foetuses with EB 2 had a severe hydrops, 1 a tachyarrhythmia, 9 a cardio-thoracic ratio >0.6, 4 a reversed flow through the ductus arteriosus. At birth 14 patients had moderate to severe EB; 4 had congestive heart failure, 9 duct-dependent pulmonary circulation and needed PGE1 infusion, which restored an adequate pulmonary flow in 5 children. 3 patients with restrictive PFO and metabolic acidosis underwent an atrioseptostomy. 4 patients needed a BT shunt associated with plasty of the right atrium or closure of the tricuspid valve. There were 4 deaths, all occurred in infants with a prenatal diagnosis: 1 at birth, 2 postoperative and 1 sudden death 53 days after surgery. Patients with prenatal heart failure displayed a positive evolution under PGE1 and did not need any intervention. At a median follow-up of 2 years, 2 patients had a Glenn palliation, the remaining 10 patients are symptoms free. The positive outcome was not related to the degree of tricuspid regurgitation, which showed the tendency to decrease with time, but with the area of functional right ventricle. Conclusions: EB is a severe cardiac disease when it presents during the fetal or the neonatal period. In this small series fetal hydrops was not related with perinatal exitus. Mortality was high and the outcome, not influenced by prenatal diagnosis, was related to the presence of functional right ventricle. All babies who survived after the neonatal period had a good outcome at medium term follow-up. However a long term follow-up is mandatory.

L. ARCIERI 1, N. ASSANTA 1, M. CANTINOTTI 1, R. MOSCHETTI 1, R. MARGARYAN 1, M. BERNABEI 1, B. MURZI 1 1 Ospedale del Cuore, Fond toscana G. Monasterio,, Massa, ITALY

Objective : Surgical approaches to single ventricle variants include fenestrated and non fenestrated Fontan operations. This study compares 10 years outcomes with these modifications of the Fontan operation at a single center. Methods : From march 2002 to march 2012 63 patients underwent a modification of the Fontan procedure in which an extracardiac inferior cavo-pulmonary conduit ( Gore-Tex) has been used in combination with a previously staged bidirectional Glenn . 37 pts were male, 27 female . Mean age was 6,2 years ( 2,1- 16,5 ) . Diagnosis were :Tricuspid Atresia 17 ( 3 fenestrated ) , Double Inlet Left Ventricle 11 ( 1 fenestrated ) , HLHS 7 ( 3 fenestrated) , Pulmonary Atresia Intact Septum 3 ( 1 fenestrated) , Unbalanced AV Canal 3 ( 1 fenestrated) , others complex forms of functional single ventricle 22 ( 10 fenestrated) . Associated procedures were performed in 22 patients ( VSD enlargement 3 , AV valve plasty 4 , aortic arch enlargement 1 , Damus Kay Stansel 1 , Left pulmonary artery plasty 2 , pulmonary valve closure 13 ) . There were no differences concerning age between fenestrated and no-fenestrated Fontan . The decision to fenestrate was based on various considerations as well, including preoperative status and anatomy , whether intracardiac procedures were performed,post bypass ventricular function, duration of cardiopulmonary bypass and whether the aorta was cross-clamped.Furthermore during the study we added to our hemodynamic guidelines for considering fenestration a pressure in the Fontan circuit following bypass >18 mm Hg and a transpulmonary pressure gradient >10 mm Hg. Results: there were no early or late mortality and no take down Fontan . . Because of continuing chilo-pericardium and chilo-torax one patient required re-do Fontan one month after a non fenestrated Fontan . At this stage the Fontan circuit was fenestrated . No statistical differences were observed concerning ICU staying, duration of drainage and hospital staying between fenestrated and no fenestrated Fontan . Follow up ( mean 4,2 years , max 9 years ) is 100% avalaible . All but 2 patients are in sinus rhythm . Two patients ( one fenestrated and one not fenestrated ) had left hemiparesis due to systemic embolism . All fenestrated Fontan are on anticoagulant therapy while all non fenestrated Fontan ,but 3 , are without . There is at moment one failing Fontan ( Plastic Bronchitis ) but the Fontan circuit has not yet been taken down . Conclusions. Proper selection of patients for modifications of the Fontan procedure resulted in excellent early and late survival with a low incidence of atrial dysrhythmia and stroke. Midterm functional outcomes were excellent.

S. TURCI 1, C. Pace 1, E. Angeli 1, G. Oppido 1, L. Ragni 2, V. Gesuete 3, G. Gargiulo 1 1 Cardiochirurgia pediatrica e dell'etŕ evolutiva - Prof. G.Gargiulo - Policlinico S.Orsola-Malpighi, Bologna, ITALY, 2 Cardiologia pediatrica e dell'etŕ evolutiva - Prof. M.Bonvicini - Policlinico S.Orsola-Malpighi, Bologna, ITALY, 3 Cardiologia - Prof. A.Branzi - Policlinico S.Orsola-Malpighi, Bologna, ITALY

Scopo della ricerca: riportare la nostra esperienza ed il follow-up a breve-medio termine dei pazienti sottoposti a trapianto di cuore in etŕ pediatrica e/o per cardiopatie congenite. Metodi impiegati: questo studio retrospettivo indaga il decorso dei 33 pazienti sottoposti a trapianto di cuore presso il nostro centro dal 1994 al 2011. Sono stati analizzati: le diagnosi pre-trapianto, l'etŕ al trapianto, il sesso, i risultati del PRA (panel reactive antibody), le precedenti procedure chirurgiche, le complicanze immediate ed a distanza, le pressioni polmonari prima e dopo il trapianto, la presenza di vasculopatia coronarica del graft ed il numero di episodi di rigetto per paziente. La diagnosi di rigetto č stata formulata sulla base dei risultati di biopsie endomiocardiche ventricolari destre, basandosi sui criteri di Dallas. La curva di sopravvivenza č stata costruita secondo Kaplan-Meyer. Risultati: lo studio include 33 pazienti (18 maschi, etŕ al trapianto 13,3+10,7, range 2 mesi - 46 anni). Le diagnosi preoperatorie sono: cardiomiopatia dilatativa (15/33; 45,4%), cardiomiopatia ipertrofica (3/33; 9,1%), cardiomiopatia restrittiva (2/33; 6,1%) e cardiopatia congenita (13/33; 39,4%). Il follow-up mediano č di 5,1 anni, la sopravvivenza attuariale a 5 anni č dell'87,8%. Due pazienti hanno presentato PRA positivo, uno prima e l'altro dopo il trapianto. Otto pazienti (8/33; 24,5%) hanno richiesto l'impianto di dispositivi di circolazione assistita come ponte al trapianto; in 7 casi ECMO con pompa centrifuga, in 1 caso BiVAD paracorporeo pulsato Berlin Heart Excor. Tutti gli 8 pazienti sono stati trapiantati: 6 pazienti sono stati dimessi in eccellente classe funzionale, due pazienti (2/8; 25%) sono deceduti dopo il trapianto, in un caso per ischemia intestinale, in un caso per sepsi. Nel gruppo di pazienti sottoposti a trapianto in prima battuta si sono verificati 3 decessi (3/25; 12%), di cui due precoci ed uno a distanza: un paziente č deceduto in 30° giornata postoperatoria per multiorgan failure, un paziente a distanza di due mesi dal trapianto per rigetto acuto e polmonite interstiziale, un paziente č deceduto per disfunzione del graft da rigetto umorale a 9 anni di distanza dal trapianto. Non sono risultate differenze nella classe funzionale, sopravvivenza, grado di rigetto al follow-up tra i pazienti sottoposti o meno ad impianto di sistemi di circolazione assistita come ponte al trapianto. Un paziente č stato sottoposto a retrapianto a distanza di 24 ore dal primo per disfunzione biventricolare acuta del graft. 3 pazienti (3/29; 10,4%) hanno sviluppato un disordine linfoproliferativo. Un paziente con resistenze polmonari elevate ed in trattamento con inibitori delle fosfodiesterasi nel preoperatorio ha mostrato una riduzione delle pressioni polmonari al controllo ad un anno dal trapianto. Undici pazienti (11/29, 38%) hanno presentato almeno un episodio di rigetto di grado 3A. Un paziente (1/29, 3,5%) ha sviluppato coronaropatia del graft all'IVUS, a distanza di 70 mesi dal trapianto. Tutti i pazienti sopravvissuti sono al domicilio, in ottima classe funzionale. Conclusioni: il trapianto cardiaco č il trattamento di scelta per cardiomiopatie e cardiopatie congenite in fase terminale, con ottimi risultati a breve-medio termine nella nostra esperienza; ancora oggi la terapia immunosoppressiva ed il rigetto cronico sono punti sui quali lavorare per migliorare i risultati a lungo termine.