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1 FOLLOW UP E EMODINAMICA P89 DUCTAL LENGTH OF STENT A PREDICTOR OF INTERSTAGE CATHETER BASED REINTERVENTION AFTER HYBRID APPROACH L. Manuri, S. Agati, M.B. Saitta, D. Poli, M. Campisi, S. Morelli, E. Iannace, F.S. Iorio, G. Pongiglione, P. Guccione Centro Cardiologico Pediatrico del Mediterraneo, IRCCS Ospedale Pediatrico Bambino Gesù, Taormina, ITALY Background Hybrid approach for palliation of Hypoplastic Left Heart Syndrome using single stage bilateral pulmonary artery bands and anterograde ductal stenting has became a well accepted option for complex congenital heart cases. Several interstage complications are now published in the literature. Rates of catheter based reintervention is one of the key point of such an approach. Cumulative experience is recently reported both from Galantowicz and colleagues that focused on development of neointimal formation causing ductal in-stent- stenosis that has most common in patients with greater initial weight and a lower stent-to-weight ratio, and from Caldarone and colleagues that stress the concept how the ductal stenting play a central role in hybrid palliation and it must cover the full length of the duct from the pulmonary artery junction to beyond the arch isthmus to reduce reintervention rates. This study aimed to review our experience retrospectively and identify possible predictors to prevent and reduce catheter based interstage morbidity. Methods Since October 2011 to April 2013, 24 consecutive patients affected by HLHS or variants that underwent single stage hybrid approach were reviewed. The form of surgical palliation was discussed at an interdisciplinary conference with cardiologists, cardiac surgeons and anesthesiologists. All procedure were performed under general anesthesia in a dedicated hybrid suite. After median sternotomy, a purse string suture was placed on the main pulmonary artery and a 7 Fr sheath was inserted. First angiography was than performed to detail ductal anatomy and possible contraindication to hybrid palliation. Selective bilateral pulmonary artery bands was then realized and, based upon the ductal length and diameter, a stent was chosen in diameter to the duct s largest dimension and long enough to cover its length. Under fluoroscopic guidance, the stent was deployed and repeat angiography performed to confirm position and shape of pulmonary artery banding. An additional stent was implanted if all ductal tissue was thought not to be covered. Results Of the 23 patients included in the study, 13 were boys (58 %) and 10 were girls (42%). The median age at hybrid palliation was 1,5 days (range, 1 7 days), and the median initial weight was 3.07 kg (range kg). Patients who required either transcatheter intervention (Group A = 5 patients; 21% ) were statistically compared with those who did not (Group B = 18 patients; 79% ). One patient was excluded from the study because the ductus was not stented due to the large size. No difference were noted between the two groups in terms of gestational week, weight at born, oxygen saturation, vital signs, ventricular function, degree of tricuspid regurgitation, pulmonary artery band gradients shown by echocardiography, diameter of the stent and diameter/weight ratio. Statistical difference were noted in length of stent (group A: 12 mm range mm versus group B: 18 mm, range mm; p<.0001) and length of stent/ weight ratio (group A: 4 versus group B: 5,3 p<.0001). Conclusion Increasing clinical experience is supporting follow-up study on the fate of ductal stenting to better understand interstage morbitidy in neonates treated by hybrid palliation. Our experience confirm the importance of length and length to weight ratio as possible predictors of interstage ductal stenosis. Differently with the experience of Holly and colleagues, in our experience the median ratio between stent diameter and weight at surgery was not statistically related to ISS.

2 P90 THE EPIDEMIOLOGICAL DATA OF RHEUMATIC HEART DISEASE IN TIME DISTANCE N. Kuneshka (Maliqari) 1, L. Teneqexhi 2, O. Cibuku 1, A. Koja 1, E. Kola 3 1 Pediatric Cardiology Unit, University Hospital Madre Teresa, Tirana, 2 Departement of Public Health, University Hospital Madre Teresa, Tirana, 3 Pediatric Intensive Care Service-University Hospital Madre Teresa, Tirana, ALBANIA Introduction. Rheumatic heart disease (RHD) remains a significant cause of valvular heart disease acquired in origin today in our country. Despite a documented decrease in the prevalence of rheumatic heart disease, these non-suppurates cardiovascular sequels of group A streptococcal pharyngitis remains a medical and public health problem. Aims. The purpose of this study is to analyze the frequency and forms of the appearance of rheumatic carditis in rheumatic fever in general and to assess by comparing the difference of frequency and factors that affect its appearance in time distance. Materials and methods. There were analyzed retrospectively the data-base of two groups of patients which are hospitalized and followed up by Pediatrics Cardiology Department. The first group includes 225 patients diagnosed and treated from ; the second group of 68 patients was diagnosed and treated from The cases included on the study were all on the first episode of the disease. The mean age of the patients is 11.9 in the first group and 10.1 in the second one. Patients with congenital heart disease, inflammatory disease, acute viral myocarditis, septic arthritis are excluded from the study. Patients were selected according to Jone s modified criteria and after the initial evaluation they were assessed on the basis of laboratory tests, ECG, Doppler Echocardiography. The social-economic conditions and the treatment of previous group A streptococcal upper respiratory infections are carefully evaluated on the study. Results and discussion. By analyzing the forms of rheumatic fever in each group, we revealed that cardiac manifestations were respectively 119 (53%) cases and 35 (51%) cases on groups. In the first group of 119 patients with rheumatic heart disease 89 (74%) had mitral and/or aortal valve disease, 6 (5%) had pericarditis, 9 (7.5%) myocarditis and 5 (4%) cases were with pancarditis. In the second group the severity and the magnitude of cardiac manifestations is significantly decreased. There are 35 cases with rheumatic heart diseases: 31(89%) just the touch of mitral valve and/or aortic valve, only 4(11%) cases with myocarditis and valve disease and no cases with pericarditis or pancarditis. There were nor pulmonary neither tricuspid involvement on both groups. In the first group the forms of presentation of rheumatic carditis at the time of diagnosis are mainly medium to serious forms, ( 66% ) while in the second group the dominant forms are mainly light ( 83% ) and not moderate or severe. It was observed that in the rural-urban distribution there are significant differences, with a high number of cases in rural areas compared to the city (68% vs. 32%). In the first group it was also observed that the most severe clinical forms of the disease were in higher percentage in rural areas. Lower socioeconomic groups had more RHD (2.7% versus 1.4%; P=0.036) and more advanced disease (64% versus 36%; P<0.001) Conclusions. Cardiac manifestations and mainly the valve touch are still the most serious sequels of acute rheumatic fever, despite the decrease of frequency and severity of the disease. Social-economics factors such as overcrowding, poor housing, poverty, difficulties in assessing health care effect on rapid spread of group A streptococcal respiratory infections, prophylaxis and/or non-compliance with it, impact negatively on the expression of the disease.

3 P91 EARLY AND LONG TERM RESULTS OF STENT IMPLANTATION FOR AORTIC COARTATION IN PEDIATRIC PATIENTS: A SINGLE CENTER EXPERIENCE S. Bondanza, M. Marasini, L.V. Zannini G.Gaslini, Genova, ITALY Aim of the study: to analyze early results and long term follow up of pediatric patients who underwent stenting for aortic coartation. Methods: we reviewed our experience on stent implantation in children with aortic coartation analyzing data collected in our database about immediate and long term results of the procedure. Between 2000 and 2012, in our Institute, we implanted stents in 30 patients (22 males and 8 females) aged between 3 and 18 years ( mean age 11±4 years ) with coartation of the aorta. In our series, 19 patients were treated for isolated native aortic coartation and 11 for recurrent coartation after surgery or balloon dilatation. We implanted 5 Palmaz stents (Cordis), 15 CP stents (NuMED) and 12 Covered CP stents (NuMED). In 2 cases 2 CP stents were positioned sequentially to cover long lesions. Results: mean systolic blood pressure before the procedure was 131±18 mmhg. After stenting, the peak systolic gradient pressure decreased from 39.5±13 to 1.5±3 mmhg(p< 0,05). We had 4 immediate complications, one migration of the stent across the aortic arch requiring immediate surgical stent removal and surgical repair of the aortic arch; two thrombosis of the femoral artery which were treated in one case with Fogarty thrombectomy and in the other case with heparin infusion, and one thrombosis of the hypoglossal artery treated with stent implantation. At follow up we observed 9 late complications (after 2 to 5 years from the procedure): 4 restenosis, in 3 cases the patients underwent effective redilatation of the stents and in 1 case the patient underwent surgical treatment of the restenosis; 4 migrations of the stent: in 3 patients the stent was displaced in abdominal aorta without clinical problems and it was left in place, in 1 patient it was found in the distal part of the aortic arch, in this last case surgical repair was required; finally one small abdominal aorta dissection occurred with no clinical manifestation. At follow up after 1 year since the procedure, we collected data about 28 patients ( 93%): the mean systolic blood pressure was 123±13 mmhg, 9 patients received medical treatment (ace inhibitors). After 5 years we have data about 15 patients ( 50%): mean systolic blood pressure was 126±10 mmhg, 4 of these patients were under medical treatment ( 2 ace inhibitors, 2 beta blockers). After 10 years we have data about 8 patients (27%),only one of them was under medical treatment ( ace inhibitors), the others had normal systolic pressure values. Ten of our patients had a stress test after 1 to 5 years from the procedure: 9 of them had normal response to the test, 1 had hypertension at the peak of exercise testing. We studied 9 patients with magnetic resonance imaging (MRI) or multislice computed thomography, only one mild restenosis of the stent was detected (this patient was the only with hypertension at the exercise test). Conclusions: in our experience, stenting in aortic coartation can be considered as an effective but challenging therapeutic option in pediatric population. Long term follow up with clinical evaluation, echocardiography, MRI and stress test should be mandatory, because complications can also occur many years after the procedure.

4 P92 LA VALVOLA AORTA BICUSPIDE NEI PRIMI VENT ANNI DI VITA: ASPETTI MORFO-FUNZIONALI G. Dolci, E.G. Milano, M.A. Prioli, M. Rebonato, F. Torelli, V. Benetti, L. Rossetti, C. Vassanelli Università degli Studi di Verona Cardiologia DU, Verona, ITALY BACKGROUND: La valvola aortica bicuspide (AoB) è la più frequente cardiopatia congenita con un incidenza dell 1-2% nella popolazione generale ed un rapporto maschi/femmine di 3:1. Può presentarsi isolata o associata ad altre anomalie cardiache congenite. La storia naturale dell AoB è caratterizzata da prematura disfunzione valvolare (stenosi aortica, insufficienza aortica, endocarditi) e/o dallo sviluppo di complicanze vascolari riguardanti l aorta toracica (dilatazione, aneurisma, dissecazione). SCOPI: Valutare la presentazione clinica e la funzione valvolare al momento della diagnosi nei pz con AoB isolata od associata a COAO (Coartazione aortica) ed età inferiore o uguale a 20 anni. Sono stati esclusi i pz con AoB associata a cardiopatie complesse. MATERIALI E METODI: Dal nostro database della Divisione di Cardiologia dell Azienda Ospedaliera Universitaria Integrata di Verona abbiamo selezionato 328 pz con diagnosi ecocardiografica certa di AoB avvenuta entro i 20 anni di vita. I pz sono stati suddivisi nelle seguenti fasce d età: inferiore o uguale ad 1anno (n=68pz), 1-5 anni (n=53pz), 5-10 (n=75pz), (n=60pz), (n=72pz) e per ciascun intervallo abbiamo valutato la funzionalità valvolare al momento della diagnosi e l associazione con la COAO. RISULTATI: Nella nostra casistica, 328 pz, l età media alla diagnosi era di 8.4±7.2 anni con un rapporto M/F=3/1. Nei pz con età inferiore o uguale ad 1 anno l associazione con la COAO era presente nel 49% dei casi. Tale percentuale si riduceva al 23% nel secondo gruppo di pz e progressivamente si stabilizzava al 12% dei pz dopo i 10 anni d età. Dal punto di vista della funzionalità valvolare la stenosi aortica alla diagnosi prevaleva sotto l anno di vita, 31% dei casi, per poi decrescere al 17% nel secondo gruppo e assestarsi all 8% dei casi nel terzo gruppo di pz. Sopra i 10 anni tale percentuale era pari al 5%. Analogamente le valvole normo-funzionanti si riducevano dal 62% nel primo gruppo fino ad arrivare al 6% nell ultima fascia d età considerata. In parallelo si riscontrava una crescita delle valvole insufficienti passando dal 6% in età inferiore o uguale ad 1 anno fino al 65% nei soggetti tra i 15 ed i 20 anni d età (Figura). CONCLUSIONI: La diagnosi di AoB nel primo anno di vita è correlata nel 49% dei casi alla diagnosi di COAO mentre nei restanti pz con AoB isolata in epoca neonatale la diagnosi è associata nel 40% dei casi a stenosi aortica. Dopo l anno di vita l incidenza della stenosi aortica decresce mentre l insufficienza aortica diventa il vizio dominante, arrivando ad una percentuale maggiore del 60% nei pz oltre i 10 anni d età. In parallelo, le valvole normo-funzionanti si riducono al 30%. La gran parte dei pz, esclusi quelli con presentazione neonatale, sono paucisintomatici ed una diagnosi precoce è fondamentale per un attento follow-up e raccomandazioni sullo stile di vita. <FILE IMAGE='660_ jpg'>

5 P93 EARLY AND LONG TERM RESULTS OF ONE AND HALF VENTRICLE REPAIR IN ADULT PATIENTS M. Carrozzini 1, M. Padalino 1, V. Vida 1, L. Leoni 2, O. Milanesi 3, G. Stellin 1 1 Pediatric and Congenital Cardiac Surgery, Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, 2 Cardiology Clinic, Department of Cardiac, Thoracic and Vascular Sciences, University of Padova, Padova, 3 Pediatric Cardiology, Department of Women and Children*s Health, University of Padova, Padova, ITALY OBJECTIVE The one and half ventricle repair (1.5 VR) represents a valid surgical option to Fontan circulation or high risk biventricular repair in a wide spectrum of congenital heart anomalies, with borderline right ventricle. Currently, good functional and clinical results are reported in paediatric patients. However in adults, 1.5 VR could represent a higher risk procedure, due to less compliant and possibly impaired cardiovascular and pulmonary systems, as a late result of previous operations and/or natural history of cardiac anomalies and aging. We describe early and long term results of 1.5 VR as a palliative surgery in a group of adult patients with complex congenital heart defects. METHODS We retrospectively reviewed our institutional database. Between February 2000 and January 2012, we found five adult patients, who underwent 1.5 VR. There were four male and one female, with a mean age at operation of 42.3 years (range years). The basic diagnoses were: Ebstein s Anomaly (n=3); TV and RV hypoplasia (n=1); TV and RV hypoplasia with pulmonary valve stenosis (n=1). An associated ASD-II or PFO was present in four patients; one patient with Ebstein s Anomay presented associated moderate MV regurgitation. Two patients had undergone previous palliative cardiac operations: Fontan (Bjork type) operation in one, Blalock-Taussig shunt in the other. Preoperative cardiac assessment included EKG, transthoracic echocardiogram and cardiac catheterization in all patients; three patients underwent additional preoperative pulmonary scintigraphy. Mean TV annulus Z-score was -3.0 (range ). Mean pulmonary vascular resistance was 1.6 WU/m2 (range 1.0 to 2.3). All available clinical and operative data were included. Follow-up data were collected from all surviving patients at last outpatient visit or by phone contact. RESULTS All underwent 1.5 VR as planned preoperatively; associated procedures were: TV repair (n=1); TV replacement and MV repair (n=1); RV-PA continuity restoration with a valved conduit (n=1); radiofrequency ablation (n=2). One patient required definitive PM implant. There were no death at operation. Postoperative complications occurred in 4 patients (80 %): arrhythmias (n=2), acute renal failure (n=1), cardiac effusion requiring pericardiocentesis (n=1), right endoventricular apical thrombus (n=1), hemidiaphragm palsy and pleural effusion requiring diaphragm plasty and pleurodesis (n=1). Mean ICU stay was 2 days (range 1 4). All patients were discharged home alive and well, after a mean hospital stay of 26 days (range 8 61), on anticoagulation regimen for 6 months. At a mean follow up of 5.9 years (range ; FU completeness: 100 %), there was 1 late death (not cardiac related, for a cerebral stroke 2 years after 1.5 VR). Among survivors, three were in NYHA class I, one in NYHA class II. In one patient, SVC thrombosis and pulmonary embolism occurred 1 month after 1.5 VR because of inappropriate anticoagulation discontinuation. There were no other late adverse events. Three patients are on anticoagulation. CONCLUSIONS In our experience, 1.5 VR showed encouraging early and long term results in adult patients. It can be considered as a palliative correction of a congenitally hypoplastic/dysfunctional RV as an alternative to Fontan operation. Upgrading the Fontan circulation to 1.5 VR can be feasible in selected cases.

6 P94 CAPACITÀ FUNZIONALE NELL ETÀ ADULTA IN PAZIENTI SOTTOPOSTI AD INTERVENTO DI FONTAN. DIFFERENZA TRA I VARI APPROCCI CHIRURGICI M. Palma 1, A. Correra 1, G. Scognamiglio 1, B. Sarubbi 1, M. D'alto 1, D. Colonna 1, G. Di Nardo 1, E. Romeo 1, N. Grimaldi 1, A. Merola 1, G.M. Di Marco 1, M.G. Russo 2 1 UOD Cardiopatie Congenite Dell'adulto, Ospedale Monaldi, AORN Dei Colli, Napoli, 2 UOD Cardiologia Pediatrica, Seconda Universita' degli Studi di Napoli, Ospedale Monaldi, AORN Dei Colli, Napoli ITALY Introduzione: In pazienti operati di correzione secondo Fontan esiste una netta discrepanza tra la capacità funzionale riferita dai pazienti ed i dati obiettivi valutati mediante metodiche standardizzate. Scopo del lavoro è stato quello di confrontare i risultati emersi dal test cardiopolmonare (CPET), test dei sei minuti (6MWT) e dosaggio del Pro-BNP in pazienti adulti già sottoposti a diversi approcci chirurgici di correzione sec Fontan. Materiali e metodi: Lo studio include 22 pazienti (10M/12F età media 27,1±9.4). 15 (7M/8F età media 24.46) di questi erano stati sottoposti ad intervento sec. Fontan mediante connessione atrio-polmonare diretta o mediante tubo intracardiaco (APC) e 7 (3M/4F età media 37.85) mediante connessione cavo-polmonare totale diretta (TCPC). Di ogni paziente è stato considerato il probnp, la frazione di eiezione del ventricolo sistemico (FE%) all esame ecocardiografico, il 6MWT, la classe funzionale (NYHA) ed i risultati del CPET. Risultati: <FILE IMAGE='656_ jpg'> La maggioranza dei pazienti riportavano soggettivamente una buona tolleranza all esercizio (10 erano classificati in I classe NYHA, 7 in II classe NYHA, e solo 5 in III classe NYHA). La funzione contrattile globale (FE%) del ventricolo sistemico non mostrava differenze significative tra i due gruppi. La capacità di esercizio valutata al 6MWT risultava superiore nei pz con TCPC rispetto a quelli con APC, rispecchiata anche da un indice di Borg superiore in questi ultimi rispetto a quelli sottoposti a TCPC. Il test cardiopolmonare mostrava una ridotta capacità funzionale e un ridotto consumo di O2 (VO2max, VO2work, Polso di O2) nei pazienti con APC rispetto al quelli con TCPC, con una normale risposta ventilatoria (VE/VCO2) in entrambi i gruppi. I livelli di pro-bnp erano significativamente più alti nei pazienti sottoposti a connessione atrio polmonare rispetto ai paziente sottoposti a connessione cavo-polmonare. Conclusioni: I livelli di pro-bnp correlano col tipo di correzione sec Fontan. La APC, che coinvolge una maggiore quantità di tessuto atriale, mostra livelli di pro-bnp più alti rispetto ai pazienti corretti mediante TCPC indipendentemente da loro stato cardiaco. E possibile dimostrare una correlazione tra livelli di probnp e capacità funzionale al 6MWT ed al CpET solo nei pz con circolazione Fontan tipo APC. Non esiste, al contrario una correlazione significativa tra livelli di pro BNP e parametri valutati al 6MWT and CpET nei pz con TCPC.

7 P95 CLINICAL PROFILE AND QUALITY OF LIFE OF ADULT PATIENTS POST FONTAN G. Bordin 1, S. Perentaler 1, A.C. Frigo 1, V. De Lucia 2, I. Spadoni 2, R. Crepaz 3, P. Michieli 1, E. Quadri 4, M. Padalino 1, R. Biffanti 1, A. Cerutti 1, B. Castaldi 1, E. Reffo 1, N. Maschietto 1, V. Vida 1, G. Stellin 1, O. Milanesi 1 1 Università di Padova, Dipartimento di Salute della Donna e del Bambino, Cardiologia Pediatrica, Padova, 2 Fondazione Toscana G. Monasterio, Dipartimento Pediatrico, Massa, 3 Ospedale di Bolzano, Dipartimento di Cardiologia, Bolzano, 4 IRCCS Policlinico San Donato, dipartimento di Cardiologia Pediatrica e Cardiopatie Congenite nell'adulto, Milano, ITALY Background and objectives. More and more patients with univentricular heart reach the adult age, thanks to the improved surgical technique and post operative management. Nevertheless the Fontan status is an ongoing disease and long term clinical outcomes, psychological consequences and quality of life (QOL) of these patients are an actual important concern. The purpose of this study is to describe the overall clinical and psychological profile, including QOL, of adult patients post Fontan, and to identify the most significant determinants of QOL. Methods. This multicenter study reviewed the history of thirty-nine patients aged between eighteen and forty-eight years in terms of: (i) type and number of surgical procedures, (ii) long term complications, (iii) medications and O2 saturation. We examined all patients using 24 hours EKG, echocardiography and exercise testing. Finally two different scores, a cardiac function and an exercise performance score, were adopted to compare the functional status with QOL features. Self report questionnaires were used to assess social conditions, ability at work (Work Ability Index questionnaire), quality of life (SWLS - Satisfaction With Life Scale questionnaire), perceived health status (SF-36 - Short form-36 questionnaire), coping strategies (Brief-Cope questionnaire) and presence of mood disorders (HADS - Hospital Anxiety and Depression Scale questionnaire). Results. The majority of the 39 patients who accepted to enter the study was unmarried (84.7%), had a high school diploma (64.1%) and was employed (66.7%). Most of them had a morphologic left ventricle (79.5%), a primary diagnosis of tricuspid atresia (51.3%) and a total cavo-pulmonary connection (82.1%). On average patients underwent 2.7 interventions (standard deviation SD- 0.9), took 2.5 medications (SD 1.8) and had an Oxygen saturation of 92% (SD 3%). 32 patients (82%) had at least one long term complications: 23 patients (59%) had arrhythmias, 11 patients (28.2%) had a permanent pacemaker; one patient (2.6%) had protein losing enteropathy, 3 patients (7.7%) had neurological impairments and 17 patients (43.6%) had hepatic dysfunction. Plastic bronchitis was not reported in our cohort. The median single ventricle ejection fraction was 59% (Q1-Q ), the median VO2max was 26.4 ml/min/kg (Q1-Q ). Adult patients with the Fontan circulation demonstrated to be anxious (median of the HADS for anxiety 13; Q1-Q ), and in a minor manner depressed (median HADS for depression 9; Q1-Q3 7-10). The more used coping strategies were Active Coping (median 7; Q1-Q3 6-7), Planning (median 6; Q1-Q3 5-7) and Acceptance (median 6; Q1-Q3 5-8). Nevertheless this population revealed to have a good QOL, with a median score in the SWLS of 25 (Q1-Q ). People with a lower cardiac function score had a worse QOL, whereas the exercise capacity seemed not to be related to it. The SF-36 questionnaire showed that the General Health is perceived as quite good (mean 64.4, SD 24.0), the Physical Function is related to both the cardiac function (p= 0.004) and the exercise performance (p= 0.026) scores, and Vitality is mildly related to the presence of long term complications (p= 0.044). Finally both the QOL and the SF-36 domains were strongly related to the Work Ability Index. Conclusions. In this group of patients post Fontan, with a mean age of 28 years and a mean follow-up of 20 years, QOL is perceived as good, even in a western environment such as the northern Italy. Furthermore QOL is not related to the severity of the disease in terms of initial diagnosis, medical history and exercise capacity. Finally adult patients post Fontan demonstrated to develop good coping strategies and to adapt daily life to their physical function, in spite of high levels of anxiety.

8 P96 LONG-TERM FOLLOW-UP AFTER SURGICAL PLASTY OF THE TRUNCAL VALVE IN NEONATAL REPAIR OF COMMON TRUNCUS ARTERIOSUS F. Petridis 1, E. Angeli 1, L. Careddu 1, R. Formigari 2, G. Oppido 1, E. Mariucci 2, M. Agulli 3, G. Gargiulo 1 1 Policlinico S.Orsola-Malpighi, Pediatric and Adult Congenital Cardiac Surgery, Bologna, 2 Policlinico S.Orsola-Malpighi, Pediatric and Adult Congenital Cardiology, Bologna, 3 Policlinico S.Orsola-Malpighi, Pediatric and Adult Congenital Cardiac Anesthesia, Bologna, ITALY Objective: In order to evaluate the long term outcome of the truncal valve after truncus arteriosus repair. Methods: A retrospective review of the clinical, echocardiographic and operative reports of all consecutive patients with truncus arteriosus operated on since 1989 until 2011 in our institution. Results and Conclusion: Thirty-five patients with TA met the inclusion criteria. Twenty-four were anatomically type I, 10 type II and one type IV. The truncal valve was bicuspid in 6 patients, quadricuspid in 9 and tricuspid in 20 patients. Interrupted aortic arch was associated in 3 patients. Mean weight at intervention was 3.1±1.2 Kgs (range Kgs), and median age was 26 days (range days). Associated genetic syndromes (22q11 del, 5p- del, CHARGE) were present in 25.6%. Preoperative truncal valve regurgitation was absent in 14 patients (40%), mild in 7 (20%), moderate to severe in 14 (39%). Abnormal coronary anatomy was found in 4/35 patients. At the time of surgery, truncal valve repair was performed in 7 (19.4%) patients. During a mean follow-up time of 83±72 months, out of the 30 early survivors, 2 patients (6.6%) died due to non cardiac reasons and 1 patient (3.3%) underwent heart transplantation after severe failure of the right ventricle. Out of the 7 patients who had neonatal truncal valve repair, 5 survived the early postoperative period. A redo valve repair was needed in 2 patients, after 3.8 years and 6.1 years, due to severe valve insufficiency in a bicuspid and a quadricuspid truncal valve respectively. Subsequently one of them with quadricuspid valve underwent truncal valve replacement at 9 years due to severe dysplasia of all the cuspids. One patient had replacement of the native quadricuspid truncal valve after 6 years; in this case the morphology of the truncal valve contraindicated the valve plasty. Freedom from truncal valve repair or replacement was 94.7% at 5 years and 67% at 10 years. Early neonatal repair was independent predictor of reoperation (p=0.025). Overall actuarial survival rate was 82% and 79% at 1 and 5 years, respectively. Truncus arteriosus is a complex pathology affected by high incidence of mortality and morbidity. Native truncal valve repair is feasible. Early age at the time of repair represent an independent risk factor for reoperation without correlation with the valve morphology.

9 P97 ANGIOPLASTICA CON IMPIANTO DI STENT NELLA COARTAZIONE AORTICA: FOLLOW UP ANATOMICO E FUNZIONALE F. Corazza, A. Donti, R. Formigari, M.E. Mariucci, M. Bonvicini Ospedale Sant'Orsola, Cardiologia Pediatrica, Bologna, ITALY SCOPO DELLO STUDIO Finalità della ricerca è stata valutare i risultati e le complicanze a medio-lungo termine dell'angioplastica aortica con impianto di stent (ST-AO) in una popolazione di pazienti affetti da Coartazione Aortica. MATERIALI E METODI E' stata condotta un'analisi prospettica ed osservazionale su un campione di 45 pazienti sottoposti a ST-AO nel periodo compreso tra gennaio 2003 e giugno L'età media dei pazienti alla procedura era di 25±15 anni (54% <18). Il rapporto maschi/femmine era di 29/16. Il peso medio dei pazienti alla procedura era di 59±17 (31-97) kilogrammi. L'indicazione alla procedura è stata posta in accordo con le linee guida ESC sul trattamento dei pazienti GUCH E stato definito successo procedurale il riscontro di un gradiente di picco (GP) post-procedurale <20 mmhg (<10: successo completo; 11-20: successo parziale) e di un rapporto diametro finale minimo dello stent/aorta diaframmatica >0,5, e ostruzione residua un GP >20 mmhg e/o un rapporto diametro finale minimo dello stent/aorta diaframmatica >0,5 ma <0,8. Al follow up i pazienti sono stati rivalutati con controlli ambulatoriali periodici, Angio-TC, Holter pressorio e Treadmill Test. All'Angio-TC è stata definita riostruzione una riduzione del diametro interno dello stent, rispetto al diametro finale intra-procedurale, >10% (severa se >30%) e ricoartazione un rapporto diametro finale minimo intrastent/diametro dell'aorta diaframmatica <0,5. All'Holter pressorio e Treadmill Test, l'ipertensione arteriosa sistemica è stata definita in base a criteri stabiliti in letteratura, per pazienti adulti o pediatrici. RISULTATI La ST-AO è risultata efficacie nel 90% dei pazienti (80%: successo completo; 10%: successo parziale). Si è osservata ostruzione residua nel 10% dei pazienti (2/45): una CoAo addominale ed una dilatazione incompleta dello stent a causa di una complicanza intra-procedurale. Si sono verificate complicanze peri-procedurali minori nel 7% (3 pazienti), maggiori nel 4% (2 pazienti) dei casi. Non è stato registrato alcun decesso. Al follow up (durata media: 2±2 anni), l Angio-TC ha mostrato un mantenimento del successo procedurale nel 91% dei pazienti (comprendente un 7% di casi con riostruzione severa), ed incidenza di ricoartazione nel 9%. Si sono verificate complicanze a distanza nel 4% dei pazienti: una frattura di stent ed una dissezione circoscritta dell'aorta a monte dello stent, entrambe senza conseguenze emodinamiche. Al controllo ambulatoriale è risultato normoteso l'84% dei pazienti, di cui il 68% in terapia medica domiciliare (pazienti normotesi prima della procedura: 51%, di cui 38% in terapia); il 16% dei pazienti è rimasto iperteso nonostante la terapia. L'Holter pressorio (dati disponibili per il 51% dei pazienti) ha evidenziato valori pressori patologici nelle 24 ore nel 35% dei pazienti: il 60% dei pazienti sottoposti alla procedura in età pediatrica ed il 15% dei pazienti sottoposti in età adulta. Al Treadmill Test (dati disponibili per il 49% dei pazienti), il 18% dei pazienti ha mostrato ipertensione arteriosa al picco: il 50% dei pazienti sottoposti alla procedura in età pediatrica, ed il 50% dei pazienti sottoposti in età adulta. CONCLUSIONI La procedura di ST-AO è risultata efficacie e gravata da una bassa incidenza di complicanze. Al follow up, il successo procedurale si è mantenuto nel 90% (con una minoranza di casi, 7%, in cui si

10 documentava una significativa riduzione del lume intrastent). Un solo paziente ha presentato complicanze parietali, prive di significatività clinica/emodinamica. Dal punto di vista funzionale, la maggior parte dei pazienti (84%) è risultata normotesa al controllo ambulatoriale. Solo una minoranza (16%) è rimasta ipertesa nonostante la terapia. La valutazione con Holter pressorio ha riscontrato una maggior incidenza di ipertensione arteriosa (35%), mentre l ipertensione arteriosa da sforzo è risultata rara (18%).

11 P98 LA FUNZIONE CARDIACA NEI PAZIENTI CON SINDROME DI SHWACHMAN-DIAMOND E. MILANO 1, M.A. Prioli 1, G. Dolci 1, V. Benetti 1, M. Rebonato 1, R. Scarsini 1, E. Nicolis 2, S. Perobelli 3, C. Vassanelli 1, M. Cipolli 1 1 Università degli Studi di Verona, Cardiologia DU, Verona, 2 Laboratorio Patologia Molecolare, Centro Fibrosi Cistica, Azienda Ospedaliera Universitaria Integrata, Verona, 3 Centro Fibrosi Cistica, Azienda Ospedaliera Universitaria Integrata, Verona, ITALY Background: La sindrome di Shwachman-Diamond (SDS) è una malattia da insufficienza midollare congenita su base ereditaria autosomica recessiva caratterizzata da neutropenia, insufficienza del pancreas esocrino, anomalie scheletriche, bassa statura e ritardo cognitivo. In questi pazienti, sono state descritte complicanze cardiache letali e studi autoptici hanno evidenziato la presenza di quadri di necrosi e fibrosi miocardica, tipicamente osservati anche in altre condizioni come la fibrosi cistica. Altre complicanze cardiache non fatali sono state descritte nei pazienti SDS sottoposti a trapianto di midollo osseo. Più recentemente, sono state dimostrate alterazioni della funzione sistolica del ventricolo sinistro in particolare sotto sforzo. Questi dati suggeriscono come i pazienti affetti da sindrome di Shwachman-Diamond potrebbero aver un aumentata suscettibilità a un danno d organo a livello miocardico. Tuttavia pochi dati sulle modificazioni della funzione ventricolare in questi pazienti sono disponibili. Obiettivi dello studio: Valutare l anatomia e le variazioni della funzione cardiaca nei pazienti con SDS. Materiali e metodi: Ventidue pazienti affetti da SDS, con diagnosi confermata dalla presenza di mutazioni genetiche, sono stati sottoposti in modo prospettico a visita cardiologica, elettrocardiogramma con misurazione degli intervalli QT/QTc, asse R e T, ed ecocardiogramma transtoracico con valutazione dell anatomia cardiaca, della funzione sistolica e diastolica del ventricolo sinistro (FE, onda s TDI, e/a, e/e, Tei Index) e del ventricolo destro (TDI, TAPSE). Nessuno dei pazienti al momento della vista era stato sottoposto a trapianto di midollo osseo. I dati sono stati confrontati con i valori di normalità per sesso ed età. Risultati: Dei 22 pazienti (età media 11,9 anni, range 6 mesi anni, 13 maschi), tutti presentavano la mutazione 258+2T >C in almeno un allele, in omozigosi in 2 casi o in eterozigosi con altre mutazioni ( TA>CT, 187 G>T, 624+1G>C) nei restanti casi. Tutti i pazienti avevano un anamnesi negativa per problemi cardiaci e al momento delle visite nessuno presentava sintomi riferibili al cuore. A livello elettrocardiografico, frequenza, ritmo, intervallo PR, QRS, asse R e T sono risultati normali in tutti i casi; l intervallo QTc medio era ms. Tre pazienti presentavano un blocco di branca destro incompleto e una paziente anomalie della ripolarizzazione. Non sono state osservate anomalie strutturali nel gruppo dei pazienti SDS ad eccezione di una paziente che presentava una pervietà del forame ovale (6 anni) e di una paziente che presentava un accentuata trabecolatura apicale del ventricolo sinistro (36.7 anni). A livello ecocardiografico, la funzione ventricolare sistolica e diastolica sono risultate normali: la funzione ventricolare sinistra sistolica è apparsa nei limiti di norma con FE media stimata intorno al 61%, TDI dell anulus mitralico a livello laterale 8.3 cm/s e Tei Index La funzione diastolica è apparsa normale con e/a stimato 1.98, e/e 5.4 e DTE 150 ms. La funzione ventricolare destra è risultata normale. I dati sono riassunti in Tabella 1. Conclusioni: I bambini e i giovani adulti affetti da SDS con mutazione del gene SBDS presentano una normale anatomia cardiaca e l incidenza di cardiopatie congenite è sovrapponibile a quella della popolazione generale. La funzione biventricolare sistolica e diastolica valutate ecograficamente risultano normali prima del trapianto di midollo osseo. Vista la rarità della patologia e delle manifestazioni cardiologiche, sarebbero necessari ulteriori approfondimenti con test da sforzo e risonanza magnetica per valutare l eventuale presenza di anomalie strutturali o funzionali latenti in questi pazienti. <FILE IMAGE='683_ jpg'>

12 P99 LONG- TERM EFFECTS OF BOSENTAN THERAPY IN PATIENTS WITH EISENMENGER SYNDROME: FIVE YEARS FOLLOW-UP B. Tchana, V. Allegri, N. Carano, A. Raymond, C. Cavalli, I. Bo, M. Caffarra, M. Sgrignoli, C. Macchi, C. Greco, L. Costi, A. Agnetti Pediatric Cardiology Unit, Department of Pediatrics, Parma University Hospital, Parma, ITALY BACKGROUND: Treatment with endothelin receptor antagonist improves symptoms, hemodynamics, functional capacity and quality of life in patients with Eisenmenger syndrome (ES). Bosentan, a safe orally active dual endothelin-receptor antagonist, has shown to be effective in short - term treatment in patients with pulmonary arterial hypertension associated with congenital heart disease, mainly by improving short-term exercise capacity. The aim of the present study is to assess the long-term efficacy and clinical benefits of bosentan monotherapy in ES patients. MATERIALS AND METHODS: 11 consecutive patients with ES, including four with Down Syndrome (DS), with the following congenital heart diseases were enrolled for treatment with bosentan: wide ventricular septal defect (3 patients), atrio ventricular canal (3 patients), pulmonary atresia, ventricular septal defect and MAPCA S (2 patients), double outlet right ventricle and ventricular septal defect (1 patient), and atrial septal defect and wide ventricular septal defect (1 patient). Their mean age at the start of treatment was 33.8 years. All patients were evaluated at baseline and during follow-up collecting clinical information with WHO functional class, systemic blood pressure, resting systemic arterial oxygen saturation (SaO2) by means of a finger pulse oximeter, performing laboratory tests, six-minute walking test (6-MWT) with heart rate and SaO2 recorded at rest and after exercise, Doppler echocardiography, strain, strain rate. RESULTS: The mean duration of treatment was 58,3 months (range months). All patients received 125 mg after the initial titration period. Bosentan therapy was generally well tolerated. No deaths or any serious adverse drug reactions were noted. Throughout the observation period AST and ALT plasma levels remained <3-fold the upper limit of normal in all but 1 patient who showed an elevation of aminotransferase levels 3 to 4 times the upper limit of normal; the dose of bosentan was reduced from 125 to 62.5 mg twice daily with complete normalization of aminotransferase levels and the patient continued receiving bosentan 62.5 mg twice daily during follow-up. WHO functional class (3.2 ± 0.4 to 2.5 ± 0.6), the 6-MWT showed an increase from baseline, of 47,2 meters (p =0.05) after 6 months and of 45,3 meters (p <0.05) after 12 months, maintained during the follow-up. Pulse oximetry remained unchanged at rest but increased at the end of 6MWT. No significant changes were found in heart rate, systemic blood pressure. In general, most echocardiographic parameters remained unchanged. However, analyses of the right ventricle showed a significant improvement in RV MPI (0.48 ± 0.17 to 0.36 ± 0.08). Left ventricle presented in an increase in mean stroke volume ( ml). In one patient with Down sindrome, after 6 months of treatment a low velocity retrograde flow appeared on doppler in main pulmonary artery, probably a ductus never report before. Regarding quality of life, all patients, except Down Syndrome patients, reported an improvement in daily life limitations caused by physical health problems and physical functioning, improvement that seemed to persist throughout the follow-up. CONCLUSIONS: Our experience confirmed that long-term bosentan monotherapy is safe, effective, and well tolerated in patients with ES, with a prolonged beneficial effect on exercise capacity. However our study presents several limitations, the sample size, the heterogeneity of underlying diagnoses (Down Syndrome, variety of cardiac defects), the lack of post treatment hemodynamics data.

13 P100 STORIA NATURALE DEI DIFETTI INTERVENTRICOLARI ISOLATI IN UNA POPOLAZIONE PEDIATRICA G. Porcedda 1, B. Castaldi 2, A. Di Palma 1, O. Milanesi 2 1 Pediatria APSS-Trento, Azienda Provinciale per i Servizi Sanitari, Trento, 2 Cardiologia Pediatrica, Dipartimento di Pediatria, Padova, ITALY Il difetto interventricolare (DIV) è la più comune cardiopatia congenita, con una prevalenza tra i neonati pari allo 0.5-1%. Sebbene esistano linee guida per il trattamento dei difetti congeniti dei pzadulti, queste raccomandazioni non possono essere estese all età pre-scolare, data l evolutività delle dimensioni del difetto e delle manifestazioni cliniche. Scopo dello studio è di valutare ad un lungo follow-up i pazienti affetti da DIV isolato, identificando il tasso di chiusura spontanea, la percentuale di soggetti in cui è necessario l intervento e la prevalenza di complicanze adducibili al DIV. Metodi: Sono stati inclusi nello studio 601 pazienti consecutivi (45% maschi), affetti da DIV isolato di età alla prima visita compresa tra 0 e 2 anni, sottoposti ad almeno 2 controlli ambulatoriali successivi con visita completa, ECG ed ecocardiogramma. La durata media di follow-up è di 6 anni. I difetti sono stati raggruppati per sede anatomica secondo la classificazione di Gatzoulis. I DIV sono stati suddivisi in piccoli, di medie ed ampie dimensioni in funzione del rapporto tra diametro del difetto e dell anulus aortico. Risultati: Il DIV era a sede perimembranosa nel 47% dei casi, muscolare nel 49% e sottopolmonare nel 4% dei pazienti. Il 63% era di piccole dimensioni, il 20 % di medie dimensioni e il 17% ampi. L 80% dei pazienti era asintomatico, il 20 % presentava uno o più dei seguenti sintomi: 14% scompenso cardiaco, 4% ridotta crescita, 15% ipertensione polmonare, 7,5% infezioni polmonari ricorrenti. Nel 55% dei pazienti (288pz) il DIV si è chiuso spontaneamente, il 33% (203 pz) ha necessitato di correzione chirurgica. La chiusura spontanea è avvenuta nel 31% dei DIV perimembranosi, nell 80% dei muscolari e nel 25% di quelli sub-polmonari. Tra quelli chiusi spontaneamente, l 87% avevano piccole dimensioni, 31,3% medie, 3% erano ampie. La chiusura chirurgica è stata riservata soprattutto ai pazienti con DIV perimembranoso (83%) e sottopolmonare (72%), mentre tra quelli muscolari è stata necessaria solo nell 8,8% dei casi. L indicazione chirurgica è stata posta per DIV ampio e scompenso cardiaco nel 77% dei pz (158), in 20 pazienti (10%) per la comparsa di insufficienza aortica, in 10 per ostruzione sub-aortica (5%) e in 8 per ostruzione all efflusso ventricolare destro (4%). Tra le sequele chirurgiche, la più frequente risulta lo shunt residuo (29 pz, 14%), di cui uno ha necessitato di re-intervento (0,5%). Due pazienti (1%) hanno richiesto un pacemaker per blocco atrioventricolare completo. Il blocco di branca destro post-chirurgico è presente nel 53% dei casi. In 6 pazienti (3%) è comparsa una neo-insufficienza aortica dopo intervento. Non si sono registrati episodi di endocardite batterica. Conclusioni: la chiusura spontanea dei DIV avviene in oltre la metà dei pazienti, più frequentemente nei difetti muscolari e in quelli di piccole dimensioni, ma è possibile anche nei DIV ampi. L insufficienza aortica è stata indicazione all intervento chirurgico nel 10% dei casi. Le principali sequele chirurgiche sono state la presenza di shunt residuo (14%), la comparsa d insufficienza aortica (3%) e la necessità di impiantare un pacemaker (1%). Le complicanze in storia naturale dei difetti interventricolari sono state estremamente rare e di modesta entità, pertanto riteniamo che in base ai dati da noi ottenuti vi sono indicazioni a favore di una cauta attesa e uno stretto follow up di questi pazienti, riservando la chirurgia precoce solo ai casi di chiaro scompenso cardiaco o di ipertensione polmonare.

14 P101 EXCERCISE CAPACITY AND STRESS ECHOCARDIOGRAPHY LONG TERM AFTER ARTERIAL SWITCH OPERATION FOR TRANSPOSITION OF THE GREAT ARTERIES AND VENTRICULAR SEPTAL DEFECT OPERATION F. Del Gaizo 1, C. Ricci 1, A. Rea 1, M. Conte 1, R. Esposito 1, G. Di Salvo 1, N. Di Virgilio 1, R. Sorrentino 1, G. Caianiello 2, M.G. Russo 1 1 Cardiologa Pediatrica AORN dei Colli, AO Monaldi, SUN Napoli, ITALY, 2 Cardiochirurgia Pediatrica, AORN dei Colli, AO Monaldi, Napoli, ITALY BACKGROUND: The arterial switch operation (ASO) is currently the treatment of choice in neonates with transposition of the great arteries (TGA). The outcome in childhood is encouraging but only limited data for long-term outcome into adulthood exist. Patients who undergo the ASO are at risk of reduced exercise capacity, with most reports focusing on chronotropic incompetence as the cause. We wanted to assess the feasibility and the physiologic responses of stress echocardiography and Cardiopulmonary test in 15 patients that underwent ASO in neonatal age. The results were compared with that of 15 patients that underwent heart surgery for ventricular septal defect (VSD) under 1 year of age. METHODS: 30 patients were reviewed for functional capacity and morphologic assessment of the left ventricle. 15 patients that underwent ASO for TGA (all had simple TGA with intact ventricular septum), and of 15 patients that underwent heart surgery for ventricular septal defect (VSD) under 1 year of age. The two groups were comparable for: age, gender, BSA and physical activity. The two groups were evaluated with: - rest EKG 12-leads; -12-leads Holter EKG 24 hours,; -Transthoracic Echocardiography at rest performed with Toshiba Artida Aplio 500 (5 MHz): parasternal views: parasternal views short and long axis; apical views: chambers; - Systolic and Diastolic function evaluated with DTI Physical stress Echocardiography performed with GE Vivid 7 (5 MHz): parasternal views: long axis; apical views: chambers; -Cardiopulmonary test. RESULTS: Both groups had not significative alteration of ST-T at EKG; few patients with TGA and more patients with VSD had right bundle branch block (3/15; 20% vs 7/15; 47%). 3/15 (20%) VSD had BAV I. There were no major arrhythmias at holter ECG evaluation. Echocardiographic parameters at rest as FE, FS, and VSX dimensions were normal in both groups. The systo-diastolic function was normal in ASO group, while the VSD group had a reduced myocardial velocity in at basal interventricular septum, compatibly with the type of operation. At physical stress echocardiography: in the ASO group, 10 (67%) had normal motion at basal and during stress, 5 (33%) with interventricular septum dyskinesia showed normal motion during stress. In the VSD group: 10 (67%) had interventricular septum dyskinesia at rest that did not recupered normal motion during stress, and 5 (33%) had normal kynesis. 7 (47%) patients operated for TGA showed an increased gradient (>30 mmhg) at right ventricle outflow tract during stress. At cardiopulmonary test: the VO2 peak, during, was normal (> 84%) in operated VSD. In 10 (67%) pz after ASO the peak of VO2 was decreased. This is compatible with a residual right ventricle outflow tract obstruction (> 30 mmhg CW gradient). Conclusions: Aim of our study was to compare two groups of patients that underwent to CEC for different congenital heart disease: patients operated for VSD and for TGA. Patients operated for VSD often showed a reduced wall motion at basal and during stress. TGA operated showed an increased RVOT obstruction during stress, that is a determinant for exercise capacity.

15 P102 BASELINE HEMODYNAMICS OF PATIENTS DEVELOPING PULMONARY ARTERIAL HYPERTENSION AFTER SHUNT CLOSURE DURING LONGTERM FOLLOW-UP E. Romeo, M. D'Alto, P. Argiento, B. Sarubbi, G. Santoro, G. Gaio, R. Calabrò, M.G. Russo Azienda Ospedaliera Monaldi, Ospedale dei Colli, Napoli, ITALY Objective: pulmonary arterial hypertension (PAH) after shunt closure is very severe condition with poor prognosis. Aim of this study was to retrospectively analyze the baseline hemodynamics (before surgery/interventional procedures) of patients developing PAH after a shunt closure. Methods: Right heart catheterization (RHC) was performed at baseline (before shunt closure) and during the follow-up. Results: 28 patients (20 female) with PAH after shunt closure (age to closure 26±21 y, age to PAH diagnosis 39±20y) have been considered. Six/28 did not have hemodynamic evaluation at baseline. Of remaining 22 patients, 8 had atrial septal defect (ASD), 11 ventricular septal defect (VSD), 1 patent ductus arteriosus, 1 ASD and VSD, and 1 atrio-ventricular septal defect. Non patient showed right heart failure immediately after the shunt closure. The time between shut closure and PAH diagnosis was 13±8 y. Table 1 shows hemodynamic data at baseline and at PAH diagnosis. At baseline evaluation 20/22 patients (90%) had a PVRi >6 Woods units/m2, 21/22 (95%) had a PVRi/SVRi >0,3, and 10/22 (45%) had a QP/QS<1,5. Conclusions: basal PVRi >6 Woods units/m2 and PVRi/SVRi >0,3 are more predictive of late development of PAH after shunt closure than QP/QS ratio <1.5. <FILE IMAGE='378_ JPG'>

16 P103 BALLON AORTIC VALVULOPLASTY IN NEONATES: PROCEDURAL RESULTS AND LONG TERM FOLLOW-UP G. Gaio, G. Santoro, C. Capogrosso, S. Scafuri, M. Carrozza, C. Iacono, M.T. Palladino, G. Caianiello, M.G. Russo Cardiology and Cardiac Surgery, Monaldi Hospital, 2nd University of Naples, Naples, ITALY BACKGROUND. Balloon aortic valvuloplasty (BAV) is an option therapy for congenital aortic stenosis (AS) in neonates. Few reports describe also a long-term outcomes. In this study, a retrospective single-institution review was performed of patients who underwent BAV for congenital neonatal AS. METHODS. From 2000 to 2012, 28 neonates underwent BAV at ages 1 day to 30 days. The mean follow-up was months. The following end points were evaluated: procedural immediate results, aortic insufficiency (AI), need of surgery, repeat BAV and death. RESULTS. The procedure was completed in all patients. In 26 patients (93%) the procedure was considered successful, with a significant reduction of the pressure gradient (from mmhg to mmhg p<0.003). in 2 patients the procedure was failure due to residual severe aortic stenosis. At last follow up: 3/28 patients (10.7%) needed surgery due to the residual steno-insufficiency post BAV; death occurred in 2/28 patients (7%, 1 of them after BAV and 1 after surgery). No patients needed a second balloon valvuloplasty. Therfore, 18 patients are in natural history and among them at last follow-up control the residual stenosis was mild in 14/18 patients (77.7%), moderate in 4/18 (22.3%), while the residual insufficiency was mild in 16/18 patients (88.8%) and moderate in 2 /18 (11.2%). None had a residual severe stenosis or insufficiency. CONCLUSIONS. Balloon valvuloplasty of aortic valve stenosis is a feasible procedure that significantly reduces gradient in the most majority of the patients. In our series no patients, at long term follow up, had a severe residual stenosis/insufficiency. Only 10% needed a surgical treatment after the balloon valvuloplasty. Mortality rate was 7%.

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