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Marfan syndrome (MIM: # 154700; MFS) is an autosomal dominant disease representing the most common form of heritable connective tissue disorder. The condition presents variable multiorgan expression, typically involving a triad of cardiovascular, eye, and skeletal manifestations. Other multisystemic features are often underdiagnosed. Moreover, the disease is characterized by age related penetrance. Diagnosis and management of MFS in the adult population are well-described in literature. Few studies are focused on MFS in the pediatric population, making the clinical approach (cardiac and multiorgan) to these cases challenging both in terms of diagnosis and serial follow-up. In this review, we provide an overview of MFS manifestations in children, with extensive revision of major organ involvement (cardiovascular ocular and skeletal). We attempt to shed light on minor aspects of MFS that can have a significant progressive impact on the health of affected children. MFS is an example of a syndrome where an early personalized approach to address a dynamic, genetically determined condition can make a difference in outcome. Applying an early multidisciplinary clinical approach to MFS cases can prevent acute and chronic complications, offer tailored management, and improve the quality of life of patients.
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The ideal aortic valve substitute for young and middle-aged adults with aortic valve disease is still debated. The Ross procedure (pulmonary autograft replacement) is the only operation that allows replacement of the diseased aortic valve with a living substitute. During the years, the use of this procedure has declined significantly due to concerns over increased intraoperative risk and especially potential long-term failure of the operation. However, several recent publications have shown that, after appropriate learning curve and in specialized centers, the Ross procedure can be performed safely in appropriately selected patients. Moreover, comparative studies suggest that the Ross procedure is associated with better long-term outcomes compared to conventional aortic valve replacement in young and middle-aged adults.
Sujet(s)
Insuffisance aortique , Prothèse valvulaire cardiaque , Valve du tronc pulmonaire , Adulte , Adulte d'âge moyen , Humains , Autogreffes , Résultat thérapeutique , Transplantation autologue , Valve aortique/chirurgie , Insuffisance aortique/chirurgie , Valve du tronc pulmonaire/chirurgie , Réintervention , Études rétrospectives , Études de suiviRÉSUMÉ
Background: The clinical impact of valvular heart disease (VHD) in adult congenital heart disease (ACHD) patients is unascertained. Aim of our study was to assess the prevalence and clinical impact of severe VHD (S-VHD) in a real-world contemporary cohort of ACHD patients. Materials and methods: Consecutive patients followed-up at our ACHD Outpatient Clinic from September 2014 to February 2021 were enrolled. Clinical characteristics and echocardiographic data were prospectively entered into a digitalized medical records database. VHD at the first evaluation was assessed and graded according to VHD guidelines. Clinical data at follow-up were collected. The study endpoint was the occurrence of cardiac mortality and/or unplanned cardiac hospitalization during follow-up. Results: A total of 390 patients (median age 34 years, 49% males) were included and S-VHD was present in 101 (25.9%) patients. Over a median follow-up time of 26 months (IQR: 12-48), the study composite endpoint occurred in 76 patients (19.5%). The cumulative endpoint-free survival was significantly lower in patients with S-VHD vs. patients with non-severe VHD (Log rank p < 0.001). At multivariable analysis, age and atrial fibrillation at first visit (p = 0.029 and p = 0.006 respectively), lower %Sat O2, higher NYHA class (p = 0.005 for both), lower LVEF (p = 0.008), and S-VHD (p = 0.015) were independently associated to the study endpoint. The likelihood ratio test demonstrated that S-VHD added significant prognostic value (p = 0.017) to a multivariate model including age, severe CHD, atrial fibrillation, %Sat O2, NYHA, LVEF, and right ventricle systolic pressure > 45 mmHg. Conclusion: In ACHD patients, the presence of S-VHD is independently associated with the occurrence of cardiovascular mortality and hospitalization. The prognostic value of S-VHD is incremental above other established prognostic markers.
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OBJECTIVES: The aim of this study was to elucidate predictors of death and reintervention after mitral valve (MV) surgery in children. METHODS: A single-centre retrospective study was performed enrolling 142 patients younger than 18 years who underwent primary index surgical mitral repair or replacement at Bambino Gesù Children's Hospital in Rome from July 1982 to April 2020. Patients with complete, transitional or partial atrioventricular septal defect and patients with single ventricle physiology were excluded. Patients were stratified according to the age group: group 1 (<1 year old), group 2 (1-5 years old) and group 3 (>5 years old). The composite primary outcome was freedom from death or transplant. The secondary outcome was freedom from redo MV surgery. RESULTS: Transplant-free survival was 89% at 5 years and 88% at 10 years. Stratified by age, group 1 had poorer outcome in comparison with other groups (log-rank test P = 0.105). Both univariate and multivariate analyses showed that age <1 year was a significant risk factor for death or transplant (P = 0.044). Age <1 year was associated with increased risk of reoperation (aHR = 3.38, P = 0.009), while the presence of genetic syndrome (aHR = 0.22) and preoperative EF% (aHR = 0.97) were protective factors for reoperation. CONCLUSIONS: The overall survival and freedom from reoperation in children undergoing MV surgery still need improvements. Younger age was a significant risk factor for death and reintervention both after repair and replacement of the MV. In particular, infants and neonates have a three-fold risk for death compared to children.
Sujet(s)
Procédures de chirurgie cardiaque , Implantation de valve prothétique cardiaque , Insuffisance mitrale , Enfant , Enfant d'âge préscolaire , Études de suivi , Implantation de valve prothétique cardiaque/effets indésirables , Humains , Nourrisson , Nouveau-né , Valve atrioventriculaire gauche/chirurgie , Insuffisance mitrale/chirurgie , Réintervention , Études rétrospectives , Résultat thérapeutiqueRÉSUMÉ
BACKGROUND: Right ventricle-pulmonary artery (RV-PA) coupling is a strong prognostic marker in several clinical settings, but few studies have focused on its role in repaired tetralogy of Fallot (rToF) with pulmonary regurgitation. AIM: To assess whether differences exist in RV-PA coupling, estimated by echocardiography, between patients with rToF and pulmonary regurgitation with or without an indication for pulmonary valve replacement (PVR). METHODS: The study population included 40 patients with rToF, who were allocated to two groups: 20 with an indication for PVR (i-PVR group); and 20 without an indication for PVR (ni-PVR group). Forty healthy controls were also included. All subjects underwent echocardiography, and cardiac magnetic resonance (CMR) was available in 27/40 patients with rToF. RV-PA coupling was assessed by echocardiographic tricuspid annular plane systolic excursion/pulmonary arterial systolic pressure (TAPSE/PASP) and right ventricular stroke volume/right ventricular end-systolic volume (RVSV/RVESV) by CMR. RESULTS: TAPSE was similar in the i-PVR and ni-PVR groups (19.0±3.4 vs 18.8±2.7mm; P=0.97) whereas RV-PA coupling was significantly worse in the i-PVR group versus the ni-PVR group (TAPSE/PASP 0.8±0.3 vs 1.1±0.5mm/mmHg; P=0.001), and in the i-PVR group versus the control group (P=0.02); there was no difference between the ni-PVR and control groups (P=0.29). CMR data confirmed the echocardiography results, with a significant difference in RV-PA coupling between the i-PVR and ni-PVR groups (RVSV/RVESV 0.9±0.2 vs 1.2±0.3mL/min/mL; P=0.01). CONCLUSIONS: This study demonstrates worse RV-PA coupling, despite normal RV systolic function, in patients with rToF with an indication for PVR. RV-PA coupling could be a sensitive marker of a progressive maladaptive RV response to long-standing volume overload in rToF before the onset of clinical symptoms and RV systolic dysfunction.
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Insuffisance pulmonaire , Valve du tronc pulmonaire , Tétralogie de Fallot , Dysfonction ventriculaire droite , Ventricules cardiaques/imagerie diagnostique , Humains , Artère pulmonaire/imagerie diagnostique , Artère pulmonaire/chirurgie , Valve du tronc pulmonaire/imagerie diagnostique , Valve du tronc pulmonaire/chirurgie , Insuffisance pulmonaire/imagerie diagnostique , Insuffisance pulmonaire/étiologie , Insuffisance pulmonaire/chirurgie , Tétralogie de Fallot/imagerie diagnostique , Tétralogie de Fallot/chirurgie , Fonction ventriculaire droiteRÉSUMÉ
Aortic reconstruction at the time of the comprehensive stage II (CSII) procedure can be complicated by compression within the aortopulmonary space resulting in airway or pulmonary artery narrowing. We describe our experience with 2 patients with hypoplastic left heart syndrome and pulmonary artery stenosis after the CSII procedure. Both patients underwent an aortic extension with a Hemashield interposition graft to open up the aortopulmonary space. The patients were discharged from the hospital. In all cases the aortopulmonary space was enlarged, and the pulmonary arteries and airway were free from compression. Aortic extension is an option to be considered in children with pulmonary artery compression who previously had a CSII procedure.
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Hypoplasie du coeur gauche , Aorte/imagerie diagnostique , Aorte/chirurgie , Enfant , Humains , Hypoplasie du coeur gauche/imagerie diagnostique , Hypoplasie du coeur gauche/chirurgie , Soins palliatifs/méthodes , Artère pulmonaire/imagerie diagnostique , Artère pulmonaire/chirurgie , Études rétrospectives , Résultat thérapeutique , Procédures de chirurgie vasculaireRÉSUMÉ
BACKGROUND: Progressive right heart chambers dilatation is frequent in the adult congenital heart disease (ACHD) population. We evaluated the immediate and mid-term response of right heart chambers to surgery performed in adulthood for lesions associated with right heart chambers enlargement. METHODS: Thirty-six adult patients with lesions associated with right heart chambers enlargement submitted to surgery were studied. We collected echocardiographic data of right ventricle (RV) mid-diameter, right atrial volume indexed, RV systolic pressure, and tricuspid annular plane systolic excursion (TAPSE) prior to surgery (T0), at 2 to 5 days (T1), and 3 to 6 months (T2) after surgery. RESULTS: At T1, we observed a significant decrease of RV mid-diameter (47.2 ± 8.4 vs. 39.6 ± 7.4 mm, P < .001), right atrial volume indexed (45.6 ± 26.6 vs. 27.2 ± 11 ml/m2, P < .001), and RV systolic pressure (39 ± 14.8 vs. 32.8 ± 11.3â mmâ Hg, P = .03). At T2, a further significant deviation in the rate of RV diameter (39.6 ± 7.4 vs. 34.5 ± 5.1 mm, P < .001), in RV systolic pressure (32.8 ± 11.3 vs. 25.3 ± 5â mmâ Hg, P = .03) and TAPSE (13.9 ± 3.2 vs. 15.8 ± 2.6 mm, P < .001) was observed. CONCLUSIONS: Positive right heart chambers remodeling occurs as early as in the immediate post-operative period in most ACHD patients operated for lesions associated with right heart chambers enlargement.
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Cardiopathies congénitales , Dysfonction ventriculaire droite , Adulte , Échocardiographie , Cardiopathies congénitales/imagerie diagnostique , Cardiopathies congénitales/chirurgie , Ventricules cardiaques/imagerie diagnostique , Ventricules cardiaques/chirurgie , Humains , Fonction ventriculaire droiteRÉSUMÉ
An alternative strategy for left ventricular (LV) venting during short-term mechanical circulatory support is use of Impella axial-flow pump. We present our transcarotid Impella 2.5 implantation technique using a polytetrafluoroethylene graft, in two children with acute heart failure treated primarily with venoarterial ECMO. The venoarterial extracorporeal membrane oxygenator and Impella support were maintained for 5 and 17 days, respectively. Transcarotid Impella implantation might be an alternative and feasible option in pediatrics patients affected by severe LV failure, as a bridge to decision or bridge to candidacy. Potentially, the Impella 2.5 device provides less invasive support for children with heart failure.
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Oxygénation extracorporelle sur oxygénateur à membrane , Défaillance cardiaque , Dispositifs d'assistance circulatoire , Pédiatrie , Enfant , Défaillance cardiaque/thérapie , Ventricules cardiaques , HumainsRÉSUMÉ
AIM: To summarize our experience on the implementation of a telemedicine service dedicated to adult congenital heart disease (ACHD) patients during the lockdown for the first wave of Coronavirus disease 2019 (COVID-19). METHODS: This is a prospective study enrolling all ACHD patients who answered a questionnaire dedicated telematic cardiovascular examination. RESULTS: A total of 289 patients were enrolled, 133 (47%) were male, 25 (9%) were affected by a genetic syndrome. The median age was 38 (29-51) years, whereas the median time interval between the last visit and the telematic follow-up was 9.5 (7.5-11.5) months. Overall, 35 patients (12%) reported a worsening of fatigue in daily life activity, 17 (6%) experienced chest pain, 42 (15%) had presyncope and 2 (1%) syncope; in addition, 28 patients (10%) presented peripheral edema and 14 (5%) were orthopneic. A total of 116 (40%) patients reported palpitations and 12 had at least one episode of atrial fibrillation and underwent successful electrical (8) or pharmacological (4) cardioversion. One patient was admitted to the emergency department for uncontrolled arterial hypertension, five for chest pain, and one for heart failure. Two patients presented fever but both had negative COVID-19 nasal swab. CONCLUSION: During the COVID-19 pandemic, the use of telemedicine dramatically increased and here we report a positive experience in ACHD patients. The postpandemic role of telemedicine will depend on permanent regulatory solutions and this early study might encourage a more systematic telematic approach for ACHD patients.
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COVID-19 , Cardiopathies congénitales , Prévention des infections , Gestion des soins aux patients , Préférence des patients/statistiques et données numériques , Télémédecine , Adulte , COVID-19/épidémiologie , COVID-19/prévention et contrôle , Femelle , Cardiopathies congénitales/épidémiologie , Cardiopathies congénitales/physiopathologie , Cardiopathies congénitales/thérapie , Humains , Prévention des infections/méthodes , Prévention des infections/organisation et administration , Italie/épidémiologie , Mâle , Évaluation des résultats et des processus en soins de santé , Gestion des soins aux patients/méthodes , Gestion des soins aux patients/statistiques et données numériques , Études prospectives , SARS-CoV-2 , Enquêtes et questionnaires , Évaluation des symptômes/méthodes , Télémédecine/méthodes , Télémédecine/organisation et administrationRÉSUMÉ
OBJECTIVES: Management of resternotomy is often a difficult challenge in patients with congenital diseases who have undergone multiple sternotomies. Our goal was to report our single-centre experience with carotid cannulation using a graft interposed during redo sternotomy in complex cardiac reintervention procedures. METHODS: We performed a retrospective review of all patients who had undergone complex redo sternotomies between January 2019 and May 2020 utilizing a cervical cannulation technique with a Gore-Tex graft interposed on the carotid artery. We classified our population study on the basis of the primary diagnosis and the type of surgery. The primary outcomes of our analysis were the evaluation of the safety of the procedure in terms of survival and freedom from neurological events. RESULTS: We analysed 22 patients who had undergone previous complex operations. The median age and weight at the time of reintervention were 130.35 (range 0.46-435) months and 31.5 (range 2.2-85) kg, respectively. Composite graft carotid cannulation provided adequate arterial flow in all patients with a median arterial flow of 3.5 l/min/m2 (range 0.6-6). One major cardiac injury occurred during sternotomy when emergency cardiopulmonary bypass (CPB) was initiated. Moreover, during their hospital stays, all patients had an uneventful recovery without neurological or vascular complications and no cervical wound infections. CONCLUSIONS: Carotid cannulation using interposition of a side graft on the common carotid artery for arterial inflow is a reliable and safe method for initiation of CPB in complex redo surgeries in patients with congenital disease. Complications directly associated with this type of cannulation are uncommon and allow surgical re-entry with overall low risks.
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Procédures de chirurgie cardiaque , Sternotomie , Procédures de chirurgie cardiaque/effets indésirables , Pontage cardiopulmonaire , Artère carotide commune , Cathétérisme , Humains , Réintervention , Études rétrospectives , Sternotomie/effets indésirablesSujet(s)
Communications interauriculaires , Communications interventriculaires , Adulte , Communications interauriculaires/imagerie diagnostique , Communications interauriculaires/chirurgie , Communications interventriculaires/imagerie diagnostique , Communications interventriculaires/chirurgie , HumainsSujet(s)
Flutter auriculaire/étiologie , Anastomose chirurgicale de Blalock-Taussig/effets indésirables , Retard de diagnostic , Hypertension pulmonaire/diagnostic , Insuffisance pulmonaire/diagnostic , Tétralogie de Fallot/chirurgie , Dysfonction ventriculaire droite/diagnostic , Flutter auriculaire/diagnostic , Flutter auriculaire/physiopathologie , Flutter auriculaire/chirurgie , Ablation par cathéter , Circulation coronarienne , Évolution de la maladie , Femelle , Hémodynamique , Humains , Hypertension pulmonaire/étiologie , Hypertension pulmonaire/physiopathologie , Adulte d'âge moyen , Soins palliatifs , Valeur prédictive des tests , Pronostic , Circulation pulmonaire , Insuffisance pulmonaire/étiologie , Insuffisance pulmonaire/physiopathologie , Récidive , Dysfonction ventriculaire droite/étiologie , Dysfonction ventriculaire droite/physiopathologieSujet(s)
Malformations multiples , Aorte thoracique/malformations , Malformations cardiovasculaires/diagnostic , Artère carotide commune/malformations , Syndrome de DiGeorge/diagnostic , Artère subclavière/malformations , Anomalies vasculaires/diagnostic , Aorte thoracique/imagerie diagnostique , Artère carotide commune/imagerie diagnostique , Angiographie par tomodensitométrie/méthodes , Humains , Imagerie tridimensionnelle , Nourrisson , MâleRÉSUMÉ
BACKGROUND: Atrial septal defect and Impella have been proposed for left ventricular unloading in venoarterial extracorporeal membrane oxygenation patients. This work aims at evaluating the haemodynamic changes in venoarterial extracorporeal membrane oxygenation patients after Impella implantation or atrial septal defect realization by a simulation study. METHODS: A lumped parameter model of the cardiovascular system was adapted to this study. Atrial septal defect was modelled as a resistance between the two atria. Venoarterial extracorporeal membrane oxygenation and Impella were modelled starting from their pressure-flow characteristics. The baseline condition of a patient undergoing venoarterial extracorporeal membrane oxygenation was reproduced starting from haemodynamic and echocardiographic data. The effects of different atrial septal defect size, Impella and venoarterial extracorporeal membrane oxygenation support were simulated. RESULTS: Impella caused an increment of mean arterial pressure up to 67%, a decrement in mean pulmonary arterial pressure up to 8%, a decrement in left ventricular end systolic volume up to 11% with a reduction up to 97% of left ventricular cardiac output. Atrial septal defect reduces left atrial pressure (19%), increases right atrial pressure (22%), increases mean arterial pressure (18%), decreases left ventricular end systolic volume (11%), increases right ventricular volume (33%) and decreases left ventricular cardiac output (55%). CONCLUSION: Impella has a higher capability in left ventricular unloading during venoarterial extracorporeal membrane oxygenation in comparison to atrial septal defect with a lower right ventricular overload.
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Simulation numérique , Oxygénation extracorporelle sur oxygénateur à membrane , Atrium du coeur/physiopathologie , Communications interauriculaires/thérapie , Ventricules cardiaques/physiopathologie , Échocardiographie , Communications interauriculaires/physiopathologie , Hémodynamique/physiologie , Humains , Modèles cardiovasculairesRÉSUMÉ
OBJECTIVES: Adults with congenital heart disease (ACHD) are a growing group with end-stage heart failure. We aim to describe the outcomes of ACHD patients undergoing assessment for orthotopic heart transplant (OHT). METHODS: Case notes of consecutive ACHD patients (>16 years) assessed for OHT between 2000 and 2016 at our centre were reviewed. Decision and outcome were reported as of 2017. Data were analysed in three groups: systemic left ventricle (LV), systemic right ventricle (RV) and single ventricle (SV). RESULTS: 196 patients were assessed (31.8 years, 27% LV, 29% RV, 44% SV). 89 (45%) patients were listed for OHT and 67 (34%) were transplanted. 41 (21%) were unsuitable or too high risk and 36 (18%) were too well for listing. Conventional surgery was undertaken in 13 (7%) and ventricular assist device in 17 (9%) with 7 (4%) bridged to candidacy. Survival from assessment was 84.2% at 1 year and 69.7% at 5 years, with no difference between groups. Patients who were considered unsuitable for OHT (HR 11.199, p<0.001) and listed (HR 3.792, p=0.030) were more likely to die than those who were considered too well. Assessments increased over the study period. CONCLUSIONS: The number of ACHD patients assessed for OHT is increasing. A third are transplanted with a small number receiving conventional surgery. Those who are unsuitable have a poor prognosis.
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Cardiopathies congénitales/complications , Défaillance cardiaque/chirurgie , Transplantation cardiaque , Ventricules cardiaques/chirurgie , Survivants , Adulte , Prise de décision clinique , Évolution de la maladie , Femelle , Cardiopathies congénitales/imagerie diagnostique , Cardiopathies congénitales/physiopathologie , Défaillance cardiaque/imagerie diagnostique , Défaillance cardiaque/étiologie , Défaillance cardiaque/physiopathologie , Transplantation cardiaque/effets indésirables , Ventricules cardiaques/malformations , Ventricules cardiaques/imagerie diagnostique , Ventricules cardiaques/physiopathologie , Humains , Mâle , Adulte d'âge moyen , Sélection de patients , Études rétrospectives , Appréciation des risques , Facteurs de risque , Résultat thérapeutique , Fonction ventriculaire gauche , Fonction ventriculaire droite , Listes d'attente , Jeune adulteRÉSUMÉ
OBJECTIVES: Aortic valve replacement (AVR) in patients with small aortic annulus (diameter ≤21 mm) is considered a challenging scenario because of technical aspects and the high risk of patient-prosthesis mismatch (PPM). The choice of the appropriate prosthesis is crucial, and at the moment, an ideal device has yet to be identified. We compare clinical and hemodynamic results after AVR with three bioprostheses with different design and characteristics. METHODS: We retrospectively evaluated 76 consecutive patients from two cardiac surgery centers who underwent AVR (Trifecta = 24; Edwards INTUITY Elite valve system = 26, and Perceval = 26) for severe aortic stenosis between 2013 and 2017. Patients selected were older than 75 years and with an annulus diameter ≤21 mm at preoperative echocardiogram. Reinterventions and combined procedures were excluded. Minimally invasive AVR was performed in 44 (57.8%) patients. Telephonic interview was obtained at 2.9 ± 0.5 years and echocardiographic follow-up at 2.2 ± 0.8 years. RESULTS: Clinical outcome was similar in the three groups. At follow-up, Trifecta patients presented significantly higher peak and mean transprosthetic pressure gradients ( P = 0.04 and 0.01). Effective orifice area and left ventricular mass regression were comparable, although an advantage was observed in Perceval patients without reaching the statistical significance. Incidence of moderate ( P = 0.2) and severe PPM ( P = 0.7) was comparable. CONCLUSIONS: Despite higher postoperative pressure gradients observed with the Trifecta valve, all three prostheses (Trifecta, Edwards INTUITY Elite, and Perceval) have proven to be reliable when implanted in small aortic annuli, with good clinical outcome and favorable left ventricular mass regression.
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Sténose aortique/chirurgie , Valve aortique/chirurgie , Implantation de valve prothétique cardiaque/méthodes , Prothèse valvulaire cardiaque/tendances , Sujet âgé , Sujet âgé de 80 ans ou plus , Valve aortique/anatomopathologie , Sténose aortique/imagerie diagnostique , Sténose aortique/anatomopathologie , Échocardiographie/méthodes , Femelle , Études de suivi , Hémodynamique/physiologie , Humains , Mâle , Conception de prothèse , Études rétrospectives , Résultat thérapeutiqueRÉSUMÉ
OBJECTIVES: The population of ageing adults with congenital heart disease (ACHD) is increasing; surgery in these patients presents major difficulties in management. A great debate has developed about whether these patients should be cared for at an adult or paediatric hospital and by an acquired or congenital cardiac surgeon. We analysed data of the surgical treatment of ACHD from the Italian cardiac surgery centres in 2016, focusing on the type of surgery performed, where these patients were operated on and by whom. METHODS: Ninety-two Italian cardiac surgery centres were contacted and 70 centres participated in this study. We collected data on the types of cardiac operations performed in congenital heart defect patients older than 18 years. In 2016, a total of 913 patients with ACHD were operated on: 440 by congenital cardiac surgeons (group I) in centres with paediatric and adult cardiac surgery units, and 473 by adult cardiac surgeons (group II) in centres with exclusively adult cardiac surgery units. RESULTS: Pathologies of the right ventricular outflow tract were the most frequent diseases treated in group I and pathologies of the left ventricular outflow tract in group II. Group I included more complex and heterogeneous cases than group II. Surgery for ACHD represented 12% of the activity of congenital cardiac surgeons and only 1% of the activity of adult cardiac surgeons. CONCLUSIONS: In Italy, ACHD patients are operated on both by congenital and adult cardiac surgeons. Congenital cardiac surgeons working in centres with both paediatric and adult cardiac surgery are more involved with ACHD patients and with more complex cases.