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Objectives: Fontan circulation presents significant challenges for patients with congenital heart disease, often necessitating heart transplantation (HTX) due to deteriorating functionality across multiple organ systems. However, the impact of prior Fontan palliation on HTX outcomes remains poorly understood, with early mortality rates suggesting a heightened risk. The aim of our study is to evaluate the long-term results after heart transplantation in patients with univentricular congenital heart disease previously palliated with Fontan circulation. Methods: A retrospective analysis was conducted on patients who underwent HTX for congenital heart disease. Patients were categorized into two groups based on the pre-HTX circulation pathway: the Failing Fontan Group (FFG) and the Biventricular Congenital Group (BCG). Data were collected from patients between 1987 and 2018. Early and late outcomes, including survival rates, were assessed and critically analyzed. Results: Of the 66 patients, 29 (43%) had a failing Fontan palliation (FFG), and 37 had biventricular congenital diseases (BCG) before heart transplantation. Early mortality (30-day) was not statistically different between the two group. The overall survival rate was 82.6 ± 13.9% at 1 year, 79.0 ± 14.9% at 5 years, 67.2 ± 17.6% at 10 years and 63.2 ± 18.2 ± at 15 years for the FFG, and 86.1 ±11.4% at 1 year, 79.5 ± 13.7% at 5 years, 75.7 ± 14.9% at 10 years, 75.7 ± 14.9% at 15 years for the BCG, with no statistically significant difference (Mantel Cox p value: 0.69, 0.89, 0.52 and 0.39, respectively). Regarding Cox-regression analysis, the long-term survival rate was not affected either by previous Fontan surgery or by the era of heart transplantation (before vs. after the year 2000). Conclusions: Although heart transplantation after Fontan palliation showed a higher risk in the early post-operative period, the medium- and long-term survival rates are comparable with biventricular circulation patients. Despite the failing Fontan patients being a challenging set of candidates for transplantation, it is a reasonable option in their treatment.

Objectives: Repair of Tetralogy of Fallot (TOF) has currently excellent results with either transventricular or transatrial approach. However, it is unclear as to which has better late outcomes and what role of residual pulmonary valve (PV) regurgitation in the long term is. We report on late clinical outcomes after repair in a large series of patients with TOF, focusing on the type of surgical technique., Methods: This analysis is a retrospective multicentre study on patients undergoing TOF repair in infancy. The exclusion criteria of the study were TOF with pulmonary atresia or absent PV., Results: We selected 720 patients who had undergone TOF repair (median age 5.7 months, interquartile range 3.7-11.7). Preoperative cyanotic spells occurred in 18%. A transatrial repair was performed in 433 (60.1%) patients. The PV was preserved in 249 (35%) patients, while the right ventricular outflow tract was reconstructed with a transannular patch (60.4%) or a conduit (4.6%) in the rest of the patients. At a median follow-up of 4 years (range 1-21, 86% complete), 10 (1.6%) patients died, while 39 (6.3%) patients required surgical reoperation and 72 (11.7%) patients required an interventional procedure. The propensity match analysis showed that the incidence of postoperative complications and adverse events at follow-up were significantly increased in patients undergoing transventricular approach repair with transannular patch (P = 0.006) and PV preservation was a significant protective factor against postoperative complications (P = 0.009, odds ratio 0.5) and late adverse events (P = 0.022)., Conclusions: Surgical repair of TOF in infancy is a safe procedure, with good late clinical outcomes. However, transatrial approach and PV preservation at repair are associated with lower early and late morbidity., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Mucopolysaccharidoses (MPS) represent a group of rare lysosomal storage disorders, with a heterogeneous clinical presentation in terms of inheritance (autosomal and X-linked recessive), age of onset (infants, children, and adults), systemic and cardiac manifestations (mild to severe disease forms). Evidence-based recommendations on the diagnosis and management of cardiovascular disease in MPS are scarce. GICEM (Gruppo Italiano Cardiologi Esperti Malattie Metaboliche) is a group of cardiologists, cardiac surgeons and pediatricians with a specific expertise in metabolic diseases including MPS. In this paper, we report our experience and recommendations on the diagnosis and management of cardiovascular aspects in MPS, with a tailored approach based on current evidence, and taking into account MPS phenotype (particularly, I, II, IVa, VI), age at presentation, and severity of systemic and cardiac manifestations.

Williams syndrome (WS) is a genetic disorder due to deficiency of elastin gene expression. It is characterized by typical somatic abnormalities and a wide range of cardiovascular malformations. Coronary artery involvement is a frequent finding of the syndrome, particularly in those patients with severe supravalvular aortic stenosis. We present the case of an 11-month-old infant affected by WS who developed severe coronary artery disease 2 months after the surgical repair of supravalvular aortic stenosis. The clinical picture and successful surgical revascularization strategy is also described., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We present a 4-year-old patient with severe rightward cardiac dislocation due to left tension pneumothorax after surgical closure of a perimembranous ventricular septal defect. The chest roentgenograms showed the "dextrocardia-like" appearance of the cardiac silhouette. Given the persistent severe cardiac dislocation despite resolution of the pneumothorax, and considering the patient's ongoing hemodynamic instability, we decided to reopen the chest to return the heart to its natural position. The procedure was uneventful, and the patient was discharged at home on postoperative day 5., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

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 affecting mortality., Methods: Between 1985 and 2011, among 839 patients who underwent HTx, 85 received transplantation for end-stage CHD. Patients were divided into four age subgroups: <1 year (8 patients, Group I), 1-10 years (20 patients, Group II), 11-18 years (24 patients, Group III) and >18 years (33 patients, Group IV) and into two time periods: 1985-2000 (47 patients) and 2001-2011 (38 patients). Anatomical diagnoses were single-ventricle defect in 37 patients (44%) and two-ventricle defect in 48 patients (56%). Seventy-three patients (86%) had undergone one or more cardiac surgical procedures prior to HTx (mean 2.4 ± 0.9). Twenty-two of them were suffering from Fontan failure. Mean pulmonary artery pressure was 25.2 ± 14.2 mmHg. Mean transpulmonary gradient was 9.4 ± 6.9 mmHg., Results: Mean follow-up after HTx was 7.8 ± 6.8 years. Survival at 1 month 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-day 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. There were 14 late deaths. Univariate analysis found that risk factors for early and late death were those related to recipient illness, such as pre-transplant creatinine, intravenous inotropic drugs, intravenous diuretics, mechanical ventilation and presence of protein-losing enteropathy (PLE). Multivariate analysis for all events (early and late deaths) identified preoperative mechanical ventilation as an independent risk factor for mortality. Number of previous procedures did not influence survival. Previous Fontan procedure did not increase mortality. We documented the reversibility of PLE in survivors., Conclusions: We demonstrated that heart transplantation for patients with CHD can be performed with the expectation of excellent results. Previous procedures, including the Fontan operation, do not reduce survival. Mortality is related to preoperative patient condition. We advocate early referral of complex CHD patients for transplant assessment and for inclusion in waiting lists before the detrimental effects of end-stage failure manifest themselves.

Objective: Primary repair is the treatment of choice in patients with tetralogy of Fallot. The timing of repair, however, remains controversial, and an initial palliative procedure might be considered a valuable option in the early management of symptomatic young infants and in those with either unfavourable anatomy, major associated lesions or chromosomal abnormalities with a poor life expectancy., Methods: We reviewed the management of 100 consecutive patients with tetralogy of Fallot who were operated upon at our department during an 8-year period from June 1995 to March 2003. The rationale for the choice of the initial management and the outcome in terms of morbidity and mortality in patients who underwent primary repair was compared to that observed in patients who had had a two-stage repair., Results: Age less than 3 months, the presence of either an unfavourable anatomy or major associated defects and genetic disorders with poor life expectancy were the indications for an initial palliation, which was carried out in 31 patients. There were no hospital deaths, and 28 of these patients underwent later repair with one hospital death (3.5%). Two patients with severe chromosomal abnormalities died at home and the remainder required a further palliation because of severely hypoplastic pulmonary arteries. Primary repair was carried out in 69 patients with one hospital death (1.4%). A transannular patch, which was used in 80% of our patients, was not an incremental risk factor for death regardless of the type of repair. Eight patients were reoperated on because of either residual right ventricular outflow tract obstruction with (four patients) or without (one patient) residual ventricular septal defect or isolated residual ventricular septal defect (three patients). All reoperations occurred in patients undergoing primary repair., Conclusions: The outcome of patients undergoing repair of tetralogy of Fallot is not influenced in terms of either mortality or morbidity by an initial palliative procedure.

We report the case of a 23-year-old man who was admitted to our Division with the diagnosis of pericardial lipoma. Chest X-ray, echocardiography and magnetic resonance imaging failed to reveal an anterior diaphragmatic hernia containing a small part of the stomach with a big prehernial lipoma that were found at surgery. We believe that in all cases of suspected pericardial lipoma a diaphragmatic hernia should be expected.

Background: The Fogarty thromboembolectomy (TE) has been considered for a long time the best treatment for acute peripheral arterial occlusion. However, recently new therapeutic options like thrombolytic agents (local or systemic) have been introduced., Methods: A total of 66 patients who underwent Fogarty TE for acute thromboembolic limb ischemia has been retrospectively studied. Patients with documented atherosclerotic pathology who were treated with other revascularization procedures were excluded. Patients were divided into four groups: 1) upper limb embolism, 2) lower limb embolism, 3) thrombosis on atherosclerotic plaque or on a graft, 4) post-traumatic thrombosis. Twelve patients underwent Fogarty TE for upper limb thromboembolic ischemia and 54 for lower limb; 57.5% of patients had atrial fibrillation., Results: Procedures were successful in 59 patients (89.4%). Further surgical procedures (bypass, arterioplasty, further embolectomy) were performed in 35.1% (19/54) of patients of groups 2 and 3 with acute lower limb ischemia. Results were very good in group 1 and 4. Seven of the 54 patients treated for lower limb ischemia underwent limb amputation (12.9%), while hospital mortality was similar for lower and upper limb (31.4 and 33.3% respectively)., Conclusions: The Fogarty TE is still a good option to revascularize acute ischemic peripheral arteries with good results in a high number of patients and should be particularly used where it is possible to perform additional techniques in case of failure.

Background: Many novel techniques have been described for "minimally invasive" congenital cardiac operations to achieve an improved cosmetic result. There is little information on incorporation of such techniques into fast-track congenital heart operations., Methods: We have developed an approach to fast-track congenital heart operations, which includes a cosmetic approach for repair of congenital heart defects without sacrificing adequate exposure or requiring specialized equipment, along with a simple approach to intraoperative anesthetic management that allows extubation in the operating room. The heart is exposed through a short midline skin incision and a full median sternotomy. The conventional technique of cannulation is performed. Between October 1997 and January 1999, 88 patients were operated on with this method. Cardiac anomalies included simple and complex ostium secundum atrial septal defect, sinus venous atrial septal defect, partial atrioventricular septal defect, simple and complex ventricular septal defect, and bicuspid aortic valve stenosis., Results: There were no operative or late deaths. The majority of patients were extubated in the operating room or within 2 hours of operation. No patient underwent reoperation and the mean length of hospital stay was 3.9 days. Sternal instability or wound infection were not observed., Conclusions: We believe that our approach to fast-track congenital heart operation is safe and effective. The surgical technique provides good exposure and has excellent cosmetic results. Moreover, it is easy to learn and, if necessary, the surgeon can quickly gain direct access to the heart. The anesthetic management facilitates early tracheal extubation and a shorter duration of postoperative stay.

Background: The incidence of coronary artery disease in patients with dextrocardia associated with situs inversus is similar to that of the general population: Nevertheless, there are few papers regarding surgical myocardial revascularization in these patients., Methods: We report two patients with dextrocardia and situs inversus who underwent myocardial revascularization by means of coronary artery bypass grafting, with the right internal mammary artery to the left anterior descending coronary artery in one case and with the left internal mammary artery as a free graft to the left anterior descending coronary artery associated with the repair of an aneurysm of the right coronary sinus of Valsalva in the second patient., Results: There were no surgical complications; the patients were discharged on the 4th and 7th postoperative days, respectively. They are alive and well after 24 and 60 months of follow-up respectively., Conclusions: This report suggests that durable myocardial revascularization in dextrocardia with situs inversus can be successfully achieved with right or left mammary artery bypass grafting to the left anterior descending coronary artery. The advantage of using an in situ mammary artery is discussed.

We report a case of cardiac echinococcosis in a patient who had sustained an acute myocardial infarction 1 month previously. The coronary angiographic study revealed an isolated complete intrinsic obstruction of the left anterior descending coronary artery in the area of the cyst. The cyst was surgically removed. Coronary artery bypass grafting was not performed because of distal myocardial scarring. Coronary arteriography should routinely be performed in all patients with cardiac echinococcosis.

The presence of coronary artery disease (CAD) evaluated with coronary angiography and eventual correction of CAD in abdominal aortic aneurysm (AAA) patients has been considered the main determinant of early and late outcome after AAA repair. This study reports our experience in CAD and AAA patients in terms of diagnosis and therapy of CAD. In a population of 126 patients (122 males, 4 females, mean age 67.5 years, range 37-81) who were candidates to elective repair for AAA with a diameter > or = 5 centimeters, we included coronary arteriography in 1) patients who were symptomatic for angina (15.9%); 2) patients with previous myocardial infarction (33.3%); 3) patients with previous coronary artery bypass (4%). We identified a group of 45 patients (35.7%) with significant CAD who had been treated before AAA surgery by coronary artery bypass grafting (CABG) in 37 cases or percutaneous coronary angioplasty (PTCA) in 8 cases. AAA repair was performed during the same hospital stay or at a later date. We did not report any morbidity and mortality related to cardiac or vascular procedures. We believe that among patients reporting cardiac symptoms (previous myocardial infarction, angina) the incidence of surgically-correctable CAD is not negligible (45/67, 67.2%). Therefore, invasive coronary study is strongly suggested in such cases to reveal and treat an eventual coronary artery stenosis prior to AAA repair. The absence of cardiac morbidity and mortality related to cardiac and vascular procedures supports this approach.

Background and Aims: Carotid endarterectomy (CEA) is often carried out to prevent cerebrovascular strokes. It is obviously important that neurological morbidity of the procedure is contained within acceptable limits (< 2%)., Methods: Between January 1991 and December 1997 a total of 239 CEA were performed in 216 patients (169 males and 47 females, mean age 66.6 +/- 14.2 years; range 43-81). Angioplasty was carried out using a precoagulated Dacron patch, except in cases in which the residual diameter of the internal carotid artery was greater than 5 mm. A Javid shunt was used selectively if stump pressure < 50 mmHg., Results: No major neurological complications were observed. A reversible focal neurological deficit was reported in 3 cases (1.2%). Neurological morbidity correlated to peripheral arterial occlusive disease appears to be correlated mainly with technical reasons or cerebral ischemia following clamping., Conclusions: The extensive use of angioplasty with patch and the selective use of a protective shunt improve the technical success rate of surgery, significantly helping to limit morbidity.

Background: Intracoronary stenting, conventional coronary artery bypass with cardiopulmonary bypass and minimally invasive direct coronary artery bypass without extracorporeal circulation are the three accepted options for revascularizing the left anterior descending coronary artery. We compare the effects of these three procedures in terms of minor myocardial damage and systemic inflammatory response., Methods: Ninety patients undergoing left anterior descending coronary artery revascularization with these three different techniques (thirty patients per group) were considered. Blood samples were collected preoperatively and immediately postoperatively, and then 24, 48 and 72 hours after the procedures to measure troponin I, creatine kinase, its MB fraction and C-reactive protein levels., Results: Postoperative levels of troponin I and MB-creatine kinase were significantly higher in conventional coronary grafting group than in PTCA-stent and in the minimally invasive surgery groups (p < 0.0003), while in both surgery groups there were higher post-operative levels of total creatine kinase and C-reactive protein (p = 0.0001)., Conclusions: Minimally invasive direct coronary artery bypass surgery and PTCA-stent are similar in terms of virtual absence of minor myocardial damage. Skeletal muscle damage and inflammatory reaction are comparable in surgical patients, but they do not appear to have any clinical relevance.

We report the case of a woman, age 65 years, who was admitted to our hospital for intense abdominal pain. Hemoglobin was 9.7 g/100 ml and computed tomography (CT) confirmed the hemorrhagic state showing intraperitoneal blood. After laparotomy a ruptured aneurysm of the left gastroepiploic artery was diagnosed. Ligation of the artery was performed with good results. This case is reported because the situs of this aneurysm is very rare.

Posttraumatic abdominal aortic pseudoaneurysm is a rare lesion. To date, fewer than 30 cases have been reported in the literature, with most of those cases involving the suprarenal aorta. Infrarenal posttraumatic abdominal aortic pseudoaneurysm following abdominal trauma has been reported in only 6 other cases. We observed such a lesion in a 62-year-old man 15 years after blunt abdominal trauma inflicted in a car accident. Back pain was the presenting symptom. Resection and Dacron graft interposition were performed without postoperative morbidity.

Background: Reinfusion of shed blood after coronary artery bypass grafting might increase the levels of cardiac enzymes with consequent difficulties in the diagnosis of perioperative myocardial infarction., Methods: Thirty consecutive patients undergoing coronary artery bypass grafting who bled at least 400 mL within the first 4 hours after operation underwent reinfusion of shed blood. Thirty consecutive patients who were not autotransfused served as control. All patients underwent enzyme determination (total creatine kinase, MB fraction, lactate dehydrogenase, and troponin I) in the shed blood and in circulating blood preoperatively, at arrival in the intensive care unit, and 6, 24, and 48 hours after operation., Results: The shed blood contained significantly higher concentration of cardiac enzymes than the circulating blood at all time intervals (p = 0.0001). The levels of creatine kinase, its MB fraction, and lactate dehydrogenase in circulating blood were significantly elevated in patients receiving autotransfusion up to 24 hours after autotransfusion. The blood levels of troponin I were not significantly different between the two group of patients at all time points. The percent fraction of MB did not increase after autotransfusion., Conclusions: The measurement of cardiac troponin I is a useful marker for the diagnosis of perioperative myocardial infarction in patients undergoing transfusion of shed blood after coronary operation.