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In 2018, the position paper ‘Imaging the adult with congenital heart disease: a multimodality imaging approach’ was published. The paper highlights, in the first part, the different imaging modalities applied in adult congenital heart disease patients. In the second part, these modalities are discussed more detailed for moderate to complex anatomical defects. Because of the length of the paper, simple lesions were not touched on. However, imaging modalities to use for simple shunt lesions are still poorly known. One is looking for structured recommendations on which they can rely when dealing with an (undiscovered) shunt lesion. This information is lacking for the initial diagnostic process, during repair and at follow-up. Therefore, this paper will focus on atrial septal defect, ventricular septal defect, and persistent arterial duct. Pre-, intra-, and post-procedural imaging techniques will be systematically discussed. This position paper will offer algorithms that might help at a glance. The document is prepared for general cardiologists, trainees, medical students, imagers/technicians to select the most appropriate imaging modality and to detect the requested information for each specific lesion. It might serve as reference to which researchers could refer when setting up a (imaging) study.

Atrial arrhythmias are common in patients with atrial septal defects (ASD) but the effects of percutaneous closure on atrial arrhythmia prevalence is unclear. We investigated the effects of ASD device closure and the impact of age at time of closure on prevalent atrial arrythmia.Meta-analysis of studies reporting atrial arrhythmia prevalence in adult patients before and after percutaneous closure was performed. Primary outcomes were prevalence of 'all atrial arrhythmia' and atrial fibrillation alone post closure. Sub-group analysis examined the effects of closure according to age in patients;40 years, ≥40 and ≥ 60 years. 25 studies were included.Meta-analysis of all studies demonstrated no reduction in all atrial arrhythmia or atrial fibrillation prevalence post-closure (OR 0.855, 95% CI 0.672 to 1.087, P = .201 and OR 0.818, 95% CI 0.645 to 1.038, P = .099, respectively). A weak reduction in all atrial arrhythmia and atrial fibrillation was seen in patients ≥40 years (OR 0.77, 95% CI 0.616 to 0.979, P = .032 and OR 0.760, 95% CI 0.6 to 0.964, P = .024, respectively) but not ≥60 years (OR 0.822, 95% CI 0.593 to 1.141, P = .242 and OR 0.83, 95% CI 0.598 to 1.152, P = .266, respectively). No data were available in patients40 years. This, and other limitations, prevents conclusive assessment of the effect of age on arrhythmia prevalence.Overall, percutaneous ASD closure is not associated with a reduction in atrial arrhythmia prevalence in this meta-analysis. A weak benefit is seen in patients ≥40 years of age, not present in patients ≥60 years.

IntroductionMedical advancements have significantly improved survival of patients with Congenital Heart Disease (CHD). Consequently, an aging CHD population are more susceptible to cardiac-related complications and co-morbidities.Early intervention, with individually-tailored, patient-centred approaches targeting physical activity, diet and stress, can reduce these modifiable risks. Lifestyle Intervention Programmes (LIP) are safe, cost-effective interventions, aiming to promote positive health behaviour change. CHD LIP efficacy research is limited.ObjectiveThis study aims to explore patients’ expectations and experiences to better identify determinants of patient adherence and aspects of LIP that need developing.MethodsQualitative study using semi-structured interviews. Group 1 included seven patients who had not taken part in a LIP to establish views and expectations of LIP. Group 2 included five patients who participated in 3-month pilot LIP at Guy's and St Thomas' to gather feedback on their experience.ResultsThree key themes were identified from Group 1 data: Expectations of Lifestyle Intervention Programme and Intentions to Engage, Views about Online Support for Lifestyle Intervention Programme, Patient Anxieties about Exercise Damaging their Heart. Four key themes were identified from Group 2 data: Insufficient Dietary Information, Brilliant Intentions Marred by Barriers, Pertinence of Plans to Patients, Positive Patient Experiences and Outcomes.ConclusionsThis study provides new insight into beliefs, expectations and experiences of patients with CHD. Findings support the value of LIP for CHD and inform recommendations for LIP development including need for health psychology, dietetic support, more education, greater focus on techniques to address barriers to adherence and scope for specifically-designed App.

Aims Recent evidence supports the occurrence of multiple hormonal and metabolic deficiency syndrome (MHDS) in chronic heart failure (CHF). However, no large observational study has unequivocally demonstrated its impact on CHF progression and outcome. The T.O.S.CA. (Trattamento Ormonale nello Scompenso CArdiaco; Hormone Treatment in Heart Failure) Registry has been specifically designed to test the hypothesis that MHDS affects morbidity and mortality in CHF patients. Methods and Results The T.O.S.CA. Registry is a prospective, multicentre, observational study involving 19 Italian centres. Thyroid hormones, insulin-like growth factor-1, total testosterone, dehydropianoandrosterone sulfate, insulin resistance, and the presence of diabetes were evaluated. A MHDS was defined as the presence of ≥2 hormone deficiencies (HDs). Primary endpoint was a composite of all-cause mortality and cardiovascular hospitalizations. Four hundred and eighty heart failure patients with ejection fraction ≤45% were enrolled. MHDS or diabetes was diagnosed in 372 patients (77.5%). A total of 271 events (97 deaths and 174 cardiovascular hospitalizations) were recorded, 41% in NO-MHDS and 62% in MHDS (P Conclusion MHDS is common in CHF and independently associated with increased all-cause mortality and cardiovascular hospitalization, representing a promising therapeutic target. Trial registration ClinicalTrials.gov identifier: NCT023358017

BACKGROUND: Anomalous aortic origin of coronary arteries (AAOCA) is a rare abnormality, whose optimal management is still undefined. We describe early outcomes in patients treated with different management strategies. METHODS: This is a retrospective clinical multicenter study including patients with AAOCA, undergoing or not surgical treatment. Patients with isolated high coronary take off and associated major congenital heart disease were excluded. Preoperative, intraoperative, anatomical and postoperative data were retrieved from a common database. RESULTS: Among 217 patients, 156 underwent Surgical repair (median age 39 years, IQR: 15-53), while 61 were Medical (median age 15 years, IQR: 8-52), in whom AAOCA was incidentally diagnosed during screening or clinical evaluations. Surgical patients were more often symptomatic when compared to medical ones (87.2% vs 44.3%, p

Objective: To analyze the more recent outcomes of fetuses with different laterality defects.Material and methods: Out of 1508 fetuses with congenital heart disease (CHD) diagnosed by echocardiograp...

Perioperative stress detection in children with congenital heart disease (CHD), particularly in the brain, is still limited. Among biomarkers, γ-amino-aminobutyric acid (GABA) assessment in biological fluids appears to be promising for its regulatory action on the cardiovascular and cerebral systems. We aimed to investigate cyanotic (C) or non-cyanotic (N) CHD children for GABA blood level changes in the perioperative period. We conducted an observational study in 68 CHD infants (C: n = 33, N: n = 35) who underwent perioperative clinical, standard laboratory and monitoring parameter recordings and GABA assessment. Blood samples were drawn at five predetermined time-points before, during and after surgery. No significant perioperative differences were observed between groups in clinical and laboratory parameters. In C, perioperative GABA levels were significantly lower than N. Arterial oxygen saturation and blood concentration significantly differed between C and N children and correlated at cardiopulmonary by-pass (CPB) time-point with GABA levels. The present data showing higher hypoxia/hyperoxia-mediated GABA concentrations in C children suggest that they are more prone to perioperative cardiovascular and brain stress/damage. The findings suggest the usefulness of further investigations to detect the “optimal” oxygen concentration target in order to avoid the side effects associated with re-oxygenation during CPB.

More than 4 millions of children with congenital heart disease (CHD) are waiting for cardiac surgery around the world. Few of these patients are treated only thanks to the support of many non-governmental organizations (NGOs). Starting in December 2019, the so-called coronavirus disease 2019 (COVID-19) has rapidly become a worldwide pandemic and has dramatically impacted on all the international humanitarian activities for congenital heart disease. We analyzed data from all the Italian congenital cardiac surgery centers with the aim to quantify the impact of the pandemic on their charities. Fifteen Italian centers participated in the study and contributed to data collection. We analyzed and compared data regarding humanitarian activities carried out abroad and on site from two periods: year 2019 (pre-COVID-19) and year 2020 (COVID-19 pandemic). In 2019, 53 international missions were carried out by Italian congenital cardiac surgeons, resulting in the treatment of 471 CHD patients. In the same period 11 Italian cardiac centers operated on 251 foreign patients in Italy. In 2020, the pandemic led to a reduction of this activity by 96% for the surgery performed overseas and 86% for the interventions carried out in Italy. In conclusion our study shows the important quantitative impact of the pandemic on the Italian humanitarian cardiac surgical activity overseas and in Italy. This shocking result highlights the failure of the systems adopted so far to solve the problem of CHD in developing countries.

This chapter discusses the role of multimodality imaging [i.e. transthoracic echocardiogram (TTE), cardiovascular magnetic resonance imaging (CMR) and computed tomography (CT)] for three congenital aortic lesions: bicuspid aortic valve, coarctation of the aorta and Marfan syndrome. The wealth of data contained in the imaging datasets has also allowed to explore innovative methodologies either within the imaging modalities themselves, increasing the amount or type of data available for the investigation, or at an image-processing stage, providing novel insights into these important congenital defects. Such imaging or imaging-based innovations include 4D flow CMR, computational fluid dynamics and statistical shape modelling, all holding potential to offer novel insights into haemodynamics, wall mechanics and morphology. From a translational standpoint, this chapter also explores the role of virtual stenting and 3D printing in the clinical practice. Overall, these innovations can play a role in informing and supporting diagnosis and prognosis and can produce new knowledge on concepts such as aortopathy progression and other complications that can affect patients with bicuspid aortic valve, coarctation of the aorta and Marfan syndrome.

Background Adolescence is a vulnerable period for patients with congenital heart disease (CHD). Transition is a process that guides these patients through adolescence and ensures a smooth transfer to adult services, in order improve adherence to medical care and reduce loss to follow-up. While the importance of a formal Transition process is widely recognised and a requirement for specialist services in the UK, the optimal structure and delivery of Transition remains a matter of debate. Aims To examine the different models of Transition currently in place in specialist CHD centres around the UK. Methods A survey of Adult CHD centres in the UK was performed. A focus was placed on the structure of the Transition service, relevant training and areas of perceived improvement. Results There were 10 responses to our survey covering 10 specialist CHD centres. All respondents were consultant adult CHD specialists, looking after patients from the age of 16 [14–17] years. All centres have a specialised Transition service, which runs from the age 13 [11–15] to 18 [16–25] years (duration of transition 5 [2–13] years). The majority of centres (80%) report providing transition care “well before” transfer to adult care, whereas 20% provide transition care at or immediately before transfer (i.e. first adult CHD appointment). Transition is delivered by physicians and clinical specialist nurses in approximately equal numbers in 9 (90%) centres and exclusively by clinical nurse specialists in 1 (10%) centre. A median of 2 [1–5] visits are planned for each patient, with 7 (70%) centres seeing patients at least twice during transition. The majority, but not all centres (70%) provide a health passport during transition. A significant number of centres felt they werer not receiving sufficient support in the following domains: financial (50%), training (30%), clinical space (30%), referrals from paediatrics (50%). All respondents felt that their Transition service had room for improvement. Other areas of improvement highlighted included reduction in loss to follow-up, difficulties in providing a Transition service to patients followed in peripheral hospitals, the need for more support from paediatric services in referring all appropriate patients, and dedicated administrative support. The vast majority of respondents (9, 90%) felt equipped with the appropriate skills to care for transition patients. However, few (2, 20%) had completed formal training in more than one area related to adolescent health and transition. Conclusions While all CHD centres have a Transition service, Transition models and delivery differs significantly. There is urgent need for research in this area to develop a unified model, greater financial support and relevant training to optimise care. Figure 1. Participating UK centres Funding Acknowledgement Type of funding source: None

Aims Atrial septal defects (ASD) are associated with atrial arrhythmias, but the arrhythmia substrate in these patients is poorly defined. We hypothesized that bi-atrial fibrosis is present and that right atrial fibrosis is associated with atrial arrhythmias in ASD patients. We aimed to evaluate the extent of bi-atrial fibrosis in ASD patients and to investigate the relationships between bi-atrial fibrosis, atrial arrhythmias, shunt fraction, and age. Methods and results Patients with uncorrected secundum ASDs (n = 36; 50.4 ± 13.6 years) underwent cardiac magnetic resonance imaging with atrial late gadolinium enhancement. Comparison was made to non-congenital heart disease patients (n = 36; 60.3 ± 10.5 years) with paroxysmal atrial fibrillation (AF). Cardiac magnetic resonance parameters associated with atrial arrhythmias were identified and the relationship between bi-atrial structure, age, and shunt fraction studied. Bi-atrial fibrosis burden was greater in ASD patients than paroxysmal AF patients (20.7 ± 14% vs. 10.1 ± 8.6% and 14.8 ± 8.5% vs. 8.6 ± 6.1% for right and left atria respectively, P = 0.001 for both). In ASD patients, right atrial fibrosis burden was greater in those with than without atrial arrhythmias (33.4 ± 18.7% vs. 16.8 ± 10.3%, P = 0.034). On receiver operating characteristic analysis, a right atrial fibrosis burden of 32% had a 92% specificity and 71% sensitivity for predicting the presence of atrial arrhythmias. Neither age nor shunt fraction was associated with bi-atrial fibrosis burden. Conclusion Bi-atrial fibrosis burden is greater in ASD patients than non-congenital heart disease patients with paroxysmal AF. Right atrial fibrosis is associated with the presence of atrial arrhythmias in ASD patients. These findings highlight the importance of right atrial fibrosis to atrial arrhythmogenesis in ASD patients.

Background Patients with repair of tetralogy of Fallot (rToF) who are approaching adulthood often exhibit pulmonary valve regurgitation, leading to right ventricle (RV) dilatation and dysfunction. The regurgitation can be corrected by pulmonary valve replacement (PVR), but the optimal surgical timing remains under debate, mainly because of the poorly understood nature of RV remodeling in patients with rToF. The goal of this study was to probe for pathologic molecular, cellular, and tissue changes in the myocardium of patients with rToF at the time of PVR. Methods and Results We measured contractile function of permeabilized myocytes, collagen content of tissue samples, and the expression of mRNA and selected proteins in RV tissue samples from patients with rToF undergoing PVR for severe pulmonary valve regurgitation. The data were compared with nondiseased RV tissue from unused donor hearts. Contractile performance and passive stiffness of the myofilaments in permeabilized myocytes were similar in rToF‐PVR and RV donor samples, as was collagen content and cross‐linking. The patients with rToF undergoing PVR had enhanced mRNA expression of genes associated with connective tissue diseases and tissue remodeling, including the small leucine‐rich proteoglycans ASPN (asporin), LUM (lumican), and OGN (osteoglycin), although their protein levels were not significantly increased. Conclusions RV myofilaments from patients with rToF undergoing PVR showed no functional impairment, but the changes in extracellular matrix gene expression may indicate the early stages of remodeling. Our study found no evidence of major damage at the cellular and tissue levels in the RV of patients with rToF who underwent PVR according to current clinical criteria.

Background Isolated coronary arteriovenous fistulas are extremely rare, accounting for 0.08–0.4% of all congenital heart disease. Closure of the fistula is recommended in cases of large dimensions, relevant left–right shunt, or ischaemic events. Thrombosis of the coronary aneurysms may occur as a postoperative complication. Case summary We report a case of a coronary fistula between the circumflex artery and coronary sinus with giant aneurysm. After a failed percutaneous closure attempt, the patient was surgically treated without major postoperative complications. Despite therapeutic anticoagulation and antiplatelet therapy, she presented at clinical follow-up with thrombosis of the dilated coronary artery without signs or symptoms of ischaemia. Discussion Management of coronary artery fistula may be challenging in cases in which initial percutaneous closure is unsuccessful. This particular case also highlights the importance of close follow-up, despite optimal therapy, to detect potentially lethal complications related to the low flow in the dilated coronary aneurysm.

Holt-Oram syndrome (HOS) is a rare, autosomal dominant heart-hand syndrome caused by mutations in the TBX5 gene. A wide spectrum of TBX5 mutations have been reported previously, most resulting in a null allele leading to haploinsufficiency. TBX5 gene duplications have been previously reported in association with typical and atypical HOS phenotypes. Ulnar-Mammary syndrome (UMS) is a distinct rare, autosomal dominant condition caused by mutations in the TBX3 gene. TBX5 and TBX3 are physically linked in cis on human chromosome 12 and contiguous chromosome 12q24 deletions comprising both TBX5 and TBX3 genes have been previously reported but to our knowledge, duplications have never been described. We report on a large German family with at least 17 affected individuals over 6 generations bearing a duplication at 12q24.21 identified on array-CGH comprising both TBX5 and TBX3 genes. Affected patients are presenting with HOS and UMS symptoms, consisting of variable limb anomalies involving the radial and the ulnar rays and cardiac findings such as congenital heart defects, persistent arterial duct or aortic stenosis, and non-classical symptoms, such as supernumerary nipples and cardiomyopathy. Fluorescence in situ hybridisation confirmed a tandem duplication at the 12q24.21 locus. This is the first report of a contiguous TBX3/TBX5 duplication associated with HOS/UMS phenotype.

Echocardiography is the mainstay in screening for pulmonary hypertension (PH). International guidelines suggest echocardiographic parameters for suspecting PH, but these may not apply to many adults with congenital heart disease (ACHD). PH is relatively common in ACHD patients and can significantly affect their exercise capacity, quality of life, and prognosis. Identification of patients who have developed PH and who may benefit from further investigations (including cardiac catheterization) and treatment is thus extremely important. A systematic review and survey of experts from the United Kingdom and Ireland were performed to assess current knowledge and practice on echocardiographic screening for PH in ACHD. This paper presents the findings of the review and expert statements on the optimal approaches when using echocardiography to assess ACHD patients for PH, with particular focus on major subgroups: patients with right ventricular outflow tract obstruction, patients with systemic right ventricles, patients with unrepaired univentricular circulation, and patients with tetralogy of Fallot with pulmonary atresia.

International audience; Advances in the diagnosis and management of congenital heart disease have led to a marked improvement in the survival of adult with congenital heart disease (ACHD) patients. However, ACHD patients are a heterogeneous population, with a large spectrum of anatomic substrates even within specific lesions. In addition, the nature of previous surgery and other intervention is highly variable rendering each patient unique and residual anatomic and haemodynamic abnormalities are very common. As the ACHD population continues to age, acquired heart disease will also require cardiac imaging assessment. It is increasingly recognized in ACHD community that the diagnostic utility of a multimodality cardiovascular approach is greater than the sum of individual tests. In ACHD patients, diagnostic information can be obtained using a variety of diagnostic tools. The aims of this document are to describe the role of each diagnostic modality in the care of ACHD patients and to provide guidelines for a multimodality approach. The goal should be to provide the most appropriate and cost-effective diagnostic pathway for each individual ACHD patient.

Aims Treatment decisions in patients with scimitar syndrome (SS) are often challenging, especially in patients with isolated SS who are often asymptomatic and who might be diagnosed accidentally. We queried a large multi-institutional registry of SS patients to evaluate the natural history of this condition and to determine the efficacy of surgical treatment in terms of survival and clinical status. Methods and results We collected data on 485 SS patients from 51 institutions; 279 (57%) patients were treated surgically (STPs) and 206 (43%) were clinically monitored (CMPs). Median age at last follow-up was 11.6 years (interquartile range 4-22 years). Overall survival probability at 30 years of age was 88% [85-92% confidence intervals (CI)] and was lower in patients with associated congenital heart disease (CHD) (P

Recent evidence supports the concept that progression of chronic heart failure (CHF) depends upon an imbalance of catabolic forces over the anabolic drive. In this regard, multiple hormonal deficiency syndrome (MHDS) significantly has impacts upon CHF progression, and is associated with a worse clinical status and increased mortality. The T.O.S.CA. (Trattamento Ormonale nello Scompenso CArdiaco; Hormone Therapy in Heart Failure) Registry (clinicaltrial.gov = NCT02335801) tests the hypothesis that anabolic deficiencies reduce survival in a large population of mild-to-moderate CHF patients. The T.O.S.CA. Registry is a prospective multicenter observational study coordinated by “Federico II” University of Naples, and involves 19 centers situated throughout Italy. Thyroid hormones, insulin-like growth factor-1, total testosterone, dehydroepiandrosterone , and insulin are measured at baseline and every year for a patient-average follow-up of 3years. Subjects with CHF are divided into two groups: patients with one or no anabolic deficiency, and patients with two or more anabolic deficiencies at baseline. The primary endpoint is the composite of all-cause mortality and cardiovascular hospitalization. Secondary endpoints include the composite of all-cause mortality and hospitalization, the composite of cardiovascular mortality and cardiovascular hospitalization, and change of VO 2 peak. Patient enrollment started in April 2013, and was completed in July 2017. Demographics and main clinical characteristics of enrolled patients are provided in this article. Detailed cross-sectional results will be available in late 2018. The T.O.S.CA. Registry represents the most robust prospective observational trial on MHDS in the field of CHF. The study findings will advance our knowledge with regard to the intimate mechanisms of CHF progression and hopefully pave the way for future randomized clinical trials of single or multiple hormonal replacement therapies in CHF.

Retrograde systolic flow in the main pulmonary artery (PA) is a hallmark echocardiographic sign of patent ductus arteriosus. We describe a case of PA aneurysm mimicking patent ductus arteriosus flow. Using 4-dimensional flow cardiac magnetic resonance imaging, this echocardiographic feature is explained and altered flow dynamics in the aneurysmal PA are visualized. (Level of Difficulty: Intermediate.), Graphical abstract, Retrograde systolic flow in the main pulmonary artery (PA) is a hallmark echocardiographic sign of patent ductus arteriosus. We describe a case of PA…

Background Irbesartan, a long acting selective angiotensin-1 receptor inhibitor, in Marfan syndrome might reduce aortic dilatation, which is associated with dissection and rupture. We aimed to determine the effects of irbesartan on the rate of aortic dilatation in children and adults with Marfan syndrome. Methods We did a placebo-controlled, double-blind randomised trial at 22 centres in the UK. Individuals aged 6–40 years with clinically confirmed Marfan syndrome were eligible for inclusion. Study participants were all given 75 mg open label irbesartan once daily, then randomly assigned to 150 mg of irbesartan (increased to 300 mg as tolerated) or matching placebo. Aortic diameter was measured by echocardiography at baseline and then annually. All images were analysed by a core laboratory blinded to treatment allocation. The primary endpoint was the rate of aortic root dilatation. This trial is registered with ISRCTN, number ISRCTN90011794. Findings Between March 14, 2012, and May 1, 2015, 192 participants were recruited and randomly assigned to irbesartan (n=104) or placebo (n=88), and all were followed for up to 5 years. Median age at recruitment was 18 years (IQR 12–28), 99 (52%) were female, mean blood pressure was 110/65 mm Hg (SDs 16 and 12), and 108 (56%) were taking β blockers. Mean baseline aortic root diameter was 34·4 mm in the irbesartan group (SD 5·8) and placebo group (5·5). The mean rate of aortic root dilatation was 0·53 mm per year (95% CI 0·39 to 0·67) in the irbesartan group compared with 0·74 mm per year (0·60 to 0·89) in the placebo group, with a difference in means of −0·22 mm per year (−0·41 to −0·02, p=0·030). The rate of change in aortic Z score was also reduced by irbesartan (difference in means −0·10 per year, 95% CI −0·19 to −0·01, p=0·035). Irbesartan was well tolerated with no observed differences in rates of serious adverse events. Interpretation Irbesartan is associated with a reduction in the rate of aortic dilatation in children and young adults with Marfan syndrome and could reduce the incidence of aortic complications. Funding British Heart Foundation, the UK Marfan Trust, the UK Marfan Association.

Introduction There is growing recognition of Fontan-associated liver disease (FALD), which encompasses a spectrum ranging from liver congestion to cirrhosis and hepatocellular carcinoma. Diagnostic pathways remain poorly defined, with no consensus on optimal screening and management modalities. Aim of this study was to assess prevalence and severity of FALD by implementation of a structured screening programme. Methods A cohort of 93 Fontan patients aged ≥ 16 years underwent a screening protocol for liver disease at our tertiary Adult Congenital Heart Disease Centre, including blood tests, liver ultrasound with elastography, and fibroscan. Results were discussed in a MDM and patients categorised according to the risk of having liver fibrosis. MRI, CT scan and/or endoscopy were also organised when appropriate. Patients were followed up yearly if the initial tests were reassuring, or started on 6-montly HCC surveillance if the initial screening suggested advanced fibrosis. Results The study cohort comprised 55 (59%) males, mean age 25.4±6.2 years, and median 18 years since Fontan surgery. Most patients were asymptomatic (79%), with good haemodynamics. Liver abnormalities on ultrasonography were evident in 51% (41 of 80 scans), including coarse echotexture (41%), hepatomegaly (12%), hyper-echoic nodules (6%) and signs of portal hypertension (15%). Liver stiffness was elevated on both ultrasound elastography (mean 7.8±3.3 kPa, Philips US ElasPQ) and fibroscan (median 20.0±6.7 kPa). Liver MRIs and triple phase liver CTs, performed in a subset of the cohort, further characterised liver abnormalities, most notably focal nodular hyperplasia (FNH) in 65% (13 of 20 MRIs). Most patients had normal biochemical liver profiles, with mean values for bilirubin 18.2 ± 10.6umol/L, ALT 26.5 ± 10.6IU/L, albumin 47.5 ± 5.3g/L and platelets 169 ±55 x109, despite hepatic imaging of parenchymal abnormalities. Two patients were found to have hepatocellular carcinomas (HCC), BCLC stage B and C at diagnosis. There was a total of 4 deaths over a median follow-up of 2.8 years, of which 2 deaths were liver-related. Conclusions Elevated hepatic stiffness and ultrasound appearance in keeping with severe fibrosis are common in adult Fontan patients, despite normal liver enzymes and synthetic function. FNH is the most common abnormality on imaging in high risk patients. The incidence of HCC justifies surveillance in this group of patients, however the optimal surveillance protocol remains to be established.

Congenital sinus of Valsalva aneurysm is a rare disorder, mostly involving the right and noncoronary sinuses, in which intracardiac rupture is more likely foreseen into the right chambers. Because of the unfavorable prognosis, which includes heart failure and sudden death, detection itself is an indication for treatment, which may be performed either surgically or percutaneously. We present a case of a four-year-old patient with aorto-right atrial tunnel, in which a transcatheter attempt of closure was performed, complicated by new onset of aortic valve regurgitation, requiring surgical intervention.

The impact of congenital heart disease on brain aging has not been extensively investigated. We evaluated cerebral microbleeds and white matter hyperintensities on brain magnetic resonance imaging in adult patients with tetralogy of Fallot (ToF). Ten ToF patients (6 women, 4 men; aged 21–58 years; New York Heart Association [NYHA] class 1–2) were prospectively enrolled and underwent a T1-weighted, a T2-weighted dark fluid, and a T2*-weighted scans. Ten age- and sex-matched controls were prospectively recruited and subjected to the same acquisition protocol. Cerebral microbleeds (CMBs) were manually counted while white matter hyperintensities (WMHs) were segmented using ITK-Snap. Wilcoxon signed-rank test, Spearman correlation, and Bland-Altman statistics were used. The median (interquartile range [IQR]) age was 45.0 (30.5–49.5) years in ToF patients and 46.0 (30.5–49.8) years in controls. The median (IQR) of the number of CMBs was 6.0 (4.0–7.8) in ToF patients and 0 (0.0–0.0) in controls (p = 0.002). The WMHs burden was 2,506 (1,557–2,900) mm3 for ToF patients and 2,212 (1,860–2,586) mm3 for controls (p = 0.160). Moreover, a positive significant correlation was found between the WMHs burden and the NYHA class (ρ = 0.80, p = 0.005). Inter-operator concordance rate for the presence/ absence of CMBs was 90%; the reproducibility for the WMHs burden was 77%. In conclusion, we found more cerebral microbleeds and a higher WMHs burden in adult ToF patients than in controls. This preliminary comparison supports the hypothesis of an early brain aging in ToF patients. Larger studies are warranted.

OBJECTIVE: Children undergoing Ross operation were expected to have longer autograft, but shorter homograft durability compared with adults. In order to define the outcome in the second decade after Ross operation in children, a nationwide review of 23 years of experience was undertaken. METHODS: 305 children underwent Ross operation in 11 paediatric units between 1990 and 2012. Age at surgery was 9.4±5.7 years, indication aortic stenosis in 103 patients, regurgitation in 109 and mixed lesion in 93. 116 (38%) patients had prior procedures. Root replacement was performed in 201 patients, inclusion cylinder in 14, subcoronary grafting in 17 and Ross-Konno in 73. RESULTS: There were 10 (3.3%) hospital and 12 late deaths (median follow-up 8.7 years). Survival was 93±2% and 89±3% and freedom from any reoperation was 76±3% and 67±6% at 10 and 15 years. 34 children had autograft 37 reoperations (25 replacement, 12 repair): three required transplantation after reoperation. Freedom from autograft reoperation was 86±3% and 75±6% at 10 and 15 years. 32 children had right heart redo procedures, and only 25 (78%) conduit replacements (15-year freedom from replacement, 89±4%). Prior operation (p=0.031), subcoronary implant (p=0.025) and concomitant surgical procedure (p=0.004) were risk factors for left heart reoperation, while infant age (p=0.015) was for right heart. The majority (87%) of late survivors were in NYHA class I, 68% free from medication and six women had pregnancies. CONCLUSIONS: Despite low hospital risk and satisfactory late survival, paediatric Ross operation bears substantial valve-related morbidity in the first two decades. Contrary to expectation, autograft reoperation is more common than homograft.

The Thoracic Aortic Research Center (TARC) of the IRCCS Policlinico San Donato (PSD) aims to promote research on thoracic aortic diseases, to disclose the scientific knowledge and clinical experience and to develop new scientific paths within the Hospital and the aortic community, in collaboration with other national and international centres. Thoracic Aortic Research Center collaborates with many centres in both Europe (e.g. University of Utrecht, the Netherlands) and the USA (e.g. University of Michigan). This has led to multiple highly regarded publications in respected cardiovascular journals and has led to several PhD programmes resulting in doctorate degrees. Within Italy, in association with the Bioengineering School of the University of Pavia, TARC has founded the “BETA-lab” ( B iomechanics for E ndovascular T reatment of the A orta laboratory), where MDs, Bioengineers, and PhD fellows conduct experimental studies using in vitro/ex vivo models of the physiologic aorta and aortic diseases. Furthermore, a database (iCardiocloud) where the medical imaging of cardiovascular patients from the PSD is structured, for in silico analysis utilizing computational fluid dynamics, and in vitro studies using also 3D printed aortic models. With the role of principal investigator or co-investigator, TARC at PSD has been participating in other several projects, including the International Registry of Acute Aortic Dissection, the International Aortic Arch Surgery Study Group, the European Registry of Endovascular Aortic Repair Complications, the ADSORB and ASSIST trials, and the GREAT registry. International collaborations have included also studies on predictors of aortic growth after dissection with the Yale University and University of Virginia, and on aortic biomarkers with the University of Tokyo.

Thanks to the improvement of surgical and interventional cardiac procedures, the majority of children with congenital heart defects (CHD) can now be expected to reach adulthood. The number of adults with congenital heart disease (ACHD) will inevitably increase, and recent data affirm that in Europe, we are actually faced with an estimated patient population of 2.3 million. These patients, particularly the adults with moderate and highly complex CHD, can be very difficult to manage, and should be treated in few experienced and specialized 'grown-up congenital heart disease' (GUCH) units, concentrating resources, patients, funding, and professional experiences. On 2000, we created a GUCH unit in our hospital that rapidly became a referral centre in Italy for number and complexity of patients treated. The present study is a presentation of how is organized our GUCH unit and a brief overview of our more recent experience with ACHD patients.

Objectives Anomalous aortic origin of the coronary artery can be associated with sudden cardiac death and ischemic events. Anatomic static characteristics mainly dictated surgical indications, although adverse events are usually related to dynamic physical effort. We developed a computational model able to simulate anomalous coronary behavior, and we aimed to assess its clinical applicability and to investigate coronary characteristics at increasing loading stress conditions. Methods We selected 5 patients with anomalous aortic origin of the coronary artery and 5 control subjects. For each of them, we construct a 3-dimensional model resembling the aortic root and coronary arteries based on 25 parameters obtained from computed tomography. Structural finite element analysis simulations were run to simulate pressure increasing in the aortic root during exercise (+40 mm Hg, +100 mm Hg with respect baseline condition, assumed at 80 mm Hg) and investigate coronary lumen characteristics. Results The 25 parameters were obtainable in all subjects with a consistent interobserver agreement. In control subjects, the right coronary artery had a more significant lumen expansion at loading conditions compared with anomalous aortic origin of coronary artery (6%-19.2% vs 1.8%-8.1%, P = .008), which also showed an inability to expand within the intramural segment. Conclusions The proposed anomalous aortic origin of coronary artery model is able to represent the pathogenic disease mechanism after being populated with patient-specific data. It can assess the impaired expansion of anomalous right coronary at loading conditions, a process that cannot be quantified in any clinical set-up. This first clinical application showed promising results on quantifying pathological behavior, potentially helping in patient-specific risk stratification.

Infective endocarditis is a life-threatening complication of congenital heart disease (CHD), but there are few studies concerning the contemporary risk profile, preceding invasive procedures and outcomes in this patient population. The aim of this study was to investigate the epidemiology of infective endocarditis (IE) in patients with CHD.Cases of IE in children and adults with CHD were prospectively recorded as part of the UK National Institute for Cardiovascular Outcomes Research (NICOR) National Congenital Heart Disease Audit. Patients were entered into the database between April 2008 and March 2016.Eight hundred episodes of IE were recorded in 736 patients with CHD. Sixty-five patients (9%) were infants (aged1 year), 235 (32%) were children (aged 1-15 years), and 436 (59%) were adults (aged15 years). The most common diagnoses were Tetralogy of Fallot (n = 150, 22.8%), ventricular septal defect (n = 129, 19.6%) and bicuspid aortic valve (n = 70, 10.7%). Dental procedures preceded 67 of 635 episodes (11%) of IE, and non-dental invasive procedures preceded 177 of 644 episodes (27.4%). The most common causative organisms were streptococci, accounting for 40% of cases. Overall in-hospital mortality was 6.7%. On multivariable analysis, adverse factors associated with in-hospital mortality were staphylococcal infection and presence of an underlying atrioventricular septal defect.Infective endocarditis in patients with CHD is an ongoing clinical challenge. In contemporary practice in tertiary congenital centers, 1 of 15 patients do not survive to hospital discharge. Streptococci remain the most common causative organism, and antecedent dental or medical procedures were undertaken in a significant minority in the 3 months before diagnosis. The presence of an atrioventricular septal defect or staphylococcal infection is associated with significantly increased risk of early mortality.

OBJECTIVES: We sought to describe early and late outcomes in a large surgical series of patients with anomalous aortic origin of coronary arteries. METHODS: We performed a retrospective multicentre study including surgical patients with anomalous aortic origin of coronary arteries since 1991. Patients with isolated high coronary takeoff and associated major congenital heart disease were excluded. RESULTS: We collected 156 surgical patients (median age 39.5 years, interquartile range 15–53) affected by anomalous right (67.9%), anomalous left (22.4%) and other anatomical abnormalities (9.6%). An interarterial course occurred in 86.5%, an intramural course in 62.8% and symptoms in 85.9%. The operations included coronary unroofing (56.4%), reimplantation (19.2%), coronary bypass graft (15.4%) and other (9.0%). Two patients with preoperative cardiac failure died postoperatively (1.3%). All survivors were discharged home in good clinical condition. At a median follow-up of 2 years (interquartile range 1–5, 88.5% complete), there were 3 deaths (2.2%), 9 reinterventions in 8 patients (5 interventional, 3 surgical); 91.2% are in New York Heart Association functional class ≤ II, but symptoms persisted in 14.2%; 48.1% of them returned to sport activity. On Kaplan–Meier analysis, event-free survival at follow-up was 74.6%. Morbidity was not significantly different among age classes, anatomical variants and types of surgical procedures. Furthermore, return to sport activity was significantly higher in younger patients who participated in sports preoperatively. CONCLUSIONS: Surgical repair of anomalous aortic origin of coronary arteries is effective and has few complications. Unroofing and coronary reimplantation are safe and are the most common procedures. The occurrence of late adverse events is not negligible, and long-term surveillance is mandatory. Most young athletes can return to an unrestrained lifestyle.

Background Atrial fibrillation (AF) is common in atrial septal defect patients (ASD) but little is known about the triggers for these arrhythmias. Hypothesis Since right atrial dilatation is well described in ASD patients we hypothesised that atrial ectopy in ASD patients would be predominantly right-sided in origin. Objective 1. To determine the origin of ectopy recorded on continuous Holter monitoring in ASD patients compared to atrial fibrillation (AF) patients with structurally normal hearts. 2. To identify echo and CMR parameters associated with ectopy in an ASD population. Methods The origin of atrial ectopic beats was determined by measuring P wave amplitude in three Holter leads and calculating the axis for each ectopic beat. Invasive validation was performed by calculating P wave axes as recorded on Holter monitoring during intracardiac pacing from multiple right and left atrial sites in patients with structurally normal hearts undergoing catheter ablation. Haemodynamic parameters from echo/MRI and invasive catherisation, when available, were recorded in the ASD patients and correlated with arrhythmia occurrence and atrial ectopic burden (figure 1). Results Analysis of Holter monitoring P wave vectors during intra-cardiac pacing across 35 sites demonstrated that a vector between 90° and 270° had a 90.5% sensitivity and 85.7% specificity for predicting an ectopic beat of left sided (vs right sided) origin. Therefore, ectopic P waves with a vector angle of 90–270° were considered left sided while those with an angle of 0–90° or 270–360° were considered right sided. 189 ectopic beats were analysed in 33 ASD patients (14 male, mean age 50.1±16.9 years). 149 ectopic beats were analysed in 37 control patients (21 male, mean age 61.6±10.5) (table 1). Right sided ectopy accounted for 71.4% of all ectopic beats studied in the ASD population and was significantly more prevalent in the ASD population than in the control population (p=0.003). Qp:Qs was significantly associated with the presence of documented atrial arrhythmias in the ASD population (p=0.04). Overall ectopic burden over 24 hours correlated significantly with LA size by 2D echo (R=0.464, p=0.008). Conclusion Right sided ectopy is more prevalent in ASD patients compared to non-ACHD AF patients. This data suggests that arrhythmia triggers are more likely to be right sided in origin compared to NCHD AA patients This observation may have implications for arrhythmia intervention strategies in this cohort. Further investigation is required to determine the role of right-sided ablation as an adjunct to pulmonary vein isolation in ASD patients with AF.

Introduction Atrial septal defects (ASDs) are associated with atrial arrhythmias (AAs) however little is known about the arrhythmia substrate in these patients. Left atrial (LA) fibrosis, detected by cardiac MRI (CMR) is well described in patients with atrial fibrillation and structurally normal hearts but fibrosis has not been described in either atria in the ASD cohort. Hypothesis We hypothesised that right atrial (RA) fibrosis is present in patients with ASDs and that the right atrium is important for arrhythmogenesis in this cohort. Aims To evaluate the presence of bi-atrial fibrosis in patients with ASDs and to identify MRI parameters associated with the presence of atrial arrhythmias. Methods Patients with uncorrected ASDs underwent CMR imaging with dedicated atrial sequences on a 1.5T MRI scanner. MR angiography was performed 90 s after infusion of 0.2 ml/kg Gadovist. 3D LGE imaging using a respiratory navigated, ECG triggered inversion recovery sequence was performed 20 min post contrast administration. The right and left atria were semi-automatically segmented using in house software. Fibrosis maps were generated from the LGE images using a maximum intensity projection technique. Fibrosis scores were generated by thresholding the maps using the image intensity ratio with a fibrosis cut off point of 0.97 times blood pool SI. Right and left atrial and ventricular dimensions were quantified from short and long axis cine imaging and aortic and pulmonary flows measured to calculate shunt fraction (Qp:Qs). Presence of atrial arrhythmia was documented for each patient. Results 14 patients were included (52 3.7 years, 10 female). Four patients had a history of atrial arrhythmia (2 atrial flutter, 1 atrial fibrillation, 1 atrial flutter and fibrillation). Mean Qp:Qs was 2.3:1. RA area was significantly greater than LA area (33.1±7.5 vs 26.6±6.8 cm2, p=0.012). RA fibrosis burden was significantly greater than LA fibrosis burden (69.7%±13.1% vs 52.7±8.1%, p Conclusions Right atrial fibrosis is present in ASD patients to a significantly greater degree than left atrial fibrosis. RA size is significantly greater in ASD patients with than without atrial arrhythmias. Further work is needed to fully define the arrhythmia substrate in terms of structural and electrical remodelling in these patients.

Anomalous aortic origin of coronary arteries (AAOCA) is a congenital disease that can lead to cardiac ischemia during intense physical activity. Although AAOCA is responsible for sudden cardiac death (SCD) among young athletes and soldiers, the mechanisms underlying the coronary occlusion during physical effort still have to be clarified. The present study investigates the correlation between geometric features of the anomaly and coronary lumen narrowing under aortic root dilatations. Idealized parametric computer-aided designed (CAD) models of the aortic root with anomalous and normal coronaries are created and static finite element (FE) simulations of increasing aortic root expansions are carried out. Different coronary take-off angles and intramural penetrations are investigated to assess their role on coronary lumen narrowing. Results show that increasing aortic and coronary pressures lead to lumen expansion in normal coronaries, particularly in the proximal tract, while the expansion of the anomalous coronaries is impaired especially at the ostium. Concerning the geometric features of the anomaly, acute take-off angles cause elongated coronary ostia, with an eccentricity increasing with aortic expansion; the impact of the coronary intramural penetration on the lumen narrowing is limited. The present study provides a proof of concept of the biomechanical reasons underlying the lumen narrowing in AAOCA during aortic expansion, promoting the role of computational simulations as a tool to assess the mechanisms of this pathology.

Background Severe hemodilution during cardiopulmonary bypass (CPB) is a risk factor for acute kidney injury (AKI) after heart operations. Many improvements to CPB technology have been proposed during the past decade to limit the hemodilution-related AKI risk. The present study is a retrospective analysis of the relationship between hemodilution during CPB and AKI in cardiac operations in the setting of different interventions applied over 14 years. Methods We retrospectively analyzed 16,790 consecutive patients undergoing heart operations from 2000 to 2013. Various risk factors for AKI were collected and analyzed, together with a number of interventions as possible modifiers of the relationship between a nadir hematocrit (HCT) value during CPB and AKI. Results The relationship between the nadir HCT value during CPB and AKI was confirmed in a multivariable analysis, with the relative risk of AKI increasing by 7% per percentage point of decrease of the nadir HCT value during CPB. The relative risk of AKI decreased by 8% per year of observation ( p = 0.001) despite a significantly increased risk of AKI ( p = 0.001). A sensitivity analysis based on differences before and after different interventions demonstrated a beneficial effect of the application of goal-directed perfusion (aimed at preserving oxygen delivery during CPB), with a reduction in the AKI rate from 5.8% to 3.1% ( p = 0.001). A policy restricting angiographic examination on the day of operation was also useful (reduction of AKI rate from 4.8% to 3.7%; p = 0.029). Conclusions A bundle of interventions mainly aimed at limiting the renal impact of hemodilution during CPB is effective in reducing the AKI rate.

Aims Supraventricular arrhythmias are a major cause of morbidity and mortality in adult patients with congenital heart disease (CHD). Intraoperative ablation offers an alternative for patients who failed ablation procedures or are requiring concomitant surgical intervention. We present our long-term results with the surgical treatment of arrhythmias in adults with CHD (ACHD) undergoing elective cardiac surgery and the clinical predictors for arrhythmia recurrence. Methods and results Between 2002 and 2013, 80 consecutive patients with CHD, mean age of 39 years, underwent intraoperative ablation with monopolar irrigated radiofrequency during cardiac surgery procedures. Significant clinical predictors of arrhythmia recurrence were determined by univariate analysis. We performed 47 right-sided Maze procedures, and 33 Cox-Maze III procedures. In 75 survivors, the ablation was effective immediately. Over an average follow-up period of 72 months (12–155 months), arrhythmias recurred in nine (20%) patients after right-sided Maze, and in six (19%) patients after Cox-Maze III. Eleven patients were controlled with medical therapy, three underwent catheter ablation of the arrhythmia, and one required a permanent pacemaker. Preoperative arrhythmia length ≥3 years ( P ≤ 0.001), tetralogy of Fallot ( P ≤ 0.006), and preoperative atrial fibrillation ( P ≤ 0.016) were associated with recurrence arrhythmia. Conversely, NYHA class

Background Despite advances in treatment of cardiogenic shock (CS), the incidence of this serious complication of acute ST-elevation myocardial infarction (STEMI) has stayed relatively constant, and rates of mortality, although somewhat improved in recent decades, remain dauntingly high. Although both percutaneous coronary intervention (PCI) and coronary artery bypass grafting (CABG) are used in patients with CS with multivessel coronary disease, the optimal revascularization strategy in this setting remains unknown. Methods We conducted a literature search and review of English language publications on CS in multiple online medical databases. Studies were included if they were (1) randomized controlled trials or observational cohort studies, (2) single-center or multicenter reports, (3) prospective or retrospective studies, and (4) contained information on PCI and CABG. Non-English language studies were excluded. Results Our search retrieved no published findings from randomized clinical trials, and only 4 observational reports evaluating PCI versus CABG. Our review of the limited available data suggests similar mortality rates with CABG and PCI in patients with STEMI and multivessel coronary disease complicated by CS. Conclusions Limited data from observational studies in patients with CS and multivessel disease suggest that CABG should be considered a complementary reperfusion strategy to PCI and may be preferred, especially when complete revascularization with PCI is not possible. Our data highlight the need for large randomized trials to further evaluate the relative benefit of PCI versus CABG in patients with multivessel coronary disease and CS using contemporary surgical and percutaneous techniques.

Transcatheter aortic valve replacement determined a paradigm shift in the treatment of high-risk patients with severe symptomatic aortic valve stenosis. Notwithstanding the impressive results of the first-generation prostheses, a fast-paced technological evolution is taking place to overcome their limitations, in particular the vascular access damage and the paravalvular leak. Nowadays, with the availability of several different devices, the expert operator can select the right prosthesis for the specific anatomical and clinical situation. As ‘One does not fit all’, the ‘Tailored TAVR Approach’ we describe will conceivably become the future of this therapy.

Mucormycosis is an angioinvasive infection caused by ubiquitous filamentous fungi of the order Mucorales. We describe a case of rhinocerebral mucormycosis presented in a paediatric patient after cardiac surgery correction of a complete atrioventricular canal defect. The rhinocerebral form of mucormycosis in our patient presented as an important epistaxis that needed immediate intubation due to blood inhalation. Furthermore, due to the worsening of pulmonary function, the patient also needed mechanical support with veno-arterial extracorporeal membrane oxygenation. The patient died as a consequence of a disseminated form of fungal infection. We describe our experience of this rare opportunistic infection and we think that early recognition of the disease could help in proper management.

In a recent paper, Burri and colleagues (1) addressed the complex theme of bioprosthesis durability in patients with congenital heart disease (CHD) operated of tricuspid valve replacement (TVR). The authors reviewed a consecutive series of 51 patients (mean age 32 years) affected by CHD (mainly Ebstein disease) operated over a 25 years period of TVR with a bioprosthesis.

A 21-year-old woman affected by a rare autosomal-dominant disorder called Singleton-Merten syndrome [(1)][1] with symptomatic severe aortic regurgitation in New York Heart Association functional class III was referred for consideration of aortic valve intervention. Her clinical history was

Congenital mitral valve disease is a real challenge in infants and small children. The implantation of expandable stented valves in mitral position has become one of the options of choice in consideration of their acceptable short-term expandable durability and subsequent feasibility of balloon expansion of these devices. We report the first case of a surgical Melody valve-in-valve procedure for early Melody valve degeneration in the mitral position. The result was good, and the procedure safe, rapid, and without an increased risk when a mechanical valve implantation will be necessary.

Background Global population aging and greater age-related incidence of ischemic, degenerative and calcific valve disease have led to an increasing number of very elderly patients being referred for valve surgery. However, their preoperative risk factors, and in-hospital and long-term outcomes have not been thoroughly investigated. Methods Three hundred seven consecutive patients 80 years and older (60% female; mean age 83±2.4 years) attending three major Italian cardiac centres to undergo valve surgery were evaluated. Seventy-nine patients underwent mitral valve surgery (isolated n=30, combined n=49) and 228 underwent aortic valve surgery (isolated n=134, combined n=94). Results The most frequent in-hospital complications were atrial arrhythmias, need for inotropic support for more than 48 h, renal insufficiency, congestive heart failure, respiratory failure, and stroke or transient ischemic attack. The in-hospital mortality rate was 9.7% (30 of 307). Multivariate logistic regression identified the following clinical variables as predictors of in-hospital death: New York Heart Association functional class IV, diabetes, hypertension, renal insufficiency at presentation, rheumatic etiology and left ventricular ejection fraction of less than 45%. Late mortality occurred in 45 of 277 patients (16.2%), but there was a substantial improvement in the New York Heart Association functional class of the 232 long-term survivors (from 3.0±0.7 to 1.7±0.6; P Conclusions Surgery seems to be an effective therapeutic option for selected symptomatic octogenarians with valve disease, associated with good long-term survival and an improved functional class. Operative mortality is related more to patients' preoperative clinical status and increased comorbidity than the type of surgery per se.

BackgroundSurgical mortality for acute type A aortic dissection reported in different experiences from single centers or surgeons varies from 7% to 30%. The International Registry of Acute Aortic Dissection, collecting patients from 18 referral centers worldwide, identifies a preoperative risk stratification scheme and a real average surgical mortality for acute type A aortic dissection in the current era.MethodsA comprehensive analysis was completed of 290 clinical variables and their relationship to surgical outcomes in 526 of 1032 patients enrolled in the International Registry of Acute Aortic Dissection from 1996 through 2001. Extracted cases, categorized according to risk profile, were defined as unstable (group I) in the presence of cardiac tamponade; shock; congestive heart failure; cerebrovascular accident; stroke; coma; myocardial ischemia, infarction, or both; electrocardiograms with new Q waves or ST elevation; acute renal failure; or mesenteric ischemia-infarction at the time of the operation. Outside of an unstable condition, patients were categorized as stable (group II).ResultsThe overall in-hospital mortality was 25.1%. Mortality in group I was 31.4% compared with 16.7% in group II (P < .001). Independent preoperative predictors of operative mortality were history of aortic valve replacement (odds ratio = 3.12), migrating chest pain (odds ratio = 2.77), hypotension as sign of acute type A aortic dissection (odds ratio = 1.95), shock or tamponade (odds ratio = 2.69), preoperative cardiac tamponade (odds ratio = 2.22), and preoperative limb ischemia (odds ratio = 2.10).ConclusionsThe International Registry of Acute Aortic Dissection experience confirms that patient selection plays an important role in determining surgical outcomes in patients with acute type A aortic dissection. Knowledge of significant risk factors for operative mortality can contribute to better management and a more defined risk assessment in patients affected by acute type A aortic dissection.

Objective:Preoperative intra-aortic balloon pump use in high-risk patients undergoing surgical coronary revascularization is still a matter of debate. The objective of this study is to determine whether the preoperative use of an intra-aortic balloon pump improves the outcome after coronary operatio

Aim To estimate the prevalence of cerebral aneurysms in patients previously treated for left cardiac myxoma (LCM). Materials and methods This prospective institutional review board-approved study included patients treated for LCM. All patients treated at our institution (IRCCS Policlinico San Donato, Italy) were telephoned and those enrolled underwent unenhanced brain magnetic resonance imaging (MRI) using sagittal T1-weighted turbo spin-echo (TSE); axial T2-weighted TSE; axial fluid-attenuated inversion-recovery; axial echo-planar diffusion-weighted; and three-dimensional time-of-flight angiographic sequences. Results Seventy-six patients were telephoned, and data regarding their clinical history since tumor resection were obtained for 49 patients (64%). Four of the 49 (8%) patients were deceased, one due to a cerebral hemorrhage from a ruptured cerebral aneurysm 8 years after tumor resection. One patient had a pacemaker preventing MRI. Of the remaining 44 patients, 31 refused MRI and 13 were enrolled (10 females; mean age 64 years). Three of the 13 (23%; two females; 59–78 years) were diagnosed with a cerebral aneurysm, from 2 mm to 4–5 mm in diameter, involving the right middle cerebral artery (n = 2) or the right internal carotid artery (n = 1). Including the deceased patient, the resulting prevalence was 4/14 (29%). Conclusion From this preliminary study, one-third of patients treated for LCM may present with a cerebral aneurysm. Longitudinal large studies are needed to further clarify this matter.

Background— Pulmonary valve replacement (PVR) after repair of tetralogy of Fallot is commonly required and is burdensome. Detailed anatomic and physiologic characteristics of survivors free from late PVR and with good exercise capacity are not well described in a literature focusing on the indications for PVR. Methods and Results— Survival and freedom from PVR were tracked in 1085 consecutive patients receiving standard tetralogy of Fallot repair in a single institution from 1964 to 2009. Of 152 total deaths, 100 occurred within the first postoperative year. Surviving patients between 10 and 50 years of age had an annual risk of death of 4 (confidence limit, 2.8–5.4) times that of normal contemporaries. To date, 189 patients have undergone secondary PVR at mean age of 20±13 years (36% of those alive at 40 years of age). A random sample of 50 survivors (age, 4–57 years) free from PVR underwent cardiovascular magnetic resonance, echocardiography, and exercise testing. These patients had mildly dilated right ventricles (right ventricular end-diastolic volume=101±26 mL/m 2 ) with good systolic function (right ventricular ejection fraction=59±7%). Most had exercise capacity within normal range ( z peak o 2 =−0.91±1.3; z e / co 2 =0.20±1.5). In patients >35 years of age with normal exercise capacity, there was mild residual right ventricular outflow tract obstruction (mean gradient, 24±13 mm Hg), pulmonary annulus diameters z , and unobstructed branch pulmonary arteries. Conclusions— An important proportion of patients require PVR late after tetralogy of Fallot repair. Patients surviving to 35 years of age without PVR and with a normal exercise capacity may have had a definitive primary repair; their right ventricular outflow tracts are characterized by mild residual obstruction and pulmonary annulus diameter z .

Background The population of adults with congenital heart disease (ACHD) is increasing and aging, and a large percentage of this population is now over 65 years of age. For this reason, it is probable that acquired coronary artery disease (CAD) will become an important issue that needs to be addressed also in these patients. We retrospectively analyzed all ACHD patients who underwent surgery in our Institution with the aim to investigate the incidence of associated CAD and the results of surgical treatment. Methods From January 2000 to December 2015, a total of 1154 ACHD underwent surgery in our center. Fifty patients (4.3%) were diagnosed with acquired CAD and required coronary artery bypass grafting. The mean age at surgery was 66 years (range 41-78 years). The primary diagnosis were atrial septal defect (n = 40 patients), Tetralogy of Fallot (n = 4 patients), ventricular septal defect (n = 2 patients), partial AV canal (n = 1), partial anomalous pulmonary venous drainage (n = 1), Ebstein's anomaly (n = 1), and subaortic stenosis (n = 1). Results Hospital mortality was 2% (one patient). During a mean follow-up of 9 years (maximum follow-up: 15 years), seven patients died (14%). The actuarial survival was 83% at 5 years and 77% at 10 years. Freedom from reoperation for coronary artery bypass grafting or percutaneous coronary intervention was 88% at 5 years and 82% at 10 years. Conclusion Acquired CAD may coexist with congenital heart defects but the association is quite rare. It typically occurs later during adulthood and it is usually associated with atrial septal defect. Acquired CAD and congenital heart defects can be treated contemporarily with good early and late results.

IntroductionPatients with repaired tetralogy of Fallot have good long-term survival but less is known about the subjectively assessed quality of life or objectively measured functional status of those who have not required subsequent pulmonary valve replacement. We assessed these parameters in a group of children and adults free from pulmonary valve replacement after tetralogy of Fallot repair.Methods and resultsA random sample of 50 subjects – 16 children and 34 adults, aged 4.1–56.7 years – who had undergone tetralogy of Fallot repair and were free from subsequent pulmonary valve replacement underwent cardiopulmonary exercise testing and completed standardised questionnaires assessing health-related quality of life and resilience. Patients were generally asymptomatic (median New York Heart Association class = I). Exercise capacity was within two standard deviations of normal for most children and adults (mean z VO2max: 0.20 ± 1.5; mean z VE/VCO2: −0.9 ± 1.3). Children reported a total health-related quality of life score similar to healthy norms (78 ± 10 versus 84 ± 1, p = 0.73). Adult survivors also reported quality of life scores comparable to healthy norms. Resilience was highly correlated with all domains of health-related quality of life (r = 0.713, p < 0.0001).ConclusionsPatients who have undergone tetralogy of Fallot repair in childhood and have not required pulmonary valve replacement have a good long-term health-related quality of life. The finding that patients with greater resilience had better health-related quality of life suggests that it may be beneficial to implement interventions to foster resilience.