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Cardiac MRI plays an important role in the diagnosis and follow-up of patients with congenital heart disease (CHD). Gadolinium-based contrast agents are often needed to overcome flow-related and off-resonance artifacts that can impair the quality of conventional noncontrast 3D imaging. As serial imaging is often required in CHD, the development of robust noncontrast 3D MRI techniques is desirable.To assess the clinical utility of noncontrast enhanced magnetization transfer and inversion recovery prepared 3D free-breathing sequence (MTC-BOOST) compared to conventional 3D whole heart imaging in patients with CHD.Prospective, image quality.A total of 27 adult patients (44% female, mean age 30.9 ± 14.8 years) with CHD.A 1.5 T; free-breathing 3D MTC-BOOST sequence.MTC-BOOST was compared to diaphragmatic navigator-gated, noncontrast T2 prepared 3D whole-heart imaging sequence (T2prep-3DWH) for comparison of vessel dimensions, lumen-to-myocardium contrast ratio (CR), and image quality (vessel wall sharpness and presence and type of artifacts) assessed by two experienced cardiologists on a 5-point scale.Mann-Whitney test, paired Wilcoxon signed-rank test, Bland-Altman plots. P 0.05 was considered statistically significant.MTC-BOOST significantly improved image quality and CR of the right-sided pulmonary veins (PV): (CR: right upper PV 1.06 ± 0.50 vs. 0.58 ± 0.74; right lower PV 1.32 ± 0.38 vs. 0.81 ± 0.73) compared to conventional T2prep-3DWH imaging where the PVs were not visualized in some cases due to off-resonance effects. MTC-BOOST demonstrated resistance to degradation of luminal signal (assessed by CR) secondary to accelerated or turbulent flow conditions. T2prep-3DWH had higher image quality scores than MTC-BOOST for the aorta and coronary arteries; however, great vessel dimensions derived from MTC-BOOST showed excellent agreement with standard T2prep-3DWH imaging.MTC-BOOST allows for improved contrast-free imaging of pulmonary veins and regions characterized by accelerated or turbulent blood flow compared to standard T2prep-3DWH imaging, with excellent agreement of great vessel dimensions.1 TECHNICAL EFFICACY: Stage 2.

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...

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.

The right ventricle (RV) is often overlooked in the evaluation of cardiac performance and treatment of left ventricular (LV) heart diseases. However, recent evidence suggests the RV may play an important role in maintaining systemic cardiac function and delivering stroke volume (SV). We used exercise cardiac magnetic resonance and biomechanical modeling to investigate the role of the RV in LV stroke volume regulation. We studied SV augmentation during exercise by pharmacologically inducing negative chronotropy (sHRi) in healthy volunteers and investigating training-induced SV augmentation in endurance athletes. SV augmentation during exercise after sHRi is achieved differently in the two ventricles. In the RV, the larger SV is driven by increasing contraction down to lower end-systolic volume (ESV; P < 0.001). In the LV, SV augmentation is achieved through an increase in end-diastolic volume (EDV; P < 0.001), avoiding contraction to a lower ESV. The same mechanism underlies the enhanced SV response observed in athletes. Changes in atrial area during SV augmentation suggest that the improved LV EDV response is sustained by the larger RV contractions. Using our biomechanical model, we explain this behavior by showing that the RV systolic function-driven regulation of LV SV optimizes the energetic cost of LV contraction and leads to minimization of the total costs of biventricular contraction. In conclusion, this work provides mechanistic understanding of the pivotal role of the RV in optimizing LV SV during exercise. It demonstrates why optimizing RV function needs to become a key part of therapeutic strategies in patients and training for athletes. NEW & NOTEWORTHY The right ventricle appears to have an important impact on maintaining systemic cardiac function and delivering stroke volume. However, its exact role in supporting left ventricular function has so far been unclear. This study demonstrates a new mechanism of ventricular interaction that provides mechanistic understanding of the key importance of the right ventricle in driving cardiac performance.

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.

Summary Screening on hospital admission to identify multidrug-resistant organism (MDRO) colonization is a frequently discussed topic. We report the results of microbiological screening in 141 Italian and 354 migrant children candidates for cardiac surgery conducted in 2015–2016. In all, 25% of Italian children and more than 65.4% of African and Romanian children carried at least one MDRO (meticillin-resistant Staphylococcus aureus; extended-spectrum β-lactamase enzymes; carbapenemase producers; and vancomycin-resistant enterococci). Based on our findings, we propose that non-geographically limited approaches are needed to improve infection prevention and control.

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.

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.

Background The Ross procedure was introduced as a long-term if not definitive solution for aortic pathology. However, the rate of reoperation is not negligible. Methods This single-center prospective study assessed the general outcome of Ross reoperation and patients' perceived quality of life compared with 2 control groups (Ross non-reoperation and mechanical aortic valve replacement). Patient's preference regarding the choice between mechanical aortic valve and Ross procedure was investigated in a subgroup that could theoretically have been directed to either of the 2 procedures. Results Between 2005 and 2017, 64 consecutive patients underwent reoperation after Ross. Median age was 31 years. Median freedom from reoperation after the Ross procedure was 136 months. An autograft reoperation was required in 49, and 25 had homograft failure. No in-hospital death was recorded. Mean follow-up was 77 months (range, 6-164 months). Quality of life was assessed with the 36-Item Short Form Health Survey questionnaire. The Ross reoperation group showed a lower score involving psychological concerns compared with the other groups. In the reoperated-on patients group, 52 had adequate aortic annulus dimensions to receive a prosthetic valve instead of a Ross procedure. When asked whether they would make the same choice, only 31% confirmed the preference. Conclusions Reoperations after Ross procedure have low mortality and morbidity. Long-term follow-up showed a high quality of life, even after reoperations. However, owing to psychological concerns after the redo operation, when choosing a Ross procedure, it is our duty to thoroughly explain to patients that a high level of disillusion is predictable in case of reoperations.

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.

BackgroundIn 2007, a partnership was initiated between a small-volume paediatric cardiac surgery unit located in Las Palmas de Gran Canaria, Spain, and a large-volume cardiac surgery unit located in Milan, Italy. The main goal of this partnership was to provide surgical treatment to children with CHD in the Canary Islands.MethodsAn operative algorithm for performing surgery in elective, urgent, and emergency cases was adopted by the this joint programme. Demographic and in-hospital variables were collected from the medical records of all the patients who had undergone surgical intervention for CHD from January, 2009 to March, 2013. Data were introduced into the congenital database of the European Congenital Heart Surgeons Association Congenital Database and the database was interrogated.ResultsIn total, 65 surgical mission trips were performed during the period of this study. The European Congenital Heart Surgeons Association Congenital Database documented 214 total patients with a mean age at operation of 36.45 months, 316 procedures in total with 198 cardiopulmonary bypass cases, 46 non-cardiopulmonary bypass cases, 26 cardiovascular cases without cardiopulmonary bypass, 22 miscellaneous other types of cases, 16 interventional cardiology cases, six thoracic cases, one non-cardiac, non-thoracic procedure on a cardiac patient with cardiac anaesthesia, and one extracorporeal membrane oxygenation case. The 30-day mortality was 6.07% (13 patients).ConclusionsA joint programme between a small-volume centre and a large-volume centre may represent a valid and reproducible model for safe paediatric cardiac surgery in the context of a peripheral region.

OBJECTIVES The existing risk stratification scores for paediatric patients undergoing cardiac surgery include the Aristotle Basic Complexity (ABC) Score, the Risk Adjustment in Congenital Heart Surgery-1 (RACHS-1) Score and the Aristotle Comprehensive Complexity (ACC) Score. They are all based on the nature of the surgical operation (ABC and RACHS-1 Scores) with possible adjustment for a number of patient conditions (ACC Score). The present study investigates if the early postoperative parameters may be used to improve the preoperative mortality risk prediction. METHODS A retrospective study on 1392 consecutive patients aged ≤12 years old, undergoing cardiac surgery with cardiopulmonary bypass and without a residual right-to-left shunt was conducted. The ABC Score and metabolic and respiratory postoperative parameters at arrival in the intensive care unit were tested for association and discriminative power for operative mortality. RESULTS The ABC yielded a c-statistic of 0.746. Additional independent predictors of operative mortality were postoperative hypoxia [Formula: see text] and arterial blood lactates. In a multivariable model including the ABC Score, postoperative hypoxia and arterial blood lactates remained independently associated with operative mortality. A modified ABC Score was created, consisting of the ABC Score plus 1.5 points in case of postoperative hypoxia plus 1 point per each 1 mmol/l of arterial blood lactates. The new model was significantly (P = 0.043) more discriminative than the ABC Score, with a c-statistic of 0.803. CONCLUSIONS Early postoperative respiratory and metabolic parameters increased the accuracy and discrimination of the ABC Score. An external validation is needed to confirm our results.

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 Hypoxia is a common feature of many congenital heart defects (CHDs) and significantly contributes to their pathophysiology. Thus, understanding the mechanism underlying cell response to hypoxia is vital for the development of novel therapeutic strategies. Certainly, the hypoxia inducible factor (HIF) has been extensively investigated and it is now recognized as the master regulator of cell defense machinery counteracting hypoxic stress. Along this line, we recently discovered and reported a novel mechanism of HIF activation, which is mediated by sialidase NEU3. Thus, aim of this study was to test whether NEU3 played any role in the cardiac cell response to chronic hypoxia in congenital cyanotic patients. Methods Right atrial appendage biopsies were obtained from pediatric patients with cyanotic/non-cyanotic CHDs and processed to obtain mRNA and proteins. Real-Time PCR and Western Blot were performed to analyze HIF-1α and its downstream targets expression, NEU3 expression, and the NEU3 mediated effects on the EGFR signaling cascade. Results Cyanotic patients showed increased levels of HIF-1α, NEU3, EGFR and their downstream targets, as compared to acyanotic controls. The same patients were also characterized by increased phosphorylation of the EGFR signaling cascade proteins. Moreover, we found that HIF-1α expression levels positively correlated with those recorded for NEU3 in both cyanotic and control patients. Conclusions Sialidase NEU3 plays a central role in activating cell response to chronic hypoxia inducing the up-regulation of HIF-1α, and this represent a possible novel tool to treat several CHD pathologies.

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.

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.

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 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.

Aims Paediatric patients with mitral valve (MV) disease have limited options for prosthetic MV replacement. Based on long experience with the stented bovine jugular vein graft (Melody valve) in the right ventricular outflow tract, we aimed to test the use of the Melody valve as a surgical implant in the mitral position in a small group of infants with congenital mitral disease. Methods and results Eight patients, aged from 3 months to 6.2 years, with congenital MV dysplasia underwent Melody valve implantation in the mitral position between March 2014 and October 2015. Once sutured to the MV annulus, the Melody valve was expanded through a balloon catheter to achieve the best diameter (12-20 mm). Two patients needed ECMO assistance after traditional MV repair and a Melody valve was successfully implanted as possible bail-out. However, neither of them recovered from multi-organ failure and both died, in spite of the well-functioning Melody prosthesis. During follow-up, only one patient underwent catheter-based balloon expansion of the valve. Conclusions The medium-term results of this procedure are encouraging. The Melody valve opens up the opportunity to carry out MV replacement in more children at an earlier time point, and it has potential to revolutionise the treatment of MV disease.

Maternal antenatal therapy with glucocorticoids (GC) is routinely used to prevent lung immaturity. The potential harmful effects on other organs, including in particular the central nervous system (CNS), are still controversial. In the present review we aimed to investigate: i) the beneficial and detrimental effects of antenatal GC treatment in both human and animal models; ii) the potential usefulness of biochemical markers such as calcium binding proteins (S100B, synaptophysin) and cytoskeletal protein of neurons and dendrites (MAP2) in the perinatal period, and iii) whether the assessment of brain markers in different biological fluids could constitute a promising tool for the monitoring of CNS function and/or developmental in fetuses and newborns whose mothers assumed GC antenatally.

Background. S100B protein, previously proposed as a consolidated marker of brain damage in congenital heart disease (CHD) newborns who underwent cardiac surgery and cardiopulmonary bypass (CPB), has been progressively abandoned due to S100B CNS extra-source such as adipose tissue. The present study investigated CHD newborns, if adipose tissue contributes significantly to S100B serum levels.Methods. We conducted a prospective study in 26 CHD infants, without preexisting neurological disorders, who underwent cardiac surgery and CPB in whom blood samples for S100B and adiponectin (ADN) measurement were drawn at five perioperative time-points.Results. S100B showed a significant increase from hospital admission up to 24 h after procedure reaching its maximum peak(P<0.01)during CPB and at the end of the surgical procedure. Moreover, ADN showed a flat pattern and no significant differences(P>0.05)have been found all along perioperative monitoring. ADN/S100B ratio pattern was identical to S100B alone with the higher peak at the end of CPB and remained higher up to 24 h from surgery.Conclusions. The present study provides evidence that, in CHD infants, S100B protein is not affected by an extra-source adipose tissue release as suggested by no changes in circulating ADN concentrations.

AIMS Nowadays, transcatheter approaches are the treatment of choice for several congenital heart defects. However, adverse events may occur during interventional procedures. Even if the complication rate has been reduced remarkably because of learning curve and technological improvements, catastrophic events are still possible. The aim of this study was to review cardiac catheter complications that required surgical treatment during or after a percutaneous procedure. METHODS AND RESULTS We evaluated retrospectively a thirteen-year experience at our centre. We examined all transcatheter procedures involving device release or implantation needing surgical rescue. We performed 3,205 interventional catheterisation procedures with device release or implantation: ASD device closure (n=2,205), PDA device occlusion (n=355), VSD device closure (n=218), aortic coarctation or recoarctation stenting (n=199), pulmonary artery stenting (n=154) and pulmonary valve implantation (n=74). Complications that required surgical treatment occurred in 1.2% of cases. Early surgery was performed in 22 cases, while in 18 patients a surgical treatment related to late complications was performed in a mean follow-up of 17 months. There were no deaths in either group. CONCLUSIONS A spectrum of CHD can be treated today by transcatheter interventional procedures with good results and a low, but not negligible, risk of complications that require a surgical operation. The risk of developing late complications makes a long-term follow-up mandatory in such patients.

Abnormal connection of the right superior caval vein to the left atrium is an uncommon systemic vein drainage anomaly, with only a few cases reported among congenital heart disease (CHD), around 20 cases published in the medical literature. The inferior vena cava connection with the left atrium, also very rare, can appear directly or in heterotaxy. Clinical suspicion arises due to the presence of cyanosis in the absence of other specific clinical signs (without other associated CHD). We present the cases of two children with abnormal superior and inferior systemic venous return. The first case is an abnormal connection of right superior vena cava to the left atrium associated with persistent left superior vena cava draining into the right atrium through the coronary sinus. The second case is an interruption of the inferior vena cava with hemiazygos continuation, drained into the left superior vena cava, which drained into the left atrium. The diagnosis was imagistic - echocardiography and angiography. Surgical treatment solutions vary from one case to another, usually following anatomic correction. Hypoxia accompanied by cyanosis must bring into question the pathology of systemic venous drainage anomaly, after other common causes have been excluded. Surgery is indicated in all cases due to the risk associated with the presence of right-to-left shunt.

Objective To evaluate the scale and clinical importance of loss to follow-up of past patients with serious congenital heart disease, using a common malformation as an example. To better understand the antecedents of loss to specialist follow-up and patients’ attitudes to returning. Design Cohort study using NHS number functionality. Content and thematic analysis of telephone interviews of subset contacted after loss to follow-up. Patients, intervention and setting Longitudinal follow-up of complete consecutive list of all 1085 UK patients with repair of tetralogy of Fallot from single institution 1964–2009. Main outcome measures Survival, freedom from late pulmonary valve replacement, loss to specialist followup, shortfall in late surgical revisions related to loss to follow-up. Patients’ narrative about loss to follow-up. Results 216 (24%) of patients known to be currently alive appear not to be registered with specialist clinics; some are seen in general cardiology clinics. Their median age is 32 years and median duration of loss to follow-up is 22 years; most had been lost before Adult Congenital services had been consolidated in their present form. 48% of the late deaths to date have occurred in patients not under specialist follow-up. None of those lost to specialist follow-up has had secondary pulmonary valve replacement while 188 patients under specialist care have. Patients lost to specialist follow-up who were contacted by telephone had no knowledge of its availability. Conclusions Loss to specialist follow-up, typically originating many years ago, impacts patient management.

Aims We aimed to assess the impact of surgical pulmonary valve replacement (PVR) for severe pulmonary regurgitation (PR) on biventricular function and its effect on exercise capacity. Methods and results Seventy-three patients (mean age 23.6 ± 11.5 years, 47 females) underwent surgical PVR for PR. Echocardiogram and magnetic resonance imaging to assess ventricular size and function, and a cardiopulmonary exercise test were performed before, and 1-year post-surgery. Median New York Heart Association class improved from 2 to 1 but peak oxygen uptake (VO2) did not change. Left ventricular (LV) cardiac output increased from 3.2 ± 0.9 to 3.5 ± 0.7 L/min ( P = 0.003). However, this was not associated with increased trans-mitral velocities (▵ E = −0.13, P = 0.004; ▵ A = 0.03, P = 0.395), or increased heart rate (−0.002%, P = 0.993). Trans-tricuspid rapid right ventricular (RV) filling increased significantly, whereas early diastolic myocardial velocity in RV wall decreased ( E velocity: 0.57 ± 0.14–0.65 ± 0.21, P = 0.034; and E / e ′ from 6.7 ± 1.9 to 14.8 ± 7.0, P < 0.0001). RV and LV late diastolic velocities and their ratio to early velocities ( A , a ′, E / A , and e ′/ a ′) correlated with pre- and/or post-PVR peak VO2. No correlations were found between indexes of systolic function and peak VO2, either before or after surgery. Doppler evidence of restrictive RV physiology resolved after elimination of PR. Conclusion Surgical PVR for PR improves RV filling and increases left ventricular stroke volume, however, this could not be demonstrated by conventional Doppler echocardiography. Diastolic ventricular function was associated with exercise capacity. Because of its load dependency, E / e ′ ratio failed in assessing diastolic function. Pre-systolic flow in pulmonary trunk in presence of severe PR does not determine intrinsic myocardial stiffness.

We sought to evaluate the long-term performance of a consecutive cohort of patients implanted with a 17-mm bileaflet mechanical prosthesis.Between January 1995 and December 2005, 78 patients (74 women, mean age=71±12 years) underwent aortic valve replacement with a 17-mm mechanical bileaflet prosthesis (Sorin Bicarbon-Slim and St. Jude Medical-HP). Preoperative mean body surface area and New York Heart Association class were 1.6±0.2 m2 and 2.6±0.8, respectively. Preoperative mean aortic annulus, indexed aortic valve area, and peak and mean gradients were 18±1.6 mm, 0.42 cm2/m2, 89±32 mm Hg, and 56±21 mm Hg, respectively. Patients were divided into two groups, according to the presence (group A, 29 patients) or absence of patient-prosthesis mismatch (group B, 49 patients). Patient-prosthesis mismatch was defined by an indexed effective orifice area less than 0.85 cm2/m2.Overall hospital mortality was 8.8%. Follow-up time averaged 86±44 months. Actuarial 5-year and 10-year survival rates were 83.7% and 65.3%, respectively. The mean postoperative New York Heart Association class was 1.3±0.6 (p0.001). Overall indexed left ventricular mass decreased from 163±48 to 120±42 g/m2 (p0.001), whereas average peak and mean prosthesis gradients were 28±9 mm Hg and 15±6 mm Hg, respectively (p0.001). Early and long-term mortality were similar between the two groups as well as long-term hemodynamic performance (mean peak gradient was 28 mm Hg and 27 mm Hg in group A and B, respectively, not significant); left ventricular mass regression occurred similarly in both groups (indexed left ventricular mass at follow-up was 136±48 and 113±40 in group A and B, respectively; not significant).Selected patients with aortic stenosis experience satisfactory clinical improvement after aortic valve replacement with modern small-diameter bileaflet prostheses.

The authors present the case of a 41-year-old woman, who presented with moderate pericardial effusion and bilateral pleural effusion 11 months following a secundum atrial septal defect closure with an Amplatzer septal occluder (ASO) device. The acute presentation responded well to diuretics and a short course of corticosteroid therapy. The patient, however, continued to experience pleuritic chest pain and a recurrence of pericardial effusion a month later. The patient showed significant symptomatic improvement with a 2-month course of down-titrating prednisolone. Six months later, the echocardiogram showed complete remission of pleural and pericardial effusion. The possibility of cardiac erosion following ASO implantation has been excluded. The aetiology of the reoccurrence of steroid-responsive pleuropericarditis remains unknown.

Background: The percutaneous closure of single atrial septal defect (ASD) is a valid alternative to surgery. Objectives: To assess the feasibility of percutaneous treatment of multiple ASDs. Methods: Between 1998 and 2007, 165 out of 1280 consecutive patients undergoing ASD percutaneous closure at our institution showed multiple defects that were classified in four categories: double atrial septal defects (d-ASD), multifenestrated atrial septal defects (f-ASD), multifenestrated defects with no signs of right heart overload (f-PFO), and complex cases (c-ASD). The following end points were taken into consideration: (1) immediate procedural success; (2) long term safety and efficacy. In this study, up to 81% of multiple ASDs were suitable for percutaneous closure. Results: Multiple device implantations were required in 47% of cases, especially in patients with d-ASD and c-ASD. Complication rate, residual shunt, and long term outcome were comparable among the four different categories. In particular, at long term follow-up (6 ± 2 years) no patient required further surgical or percutaneous treatment and complete closure was confirmed in 99% of cases. Conclusions: Percutaneous closure of multiple ASDs is feasible and associated with a good outcome. A thorough identification and analysis of morphological aspects are mandatory in order to select the appropriate device and the optimal strategy. © 2010 Wiley-Liss, Inc.

Cerebral monitoring constitutes an emerging issue in perinatal medicine. Near Infrared Spectroscopy (NIRS) monitors brain oxygenation status in sick infants although data in healthy infants are lacking. The present study investigates whether NIRS parameters change according to gestational age and correlate with S100B protein. We recruited 64 healthy newborns (weeks' gestation: 30-42 wks) in which we performed in the first 6-hours after birth routine clinical, radiological and laboratory variables, cerebral oxygen saturation (rSO2), fractional cerebral tissue oxygen extraction (FTOE) values and S100B urine assessment. rSO2 and FTOE correlated (R=-0.73; R=0.51; P less than 0.01, for both) with gestational age. Highest rSO2 and the lowest FTOE peaks (P less than 0.001) were found at 30-33 wks. From 34 wks onwards, rSO2 progressively decreased and FTOE increased reaching their lower dip/peak (P less than 0.001) at 38-39 weeks. A significant correlation between S100B and NIRS parameters (rSO2: r=0.77; FTOE: r=-0.69; P less than 0.01) has been found. The present study shows that NIRS parameters and S100B protein correlation may be of help in brain function monitoring.

We investigated the levels of S100 beta protein (S100B) in the serum of fetuses with neural tube defects (NTD), and their mother. Samples from 20 fetuses with NTD and 30 controls at the same gestational age, and their mothers, were studied. S100B protein levels were determined using Lia-mat Sangtec. kit. S100B concentrations were significantly higher in NTD fetuses (median 2.71 microg/L) than in control subjects (median 0.98 microg/L). Increased S100B levels were also found in mothers carrying fetuses with NTD compared to control and uncomplicated pregnancies. This study indicates that NTD is associated with increased serum concentration of S100B in fetuses and mothers. Moreover, it gives information on S100B levels in the fetal circulation in early-mid gestation.

Background Reoperations represent relatively frequent events in adults with congenital heart disease (ACHD). Cardiac operations in these patients present major difficulties in management and technique. Although reoperations in ACHD are becoming increasingly frequent, limited knowledge exists regarding perioperative risk factors. Methods The study included 164 ACHD patients who underwent cardiac reoperations between January 2002 and December 2007 at our institution. Preoperative and intraoperative data were analyzed to identify morbidity and mortality risk factors. Results Reoperations included pulmonary valve implantation or conduit replacement in 60, aortic valve/root procedures in 36, residual atrial or ventricular septal defect closure in 19, and Fontan operation/conversion in 19. Hospital mortality was 3.6%. The mean mechanical ventilation time was 26 hours. Mean intensive care unit stay was 3.1 days. Severe postoperative complications occurred in 24 (15.1%). Cardiopulmonary bypass time (p = 0.001), Fontan operation/conversion (p = 0.001), preoperative hematocrit (p = 0.004), previous number of operations (p = 0.001), and preoperative congestive heart failure (p = 0.021) were associated with severe morbidity. No factor was associated with death. Conclusions Reoperations in ACHD are mostly due to right ventricular outflow tract lesions and were associated with a low mortality rate if performed in a center with a considerable activity and a dedicated program. Severe morbidity is relatively frequent and is generally associated with the preoperative (high hematocrit due to cyanosis, congestive heart failure, and the number of previous operations) and operative (Fontan operation/conversion and cardiopulmonary bypass duration) conditions of the patient.

Background: Thrombocytopenia after cardiac operations is a common event in both adult and pediatric patients. Late thrombocytopenia (LTCP) is a less common event that is still without a well-recognized cause. This study explores the role of heparin-induced thrombocytopenia (HIT) and other factors (complexity of the operation, temperature management, and drug use) in determining LTCP. Methods: We conducted an observational study of 63 consecutive patients aged

Near Infrared Spectroscopy (NIRS) is an emerging technique for brain oxygenation monitoring in newborns complicated by acute and chronic hypoxia. However, data regarding cerebral oxygenation normal values are still lacking and matter of debate. Therefore, we investigate whether NIRS parameters in healthy preterm/term infants are gestational age and delivery modalities dependent and correlate with standard monitoring parameters. From January to December 2007, 100 healthy newborns with gestational age from 30 to 42 weeks' gestation were evaluated. Routine laboratory variables, daily clinical and neurological evaluation and ultrasound imaging were performed. The regional cerebral oxygen saturation (rSO2) and fractional cerebral tissue oxygen extraction (FTOE) were measured by NIRS in the first 6-hours after birth. Data were recorded by MetaVision ICU X-Edition software and analyzed by SPSS statistical package. rSO2 and FTOE correlated (R=-0.77; R=0.41; P

Background Scimitar syndrome is a rare congenital heart disease. To evaluate the results of managing this malformation surgically, we have embarked on a multicenter Italian study involving seven different centers and reporting the largest published series in the medical literature. Methods From January 1997 to December 2007, 26 patients with scimitar syndrome who underwent surgical correction were included. Primary outcomes include hospital mortality and the efficacy of repair at the follow-up. Results Median age was 11 years (interquartile range, 1.8 to 19.9 years). Nineteen patients (73%) presented with symptoms including upper respiratory tract infections (n = 13), recurrent pneumonia (n = 10), cardiac failure (n = 4), and cyanosis (n = 2). Associated cardiac anomalies were present in 16 patients (63%). Surgical repair included intraatrial baffle repair in 18 patients (69%; group 1), and reimplantation of the "scimitar vein" onto the left atrium in 8 patients (31%; group 2). One patient died in hospital (3.8%; group 1). Postoperative complications were less frequently reported in group 1 (4 of 18 patients, 22%) compared with group 2 (5 of 8 patients, 62%). Median follow-up time was 4 years (interquartile range, 1.8 to 9.7 years). There was 1 late death (1/25 patients, 4%; group 2). Four patients (16%) showed a complete occlusion of the scimitar drainage (2 in group 1, 12%; 2 in group 2, 25%) and 3 patients (12%) required balloon dilation or stenting for scimitar vein stenosis (1 in group 1, 6%; 2 in group 2, 25%). Conclusions The intraatrial baffle repair seems to have a lower incidence of postoperative complications and a better patency rate, at last follow-up, than the reimplantation of the scimitar vein onto the left atrium.

Aims To prospectively evaluate homograft function with cardiac magnetic resonance (CMR) imaging 1 year after insertion into the pulmonary position, and to assess the impact of in situ homograft geometry, surgical factors, and ‘intrinsic’ homograft properties on early valve incompetence. Methods and results A total of 60 patients (mean age 21 ± 10 years; 35 females) with congenital heart disease underwent pulmonary valve replacement with homograft insertion and were prospectively enrolled into a study protocol that included serial echocardiography and CMR 1 year after surgery. None of the patients had homograft stenosis but 10 (17%) had significant homograft incompetence (i.e. pulmonary regurgitation fraction >20% on CMR). A higher incidence of ‘eccentric’ pulmonary forward flow pattern ( P < 0.001, Fisher's exact test), more acute ‘homograft distortion angle’ ( P < 0.001), larger relative ‘annular’ size ( P < 0.01), and greater pre-homograft right ventricular outflow tract (RVOT) diameters ( P = 0.01) at CMR was seen in those with worse homograft function. In a backward multivariate linear regression model, ‘eccentric’ pulmonary forward flow pattern ( r part = 0.36, P < 0.001), ‘homograft distortion angle’ ( r part = 0.31, P = 0.001), and pre-homograft RVOT diameter ( r part = 0.19, P = 0.03) were independently associated with the degree of pulmonary regurgitation (in %) at 1 year. Conclusion Using CMR, in this prospective cohort study, we have shown that significant valve incompetence is present in one-sixth of patients after homograft insertion into the pulmonary position, and that alterations in the in situ homograft geometry were associated with the likelihood of developing valve incompetence. These findings imply that mechanical factors may have an important impact on homograft performance.

Background Percutaneous closure of atrial septal defect (ASD) is a valid alternative to surgical approach. Current device has significantly improved the success rate also in complex cases. The aim of this study is to assess the impact of age, defect size, and morphologic features on successfully percutaneous ASD closure. Methods Between January 2000 and September 2004, 1,013 consecutive patients underwent closure of an isolated type II ASD at our institution. The following outcomes have been evaluated: (1) role of percutaneous ASD closure as alternative to surgical repair, in current daily practice; (2) impact of age on the selected closure approach; (3) analysis of morphologic variety of ASD and its effect on the closure technique; (4) possible role of specific device selection according to ASD morphology to improve procedural success. Results During the study period, up to 80% of secundum ASDs were suitable for percutaneous closure with the currently available devices. Need for surgical ASD closure was more common in pediatric patients, likely reflecting the more frequent diagnosis of larger and complex defects at a young age. Accurate ADS morphology assessment and appropriate device selection are key elements to obtain procedural success. In particular, among all the ASD characteristics, the rim absence is the main limiting factor to a successful percutaneous ASD closure. A trend of reduction in peri-procedural adverse events was observed during the study period, with complications needing immediate cardiac surgery occurred only in 1% of cases. Conclusions Percutaneous ASD closure is feasible and associated with low complication rate. A thorough analysis of morphologic aspects is mandatory in order to select the appropriate device and the optimal approach. Surgical closure remains the treatment of choice in selected patients.

Background This is a clinical investigation of the mid- to long-term follow-up of the Ross procedure in adult patients. The primary end point is to explore the incidence and risk factors for a reoperation on the pulmonary autograft. The secondary end points are to explore the incidence of neoaortic root dilation and valve regurgitation, and the echocardiographic profile leading to a reoperation. Methods Ross operations were done in 110 adults who received at least two echocardiographic examinations for a mean follow-up time of 82 months (range, 5 to 155 months). Kaplan-Meier and Cox regression analyses were applied to assess freedom from events and risk factors for events. Results Freedom from reoperation on the pulmonary autograft, neoaortic root dilation, and moderate-severe neoaortic valve regurgitation were, respectively, 91.4%, 50%, and 70% at 12 years. The main risk factor for a reoperation was the degree of neoaortic valve regurgitation within the first 2 years of follow-up. Patients requiring an early (≤4 years) reoperation had early and severe pulmonary autograft valve regurgitation, and no neoaortic root dilation. Patients needing a late (>4 years) reoperation had severe neoaortic root dilation and severe neoaortic valve regurgitation. The left ventricular end-diastolic diameter at the third year of follow-up was a risk factor for late reoperation. Conclusions The Ross operation in adults is a safe procedure with good clinical results in mid- to long-term follow-up. Early reoperations are due to early neoaortic valve regurgitation, wheras late reoperations are due to progressive neoaortic root dilation.

Percutaneous closure of secundum atrial septal defects may be complicated by immediate or late device embolization. Percutaneous retrieval techniques are usually successful even if sometimes surgery is required. The optimal selection of an atrial septal defect and the choice of appropriate device sizes are fundamental to reduce the incidence of embolization. We describe a case of late device embolization occurring two years after transcatheter atrial septal defect closure and for which percutaneous retrieval was unsuccessfully attempted.

Background Postoperative bleeding after heart operations is still a common finding, leading to allogeneic blood products transfusion. Fibrinogen and coagulation factors deficiency are possible determinants of bleeding. The experimental hypothesis of this study is that a first‐line fibrinogen supplementation avoids the need for fresh frozen plasma ( FFP ) and reduces the need for any kind of transfusions. Methods and Results This was a single‐center, prospective, randomized, placebo‐controlled, double‐blinded study. One‐hundred sixteen patients undergoing heart surgery with an expected cardiopulmonary bypass duration >90 minutes were admitted to the study. Patients in the treatment arm received fibrinogen concentrate after protamine administration; patients in the control arm received saline solution. In case of ongoing bleeding, patients in the treatment arm could receive prothrombin complex concentrates ( PCC s) and those in the control arm saline solution. The primary endpoint was avoidance of any allogeneic blood product. Patients in the treatment arm had a significantly lower rate of any allogeneic blood products transfusion (odds ratio, 0.40; 95% confidence interval, 0.19 to 0.84, P =0.015). The total amount of packed red cells and FFP units transfused was significantly lower in the treatment arm. Postoperative bleeding was significantly ( P =0.042) less in the treatment arm (median, 300 mL; interquartile range, 200 to 400 mL) than in the control arm (median, 355 mL; interquartile range, 250 to 600 mL). Conclusions Fibrinogen concentrate limits postoperative bleeding after complex heart surgery, leading to a significant reduction in allogeneic blood products transfusions. No safety issues were raised. Clinical Trial Registration URL: http://www.clinicaltrials.gov . Unique identifier: NCT01471730.

Aims The aim of the article is to study the safety, efficacy, and follow-up of percutaneous closure of muscular ventricular septal defect (mVSD) and perimembranous ventricular septal defect (pmVSD) in children. Methods and results Between January 2000 and June 2005, among 140 patients who underwent percutaneous closure of an mVSD or a pmVSD, 88 were aged lt;12. Two different Amplatzer devices were used: the mVSD occluder and the pmVSD occluder. Mean age and weight at procedure were 4.5±3.3 years and 18.7±11.2 kg, respectively. Percutaneous closure was successfully achieved in 83 subjects (94%). No deaths occurred. Thirteen patients (14.7%) had early complications: device embolization ( n =4), vascular complications ( n =3), and rhythm abnormalities ( n =6). These were transient complications in all but one case [1.1% complete atrioventricular block (cAVB) needing pacemaker implantation]. During a median follow-up of 24 months, three subjects treated for a pmVSD needed pacemaker implantation due to the occurrence of cAVB. Multivariable analysis using Cox's proportional hazard regression showed that age was the only risk factor associated with the occurrence of cAVB ( P =0.028; relative risk: 0.25). Conclusion Percutaneous closure of mVSD and pmVSD in children can be performed safely and successfully. The occurrence of cAVB is a major concern in young children with pmVSD.

Objective: Pulmonary regurgitation may cause progressiveright ventricular dilatation and dysfunction in adult patients previously repaired for right ventricular outflow tract obstruction (RVOTO), and who require subsequent valve implantation for relief of these symptoms. Right ventricular recovery after pulmonary valve implantation (PVI) may be closely linked to the functional importance of the structural presence of an aneurysm or akinetic segment in the RVOT area. To test this concept, the impact of the right ventricular restoration with a new surgical ventriculoplasty technique is evaluated following pulmonary valve implantation in patients with severe pulmonary regurgitation and right ventricular dilatation. Methods: Sixteen patients with severe pulmonary valve regurgitation (PVR) and right ventricular dilatation with RVOT aneurysm underwent right ventricular remodelling since January 2002. Each underwent preoperative evaluation by Doppler echocardiography, magnetic resonance imaging (MRI), and right ventricular myocardial acceleration during isovolumic contraction (IVC). The surgical procedure included pulmonary valve implantation and RVOT restoration achieved by removal of the aneurysm tissue, coupled with a ventriculoplasty to reduce volume, accomplished by creating a satisfactory RVOT dimension by placing with 2-0 Gortex suture to allow acceptance of a 26 Hegar dilator to avoid restriction. Thirteen associate surgical procedures were added in nine patients. Results: All patients survived the operative procedure and underwent a 16-month follow-up interval. A reduction of cardio thoracic index and a clinical improvement occurred in each patient. Significant reduction of RVEDV and RVESV and increased right ventricular ejection fraction was observed, and IVC changed from 0.7 0.5 m/s 2 to 1.3 0.6 m/s 2 in the 13 patients that underwent MRI and IVC during the preoperative control interval and 6 months after the procedure. Conclusions: This preliminary database implies that the right ventricular restoration is a simple and effective procedure, and introduces a structural component that should be added during pulmonary valve implantation in patients with severe right ventricular dilatation and underlying aneurysm or akinesia of the right ventricular outflow tract.

Background Anterior myocardial infarction leads a sequence of structural changes that alter the size and the shape of the left ventricle. Efforts to assess shape have been made by global left ventricular (LV) chamber analysis (sphericity index, SI) but this analysis does not detect regional shape abnormalities like those at the apical level, which precede global ventricular dilatation. Objective The present study will introduce a new analysis of regional apical changes in 52 normal subjects and in 92 patients with previous anterior myocardial infarction. Methods All patients had transthoracic echocardiogram and multiple views were obtained (long axis, 4CH, 2CH and short axis view). From the 4CH view the long and the short axes were measured and their ratio was calculated (sphericity index). In the same view, the apical axis length was also measured and the ratio between apical and short axis length was calculated (apical conicity index, ACI). Results Patients had all the measured parameters significantly worse than normal, except the sphericity index which remained unchanged. Ventricular length and width increased following anterior MI but the ratio between the two measurements did not change. Conversely, apical conicity index is significantly different following anterior MI, thereby indicating anterior infarction produces a less conical shape. SI and ACI differed when correlations were made in the relationship of mitral valve function; SI correlates with the degree of mitral regurgitation (MR) and with the distance of papillary muscles, conversely ACI shows an inverse correlation with the determinants of mitral regurgitation. These observations reflect differences between apical versus global dilatation in ischemic cardiomyopathy, so that mitral function is better (lower tenting area and lower coaptation height) when the apex is markedly dilated in respect to the short axis (high conicity index). In contrast, mitral function is impaired (bigger distance between papillary muscles and higher degree of mitral regurgitation), when sphericity index is high. Conclusions Sphericity index fails to detect regional apical shape abnormalities. To address this focal change, we introduce a simple new measure termed apical conicity index, which is abnormal in patients with myocardial infarction, and can be useful to evaluate changes induced by the subsequent surgical approach of ventricular re-shaping.

During cardiac operations with cardiopulmonary bypass surgery, antithrombin is consumed and low levels of antithrombin activity are commonly observed at admission to the intensive care unit (ICU). This study investigates the association between antithrombin activity at admission to the ICU (ICU-antithrombin activity) and various outcome variables.The authors conducted a prospective, observational cohort study.The study was conducted at a university hospital.The study consisted of 647 consecutive patients who had undergone cardiac surgery with cardiopulmonary bypass.ICU-antithrombin activity significantly (p.001) decreased with respect to preoperative values. As seen with univariate analysis, low levels of ICU-antithrombin activity were significantly associated with higher blood loss, prolonged mechanical ventilation time and ICU stay, a higher incidence of allogeneic blood products use, surgical reexploration, low cardiac output syndrome, adverse neurologic events, thromboembolic events, renal dysfunction, and hospital mortality. When corrected for the other explanatory variables, low levels of ICU-antithrombin activity remained independently associated with a prolonged ICU stay (p = .003) and with a higher incidence of surgical reexploration (p = .023), adverse neurologic events (p = .001), and thromboembolic events (p = .036). An ICU-antithrombin activity value of58% was found to be predictive of prolonged ICU stay, with a sensitivity of 67% and a specificity of 83%.Low levels of ICU-antithrombin activity are associated with a poor outcome in cardiac surgery; ICU-antithrombin activity is predictive of prolonged ICU stay.

N-terminal pro-brain natriuretic peptide (NT-pro-BNP) levels have been shown to be increased at baseline in patients undergoing surgical ventricular restoration (SVR) of the left ventricle. However, changes in the values of this marker in the early postoperative period and its prognostic significance remain less known in these patients. We evaluated 31 consecutive patients undergoing SVR who had NT-pro-BNP determined a day before SVR and from postoperative days 0 to 4. Major morbidity was defined asor =1 of the following: ventilation48 hours, stroke, acute renal failure, low cardiac output, reoperation, or mediastinitis. The association of preoperative NT-pro-BNP with perioperative outcomes was assessed using multivariable logistic regression analysis. Receiver operating characteristic curve was used to test its discrimination power. Major morbidity occurred in 16 patients (52%) with only 1 death within 30 days of SVR. Mean preoperative NT-pro-BNP was 4.5-fold higher in patients with postoperative major morbidity than in those without it (3,022 +/- 2,981 vs 676 +/- 533 pg/ml, p = 0.007). On multivariate analysis, preoperative NT-pro-BNP was independently associated with major morbidity after adjusting for baseline confounding, particularly age, ejection fraction, and European System for Cardiac Operative Risk Evaluation (odds ratio 1.002, 95% confidence interval 1.001 to 1.003, p = 0.032). Preoperative NT-pro-BNP had a high discrimination power on receiver operating characteristic analysis for major morbidity (area under the curve 0.84, sensitivity 68%, and specificity 88% for 1,304 pg/ml). Although NT-pro-BNP levels decreased after SVR in patients without major morbidity, their levels remained persistently increased in those with it. In conclusion, preoperative NT-pro-BNP determination may be of value in stratifying the risk for major morbidity after SVR.