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Background: Balloon valvuloplasty is the primary treatment for congenital aortic valve stenosis in our centre. We sought to determine independent predictors of reintervention (surgical repair or repeated balloon dilation) after primary valvuloplasty. Methods: We retrospectively studied patients with congenital aortic valve stenosis who underwent balloon valvuloplasty during 2004–2018. The following risk factors were analysed: aortic valve insufficiency after balloon valvuloplasty >+1/4, post-procedural gradient across the aortic valve ≥35 mmHg, pre-interventional gradient across the valve, annulus size, use of rapid pacing, and balloon/annulus ratio. Primary outcome was aortic valve reintervention. Results: In total, 99 patients (median age 4 years, range 1 day to 26 years) underwent balloon valvuloplasty for congenital aortic valve stenosis. After a mean follow-up of 4.0 years, 30% had reintervention. Adjusted risks for reintervention were significantly increased in patients with post-procedural aortic insufficiency grade >+1/4 and/or residual gradient ≥35 mmHg (HR 2.55, 95% CI 1.13–5.75, p = 0.024). Pre-interventional gradient, annulus size, rapid pacing, and balloon/annulus ratio were not associated with outcome. Conclusion: Post-procedural aortic valve insufficiency grade >+1/4 and/or residual gradient ≥35 mmHg in patients undergoing balloon valvuloplasty for congenital aortic valve stenosis confers an increased risk for reintervention in mid-term follow-up.

The majority of patients with congenital heart disease (CHD) who have undergone open heart surgery during childhood are possible candidates for additional transcatheter or surgical interventions. One fifth of these conditions usually involve the right ventricular outflow tract (RVOT). Percutaneous pulmonary valve replacement (PPVR) has been widely established as an alternative, less invasive option to surgical pulmonary valve replacement (SPVR). The variability of RVOT anatomy and size, the relative course of the coronary arteries, and the anatomy of the pulmonary artery branches are factors that determine the success of the intervention and the complication rates. Careful and reliable pre-interventional imaging warrants the selection of suitable candidates and minimizes the risk of complications. 2D and 3D fluoroscopy have been extensively used during pre- and peri-interventional assessment. Established imaging techniques such as cardiovascular magnetic resonance (CMR) and computed tomography (CT) and newer techniques such as fusion imaging have proved to be efficient and reliable tools during pre-procedural planning in patients assessed for PPVR.

BACKGROUND: Cardiac surgery is a technically demanding field with an appreciable learning curve that extends beyond formal training. Minimally invasive congenital cardiac surgery has one of the steepest learning curves. Early complications often discourage surgeons, particularly those at lower volume centers, from pursuing innovative approaches. Over the past three years, we have utilized a number of minimally invasive approaches including pulmonary valve replacement, anomalous aortic origin coronary artery repair, atrial septal defect repair, epicardial lead placement, and partial anomalous pulmonary venous return. Herein we report on our experience performing minimally invasive congenital cardiac surgery, lessons learned, and how our approach has evolved. METHODS: We performed a single institution, retrospective review, wherein continuous variables were reported as median (interquartile range). RESULTS: Between September 2017 and May 2020, minimally invasive approaches were attempted on 49 patients with a median age of 19 years (14–47 years) for nine distinct congenital cardiac diagnoses. Seven patients (14%) required conversion to larger incisions, including four patients or 36% of those undergoing anomalous aortic origin of a coronary artery repair. Patients who were converted had a higher body mass index 33.1 (31.7–37.8) than those who were not (24.2, 20.8–29.3) (P=0.009). CONCLUSIONS: Minimally invasive approaches for congenital cardiac conditions require a team approach. Patients with a body mass index greater than 30 should be counseled on the higher rate of conversion. We no longer perform minimally invasive anomalous aortic origin of a coronary artery repair given the high rate of conversions and complications. Surgeons attempting this procedure should do so cautiously.

Objective:Rapid right ventricular pacing during balloon aortic valvuloplasty is commonly used to achieve balloon stability in children and adults. There is no consensus for the use of the technique in neonates and infants. We sought to review our institutional experience with rapid right ventricular pacing-assisted balloon aortic valvuloplasty across all age groups and evaluate the safety and effectiveness of the technique in the sub-group of neonates and infants Methods:Retrospective study between February, 2011 and February, 2020.Results:A total of 37 patients (Group I: 21 neonates/infants Conclusions:Rapid right ventricular pacing can be expanded in neonates and infants to potentially decrease the incidence of aortic regurgitation and reintervention rates, hence avoiding high-risk surgical bail-out procedures for severe aortic regurgitation in the first year of life. Myocardial ischemia may predispose to ventricular dysrhythmias during rapid right ventricular pacing.

The vast majority of patients with congenital heart disease (CHD) survive into adulthood, but many face lifelong complications, which often result in a hospital admission. The increasing number of hospitalizations in adults with CHD (ACHD) poses a significant challenge for healthcare systems globally, especially as heart failure (HF) is becoming increasingly common in this population and is the leading cause of morbidity and mortality. Besides HF, other major contributors to this increase in admission volume are hospitalizations related to mild lesions, comorbidities and pregnancies. Ιn-hospital mortality ranges between 0.8 and 6.1%, while hospitalizations related to HF predict medium-term mortality in ACHD population. Understanding the predictors of hospitalization and in-hospital mortality is, therefore, important for ACHD healthcare providers, who should identify patients at risk that require escalation of treatment and/or close monitoring. This article reviews the available literature on hospitalization patterns in ACHD patients, with a focus on HF-related hospital admissions and specific diagnostic subgroups.

Purpose of review Aberrant subclavian artery (ASCA) represents one of the most common congenital vascular anomalies of the aortic arch. The majority of ASCAs are associated with an aneurysm which occurs at their origin from the descending thoracic aorta, namely, the Kommerell's diverticulum. In this review, we discuss recent data with regards to indications of treatment and surgical management of these anatomical variants. Recent findings Various surgical methods have been described for the repair of ASCA and Kommerell's diverticulum. Traditionally, treatment included open surgery; however, recent studies describe a shift of conventional treatment to more hybrid or endovascular approaches. The heterogeneity in the anatomy and presentation of these clinical entities as well as patient-related factors have prevented conduction of randomized trials for the best available modality of treatment. This dearth of literature is well depicted in the current guidelines. Summary The optimal surgical procedure has to be tailored to every patient according to the presentation, individual anatomy, and patient's risk profile. Conventional surgery for ASCA and Kommerell's diverticulum has acceptable mortality and complication rates, whereas hybrid repairs report encouraging results. Further studies are required to provide sufficient evidence which will formulate a clear frame of treatment indications and optimal surgical methods, as well as evaluate long-term results following hybrid repair.

Device closure is the first-line treatment for most atrial septal defects (ASDs). Minimally invasive cardiac surgery (MICS) has been found safe and effective for ASD closure with comparable mortality/morbidity and superior cosmetic results compared to conventional median sternotomy. Our goal was to compare percutaneous versus MICS of ASDs. A systematic review was performed using PubMed and the Cochrane Library (end-of-search date on May 22, 2019). Meta-analyses were conducted using fixed and random effects models. In the present systematic review, we analyzed six studies including 1577 patients with ASDs who underwent either MICS (n = 642) or device closure (n = 935). Treatment efficacy was significantly higher in the MICS (99.8%; 95% CI 98.9-99.9) compared to the device closure group (97.3%; 95% CI 95.6-98.2), (OR 0.1; 95% CI 0.02-0.6). Surgical patients experienced significantly more complications (16.2%; 95% CI 13.0-19.9) compared to those that were treated with a percutaneous approach (7.1%; 95% CI 5.0-9.8), (OR 2.0; 95% CI 1.2-3.2). Surgery was associated with significantly longer length of hospital stay (5.6 ± 1.7 days) compared to device closure (1.3 ± 1.4 days), (OR 4.8; 95% CI 1.1-20.5). Residual shunts were more common with the transcatheter (3.9%; 95% CI 2.7-5.5) compared to the surgical approach (0.95%; 95% CI 0.3-2.4), (OR 0.1; 95% CI 0.06-0.5). There was no difference between the two techniques in terms of major bleeding, hematoma formation, transfusion requirements, cardiac tamponade, new-onset atrial fibrillation, permanent pacemaker placement, and reoperation rates. MICS for ASD is a safe procedure and compares favorably to transcatheter closure. Despite longer hospitalization requirements, the MICS approach is feasible irrespective of ASD anatomy and may lead to a more effective and durable repair.

Background Advances in three-dimensional reconstruction techniques and computational fluid dynamics of coronary CT angiography (CCTA) data sets make feasible evaluation of endothelial shear stress (ESS) in the vessel wall. Purpose To investigate the relationship between CCTA-derived computational fluid dynamics metrics, anatomic and morphologic characteristics of coronary lesions, and their comparative performance in predicting impaired coronary vasodilating capability assessed by using PET myocardial perfusion imaging (MPI). Materials and Methods In this retrospective study, conducted between October 2019 and September 2020, coronary vessels in patients with stable chest pain and with intermediate probability of coronary artery disease who underwent both CCTA and PET MPI with oxygen 15-labeled water or nitrogen 13 ammonia and quantification of myocardial blood flow were analyzed. CCTA images were used in assessing stenosis severity, lesion-specific total plaque volume (PV), noncalcified PV, calcified PV, and plaque phenotype. PET MPI was used in assessing significant coronary stenosis. The predictive performance of the CCTA-derived parameters was evaluated by using area under the receiver operating characteristic curve (AUC) analysis. Results There were 92 coronary vessels evaluated in 53 patients (mean age, 65 years ± 7; 31 men). ESS was higher in lesions with greater than 50% stenosis versus those without significant stenosis (mean, 15.1 Pa ± 30 vs 4.6 Pa ± 4 vs 3.3 Pa ± 3; P = .004). ESS was higher in functionally significant versus nonsignificant lesions (median, 7 Pa [interquartile range, 5-23 Pa] vs 2.6 Pa [interquartile range, 1.8-5 Pa], respectively; P ≤ .001). Adding ESS to stenosis severity improved prediction (change in AUC, 0.10; 95% CI: 0.04, 0.17; P = .002) for functionally significant lesions. Conclusion The combination of endothelial shear stress with coronary CT angiography (CCTA) stenosis severity improved prediction of an abnormal PET myocardial perfusion imaging result versus CCTA stenosis severity alone. © RSNA, 2021 Online supplemental material is available for this article. See also the editorial by Kusmirek and Wieben in this issue.

Ventricular septal defects (VSDs) are the most common type of congenital heart disease. Transcatheter device closure of a VSD is an established method of treatment with excellent reported results. ...

In most recent decades, the area of pediatric and congenital cardiac catheterization has evolved into a predominantly interventional discipline, with diagnostic cardiac catheterizations being performed more seldomly. This is due to the vast evolution of noninvasive cross-sectional imaging techniques such as intracardiac echocardiography (ICE), 2D and three-dimensional (3D) transesophageal echocardiography (TEE), cardiac computed tomography (CT) and magnetic resonance imaging (MRI), rotational angiography with 3D roadmap, holography and 3D printing, which have largely replaced diagnostic cardiac catheterization procedures.

The perception that women represent a low-risk population for cardiovascular (CV) disease (CVD) needs to be reconsidered. Starting from risk factors, women are more likely to be susceptible to unhealthy behaviors and risk factors that have different impact on CV morbidity and mortality as compared to men. Despite the large body of evidence as regards the effect of lifestyle factors on the CVD onset, the gender-specific effect of traditional and non-traditional risk factors on the prognosis of patients with already established CVD has not been well investigated and understood. Furthermore, CVD in women is often misdiagnosed, underestimated, and undertreated. Women also experience hormonal changes from adolescence till elder life that affect CV physiology. Unfortunately, in most of the clinical trials women are underrepresented, leading to the limited knowledge of CV and systemic impact effects of several treatment modalities on women's health. Thus, in this consensus, a group of female cardiologists from the Hellenic Society of Cardiology presents the special features of CVD in women: the different needs in primary and secondary prevention, as well as therapeutic strategies that may be implemented in daily clinical practice to eliminate underestimation and undertreatment of CVD in the female population.

Anomalous aortic origin of a coronary artery is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. The surgical treatment of this anomaly is highly variable and controversial and is achieved by implementing elaborate techniques of anatomic repair or by simple coronary artery bypass grafting. This review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Two independent reviewers determined whether studies met the inclusion criteria. Eligible papers were published in English, clinical studies describing surgical repair of anomalous aortic origin of a coronary artery including coronary artery bypass in adults, and enrolled >5 patients. In the absence of multicentre trials, 7 single-center retrospective series were included, which demonstrated comparable short- and midterm outcomes of anatomic repair and coronary artery bypass in adults with anomalous aortic origin of a coronary artery. Coronary unroofing was the preferred technique when the anomalous artery has an intramural component, but there is a risk of aortic insufficiency. Anatomic repair is technically demanding and should be carried out in experienced centers. Coronary artery bypass with internal thoracic artery or vein grafts is technically straightforward with low operative risk and comparable midterm outcomes, however, long-term outcomes are unknown. Coronary artery bypass grafting is the technique of choice for older patients, in those with concomitant coronary artery disease, as a bailout procedure for failed anatomic repair, or in centers without experience in anatomic repair for anomalous aortic origin of a coronary artery.

Background: Type 2 diabetes mellitus (DM) is associated with a considerable risk of cardiovascular and renal diseases, including heart failure. Sodium-glucose co-transporter 2 (SGLT2) inhibitors have demonstrated unprecedented cardiorenal protective effects in large-scale clinical trials of patients with or without diabetes and either established cardiovascular disease (CV) or multiple CV risk factors. Objective: Herein we aim to focus on the role of SGLT2 inhibitors regarding the improvement in heart failure outcomes and the proposed mechanisms of action by which these drugs confer their beneficial effect. Methods: PubMed, Embase, and Google Scholar databases were searched to identify eligible articles that are comprehensively summarized and discussed in this study. Results: The most commonly discussed mechanisms of action are diuresis and natriuresis, reduction in preload, afterload, and ventricular mass, as well as stimulation of erythropoietin production and improved myocardial energetics. SGLT2 inhibitors improve outcomes in patients with established heart failure (HF) and reduce the risk of death and HF admissions in patients with established chronic HF with reduced ejection fraction (HFrEF), either with or without diabetes. Conclusion: Potential key mechanisms that may explain the notable cardioprotective benefits of SGLT2 inhibitors have been outlined. These agents have recently received class Ia recommendation in specific groups of people with DM to lower the risk of hospitalization for HF and risk of death, while these benefits may also extend to people without diabetes. It remains to be seen whether they will also emerge as treatment approaches in the acute phase of CV episodes.

BACKGROUND Balloon aortic valvuloplasty (BAV) is a well-established treatment modality for congenital aortic valve stenosis. AIM To evaluate the role of rapid right ventricular pacing (RRVP) in balloon stabilization during BAV on aortic regurgitation (AR) in pediatric patients. METHODS A systematic review of the MEDLINE, Cochrane Library, and Scopus databases was conducted according to the PRISMA guidelines (end-of-search date: July 8, 2020). The National Heart, Lung, and Blood Institute and Newcastle-Ottawa scales was utilized for quality assessment. RESULTS Five studies reporting on 72 patients were included. The studies investigated the use of RRVP-assisted BAV in infants (> 1 mo) and older children, but not in neonates. Ten (13.9%) patients had a history of some type of aortic valve surgical or catheterization procedure. Before BAV, 58 (84.0%), 7 (10.1%), 4 (5.9%) patients had AR grade 0 (none), 1 (trivial), 2 (mild), respectively. After BAV, 34 (49.3%), 6 (8.7%), 26 (37.7%), 3 (4.3%), patients had AR grade 0, 1, 2, and 3 (moderate), respectively. No patient developed severe AR after RRVP. One (1.4%) developed ventricular fibrillation and was defibrillated successfully. No additional arrhythmias or complications occurred during RRVP. CONCLUSION RRVP can be safely used to achieve balloon stability during pediatric BAV, which could potentially decrease AR rates.

Bradyarrhythmias are a common complication following pediatric OHT and may require permanent pacemaker implantation (PPM). The purpose of this study was to investigate the incidence, predictors, and outcomes of children undergoing PPM implantation following OHT. A PRISMA-compliant systematic literature review was performed using the PubMed database and the Cochrane Library (end-of-search date: January 27, 2019). The Newcastle-Ottawa scale and the Joanna Briggs Institute tool were used to assess the quality of cohort studies and case reports, respectively. We analyzed data from a total of 11 studies recruiting 7198 pediatric patients who underwent heart transplant. PPM implantation was performed in 1.9% (n = 137/7,198; 95% CI: 1.6-2.2) of the patients. Most patients underwent dual-chamber pacing (46%, 95% CI: 32.6-59.7). Male-to-female ratio was 1.3:1. Mean patient age at the time of OHT was 10.1 ± 6.3. Overall, biatrial anastomosis was used in 62.2% (95% CI: 52.8-70.6) of the patients. The bicaval technique was performed in the remaining 37.8% (95% CI: 29.4-47.1). Sinus node dysfunction was the most frequent indication for PPM implantation (54.4%; 95% CI: 42.6-65.7) followed by AV block (45.6%; 95% CI: 34.3-57.3). The median time interval between OHT and PPM implantation ranged from 17 days to 12.5 years. All-cause mortality was 27.9% (95% CI: 18.6-39.6) during a median follow-up of 5 years. PPM implantation is rarely required after pediatric OHT. The most common indication for pacing is sinus node dysfunction, and patients undergoing biatrial anastomosis may be more likely to require PPM.

Patients with Williams syndrome often present with abnormalities of the vascular wall of the aorta and/or the pulmonary artery. Surgery may result in restenosis of the affected vessel. Herein, we report a case of an infant with multiple recurrences of aortic coarctation successfully treated with Zotarolimus drug-eluting stent.

ObjectiveDespite the progress in the management of patients with adult congenital heart disease (ACHD), a significant proportion of patients still develop pulmonary hypertension (PH). We aimed to highlight the rate of the complications in PH-ACHD and the predicting factors of cumulative mortality risk in this population.MethodsData were obtained from the cohort of the national registry of ACHD in Greece from February 2012 until January 2018.ResultsOverall, 65 patients receiving PH-specific therapy were included (mean age 46.1±14.4 years, 64.6% females). Heavily symptomatic (New York Heart Association (NYHA) class III/IV) were 53.8% of patients. The majority received monotherapy, while combination therapy was administered in 41.5% of patients. Cardiac arrhythmia was reported in 30.8%, endocarditis in 1.5%, stroke in 4.6%, pulmonary arterial thrombosis in 6.2%, haemoptysis in 3.1% and hospitalisation due to heart failure (HF) in 23.1%. Over a median follow-up of 3 years (range 1–6), 12 (18.5%) patients died. On univariate Cox regression analysis history of HF hospitalisation emerged as a strong predictor of mortality (HR 8.91, 95% CI 2.64 to 30.02, pConclusionsLong-term complications are common among patients with PH-ACHD. Hospitalisations for HF predict mortality and should be considered in the risk stratification of this population.

We present the case of a 3-year-old boy with bicuspid aortic valve, aortic coarctation, and left ventricular non-compaction. The diagnosis was made post-natally with ultrasonography and was verified by cardiac MRI. Aortic coarctation was initially repaired surgically. At age 3 months, recoarctation and heart failure developed. Balloon angioplasty was performed with immediate improvement. At age 3 years, the patient remains asymptomatic and normotensive.

BackgroundCardiac haemangiomas are extremely rare tumours with equivocal surgical outcomes. Haemangiomas appearing on other sites of the body have been successfully treated with oral propranolol. To the best of our knowledge, such treatment has not been tried to date for cardiac location of haemangiomas.Case summaryWe report two cases of neonatal cardiac haemangiomas, and we describe their presentation and characteristics, as well as how these were successfully treated with oral propranolol, with complete regression of the tumours within the 1st year of life.DiscussionDespite the rarity of cardiac haemangiomas, their presentation and complications could be dramatic with side-effects spanning from intracardiac space occupying phenomena to Kasabach–Merritt syndrome. Propranolol therapy, having been established for long now in the treatment of skin haemangiomas, should also be considered in cases of cardiac haemangiomas, particularly in the neonatal and infantile population.

We describe a case of right coronary artery occlusion in a neonate with critical aortic stenosis. We hypothesize that the thrombus was either formed in situ resulting from low cardiac output or secondary to an atrial arrhythmia in the presence of severe left ventricular diastolic dysfunction that may have occurred during air-transfer to our facility. We conclude that attention should be given to the coronary arteries in low-flow critical aortic stenosis, particularly in neonates and infants whose stroke volume is extremely low.

While virus epidemics are nothing new to man, the scale, speed of global spread and immediacy of the COVID-19 pandemic have been truly unprecedented [ 1 ]. The entire world has been turned on its head in less than a few months, with major implications beyond disease burden and loss of life, threatening the economic status quo and human psychosocial balance and wellbeing not only for patients, but for all of us. The primary aim of our Call for Action Viewpoint was to support and protect our adult congenital heart disease (ACHD) patients and their needs during these challenging and uncertain times. This goal had to be met while we, as individuals, teams, institutions and nations, came together in a global effort to combat this aggressive virus, that appears to spare no organs or systems, nor any borders, geographic or other. As with any crisis, there is always opportunity: we are submitting herewith a vision for a different and better model of ACHD care, and for a better life journey and health care experience for our patients, that should be in place in the aftermath of the Covid-19 pandemic. Many of the points and principles discussed in this article, need not be confined to ACHD patients, but have a broader reach. And we must not forget nor neglect the most vulnerable in society at this time, namely the elderly, disabled and other dependent or disadvantaged groups in this “We Are One” global operation. Last but not least, this maybe the time to take better care of ourselves (and others) and reflect on life.

Levoatriocardinal vein (LACV) is a rare congenital anomaly of the systemic veins. We describe the case of a 43-year-old patient who presented with a transient ischemic attack episode, desaturation, and ventricular arrhythmias. Transesophageal echocardiography diagnosed a patent foramen ovale, and magnetic resonance imaging revealed 2 small myocardial infarcts, despite normal coronaries on subsequent angiography. Echocardiography and cardiac computed tomography revealed an LACV with bidirectional flow. The patient underwent percutaneous LACV and patent foramen ovale closure, with immediate saturation increase after occlusion of the LACV. To the best of our knowledge, this is the first time an LACV is identified as the cause of recurrent thromboembolic events.

Transcatheter aortic and pulmonary valves have been used to treat stenosis or regurgitation after prior surgical tricuspid valve (TV) replacement or repair. Little is known about intermediate-term valve-related outcomes after transcatheter tricuspid valve replacement (TTVR), including valve function, thrombus, and endocarditis.The authors sought to evaluate mid-term outcomes in a large cohort of patients who underwent TTVR after surgical TV repair or replacement, with a focus on valve-related outcomes.Patients who underwent TTVR after prior surgical TV replacement or repair were collected through an international registry. Time-related outcomes were modeled and risk factors assessed.Data were collected for 306 patients who underwent TTVR from 2008 through 2017 at 80 centers; 52 patients (17%) had a prior history of endocarditis. Patients were followed for a median of 15.9 months after implantation (0.1 to 90 months), with 64% of patients estimated to be alive without TV reintervention or a valve-related event at 3 years. The cumulative 3-year incidence of death, reintervention, and valve-related adverse outcomes (endocarditis, thrombosis, or significant dysfunction) were 17%, 12%, and 8%, respectively. Endocarditis was diagnosed in 8 patients 2 to 29 months after TTVR, for an annualized incidence rate of 1.5% per patient-year (95% confidence interval: 0.45% to 2.5%). An additional 8 patients were diagnosed with clinically relevant valve thrombosis, 3 in the short term, 2 within 2 months, and 3 beyond 6 months. Only 2 of these 8 patients received anticoagulant therapy before thrombus detection (p = 0.13 vs. patients without thrombus). Prior endocarditis was not a risk factor for reintervention, endocarditis, or valve thrombosis, and there was no difference in valve-related outcomes according to TTVR valve type.TV dysfunction, endocarditis, and leaflet thrombosis were uncommon after TTVR. Patients with prior endocarditis were not at higher risk for endocarditis or other adverse outcomes after TTVR, and endocarditis occurred with similar frequency in different valve types. Though rare, leaflet thrombosis is an important adverse outcome, and further study is necessary to determine the appropriate level of prophylactic therapy after TTVR.

A male term patient was delivered electively due to prenatal diagnosis of d-transposition of great arteries. Postpartum arterial saturation measured 65%. Transthoracic echo revealed an extremely aneurysmal atrial septum with no obvious atrial septal defect. Emergency Rashkind procedure was deemed necessary. Multiple attempts to advance the septostomy catheter to the left atrium resulted in tenting of the septum with no obvious interatrial flow. The aneurysmal septum was therefore advanced with caution and perforated within the left lower pulmonary vein under echocardiographic guidance. The patient tolerated the procedure well and a successful arterial switch operation was performed eight days later. Stabilization and perforation of an aneurysmal, intact atrial septum within the pulmonary vein may be a helpful maneuver to complete the Rashkind procedure.

Aorto-ventricular tunnel is a rare congenital malformation, and aorto-right ventricular tunnel (ARVT) is an even less common entity. Here, we report the case of a 3-month-old female who underwent successful surgical closure of ARVT. The origin of the right coronary artery was proximal to the ostium of the tunnel.

We sought to describe the acute results and short- to medium-term durability of transcatheter tricuspid valve-in-valve (TVIV) implantation within surgical bioprostheses among patients with Ebstein anomaly (EA). Cases were identified from a voluntary, multicenter, international registry of 29 institutions that perform TVIV. Demographic, clinical, procedural, and follow-up data were analyzed. Eighty-one patients with EA underwent TVIV from 2008 to 2016. Thirty-four patients (42%) were New York Heart Association (NYHA) class 3/4 at time of TVIV. The most common indication for TVIV was the presence of moderate or severe tricuspid regurgitation (40%). Most patients received a Melody valve (64%). TVIV was ultimately successful in all patients, and there was no procedural mortality. Four patients (5%) developed acute valve thrombosis, 4 patients (5%) developed endocarditis, and 9 patients (11%) developed valve dysfunction not related to thrombosis or endocarditis. Eight patients (10%) underwent reintervention (2 transcatheter, 6 surgical) due to thrombosis (3), endocarditis (2), other valve dysfunction (2), and patient-prosthesis mismatch without valve dysfunction (1). Among 69 patients who were alive without reintervention at latest follow-up, 96% of those with NYHA status reported were class 1/2, a significant improvement from baseline (62% NYHA class 1/2, p

Background The majority of patients with congenital heart disease (CHD), nowadays, survives into adulthood and is faced with long-term complications. We aimed to study the basic demographic and clinical characteristics of adult patients with congenital heart disease (ACHD) in Greece. Methods A registry named CHALLENGE (Adult Congenital Heart Disease Registry. A registry from Hellenic Cardiology Society) was initiated in January 2012. Patients with structural CHD older than 16years old were enrolled by 16 specialized centers nationwide. Results Out of a population of 2115 patients with ACHD, who have been registered, (mean age 38years (SD 16), 52% women), 47% were classified as suffering from mild, 37% from moderate and 15% from severe ACHD. Atrial septal defect (ASD) was the most prevalent diagnosis (33%). The vast majority of ACHD patients (92%) was asymptomatic or mildly symptomatic (NYHA class I/II). The most symptomatic patients were suffering from an ASD, most often the elderly or those under targeted therapy for pulmonary arterial hypertension. Elderly patients (>60years old) accounted for 12% of the ACHD population. Half of patients had undergone at least one open-heart surgery, while 39% were under cardiac medications (15% under antiarrhythmic drugs, 16% under anticoagulants, 16% under medications for heart failure and 4% under targeted therapy for pulmonary arterial hypertension). Conclusions ACHD patients are an emerging patient population and national prospective registries such as CHALLENGE are of unique importance in order to identify the ongoing needs of these patients and match them with the appropriate resource allocation.

MRI- or combined x-ray and MRI (XMR)-guided catheterization was introduced as an alternative to x-ray-guided catheterization to reduce radiation exposure and offer more comprehensive anatomical, hemodynamic and physiological data. However, developments have been slow to come into routine clinical practice. We report a 10-year experience of solely MRI-guided and XMR catheterization in patients at our institution, review the developments in clinical MRI-guided and XMR catheterization and discuss future perspectives. This includes further results from our clinical trial on MRI-guided cardiac interventions.

Iron deficiency is common in cyanotic congenital heart disease (CHD) and results in reduced exercise tolerance. Currently, iron replacement is advocated with limited evidence in cyanotic CHD. We investigated the safety and efficacy of iron replacement therapy in this population.Twenty-five iron-deficient cyanotic CHD patients were prospectively studied between August 2008 and January 2009. Oral ferrous fumarate was titrated to a maximum dose of 200mg thrice-daily. The CAMPHOR QoL questionnaire, 6 minute walk test (6MWT) and cardiopulmonary exercise testing were conducted at baseline and after 3 months of treatment.Mean age was 39.9 ± 10.9 years, 80% females. Fourteen had Eisenmenger syndrome, 6 complex cyanotic disease and 5 Fontan circulation. There were no adverse effects necessitating termination of treatment. After 3 months of treatment, hemoglobin (19.0 ± 2.9 g/dL to 20.4 ± 2.7 g/dL, p0.001), ferritin (13.3 ± 4.7 μg/L to 54.1 ± 24.2 μg/L, p0.001) and transferrin saturation (17.8 ± 9.6% to 34.8 ± 23.4%, p0.001) significantly increased. Significant improvements were also detected in the total CAMPHOR score (20.7 ± 10.9 to 16.2 ± 10.4, p=0.001) and 6MWT distance (371.7 ± 84.7 m to 402.8.0±74.9m, p=0.001). Peak VO(2) remained unchanged (40.7 ± 9.2% to 43.8 ± 12.4% of predicted, p=0.15).Three months of iron replacement therapy in iron-deficient cyanotic CHD patients was safe and resulted in significant improvement in exercise tolerance and quality of life. Identification of iron deficiency and appropriate replacement should be advocated in these patients.

To describe and assess the use of the maximum colour flow diameter (CFDmax) in guiding transcatheter closure of atrial septal defects (ASDs) and compare it with the standard balloon sizing method.Balloon sizing of ASDs has been the standard method of assessing the size of ASDs and selecting a closure device. However, overestimation of the defect by stretching it may result in complications such as erosion of the atrial wall or aortic root, particularly in the presence of a deficient aortic rim and the long-term effects of oversized defects are unknown.Retrospective and prospective assessment of 115 consecutive patients who underwent ASD closure during three different time periods, when change in local practice occurred. Fifty patients underwent ASD closure with balloon sizing (period A), 15 with the aid of balloon sizing and additional estimation of the CFDmax (period B) and 50 with sole assessment of the CFDmax (period C).The mean difference between the device used and the 2D defect size decreased from 6.9 to 5.1mm (p0.01) and the percentage of device oversizing decreased from 38% to 31%. The complication rate decreased from 14% to 2%, (p=0.03), whilst the procedure and screening times decreased from 70 to 43min, (p0.001) and 14.2 to 8.4min, (p0.001), respectively.Transcatheter closure of ASDs guided solely by the CFDmax is feasible and safe and results in decreased procedure and screening times, whilst maintaining a low complication rate.

In neonates and infants with a shunt-dependent pulmonary circulation, stenosis of the shunt needs to be treated aggressively to prevent a fatal outcome. We report the technique and outcome in seven consecutive neonates and infants with Blalock–Taussig shunt stenosis, who were treated with intraluminal stents. Stenting of classical or modified Blalock–Taussig shunts is technically feasible and can be used as a rescue procedure. Neointimal dissection is less likely compared with balloon angioplasty of the shunt alone.

Owing to their noninvasive nature, coronary artery stents have become popular demand for patients undergoing percutaneous coronary intervention. Late restenosis, in-stent restenosis, and late thrombosis, all mediated by the denuded endothelium, represent the most recurrent failures of vascular stent induction. Higher patency rates of stents can be achieved by restoring the native internal environment of the vessel-an endothelium monolayer. This active organ inhibits the inflammatory reaction to injury responsible for thrombus and intimal hyperplasia, thereby providing a novel therapeutic option to combat the unacceptably high prevalence of restenosis. As the climax of the nanotechnology era approaches, tissue engineering is being explored by means of exploiting the multipotent abilities of stem cells and their adherence to bioactive surface nanocomposite polymers. The endothelium can be reconstructed from neighboring intact endothelium and adherence of circulating endothelium progenitor cells. The latter takes place via a series of signaling events: mobilization, adhesion, chemoattraction, migration, proliferation, and finally their differentiation in mature endothelial cells. A nanotopography surface can orchestrate endothelium formation, attributable to cellular interactions promoted by its nanosize. This review encompasses the prospect of in situ endothelialization, the mechanisms regulating the process, and the advantages of using a new generation of bioactive nanocomposite materials for coating metal stent scaffolds.

Clinical practice is discrepant regarding routine enlargement of the interatrial communication (IAC) in patients with right-sided atrioventricular valve atresia. We determined the percentage and risk factors of those who develop a restrictive IAC.Medical records were reviewed for patients treated from 1985 to 2006, including those admitted in the first 6 weeks of life (group A), and those referred at a later age (group B), some of whom had routine atrial septal procedures. In group A, we analyzed the initial postnatal echocardiogram.Group A consisted of 79 patients, 16 of whom had an atrial septectomy as part of the initial surgical procedure. Of the remaining 63 patients, 9 (14%) developed atrial septal restriction. In group A, an atrial septal aneurysm (ASA) (OR 16, P = .006) and IAC diameter5 mm (OR 13, P = .009) were associated with atrial septal restriction. Atrial septal restriction occurred in 80% of patients with both features, 20% with IACor = 5 mm and ASA, 18% with IAC5 mm and no ASA, and 2% with neither feature. Group B consisted of 95 patients, 27 of whom had an atrial septal procedure. Of the remaining 68 patients, 11 (16%) developed atrial septal restriction.Routine enlargement of the IAC is not necessary in patients with right-sided atrioventricular valve atresia. Patients with ASA or IAC5 mm are at increased risk for development of atrial septal restriction.

Objectives The purpose of this research was to investigate the causes and symptoms of superior vena cava (SVC) obstruction or occlusion and report on the long-term results of transcatheter therapy. Background Information on transcatheter therapy for SVC obstruction is limited. Methods Superior vena cava catheterization interventions between August 1984 and April 2006 were reviewed. Patients were divided into 2 subgroups depending on whether or not they had previously undergone congenital cardiac surgery. Results Sixty-three patients with median age of 3.7 years (range 1 month to 42 years) and weight of 13.3 kg (range 3 to 114 kg) were treated. Fifty patients (79%) were symptomatic, although only 50% had symptoms suggestive of SVC obstruction. Superior vena cava syndrome was more common in the non-cardiac surgical group (52% vs. 10%, p = 0.001). The mean gradient and SVC diameter improved from 10.8 ± 5.8 mm Hg to 2.6 ± 2.2 mm Hg (p 5 years at the time of intervention. Conclusions Superior vena cava-related symptoms occur in only 50% of patients with hemodynamically significant SVC obstruction. Endovascular therapy is successful in relieving the stenosis and associated symptoms with good long-term results.

Aims Exercise limitation is common post-Fontan. Hybrid X-ray and magnetic resonance imaging (XMR) catheterization allows haemodynamic assessment by means of measurement of ventricular volumes and flow in major vessels with simultaneous invasive pressures. We aim to assess haemodynamic response to stress in patients with hypoplastic left heart syndrome (HLHS) post-Fontan. Methods and results Prospective study of 13 symptomatic children (NHYA 2) with HLHS post-Fontan using XMR catheterization. Three conditions were applied: baseline (Stage 1), dobutamine at 10 µg/kg/min (Stage 2), and dobutamine at 20 µg/kg/min (Stage 3). Seven consecutive patients received inhaled nitric oxide (iNO) at peak stress. Control MRI data were from normal healthy adults. In the HLHS patients, baseline mean pulmonary vascular resistance (PVR) was 1.51 ± 0.59 WU m2 and aortopulmonary collateral flow was 17.7 ± 13.6% of systemic cardiac output. Mean right ventricular end-diastolic pressure was 6.7 ± 2.5 mmHg which did not rise with stress. Cardiac index (CI) increased at Stage 2 in HLHS (40%) and controls (61%) but continued to increase at Stage 3 only in controls (19%) but not in HLHS. The blunted rise in CI in HLHS was due to a continuing fall in end-diastolic volume throughout stress, with no significant change in PVR or CI at peak stress in response to iNO. Conclusion Cardiac output post-Fontan in HLHS at peak stress is blunted due to a limitation in preload which is not responsive to inhaled pulmonary vasodilators in the setting of normal PVR.

Objectives This study sought to evaluate the use of covered Cheatham-platinum (CP) stents in the treatment of aortic coarctation (CoA). Background Aortic aneurysms and stent fractures have been encountered after surgical and transcatheter treatment for CoA. Covered stents have previously been used in the treatment of abdominal and thoracic aneurysms in adults. We implanted covered CP stents as a rescue treatment in patients with CoA aneurysms or previous stent-related complications and in patients at risk of developing complications because of complex CoA anatomy or advanced age. Methods Thirty-three covered CP stents were implanted in 30 patients; 16 patients had had previous procedures. The remaining patients had complex or near-atretic CoA. Results The mean patient age and weight were 28 (±17.5) years (range 8 to 65 years), and 62 (±13) kg (range 28 to 86 kg), respectively. The systolic gradient across the CoA decreased from a mean (±SD) of 36 ± 20 mm Hg before to a mean of 4 ± 4 mm Hg after the procedure (p Conclusions Covered CP stents may be used as the therapy of choice in patients with complications after CoA repairs, whereas they provide a safe alternative to conventional stenting in patients with severe and complex CoA lesions or advanced age.

Aims: Transcatheter implantation of valved stents (Melody and Edwards valves) for replacement of the pulmonary valve is currently an established procedure. We reviewed our experience on implantation of such valves in the tricuspid valve position. Methods and results: Transcatheter valve implantation in the tricuspid position was attempted in five patients. Four patients had predominantly tricuspid valve regurgitation, two of whom also had tricuspid valve stenosis. All patients had severely symptomatic right heart failure. Patient median age and weight were 12 years and 50 kg (range six-43 years and 13-68 kg, respectively). All patients had a bioprosthetic valve already in place. The mean gradient across the tricuspid valve decreased from 12 to 3 mmHg after the procedure. Median procedure time and fluoroscopy time were 100 and 39 min (range 60-180 and 30-57 min, respectively). The patients’ functional class improved from NYHA Class III to II in three and from Class III to I in two patients during a follow-up period of 15-22 months. Conclusions: Transcatheter replacement of malfunctioning bioprosthetic valves in the tricuspid position using valved stents is an attractive alternative to repeat surgery in high-risk or multi-operated patients. Longer follow-up and a larger number of patients are required to establish the long-term benefit of the procedure and freedom from reinterventions.

Atrial septal restriction is estimated to occur in up to 22% of patients with hypoplastic left heart syndrome and is associated with poor prognosis postnatally. Atrial septal aneurysms are known to be more prevalent in patients with congenital heart disease and atrial septal restriction due to elevated atrial pressures in fetal life. We report the case of a newborn with hypoplastic left heart syndrome, restrictive interatrial communication, and atrial septal aneurysm. The latter led to formation of a large intralimbic hematoma within it, which resulted in complete obstruction of the interatrial communication after birth, which led to death. We suggest that in addition to the known associated problems encountered in patients with hypoplastic left heart syndrome and restrictive interatrial communication, aneurysmal formation of the interatrial septum should alert the fetal and postnatal echocardiographer further, and careful assessment and follow-up is recommended.

Clinically significant mitral prosthetic paravalvular leaks may occur in up to 5% of patients following surgical mitral valve replacement. Successful closure may offer relief from symptoms of heart failure and hemolysis, but surgical closure is associated with increased morbidity and mortality. Alternative transcatheter closure techniques are being developed. We present a patient who was successfully treated via a transapical approach with deployment of an amplatzer duct occluder type I closure device under real-time two-dimensional transesophageal echocardiography.

Aortopulmonary window is a communication between the main pulmonary artery and the ascending aorta in the presence of two separate semilunar valves. The combination of an aortopulmonary window with interrupted aortic arch is rare. We discuss the unique case of an extremely premature infant weighing 1.7 kilograms who underwent cardiovascular magnetic resonance imaging as a pre-operative assessment in a high-field open 1.0 Tesla magnetic resonance imaging system as a one-stop investigation before complete repair.