Stent Implantation Across the Atrial Flow Regulator Device.

The Atrial Flow Regulator (AFR) is a self-expandable double-disc device with a central fenestration, intended to maintain a calibrated communication across the interatrial septum. We reported for the first time a stent implantation across an AFR device in an adolescent born with complex congenital heart disease with duct-dependent systemic circulation and severe combined pulmonary hypertension.

Coronary artery compression by pulmonary artery stenting after arterial switch operation: a novel indication for coronary compression test.

Pulmonary artery stenosis is one of the most common complications after arterial switch operation. Stent implantation is an effective treatment with some risks related to coronary artery compression. We present the case of a patient affected by pulmonary stenosis after arterial switch operation. Coronary compression testing demonstrated compression of the left coronary artery from the balloon inflated in the left pulmonary artery. We should systematically consider the use of coronary balloon testing before stent implantation in patients with a history of switch operation.

The use of a numerical model to simulate the cavo-pulmonary assistance in Fontan circulation: a preliminary verification.

The lack of an established experience on the use of VAD for the cavo-pulmonary assistance leads to the need of dedicated VADs development and animal experiments. A dedicated numerical model could support clinical and experimental strategies design and new VADs testing. The aim of this work is to perform a preliminary verification of a lumped parameter model of the cardiovascular system to simulate Fontan physiology and the effect of cavo-pulmonary assistance. Literature data of 4 pigs were used to simulate animals' baseline, and then the model was tested in simulating Fontan circulation and cavo-pulmonary-assisted condition comparing the simulation outcome (Sim) with measured literature data (Me). The results show that the numerical model can well reproduce experimental data in all three conditions (baseline, Fontan and assisted Fontan) [cardiac output (l/min): Me = 2.8 ± 1.7, Sim = 2.8 ± 1.8; ejection fraction (%): Me = 57 ± 17, Sim = 54 ± 17; arterial systemic pressure (mmHg): Me = 41.8 ± 18.6, Sim = 43.8 ± 18.1; pulmonary arterial pressure (mmHg): Me = 15.4 ± 8.9, Sim = 17.7 ± 9.9; caval pressure (mmHg): Me = 6.8 ± 4.1, Sim = 7 ± 4.6]. Systolic elastance, arterial systemic and arterial pulmonary resistances increase (10, 69, and 100 %) passing from the biventricular circulation to the Fontan physiology and then decrease (21, 39, and 50 %) once the VAD was implanted. The ventricular external work decreases (71 %) passing from the biventricular circulation to the Fontan physiology and it increases three times after the VAD implantation in parallel with the VAD power consumption. A numerical model could support clinicians in an innovative and challenging field as the use of VAD to assist the Fontan physiology and it could be helpful to personalize the VAD insertion on the base of ventricular systo-diastolic function, circulatory parameters and energetic variables.

The Impact of Specific Viruses on Clinical Outcome in Children Presenting with Acute Heart Failure.

The presence and type of viral genomes have been suggested as the main etiology for inflammatory dilated cardiomyopathy. Information on the clinical implication of this finding in a large population of children is lacking. We evaluated the prevalence, type, and clinical impact of specific viral genomes in endomyocardial biopsies (EMB) collected between 2001 and 2013 among 63 children admitted to our hospital for acute heart failure (median age 2.8 years). Viral genome was searched by polymerase chain reaction (PCR). Patients underwent a complete two-dimensional echocardiographic examination at hospital admission and at discharge and were followed-up for 10 years. Twenty-seven adverse events (7 deaths and 20 cardiac transplantations) occurred during the follow-up. Viral genome was amplified in 19/63 biopsies (35%); PVB19 was the most commonly isolated virus. Presence of specific viral genome was associated with a significant recovery in ejection fraction, compared to patients without viral evidence (p < 0.05). In Cox-regression analysis, higher survival rate was related to virus-positive biopsies (p < 0.05). When comparing long-term prognosis among different viral groups, a trend towards better prognosis was observed in the presence of isolated Parvovirus B19 (PVB19) (p = 0.07). In our series, presence of a virus-positive EMB (mainly PVB19) was associated with improvement over time in cardiac function and better long-term prognosis.

Simulation of Ventricular, Cavo-Pulmonary, and Biventricular Ventricular Assist Devices in Failing Fontan.

Considering the lack of donors, ventricular assist devices (VADs) could be an alternative to heart transplantation for failing Fontan patients, in spite of the lack of experience and the complex anatomy and physiopathology of these patients. Considering the high number of variables that play an important role such as type of Fontan failure, type of VAD connection, and setting (right VAD [RVAD], left VAD [LVAD], or biventricular VAD [BIVAD]), a numerical model could be useful to support clinical decisions. The aim of this article is to develop and test a lumped parameter model of the cardiovascular system simulating and comparing the VAD effects on failing Fontan. Hemodynamic and echocardiographic data of 10 Fontan patients were used to simulate the baseline patients' condition using a dedicated lumped parameter model. Starting from the simulated baseline and for each patient, a systolic dysfunction, a diastolic dysfunction, and an increment of the pulmonary vascular resistance were simulated. Then, for each patient and for each pathology, the RVAD, LVAD, and BIVAD implantations were simulated. The model can reproduce patients' baseline well. In the case of systolic dysfunction, the LVAD unloads the single ventricle and increases the cardiac output (CO) (35%) and the arterial systemic pressure (Pas) (25%). With RVAD, a decrement of inferior vena cava pressure (Pvci) (39%) was observed with 34% increment of CO, but an increment of the single ventricle external work (SVEW). With the BIVAD, an increment of Pas (29%) and CO (37%) was observed. In the case of diastolic dysfunction, the LVAD increases CO (42%) and the RVAD decreases the Pvci, while both increase the SVEW. In the case of pulmonary vascular resistance increment, the highest CO (50%) and Pas (28%) increment is obtained with an RVAD with the highest decrement of Pvci (53%) and an increment of the SVEW but with the lowest VAD power consumption. The use of numerical models could be helpful in this innovative field to evaluate the effect of VAD implantation on Fontan patients to support patient and VAD type selection personalizing the assistance.

Coronary plaque composition assessed by intravascular ultrasound virtual histology: association with long-term clinical outcomes after heart transplantation in young adult recipients.

OBJECTIVES: To assess coronary plaque composition by virtual histology intravascular ultrasound (VH-IVUS) analysis in young adult recipients and to correlate these findings with time from heart transplant (HTx) and long-term outcomes. BACKGROUND: Rapid progression of coronary allograft vasculopathy after heart transplantation is a powerful predictor of mortality and clinical events at long-term. METHODS: Forty consecutive young adult recipients transplanted during childhood undergoing VH-IVUS during coronary surveillance have been prospectively included in this study. According to the time interval from HTx to VH-IVUS assessment, our cohort was divided into two groups (group A: ≤5 years, n = 13; group B: >5 years, n = 27). RESULTS: Group B showed an higher percentage of necrotic core and dense calcium (12 ± 2 vs. 5 ± 1%, P = 0.04; 8.2 vs. 2.1%, P = 0.03; respectively). An "inflammatory plaque" (necrotic core and dense calcium ≥30%) was detected in 34.8% of patients in group B and in none among group A patients (P = 0.03). Patients in group B had a number of adverse clinical events significantly higher than group A patients (53.8 vs. 14.3%; HR 4.45; 95% CI 1.62-12.16; P = 0.029) at long-term follow-up (4.2 years). The multivariate regression analysis showed that age (HR 1.5; 95% CI 1.1-2.0; P = 0.007), time from HTx (HR 1.8; 95% CI 1.6-4.8; P = 0.02), and inflammatory plaque (HR 2.4; 95% CI 1.1-5.3; P = 0.03) were independent predictors of adverse clinical events. CONCLUSIONS: This study supports the hypothesis that time-dependent differences in plaque composition, as assessed by VH-IVUS, occur after HTx in young adult recipients, probably determining an increased risk of long-term clinical events.

Use of the CardioMEMS Device in Children and Patients with Congenital Heart Disease: A Literature Review.

The CardioMEMS HF System (Abbott, Abbott Park, IL) is the first FDA- and CE-Mark-approved device for monitoring patients with heart failure, significantly reducing hospitalizations and improving the quality of life for NYHA class III non-congenital adult patients. This device, implanted percutaneously, allows the direct monitoring of pulmonary arterial pressure with the wireless transfer of pressure data to the clinician, who can adjust the therapy remotely. Limited experience exists regarding its use in patients with congenital heart disease (CHD). CardioMEMS device implantation is feasible and safe in selected adults and children with CHD. The potential of the device to reduce heart failure hospitalizations in this population is enormous, but further multi-center studies are needed to demonstrate its efficacy.

BeGraft Aortic Stents: A European Multi-Centre Experience Reporting Acute Safety and Efficacy Outcomes for the Treatment of Vessel Stenosis in Congenital Heart Diseases.

BACKGROUND: Stent implantation has become the preferred method of treatment for treating vessel stenosis in congenital heart diseases. The availability of covered stents may decrease complications and have an important role in the management of patients with complex anatomy. AIM: This study aims to evaluate the feasibility and safety of the pre-mounted cobalt-chromium stent-graft-covered ePTFE Aortic BeGraft in a broad spectrum of vascular lesions. METHODS: This is a multicenter retrospective results analysis of 107 implanted BeGraft stents between 2016 and 2022 in six different European centers. RESULTS: One hundred and four patients with a mean age of thirteen years (range 1-70 years) and with the body weight of 56.5 kg (range 11-115 kg) underwent the BeGraft stent implantation. Stents were implanted in the following conditions: aortic coarctation (74 patients), RVOT dysfunction (12 patients), Fontan circulation (7 patients), and miscellaneous (11 subjects with complex CHD). All the stents were implanted successfully. The median stent diameter was 16 mm (range 7-24 mm), and the median length was 39 mm (range 19-49 mm). Major complications occurred in five subjects (4.7%). During a median follow-up of fourteen (1-70) months, stents' re-dilatation was performed in five patients. CONCLUSIONS: The BeGraft stent can be used safely and effectively in a wide spectrum of congenital heart diseases. Whether these good results will be stable in the longer term still needs to be investigated in a follow-up given its recent introduction into clinical practice, in particular regarding stent fracture or neointimal proliferation.

Percutaneous treatment of abdominal coarctation in children using a covered stent.

Coarctation of the abdominal aorta is extremely rare. It generally involves a long segment of the descending aorta and causes uncontrolled and unexplainable hypertension in children. The therapeutic choice is very challenging because acute and chronic complications are reported for both the surgical and the percutaneous approaches. The two reported cases of abdominal coarctation were treated primarily and successfully through the use of covered stents. Three covered stents were implanted in two children. No complication occurred with either procedure. At this writing, an 18-month follow-up assessment has found the patients in good health with no restenosis at the coarctation site. Covered stent implantation in children with abdominal coarctation is a feasible, safe, and effective procedure. It provides adequate relief of symptoms and reduces the risk of aneurysm formation. To avoid covering important side branches with polytetrafluoroethylene, this type of procedure must be preceded by precise study of the aorta and its branches.

Endomyocardial Biopsy in Pediatric Myocarditis and Dilated Cardiomyopathy: A Tool in Search for a Role.

Endomyocardial biopsy (EMB) is a well-known diagnostic tool for the investigation and treatment of myocardial diseases and remains the gold standard for the diagnosis of myocarditis. Due to its invasiveness, with a complication rate ranging from 1 to 15%, its role in the diagnostic work-up of pediatric heart failure is not well established. The aim of this review is to define the role of EMB as diagnostic technique in the work up of children presenting with severe left ventricular dysfunction with the support of our center experience.

Transcatheter interventions in patients with a Fontan circulation: Current practice and future developments.

The Fontan operation represents the last of multiple steps that are offered a wide range of congenital cardiac lesions with a single ventricle (SV) physiology. Nowadays this surgical program consists of a total cavopulmonary connection (TCPC), by anastomosing systemic veins to the pulmonary arteries (PAs), excluding the right-sided circulation from the heart. As a result of imaging, surgical, percutaneous, and critical care improvements, survival in this population has steadily increased. However, the Fontan physiology chronically increases systemic venous pressure causing systemic venous congestion and decreased cardiac output, exposing patients to the failure of the Fontan circulation (FC), which is associated with a wide variety of clinical complications such as liver disease, cyanosis, thromboembolism, protein-losing enteropathy (PLE), plastic bronchitis (PB), and renal dysfunction, ultimately resulting in an increased risk of exercise intolerance, arrhythmias, and premature death. The pathophysiology of the failing Fontan is complex and multifactorial; i.e., caused by the single ventricle dysfunction (diastolic/systolic failure, arrhythmias, AV valve regurgitation, etc.) or caused by the specific circulation (conduits, pulmonary vessels, etc.). The treatment is still challenging and may include multiple options and tools. Among the possible options, today, interventional catheterization is a reliable option, through which different procedures can target various failing elements of the FC. In this review, we aim to provide an overview of indications, techniques, and results of transcatheter options to treat cavopulmonary stenosis, collaterals, impaired lymphatic drainage, and the management of the fenestration, as well as to explore the recent advancements and clinical applications of transcatheter cavopulmonary connections, percutaneous valvular treatments, and to discuss the future perspectives of percutaneous therapies in the Fontan population.

Congenital Heart Disease: Growth Evaluation and Sport Activity in a Paediatric Population.

(1) Objective: To evaluate: (i) the associations of age and disease severity with anthropometric indices and weight status, (ii) the difference in the frequency of sports activity among different levels of disease severity in paediatric patients with congenital heart disease (CHD). (2) Methods: Clinical data of Caucasian children (aged 2-18 years) diagnosed with CHD (2005-2018) were retrospectively collected from the electronic register of outpatient visits. Of the 475 children with CHD, 368 children and their 1690 complete anthropometric measurements were eligible for inclusion in our analysis. (3) Results: Significant increase with age was observed for weight z-score [beta (95%CI): 0.03 (0.02, 0.05) for one-unit of age] and BMI z-score [0.06 (0.03, 0.08)] but not for height z-score. The probability of being underweight and overweight/obese increased and decreased with disease severity, respectively. The obesity probability of patients with mild CHD (0.06 [95%CI: 0.03, 0.08]) was not statistically distinguishable from that of patients with moderate CHD (0.03 [95%CI: 0.02, 0.05]), whereas it was lower in patients with severe CHD (0.004 [95%CI: 0.0, 0.009]). No obese patients with a univentricular heart defect were observed. Days spent in sport activities were equal to 1.9 [95%CI: 1.6, 2.2] days/week, 1.9 [1.5, 2.2], 1.4 [1.1, 1.7] and 0.7 [0.1, 1.3] in patients with mild, moderate, severe and univentricular CHD, respectively. (4) Conclusions: The risk of being overweight and obese should not be underestimated in paediatric patients diagnosed with CHD, especially in children with mild or moderate heart defects. It could be prevented or reduced by promoting a healthy lifestyle.

Bicuspid Aortic Valve Disease from Infancy to Older Age: A 25-Year Experience from an Italian Referral Center.

AIM: Bicuspid aortic valve (BAV) is the most common congenital heart defect, with considerable risk of morbidity and mortality. The purpose of the study was to analyze clinical and echocardiographic presentation of BAV in a large-volume tertiary Italian center and to test their interaction with full age span, sex, and first diagnosis versus second referral. METHODS: Consecutive patients of all ages diagnosed with BAV at our center from January 1988 to December 2012 were retrospectively included. Exclusion criteria were as follows: associated complex congenital cardiac disease, systemic syndrome, and previous cardiac surgery. RESULTS: Eligible patients were 790, divided by age quartiles. Seventy-two percent of patients had any grade BAV dysfunction. Aortic valve stenosis was more frequent in the first (24%) and fourth (24%) quartiles. This corresponds to a double-peak stenosis severity curve, being more severe at a very young age and in the elderly. Aortic valve regurgitation was more prevalent in each quartile than stenosis, with a prevalence of 72% in the second quartile and 77% in the third quartile. This corresponds to a single-peak regurgitation severity curve, being more severe in the fourth and fifth decades of life. Patients with previously diagnosed BAV had more significant valve dysfunction in comparison to patients with first diagnosis of BAV, either stenosis (15% vs. 21%, P = 0.024) or regurgitation (58% vs. 68%, P = 0.006). CONCLUSION: The dominant BAV dysfunction in this large Northern Italian community is regurgitation, with higher severity of disease in the fourth and fifth decades of life.

Impact of hard lockdown on interventional cardiology procedures in congenital heart disease: a survey on behalf of the Italian Society of Congenital Heart Disease.

The Coronavirus disease 2019 (COVID-19) pandemic has thoroughly and deeply affected the provision of healthcare services worldwide. In order to limit the in-hospital infections and to redistribute the healthcare professionals, cardiac percutaneous intervention in Pediatric and Adult Congenital Heart Disease (ACHD) patients were limited to urgent or emergency ones. The aim of this article is to describe the impact of the COVID-19 pandemic on Pediatric and ACHD cath laboratory activity during the so-called 'hard lockdown' in Italy. Eleven out of 12 Italian institutions with a dedicated Invasive Cardiology Unit in Congenital Heart Disease actively participated in the survey. The interventional cardiology activity was reduced by more than 50% in 6 out of 11 centers. Adolescent and ACHD patients suffered the highest rate of reduction. There was an evident discrepancy in the management of the hard lockdown, irrespective of the number of COVID-19 positive cases registered, with a higher reduction in Southern Italy compared with the most affected regions (Lombardy, Piedmont, Veneto and Emilia Romagna). Although the pandemic was brilliantly addressed in most cases, we recognize the necessity for planning new, and hopefully homogeneous, strategies in order to be prepared for an upcoming new outbreak.

Transcatheter Valve-in-Mitral Homograft in Tricuspid Position: First-in-Human Report.

A young woman with concomitant symptomatic severe stenosis and regurgitation was managed by percutaneous valve implantation because of a failing mitral homograft in the tricuspid position. The implant procedure was performed using an Edwards S3 23-mm valve. Good hemodynamic results were achieved, and, at intermediate follow-up, she did not require hospitalization and was free of symptoms.

Ischemia induced by transesophageal atrial pacing stress echocardiography predicts long-term mortality.

OBJECTIVES: It was the aim of this study to investigate the long-term value of transesophageal atrial pacing in predicting death in patients with known or suspected coronary artery disease. BACKGROUND: Exercise, dobutamine and dipyridamole stress echocardiography are all effective in predicting cardiac death. Transesophageal atrial pacing stress echocardiography (TAPSE) is a safe alternative to pharmacologic tests, but no information is available on prognosis with TAPSE. METHODS: One thousand and ten TAPSE were performed in 975 consecutive patients. TAPSE was feasible in 970 tests (96%); after exclusion of the 35 patients with more than 1 TAPSE and those 42 lost at follow-up (mean 4.5 +/- 3.7 years, median 6 years), the final population consisted of 857 patients (675 males, 58 +/- 9 years old). The Cox model was used to analyze the association of clinical, resting and TAPSE variables with cardiac death. RESULTS: TAPSE was abnormal in 281 (32%) patients. There were 46 cardiac-related deaths (5%), 25 among the 281 patients with an abnormal test (8.9%) and 21 among the 576 patients with a normal test (3.6%). The predictors of cardiac death were age, previous revascularization, resting wall motion score index and its variation during TAPSE. Abnormal TAPSE significantly increases the value of models predicting cardiac death. Moreover, cardiac mortality increased progressively with the extent of the induced ischemia. CONCLUSIONS: TAPSE is a useful tool in predicting death in patients with known or suspected coronary artery disease and might be considered an alternative to pharmacologic stressors.

Is late luminal loss an accurate predictor of the clinical effectiveness of drug-eluting stents in the coronary arteries?

Late luminal loss after percutaneous coronary intervention, although not normally distributed, has been shown to be monotonically correlated with the probability of binary angiographic restenosis after drug-eluting stent implantation. A recently proposed method has been shown to predict the restenosis rate after sirolimus-eluting stent and paclitaxel-eluting stent implantation using a power transformation of the value of late loss obtained after implantation of the 2 stent types. We used the same method to compute and compare restenosis rates from late loss values observed in the "head-to-head" randomized sirolimus-eluting stent versus paclitaxel-eluting stent comparisons available thus far. Our results showed that the model proposed has a poor overall ability to predict the real incidence and relative risk of restenosis because the use of late loss as an end point tended to overestimate the difference in restenosis, and the derived effect estimate of 1 stent compared with that of the other seemed to be overemphasized with respect to the real risk. We thus believe that further investigations are needed to appraise the real clinical effect of late loss and its reliability as an end point in direct comparative drug-eluting stent trials before its use can be recommended as a clinical surrogate.

Comparison of the long-term survival benefits associated with revascularization or medical therapy in patients with known coronary artery disease undergoing transesophageal atrial pacing stress echocardiography.

Although the utility of stress echocardiography for the diagnosis and prognostic evaluation of patients with suspected coronary artery disease (CAD) has been widely reported, few studies have evaluated the role of revascularization in relation to the presence of inducible ischemia during stress in patients with known CAD. The study population consisted of 295 consecutive patients who underwent transesophageal atrial pacing stress echocardiography (TAPSE) in the echocardiographic laboratory of our division between January 1988 and September 1997, in whom coronary angiography was performed within 10 days of the test. Patients were then assigned to revascularization or medical treatment according to the treatment given within 60 days of TAPSE. Cardiac-related deaths were higher in medically treated (19 of 135) than in revascularized (8 of 160) patients (p = 0.03). Parameters measured with TAPSE, i.e., positivity of the test, change in wall motion score index (DeltaWMSI and peak WMSI) were significantly related to mortality in medically treated patients but not in revascularized patients. At multivariate analysis, DeltaWMSI remained the most powerful predictor of cardiac death in medically treated patients (p = 0.005). Mortality progressively increased with increments in extent of inducible ischemia among medically treated patients (5 of 71 patients in DeltaWMSI 0, 3 of 27 in DeltaWMSI 0 to 25, 11 of 37 patients in DeltaWMSI >25) but not among revascularized patients (3 of 58 patients in DeltaWMSI 0, 2 of 51 in DeltaWMSI 0 to 25, 3 of 51 patients in DeltaWMSI >25). The survival curve in medically treated patients with ischemia in a remote zone (24 patients, 8 deaths) was worse than in other groups of medically treated patients (41 patients, 6 deaths). In conclusion, in patients with known CAD, the presence and extent of ischemia as evaluated with TAPSE worsens survival, if revascularization is not performed. In patients without ischemia at TAPSE, revascularization or medical therapy are equally effective.