Outcomes of transcatheter pulmonary SAPIEN 3 valve implantation: an international registry.

BACKGROUND AND AIMS: Transcatheter pulmonary valve implantation (TPVI) is indicated to treat right-ventricular outflow tract (RVOT) dysfunction related to congenital heart disease (CHD). Outcomes of TPVI with the SAPIEN 3 valve that are insufficiently documented were investigated in the EUROPULMS3 registry of SAPIEN 3-TPVI. METHODS: Patient-related, procedural, and follow-up outcome data were retrospectively assessed in this observational cohort from 35 centres in 15 countries. RESULTS: Data for 840 consecutive patients treated in 2014-2021 at a median age of 29.2 (19.0-41.6) years were obtained. The most common diagnosis was conotruncal defect (70.5%), with a native or patched RVOT in 50.7% of all patients. Valve sizes were 20, 23, 26, and 29 mm in 0.4%, 25.5%, 32.1%, and 42.0% of patients, respectively. Valve implantation was successful in 98.5% [95% confidence interval (CI), 97.4%-99.2%] of patients. Median follow-up was 20.3 (7.1-38.4) months. Eight patients experienced infective endocarditis; 11 required pulmonary valve replacement, with a lower incidence for larger valves (P = .009), and four experienced pulmonary valve thrombosis, including one who died and three who recovered with anticoagulation. Cumulative incidences (95%CI) 1, 3, and 6 years after TPVI were as follows: infective endocarditis, 0.5% (0.0%-1.0%), 0.9% (0.2%-1.6%), and 3.8% (0.0%-8.4%); pulmonary valve replacement, 0.4% (0.0%-0.8%), 1.3% (0.2%-2.4%), and 8.0% (1.2%-14.8%); and pulmonary valve thrombosis, 0.4% (0.0%-0.9%), 0.7% (0.0%-1.3%), and 0.7% (0.0%-1.3%), respectively. CONCLUSIONS: Outcomes of SAPIEN 3 TPVI were favourable in patients with CHD, half of whom had native or patched RVOTs.

Right disc thrombosis of the new Gore Cardioform ASD Occluder.

BACKGROUND: The GCA is a well performing device in terms of efficacy despite complex anatomies (aortic rim <5 mm and ASD diameter >17 mm) with a good safety profile. AIMS: To evaluate atrial septal defect (ASD) features impacting on right disc device thrombosis in patients who underwent Gore Cardioform ASD Occluder (GCA) implantation. METHODS: A total of 44 consecutive patients undergoing percutaneous ASD with GCA device from January 2020 to September 2022 at our tertiary care Center were evaluated. The minimum follow-up was 6 months. RESULTS: The patients were stratified in two groups according to a cut-off value of ASD diameter equal to 20 mm at sizing balloon, derived from ROC analysis (AUC = 0.894; p = 0.024). Baseline characteristics were comparable between groups in terms of age, sex, weight, height, and interatrial septum dimensions. Patients with ASD > 20 mm (n = 9) had a higher ASD/device dimension ratio, both at echocardiography (p = 0.009) and at sizing balloon (p = 0.001), longer fluoroscopic time (p = 0.022), and higher incidence of device thrombosis (0.006). Right disc thrombosis was observed in three patients of the ASD > 20 mm group, always in the inferior portion of the right disc. On univariate analysis, ASD diameter at sizing balloon (OR 1.360; p = 0.036) was the only positive predictor of device thrombosis. CONCLUSIONS: Right disc thrombosis of the GCA device may be under-recognized at follow-up, hence deserving clinical attention, especially in those patients with larger ASD diameters.

Solving Intraoperative Complications During Endovascular Repair of Late Contained Ruptured Aortic Pseudoaneurysm After Surgical De-coarctation: Case Report and Systematic Review of Literature.

AIM: We present a case of successful endovascular repair of late ruptured aortic anastomotic pseudoaneurysm following previous left subclavian artery-descending thoracic aorta bypass and concomitant emergency thoracic endovascular aortic repair (TEVAR) of complicated endovascular aortic de-coarctation never previously described is also presented. A review of the intraoperative, 30-day, and follow-up morbidity, mortality, and complications of TEVAR as endovascular treatment of late aneurysm/pseudoaneurysms after surgical aortic de-coarctation was also performed. METHODS: The systematic review was performed in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) statement and following PICO model. A literature search was conducted on MEDLINE, PubMed, EMBASE, Cochrane Library, Google Scholar, Science Direct, and Web of Science using the words "tevar of late complication of aortic coarctation surgical repair" and "endovascular repair of anastomotic pseudoaneurysm in coarctation" up to June 17, 2022. Data were extracted from study documents about study design, patient's demographics and comorbidities, details about primary surgical repair, type of late complication, time between open surgery and occurrence of complications, details of the secondary endovascular procedure with technical success, early, and follow-up mortality and morbidity. RESULTS: A total of 18 papers were included with 78 patients (48 men, 69.5%). The most frequent type of primary open surgical repair was patch aortoplasty (46, 58.9%). Focusing on aneurysm and pseudoaneurysm as late complications, most of the patients were asymptomatic (45, 57.7%). All patients underwent TEVAR, 14 of them (17.9%) in urgent/emergent setting. The technical success was 98.7%, with 1 intraoperative death due to rupture of the aorta. In total, 31 patients out of 78 (39.7%) showed different complications in the immediate postoperative time, with type II endoleak being the most observed (8/31, 25.8%). The mean follow-up time was about 2 years (26.5 months, range 3-92). Overall, 30-day mortality was 2.6%. Complications occurred in 30 patients (39.4%), 23 of them resolved during the follow-up period. CONCLUSIONS: With the limit of low-quality data, TEVAR can be considered a safe and effective option for the treatment of late complications after open surgery for aortic coarctation, even in urgent settings. CLINICAL IMPACT: Different specialists have to face the technical complexities and risks related to treatment of late complications after surgical de-coartaction, which can be either surgical or endovascular, and depend on patient's ages. Although covered stents appear to have some protection from the development of stent fractures, doesn't provide complete protection from late aneurysm formation. In this setting, TEVAR may represent a valuable option, combining the advantages of the covered stent with those of a device that can cover a wider range of aortic length, especially in adult patients. This study shows thoracic endovascular repair can be considered a safe and effective option in clincal practice for the treatment of late complications after open surgery for AC, even in urgent settings.

Right and left ventricle native T1 mapping in systolic phase in patients with congenital heart disease.

BACKGROUND: T1 mapping is emerging as a powerful tool in cardiac magnetic resonance (CMR) to evaluate diffuse fibrosis. However, right ventricular (RV) T1 mapping proves difficult due to the limited wall thickness in diastolic phase. Several studies focused on systolic T1 mapping, albeit only on the left ventricle (LV). PURPOSE: To estimate intra- and inter-observer variability of native T1 (nT1) mapping of the RV, and its correlations with biventricular and pulmonary function in patients with congenital heart disease (CHD). MATERIAL AND METHODS: In this retrospective, observational, cross-sectional study we evaluated 36 patients with CHD, having undergone CMR on a 1.5-T scanner. LV and RV functional evaluations were performed. A native modified look-locker inversion recovery short-axis sequence was acquired in the systolic phase. Intra- and inter-reader reproducibility were reported as complement to 100% of the ratio between coefficient of reproducibility and mean. Spearman ρ and Mann-Whitney U-test were used to compare distributions. RESULTS: Intra- and inter-reader reproducibility was 84% and 82%, respectively. Median nT1 was 1022 ms (interquartile range [IQR] 1108-972) for the RV and 947 ms (IQR 986-914) for the LV. Median RV-nT1 was 1016 ms (IQR 1090-1016) in patients with EDVI ≤100 mL/m2 and 1100 ms (IQR 1113-1100) in patients with EDVI >100 mL/m2 (P = 0.049). A significant negative correlation was found between RV ejection fraction and RV-nT1 (ρ = -0.284, P = 0.046). CONCLUSION: Systolic RV-nT1 showed a high reproducibility and a negative correlation with RV ejection fraction, potentially reflecting an adaptation of the RV myocardium to pulmonary valve/conduit (dys)-function.

Acute and midterm outcomes of the post-approval MELODY Registry: a multicentre registry of transcatheter pulmonary valve implantation.

AIMS: The post-approval MELODY Registry aimed to obtain multicentre registry data after transcatheter pulmonary valve implantation (TPVI) with the Melody™ valve (Medtronic plc.) in a large-scale cohort of patients with congenital heart disease (CHD). METHODS AND RESULTS: Retrospective analysis of multicentre registry data after TPVI with the Melody™ valve. Eight hundred and forty-five patients (mean age: 21.0 ± 11.1 years) underwent TPVI in 42 centres between December 2006 and September 2013 and were followed-up for a median of 5.9 years (range: 0-11.0 years). The composite endpoint of TPVI-related events during follow-up (i.e. death, reoperation, or reintervention >48 h after TPVI) showed an incidence rate of 4.2% per person per year [95% confidence interval (CI) 3.7-4.9]. Transcatheter pulmonary valve implantation infective endocarditis (I.E.) showed an incidence rate of 2.3% per person per year (95% CI 1.9-2.8) and resulted in significant morbidity and in nine deaths. In multivariable Cox proportional hazard models, the invasively measured residual right ventricle (RV)-to-pulmonary artery (PA) pressure gradient (per 5 mmHg) was associated with the risk of the composite endpoint (adjusted hazard ratio: 1.21, 95% CI 1.12-1.30; P < 0.0001) and the risk of TPVI I.E. (adjusted hazard ratio: 1.19, 95% CI 1.07-1.32; P = 0.002). Major procedural complications (death, surgical, or interventional treatment requirement) occurred in 0.5%, 1.2%, and 2.0%, respectively. Acutely, the RV-to-PA pressure gradient and the percentage of patients with pulmonary regurgitation grade >2 improved significantly from 36 [interquartile range (IQR) 24-47] to 12 (IQR 7-17) mmHg and 47 to 1%, respectively (P < 0.001 for each). CONCLUSION: The post-approval MELODY Registry confirms the efficacy of TPVI with the Melody™ valve in a large-scale cohort of CHD patients. The residual invasively measured RV-to-PA pressure gradient may serve as a target for further improvement in the composite endpoint and TPVI I.E. However, TPVI I.E. remains a significant concern causing significant morbidity and mortality.

Use of 65 cm large caliber Dryseal sheaths to facilitate delivery of the Edwards SAPIEN valve to dysfunctional right ventricular outflow tracts.

BACKGROUND: The Edwards SAPIEN valve and its delivery system may complicate transit through the right heart during transcatheter pulmonary valve replacement (tPVR). We report our early experience using a large diameter, 65 cm delivery sheath to facilitate delivery of the SAPIEN valve to the right ventricular outflow tract (RVOT). METHODS: Retrospective analysis of all patients from three large congenital heart centers undergoing tPVR with the Edwards SAPIEN valve delivered with the 65 cm Gore Dryseal Sheath. RESULTS: Over a 12 month period, 30 patients (17 female) with median age 17.5 years (range 8-72) underwent attempted tPVR with the SAPIEN valve delivered using the 65 cm Dryseal sheath (20-26Fr). All procedures resulted in successful valve delivery to the target area. Twenty patients had a native RVOT. The most commonly used valve diameter was 29 mm (n = 15) with the majority of cases requiring a 26Fr Dryseal sheath (n = 20). One patient with severe RVOT stenosis underwent prestenting. Median procedure time was 100 min (59-225). No patient had increase in tricuspid valve regurgitation as a consequence of valve delivery. One patient required a synchronous cardioversion for intraprocedural VT and another required ECMO postprocedure due to severe pre-existing left ventricular dysfunction. On median follow-up of 5 months, all patients had mild or less pulmonary regurgitation. Median peak Doppler velocity across the pulmonary valve was 2.2 m/s (1.7-4). There were no clinically relevant complications relating to vascular access. CONCLUSIONS: Using 65 cm Dryseal sheaths facilitates delivery of SAPIEN valves in patients with dysfunctional RVOTs.

Molding the shape of congenial and structural interventional cardiology: interviews with directors of major congresses.

The range and number of educational and networking events that are available for fellows, trainees, and junior faculty to attend grows every year. Each meeting useful in its own way; each adding value to the development and the growth of an interventionist. Within paediatric, congenital, and structural heart disease, three of the standout meetings are: Pediatric and Interventional Cardiac Symposium (PICS-AICS), Congenital and Structural Interventions (CSI), and International Workshop on Interventional Pediatric and Adult Congenital Cardiology (IPC). All of these were started by leaders in our field; people known to be passionate educators and innovators. International congresses focusing more broadly on congenital cardiac disease in children and adults are rare. These forums allow more interdisciplinary discussions between the interventionist, surgeon, and non-invasive specialists. Purely interventional meetings are essential to allow colleagues to debate and explore the nuances and intricacies of technique and approach, developing concepts to be challenged in wider forums. During the recent 21st PICS-AICS meeting Prof. Ziyad M. Hijazi, Shakeel A. Qureshi, Mario Carminati, and Dr Damien Kenny shared their time to engage in frank, recorded conversations which provide a unique insight in to the process and concepts behind three of our most important educational congresses.

Novel JAG1 Deletion Variant in Patient with Atypical Alagille Syndrome.

Alagille syndrome (AGS) is an autosomal-dominant disorder characterized by various degrees of abnormalities in the liver, heart, eyes, vertebrae, kidneys, face, vasculature, skeleton, and pancreas. This case report describes a newborn child exhibiting a congenital neural tube defect and peculiar craniofacial appearance characterized by a prominent forehead, deep-set eyes, bulbous nasal tip, and subtle upper lip. Just a few hours after birth, congenital heart disease was suspected for cyanosis and confirmed by heart evaluation. In particular, echocardiography indicated pulmonary atresia with ventricular septal defect with severe hypoplasia of the pulmonary branches (1.5 mm), large patent ductus arteriosus and several major aortopulmonary collateral arteries. Due to the association of peculiar craniofacial appearance and congenital heart disease, a form of Alagille syndrome was suspected. In addition, on the fifth day after birth, the patient developed jaundice, had acholic stools, and high levels of conjugated bilirubin and gamma-glutamyltransferase (GGT) were detected in the blood. Genetic testing revealed the novel variant c.802del in a single copy of the JAG1 gene. No variants in the NOTCH2 gene were detected. To the best of our knowledge, this is the first clinical description of a congenital neural tube defect in a molecularly confirmed Alagille patient. This work demonstrates a novel pathogenic heterozygous JAG1 mutation is associated with an atypical form of Alagille syndrome, suggesting an increased risk for neural tube defects compared to other Alagille patients.

Blood-threshold CMR volume analysis of functional univentricular heart.

PURPOSE: To validate a blood-threshold (BT) segmentation software for cardiac magnetic resonance (CMR) cine images in patients with functional univentricular heart (FUH). MATERIALS AND METHODS: We evaluated retrospectively 44 FUH patients aged 25 ± 8 years (mean ± standard deviation). For each patient, the epicardial contour of the single ventricle was manually segmented on cine images by two readers and an automated BT algorithm was independently applied to calculate end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), ejection fraction (EF), and cardiac mass (CM). Aortic flow analysis (AFA) was performed on through-plane images to obtain forward volumes and used as a benchmark. Reproducibility was tested in a subgroup of 24 randomly selected patients. Wilcoxon, Spearman, and Bland-Altman statistics were used. RESULTS: No significant difference was found between SV (median 57.7 ml; interquartile range 47.9-75.6) and aortic forward flow (57.4 ml; 48.9-80.4) (p = 0.123), with a high correlation (r = 0.789, p < 0.001). Intra-reader reproducibility was 86% for SV segmentation, and 96% for AFA. Inter-reader reproducibility was 85 and 96%, respectively. CONCLUSION: The BT segmentation provided an accurate and reproducible assessment of heart function in FUH patients.

Closure of patent foramen ovale defects using GORE® CARDIOFORM septal occluder: Results from a prospective European multicenter study.

AIMS: The GORE® CARDIOFORM Septal Occluder (GSO) is a novel device designed for rapid and effective closure of patent foramen ovale (PFO) which has distinctive features making it suitable for a broad spectrum of anatomical variations. We report the procedural and 6 months follow-up results of the first prospective, multicenter study using GSO. METHODS AND RESULTS: This single-arm study included 150 subjects undergoing closure of PFO in 10 European centers. In 149 out of 150 patients implantation of a GSO device was successful. One patient had a different PFO-closure device implanted. Periprocedural complications were few including one patient with suspected transient ischemic attack, two access site bleedings, and one patient with AV-fistula. No device embolization occurred. During the 6-month follow-up period one patient had a transient asymptomatic thrombus on the device and four patients (2.6%) were diagnosed new onset paroxysmal atrial fibrillation, which were successfully treated. No thrombembolic events occurred. Closure was successful in 94.2% of subjects at discharge evaluation and 96.9% at 6 months follow-up. CONCLUSION: This prospective, multicenter study adds to previous published data and suggests that GSO is a versatile device for PFO closure with high procedural and closure success rates and low complication rates through mid-term follow-up. © 2017 Wiley Periodicals, Inc.

Goose-neck snare-assisted transcatheter ASD closure: A safety procedure for large and complex ASDs.

OBJECTIVE: To report on a new technique that increases the safety of percutaneous atrial septal defect (ASD) closure using a goose-neck snare system. BACKGROUND: ASD transcatheter closure is a widespread procedure. However, in some cases, ASDs may be large and with soft rims. In these situation, a potential risk exists for device malposition or embolization. METHODS: When transesophageal echocardiography (TEE) evaluation and balloon sizing showed large defects with floppy rims the chosen Amplatzer device was implanted in a standard way. In large defects with floppy rims, before release a 5-mm goose-neck snare with its 4 Fr catheter was placed across the delivery cable and fixed to catch the screwing mechanism of implanted Amplatzer device. The delivery cable was unscrewed and the device reached its final position without any tension. If the position was considered satisfactory the device was released from the goose-neck snare. RESULTS: Thirteen patients had a snare-assisted ASD transcatheter closure. Median device size was 24 mm (range 14-38 mm). Retrieval or repositioning of the device using the goose-neck snare was performed in four cases: in three patients, because of device malposition after delivery cable release and in one patient, because of unsuitability of closure of a second significant defect. Furthermore, in two subjects with multiple ASDs, a second fenestration looked quite significant with the device still attached to the delivery cable while it appeared smaller after release. CONCLUSIONS: Snare-assisted Amplatzer ASD device placement is a new method for ASD percutaneous closure and adds safety to the procedure.

The care for adults with congenital heart disease: organization and function of a grown-up congenital heart disease unit.

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

Treatment of right ventricular outflow tract dysfunction: a multimodality approach.

The right timing to replace the pulmonary valve in a patient with dysfunction of the right ventricular outflow tract is unknown. Both percutaneous pulmonary valve and surgical prosthesis are suitable options. In every patient, the right ventricle (RV) remodels and recovers differently after pulmonary replacement. Therefore, it is difficult to identify the best treatment option and to predict the long-term results. In the last few years, we focused our research on optimizing the characterization of these patients through advanced cardiovascular imaging in order to find possible variables, parameters, and reproducible measurements that can help us in the decision-making process. The aim of the present article is to present our ongoing research lines that focus on the characterization and optimal treatment approach to the dysfunction of the RVOT.

International cooperation in healthcare: model of IRCCS Policlinico San Donato and Bambini Cardiopatici nel Mondo Association for congenital heart diseases.

Cooperation activity in training programmes promoted by IRCCS Policlinico San Donato and Bambini Cardiopatici nel Mondo Association is a model of cooperation between people as an alternative intervention in promoting the right to healthcare, especially offering programmes of training and medical care on cardiovascular and congenital heart disease. This new strategy, implemented in several developing countries, has absolutely contributed to the improvement of the medical services concerning the diagnostic and surgical approach in the treatment of paediatric and adult cardiovascular disease. To strengthen this kind of activity, both IRCCS Policlinico San Donato and Bambini Cardiopatici nel Mondo have introduced a global perspective aiming at the realization of surgical missions 'in situ', building new cardiac surgery units in collaboration with the local partners, which are mainly university hospitals. They, furthermore, support financially the scholarship and accommodation in favour of medical and paramedical staff.

The Edwards Valeo lifestents in the treatment and palliation of congenital heart disease in infants and small children.

BACKGROUND: Intravascular stenting is the procedure of choice in the treatment of vascular stenoses. However, in infants and small children large sheaths are needed, and adult-size stents cannot be implanted. The Valeo Biliary Lifestent (Edwards Lifesciences Irvine, CA) is low profile and can be dilated up to 18-20 mm. We aimed to report on early and mid-term results with the use of Valeo stents in infants and children with congenital heart disease. METHODS: Twenty-five subjects were treated in our experience. Mean age and weight were 39 ± 35 months (range 1-132) and 10.4 ± 6.7 kg (range 3-30), respectively. Two groups of patients were: Group A: patients with pulmonary artery stenosis (21 subjects); Group B subjects in whom stenting was applied in other vessel or heart sites (four subjects). RESULTS: A total of 35 stents were successfully used. Fluoroscopy time was 32 ± 11 min. No intra-operative death or hospital mortality was recorded. Stent post dilatation was performed in nine patients. The incidence of complication was 12% (3/25) (two subjects developed transient hypotension and bradycardia which required inotropic treatment, 1 patient developed mild lung bleeding). There was a significant improvement of angiographic appearance and RV pressure (RV/AO systolic pressure ratio before 1 ± 02 (range 0.8-1.5) versus after the procedure 0.6 ± 0.2 (range 0.4-0.9) P < 0.001). In group B stents were successfully implanted in aortic recoarctation, interatrial septum and in two modified BT shunts. At a median follow-up of 18 months (range 1-24 months) results remained stable and no complications occurred. In particular no stent fractures were seen. Furthermore, redilation was performed safely and successfully in three subjecs up to 18 months after the first implantation. CONCLUSIONS: In our series, Valeo Lifestents have proven to be effective and with low incidence of complication in various anatomical settings, in low weight infants and in early post-operative course. Large series and longer follow-up are mandatory.

Four-year cardiac magnetic resonance (CMR) follow-up of patients treated with percutaneous pulmonary valve stent implantation.

OBJECTIVES: To investigate follow-up after percutaneous pulmonary valve implantation (PPVI). METHODS: Forty patients with pulmonary conduit dysfunction (males/females 24/16; 21 ± 08 years; 12 tetralogy of Fallot, 11 aortic valve disease, 17 other congenital heart disease) were planned for CMR before PPVI and repeated 7 times up to 48 months. CMR prospective results regarded: pressure gradient (PG) and regurgitation fraction (RF); end-diastolic volume, end-systolic volume, and stroke volume indexed to body surface area (EDVI, ESVI, and SVI); ejection fraction (EF) of right and left ventricles (RV, LV). A Friedman test was used for comparisons. RESULTS: Overall, PG (31 ± 06 to 16 ± 4 mmHg), RF (16 ± 17 to 0.3 ± 1 %), RVEDVI (82 ± 38 to 58 ± 12 ml/m(2)), and RVESVI (44 ± 12 to 30 ± 13 ml/m(2)) declined (p < 0.001), RVEF (49 ± 13 to 58 ± 12 %) and RVSVI (from 38 ± 14 to 40 ± 8 ml/m(2)) increased (p < 0.001), LVEDVI (67 ± 17 to 73 ± 18 ml/m(2)) and LVSVI (37 ± 11 to 43 ± 10 ml/m(2)) increased (p = 0.034 and p < 0.001). Two patients had valve fracture at 24 and 36 months and underwent surgery. One patient had stent restenosis at 24 months and underwent percutaneous retreatment. Baseline/follow-up CMR did not predict PPVI failure. CONCLUSIONS: CMR demonstrated restored pulmonary conduit function, reduced RV volumes and increased RV and LV function but did not predict valve fracture/restenosis. KEY POINTS: • A CMR 4-year follow-up after PPVI showed restored pulmonary conduit function • RV volumes were significantly reduced • RV function was significantly better in terms of increased EF and SVI • LV function was significantly better in terms of increased EDVI and SVI • Baseline/follow-up CMR did not predict three cases of PPVI failure.

Evaluation of right ventricular function in adults with congenital heart defects.

The right ventricle (RV) is of lesser importance in acquired heart disease, but its role is of increasing importance in congenital heart disease (CHD). Despite major progress being made, precise measurements of the RV are challenging because of its peculiar anatomical structure that is not adaptable to any planar geometrical assumption. This is particularly true in adult patients with CHD where the RV shape eludes any standardization, it may assume various morphologies, and its modality of contraction depends on previous surgical treatment and/or pathophysiological conditions. However, reliable and repeatable quantification of RV dimensions and function for these patients are essential to provide appropriate timing for intervention to optimize outcomes. In this population, echocardiographic evaluation should not be limited to an observational and subjective functional assessment of the RV but must provide quantitative values repeatable and clinically reliable to help the decision-making process. The aim of this review was to discuss the echocardiographic approach to the RV in adult patients with CHD in general and in specific cases of pressure or volume overload.

From bare to covered: 15-year single center experience and follow-up in trans-catheter stent implantation for aortic coarctation.

BACKGROUND: Bare stents has become the first line therapy for aortic coarctation. Covered stents has been reported more recently in clinical practice. OBJECTIVES: The present study, reports comparatively 15-year experience of bare and covered stent implantation for aortic coarctation in a single tertiary referral center. METHODS: From 1997 to 2011, 143 patients with native or postoperative aortic coarctation were treated at our institution. Seventy-one subjects (median age 17 years (range from 4 to 70 years) underwent bare stent implantation (Group 1) while 72 patients (median age of 17.5 years (range from 6 to 68 years) underwent covered stent implantation (Group 2). RESULTS: Success rate in the whole group was 95%. More complex and tighter coarctations were treated using covered stents. Incidence of related-procedure adverse events was higher in Group 1 than in group 2 (21.1% vs. 8.3% P = 0.035). Aortic wall complications occurred in 7% of patients in Group 1 (one death) and 0% in Group 2 (P = 0.028). Subjects in Group 1 had a longer follow-up (median 85 vs. 35 months; P < 0.001). Independent predictors associated with reintervention included the presence of complex lesions (HR: 2.70; CI: 1.15-6.32), balloon diameter used <14 mm (HR: 3.76; CI: 1.48-9.55), and immediate residual gradient >10 mm Hg (HR: 4.30; CI: 1.96-9.47). CONCLUSIONS: Both bare and covered stent implantation for aortic coarctation is a safe and efficacious treatment. By using covered stent implantation the spectrum of patients treated has increased with lower rates of acute and late complications.

The impact of actual and perceived disease severity on pre-operative psychological well-being and illness behaviour in adult congenital heart disease patients.

OBJECTIVES: The purpose of this study was to investigate whether the objective medical parameters related to congenital heart disease and patients' ratings of cardiac disease severity were related to psychological well-being and illness behaviour during the pre-operative period. METHODS: A total of 143 patients (63 male patients; 16-73 years old) with congenital heart disease evaluated the severity of their cardiac condition using a numerical rating scale ranging from 0, indicating the least severe condition, to 100, indicating the most severe condition. Psychological well-being was assessed using the Psychological General Well-Being Index (total score ≤ 60 indicating severe distress) and illness behaviour using the Illness Behavior Questionnaire. RESULTS: Pre-operative psychological well-being was not related to the objective medical parameters reflecting cardiac disease severity. In contrast, total psychological well-being scores correlated significantly with patients' subjective ratings of disease severity (p < 0.001). When compared with the reference values from the Italian population, the mean scores of the patients on psychological well-being were similar. As regards the Illness Behavior Questionnaire, the scores on denial were higher and those on hypochondria were lower compared with other hospitalised patients. CONCLUSIONS: This study shows that the perception of cardiac disease severity, and not the medical parameters in congenital heart disease, is related to the patients' pre-operative psychological state. Thus, more importance needs to be given to assessing the patients' pre-operative perception and psychological state independently of cardiac severity. Targeted interventions with regard to the cardiac condition are recommended.

Cardiac magnetic resonance before and after percutaneous pulmonary valve implantation.

PURPOSE: To measure the magnetic resonance (MR) artefact produced by a percutaneous pulmonary valve stent and to evaluate the changes in volumetric and functional right ventricle (RV) parameters due to percutaneous pulmonary valve implantation (PPVI). MATERIALS AND METHODS: A Melody valve was studied in vitro using clinical cardiac MR (CMR) sequences. In vivo, we analysed the CMR examinations obtained before and after PPVI of 27 consecutive patients. The echocardiography pressure gradient (PG) and catheter PG were measured. The Wilcoxon test was used for comparisons. RESULTS: In vitro, the least difference between artefact extent and actual valve size (0.1 mm) was obtained with a steady-state free precession (SSFP) sequence. In vivo, RV end-diastolic volume (ml/m(2)), end-systolic volume (ml/m(2)) and ejection fraction (%) were 79 ± 42, 43 ± 41 and 49 ± 13 before PPVI and 64 ± 21 (p = 0.054), 30 ± 14 (p = 0.021), and 54 ± 12 (p = 0.018) 6 months after PPVI, respectively. The PG and regurgitation fraction (RF) were 36 ± 15 mmHg and 14 ± 18 % before PPVI and 13 ± 15 mmHg (p < 0.001) and 2 ± 5 % (p = 0.013) after PPVI, respectively. No significant differences were found comparing the PG measured with CMR, echocardiography and catheter. CONCLUSIONS: We showed in vitro that the SSFP sequence produced the most accurate valve measurement. After PPVI, CMR showed a strong decrease of PG and RF with a significant improvement of RV function.