Transcatheter closure of large postinfarct ventricular septal defect: Initial results of prototype Occlutech® device including the first-in-human.

BACKGROUND: Postinfarct ventricular septal rupture is a serious complication in delayed or failed reperfusion with a grim prognosis. The optimal timing and treatment option remain debatable in the absence of randomized controlled trials. Percutaneous device closure is a well-reported and less invasive treatment option but recent imaging studies indicate that majority of defects are too large to be adequately covered by the currently Conformite Europeenne and Food and Drug Administration approved occluder devices. METHODS: Six patients presented with large and complex postinfarct ventricular septal ruptures, considered unsuitable for the Amplatzer post-infarct ventricular septal defect Occluder, so were treated using the prototype Occlutech® 36 mm PI-VSD occluder, including the first-in-human use. RESULTS: The prototype device was successfully deployed in all patients with satisfactory immediate results and shunt reduction. Three patients (50%) in cardiogenic shock did not survive beyond discharge, of which two were complicated by device dislodgement or embolization. CONCLUSIONS: Percutaneous closure of large postinfarct ventricular septal ruptures is possible using newer device with a wider coverage. Further device refinement is necessary to improve treatment outcomes.

A practical guideline for performing a comprehensive transthoracic echocardiogram in the congenital heart disease patient: consensus recommendations from the British Society of Echocardiography.

Transthoracic echocardiography is an essential tool in the diagnosis, assessment, and management of paediatric and adult populations with suspected or confirmed congenital heart disease. Congenital echocardiography is highly operator-dependent, requiring advanced technical acquisition and interpretative skill levels. This document is designed to complement previous congenital echocardiography literature by providing detailed practical echocardiography imaging guidance on sequential segmental analysis, and is intended for implementation predominantly, but not exclusively, within adult congenital heart disease settings. It encompasses the recommended dataset to be performed and is structured in the preferred order for a complete anatomical and functional sequential segmental congenital echocardiogram. It is recommended that this level of study be performed at least once on all patients being assessed by a specialist congenital cardiology service. This document will be supplemented by a series of practical pathology specific congenital echocardiography guidelines. Collectively, these will provide structure and standardisation to image acquisition and reporting, to ensure that all important information is collected and interpreted appropriately.

Isolating the Effect of Arch Architecture on Aortic Hemodynamics Late After Coarctation Repair: A Computational Study.

Objectives: Effective management of aortic coarctation (CoA) affects long-term cardiovascular outcomes. Full appreciation of CoA hemodynamics is important. This study aimed to analyze the relationship between aortic shape and hemodynamic parameters by means of computational simulations, purposely isolating the morphological variable. Methods: Computational simulations were run in three aortic models. MRI-derived aortic geometries were generated using a statistical shape modeling methodology. Starting from n = 108 patients, the mean aortic configuration was derived in patients without CoA (n = 37, "no-CoA"), with surgically repaired CoA (n = 58, "r-CoA") and with unrepaired CoA (n = 13, "CoA"). As such, the aortic models represented average configurations for each scenario. Key hemodynamic parameters (i.e., pressure drop, aortic velocity, vorticity, wall shear stress WSS, and length and number of strong flow separations in the descending aorta) were measured in the three models at three time points (peak systole, end systole, end diastole). Results: Comparing no-CoA and CoA revealed substantial differences in all hemodynamic parameters. However, simulations revealed significant increases in vorticity at the site of CoA repair, higher WSS in the descending aorta and a 12% increase in power loss, in r-CoA compared to no-CoA, despite no clinically significant narrowing (CoA index >0.8) in the r-CoA model. Conclusions: Small alterations in aortic morphology impact on key hemodynamic indices. This may contribute to explaining phenomena such as persistent hypertension in the absence of any clinically significant narrowing. Whilst cardiovascular events in these patients may be related to hypertension, the role of arch geometry may be a contributory factor.

Wave Reflection and Ventriculo-Arterial Coupling in Bicuspid Aortic Valve Patients With Repaired Aortic Coarctation.

BACKGROUND: Ventriculo-arterial (VA) coupling in bicuspid aortic valve (BAV) patients can be affected by the global aortopathy characterizing BAV disease and the presence of concomitant congenital lesions such as aortic coarctation (COA). This study aimed to isolate the COA variable and use cardiovascular magnetic resonance (CMR) imaging to perform wave intensity analysis non-invasively to shed light on VA coupling changes in BAV. The primary hypothesis was that BAV patients with COA exhibit unfavorable VA coupling, and the secondary hypothesis was that BAV patients with COA exhibit increased wave speed as a marker of reduced aortic distensibility despite successful surgical correction. METHODS: Patients were retrospectively identified from a CMR database and divided into two groups: isolated BAV and BAV associated with repaired COA. Aortic and ventricular dimensions, global longitudinal strain (GLS), and ascending aortic flow data and area were collected and used to derive wave intensity from CMR data. The main variables for the analysis included all wave magnitudes (forward compression/expansion waves, FCW and FEW, respectively, and reflected backward compression wave, BCW) and wave speed. RESULTS: In the comparison of patients with isolated BAV and those with BAV associated with repaired COA (n = 25 in each group), no differences were observed in left ventricular ejection fraction, GLS, or ventricular volumes, whilst significant increases in FCW and FEW magnitude were noted in the BAV and repaired COA group. The FCW inversely correlated with age and aortic size. Whilst the BCW was not significantly different compared with that in patients with/without COA, its magnitude tends to increase with a lower COA index. Patients with repaired COA exhibited higher wave speed velocity. Aortic wave speed (inversely related to distensibility) was not significantly different between the two groups. CONCLUSION: In the absence of a significant restenosis, VA coupling in patients with BAV and COA is not negatively affected compared to patients with isolated BAV. A reduction in the magnitude of the early systolic FCW was observed in patients who were older and with larger aortic diameters.

Echocardiographic assessment of the tricuspid and pulmonary valves: a practical guideline from the British Society of Echocardiography.

Transthoracic echocardiography is the first-line imaging modality in the assessment of right-sided valve disease. The principle objectives of the echocardiographic study are to determine the aetiology, mechanism and severity of valvular dysfunction, as well as consequences on right heart remodelling and estimations of pulmonary artery pressure. Echocardiographic data must be integrated with symptoms, to inform optimal timing and technique of interventions. The most common tricuspid valve abnormality is regurgitation secondary to annular dilatation in the context of atrial fibrillation or left-sided heart disease. Significant pulmonary valve disease is most commonly seen in congenital heart abnormalities. The aetiology and mechanism of tricuspid and pulmonary valve disease can usually be identified by 2D assessment of leaflet morphology and motion. Colour flow and spectral Doppler are required for assessment of severity, which must integrate data from multiple imaging planes and modalities. Transoesophageal echo is used when transthoracic data is incomplete, although the anterior position of the right heart means that transthoracic imaging is often superior. Three-dimensional echocardiography is a pivotal tool for accurate quantification of right ventricular volumes and regurgitant lesion severity, anatomical characterisation of valve morphology and remodelling pattern, and procedural guidance for catheter-based interventions. Exercise echocardiography may be used to elucidate symptom status and demonstrate functional reserve. Cardiac magnetic resonance and CT should be considered for complimentary data including right ventricular volume quantification, and precise cardiac and extracardiac anatomy. This British Society of Echocardiography guideline aims to give practical advice on the standardised acquisition and interpretation of echocardiographic data relating to the pulmonary and tricuspid valves.

A practical guideline for performing a comprehensive transthoracic echocardiogram in adults: the British Society of Echocardiography minimum dataset.

Since cardiac ultrasound was introduced into medical practice around the middle twentieth century, transthoracic echocardiography has developed to become a highly sophisticated and widely performed cardiac imaging modality in the diagnosis of heart disease. This evolution from an emerging technique with limited application, into a complex modality capable of detailed cardiac assessment has been driven by technological innovations that have both refined 'standard' 2D and Doppler imaging and led to the development of new diagnostic techniques. Accordingly, the adult transthoracic echocardiogram has evolved to become a comprehensive assessment of complex cardiac anatomy, function and haemodynamics. This guideline protocol from the British Society of Echocardiography aims to outline the minimum dataset required to confirm normal cardiac structure and function when performing a comprehensive standard adult echocardiogram and is structured according to the recommended sequence of acquisition. It is recommended that this structured approach to image acquisition and measurement protocol forms the basis of every standard adult transthoracic echocardiogram. However, when pathology is detected and further analysis becomes necessary, views and measurements in addition to the minimum dataset are required and should be taken with reference to the appropriate British Society of Echocardiography imaging protocol. It is anticipated that the recommendations made within this guideline will help standardise the local, regional and national practice of echocardiography, in addition to minimising the inter and intra-observer variation associated with echocardiographic measurement and interpretation.

Left heart matters in Ebstein's anomaly: the importance of a closer follow up-a case report.

BACKGROUND: Ebstein's anomaly (EA) is mainly thought of as a right heart condition, however, congenital left-sided lesions can co-exist. Therefore, it is paramount to include the left side of the heart as part of a routine investigation in these patients. We present a 57-year-old symptomatic patient with EA and progressive tricuspid regurgitation (TR) associated with acquired left ventricular outflow obstruction (LVOTO). CASE SUMMARY: A 57-year-old women, known to have severe EA presented with shortness of breath and chest pain on exertion secondary to progression of the tricuspid valve regurgitation and right ventricle dilatation leading to a dynamic compression of the left outflow tract requiring surgical intervention. DISCUSSION: Left ventricular obstruction secondary to severe TR and dilation of the right ventricle can present and remain silent at rest but becoming significant on exertion. Therefore, we recommend that all patients with EA and significant TR undergo exercise echocardiography at regular intervals to specifically look for acquired dynamic LVOTO.

Third time lucky: challenging secundum atrial septal defect.

Secundum atrial septal defect is the most common form of interatrial communication. Atrial septal defects can present in young adults with a variety of clinical presentations, including breathlessness on effort, palpitations, or stroke. Clinical heart failure and resting desaturation are both rarely seen in young patients. We present a case of a young man with a secundum atrial septal defect and a diagnosis of constrictive pericarditis, only made after two attempts at surgical correction of the atrial septal defect, with pericardiectomy at the third attempt and subsequent symptomatic improvement.

Echocardiographic Screening for Pulmonary Hypertension in Congenital Heart Disease: JACC Review Topic of the Week.

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

Early experience with the Occlutech PLD occluder for mitral paravalvar leak closure through a hybrid transapical approach.

AIMS: We sought to evaluate the feasibility and efficacy of hybrid transapical closure of paravalvar mitral leaks using a new Occlutech PLD occluder in patients with heart failure and/or haemolytic anaemia. METHODS AND RESULTS: Retrospective analysis of clinical and procedural data was undertaken for patients who had attempted closure of paravalvar mitral leaks via a hybrid transapical approach with the Occlutech PLD occluder. Eight patients (four males, median age 69 years) underwent closure of 10 mitral paravalvar leaks using eight Occlutech PLD occluders and two AMPLATZER Vascular Plugs (AVP II). Successful deployment, with significant reduction of the paravalvar leak was achieved in seven patients with short procedure (median 131 min) and fluoroscopy times (median 22 min). One patient had mechanical interference with prosthetic valve function, requiring surgery. Another patient with a high EuroSCORE (48.8%) died of multi-organ failure two days after the procedure. Clinical improvement in either heart failure or haemolysis was seen in all discharged patients. CONCLUSIONS: In our series of patients with challenging anatomy, the Occlutech PLD occluders performed well when implanted via a hybrid transapical approach. Further work is needed to assess this methodology fully for a wider population and to assess other deployment approaches for this promising new occluder.

Self-expanding stent implantation in arterial duct during hybrid palliation of hypoplastic left heart syndrome: midterm experience with a specially designed stent.

AIMS: The lack of a specifically designed stent for the arterial duct has led to the off-label use of stents during hybrid palliation of hypoplastic left heart syndrome (HLHS). We evaluated the implantation and performance of a specially designed self-expanding stent in hybrid palliation of HLHS. METHODS AND RESULTS: We implanted 39 sinus-SuperFlex (SSF) stents in 24 patients at a median age of seven days (range 2-27 days) and median weight of 2.85 kg (range 1.3-3.8 kg). A single stent was implanted in nine patients and two overlapping stents in 15 patients. There was one intraoperative death, not related to ductal stenting and one additional in-hospital death. During median follow-up of 137 days (range 38-522 days), nine patients required 11 interstage interventions. Four patients have undergone a biventricular repair, 11 have undergone the next stage of univentricular palliation and seven are awaiting a second-stage palliation. CONCLUSIONS: The SSF stent provides effective maintenance of ductal patency in patients undergoing hybrid palliation of HLHS and its variants. It conforms to the ductal anatomy but the lack of stents longer than 20 mm has required overlapping stents in longer ducts. This has not been of haemodynamic consequence.

Acceptance and psychological impact of implantable defibrillators amongst adults with congenital heart disease.

BACKGROUND: The psychological impact of implantable cardioverter defibrillators (ICDs) in adults with congenital heart disease (ACHD) has not been established. OBJECTIVE: To compare device acceptance, quality of life, anxiety and depression between ACHD patients with ICDs (ICD-Congenital), with pacemakers (PPM-Congenital), with no devices (No Device-Congenital) and non-ACHD patients with ICDs (ICD-Non-Congenital). METHODS: A total of 147 ACHD and 46 non-ACHD patients (age 45.0±14.7 years, 56.5% males) completed the Florida Patient Acceptance Survey (FPAS), the 36-item Short Form Health Survey (SF-36) and Hospital Anxiety & Depression Scale (HADS). RESULTS: ICD-Congenital patients (n=59) showed lower device acceptance compared to PPM-Congenital patients (n=41), p=0.04, and reported worse quality of life (p=0.001) and higher prevalence of depression (p=0.009) when compared to No Device-Congenital (n=47) patients. ICD-Congenital and ICD-Non-Congenital patients (n=46) showed similar mental and physical health, device acceptance, anxiety and depression. Within ICD-Congenital, patients with poorest device acceptance (FPAS <67, "Non-Acceptors") showed significantly lower mental health scores (p=0.008), and higher levels of anxiety (p=0.02) and depression (p=0.01) compared to "Acceptors" (FPAS ≥67). "Non-Acceptors" were younger at survey (p=0.006), younger at ICD implantation (p=0.01) and were less likely to have received appropriate shocks (p=0.03). CONCLUSION: Younger age and lack of appropriate ICD shocks are risk factors for poor ICD acceptance. Device acceptance is lower in adults with congenital heart disease who receive an ICD than those who receive pacemakers. Appropriate screening for anxiety and depression may be warranted for ACHD patients considered for ICD implantation or already living with ICDs.