BACKGROUND:Pulmonary regurgitation is common during follow-up of patients after surgical repair of tetralogy of Fallot and other right ventricular outflow tracts (RVOTs). Many percutaneous pulmonary valves have been used but are limited to smaller RVOTs. OBJECTIVES:Since August 2016, a multicenter CE (Conformité Européenne) study was initiated to evaluate a self-expandable VenusP-valve. We aimed to report the acute and 3-year follow-up results. METHODS:A total of 81 patients with pulmonary regurgitation were recruited for VenusP-valve implantation and assessed for a 3-year period. RESULTS:In all patients, VenusP-valves were successfully implanted. The mean age was 26.5 ± 13.3 years and the mean weight 59.5 ± 15.6 kg. There was no early procedure-related or late mortality. One patient experienced guidewire perforation of a branch pulmonary artery, causing hemoptysis, and 1 had ventricular tachycardia, at the end of the procedure. During follow-up, 1 patient developed runs of ventricular tachycardia and needed an implantable cardioverter-defibrillator and ablation of the RVOT 5 months after valve implantation. One developed endocarditis 11 months after implantation. After medical treatment, the valve has continued to function normally. One patient developed thrombus on the distal flare 3 years after implantation and was treated with anticoagulants. During 3-year follow-up, valve function has remained satisfactory and right ventricular remodeling has occurred in all patients. CONCLUSIONS:We report the 3-year CE study results of percutaneous pulmonary valve implantation in patients with severe pulmonary regurgitation. The valve has shown promising safety and durability. Long-term evaluation is warranted.

BACKGROUND:Coronary artery fistulas (CAFs) are rare anomalies that can cause ischemia, arrhythmias, and sudden cardiac death. Conventional closure techniques often fail in anatomically complex cases. SUMMARY:We present the first-in-human use of a customized, partially covered G-Armor pulmonary artery (PA) stent to manage a left main-to-right pulmonary artery fistula in a patient with Shone complex. The intervention was planned using a three-dimensionally printed model, allowing precise stent customization to avoid obstruction of key pulmonary branches. DISCUSSION:This case shows the feasibility of using customized endovascular devices to address complex CAFs in high-risk patients. The intervention improved antegrade coronary perfusion and clinical outcomes. NOVELTY:To the best of our knowledge, this is the first reported case of a customized partially covered PA stent for CAF management. TAKE-HOME MESSAGE:In anatomically complex and high-risk CAFs, customized stenting offers a viable solution when conventional therapies fail.

BACKGROUND:Covered stent correction for a sinus venosus atrial septal defect (SVASD) was first performed in 2009. This innovative approach was initially viewed as experimental and was reserved for highly selected patients with unusual anatomic variants. In 2016, increasing numbers of procedures began to be performed, and in several centers, it is now offered as a standard of care option alongside surgical repair. However, covered stent correction for SVASD is not recognized by regulatory authorities, and in the minds of many pediatric and adult congenital cardiologists and surgeons, the condition is viewed as treatable only by cardiac surgery with cardiopulmonary bypass. METHODS:In April 2023, all centers identified from international conferences, publications, and colleague networks to be undertaking covered stent correction for SVASD were invited to participate in a retrospective audit of their procedures. RESULTS:Data were received on 381 patients from 54 units over a 12-year period with 90% of procedures being performed over the past 5 years. Balloon-expandable stents (8 types) were used in the majority; self-expanding stents (4 types) were used in 4.5%. The commonest stent was the 10-zig covered Cheatham Platinum stent in 62% of cases. In 10 procedures, the stent embolized requiring surgical retrieval and repair of the defect, resulting in technically successful implantation in 371 of 381 (97.4%). Major complications (surgical drainage of tamponade, pacemaker implantation, surgery for pulmonary vein occlusion, and late stent removal) occurred in 5 patients (1.3%). Repeat catheterization to correct residual leaks was required in 7 patients (1.8%). Thus, 359 of 381 patients (94.2%) had successful correction without major complications or additional catheter interventions. CONCLUSIONS:This article details the exponential uptake of covered stent correction for SVASD during the past 5 years. Cardiopulmonary bypass was avoided in the majority of patients, and major complications were infrequent. Prospective registries with standardized definitions, inclusion criteria, and follow-up and comparative studies with surgery are now required to help support the extension of covered stent correction as an alternative standard-of-care option for patients with an SVASD.

This document has been developed as an expert consensus document by the Pediatric and Congenital Interventional Cardiovascular Society (PICS), the Association for European Paediatric and Congenital Cardiology (AEPC), the Asia-Pacific Pediatric Cardiac Society (APPCS), the Cardiac Society of Australia and New Zealand (CSANZ), Society for Cardiovascular Angiography & Interventions (SCAI), and the Latin American Society of Interventional Cardiology (SOLACI), with additional endorsement from the Congenital Cardiac Anesthesia Society (CCAS) and the American Association of Physicists in Medicine (AAPM, Sections 7 and 11).

The GORE CARDIOFORM atrial septal defect (ASD) Occluder (GCA) is composed of a platinum-filled nitinol wire frame covered with expanded polytetrafluoroethylene, making it softer and more conformable compared with nitinol mesh devices. After the ASSURED clinical study confirmed the efficacy and safety of the device, it received U.S. Food and Drug Administration approval and a European conformity mark. Our aim was to understand the learning curve implicated in using the GCA for ASD closure in paediatric and adult patients as well as to study the early outcomes. To this end, a review of ASD device closures with GCA in 4 UK centres was conducted between January 2020 and January 2023. Implantation success was the primary outcome; the secondary outcomes were serious adverse events, including new onset arrhythmia. In all, 135 patients were included, and 128 (95%) had successful ASD device closure with GCA. The median patient age was 49 years, the median defect size was 18 mm, and the median device size was 37 mm. The median follow-up time was 6 months (interquartile range 1-14). One device embolisation occurred, and 15 patients (12% of GCA implantations) developed new onset arrhythmia - this was not related to patient age, defect diameter or device oversizing but was positively associated with device size. With growing experience using GCA, the device can be applied to a wide variety of ASD sizes and morphologies. Given the number of successful implantations with an absence of aortic erosion, as well as the ability to perforate through the device should procedures be required in the left atrium, the GCA device is an important addition for interventionists who close atrial septal defects.

To reflect new developments in imaging, indications, and techniques in catheterisation of patients with CHD, the training recommendations of the Association for European Paediatric and Congenital Cardiology (AEPC) for the training in interventional catheterisation for CHD have been reviewed and updated after more than 7 years. They include detailed information about knowledge, skills, and approach to clinical practice expected from trainees at basic, intermediate, and advanced level.

Atrial fibrillation (AF) is common in adults with unrepaired atrial septal defects (ASDs). Sinus venosus (SV) ASDs associated with partial anomalous pulmonary venous return (PAPVR) are traditionally managed surgically. We report the first AF catheter ablation in a patient with SV ASD and PAPVR preceding transcatheter ASD repair with a covered stent. (Level of Difficulty: Advanced.)

Infants with complex cyanotic CHD can become symptomatic from insufficient pulmonary blood supply following either ductal closure or due to outflow tract obstruction. Blalock-Taussig shunt mortality remains significant and recent studies have highlighted the advantages of using transcatheter alternatives. We present here our experience in changing our primary choice of palliation from the Blalock-Taussig shunt to transcatheter palliation with either a ductal stent or, if antegrade flow is present, a right ventricular outflow tract stent.This is a retrospective, single-unit cohort study. Eighty-seven infants underwent palliation for insufficient pulmonary blood flow at under 3 months of age between 2012 and 2019. On an intention-to-treat basis, 29 underwent insertion of a Blalock-Taussig shunt, 36 duct stents, and 22 right ventricular outflow tract stents at median ages of 15, 9, and 32 days, respectively, and median weights of 3.3, 3.1, and 3.1 kg, respectively. No primary Blalock-Taussig shunts have been performed in our institution since 2017.At 30-days there had been one death in each group (univariable p = 0.93) and deaths prior to repair totalled three in the shunt group, four in the ductal stent group, and two in the right ventricular outflow tract stent group (univariable p = 0.93). Reintervention on the pulmonary circuit prior to next stage of surgery was more frequent in those undergoing transcatheter intervention, reaching statistical significance by logrank (p = 0.012).In conclusion, within this work we provide further evidence of the safety and efficacy of transition from a primary surgical to primary transcatheter palliation pathway in infants with insufficient pulmonary blood supply.

OBJECTIVES:Since percutaneous pulmonary valve implantation (PPVI) was introduced to prolong the lifetime of surgically placed right ventricular to pulmonary artery conduits, valve technology has evolved and the indications for PPVI expanded to native and larger right ventricular outflow tracts. We explore how indications, patient populations and outcomes compare to surgical pulmonary valve replacement (PVR).METHODS:This is a retrospective cohort study of PPVI and PVR procedures between 1998 and 2020 at a single UK centre. One hundred and twenty-eight patients underwent PPVI and 365 patients PVR. Primary outcome measures were survival, infective endocarditis and reintervention.RESULTS:The most common indication for PVR was replacement of the native pulmonary valve for pulmonary regurgitation whereas PPVI was more commonly used to treat pulmonary stenosis in a previously placed bioprosthetic conduit or valve. Treatment indications for PPVI expanded over the study to include the native right ventricular outflow tract. Survival was similar for PPVI and PVR (92% PPVI and 96.8% PVR at 5 years; 85.8% PPVI and 95.1% PVR at 10 years). Preprocedural New York Heart Association class 3 and 4 was the most important predictor of poor outcome. Annualized infective endocarditis rate was significantly higher for the Melody PPVI (0.024 vs 0.0024/person/year for PVR, P < 0.05). Both groups showed significant symptomatic improvement postprocedure with remodelling of ventricular volumes and improvement in cardiac output. Long-term follow-up for PVR showed half of patients will need replacement at 10-15 years post-index procedure.CONCLUSIONS:An increasing number of patients requiring PVR can now be treated percutaneously. A lifetime strategy for re-valving should be considered at the first valve implant.

Objective To bring together patients, parents, charities and clinicians in a Priority Setting Partnership to establish national clinical priorities for research in children and adults with congenital heart disease. Methods The established James Lind Alliance methodology was used to identify and prioritise research on the management of congenital heart disease, focusing on diagnosis, treatment and outcomes. An initial open survey was used to gather potential uncertainties which were filtered, categorised, converted into summary questions and checked against current evidence. In a second survey, respondents identified the unanswered questions most important to them. At two final workshops, patients, parents, charities and healthcare professionals agreed the top 10 lists of priorities for child/antenatal and adult congenital heart disease research. Results 524 respondents submitted 1373 individual questions, from which 313 out of scope or duplicate questions were removed. The remaining 1060 questions were distilled into summary questions and checked against existing literature, with only three questions deemed entirely answered and removed. 250 respondents completed the child/antenatal survey (56 uncertainties) and 252 completed the adult survey (47 uncertainties). The questions ranked the highest by clinicians and non-clinicians were taken forward to consensus workshops, where two sets of top 10 research priorities were agreed. Conclusions Through an established and equitable process, we determined national clinical priorities for congenital heart disease research. These will be taken forward by specific working groups, a national patient and public involvement group, and through the establishment of a UK and Ireland network for collaborative, multicentre clinical trials in congenital heart disease.

AIMS:Post-infarction ventricular septal defect (PIVSD) is a mechanical complication of acute myocardial infarction (AMI) with a poor prognosis. Surgical repair is the mainstay of treatment, although percutaneous closure is increasingly undertaken.METHODS AND RESUTS:Patients treated with surgical or percutaneous repair of PIVSD (2010-2021) were identified at 16 UK centres. Case note review was undertaken. The primary outcome was long-term mortality. Patient groups were allocated based upon initial management (percutaneous or surgical). Three-hundred sixty-two patients received 416 procedures (131 percutaneous, 231 surgery). 16.1% of percutaneous patients subsequently had surgery. 7.8% of surgical patients subsequently had percutaneous treatment. Times from AMI to treatment were similar [percutaneous 9 (6-14) vs. surgical 9 (4-22) days, P = 0.18]. Surgical patients were more likely to have cardiogenic shock (62.8% vs. 51.9%, P = 0.044). Percutaneous patients were substantially older [72 (64-77) vs. 67 (61-73) years, P < 0.001] and more likely to be discussed in a heart team setting. There was no difference in long-term mortality between patients (61.1% vs. 53.7%, P = 0.17). In-hospital mortality was lower in the surgical group (55.0% vs. 44.2%, P = 0.048) with no difference in mortality after hospital discharge (P = 0.65). Cardiogenic shock [adjusted hazard ratio (aHR) 1.97 (95% confidence interval 1.37-2.84), P < 0.001), percutaneous approach [aHR 1.44 (1.01-2.05), P = 0.042], and number of vessels with coronary artery disease [aHR 1.22 (1.01-1.47), P = 0.043] were independently associated with long-term mortality.CONCLUSION:Surgical and percutaneous repair are viable options for management of PIVSD. There was no difference in post-discharge long-term mortality between patients, although in-hospital mortality was lower for surgery.

We present five cases of sinus-SuperFlex-DS stent stenosis during early follow up that resulted in inadequate ductal patency and required urgent re-stenting with a balloon-expandable stent. This causes concern that these stents lack sufficient radial force against ductal constriction and if used need to be kept under close scrutiny.

We are pleased that our short article highlighting concerns over Sinus-SuperFlex-DS stent deformation occurring after deployment in the patent ductus as part of the Hybrid procedure has attracted controversy. Professor Schranz is correct to point out that the hybrid procedure is used by a handful of institutions around the world as first line therapy for hypoplastic left heart syndrome (HLHS) but most institutions have not followed this route reserving the hybrid procedure for cases too unwell to undergo a primary Norwood or abandoning the hybrid procedure altogether. We recently conducted an analysis of the Hybrid procedure undertaken in at risk infants in four centers across the UK and compared this with outcomes for those infants undergoing primary Norwood. Interestingly, we found that survival was not different despite there being marked differences in the two groups at baseline. We therefore agree with the author that Hybrid continues to have an important place in the management of HLHS, at least in the very sickest of infants. We read with interest Professor Schranz's comments in which he points out that, rather than being novel our case series simply adds to the growing evidence of stent compression following the use of the Sinus super-flex ductal stent. Far from offering guidelines for practice, we simply intended to highlight to readers of the journal one of the potential complications that can occur, when stenting the duct using the only specifically CE marked device. Any complication profile has, of course to be weighed up against the advantages of a piece of equipment and in the case of the superflex stent there are many-in particular its ease of use and low delivery profile. Interventionists are well adept at balancing advantages and disadvantages of equipment if they are aware of them. Very few implanting centers can rival Giessen for length and breadth of experience in ductal stenting. We are therefore surprised that Professor Schranz takes issue with our advice to keep these stents under close scrutiny. It would seem very prudent, if not essential, for units performing Hybrid to adopt careful echocardiographic screening protocols that monitor for signs of such an eventuality. This we have done and we stand by reporting it to the wider interventional community for their benefit. There is insufficient information in such a brief report to make critical comments about duct morphology, stent position, and stent choice and we will not endeavor to respond to these remarks. The true measure of any piece of equipment or indeed procedure is not necessarily performance in the hands of the highest volume “experts” but how it translates to the “everyday” and in this regard whilst we accept some of the criticisms of our “imperfect” practice the truth is this is real world use of the stent, within a large volume program in the only environment the stent was intended for. We do not believe that a structural issue of this sort can be fully ameliorated by the steps Professor Schranz offers such as an altered prostaglandin strategy or the use of one cyclooxygenase inhibitor or another. As a final comment, as all experienced interventionalists know, there can never be a completely “right way” to do any procedure, and whilst we have all learned enormously from the eponymous “Giessen” or “Columbus” approaches, the vulnerable neonate with an arterial duct dependent circulation remains an extreme challenge and one for which we hope that technical lessons will continue to be learned and shared (no matter how apparently foolish) amongst colleagues long after the baton has been handed on to future generations.

HomeCirculationVol. 144, No. 14Hybrid Palliation for Hypoplastic Left Heart Syndrome: Association With Contemporary Outcomes Free AccessLetterPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyRedditDiggEmail Jump toFree AccessLetterPDF/EPUBHybrid Palliation for Hypoplastic Left Heart Syndrome: Association With Contemporary Outcomes Andrew B. Ho, MD, Ines Hribernik, MD, Daniel Shillaker, John Thomson, MD, Abdul Salam, MD, Nathalie Dedieu, MD, Alessandro Giardini, MD, Graham Derrick, MD, Barry O'Callaghan, MD, Jack Gibb, MD, Francisco Gonzalez-Barlatay, MD, Demitris Taliotis, MD, Melonie Johns, MD, Nicholas Hayes, MD and James R. Bentham, MD, PhD Andrew B. HoAndrew B. Ho https://orcid.org/0000-0001-9410-4609 Paediatric Cardiology, Southampton General Hospital, United Kingdom (A.B.H., M.J., N.H.). Department of Congenital Cardiology, Leeds General Infirmary, United Kingdom (A.B.H., I.H., J.T., J.R.B.). , Ines HribernikInes Hribernik Department of Congenital Cardiology, Leeds General Infirmary, United Kingdom (A.B.H., I.H., J.T., J.R.B.). , Daniel ShillakerDaniel Shillaker Sheffield Medical School, United Kingdom (D.S.). , John ThomsonJohn Thomson Department of Congenital Cardiology, Leeds General Infirmary, United Kingdom (A.B.H., I.H., J.T., J.R.B.). , Abdul SalamAbdul Salam Paediatric Cardiology, Great Ormond Street Hospital, London, United Kingdom (A.S., N.D., A.G., G.D.). , Nathalie DedieuNathalie Dedieu Paediatric Cardiology, Great Ormond Street Hospital, London, United Kingdom (A.S., N.D., A.G., G.D.). , Alessandro GiardiniAlessandro Giardini Paediatric Cardiology, Great Ormond Street Hospital, London, United Kingdom (A.S., N.D., A.G., G.D.). , Graham DerrickGraham Derrick Paediatric Cardiology, Great Ormond Street Hospital, London, United Kingdom (A.S., N.D., A.G., G.D.). , Barry O'CallaghanBarry O'Callaghan Paediatric Cardiology, Bristol Royal Hospital for Children, United Kingdom (B.O'C., J.G., F.G.-B., D.T.). , Jack GibbJack Gibb https://orcid.org/0000-0002-2982-0649 Paediatric Cardiology, Bristol Royal Hospital for Children, United Kingdom (B.O'C., J.G., F.G.-B., D.T.). , Francisco Gonzalez-BarlatayFrancisco Gonzalez-Barlatay Paediatric Cardiology, Bristol Royal Hospital for Children, United Kingdom (B.O'C., J.G., F.G.-B., D.T.). , Demitris TaliotisDemitris Taliotis Paediatric Cardiology, Bristol Royal Hospital for Children, United Kingdom (B.O'C., J.G., F.G.-B., D.T.). , Melonie JohnsMelonie Johns Paediatric Cardiology, Southampton General Hospital, United Kingdom (A.B.H., M.J., N.H.). , Nicholas HayesNicholas Hayes Paediatric Cardiology, Southampton General Hospital, United Kingdom (A.B.H., M.J., N.H.). and James R. BenthamJames R. Bentham Correspondence to: James R. Bentham, MD, PhD, Yorkshire Heart Centre, Leeds General Infirmary, Great George St, Leeds LS1 3EX, United Kingdom. Email E-mail Address: [email protected] https://orcid.org/0000-0003-0559-1316 Department of Congenital Cardiology, Leeds General Infirmary, United Kingdom (A.B.H., I.H., J.T., J.R.B.). Originally published4 Oct 2021https://doi.org/10.1161/CIRCULATIONAHA.121.055183Circulation. 2021;144:1189–1191The Norwood procedure is the palliation preferred by most cardiac programs for patients with hypoplastic left heart syndrome (HLHS) and anatomic variants.1,2 Although improvements in long-term outcomes have been observed for these patients, mortality continues to be among the highest of all neonatal surgical procedures.2 Focusing on improved survival, different surgical strategies have been pursued.3–5 One example is the hybrid procedure. First described in 1993 by one of the study centers, it involves placement of bilateral pulmonary artery bands and stenting of the arterial duct and allows for growth or stabilization before the next-stage surgery.3 Early improvement in survival with a hybrid approach is tempered by perception of higher cumulative mortality and morbidity, such that few centers choose hybrid as a primary HLHS strategy. Some centers, including those in this study, perform hybrid palliations only in patients with significant risk factors, such as prematurity, low birth weight, and noncardiac comorbidity. Subsequent surgical options include a standard Norwood, combined first- and second-stage Norwood, cardiac transplantation, biventricular repair, or reorientation to comfort care.5This study compares the outcomes of high-risk infants following an initial hybrid procedure with those undergoing a Norwood procedure in a multicenter prospectively collected contemporary series.From 4 large pediatric cardiology centers in the United Kingdom, the cases comprised all patients with HLHS physiology undergoing hybrid palliation between January 2013 and January 2020 for 3 units and to January 2019 for a fourth, with follow-up to January 2021. The control group consisted of patients undergoing a Norwood procedure at <30 days of age and operated on in the same time period. All centers contribute to a mandatory validated prospective audit of cardiac outcomes with patient informed consent, and contributing centers searched their databases to identify all cases that met these inclusion criteria. Institutional review board approval was given for the study. The data supporting the findings from this study are available on request from the corresponding author.The primary outcome was survival to bidirectional cavopulmonary anastomosis (Norwood stage 2) or complete biventricular repair. Secondary outcomes were overall survival, need for reintervention, and branch pulmonary artery sizes. Post hoc, we performed calculation of a modified Aristotle score to better describe the differing risk profiles between the groups as previously described.5One hundred forty neonates underwent a primary Norwood procedure (median age, 5 days [interquartile range, 4–8 days]; weight, 3.28 kg [interquartile range, 2.9–3.6 kg]). Forty-six neonates underwent a hybrid procedure (median age, 7 days [interquartile range, 4–12 days]; weight, 2.5 kg [interquartile range, 2.3–3.0 kg]). Baseline demographics differed significantly between the 2 groups in index weight, prematurity (odds ratio, 27 [95% CI, 5.8–126]), preprocedural necrotizing enterocolitis (odds ratio, 17 [95% CI, 1.9–149]), preprocedural ventilation (odds ratio, 2.7 [95% CI, 1.3–5.8]), and the presence of other comorbidities (odds ratio, 3.1 [95% CI, 1.0–96]). The modified Aristotle score was 0.6 in the Norwood group and 2.9 in the hybrid group (P<0.001).5Of 46 hybrids, 19 comprised placement of pulmonary artery bands only, and all others (27) used a hybrid approach for ductal stent placement through the main pulmonary artery. Two hybrids (4.3%) required extracorporeal membrane oxygenation after the procedure. Twenty-four underwent reintervention before the next-stage surgery (52%), with the majority being atrial septal procedures. One patient in the group undergoing a combined second stage required extracorporeal membrane oxygenation in the postoperative period following next-stage surgery (10%). Of 140 primary Norwood procedures, 18 (13%) required extracorporeal membrane oxygenation postoperatively and 51 required interstage reintervention (36%).The primary outcome of survival to Glenn shunt or complete repair, and overall mortality by Kaplan-Meier with a median follow-up of 2 years, as well, is shown in the Figure. There was no difference in survival to stage 2 by the initial approach by univariable analysis (P=0.59). There was no statistically significant difference in overall survival between hybrid and primary Norwood groups by log-rank (P=0.5). Redefining 3 patients in both groups with an ongoing requirement for an arterial shunt as not having achieved Glenn circulations, there remains no difference in survival to Glenn between primary Norwood and hybrid procedures.Download figureDownload PowerPointFigure. Comparison of hybrid palliation for hypoplastic left heart syndrome with a primary Norwood approach in 186 infants. A, Summary flow diagram of operative outcomes. B, Kaplan-Meier of mortality by group. P for difference by log-rank=0.5. C, Primary outcomes for both hybrid and primary Norwood groups was mortality at 30 days, interstage (pre-Glenn), following Glenn/complete repair, and at 1 year.Following achievement of Glenn or biventricular physiology, reintervention before discharge was more common (P<0.001) in the hybrid group. There was no statistically significant difference between operative times of equivalent procedures between groups. There was no difference in the average Nakata index (P=0.78) between the hybrid (181 [158–204]) and the Norwood groups (273 [123–224]). Branch pulmonary artery repair was performed more frequently after a hybrid procedure (P<0.025).The hybrid group began with significantly more risk factors for an adverse outcome. Despite this, we demonstrate no difference in survival to next-stage surgery. The strength of the present study in this context lies in supporting the conclusion that despite being a more adverse group, we see no difference in medium-term outcomes. Reintervention between all stages is extremely well-recognized as one of the costs of this type of palliation.Hybrid palliation in the highest-risk infants can be used with similar survival as with an initial Norwood approach in standard-risk patients.Nonstandard Abbreviations and AcronymsHLHShypoplastic left heart syndromeSources of FundingNone.Disclosures None.Footnoteshttps://www.ahajournals.org/journal/circFor Sources of Funding and Disclosures, see page 1191.Correspondence to: James R. Bentham, MD, PhD, Yorkshire Heart Centre, Leeds General Infirmary, Great George St, Leeds LS1 3EX, United Kingdom. Email Jamie.[email protected]netReferences1. Ohye RG, Schranz D, D'Udekem Y. Current therapy for hypoplastic left heart syndrome and related single ventricle lesions.Circulation. 2016; 134:1265–1279. doi: 10.1161/CIRCULATIONAHA.116.022816LinkGoogle Scholar2. Newburger JW, Sleeper LA, Gaynor JW, Hollenbeck-Pringle D, Frommelt PC, Li JS, Mahle WT, Williams IA, Atz AM, Burns KM, et al.; Pediatric Heart Network Investigators. Transplant-free survival and interventions at 6 years in the SVR Trial.Circulation. 2018; 137:2246–2253. doi: 10.1161/CIRCULATIONAHA.117.029375LinkGoogle Scholar3. Gibbs JL, Wren C, Watterson KG, Hunter S, Hamilton JR. Stenting of the arterial duct combined with banding of the pulmonary arteries and atrial septectomy or septostomy: a new approach to palliation for the hypoplastic left heart syndrome.Br Heart J. 1993; 69:551–555. doi: 10.1136/hrt.69.6.551CrossrefMedlineGoogle Scholar4. Galantowicz M, Yates AR. Improved outcomes with the comprehensive stage 2 procedure after an initial hybrid stage 1.J Thorac Cardiovasc Surg. 2016; 151:424–429. doi: 10.1016/j.jtcvs.2015.10.023CrossrefMedlineGoogle Scholar5. Lloyd DF, Cutler L, Tibby SM, Vimalesvaran S, Qureshi SA, Rosenthal E, Anderson D, Austin C, Bellsham-Revell H, Krasemann T. Analysis of preoperative condition and interstage mortality in Norwood and hybrid procedures for hypoplastic left heart syndrome using the Aristotle scoring system.Heart. 2014; 100:775–780. doi: 10.1136/heartjnl-2013-304759CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetails October 5, 2021Vol 144, Issue 14Article InformationMetrics Download: 214 © 2021 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.121.055183PMID: 34606304 Originally publishedOctober 4, 2021 KeywordsFontan procedurehypoplastic left heart syndromeNorwood procedurespulmonary arterypulmonary arteryPDF download SubjectsCardiovascular SurgeryQuality and Outcomes

Background Covered stent correction of sinus venosus ASDs (SVASD) is a relatively new technique. Challenges include anchoring a sufficiently long stent in a nonstenotic superior vena cava (SVC) and expanding the stent at the wider SVC-RA junction without obstructing the anomalous right upper pulmonary vein (RUPV). The 10-zig covered Cheatham-platinum (CCP) stent has the advantage of being available in lengths of 5-11 cm and dilatable to 34 mm in diameter. Methods An international registry reviewed the outcomes of 10-zig CCP stents in 75 patients aged 11.4-75.9 years (median 45.4) from March 2016. Additional stents were used to anchor the stent in the SVC or close residual shunts in 33/75. An additional stent was placed in 4/5 (80%) with 5/5.5 cm CCPs, 18/29 (62%) with 6 cm CCPs, 5/18 (28%) with 7 cm CCPs, 5/22 (23%) with 7.5/8 cm CCPs and 0/1 with an 11 cm CCP. A "protective" balloon catheter was inflated in the RUPV in 17. Results Early stent embolization in two patients required surgical removal and defect repair and tamponade was drained in one patient. The CT at 3 months showed occlusion of the RUPV in one patient. Follow up is from 2 months to 5.1 years (median 1.8 years). QP:QS has reduced from 2.5 +/- 0.5 to 1.2 +/- 0.36 (p < .001) and RVEDVi from 149.1 +/- 35.4 to 95.6 +/- 21.43 ml/m2 (p < .001). Conclusions Ten-zig CCPs of 7-8 cm appear to provide reliable SVASD closure with a low requirement for additional stents. Careful selection of patients and meticulous attention to detail is required to avoid complications.

Patent foramen ovale (PFO) is implicated in the pathogenesis of a number of medical conditions but to date only one official position paper related to left circulation thromboembolism has been published. This interdisciplinary paper, prepared with the involvement of eight European scientific societies, reviews the available evidence and proposes a rationale for decision making for other PFO-related clinical conditions. In order to guarantee a strict evidence-based process, we used a modified grading of recommendations, assessment, development, and evaluation (GRADE) methodology. A critical qualitative and quantitative evaluation of diagnostic and therapeutic procedures was performed, including assessment of the risk/benefit ratio. The level of evidence and the strength of the position statements were weighed and graded according to predefined scales. Despite being based on limited and observational or low-certainty randomised data, a number of position statements were made to frame PFO management in different clinical settings, along with suggestions for new research avenues. This interdisciplinary position paper, recognising the low or very low certainty of existing evidence, provides the first approach to several PFO-related clinical scenarios beyond left circulation thromboembolism and strongly stresses the need for fresh high-quality evidence on these topics.