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.

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

There has been marked innovation and progress in the techniques, technologies and outcomes in the field of paediatric and congenital cardiac catheter intervention over the past 4 decades. In the modern era, it is now possible to treat many forms of congenital heart disease through minimally invasive percutaneous therapies, with associated reduction in morbidity for this group of patients. This article provides an overview of current practice and developments in this field, with particular focus on the interventional procedures commonly performed in the paediatric population.

Although often used as a diagnosis in its own right, double outlet right ventricle is more accurately merely a description of the ventriculo-arterial connection in a congenitally malformed heart, and use of the term must always be allied with further description of the anatomy. In this chapter we describe a term infant born at 3.4 kg following an antenatal diagnosis of double outlet right ventricle (DORV) with noncommitted ventricular septal defect (VSD), side-by-side great arteries and pulmonary stenosis. There was a duct-dependent pulmonary circulation in the neonatal period, and the infant underwent placement of a Blalock-Taussig shunt. Following multi-modality cardiac imaging, he underwent to complete surgical repair at 16 months of age, with VSD enlargement, tunnelled closure of the VSD to commit the left ventricle to the aorta, and augmentation of the pulmonary outflow tract. Through the chapter, we emphasise the importance of a detailed anatomical description and thorough investigation in this family of defects, in order to allow an understanding of the physiology, clinical behaviour and the management options available for individual patients. Learning points in the chapter include discussion regarding the definitions of DORV, detailed descriptions of the major anatomical groups and a synopsis of the more common surgical options.

Background Disconnected branch pulmonary arteries with a systemic arterial origin of the disconnected vessel is a rare, but well-described entity. Most will have ductal tissue connecting the pulmonary artery to the aorta. Case summary We describe in this paper the haemodynamic result in three neonates presenting with ductal origin of a single branch pulmonary artery in the context of trans-catheter stenting procedures to maintain or re-recruit vessel patency. All were faced with potential or actual ductal closure and proceeded to trans-catheter stenting to re-cannalate the duct-dependent pulmonary artery. Two patients with otherwise normal anatomy struggled post-procedure with pulmonary hypertension and right ventricular dilatation. Both required surgical re-anastomosis of the disconnected pulmonary artery during the same admission—one 26 days post-stenting following failure to wean from high-flow respiratory support and the second 8 days post-stenting following failed extubation. In contrast, a patient with tetralogy of Fallot born at 2.5 kg underwent sequential stenting of the right ventricular outflow tract and then the left-sided ductus. He had a good post-procedural course and thrived for several months before complete repair. Discussion We describe the clinical courses and discuss the resultant haemodynamics, highlighting the importance of flow to each lung, the resulting haemodynamic implications and the compounding effects of additional lesions.

Tricuspid regurgitation in the context of hypoplastic left heart syndrome (HLHS) carries a significant burden of both mortality and morbidity. We report successful hybrid trans-atrial implantation of an Edwards Sapien XT valve into a dysfunctional bioprosthetic valve in the tricuspid position of a 9-year-old patient with HLHS. There was an immediate fall in the Fontan pressures combined with significant clinical improvement and the valve continues to function well nearly 3 years post implant. To the best of our knowledge, this is the first described implantation of a transcatheter valve in this specific position and anatomy.

Background Classical management of small infants with tetralogy of Fallot has involved placement of a Blalock-Taussig shunt followed later by complete repair, rather than primary complete repair which is the strategy adopted in larger infants. Some advantages of early complete repair compared to a staged strategy have been shown. We sought to review our institutional outcomes. Methods Patients under 3-months old undergoing complete surgical repair of tetralogy of Fallot in our institution between 2005 and 2015 were retrospectively reviewed and compared with an older control group matched by anatomical diagnosis and outflow tract intervention. Results Fourteen index cases (group A) and 14 controls (group B) were identified. At surgery, the median age was 43 days and weight 4.2 kg in group A, and 130 days and 6.1 kg in group B. Nine of 14 in group A were admitted for surgery as emergencies compared to none in group B. Peak inotrope score (22.3 vs. 12.8, p = 0.02) and intensive care unit stay (4.4 vs. 2.6 days, p = 0.02) were higher in group A. Bypass and crossclamp times, duration of intubation, and total length of stay did not differ. Conclusions We conclude that although babies undergoing early repair of tetralogy of Fallot have an increased need for intensive care support in the early postoperative period, the total length of stay is not prolonged despite more emergency admissions. As it is known that early repair may reduce long-term morbidity, we propose consideration of earlier complete repair of tetralogy of Fallot.

Here, we report a four-day old neonate presenting with cardiovascular collapse secondary to a descending aorta to right atrial fistula. Echocardiography was suggestive of the diagnosis and confirmed with computed tomographic (CT) imaging. The fistula was successfully occluded via cardiac catheterization using an Amplatzer Vascular Plug II implanted from the right atrial aspect. This report includes the first CT imaging of this extremely rare congenital defect as well as detailing the first successful transcatheter occlusion of this particular lesion.

Aims: The pathophysiological entity of a persisting left-sided superior caval vein draining into the roof of the left atrium represents an extreme form of coronary sinus de-roofing. This is an uncommon, but well-documented condition associated with systemic desaturation due to a right-to-left shunt. Depending on the size of the coronary ostium, the defect may also present with right-sided volume loading. We describe two patients, both of whom presented with desaturation, and highlight the important anatomical features underscoring management. Methods and Results: Both patients were managed interventionally with previous assessment of the size of the coronary sinus ostium through cross-sectional imaging. This revealed a restrictive interatrial communication at the right atrial mouth of the coronary sinus in both patients, which permitted an interventional approach, as the residual left-to-right shunt subsequent to closure of the aberrant vessel would be negligible. At intervention, test occlusion of the left superior caval vein allowed assessment of decompressing vessels before successful occlusion using an Amplatzer Vascular Plug. Conclusions: Persistence of a left superior caval vein draining to the left atrium may be associated with an interatrial communication at the mouth of the unroofed coronary sinus. The ostium of the de-roofed coronary sinus can be atretic, restrictive, normally sized, or enlarged. Careful assessment of the size of this defect is required before treatment. In view of its importance, which has received little attention in the literature to date, we suggest an additional consideration to the classification of unroofed coronary sinus.

Isolated congenital complete heart block (CCHB) is a rare disease with significant associated morbidity and mortality. A diagnosis is often made in fetal life, but data regarding long-term outcomes are limited, and fetal therapy to improve prognosis is controversial. In our institution, 85 fetuses were diagnosed with CCHB from 1981 to 2013 in 80 mothers. There were 37 anti Ro-positive pregnancies, 36 both anti-Ro and anti-La positive, 10 antibody negative, and 2 of unknown antibody status. Antenatal treatments were given in 14 fetuses, with 8 given fluorinated steroids, 4 beta sympathomimetics, and both in 2. Of the original 85, 74 babies survived to delivery. Fetal hydrops was the only risk factor found to be significantly associated with intrauterine death (p < 0.001). Four babies died before pacemaker implantation, 56 have had pacemakers implanted, and 14 are pacemaker free. The Kaplan-Meier estimate for median time to pacemaker implantation was 2.6 years, with 15 implanted in the neonatal period. There have been 14 postnatal deaths, with a Kaplan-Meier estimate of survival at 30 years of 76.8% (95% confidence interval 65% to 90%). Dilated cardiomyopathy was uncommon, occurring in 6 patients. Prematurity and hydrops were associated with increased postnatal mortality (p = 0.02 and 0.005, respectively). In conclusion, we present the largest single-unit experience of prenatally diagnosed CCHB in the published literature. Our cohort was conservatively managed, with survival similar to those previously published. These data offer insight into the long-term natural history of CCHB. (C) 2015 Elsevier Inc. All rights reserved.