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ObjectiveTo 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.MethodsThe 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.Results524 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.ConclusionsThrough 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.

OBJECTIVE Our goal was to present 2 decades of our experience with the Ross procedure and its sequential modifications, adopted since 2010, to improve the reoperation rate. METHODS We performed a single-centre, retrospective review of database information and medical notes about the implantation technique: the freestanding root. We compared era 1 (1997–2009) and era 2 (2010–2019). RESULTS Between 1997 and 2019, a total of 214 Ross procedures were performed (71% men, median age 24 years) [interquartile range (IQR) 15–38]. Of these, 87% had various forms of congenital-dysplastic aortic valves. The median cross-clamping and bypass times were 173 (IQR 148–202) and 202 (IQR 182–244) min. The median postoperative stay was 6 days (2–77). Thirty-day mortality was 0.5%. The median follow-up time was 8.2 years (IQR 3.9–13.2). Survival at 10 and 20 years was 97% and 95%; freedom from greater than moderate aortic regurgitation or aortic valve intervention was 91% and 80%; and 93% of the patients were in New York Heart Association functional class I. Twenty (21%) patients operated on during era 1 and 6 (9%) during era 2 underwent autograft reoperations. The median follow-up time was 14.3 (IQR 11.5–17.4) and 4.8 (IQR 2.5–7) years. Freedom from autograft reoperation was 87% and 69% at 10 and 20 years, with no significant difference between eras. Freedom from homograft reoperation was 96% and 76% at 10 and 20 years. The presence of aortic regurgitation, infective endocarditis and era 1 were predictors of autograft reoperation. Male gender and era 1 were predictors of neoaortic root dilatation. CONCLUSIONS The contemporary modified Ross procedure continues to deliver excellent results and should remain part of the strategy to treat children and young adults requiring aortic valve replacement.

There are marked gender differences in all etiologies of advanced heart failure. We sought to determine whether there is evidence of gender-specific decision making for transplant assessments, and how gender effects outcomes.Retrospective analysis of adult heart transplant assessments at a single UK center between April 2015 and March 2020.Females were 32% of referrals (N = 137 females, 285 males), with marked differences between diagnoses - 11% ischemic and 43% of adult congenital. Females were younger, shorter, weighed less, and had lower pulmonary pressures. Females were much less likely to receive a ventricular assist device (13%). Blood type "O" females were relatively more likely compared to males to receive a transplant (45%). Comparing males and females who received a ventricular assist device, both had similar levels of high pulmonary pressures, indicating consistent decision-making based on hemodynamics to implant a device. Overall survival was better for females (in noncongenital patients), and this was due to female patients who were not accepted for transplant or a ventricular assist device being more often "too well for transplant," rather than in males when they were more often "unsuitable."Marked gender differences exist at all stages of the heart transplant assessment pathway. Appropriate decision-making based on clinical grounds is shown with less transplants in male blood type "O"s and hemodynamic criteria for ventricular assist device implantation in both genders. Further studies are needed to determine if there is a wider community bias in advanced heart failure treatments for females.

Background: The adult congenital heart disease (ACHD) population is growing in size and complexity. This study evaluates whether present ambulatory care adequately detects problems and considers costs. Methods: A UK single-centre study of clinic attendances amongst 100 ACHD patients (40.4 years, median ACHD AP class 2B) between 2014 and 2019 and the COVID-19 restrictions period (March 2020–July 2021). Results: Between 2014 and 2019, there were 575 appointments. Nonattendance was 10%; 15 patients recurrently nonattended. Eighty percent of appointments resulted in no decision other than continued review. Electrocardiograms and echocardiograms were frequent, but new findings were rare (5.1%, 4.0%). Decision-making was more common with the higher ACHD AP class and symptoms. Emergency admissions (n = 40) exceeded elective (n = 25), with over half following unremarkable clinic appointments. Distance travelled to the ACHD clinic was 14.9 km (1.6–265), resulting in 433–564 workdays lost. During COVID 19, there were 127 appointments (56% in-person, 41% telephone and 5% video). Decisions were made at 37% in-person and 19% virtual consultations. Nonattendance was 3.9%; there were eight emergency admissions. Conclusion: The main purpose of the ACHD clinic is surveillance. Presently, the clinic does not sufficiently predict or prevent emergency hospital admissions and is costly to patient and provider. COVID-19 has enforced different methods for delivering care that require further evaluation.

OBJECTIVES Ventricular assist device (VAD) for systemic right ventricular (RV) failure patients post-atrial switch, for transposition of the great arteries (TGA), and those with congenitally corrected TGA has proven useful to reduce transpulmonary gradient and bridge-to-transplantation. The purpose of this study is to describe our experience of VAD in systemic RV failure and our move towards concomitant tricuspid valve replacement (TVR). METHODS This is a single-centre retrospective study of consecutive adult patients receiving HeartWare VAD for systemic RV failure between 2010 and 2019. From 2017, concomitant TVR was performed routinely. Demographic, clinical variables and echocardiographic and haemodynamic measurements pre- and post-VAD implantation were recorded. Complications on support, heart transplantation and survival rates were described. RESULTS Eighteen patients underwent VAD implantation. Moderate or severe systemic tricuspid regurgitation was present in 83.3% of patients, and subpulmonic left ventricular impairment in 88.9%. One-year survival was 72.2%. VAD implantation was technically feasible and successful in all but one. Post-VAD, transpulmonary gradient fell from 16 (15–22) to 10 (7–13) mmHg (P = 0.01). Patients with TVR (n = 6) also demonstrated a reduction in mean pulmonary and wedge pressures. Furthermore, subpulmonic left ventricular end-diastolic dimension (44.3 vs 39.6 mm; P = 0.03) and function improved in this group. After 1 year of support, 72.2% of patients were suitable for transplantation. CONCLUSIONS VAD is an effective strategy as bridge-to-candidacy and bridge-to-transplantation in patients with end-stage systemic RV failure. Concomitant TVR at the time of implant is associated with better early haemodynamic and echocardiographic results post-VAD.

Background: In recent years, patient-reported outcomes (PROs) have received increasing prominence in cardio-vascular research and clinical care. An understanding of the variability and global experience of PROs in adults with congenital heart disease (CHD), however, is still lacking. Moreover, information on epidemiological characteristics and the frailty phenotype of older adults with CHD is minimal. The APPROACH-IS II study was established to address these knowledge gaps. This paper presents the design and methodology of APPROACH-IS II.Methods/design: APPROACH-IS II is a cross-sectional global multicentric study that includes Part 1 (assessing PROs) and Part 2 (investigating the frailty phenotype of older adults). With 53 participating centers, located in 32 countries across six continents, the aim is to enroll 8000 patients with CHD. In Part 1, self-report surveys are used to collect data on PROs (e.g., quality of life, perceived health, depressive symptoms, autonomy support), and explanatory variables (e.g., social support, stigma, illness identity, empowerment). In Part 2, the cognitive functioning and frailty phenotype of older adults are measured using validated assessments.Discussion: APPROACH-IS II will generate a rich dataset representing the international experience of individuals in adult CHD care. The results of this project will provide a global view of PROs and the frailty phenotype of adults with CHD and will thereby address important knowledge gaps. Undoubtedly, the project will contribute to the overarching aim of improving optimal living and care provision for adults with CHD., KU Leuven - University of Leuven; Research Foundation Flanders [1159522N, 12E9819N]; Swedish Heart and Lung Foundation [20190525]; Research Council of Aga Khan University in Karachi in Pakistan; Maltese Cardiac Society; Children's Heart Unit Fund; UnIC (Unidade de Investigacao Cardiovascular da FMUP); Italian Ministry of Health, This work is supported by KU Leuven - University of Leuven, Research Foundation Flanders through grants 1159522N and 12E9819N, Swedish Heart and Lung Foundation through grant 20190525, the University Research Council of the Aga Khan University in Karachi in Pakistan, Maltese Cardiac Society, UnIC (Unidade de Investigacao Cardiovascular da FMUP) supported the costs of the translations into Portuguese used in Portugal, Children's Heart Unit Fund ( www.chuf.org.uk), Newcastle upon Tyne through their funding of the CHUF Fontan nurse specialist. This study was partially supported by Ricerca Corrente funding from the Italian Ministry of Health to IRCCS Policlinico San Donato.

Brief introduction: In healthy adults, the right atrium (RA) serves as a reservoir for the systemic flow return from the superior vena cava (SVC) and inferior vena cava (IVC), preparing the two flows to be transferred to the right ventricle (RV) and pulmonary circulation. This study aims to quantify the haemodynamics of the RA and the associated SVC and IVC inflows, which have not been fully understood to date. Basic Methods Eighteen adults with structurally normal hearts underwent 4D flow magnetic resonance imaging. The cardiac cycle was resolved to 20 temporal phases with a spatial resolution of 3×3×3 mm cube. Analysis included objective visualisation of the flow structures in the RA identified by three different vortex identification criteria, kinetic energy (KE), enstrophy and dissipation. KE and helicity flux were also assessed in both caval veins. Main Results Vortex identification methods confirmed that in the majority of participants the blood flow from the caval veins filling the RA during ventricular systole is not chaotic, but rather forms an organised pattern of a single coherent forward turning vortex structure. Thirteen participants displayed a single vortex flow structure, four showed multiple vortices and one had a helical flow pattern without a clear vortex structure. A strong positive correlation exists between the flow KE and enstrophy density. Conclusions and Implications This study suggests that flow energy in the RA is mainly rotational, part of which is convected by the highly helical SVC and IVC inflows. Multiple vortices tend to be associated with higher dissipation rates in the central RA region due to turbulence. The rotational nature of the flow in the RA maintains KE better than non-rotational flow. RA flow characteristics are highly related to the helicity content in the caval veins, as well as the KE flux intensity. Lower caval helicity or IVC KE flux dominance tends to favour single vortex formation while the opposite tends to lead to multiple vortices or the rare helical flow patterns. Atria lacking single vortex flow are inclined to have a larger energy input from atrial contraction. Conflict of Interest n/a

ObjectivesAdults with congenital heart disease (ACHD) are a growing group with end-stage heart failure. We aim to describe the outcomes of ACHD patients undergoing assessment for orthotopic heart transplant (OHT).MethodsCase notes of consecutive ACHD patients (>16 years) assessed for OHT between 2000 and 2016 at our centre were reviewed. Decision and outcome were reported as of 2017. Data were analysed in three groups: systemic left ventricle (LV), systemic right ventricle (RV) and single ventricle (SV).Results196 patients were assessed (31.8 years, 27% LV, 29% RV, 44% SV). 89 (45%) patients were listed for OHT and 67 (34%) were transplanted. 41 (21%) were unsuitable or too high risk and 36 (18%) were too well for listing. Conventional surgery was undertaken in 13 (7%) and ventricular assist device in 17 (9%) with 7 (4%) bridged to candidacy. Survival from assessment was 84.2% at 1 year and 69.7% at 5 years, with no difference between groups. Patients who were considered unsuitable for OHT (HR 11.199, pConclusionsThe number of ACHD patients assessed for OHT is increasing. A third are transplanted with a small number receiving conventional surgery. Those who are unsuitable have a poor prognosis.

Aims:Most reports on the outcome of children who present with heart failure, due to heart muscle disease, are from an era when ventricular assist devices were not available. This study provides outcome data for the current era where prolonged circulatory support can be considered for most children.Methods & Results:Data was retrieved on 100 consecutive children, who presented between 2010 – 2016, with a first diagnosis of unexplained heart failure. Hospital outcome was classified as either death, transplantation, recovery of function or persistent heart failure. Median age at presentation was 24 months and 58% were < 5 years old. Hospital mortality was 12% and 59% received a heart transplant. Most, 79%, of the transplants were carried out on patients with a device. Recovery of function was observed in 18% and 10% stabilised on oral therapy. Eighty-four percent of the deaths occurred in the Conclusion:Heart failure in children who require referral to a transplant unit is a serious illness with a high chance of either transplantation or death. Modifications in assist devices will be required to improve safety, especially for children < 5 years old where the donor wait may be prolonged. The identification of children who may recover function requires further study.

Objective To identify the key contributors to postoperative mortality in patients undergoing orthotopic heart transplantation (OHT) for late Fontan failure. Methods This retrospective review of failing Fontan patients who underwent OHT in our tertiary care center between 2007 and 2019 included adult patients with congenital heart disease and single ventricle physiology who were palliated with a Fontan circulation for >1 year. We excluded patients undergoing combined heart-liver transplantation. Results The study cohort comprised 31 patients, including 18 males (58.1%), with a mean weight of 58.4 kg, median age at Fontan of 6.9 years (interquartile range [IQR], 2-38 years), and a median age at OHT of 27.1 years (IQR, 16.7-53.3). Almost all (93.5%) of the patients were in New York Heart Association class III-IV, and the majority (74.2%) were in Interagency Registry for Mechanically Assisted Circulatory Support class 3. Overall survival at 30 days, 1 year, and 5 years after OHT was 81%, 71%, and 67%, respectively. Major intraoperative bleeding was associated with increased mortality after OHT (odds ratio, 30; 95% confidence interval, 2.8-322; P = .002). Neither preoperative systemic ventricular function nor the development of primary graft dysfunction (PGD) was significantly associated with postoperative death. Nevertheless, PGD determined significant morbidity of this population. Conclusions In our cohort, major intraoperative bleeding was the key factor associated with mortality after OHT for late Fontan failure. Novel strategies for the prevention and management of postoperative bleeding will improve outcomes in this group of patients.

Objective. In this paper we elucidate the asymmetric flow pattern and the haemodynamic quantity distributions and correlations in the pulmonary artery (PA) vasculature in healthy adults having structurally normal hearts, to provide reference on the flow characteristics in the PA and the right ventricle. Approach. Velocity data are acquired non-invasively from 18 healthy volunteers by 4D flow magnetic resonance imaging, resolved to 20 phases with spatial resolution 3 × 3 × 3 mm3. Interpolation is applied to improve the accuracy in quantifying haemodynamic quantities including kinetic energy, rotational energy, helicity and energy dissipation rate. These quantities are volumetrically normalised to remove size dependency, representing densities or local intensity. Main results. Flow asymmetry in the PA is quantified in terms of all the flow dynamic quantities and their correlations. The right PA has larger diameter and higher peak stroke velocity than the left PA. It also has the highest rotational energy intensity. Counter-rotating helical streams in the main PA appear to be associated with the unidirectional helical flow noticed in the left and the right PA near the peak systole. Significance. This study provides a fundamental basis of normal flow in the PA. It implies the validity to use these flow pattern-related quantitative measures to aid with the identification of abnormal PA flow non-invasively, specifically for detecting abnormalities in the pulmonary circulation and response to therapy, where haemodynamic flow is commonly characterised by increased vortical and helical formations.

Objective: In healthy adults, the right atrium (RA) serves as a reservoir for the systemic flow return from the superior vena cava (SVC) and inferior vena cava (IVC), preparing the two flows to be transferred to the right ventricle (RV) and pulmonary circulation. This study aims to quantify the haemodynamics of the RA and the associated SVC and IVC inflows, which have not been fully understood to date. Approach: Eighteen adults with structurally normal hearts underwent 4D flow magnetic resonance imaging. The cardiac cycle was resolved to 20 temporal phases with a spatial resolution of 3x3x3mm3. Analysis included objective visualisation of the flow structures in the RA identified by three different vortex identification criteria, kinetic energy (KE), enstrophy and dissipation. KE and helicity flux were also assessed in both caval veins. Main results: Vortex identification methods confirmed that in the majority of subjects the blood flow from the caval veins filling the RA during ventricular systole is not chaotic, but rather forms an organised pattern of a single coherent forward turning vortex structure. Thirteen subjects displayed a single vortex flow structure, four showed multiple vortices and one had a helical flow pattern without a clear vortex structure. A strong positive correlation exists between the flow KE and enstrophy density. Significance: This suggests that flow energy in the RA is mainly rotational, part of which is convected by the highly helical SVC and IVC inflows. Multiple vortices tend to be associated with higher dissipation rates in the central RA region due to turbulence. The rotational nature of the flow in the RA maintains KE better than non-rotational flow. RA flow characteristics are highly related to the helicity content in the caval veins, as well as the KE flux intensity. Lower caval helicity or IVC KE flux dominance tends to favour single vortex formation while the opposite tends to lead to multiple vortices or the rare helical flow patterns. Atria lacking single vortex flow are inclined to have a larger energy input from atrial contraction.

PurposeAnecdotal reports suggest that children and young adults with CHD frequently experience pain in their legs. The purpose of this pilot study, performed by Little Hearts Matter patient organisation, was to assess the burden of leg pains in this group and begin to investigate associated factors and consequences for daily living.MethodsAn internet-based survey was distributed by Little Hearts Matter patient organisation. After anonymisation and collation, responses were analysed and compared with their healthy siblings.ResultsOf the 220 patients who responded, 94% reported leg pains compared with 30% of siblings (n=107; pConclusionLeg pains are more frequent in those with CHD compared with their healthy siblings. Aetiology is uncertain, but pains share many common characteristics with benign “growing pains”.

The single ventricle patient population comprises the most complex cohort presenting to the cardiac transplant team, in terms of demographics, anatomic substrate, and unique physiology. It is also the most rapidly growing diagnostic group presenting for heart transplantation. In this manuscript, we aim to describe the changing landscape of transplantation in single ventricle conditions through reflection on our own institution’s practice and experience, alongside contemporary literature review. Single ventricle patients are heterogeneous in terms of age, anatomic diagnosis and physiology according to surgical stage of repair. Progress in surgical palliative strategies has impacted upon the present composition of the population, with growing numbers of hypoplastic left heart syndrome patients and those with late physiology failure following Fontan completion. Multiple prior surgeries, immunological sensitivity and multi-organ involvement impart high peri-operative risk but can be mitigated in part by careful pre-operative planning by a dedicated multi-disciplinary team addressing issues such as planning of concurrent reconstructive surgery, minimizing the post-operative effect of collaterals, timely harvesting, oversizing of donor organs to minimize graft failure, and strategies to address anticipated post-operative elevation in pulmonary vascular resistance. Determining optimal timing for transplant in these patients remains unclear, but understanding the risk of alternative surgical options can help guide decision making with regards to listing.

Purpose Ventricular assist device (VAD) for systemic right ventricular failure (SRVF) post atrial switch for transposition of the great arteries (TGA) and congenitally corrected TGA (ccTGA) has proven useful to reduce transpulmonary gradient (TPG) and bridge-to-transplantation. The hemodynamic impact of severe tricuspid regurgitation (TR), potential role of systemic tricuspid valve replacement (TVR) and long-term outcomes are still unknown. The purpose of this study is to describe our experience of VAD in SRVF and our move towards concomitant TVR. Methods Single-centre retrospective study of consecutive adult patients receiving HeartWare-VAD for SRVF between 2010 and 2020. From 2017, concomitant TVR was performed routinely. Demographic, clinical variables and echocardiographic and haemodynamic measurements pre and post VAD implantation were recorded. Complications on support, heart transplantation and survival rates were described. Results 24 patients underwent VAD implantation. Moderate or severe systemic TR was present in 83% of patients, and subpulmonic left ventricular (LV) impairment in 79%. One and two-year survival rates were 75% and 60% respectively. VAD implantation was technically feasible and successful in all but one. Post VAD, TPG fell from 16 (15-20) to 10 (7-13) mmHg; p=0.002. Patients with TVR (n=12) also demonstrated reduction in pulmonary and atrial pressures (figure). Subpulmonic LV end-diastolic dimension (50 vs 42mm; p=0.05) and systolic function improved in this group. On the other hand a high prevalence of subpulmonic LV failure was noted in the non TVR group (50% vs 8.3%; p=0.03) resulting in higher mortality. After six months on support, 63% of patients were suitable for transplantation. Conclusion VAD implantation for patients with SRVF in TGA or ccTGA is feasible, and significantly reduces the TPG to transplantable limits. Concomitant TVR at the time of implant associates better haemodynamic and echocardiographic results.

Management of adults with failing Fontan physiology poses many challenges, especially as transplantation offers the only realistic alternative to palliative care. We present the first combined heart and liver transplant performed in Europe, for a late survivor of single ventricle palliation with the Fontan circulation. In addition to the conventional medical and surgical challenges posed, we highlight the management of the associated multi-organ failure with focus on the liver and novel strategies for assessment and optimization.

OBJECTIVE The Fontan operation greatly improves survival for single ventricle congenital heart disease patients but creates a physiology that leads to long-term multi-organ dysfunction. A non-invasive screening tool that can identify impending decline is sought. The objective of this pilot study was to assess the microcirculation in Fontan-palliated patients by measuring tissue oxygen saturation (StO2) in superficial and deeper tissues. APPROACH Three patient cohorts were studied: Fontan group (n = 8) and two patient control groups, liver disease group (n = 8) and tetralogy of Fallot group (n = 9). 22 healthy controls were also examined. Superficial and deeper StO2 was measured at the forearm, thenar eminence, index and ring fingers of both arms using the LEA O2C spectrophotometry device. MAIN RESULTS Superficial StO2 was reduced in Fontan patients compared to healthy controls (p = 0.002) and tetralogy patients (p = 0.016), but not compared to the liver group (p = 0.313). Deeper StO2 was similar between groups (p = 0.112). The gap between deeper and superficial StO2 was raised in Fontan patients compared to healthy controls (p = 0.001) and tetralogy patients (p = 0.037), but not compared to the liver group (p = 0.504). There was no clinically relevant difference in StO2 between the left and right arms, and the variation in StO2 according to measurement site was similar between the four groups. SIGNIFICANCE Vascular optical spectrophotometry is a feasible non-invasive measure of micro-circulatory function that can easily be performed in the clinic setting and may have utility in patients with Fontan circulations. Further, we provide important normal range data in the healthy control population which can be used to design future studies.

Purpose Occasionally, single ventricle (SV) patients survive into adulthood without Fontan palliation; with the theoretical advantage of potentially lower inferior caval vein pressures. We studied liver and renal function in an adult cohort of SV patients who had not undergone Fontan completion (F-) and a group who had Fontan completion (F+) in adult life. Methods Retrospective case note analysis between 1990–2017. Results There were 12 SV patients in the F- group [9 (75%) male, age 41 years (18–52), 8 (67%) had previous surgery, aortopulmonary shunt: 3, Glenn: 5]. Outcomes: 2 deaths (at 42 and 24 years), 1 assessed for transplant, 2 for TCPC completion, 7 balanced circulations. At last review, saturations were 78% (69–85), Hb 192 g/L (159–252), creatinine 88 μmol/L (59–129), eGFR 65 (59– >90), albumin 46 g/L (35–50), bilirubin 20 μmol/L (12–46) and platelets 220 (105–333). Abdominal USS was available in 5: normal liver (2), mild-moderate fibrosis (2), cirrhosis (1). There were 8 SV patients in the F+ group, [3 (37%) male, age 46 years (23–55), age at Fontan completion 28 years (16–46)]. Follow-up was 13 years (2–24). Outcomes 1 transplant, 4 failing Fontan physiology, 3 well. At last review, saturations were 95% (75–96), Hb 163 g/L (108–171), creatinine 76 μmol/L (66–148), eGFR 72 (36– >90), albumin 47 g/L (30–52), bilirubin 13 μmol/L (10–39) and platelets 216 (137–429). Abdominal USS was available in 5: normal liver (2), mild-moderate fibrosis (2), severe fibrosis (1). Conclusion In both groups, synthetic liver function was preserved and a minority showed advanced fibrosis/cirrhosis on USS. Renal function was impaired, more prominently in the non-Fontan group, likely reflecting the effect of chronic hypoxia. Strategies to delay or avoid Fontan completion may not preserve liver function.

BackgroundThe population of women of childbearing age palliated with a Fontan repair is increasing. The aim of this study was to describe the progress of pregnancy and its outcome in a cohort of patients with a Fontan circulation in the UK.MethodsA retrospective study of women with a Fontan circulation delivering between January 2005 and November 2016 in 10 specialist adult congenital heart disease centres in the UK.Results50 women had 124 pregnancies, resulting in 68 (54.8%) miscarriages, 2 terminations of pregnancy, 1 intrauterine death (at 30 weeks), 53 (42.7%) live births and 4 neonatal deaths. Cardiac complications in pregnancies with a live birth included heart failure (n=7, 13.5%), arrhythmia (n=6, 11.3%) and pulmonary embolism (n=1, 1.9%). Very low baseline maternal oxygen saturations at first obstetric review were associated with miscarriage. All eight women with saturations of less than 85% miscarried, compared with 60 of 116 (51.7%) who had baseline saturations of ≥85% (p=0.008). Obstetric and neonatal complications were common: preterm delivery (n=39, 72.2%), small for gestational age (ConclusionWomen with a Fontan circulation have a high rate of miscarriage and, even if pregnancy progresses to a viable gestational age, a high rate of obstetric and neonatal complications.

Cardiogenic shock (CS) describes the physiologic state in which reduced cardiac output and resultant tissue hypoxia occur in the presence of adequate intravascular volume. Among patients hospitalized with myocardial infarction (MI), CS is the foremost cause of death. Women are more susceptible to CS than men in the setting of ST segment increase MI. Introduction of early revascularization strategies and mechanical ventricular support have seen a decrease in short-term mortality from CS. However, the prognosis following CS remains poor. This article examines the prevalence, causes, pathophysiology, and therapeutic options for CS among women.

Sudden cardiac death in congenital heart disease is related to increased right ventricular end-diastolic volume (RVEDV), abnormalities of QRS duration, and QRS, JT, and QT dispersions. Surgical pulmonary valve replacement and percutaneous pulmonary valve implantation (PPVI) decrease RVEDV, but the effects of PPVI on surface electrocardiographic parameters are unknown. PPVI represents a pure model of RV mechanical and electrophysiologic changes after replacement. This prospective study sought to determine the effects of PPVI on surface electrocardiographic parameters: Ninety-nine PPVI procedures in patients with congenital heart disease (23.1 ± 10 years of age) were studied before, after, and 1 year after PPVI with serial electrocardiograms and echocardiogram/magnetic resonance images. Forty-three percent had pulmonary stenosis, 27% pulmonary regurgitation (PR), and 29% mixed lesions. In those with predominantly PR (n = 26), QRS duration decreased significantly (135 ± 27 to 128 ± 29 ms, p = 0.007). However, in the total cohort no significant change in QRS duration at 1 year was observed (137 ± 29 to 134 ± 29 ms). Corrected QT interval and QRS, QT, and JT dispersions significantly decreased at 1 year (p ≤0.001). RVEDV correlated with preprocedure QRS duration (r = 0.34, p

Percutaneous pulmonary valve implantation is a potential treatment for right ventricular outflow tract (RVOT) dysfunction. However, RVOT implantation site varies among subjects and the success of the procedure depends on RVOT morphology selection. The aim of this study was to use in vitro testing to establish percutaneous valve competency in different previously defined RVOT morphologies. Five simplified RVOT geometries (stenotic, enlarged, straight, convergent, and divergent) were manufactured by silicone dipping. A mock bench was developed to test the percutaneous valve in the five different RVOTs. The bench consists of a volumetric pulsatile pump and of a hydraulic afterload. The pump is made of a piston driven by a low inertia programmable motor. The hydraulic afterload mimics the pulmonary input impedance and its design is based on a three element model of the pulmonary circulation. The mock bench can replicate different physiological and pathological hemodynamic conditions of the pulmonary circulation. The mock bench is here used to test the five RVOTs under physiological-like conditions: stroke volume range 40-70 mL, frequency range 60-80 bpm. The valved stent was implanted into the five different RVOT geometries. Pressures upstream and downstream of the valved stent were monitored. Flow rates were measured with and without the valved stent in the five mock RVOTs, and regurgitant fraction compared between the different valved stent RVOTs. The percutaneous valved stent drastically reduced regurgitant flow if compared with the RVOT without the valve. RVOT geometry did not significantly influence the flow rate curves. Mean regurgitant fractions varied from 5% in the stenotic RVOT to 7.3% in the straight RVOT, highlighting the influence of the RVOT geometry on valve competency. The mock bench presented in this study showed the ability to investigate the influence of RVOT geometry on the competence of valved stent used for percutaneous pulmonary valve treatment.

Objective: To analyse the potential benefit of restoration of pulmonary valvar competence in patients with severe pulmonary regurgitation (PR) and pulmonary hypertension (PH) associated with congenital heart disease. Design: Retrospective study. Setting: Tertiary paediatric and adult congenital heart cardiac centre. Interventions: Percutaneous pulmonary valve implantation (PPVI). Patients: All patients who underwent PPVI for treatment of PR in the presence of PH (mean PAP >25 mm Hg). Results: Seven patients with severe PH as a result of congenital heart disease and severe PR underwent PPVI. The valve implantation procedure was feasible and uncomplicated in all seven cases, successfully abolishing PR. There was a significant increase in diastolic (15.4 (7.3) to 34.0 (8.5) mm Hg; p = 0.007) and mean (29.7 (8.1) to 41.3 (12.9) mm Hg; p = 0.034) pulmonary artery pressures, and an improvement in NYHA functional class (from median IV to median III; p

Aims To investigate the early clinical and physiological consequences of relieving chronic right ventricular (RV) volume overload with percutaneous pulmonary valve implantation (PPVI). Methods and results We selected 17 patients (age 21.2 ± 8.7 years), from a total of 125 who underwent PPVI, because they had important pulmonary regurgitation (PR) [regurgitant fraction > 25% on magnetic resonance (MR)] and an echocardiographic gradient < 50 mmHg across the RV outflow tract. Cardiopulmonary exercise testing, tissue Doppler and MR were performed before and within 3 months of PPVI. Following PPVI, PR (40.7 ± 7.3 to 4.1 ± 6.1%, P < 0.001) and RV end-diastolic volume fell (115.4 ± 33.1 to 98.9 ± 32.0 mL/m2, P = 0.001); effective RV stroke volume increased (34.3 ± 7.8 to 44.4 ± 9.3 mL/m2, P < 0.001). Left ventricular end-diastolic volume (66.6 ± 18.0 to 73.4 ± 16.5 mL/m2, P = 0.014), stroke volume (38.4 ± 11.1 to 46.4 ± 10.2 mL/m2, P = 0.001) and ejection fraction (57.8 ± 8.1 to 63.5 ± 5.2 mL/m2, P = 0.001) increased. Pulmonary artery diastolic pressure (8.9 ± 4.5 to 12.5 ± 5.2 mmHg, P = 0.041) and mitral E /Ea increased (from 9.0 ± 2.0 to 11.6 ± 3.1, P = 0.003). Patients felt better, but standard measures of exercise capacity were unchanged. Conclusion PPVI relieves PR and restores compensatory cardiac performance. The lack of improvement in exercise parameters suggests that, in contrast to pressure overload, the contractile reserve of chronically volume-overloaded myocardium is limited.

Background— We analyzed the incidence, risk factors and treatment options for stent fracture after percutaneous pulmonary valve (PPV) implantation (PPVI). Methods and Results— After PPVI, 123 patients had chest x-ray in anteroposterior and lateral projection, echocardiography, and clinical evaluation during structured follow-up. Of these 123 patients, 26 (21.1%) developed stent fracture 0 to 843 days after PPVI (stent fracture–free survival at 1 year, 85.1%; at 2 years, 74.5%; and at 3 years, 69.2%). Stent fracture was classified as type I: no loss of stent integrity (n=17); type II: loss of integrity with restenosis on echocardiography (n=8); and type III: separation of fragments or embolization (n=1). In a multivariate Cox regression, we analyzed various factors, of which 3 were associated with a higher risk of stent fracture: implantation into “native” right ventricular outflow tract ( P =0.04), no calcification along the right ventricular outflow tract (judged with fluoroscopy, P =0.02), recoil of PPV (qualitatively, PPV diameter in frontal or lateral plane with fully inflated balloon > diameter after balloon deflation, P =0.03). Substernal PPV location, high-pressure post-PPVI dilatation of PPV, pre-PPVI right ventricular outflow tract gradients, and other indicators of PPV compression or asymmetry did not pose increased risk. Patients with type I fracture remain under follow-up. Patients with type II fracture had 2nd PPVI or are awaiting such procedure, and 1 patient with type III fracture required surgical explantation. Conclusion— Stent fracture after PPVI can be managed effectively by risk stratification, systematic classification, and anticipatory management strategies. Serial x-ray and echocardiography are recommended for surveillance.

To determine if magnetic resonance (MR) imaging data can be used to create rigid models that are accurate representations of the right ventricular outflow tract (RVOT) and pulmonary trunk anatomy and if such models can be used to refine the selection of patients for percutaneous pulmonary valve implantation (PPVI).Institutional review board approval and informed patient consent were obtained. Twelve patients' MR data were analyzed and elaborated for input into a rapid prototyping (RP) system. RP models were successfully built and presented to two experienced cardiologists, who were retrospectively asked if they would have attempted PPVI. Their responses were compared with the documented decisions and outcomes of PPVI.For four subjects, both cardiologists correctly determined, on the basis of MR image or three-dimensional (3D) RP model findings, that PPVI should not have been attempted. Two patients in whom PPVI was attempted were considered to be unsuitable for the procedure after balloon sizing, and in another two patients, implantation was unsuccessful because of device instability. For the four patients in whom PPVI was suitable and the four in whom it was unsuitable, observers 1 and 2 correctly determined suitability for PPVI in four and two patients, respectively, by using the MR images alone. Both observers correctly determined the suitability of five patients by using the 3D models alone.Using 3D RP models resulted in more accurate selection of patients for PPVI than did using MR images.

Background Patent foramen ovale (PFO) occurs in a quarter of the population but half of those with cryptogenic stroke (CS). Difficulty in identifying the pathogenic PFO versus the innocent bystander has contributed to controversy surrounding outcomes following PFO closure. We aimed to investigate whether right atrial flow patterns could help define the mechanism for CS in the setting of PFO. Methods 4D flow cardiac magnetic resonance (CMR) was performed in 10 subjects (age 43±6 years, 4 male) with proven PFO and CS and in 10 controls (age 42±7years, 4 male) with normal trans-thoracic echocardiograms. CMR was performed at 3T (Achieva; Philips) with a 6-channel cardiac array. A retrospectively ECG-gated and respiratory-gated TFE sequence (TE/TR/flip:3.7/6.3ms/8°, VENC:150m/s, FOV:240mm(AP)x240mm(FH)x142mm (LR), spatial resolution:3mm 3 , temporal resolution:5055ms, 20 phases, SENSE, factor 2) was used. Analysis was performed with GTflow v2.0 (Gyrotools). Contours were placed manually in the superior vena cava (SVC) and inferior vena cava (IVC) in the axial plane at the junction with the right atrium. Net flow was assessed at these points. The relative position of the SVC and IVC at these points was measured. A pathline analysis of atrial flow was performed (SVC:red, IVC:green). Results

Background— Right ventricular outflow tract obstruction (RVOTO) is a common problem after repair of congenital heart disease. Percutaneous pulmonary valve implantation (PPVI) can treat this condition without consequent pulmonary regurgitation or cardiopulmonary bypass. Our aim was to investigate the clinical and physiological response to relieving RVOTO. Methods and Results— We studied 18 patients who underwent PPVI for RVOTO (72% male, median age 20 years) from a total of 93 who had this procedure for various indications. All had a right ventricular outflow tract (RVOT) gradient >50 mm Hg on echocardiography without important pulmonary regurgitation (less than mild or regurgitant fraction P P −1 · min −1 , P =0.002) and anaerobic (14.4 to 16.2 mL · kg −1 · min −1 , P =0.002) exercise capacity improved. Myocardial systolic velocity improved acutely (tricuspid 4.8 to 5.3 cm/s, P =0.05; mitral 4.7 to 5.5 cm/s, P =0.01), whereas isovolumic acceleration was unchanged. The tricuspid annular velocity was not maintained on intermediate follow-up. Right ventricular end-diastolic volume (99.9 to 89.7 mL/m 2 , P 2 , P =0.06) and ejection fraction (48.0% to 56.8%, P =0.01) increased. Left ventricular end-diastolic volume (72.5 to 77.4 mL/m 2 , P =0.145), stroke volume (45.3 to 50.6 mL/m 2 , P =0.02), and ejection fraction (62.6% to 65.8%, P =0.03) increased. Conclusions— PPVI relieves RVOTO, which leads to an early improvement in biventricular performance. Furthermore, it reduces symptoms and improves exercise tolerance. These findings have important implications for the management of this increasingly common condition.

Background— Right ventricular outflow tract (RVOT) reconstruction with valved conduits in infancy and childhood leads to reintervention for pulmonary regurgitation and stenosis in later life. Methods and Results— Patients with pulmonary regurgitation with or without stenosis after repair of congenital heart disease had percutaneous pulmonary valve implantation (PPVI). Mortality, hemodynamic improvement, freedom from explantation, and subjective and objective changes in exercise tolerance were end points. PPVI was performed successfully in 58 patients, 32 male, with a median age of 16 years and median weight of 56 kg. The majority had a variant of tetralogy of Fallot (n=36), or transposition of the great arteries, ventricular septal defect with pulmonary stenosis (n=8). The right ventricular (RV) pressure (64.4±17.2 to 50.4±14 mm Hg, P P P P −1 · m −2 , P −1 · m −2 , P =0.005) and effective RV stroke volume (37±7 versus 42±9 mL · beat −1 · m −2 , P =0.006) in 28 patients (age 19±8 years). A further 16 subjects, on metabolic exercise testing, showed significant improvement in V̇ o 2 max (26±7 versus 29±6 mL · kg −1 · min −1 , P Conclusions— PPVI is feasible at low risk, with quantifiable improvement in MRI-defined ventricular parameters and pulmonary regurgitation, and results in subjective and objective improvement in exercise capacity.

To the Editor, We read with great interest the report by van Melle et al 1 who conducted a retrospective multicentre European study to assess surgical options for the failing Fontan. First, we would like to acknowledge the effort made to bring together the results of these infrequently performed, difficult interventions. However, we would also like to highlight concerns with the conclusions reached from this historical mixed registry data. The authors state that a …

Occasionally complex congenital cardiac disease presents for the first time in adulthood, encouraging us to reflect anew on our understanding of familiar conditions. A 59-year-old woman, who had had two normal pregnancies, attended with breathlessness and was found to have a double inlet left ventricle with concordant ventriculoarterial connections and a straddling tricuspid valve adherent to the malaligned ventricular septum.