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Introduction Nosocomial pneumonia is a common infection associated with high mortality in hospitalized patients. Nosocomial pneumonia, caused by gram-negative bacteria, often occurs in the elderly and patients with co-morbid diseases. Methods Original research using a prospective cross-sectional design was conducted on 281 patients in an intensive care unit setting with nosocomial pneumonia between July 2015 and July 2019. For each nosocomial pneumonia case, data regarding comorbidities, risk factors, patient characteristics, Charlson comorbidity index (CCI), Systemic Inflammatory Response Syndrome (SIRS), and quick Sepsis-Related Organ Failure Assessment (qSOFA) points and treatment outcomes were collected. Data were analyzed by SPSS 22.0. Results Nosocomial pneumonia due to gram-negative bacteria occurred in patients with neurological disorders (34.87%), heart diseases (16.37%), chronic renal failure (7.12%), and post-surgery (10.68%). Worse outcomes attributed to nosocomial pneumonia were high at 75.8%. Mechanical ventilation, change of antibiotics, and CCI ≥ 3 and qSOFA ≥ 2 were significantly negative prognostic factors (p 0.05). The pathogens were significant factors that influence treatment effects, but they weren’t independent risk factors for poor outcomes (p = 0.823). Conclusions Patients with nosocomial pneumonia hospitalized in intensive care units are usually associated with many underlying diseases, including neurological diseases. Mechanical ventilation, a change in antibiotics, CCI ≥ 3, and qSOFA ≥ 2 are also associated with a worse prognosis of nosocomial pneumonia. CCI and qSOFA might be used in predicting the outcome of nosocomial pneumonia. Supplementary Information The online version contains supplementary material available at 10.1007/s41030-021-00175-4.

Purpose To compare the prevalence of psychiatric comorbidity between patients with complex regional pain syndrome (CRPS) of the hand and non-CRPS patients and to assess the association between biopsychosocial (BPS) complexity profiles and psychiatric comorbidity in a comparative study. Patients and methods We included a total of 103 patients with CRPS of the hand and 290 patients with chronic hand impairments but without CRPS. Psychiatric comorbidities were diagnosed by a psychiatrist, and BPS complexity was measured by means of the INTERMED. The odds ratios (OR) of having psychiatric comorbidities according to BPS complexity were calculated with multiple logistic regression (adjusted for age, sex, and pain). Results Prevalence of psychiatric comorbidity was 29% in CRPS patients, which was not significantly higher than in non-CRPS patients (21%, relative risk=1.38, 95% CI: 0.95 to 2.01 p=0.10). The median total scores of the INTERMED were the same in both groups (23 points). INTERMED total scores (0-60 points) were related to an increased risk of having psychiatric comorbidity in CRPS patients (OR=1.46; 95% CI: 1.23-1.73) and in non-CRPS patients (OR=1.21; 95% CI: 1.13-1.30). The four INTERMED subscales (biological, psychological, social, and health care) were correlated with a higher risk of having psychiatric comorbidity in both groups. The differences in the OR of having psychiatric comorbidity in relation to INTERMED total and subscale scores were not statistically different between the two groups. Conclusion The total scores, as well as all four dimensions of BPS complexity measured by the INTERMED, were associated with psychiatric comorbidity, with comparable magnitudes of association between the CRPS and non-CRPS groups. The INTERMED was useful in screening for psychological vulnerability in the two groups.

Cardiac MRI and CT are increasingly used in the diagnosis and management of patients with congenital heart disease as an imaging adjunct to echocardiography. The benefits and limitations of both modalities are highlighted, with a focus on the anatomical, functional and haemodynamic information that can be gained from the different modalities. Deciding on the imaging modality of choice must also take into account patient factors such as age, compliance, the type of congenital heart disease, and previous procedures. Future developments in CT and MRI are also discussed.

IntroductionMedical advancements have significantly improved survival of patients with Congenital Heart Disease (CHD). Consequently, an aging CHD population are more susceptible to cardiac-related complications and co-morbidities.Early intervention, with individually-tailored, patient-centred approaches targeting physical activity, diet and stress, can reduce these modifiable risks. Lifestyle Intervention Programmes (LIP) are safe, cost-effective interventions, aiming to promote positive health behaviour change. CHD LIP efficacy research is limited.ObjectiveThis study aims to explore patients’ expectations and experiences to better identify determinants of patient adherence and aspects of LIP that need developing.MethodsQualitative study using semi-structured interviews. Group 1 included seven patients who had not taken part in a LIP to establish views and expectations of LIP. Group 2 included five patients who participated in 3-month pilot LIP at Guy's and St Thomas' to gather feedback on their experience.ResultsThree key themes were identified from Group 1 data: Expectations of Lifestyle Intervention Programme and Intentions to Engage, Views about Online Support for Lifestyle Intervention Programme, Patient Anxieties about Exercise Damaging their Heart. Four key themes were identified from Group 2 data: Insufficient Dietary Information, Brilliant Intentions Marred by Barriers, Pertinence of Plans to Patients, Positive Patient Experiences and Outcomes.ConclusionsThis study provides new insight into beliefs, expectations and experiences of patients with CHD. Findings support the value of LIP for CHD and inform recommendations for LIP development including need for health psychology, dietetic support, more education, greater focus on techniques to address barriers to adherence and scope for specifically-designed App.

Background: Myocardial ischemia occurs in pediatrics, as a result of both congenital and acquired heart diseases, and can lead to further adverse cardiac events if untreated. The aim of this work is to assess the feasibility of fully automated, high resolution, quantitative stress myocardial perfusion cardiac magnetic resonance (CMR) in a cohort of pediatric patients and to evaluate its agreement with the coronary anatomical status of the patients.Methods: Fourteen pediatric patients, with 16 scans, who underwent dual-bolus stress perfusion CMR were retrospectively analyzed. All patients also had anatomical coronary assessment with either CMR, CT, or X-ray angiography. The perfusion CMR images were automatically processed and quantified using an analysis pipeline previously developed in adults.Results: Automated perfusion quantification was successful in 15/16 cases. The coronary perfusion territories supplied by vessels affected by a medium/large aneurysm or stenosis (according to the AHA guidelines), induced by Kawasaki disease, an anomalous origin, or interarterial course had significantly reduced myocardial blood flow (MBF) (median (interquartile range), 1.26 (1.05, 1.67) ml/min/g) as compared to territories supplied by unaffected coronaries [2.57 (2.02, 2.69) ml/min/g, p < 0.001] and territories supplied by vessels with a small aneurysm [2.52 (2.45, 2.83) ml/min/g, p = 0.002].Conclusion: Automatic CMR-derived MBF quantification is feasible in pediatric patients, and the technology could be potentially used for objective non-invasive assessment of ischemia in children with congenital and acquired heart diseases.

Overlapping clinical features promoted the discussion of whether Kawasaki disease (KD) and PIMS-TS share pathophysiological features and disease outcomes. Medical records from English patients with KD (2015-02/20, N = 27) and PIMS-TS (02/2020-21, N = 34) were accessed to extract information. Children with PIMS-TS were older and more frequently of minority ethnicity background. They patients more commonly exhibited cytopenias and hyperferritinemia, which associated with diffuse cardiac involvement and functional impairment. In some PIMS-TS cases, cardiac pathology developed late, but outcomes were more favorable. In both, KD and PIMS-TS, baseline coronary diameter was a predictor of outcomes. PIMS-TS treatment more frequently included respiratory and cardiovascular support, and corticosteroids with IVIG. Cardiac involvement in PIMS-TS may be the result of a cytokine storm. Though more severe and diffuse when compared to KD, cardiac involvement of PIMS-TS has a more favorable prognosis, which may, after recovery, mitigate the need for long-term follow up.

[Background] Improvements in outcomes for patients with congenital heart disease (CHD) have increased the need for diagnostic and interventional procedures. Cumulative radiation risk is a growing concern. MRI-guided interventions are a promising ionizing radiation-free, alternative approach., [Purpose] To assess the feasibility of MRI-guided catheterization in young patients with CHD using advanced visualization passive tracking techniques., [Study Type] Prospective., [Population] A total of 30 patients with CHD referred for MRI-guided catheterization and pulmonary vascular resistance analysis (median age/weight: 4 years / 15 kg)., [Field Strength/Sequence] 1.5T; partially saturated (pSAT) real-time single-shot balanced steady-state free-precession (bSSFP) sequence., [Assessment] Images were visualized by a single viewer on the scanner console (interactive mode) or using a commercially available advanced visualization platform (iSuite, Philips). Image quality for anatomy and catheter visualization was evaluated by three cardiologists with >5 years' experience in MRI-catheterization using a 1–5 scale (1, poor, 5, excellent). Catheter balloon signal-to-noise ratio (SNR), blood and myocardium SNR, catheter balloon/blood contrast-to-noise ratio (CNR), balloon/myocardium CNR, and blood/myocardium CNR were measured. Procedure findings, feasibility, and adverse events were recorded. A fraction of time in which the catheter was visible was compared between iSuite and the interactive mode., [Statistical Tests] T-test for numerical variables. Wilcoxon signed rank test for categorical variables., [Results] Nine patients had right heart catheterization, 11 had both left and right heart catheterization, and 10 had single ventricle circulation. Nine patients underwent solely MRI-guided catheterization. The mean score for anatomical visualization and contrast between balloon tip and soft tissue was 3.9 ± 0.9 and 4.5 ± 0.7, respectively. iSuite provided a significant improvement in the time during which the balloon was visible in relation to interactive imaging mode (66 ± 17% vs. 46 ± 14%, P, [Data Conclusion] MRI-guided catheterizations were carried out safely and is feasible in children and adults with CHD. The pSAT sequence offered robust and simultaneous high contrast visualization of the catheter and cardiac anatomy.

The novel Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) is the pathogen responsible for Coronavirus Disease 2019 (COVID-19). Whilst most children and young people develop mild symptoms, recent reports suggest a novel paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS). Case definition and classification are preliminary, treatment is empiric and disease-associated outcomes are unclear. Here, we report 29 patients with PIMS-TS who were diagnosed, admitted and treated in the English North West between March and June 2020. Consistent with patterns observed internationally, cases peaked approximately 4 weeks after the initial surge of COVID-19-like symptoms in the UK population. Clinical symptoms included fever (100%), skin rashes (72%), cardiovascular involvement (86%), conjunctivitis (62%) and respiratory involvement (21%). Some patients had clinical features partially resembling Kawasaki disease (KD), toxic shock syndrome and cytokine storm syndrome. Male gender (69%), black, Asian and other minority ethnicities (BAME, 59%) were over-represented. Immune modulating treatment was used in all, including intravenous immunoglobulin (IVIG), corticosteroids and cytokine blockers. Notably, 32% of patients treated with IVIG alone went into remission. The rest required additional treatment, usually corticosteroids, with the exception of two patients who were treated with TNF inhibition and IL-1 blockade, respectively. Another patient received IL-1 inhibition as primary therapy, with associated rapid and sustained remission. Randomized and prospective studies are needed to investigate efficacy and safety of treatment, especially as resources of IVIG may be depleted secondary to high demand during future waves of COVID-19.

Background: The aim of the study was to document cardiovascular clinical findings, cardiac imaging and laboratory markers in children presenting with the novel multisystemic inflammatory syndrome associated with COVID-19. Methods: A real-time internet based survey was sent via the member mailing database for Association for European Paediatric and Congenital Cardiologists (AEPC) working groups for Cardiac Imaging and Cardiovascular Intensive Care member. Inclusion criteria was children 0-18 years admitted to hospital between March 1 and June 6, 2020 with diagnosis of an inflammatory syndrome and acute cardiovascular complications. Findings: A total of 286 children from 55 centres from 17 European countries were included. The median age was 8·4 years (IQR 3·8-12·4 years) and 67% were males. Most common cardiovascular complications were shock (40%), cardiac arrhythmias (35%), pericardial effusion (28%) and coronary artery dilatation (24%). Reduced left ventricular ejection fraction was present in 52% of patients and 93% had raised cardiac troponin (cTnT). The biochemical markers of inflammation were raised in majority of patients on admission: elevated CRP (99%), ferritin (79%), procalcitonin (96%), NT-proBNP (93%), IL-6 level (88%) and D-dimers (90%). There was a statistically significant correlation between degree of elevation in cardiac and biochemical parameters and need of intensive care support (p

Superior sinus venosus atrial septal defect is a communication between the two atria located above the upper margin of the oval fossa, immediately caudal to the junction of the superior vena cava and the right atrium. Commonly, it is associated with partial anomalous pulmonary venous drainage, particularly of the right upper pulmonary vein. Surgical repair has been the gold-standard approach to close this congenital anomaly, with excellent results. However, some recent studies have shown promising results from a trans-catheter approach by placing a covered stent in the superior vena cava to close the defect and redirect the anomalous pulmonary venous return posterior to the stent and into the left atrium. This procedure has proven effective and feasible in selected patients. Additionally, the development of a percutaneous approach has opened the possibility of treating patients with contraindications to surgical correction. Procedural planning using 3D printed models, simulation, holographic augmented reality, and interrogation of the superior vena cava with a balloon has made it possible to increase the effectiveness and safety of the transcatheter correction of this type of atrial septal defects. Based on our experience and literature data, transcatheter correction of sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage may be an alternative to surgery in adult patients and would reduce the need for cardiopulmonary bypass. The short and medium-term results are promising, but longer follow-up studies on the use of this technique are needed to validate the procedure.

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.

Retrograde systolic flow in the main pulmonary artery (PA) is a hallmark echocardiographic sign of patent ductus arteriosus. We describe a case of PA aneurysm mimicking patent ductus arteriosus flow. Using 4-dimensional flow cardiac magnetic resonance imaging, this echocardiographic feature is explained and altered flow dynamics in the aneurysmal PA are visualized. (Level of Difficulty: Intermediate.), Graphical abstract, Retrograde systolic flow in the main pulmonary artery (PA) is a hallmark echocardiographic sign of patent ductus arteriosus. We describe a case of PA…

Background The superior sinus venosus atrial septal defect (SVASD) is characterized by deficiency of the common wall between the superior vena cava (SVC) and the right upper pulmonary vein (RUPV), which is no longer committed to the left atrium. Objectives This study sought to evaluate the potential for redirecting the SVC and RUPV flow to the right and left atria, respectively, by implantation of a covered stent in the SVC. Methods Review of 48 consecutive adult SVASD patients undergoing assessment for correction. Pre-procedural evaluation included cross-sectional imaging and ex vivo simulation using printed or virtual 3-dimensional models. Results Transcatheter correction was performed in 25 patients, with a further 6 awaiting stent implantation. Only 8 patients were deemed technically unsuitable. The procedure involved balloon test inflation in the anticipated stent landing zone with simultaneous transesophageal echocardiography and pulmonary venography to confirm defect closure and unobstructed pulmonary venous drainage, followed by deployment of a 10-zig covered Cheatham platinum stent. Stents of lengths between 5 and 8 cm were implanted. A second, uncovered stent was used for anchoring in 9 patients. The RUPV was protected with a high-pressure balloon during stent implantation to prevent pulmonary venous obstruction in 4 patients. The median follow-up period was 1.4 (interquartile range: 0.8 to 1.7) years, with no mortality. Stent embolization occurred in 1 patient; another required drainage of hemopericardium. Cardiac computed tomography after 3 months confirmed unobstructed pulmonary venous return. At latest follow-up, a residual shunt was present in 1 patient. Conclusions Transcatheter correction of SVASD may be considered as an alternative to surgery in a substantial proportion of patients.

The main criteria used for deciding on surgery in children with presumed antenatally detected pelviureteric junction obstruction (PPUJO) are the level of hydronephrosis (ultrasonography), the level of differential renal function (DRF) and the quality of renal drainage after a furosemide challenge (renography), the importance of each factor being far from generally agreed. Can we predict, on the basis of ultrasound parameters, the patient in whom radionuclide renography can be avoided?We retrospectively analysed the medical charts of 81 consecutive children with presumed unilateral PPUJO detected antenatally. Ultrasound and renographic studies performed at the same time were compared. Anteroposterior pelvic diameter (APD) and calyceal size were both divided into three levels of dilatation. Parenchymal thickness was considered either normal or significantly decreased. Acquisition of renograms under furosemide stimulation provided quantification of DRF, quality of renal drainage and cortical transit.The percentages of patients with low DRF and poor drainage were significantly higher among those with major hydronephrosis, severe calyceal dilatation or parenchymal thinning. Moreover, impaired cortical transit, which is a major risk factor for functional decline, was seen more frequently among those with very severe calyceal dilatation. However, none of the structural parameters obtained by ultrasound examination was able to predict whether the level of renal function or the quality of drainage was normal or abnormal. Alternatively, an APD30 mm, a calyceal dilatation of10 mm and a normal parenchymal thickness were associated with a low probability of decreased renal function or poor renal drainage.In the management strategy of patients with prenatally detected PPUJO, nuclear medicine examinations may be postponed in those with an APD30 mm, a calyceal dilatation of10 mm and a normal parenchymal thickness. On the contrary, precise estimation of DRF and renal cortical transit should be performed in patients with APD30 mm, major calyceal dilatation and/or parenchymal thinning.

The main purposes of this study are to demonstrate the synergistic anticancer drug systems with the combined doxorubicin (D) and paclitaxel (P) via the aid of cell penetrating and cell targeting moieties for enhancing the cancer therapeutic effect. Firstly, the synergistic effect of combined free drugs (D/P) was investigated to obtain the suitable dose combination for subsequent studies. The combination of free drugs D/P at molar ratio of 1/0.2 shows synergistic therapeutic effect compared with the treatment of a free single drug D or P. Secondly, sustainable release systems of two single drug-loaded micelles, (i) co-delivered D-FOL micelle & P-FOL micelle system and (ii) co-delivered D-TAT/FOL micelle & P-TAT/FOL micelle system, at D/P molar ratio of 1/0.2 were investigated. The results show synergistic effect with the higher efficacy of the TAT/FOL system compared to FOL only system. Finally, a dual D/P-loaded system with sustainable release rate, synergistic drug interaction, selective targeting to cancer cells and high cell penetrating ability was designed. The D/P-TAT/FOL micelles exhibit an IC50 value of 0.172 μM D/0.043 μM P, which is much lower than the IC50 values of the single drug-loaded micelles without functionalization (3.873 μM for D-micelles and 0.790 μM for P-micelles). Overall, this newly developed dual encapsulation of D and P in the multifunctional carrier would be a promising technology for cancer treatment.

Aim Data on arrhythmia outcome following device closure of atrial septal defect (ASD) are lacking. This study provides medium-term follow-up data on atrial arrhythmias in patients who were ≥40 years of age at the time of transcatheter ASD closure. Methods and results It is a retrospective review. Mean age of the 159 patients was 57 years. Median follow-up was 3.6 years (range 6 months–10.9 years). Patients were classified, according to arrhythmia status prior to ASD closure, into Group I, no history of atrial arrhythmia ( n = 119, mean age 55.5 years); Group II, paroxysmal atrial arrhythmia ( n = 18, mean age 55.7 years); and Group III, persistent atrial fibrillation ( n = 22, mean age 65.7 years). Group III patients were significantly older, had larger left atrial size, and had higher mean pulmonary arterial pressure than Group I and II patients ( P < 0.001). Prior to closure, radiofrequency ablation was carried out in 12/18 (66%) of Group II and 3/22 (14%) of Group III. After device closure, 7 patients (6%) of Group I developed new atrial fibrillation. Fifty per cent (9/18) of Group II but only 9% (2/22) of Group III were in sinus rhythm on follow-up. Conclusion Device closure alone in patients with persistent atrial arrhythmia is not likely to restore sinus rhythm in the medium term. New atrial arrhythmia occurred in 6% of patients who were in sinus rhythm prior to device closure. At least 50% of the patients with paroxysmal atrial arrhythmia continue to have significant atrial arrhythmia following device closure, and the role of ablation prior to closure in patients with a history of arrhythmia requires refinement.

Background Mechanical circulatory support in the pediatric population is currently limited to pulsatile ventricular assist devices (VAD). In recent years, the use of durable, newer generation, continuous flow devices have increased substantially among adults with end-stage heart failure. We examined the extended role of this device in the pediatric population (aged less than 18 years). Methods Between 2010 and 2015, 12 patients (median age 7.1 years; range, 3.7 to 17.0; one third of patients were aged 5 years or less) received a HeartWare ventricular assist device (HVAD; HeartWare, Framingham, MA), 11 for cardiomyopathy and 1 for posttransplant rejection. Right VAD support (n = 5; 42%) was provided by a short-term device (Levitronix, Zurich, Switzerland). Results Overall, 1 patient died (day 638), 8 patients (67%) underwent transplantation, 1 patient (8.3%) recovered, and 2 patients (17%) remain on HVAD. The mean length of support was 150 days (range, 16 to 638). Four patients (33.3%) were discharged home (all left VAD). In the left VAD group (n = 7), 3 patients subsequently received transplants (days 185, 201, and 234, respectively), 1 recovered (day 149), 1 died (day 638), 1 remained on HVAD (day 198), and 1 needed conversion to biventricular assist device (BIVAD [day 73]). In the BIVAD group (n = 5), right VAD was weaned in 3 (60%), all subsequently received transplants, and 2 remained on BIVAD support until transplant (days 16 and 17, respectively). One BIVAD patient required conversion to central cannulation for longer-term support. Four BIVAD patients (80%) were in Interagency Registry for Mechanically Assisted Circulatory Support level 1 before VAD compared with 2 (29%) in the left VAD group ( p = not significant). The actuarial survival rate was 100% at 1 year with no neurologic events. Conclusions The third-generation, continuous flow device can provide durable support in the pediatric population. The selection strategy for patients who benefit most from the device continues to evolve. It is anticipated that a smaller design in the future will benefit an even wider pediatric population with heart failure.

Objective: When taking into account the Society of Nuclear Medicine (SNM) and European Association of Nuclear Medicine (EANM) guidelines on diuretic renography in children, we see important divergences in the preparation, acquisition, processing, and interpretation of the results. In this study, we estimated the quality of renal drainage according to these two guidelines. Patients and methods: Ninety kidneys with hydronephrosis or severe vesicorenal reflux were processed and analyzed according to the SNM (estimation of the T1/2 of the washout curve and conclusions about the possible existence of obstruction) and EANM guidelines (estimation of normalized residual activity or NORA, and output efficiency or OE on the late postmicturition images, without any conclusion about the possible existence of obstruction). Among the 90 kidneys, 39, 20, and 31 were considered to have normal, equivocal, or poor drainage (obstruction), respectively, when the SNM guidelines were used. Results: All 39 kidneys with good drainage according to SNM were also considered as normal using the EANM guidelines. Among the 20 equivocal cases based on T1/2, concordance between the two guidelines was obtained in only 35 and 0% when using NORA and OE (OE was considered normal in all 20 cases), respectively. Among the 31 cases defined as obstructed by SNM, only five were classified as 'poor drainage' using NORA and no single case was classified thus using OE. Conclusion: This limited study demonstrates that the use of different guidelines can lead to different interpretations of the results. This is unacceptable, and an agreement between both pediatric task groups of the SNM and EANM is urgently required.

Background We describe our current unit approach and report interstage results in the HLHS with Intact atrial septum (IAS) or highly restrictive interatrial communication (HRIC). Method A retrospective review of the institutional HLHS programme (2005–2014). Advances in fetal diagnosis lead to delivery planning in hybrid theatre: median sternotomy and interventional defect creation/enlargement. Results 9 neonates (4 IAS and 5 HRIC) and 5 required immediate intervention postnatally. Delayed/insufficient septostomy in 3 HRIC resulted in death before stage I Norwood even with ECMO support in 2/3. Of the other 6, 2 had trans-atrial stent placement and 1 trans-atrial balloon septostomy (3 had surgical septectomy). 5/6 underwent concomitant bilateral pulmonary artery banding. All the 6 patients reached the Norwood procedure after 27 ± 21 days and 50% required ECMO postoperatively. Trans-atrial stenting resulted in less pre Norwood morbidities. There was no stent-related complications. There was no hospital mortality after Norwood and current inter-stage survival is 100%: 5 patients underwent successfully second-stage palliation, 1 of them had the Fontan completion and subsequently transplanted. Conclusion Together with advanced fetal diagnosis, effective left atrial decompression especially with trans-atrial stunting using hybrid technique and availability of mechanical support can improve the outcome of HLHS/IAS/HRIC with low interstage morbidity and excellent survival.