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Fetal critical aortic stenosis with evolving hypoplastic left heart syndrome (CAS-eHLHS) causes biomechanical and functional aberrations, leading to a high risk of progression to hypoplastic left heart syndrome (HLHS) at birth. Fetal aortic valvuloplasty (FAV) can resolve outflow obstruction and may reduce progression risk. However, it is currently difficult to accurately predict which patients will respond to the intervention and become functionally biventricular (BV) at birth, as opposed to becoming functionally univentricular (UV). This prediction is important for patient selection, parental counselling, and surgical planning. Therefore, we investigated whether biomechanics parameters from pre-FAV image-based computations could robustly distinguish between CAS-eHLHS cases with BV or UV outcomes in a retrospective cohort. To do so we performed image-based finite element biomechanics modelling of nine CAS-eHLHS cases undergoing intervention and six healthy fetal control hearts, and found that a biomechanical parameter, peak systolic myofibre stress, showed a uniquely large difference between BV and UV cases, which had a larger magnitude effect than echocardiography parameters. A simplified equation was derived for quick and easy estimation of myofibre stress from echo measurements via principal component analysis. When tested on a retrospective cohort of 37 CAS-eHLHS cases, the parameter outperformed other parameters in predicting UV versus BV outcomes, and thus has a high potential of improving outcome predictions, if incorporated into patient selection procedures. Physiologically, high myocardial stresses likely indicate a healthier myocardium that can withstand high stresses and resist pathological remodelling, which can explain why it is a good predictor of BV outcomes. KEY POINTS: Predicting the morphological birth outcomes (univentricular versus biventricular) of fetal aortic valvuloplasty for fetal aortic stenosis with evolving HLHS is important for accurate patient selection, parental counselling and management decisions. Computational simulations show that a biomechanics parameter, pre-intervention peak systolic myofibre stress, is uniquely robust in distinguishing between such outcomes, outperforming all echo parameters. An empirical equation was developed to quickly compute peak systolic myofibre stress from routine echo measurements and was the best predictor of outcomes among a wide range of parameters tested.
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Estenose da Valva Aórtica , Síndrome do Coração Esquerdo Hipoplásico , Recém-Nascido , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/terapia , Síndrome do Coração Esquerdo Hipoplásico/etiologia , Estudos Retrospectivos , Estenose da Valva Aórtica/diagnóstico por imagem , Coração Fetal , MiocárdioRESUMO
BACKGROUND: In 2020, reports revealed cases called multisystem inflammatory syndrome in children and adolescents temporally related to COVID-19 or multisystem inflammatory syndrome in children. A small proportion of patients suffer from persistent left ventricular dysfunction at discharge. The primary aim was to investigate if myocardial impairment persists during follow-up in these patients. METHODS: Children fulfilling the criteria for multisystem inflammatory syndrome in children with cardiac involvement hospitalized between December 2020 and February 2022 were included in this retrospective single centre study. Cardiac MRI was performed six months after the onset of symptoms to evaluate possible persistent myocardial damage. RESULTS: Fifteen patients (80% male) with a median age of 8 years (interquartile range 4.5 - 13.5 years) were included. Upon admission, eight patients (53%) presented with reduced left ventricular function, with a median left ventricular ejection fraction of 54% (interquartile range 49.5%-61.5%) on transthoracic echocardiography. Elevated levels of cardiac-specific markers were found in 14 patients (93%). Cardiac MRI was performed in 12 patients at a median of 190.5 days after the onset of symptoms. Nine patients (75%) had normal left ventricular function, with a median left ventricular ejection fraction of 59.45%, while the remaining patients showed mildly to moderately reduced values. None of the patients showed signs of late gadolinium enhancement, indicating the absence of persistent myocardial scarring. CONCLUSION: During a follow-up of 6.2 months, mild to moderate cardiac impairment was revealed in 25% of patients evaluated by cardiac MRI. Although a majority of patients do not show signs of cardiac impairment, close follow-ups should be performed in a proportion of patients.
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COVID-19 , Doenças do Tecido Conjuntivo , Síndrome de Resposta Inflamatória Sistêmica , Criança , Humanos , Masculino , Adolescente , Pré-Escolar , Feminino , COVID-19/complicações , Seguimentos , Estudos Retrospectivos , Volume Sistólico , Meios de Contraste , Função Ventricular Esquerda , Gadolínio , Imageamento por Ressonância MagnéticaRESUMO
Fetuses with critical aortic stenosis, severe mitral regurgitation and left atrial enlargement are at risk to develop heart failure and intrauterine death. We present two cases with this physiology who underwent fetal aortic balloon valvuloplasty at 25 + 5 and 28 + 2 weeks as their only intervention without requiring postnatal treatment during short- to medium-term follow-up of 1.4 and 4.9 years.
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Estenose da Valva Aórtica , Valvuloplastia com Balão , Insuficiência da Valva Mitral , Feminino , Gravidez , Humanos , Seguimentos , Insuficiência da Valva Mitral/complicações , Insuficiência da Valva Mitral/diagnóstico , Feto , Estenose da Valva Aórtica/complicações , Estenose da Valva Aórtica/diagnóstico , Estenose da Valva Aórtica/cirurgia , Vitaminas , Átrios do Coração , Resultado do TratamentoRESUMO
Lymphatic flow disorders are rare but devastating complications in children with congenital heart disease. T2-weighted magnetic resonance lymphography and intranodal dynamic contrast magnetic resonance lymphangiography are imaging modalities that can depict central lymphatic anatomy and flow pattern. Our objective was to describe the technical aspects and our imaging findings of central lymphatic abnormalities and their impact on patient management and outcomes: We conducted a retrospective review of 26 children with congenital heart disease who presented for lymphatic imaging between 2015 and 2020 at our institution. Eleven had postoperative chylothorax, six had plastic bronchitis, seven had protein-losing enteropathy and three had Noonan syndrome. Our lymphatic imaging demonstrated severely abnormal lymphatic flow in all of the children, but only minor abnormalities in protein-losing enteropathy. No major procedure-related complication occurred. Lymphatic interventions were performed in six patients, thoracic duct decompression in two patients and chylothorax revision in three patients. This led to symptomatic improvements in all of the patients: Lymphatic imaging is safe and essential for the diagnosis of lymphatic flow disorders and therapy planning. Our intranodal lymphangiography depicts an abnormal lymphatic flow pattern from the central lymphatics but failed to demonstrate an abnormal lymphatic flow in protein-losing enteropathy. These imaging techniques are the basis for selective lymphatic interventions, which are promising to treat lymphatic flow disorders.
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Quilotórax , Cardiopatias Congênitas , Enteropatias Perdedoras de Proteínas , Humanos , Criança , Linfografia/métodos , Quilotórax/diagnóstico por imagem , Quilotórax/terapia , Quilotórax/complicações , Sistema Linfático/patologia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Imageamento por Ressonância Magnética/métodosRESUMO
INTRODUCTION: Fetal cardiac interventions (FCIs) were introduced to change the natural history of some congenital heart defects. The aim of this study was to analyze the complications and management strategies associated with FCI at our institution. METHODS: The local FCI database was retrospectively reviewed for all fetuses who underwent FCI in our center since 2000 regarding complications and fetal outcome. RESULTS: 213 FCIs have been performed in 165 fetuses since October 2000: 60 with fetal pulmonary valvuloplasty, 4 with atrial septostomy, 7 with atrial septal stents, and 142 with fetal aortic valvuloplasty (FAV). The median gestational age at intervention was 27+1/7 weeks (21+4/7-38+3/7) for all interventions. The most common complications needing treatment were bradycardia (37%) and pericardial effusions (12%). FAV procedure-related mortality was significantly lower in the recent period since 2014 (14% early vs. 4% recent era, p = 0.03) due to a learning curve and improved management strategies. There were no relevant maternal complications. Premature deliveries occurred in 22.8% percent of all patients. CONCLUSIONS: Complications during FCI were frequent and must be expected. Strategies to reduce their prevalence as well as timely and correct treatment are mandatory to keep mortality rates low.
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Cardiopatias Congênitas , Ultrassonografia Pré-Natal , Gravidez , Feminino , Humanos , Lactente , Estudos Retrospectivos , Prevalência , Cardiopatias Congênitas/epidemiologia , Cardiopatias Congênitas/cirurgia , Coração Fetal/diagnóstico por imagemRESUMO
INTRODUCTION: Aetiology of protein-losing enteropathy in single-ventricle type CHD is multi-factorial. REPORT: We describe two Fontan patients with protein-losing enteropathy who presented with cytomegalovirus-associated colitis. DISCUSSION: Fontan patients display risk factors for cytomegalovirus-induced gastroenteropathy that may affect lymph angiogenesis, disease development, and progression. CONCLUSION: Cytomegalovirus enteropathy may be common among Fontan patients who suffer from protein-losing enteropathy. Polymerase chain reaction is important for detection.
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Colite/etiologia , Infecções por Citomegalovirus/diagnóstico , Técnica de Fontan/efeitos adversos , Enteropatias Perdedoras de Proteínas/etiologia , Criança , Pré-Escolar , Colite/virologia , Infecções por Citomegalovirus/complicações , Cardiopatias Congênitas/cirurgia , Humanos , Masculino , Complicações Pós-Operatórias , Enteropatias Perdedoras de Proteínas/virologia , Fatores de RiscoRESUMO
Data from neurological and radiological research show an abnormal neurological development in patients treated for hypoplastic left heart syndrome. Thus, the aim of this study was to survey the quality of life scores in comparison with healthy children and children with other heart diseases (mild, moderate, and severe heart defects, heart defects in total). Children with hypoplastic left heart syndrome (aged 6.3-16.9 years) under compulsory education requirements, who were treated at the Children's Heart Center Linz between 1997 and 2009 (n = 74), were surveyed. Totally, 41 children and 44 parents were examined prospectively by psychologists according to Pediatric Quality of Life Inventory, a health-related quality of life measurement. The results of the self-assessments of health-related quality of life on a scale of 1-100 showed a wide range, from a minimum of 5.00 (social functioning) to a maximum of 100 (physical health-related summary scores, emotional functioning, school functioning), with a total score of 98.44. The parents' assessments (proxy) were quite similar, showing a range from 10 (social functioning) up to 100. Adolescent hypoplastic left heart syndrome patients rated themselves on the same level as healthy youths and youths with different heart diseases. The results show that patients with hypoplastic left heart syndrome aged 6-16 years can be successfully supported and assisted in their psychosocial development even if they show low varying physical and psychosocial parameters. The finding that adolescent hypoplastic left heart syndrome patients estimated themselves similar to healthy individuals suggests that they learnt to cope with a severe heart defect.
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Adaptação Psicológica , Nível de Saúde , Síndrome do Coração Esquerdo Hipoplásico/psicologia , Qualidade de Vida/psicologia , Instituições Acadêmicas , Adolescente , Criança , Feminino , Seguimentos , Humanos , Masculino , Estudos Retrospectivos , Inquéritos e QuestionáriosRESUMO
Neurological and radiologic research results show an abnormal cerebral microstructure as well as abnormal neurodevelopment in patients treated for hypoplastic left heart syndrome. The aim of this study was to assess the varying cognitive performance these children have developed in dependence upon prenatal diagnosis, surgical techniques, surgical learning effects, anatomy, perfusion techniques, gender, pedagogic, and sociodemographic parameters in comparison to age-adjusted normative values. School-age children (6.3-16.9 years) with hypoplastic left heart syndrome, who were treated at the Children's heart Center Linz between 1997 and 2009, (n = 74), were surveyed in reference to cognitive achievements. 43 patients were examined prospectively by psychologists using the Wechsler intelligence scale for children IV in order to determine the respective total intelligence quotient index for each child's developmental stage. The mean index was 84.5 (percentile rank 26.4). The statistical spread and standard deviation ranged from a minimum of 40 to a maximum of 134 ± 20.8. The results for verbal comprehension, perceptual reasoning, and processing speed corresponded with total index results and were thus lower than the mean value of the normative values. The assessment of working memory showed results in the average. Prenatal diagnosis, type of lung perfusion, anatomy, and various cerebral perfusion techniques did not significantly affect the cognitive results of the patients. The results show that hypoplastic left heart syndrome patients can be successfully tutored formally as well as personally in cognitive areas, although when compared to healthy children, they showed lower results for intellectual area parameters.
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Transtornos Cognitivos/diagnóstico , Síndrome do Coração Esquerdo Hipoplásico/complicações , Transtornos do Neurodesenvolvimento/diagnóstico , Adolescente , Criança , Transtornos Cognitivos/etiologia , Feminino , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Testes de Inteligência , Masculino , Transtornos do Neurodesenvolvimento/etiologia , Testes Neuropsicológicos , Procedimentos de Norwood/métodosRESUMO
OBJECTIVES: The standard surgical management of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis is the Rastelli operation. Recently, en bloc rotation of the arterial trunk, by cutting out the aortic and the pulmonary root in one block and by rotating it 180°, has been introduced as a new option for anatomical repair. METHODS: To evaluate the effects of this surgical method on the conduction system, pre-operative, post-operative, and follow-up electrocardiograms as well as patient charts were reviewed retrospectively. A total of 16 consecutive patients with transposition of the great arteries and left outflow tract obstruction were treated with en bloc rotation. RESULTS: During the post-operative period, there were two patients with complete atrio-ventricular block, one with junctional ectopic tachycardia, one with ventricular tachycardia, and one with supraventricular tachycardia. None of the patients had a typical right bundle branch block pattern before surgery; however, this pattern was detectable after surgery in eight out of 16 patients (50%), which persisted during the follow-up. All patients without typical right bundle branch block pattern showed a median QRS duration of 65 ms (54-112 ms) before surgery, 62 ms (54-122 ms) after surgery, and 84 ms (66-128 ms) at the last follow-up visit. This compares well with a similar Rastelli cohort, where a right bundle branch block prevalence of 77% was reported. Out of 16 patients, 12 showed non-specific ST changes and negative T-waves, which persisted during follow-up with an unknown significance for the future. CONCLUSION: Our data suggest that en bloc rotation of the arterial trunk seems not to have more negative effects on the conduction system than the Rastelli operation.
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Síndrome de Brugada/fisiopatologia , Procedimentos Cirúrgicos Cardíacos/métodos , Comunicação Interventricular/cirurgia , Complicações Pós-Operatórias , Estenose da Valva Pulmonar/cirurgia , Transposição dos Grandes Vasos/cirurgia , Obstrução do Fluxo Ventricular Externo/cirurgia , Anormalidades Múltiplas/cirurgia , Aorta/cirurgia , Transposição das Grandes Artérias , Áustria , Doença do Sistema de Condução Cardíaco , Criança , Pré-Escolar , Eletrocardiografia , Ventrículos do Coração/cirurgia , Humanos , Lactente , Estudos Retrospectivos , Resultado do Tratamento , Tronco Arterial/cirurgiaRESUMO
Fetal critical aortic stenosis with evolving hypoplastic left heart syndrome (CAS-eHLHS) can progress to a univentricular (UV) birth malformation. Catheter-based fetal aortic valvuloplasty (FAV) can resolve stenosis and reduce the likelihood of malformation progression. However, we have limited understanding of the biomechanical impact of FAV and subsequent LV responses. Therefore, we performed image-based finite element (FE) modeling of 4 CAS-eHLHS fetal hearts, by performing iterative simulations to match image-based characteristics and then back-computing physiological parameters. We used pre-FAV simulations to conduct virtual FAV (vFAV) and compared pre-FAV and post-FAV simulations. vFAV simulations generally enabled partial restoration of several physiological features toward healthy levels, including increased stroke volume and myocardial strains, reduced aortic valve (AV) and mitral valve regurgitation (MVr) velocities, reduced LV and LA pressures, and reduced peak myofiber stress. FAV often leads to aortic valve regurgitation (AVr). Our simulations showed that AVr could compromise LV and LA depressurization but it could also significantly increase stroke volume and myocardial deformational stimuli. Post-FAV scans and simulations showed FAV enabled only partial reduction of the AV dissipative coefficient. Furthermore, LV contractility and peripheral vascular resistance could change in response to FAV, preventing decreases in AV velocity and LV pressure, compared with what would be anticipated from stenosis relief. This suggested that case-specific post-FAV modeling is required to fully capture cardiac functionality. Overall, image-based FE modeling could provide mechanistic details of the effects of FAV, but computational prediction of acute outcomes was difficult due to a patient-dependent physiological response to FAV.
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The lymphatic system plays a central role in some of the most devastating complications associated with congenital heart defects. Diseases like protein-losing enteropathy, plastic bronchitis, postoperative chylothorax, and chylous ascites are now proven to be lymphatic in origin. Novel imaging modalities, most notably, noncontrast magnetic resonance lymphangiography and dynamic contrast-enhanced magnetic resonance lymphangiography, can now depict lymphatic anatomy and function in all major lymphatic compartments and are essential for modern therapy planning. Based on the new pathophysiologic understanding of lymphatic flow disorders, innovative minimally invasive procedures have been invented during the last few years with promising results. Abnormal lymphatic flow can now be redirected with catheter-based interventions like thoracic duct embolization, selective lymphatic duct embolization, and liver lymphatic embolization. Lymphatic drainage can be improved through surgical or interventional techniques such as thoracic duct decompression or lympho-venous anastomosis.
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Background: Children with congenital heart defects (CHD) are at risk for a range of developmental disabilities that challenge cognition, executive functioning, self-regulation, communication, social-emotional functioning, and motor skills. Ongoing developmental surveillance is therefore key to maximizing neurodevelopmental outcome opportunities. It is crucial that the measures used cover the spectrum of neurodevelopmental domains relevant to capturing possible predictors and malleable factors of child development. Objectives: This work aimed to synthesize the literature on neurodevelopmental measures and the corresponding developmental domains assessed in children aged 1-8 years with complex CHD. Methods: PubMed was searched for terms relating to psycho-social, cognitive and linguistic-communicative outcomes in children with CHD. 1,380 papers with a focus on complex CHD that reported neurodevelopmental assessments were identified; ultimately, data from 78 articles that used standardized neurodevelopmental assessment tools were extracted. Results: Thirty-nine (50%) of these excluded children with syndromes, and 9 (12%) excluded children with disorders of intellectual development. 10% of the studies were longitudinal. The neurodevelopmental domains addressed by the methods used were: 53% cognition, 16% psychosocial functioning, 18% language/communication/speech production, and 13% motor development-associated constructs. Conclusions: Data on social communication, expressive and receptive language, speech motor, and motor function are underrepresented. There is a lack of research into everyday use of language and into measures assessing language and communication early in life. Overall, longitudinal studies are required that include communication measures and their interrelations with other developmental domains.
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Children with hypoplastic left heart syndrome share unique hemodynamic features that alter lymphatic integrity at all stages of palliation. Lymphatic congestion is almost universal in this patient group to some extent. It may lead to reversal of lymphatic flow, the development of abnormal lymphatic channels and ultimately decompression and loss of protein rich lymphatic fluid into extra lymphatic compartments in prone individuals. Some of the most devastating complications that are associated with single ventricle physiology, notably plastic bronchitis and protein losing enteropathy, have now been proven to be lymphatic in origin. Based on the new pathophysiologic concept new diagnostic and therapeutic strategies have recently been developed. Dynamic contrast magnetic resonance lymphangiography is now mainstay in diagnosis of lymphatic insufficiency and allows a thorough assessment of anatomy and function of the main lymphatic compartments through intranodal, intrahepatic and intramesenteric lymphatic imaging. Contrast enhanced ultrasound can evaluate thoracic duct patency and conventional fluoroscopic lymphangiography has been refined for evaluation of patients where magnetic resonance imaging cannot be performed. Novel lymphatic interventional techniques, such as thoracic duct embolization, selective lymphatic duct embolization and liver lymphatic embolization allow to seal abnormal lymphatic networks minimally invasive and have shown to resolve symptoms. Innominate vein turn-down procedures, whether surgical or interventional, have been designed to reduce lymphatic afterload and increase systemic preload effectively in the failing Fontan circulation. Outflow obstruction can now be managed with new microsurgical techniques that create lympho-venous anastomosis. Short term results for all of these new approaches are overall promising but evidence is sparse and long-term outcome still has to be defined. This review article aims to summarize current concepts of lymphatic flow disorders in single ventricle patients, discuss new emerging diagnostic and therapeutic strategies and point out lacks in evidence and needs for further research on this rapidly growing topic.
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Fetuses with critical aortic stenosis (FAS) are at high risk of progression to HLHS by the time of birth (and are thus termed "evolving HLHS"). An in-utero catheter-based intervention, fetal aortic valvuloplasty (FAV), has shown promise as an intervention strategy to circumvent the progression, but its impact on the heart's biomechanics is not well understood. We performed patient-specific computational fluid dynamic (CFD) simulations based on 4D fetal echocardiography to assess the changes in the fluid mechanical environment in the FAS left ventricle (LV) directly before and 2 days after FAV. Echocardiograms of five FAS cases with technically successful FAV were retrospectively analysed. FAS compromised LV stroke volume and ejection fraction, but FAV rescued it significantly. Calculations to match simulations to clinical measurements showed that FAV approximately doubled aortic valve orifice area, but it remained much smaller than in healthy hearts. Diseased LVs had mildly stenotic mitral valves, which generated fast and narrow diastolic mitral inflow jet and vortex rings that remained unresolved directly after FAV. FAV further caused aortic valve damage and high-velocity regurgitation. The high-velocity aortic regurgitation jet and vortex ring caused a chaotic flow field upon impinging the apex, which drastically exacerbated the already high energy losses and poor flow energy efficiency of FAS LVs. Two days after the procedure, FAV did not alter wall shear stress (WSS) spatial patterns of diseased LV but elevated WSS magnitudes, and the poor blood turnover in pre-FAV LVs did not significantly improve directly after FAV. FAV improved FAS LV's flow function, but it also led to highly chaotic flow patterns and excessively high energy losses due to the introduction of aortic regurgitation directly after the intervention. Further studies analysing the effects several weeks after FAV are needed to understand the effects of such biomechanics on morphological development.
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Insuficiência da Valva Aórtica , Estenose da Valva Aórtica , Síndrome do Coração Esquerdo Hipoplásico , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Estudos Retrospectivos , Estenose da Valva Aórtica/diagnóstico por imagem , FetoRESUMO
BACKGROUND: Echocardiographic quantification of fetal cardiac strain is important to evaluate function and the need for intervention, with both two-dimensional (2D) and three-dimensional (3D) strain measurements currently feasible. However, discrepancies between 2D and 3D measurements have been reported, the etiologies of which are unclear. This study sought to determine the etiologies of the differences between 2D and 3D strain measurements. METHODS: A validated cardiac motion-tracking algorithm was used on 3D cine ultrasound images acquired in 26 healthy fetuses. Both 2D and 3D myocardial strain quantifications were performed on each image set for controlled comparisons. Finite element modeling of 2 left ventricle (LV) models with minor geometrical differences were performed with various helix angle configurations for validating image processing results. RESULTS: Three-dimensional longitudinal strain (LS) was significantly lower than 2D LS for the LV free wall and septum but not for the right ventricular (RV) free wall, while 3D circumferential strain (CS) was significantly higher than 2D CS for the LV, RV, and septum. The LS discrepancy was due to 2D long-axis imaging not capturing the out-of-plane motions associated with LV twist, while the CS discrepancy was due to the systolic motion of the heart toward the apex that caused out-of-plane motions in 2D short-axis imaging. A timing mismatch between the occurrences of peak longitudinal and circumferential dimensions caused a deviation in zero-strain referencing between 2D and 3D strain measurements, contributing to further discrepancies between the 2. CONCLUSIONS: Mechanisms for discrepancies between 2D and 3D strain measurements in fetal echocardiography were identified, and inaccuracies associated with 2D strains were highlighted. Understanding of this mechanism is useful and important for future standardization of fetal cardiac strain measurements, which we propose to be important in view of large discrepancies in measured values in the literature.
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Ecocardiografia Tridimensional , Humanos , Ecocardiografia Tridimensional/métodos , Ventrículos do Coração/diagnóstico por imagem , Ecocardiografia/métodos , Função Ventricular Esquerda , Feto , Reprodutibilidade dos TestesRESUMO
During fetal life some cardiac defects may lead to diminished left heart growth and to the evolution of a form of hypoplastic left heart syndrome (HLHS). In fetuses with an established HLHS, severe restriction or premature closure of the atrial septum leads to left atrial hypertension and remodeling of the pulmonary vasculature, severely worsening an already poor prognosis. Fetal therapy, including invasive fetal cardiac interventions and non-invasive maternal hyperoxygenation, have been introduced to prevent a possible progression of left heart hypoplasia, improve postnatal outcome, or secure fetal survival. The aim of this review is to cover patient selection and possible hemodynamic effects of fetal cardiac procedures and maternal hyperoxygenation in fetuses with an evolving or established hypoplastic left heart syndrome.
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In cases of fetal aortic stenosis and evolving Hypoplastic Left Heart Syndrome (feHLHS), aortic stenosis is associated with specific abnormalities such as retrograde or bidirectional systolic transverse arch flow. Many cases progressed to hypoplastic left heart syndrome (HLHS) malformation at birth, but fetal aortic valvuloplasty can prevent the progression in many cases. Since both disease and intervention involve drastic changes to the biomechanical environment, in-vivo biomechanics likely play a role in inducing and preventing disease progression. However, the fluid mechanics of feHLHS is not well-characterized. Here, we conduct patient-specific echocardiography-based flow simulations of normal and feHLHS left ventricles (LV), to understand the essential fluid dynamics distinction between the two cohorts. We found high variability across feHLHS cases, but also the following unifying features. Firstly, feHLHS diastole mitral inflow was in the form of a narrowed and fast jet that impinged onto the apical region, rather than a wide and gentle inflow in normal LVs. This was likely due to a malformed mitral valve with impaired opening dynamics. This altered inflow caused elevated vorticity dynamics and wall shear stresses (WSS) and reduced oscillatory shear index at the apical zone rather than mid-ventricle. Secondly, feHLHS LV also featured elevated systolic and diastolic energy losses, intraventricular pressure gradients, and vortex formation numbers, suggesting energy inefficiency of flow and additional burden on the LV. Thirdly, feHLHS LV had poor blood turnover, suggesting a hypoxic environment, which could be associated with endocardial fibroelastosis that is often observed in these patients.
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Estenose da Valva Aórtica , Síndrome do Coração Esquerdo Hipoplásico , Estenose da Valva Aórtica/diagnóstico por imagem , Feminino , Coração Fetal/diagnóstico por imagem , Ventrículos do Coração/diagnóstico por imagem , Humanos , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/etiologia , Síndrome do Coração Esquerdo Hipoplásico/prevenção & controle , Recém-Nascido , Gravidez , Ultrassonografia Pré-Natal/efeitos adversosRESUMO
OBJECTIVES: Fontan patients are at lifelong risk for developing complications, which may result in Fontan failure. Survival rates after heart transplantation (HTX) are still unsatisfying in these patients. Long-term survival of extracardiac Fontan patients in the modern era was investigated. The objective of this study was to investigate if surgical and interventional procedures in patients with protein-losing enteropathy (PLE) and/or plastic bronchitis (PB) and a failing Fontan circulation can postpone or avoid HTX. METHODS: Retrospective data collection of all children who underwent a Fontan procedure between January 1999 and July 2021 at our centre was performed. Patients were surveyed regarding the occurrence of PLE or PB and their outcome was reported descriptively. HTX-free survival of patients who underwent a rescue procedure due to PLE/PB was evaluated. RESULTS: Three hundred and seventy [94.1% (95% confidence interval, 91.4-96.3)] Fontan patients were free of HTX or death at last follow-up after a median follow-up time of 6.7 years. PB/PLE was diagnosed in 34 patients during the observation period. A rescue procedure was undertaken in 16 pts. at a median time of 6.5 months (range: 1 day to 9.4 years) since the initial diagnosis of PLE/PB. In these patients, HTX-free survival was 75% (95% confidence interval, 47.6-92.7) at a median follow-up time of 4.0 years after the procedure. Range: 3.5 months to 13.9 years. CONCLUSIONS: Extracardiac Fontan patients in the modern era expect reasonable HTX-free survival rates. Surgical and/or interventional rescue strategies for Fontan failure can postpone HTX for a sustained period of time.
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Bronquite , Técnica de Fontan , Cardiopatias Congênitas , Transplante de Coração , Enteropatias Perdedoras de Proteínas , Criança , Humanos , Complicações Pós-Operatórias , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
Objective: Tetralogy of Fallot patients with pulmonary atresia (TOFPA) have a largely varying source of pulmonary perfusion with often hypoplastic and even absent central pulmonary arteries. A retrospective single center study was undertaken to assess outcome of these patients regarding type of surgical procedures, long-term mortality, achievement of VSD closure and analysis of postoperative interventions. Methods: 76 consecutive patients with TOFPA operated between 01.01.2003 and 31.12.2019 are included in this single center study. Patients with ductus dependent pulmonary circulation underwent primary single stage full correction including VSD closure and right ventricular to pulmonary conduit implantation (RVPAC) or transanular patch reconstruction. Children with hypoplastic pulmonary arteries and MAPCAs without double supply were predominantly treated by unifocalization and RVPAC implantation. The follow up period ranges between 0 and 16,5 years. Results: 31 patients (41%) underwent single stage full correction at a median age of 12 days, 15 patients could be treated by a transanular patch. 30 days mortality rate in this group was 6%. In the remaining 45 patients the VSD could not be closed during their first surgery which was performed at a median age of 89 days. A VSD closure was achieved later in 64% of these patients after median 178 days. 30 days mortality rate after the first surgery was 13% in this group. The estimated 10-year-survival rate after the first surgery is 80,5% ± 4,7% showing no significant difference between the groups with and without MAPCAs (p > 0,999). Median intervention-free interval (surgery and transcatheter intervention) after VSD closure was 1,7 ± 0,5 years [95% CI: 0,7-2,8 years]. Conclusions: A VSD closure could be achieved in 79% of the total cohort. In patients without MAPCAs this was possible at a significant earlier age (p < 0,01). Although patients without MAPCAs predominantly underwent single stage full correction at newborn age, the overall mortality rate and the interval until reintervention after VSD closure did not show significant differences between the two groups with and without MAPCAs. The high rate of proven genetic abnormalities (40%) with non-cardiac malformations did also pay its tribute to impaired life expectancy.
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OBJECTIVES: The aortic arch enlargement in the Norwood procedure is classically carried out using a curved homograft patch on the inner curvature of the neoaortic arch. The study investigates the outcome of a newly used artificial patch from a vascular prosthesis as an alternative to a homograft patch. METHODS: Since April 2007, we used curved polytetrafluorethylene (PTFE) patches cut out of a prosthesis as an alternative to homograft patches for the aortic arch reconstruction. The decision for either patch material was made due to anatomic reasons, preferring PTFE patches in larger aortas. In this study, 224 Norwood patients, operated between April 2007 and April 2018, were analysed. A total of 104 patients received a PTFE patch (group PTFE), and 120 patients got a pulmonary homograft patch (group homograft). A single-centre retrospective analysis was carried out concerning postoperative course and long-term follow-up regarding aortic arch interventions and reoperations and comparing the 2 material groups. RESULTS: There were no material associated operative or postoperative complications. In-hospital mortality was 13% in group PTFE. Six children died late during follow-up (6%). One aortic isthmus dilatation (1%) was carried out 12 months after the Norwood procedure in this group, no arch reoperation was necessary during the complete follow-up. CONCLUSIONS: The curved PTFE patch showed good qualities in operative technical demands and excellent long-term results. In selected cases of hypoplastic left heart syndrome, it can be well used as alternative to the pulmonary homograft.