RESUMO
BACKGROUND: Iron deficiency (ID) has been reported in patients with congenital heart disease. There is, however, a scarcity of data on its prevalence in patients with a Fontan circulation. The aim of this study is to investigate the prevalence of ID in Fontan patients and to investigate the association between ID and exercise capacity in this population. METHODS AND RESULTS: Blood count and haematological parameters were determined in plasma of 61 Fontan patients (51% female, mean age 29±9 years). ID was defined as transferrin saturation (TSAT) ≤19.8%. The prevalence of ID was 36% (22/61 patients). Especially among women, the diagnosis of ID was highly prevalent (52%) despite normal haemoglobin levels (153.7±18.4 g/L). Mean ferritin levels were 98±80 µg/L and mean TSAT levels were 22%±12%. Cardiopulmonary exercise testing was performed in 46 patients (75%). Patients with ID had a lower peak oxygen uptake (VÌO2peak) (1397±477 vs 1692±530 mL/min; p=0.039), although this relationship was confounded by sex. The presence of ID increased the likelihood of not achieving a respiratory exchange ratio (RER) ≥1.1 by 5-fold (p=0.035). CONCLUSION: ID is highly prevalent among patients with a Fontan circulation. VÌO2peak is lower in patients with ID. Fontan patients with ID are less likely to achieve an RER≥1.1 during cardiopulmonary exercise testing.
Assuntos
Teste de Esforço , Tolerância ao Exercício , Técnica de Fontan , Cardiopatias Congênitas , Humanos , Feminino , Masculino , Técnica de Fontan/efeitos adversos , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/sangue , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/epidemiologia , Tolerância ao Exercício/fisiologia , Adulto , Prevalência , Adulto Jovem , Biomarcadores/sangue , Anemia Ferropriva/sangue , Anemia Ferropriva/epidemiologia , Anemia Ferropriva/diagnóstico , Anemia Ferropriva/fisiopatologia , Consumo de Oxigênio/fisiologia , Ferro/sangue , Deficiências de Ferro , Adolescente , Ferritinas/sangueRESUMO
Purpose To evaluate lymphatic abnormalities before and after Fontan completion using noncontrast lymphatic imaging and relate findings with postoperative outcomes. Materials and Methods This study is a retrospective review of noncontrast T2-weighted lymphatic imaging performed at The Children's Hospital of Philadelphia from June 2012 to February 2023 in patients with single ventricle physiology. All individuals with imaging at both pre-Fontan and Fontan stages were eligible. Lymphatic abnormalities were classified into four types based on severity and location of lymphatic vessels. Classifications were compared between images and related to clinical outcomes such as postoperative drainage and hospitalization, lymphatic complications, heart transplant, and death. Results Forty-three patients (median age, 10 years [IQR, 8-11]; 20 [47%] boys, 23 [53%] girls) were included in the study. Lymphatic abnormalities progressed in 19 individuals after Fontan completion (distribution of lymphatic classifications: type 1, 23; type 2, 11; type 3, 6; type 4, 3 vs type 1, 10; type 2, 18; type 3, 10; type 4, 5; P = .04). Compared with individuals showing no progression of lymphatic abnormalities, those progressing to a high-grade lymphatic classification had longer postoperative drainage (median time, 9 days [IQR, 6-14] vs 17 days [IQR, 10-23]; P = .04) and hospitalization (median time, 13 days [IQR, 9-25] vs 26 days [IQR, 18-30]; P = .03) after Fontan completion and were more likely to develop chylothorax (12% [three of 24] vs 75% [six of eight]; P < .01) and/or protein-losing enteropathy (0% [0 of 24] vs 38% [three of eight]; P < .01) during a median follow-up of 8 years (IQR, 5-9). Progression to any type was not associated with an increased risk of adverse events. Conclusion The study demonstrated that lymphatic structural abnormalities may progress in select individuals with single ventricle physiology after Fontan completion, and progression of abnormalities to a high-grade classification was associated with worse postoperative outcomes. Keywords: Congenital Heart Disease, Glenn, Fontan, Lymphatic Imaging, Cardiovascular MRI Supplemental material is available for this article. Published under a CC BY 4.0 license.
Assuntos
Técnica de Fontan , Anormalidades Linfáticas , Imageamento por Ressonância Magnética , Humanos , Técnica de Fontan/efeitos adversos , Masculino , Feminino , Estudos Retrospectivos , Criança , Anormalidades Linfáticas/diagnóstico por imagem , Anormalidades Linfáticas/cirurgia , Anormalidades Linfáticas/patologia , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/etiologia , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/diagnóstico por imagemRESUMO
BACKGROUND: Due to their relationship with clinical progression, follow-up of exercise capacity and muscle strength is important for optimal disease management in patients who have undergone the Fontan procedure. We aimed to retrospectively analyze exercise capacity and muscle strength trajectory over approximately 2 years. METHODS: Exercise capacity was assessed using an exercise stress test with the modified Bruce protocol on a treadmill, hand grip and knee extensor strength using a hand dynamometer, and body composition using a bioelectrical impedance device. Exercise capacity, muscle strength, and body composition follow-up data recorded between 2020 and 2022 were compared. RESULTS: Fifteen patients [median age from 17 (first assessment) to 18 years (last assessment), 5 females)] with a 20-month median follow-up time were analyzed retrospectively. There was an increase in weight, height, body mass index, and body fat weight (p<0.05). There was a tendency for increased handgrip strength (%) (p=0.069), but no significant difference was observed in the knee extensor strength of patients during the follow-up period (p>0.05). The changes in heart rate (HR) and oxygen saturation were higher in the last test than in the first test (p<0.05). Maximum HR (HRmax), % predicted HRmax and HR reserve recorded during the test and HR 1 minute after the test were similar between the first and last tests (p>0.05). CONCLUSIONS: After 20 months of follow-up, exercise capacity and muscle strength did not decline; instead, the body mass index and fat weight increased. Patients who have undergone the Fontan procedure may not be experiencing a decline in exercise capacity and muscle strength over relatively short time periods during childhood, adolescence, and early adulthood.
Assuntos
Tolerância ao Exercício , Técnica de Fontan , Força Muscular , Humanos , Feminino , Masculino , Estudos Retrospectivos , Força Muscular/fisiologia , Adolescente , Seguimentos , Tolerância ao Exercício/fisiologia , Teste de Esforço , Força da Mão/fisiologia , Composição CorporalRESUMO
Previous omics research in patients with complex congenital heart disease and single-ventricle circulation (irrespective of the stage of palliative repair) revealed alterations in cardiac and systemic metabolism, inter alia abnormalities in energy metabolism, and inflammation, oxidative stress or endothelial dysfunction. We employed an affinity-proteomics approach focused on cell surface markers, cytokines, and chemokines in the serum of 20 adult Fontan patients with a good functioning systemic left ventricle, and we 20 matched controls to reveal any specific processes on a cellular level. Analysis of 349 proteins revealed 4 altered protein levels related to chronic inflammation, with elevated levels of syndecan-1 and glycophorin-A, as well as decreased levels of leukemia inhibitory factor and nerve growth factor-ß in Fontan patients compared to controls. All in all, this means that Fontan circulation carries specific physiological and metabolic instabilities, including chronic inflammation, oxidative stress imbalance, and consequently, possible damage to cell structure and alterations in translational pathways. A combination of proteomics-based biomarkers and the traditional biomarkers (uric acid, γGT, and cholesterol) performed best in classification (patient vs. control). A metabolism- and signaling-based approach may be helpful for a better understanding of Fontan (patho-)physiology. Syndecan-1, glycophorin-A, leukemia inhibitory factor, and nerve growth factor-ß, especially in combination with uric acid, γGT, and cholesterol, might be interesting candidate parameters to complement traditional diagnostic imaging tools and the determination of traditional biomarkers, yielding a better understanding of the development of comorbidities in Fontan patients, and they may play a future role in the identification of targets to mitigate inflammation and comorbidities in Fontan patients.
Assuntos
Biomarcadores , Proteínas Sanguíneas , Técnica de Fontan , Inflamação , Proteômica , Humanos , Adulto , Masculino , Inflamação/metabolismo , Feminino , Proteínas Sanguíneas/metabolismo , Técnica de Fontan/efeitos adversos , Biomarcadores/sangue , Proteômica/métodos , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/metabolismo , Cardiopatias Congênitas/sangue , Cardiopatias Congênitas/patologia , Fibrose , Adulto Jovem , Neovascularização Patológica/metabolismo , Estresse Oxidativo , AngiogêneseRESUMO
This article will detail Dr. James Tweddell's operative technique and considerations for 26 all three stages of SVP in patients with HLHS. This will include the Norwood Procedure with 27 Right-Ventricle-to-Pulmonary-Artery Conduit (Sano Modification), Bidirectional Glenn 28 (Superior Cavopulmonary Connection), and Extracardiac, Fenestrated Fontan Completion (Total 29 Cavopulmonary Connection). These techniques are the culmination of over 20 years of experience 30 and represent the final techniques he employed at both Children's Hospital of Wisconsin and 31 Cincinnati Children's Hospital Medical Center.
Assuntos
Técnica de Fontan , Síndrome do Coração Esquerdo Hipoplásico , Procedimentos de Norwood , Cuidados Paliativos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/história , Humanos , Técnica de Fontan/história , Técnica de Fontan/métodos , Procedimentos de Norwood/história , História do Século XX , Resultado do Tratamento , História do Século XXI , Ventrículos do Coração/cirurgia , Ventrículos do Coração/anormalidadesRESUMO
The Fontan physiology is a surgically created circulation for patients with a single functioning ventricle. Patients with this circulation tend to have lower exercise tolerance compared to those with a normal circulation. Important computational and experimental work has been done to investigate this reduction in exercise tolerance. However, there are few systematic modeling studies that focus on the effect of several surgically determined parameters within the same framework. We propose a mathematical model to describe the Fontan circulation under exercise. We then formulate a heuristic based on clinical data from Fontan patients to estimate exercise tolerance. The model is used to investigate the effect of three important surgically determined parameters on exercise tolerance: the systemic arterial compliance, the systemic-venous to pulmonary-venous fenestration, and the resistance of the total cavopulmonary connection.
Assuntos
Tolerância ao Exercício , Técnica de Fontan , Humanos , Modelos BiológicosRESUMO
OBJECTIVES: This prospective cohort study aimed to investigate changes in intracranial pressure (ICP) and cerebral hemodynamics in infants with congenital heart disease undergoing the Glenn procedure, focusing on the relationship between superior vena cava pressure and estimated ICP. DESIGN: A single-center prospective cohort study. SETTING: The study was conducted in a cardiac center over 4 years (2019-2022). PATIENTS: Twenty-seven infants with congenital heart disease scheduled for the Glenn procedure were included in the study, and detailed patient demographics and primary diagnoses were recorded. INTERVENTIONS: Transcranial Doppler (TCD) ultrasound examinations were performed at three time points: baseline (preoperatively), postoperative while ventilated (within 24-48 hr), and at discharge. TCD parameters, blood pressure, and pulmonary artery pressure were measured. MEASUREMENTS AND MAIN RESULTS: TCD parameters included systolic flow velocity, diastolic flow velocity (dFV), mean flow velocity (mFV), pulsatility index (PI), and resistance index. Estimated ICP and cerebral perfusion pressure (CPP) were calculated using established formulas. There was a significant postoperative increase in estimated ICP from 11 mm Hg (interquartile range [IQR], 10-16 mm Hg) to 15 mm Hg (IQR, 12-21 mm Hg) postoperatively (p = 0.002) with a trend toward higher CPP from 22 mm Hg (IQR, 14-30 mm Hg) to 28 mm Hg (IQR, 22-38 mm Hg) postoperatively (p = 0.1). TCD indices reflected alterations in cerebral hemodynamics, including decreased dFV and mFV and increased PI. Intracranial hemodynamics while on positive airway pressure and after extubation were similar. CONCLUSIONS: Glenn procedure substantially increases estimated ICP while showing a trend toward higher CPP. These findings underscore the intricate interaction between venous pressure and cerebral hemodynamics in infants undergoing the Glenn procedure. They also highlight the remarkable complexity of cerebrovascular autoregulation in maintaining stable brain perfusion under these circumstances.
Assuntos
Circulação Cerebrovascular , Cardiopatias Congênitas , Hemodinâmica , Pressão Intracraniana , Ultrassonografia Doppler Transcraniana , Humanos , Lactente , Estudos Prospectivos , Feminino , Masculino , Pressão Intracraniana/fisiologia , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/fisiopatologia , Cardiopatias Congênitas/diagnóstico por imagem , Circulação Cerebrovascular/fisiologia , Ultrassonografia Doppler Transcraniana/métodos , Hemodinâmica/fisiologia , Estudos de Coortes , Técnica de Fontan , Veia Cava Superior/fisiopatologia , Veia Cava Superior/diagnóstico por imagemRESUMO
Lymphatic flow abnormalities are central to the development of protein losing enteropathy, plastic bronchitis, ascites and pleural effusions in patients palliated to the Fontan circulation. These complications can occur in isolation or multicompartmental (two or more). The treatment of multicompartmental lymphatic failure aims at improving thoracic duct drainage. Re-routing the innominate vein to the pulmonary venous atrium decompresses the thoracic duct, as atrial pressure is lower than systemic venous pressure in Fontan circulation. Transcatheter thoracic duct decompression is a new minimally invasive procedure that involves placing covered stents from the innominate vein to the atrium. Patients undergoing this procedure require multiple general anesthetics, presenting challenges in managing the sequelae of disordered lymphatic flow superimposed on Fontan physiology. We reviewed the first 20 patients at the Center for Lymphatic Imaging and Intervention at a tertiary care children's hospital presenting for transcatheter thoracic duct decompression between March 2018 and February 2023. The patients ranged in age from 3 to 26 years. The majority had failed prior catheter-based lymphatic intervention, including selective embolization of abnormal lympho-intestinal and lympho-bronchial connections to treat lymphatic failure in a single compartment. Fourteen had failure in three lymphatic compartments. Patients were functionally impaired (ASA 3-5) with significant comorbidities. Concurrent with thoracic duct decompression, three patients required fenestration closure for the resultant decrease in oxygen saturation. Ten patients had improvement in symptoms, seven had no changes and three have limited follow up. Five (25%) of these patients were deceased as of January 2024 due to non-lymphatic complications from Fontan failure.
Assuntos
Técnica de Fontan , Ducto Torácico , Humanos , Técnica de Fontan/efeitos adversos , Masculino , Feminino , Ducto Torácico/cirurgia , Pré-Escolar , Criança , Adolescente , Adulto , Adulto Jovem , Descompressão Cirúrgica/métodos , Anestesia/métodos , Complicações Pós-Operatórias/terapia , Complicações Pós-Operatórias/etiologia , Cuidados Paliativos/métodos , Doenças Linfáticas/terapia , Doenças Linfáticas/etiologia , Estudos RetrospectivosRESUMO
OBJECTIVES: Patients after the Norwood procedure are prone to postoperative instability. Extracorporeal membrane oxygenation (ECMO) can help to overcome short-term organ failure. This retrospective single-centre study examines ECMO weaning, hospital discharge and long-term survival after ECMO therapy between Norwood and bidirectional Glenn palliation as well as risk factors for mortality. METHODS: In our institution, over 450 Norwood procedures have been performed. Since the introduction of ECMO therapy, 306 Norwood operations took place between 2007 and 2022, involving ECMO in 59 cases before bidirectional Glenn. In 48.3% of cases, ECMO was initiated intraoperatively post-Norwood. Patient outcomes were tracked and mortality risk factors were analysed using uni- and multivariable testing. RESULTS: ECMO therapy after Norwood (median duration: 5 days; range 0-17 days) saw 31.0% installed under CPR. Weaning was achieved in 46 children (78.0%), with 55.9% discharged home after a median of 45 (36-66) days. Late death occurred in 3 patients after 27, 234 and 1541 days. Currently, 30 children are in a median 4.8 year (3.4-7.7) follow-up. At the time of inquiry, 1 patient awaits bidirectional Glenn, 6 are at stage II palliation, Fontan was completed in 22 and 1 was lost to follow-up post-Norwood. Risk factor analysis revealed dialysis (P < 0.001), cerebral lesions (P = 0.026), longer ECMO duration (P = 0.002), cardiac indication and lower body weight (P = 0.038) as mortality-increasing factors. The 10-year mortality probability after ECMO therapy was 48.5% (95% CI 36.5-62.9%). CONCLUSIONS: ECMO therapy in critically ill patients after the Norwood operation may significantly improve survival of a patient cohort otherwise forfeited and give the opportunity for successful future-stage operations.
Assuntos
Oxigenação por Membrana Extracorpórea , Procedimentos de Norwood , Humanos , Oxigenação por Membrana Extracorpórea/métodos , Oxigenação por Membrana Extracorpórea/mortalidade , Procedimentos de Norwood/mortalidade , Procedimentos de Norwood/efeitos adversos , Estudos Retrospectivos , Feminino , Masculino , Resultado do Tratamento , Recém-Nascido , Lactente , Técnica de Fontan/efeitos adversos , Técnica de Fontan/mortalidade , Fatores de RiscoRESUMO
The Fontan operation is the current standard of care for single-ventricle congenital heart disease. Individuals with a Fontan circulation (FC) exhibit central venous hypertension and face life-threatening complications of hepatic fibrosis, known as Fontan-associated liver disease (FALD). The fundamental biology and mechanisms of FALD are little understood. Here, we generated a transcriptomic and epigenomic atlas of human FALD at single-cell resolution using multiomic snRNA-ATAC-seq. We found profound cell type-specific transcriptomic and epigenomic changes in FC livers. Central hepatocytes (cHep) exhibited the most substantial changes, featuring profound metabolic reprogramming. These cHep changes preceded substantial activation of hepatic stellate cells and liver fibrosis, suggesting cHep as a potential first "responder" in the pathogenesis of FALD. We also identified a network of ligand-receptor pairs that transmit signals from cHep to hepatic stellate cells, which may promote their activation and liver fibrosis. We further experimentally demonstrated that activins A and B promote fibrotic activation in vitro and identified mechanisms of activin A's transcriptional activation in FALD. Together, our single-cell transcriptomic and epigenomic atlas revealed mechanistic insights into the pathogenesis of FALD and may aid identification of potential therapeutic targets.
Assuntos
Técnica de Fontan , Células Estreladas do Fígado , Hepatócitos , Hepatopatias , Humanos , Epigenômica , Técnica de Fontan/efeitos adversos , Células Estreladas do Fígado/metabolismo , Células Estreladas do Fígado/patologia , Hepatócitos/metabolismo , Fígado/patologia , Fígado/metabolismo , Cirrose Hepática/etiologia , Cirrose Hepática/patologia , Hepatopatias/etnologia , Hepatopatias/patologia , Multiômica , Análise de Célula Única , TranscriptomaRESUMO
BACKGROUND: The Fontan operation is used to palliate single ventricle congenital heart defects (CHD) but poses significant morbidity and mortality risks. We present the design, planned analyses, and rationale for a long-term Fontan cohort study aiming to examine the association of patient characteristics at the time of Fontan with post-Fontan morbidity and mortality. METHODS AND RESULTS: We used the Pediatric Cardiac Care Consortium (PCCC), a US-based, multicenter registry of pediatric cardiac surgeries to identify patients who underwent the Fontan procedure for single ventricle CHD between 1 and 21 years of age. The primary outcomes are in-hospital Fontan failure (death or takedown) and post-discharge mortality through 2022. A total of 1461 (males 62.1%) patients met eligibility criteria and were included in the analytical cohort. The median age at Fontan evaluation was 3.1 years (IQR: 2.4-4.3). While 95 patients experienced in-hospital Fontan failure (78 deaths and 17 Fontan takedown), 1366 (93.5%) survived to discharge with Fontan physiology and formed the long-term analysis cohort. Over a median follow-up of 21.2 years (IQR: 18.4-24.5) 184 post-discharge deaths occurred. Thirty-year post Fontan survival was 75.0% (95% CI: 72.3%-77.8%) for all Fontan types with higher rates for current techniques such as lateral tunnel and extracardiac conduit 77.1% (95% CI: 73.5-80.8). CONCLUSION: The PCCC Fontan study aims to identify predictors for post-Fontan morbidity and mortality, enabling risk- stratification and informing surveillance practices. Additionally, the study may guide therapeutic interventions aiming to optimize hemodynamics and enhance Fontan longevity for individual patients.
Assuntos
Técnica de Fontan , Cardiopatias Congênitas , Sistema de Registros , Humanos , Técnica de Fontan/métodos , Masculino , Feminino , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/mortalidade , Pré-Escolar , Criança , Adolescente , Lactente , Adulto Jovem , Cuidados Paliativos/métodos , Estados Unidos/epidemiologia , Ventrículos do Coração/fisiopatologia , Ventrículos do Coração/anormalidades , Ventrículos do Coração/cirurgia , Complicações Pós-Operatórias/epidemiologia , Estudos de Coortes , Fatores de TempoRESUMO
This Viewpoint discusses the impact of sinus node dysfunction and escape junctional rhythm associated with the Fontan procedure on patient outcomes.
Assuntos
Técnica de Fontan , Humanos , Eletrocardiografia , Cardiopatias Congênitas/cirurgia , MasculinoRESUMO
BACKGROUND: The Fontan operation is a palliative technique for patients born with single ventricle heart disease. The superior vena cava (SVC), inferior vena cava (IVC), and hepatic veins are connected to the pulmonary arteries in a total cavopulmonary connection by an extracardiac conduit or a lateral tunnel connection. A balanced hepatic flow distribution (HFD) to both lungs is essential to prevent pulmonary arteriovenous malformations and cyanosis. HFD is highly dependent on the local hemodynamics. The effect of age-related changes in caval inflows on HFD was evaluated using cardiac magnetic resonance data and patient-specific computational fluid dynamics modeling. METHODS: SVC and IVC flow from 414 patients with Fontan were collected to establish a relationship between SVC:IVC flow ratio and age. Computational fluid dynamics modeling was performed in 60 (30 extracardiac and 30 lateral tunnel) patient models to quantify the HFD that corresponded to patient ages of 3, 8, and 15 years, respectively. RESULTS: SVC:IVC flow ratio inverted at ≈8 years of age, indicating a clear shift to lower body flow predominance. Our data showed that variation of HFD in response to age-related changes in caval inflows (SVC:IVC, 2, 1, and 0.5 corresponded to ages, 3, 8, and 15+, respectively) was not significant for extracardiac but statistically significant for lateral tunnel cohorts. For all 3 caval inflow ratios, a positive correlation existed between the IVC flow distribution to both the lungs and the HFD. However, as the SVC:IVC ratio changed from 2 to 0.5 (age, 3-15+) years, the correlation's strength decreased from 0.87 to 0.64, due to potential flow perturbation as IVC flow momentum increased. CONCLUSIONS: Our analysis provided quantitative insights into the impact of the changing caval inflows on Fontan's long-term HFD, highlighting the importance of SVC:IVC variations over time on Fontan's long-term hemodynamics. These findings broaden our understanding of Fontan hemodynamics and patient outcomes.
Assuntos
Técnica de Fontan , Cardiopatias Congênitas , Humanos , Pré-Escolar , Criança , Adolescente , Veia Cava Superior/diagnóstico por imagem , Veia Cava Superior/cirurgia , Veia Cava Superior/fisiologia , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/cirurgia , Fígado/diagnóstico por imagem , Hemodinâmica/fisiologia , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/cirurgia , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgiaAssuntos
Arritmias Cardíacas , Técnica de Fontan , Cardiopatias Congênitas , Humanos , Técnica de Fontan/efeitos adversos , Arritmias Cardíacas/fisiopatologia , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/cirurgia , Arritmias Cardíacas/diagnóstico , Cardiopatias Congênitas/cirurgia , Resultado do TratamentoRESUMO
AIMS: The extracardiac conduit-Fontan (ECC) has become the preferred technique for univentricular heart palliation, but there are currently no data on the incidence of long-term arrhythmias. This study investigated the incidence of arrhythmias and relation to single ventricle morphology in the long-term follow-up (FU) in ECC. METHODS AND RESULTS: All patients with ECC performed in our Centre between 1987 and 2017 were included (minimum FU 5 years). Of 353 consecutive patients, 303 [57.8% males, aging 8-50 (median 20) years at last FU] were considered and divided into two groups depending on left (194 in Group 1) or right (109 in Group 2) ventricular morphology. Eighty-five (28%) experienced ≥1 arrhythmic complications, with early and late arrhythmias in 17 (5.6%) and 73 (24.1%) patients, respectively. Notably, late bradyarrhythmias occurred after 6 years in 21 (11%) patients in Group 1, and in 15 (13.8%) in Group 2 [P = 0.48]. Late tachyarrhythmias occurred in 55 (18.2%) patients after 12 years: 33 (17%) in Group 1 and 22 (20.2%) patients in Group 2 [P = 0.5]. Ventricular tachycardias (VT) were documented after 12.5 years in 14 (7.2%) patients of Group 1 and 15 (13.8%) of Group 2 [P = 0.06] with a higher incidence in Group 2 during the FU [P = 0.005]. CONCLUSION: Extracardiac conduit is related to a significant arrhythmic risk in the long-term FU, higher than previously reported. Bradyarrhythmias occur earlier but are less frequent than tachyarrhythmias. Interestingly, patients with systemic right ventricle have a significantly higher incidence of VT, especially in a very long FU.
Assuntos
Arritmias Cardíacas , Técnica de Fontan , Ventrículos do Coração , Humanos , Masculino , Feminino , Técnica de Fontan/efeitos adversos , Incidência , Criança , Adolescente , Adulto , Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/fisiopatologia , Arritmias Cardíacas/diagnóstico , Pessoa de Meia-Idade , Adulto Jovem , Ventrículos do Coração/fisiopatologia , Ventrículos do Coração/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/epidemiologia , Estudos Retrospectivos , Fatores de Tempo , Coração Univentricular/cirurgia , Coração Univentricular/epidemiologia , Coração Univentricular/fisiopatologia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Fatores de RiscoAssuntos
Varizes Esofágicas e Gástricas , Técnica de Fontan , Cuidados Paliativos , Humanos , Técnica de Fontan/efeitos adversos , Varizes Esofágicas e Gástricas/etiologia , Varizes Esofágicas e Gástricas/diagnóstico , Masculino , Feminino , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/complicações , Programas de Rastreamento/métodos , AdultoRESUMO
We present the case of a 52-year-old woman with cardiogenic shock and refractory right ventricular failure due to spontaneous dissection of the right coronary artery. She remained dependent on mechanical support for several weeks. Both a right ventricular assist device implant and a bidirectional cavopulmonary anastomosis were explored as long-term support options. A history of malignancy and possible right ventricular functional recovery resulted in a decision in favour of the bidirectional cavopulmonary anastomosis and concomitant tricuspid valve annuloplasty. Postoperatively her clinical condition improved significantly, and she could be discharged home. Echocardiography showed normalization of right ventricular dimensions and slight improvement of right ventricular function.
Assuntos
Insuficiência Cardíaca , Infarto do Miocárdio , Humanos , Feminino , Pessoa de Meia-Idade , Insuficiência Cardíaca/cirurgia , Insuficiência Cardíaca/etiologia , Infarto do Miocárdio/cirurgia , Infarto do Miocárdio/complicações , Disfunção Ventricular Direita/cirurgia , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/fisiopatologia , Técnica de Fontan/efeitos adversos , Coração Auxiliar , Ventrículos do Coração/cirurgia , Ventrículos do Coração/diagnóstico por imagemRESUMO
Whole blood analysis can evaluate numerous parameters, including pH, pCO2, pO2, HCO3 - , base excess, glucose, electrolytes, lactate, blood urea nitrogen, creatinine, bilirubin, and hemoglobin. This valuable tool enables clinicians to make more informed decisions about patient care. However, the current body of literature describing perioperative whole blood analysis in Dorset sheep (Ovis aries) is small, so clinicians lack adequate information to guide their decision-making when evaluating test results. We evaluated arterial and venous whole blood pH, bicarbonate, pCO2, lactate, creatinine, and blood urea nitrogen before and for the first 24 hours after surgery in 2 cohorts of male and female Ovis arie s undergoing one of 2 major cardiovascular procedures, a Single-Stage Fontan or an inferior vena cava to pulmonary artery extracardiac conduit implantation (IP-ECC). The cohort undergoing a Single-Stage Fontan, which is the more complex procedure, exhibited greater deviation from baseline measurements than did the cohort undergoing the IP-ECC for lactate, bicarbonate, and creatinine. The cohort undergoing the IP-ECC showed no significant deviation from baseline for any parameters, potentially indicating a better safety margin than expected when compared with the Single-Stage Fontan. Together, these results indicate the clinical value of arterial and venous whole blood measurements in perioperative management of sheep and can provide a reference for clinicians managing sheep after significant cardiovascular procedures.
Assuntos
Técnica de Fontan , Animais , Feminino , Masculino , Ovinos , Creatinina/sangue , Concentração de Íons de Hidrogênio , Nitrogênio da Ureia Sanguínea , Bicarbonatos/sangue , Análise Química do Sangue/veterinária , Ácido Láctico/sangue , Dióxido de Carbono/sangue , Carneiro Doméstico/sangueRESUMO
Introducción: Se ha postulado que el uso de vasopresina tendría efectos beneficiosos en el postoperatorio de cirugía cardiovascular. Objetivo: Evaluar la respuesta a la vasopresina en el postoperatorio (POP) de cirugía de Fontan de nuestra población. Métodos: Estudio de casos y controles anidados en una cohorte retrospectiva. Se incluyeron pacientes con cirugía de Fontan entre 2014 y 2019. Se registraron variables demográficas, datos del cateterismo pre-Fontan, días de asistencia respiratoria mecánica (ARM), necesidad de inotrópicos, diuréticos, diálisis, dieta hipograsa, octreotide, sildenafil y nutrición parenteral total (NPT); balance de fluidos al primer y segundo día POP, necesidad de cateterismo en el POP, días de permanencia de tubo pleural, días de internación, necesidad de reinternación y mortalidad. Se compararon los grupos con y sin vasopresina utilizando la prueba de Mann- Whitney-Wilcoxon test. Se consideró significativa una p < 0.05. Resultados: Del total analizado, 35 pacientes recibieron vasopresina. En el grupo control fueron 58 pacientes con características similares de gravedad sin vasopresina. No se encontraron diferencias en la evolución postoperatoria entre ambos grupos. El grupo con vasopresina recibió en mayor proporción dieta hipograsa. Conclusiones: En nuestra serie el uso de vasopresina no marcó diferencias significativas en términos de morbimortalidad con relación al grupo control (AU)
Introduction: The use of vasopressin has been suggested to have beneficial effects in the postoperative period after cardiovascular surgery. Objective: To evaluate the response to vasopressin in the postoperative period (POP) of Fontan surgery in our population. Methods: Nested case-control study in a retrospective cohort. Patients who underwent Fontan surgery between 2014 and 2019 were included. Demographic variables, pre-Fontan catheterization data, days of mechanical ventilation (MRA), need for inotropics, diuretics, dialysis, low-fat diet, octreotide, sildenafil and total parenteral nutrition (TPN); fluid balance at first and second day POP, need for catheterization at POP, duration of chest tube drainage, days of hospitalization, need for readmission, and mortality were recorded. Groups with and without vasopressin were compared using the Mann-Whitney- Wilcoxon test. A p < 0.05 was considered significant. Results: Of all patients analyzed, 35 received vasopressin. The control group consisted of 58 patients with similar severity characteristics who did not receive vasopressin. No differences were found in the postoperative outcome between the two groups. The vasopressin group received a higher proportion of low-fat diet. Conclusions: In our series the use of vasopressin did not show significant differences in terms of morbidity and mortality compared to the control group (AU)