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It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Up to 70 000 patients may be alive worldwide today with Fontan circulation, and this population is expected to double in the next 20 years. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output. The addition of this acquired abnormal circulation to innate abnormalities associated with single-ventricle congenital heart disease exposes these patients to a variety of complications. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation. Abnormalities in body composition, bone structure, and growth have been detected. Liver fibrosis and renal dysfunction are common and may progress over time. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications. This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. Gaps in knowledge and areas for future focus of investigation are highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for these unique individuals.
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OBJECTIVE: To investigate potential relationships between neuropsychologic functioning and cardiac, gastroenterologic/hepatologic, and pulmonary complications in the single ventricle heart disease (SVHD) post-Fontan population. STUDY DESIGN: Following the initiation of a Fontan Multidisciplinary Clinic, patients with SVHD were evaluated systematically according to a clinical care pathway, and data from multiple subspecialty evaluations were collected prospectively from 2016 to 2019. Biomarkers of cardiology, pulmonary, and hepatology/gastroenterology functioning were abstracted, along with neuropsychologic testing results. Bivariate correlations and regression analyses examined cross-sectional relationships between physiologic predictors and neuropsychologic outcomes. RESULTS: The sample included a cohort of 68 youth with SVHD age 3-19 years, after Fontan palliation. Sleep-disordered breathing was related to poorer visual-motor integration skills (r = -0.33; P < .05) and marginally related to poorer executive functioning (r = -0.33; P = .05). Lower arterial blood oxygen content was related to poorer executive functioning (r = .45; P < .05). Greater atrioventricular valve regurgitation was related to lower parent-rated adaptive functioning (ρ = -0.34; P < .01). These results were maintained in regression analyses controlling for history of stroke and/or seizures. CONCLUSIONS: We demonstrated associations between neuropsychologic functioning and potentially modifiable aspects of physiologic functioning in a prospectively evaluated cohort of patients with SVHD with Fontan physiology. Our findings emphasize the importance of multidisciplinary screening and care after a Fontan procedure and suggest avenues for intervention that may improve patient outcomes and quality of life.
Assuntos
Técnica de Fontan , Complicações Pós-Operatórias/fisiopatologia , Complicações Pós-Operatórias/psicologia , Coração Univentricular/cirurgia , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Masculino , Testes Neuropsicológicos , Complicações Pós-Operatórias/epidemiologia , Estudos Prospectivos , Adulto JovemRESUMO
More children with single ventricle heart disease are surviving after Fontan surgery. This circulation has pervasive effects on multiple organ systems and has unique modes of failure. Many centers have created multidisciplinary programs to care for these patients. Our aim was to survey such programs to better understand current approaches to care. We hypothesized that significant variability in surveillance testing strategy would be present. Eleven academic institutions with established Fontan care programs performing a combined estimated 300 Fontan surgeries per year, with a total population of 1500-2000 Fontan patients, were surveyed using a REDCap survey regarding surveillance testing and basic practice philosophies. Fontan care programs were structured both as consultative services (64%) and as the primary clinical team (9%). Electrocardiograms (73%) and echocardiograms (64%) were most commonly obtained annually. Serum studies, including complete blood count (73%), complete metabolic panel (73%), and Brain-type natriuretic peptide (54%), were most commonly obtained annually. Hepatic testing consisted of liver ultrasound in most centers, obtained biennially (45%) or > every 2 years (45%). Liver biopsy was not routinely recommended (54%). Neurodevelopmental outcomes were assessed at most institutions (54%), with a median frequency of every 3-4 years. There is considerable variability in the surveillance testing regimen and management strategy after a Fontan procedure at surveyed programs. There is an urgent need for surveillance guidelines to reduce variability, define quality metrics, streamline collaborative practice, and prospective research to better understand the complex adaptations of the body to Fontan physiology.
Assuntos
Procedimentos Clínicos , Técnica de Fontan/efeitos adversos , Ventrículos do Coração/anormalidades , Avaliação de Processos e Resultados em Cuidados de Saúde , Criança , Pré-Escolar , Feminino , Cardiopatias Congênitas/cirurgia , Ventrículos do Coração/cirurgia , Humanos , Masculino , Avaliação de Programas e Projetos de Saúde , Inquéritos e QuestionáriosRESUMO
Echocardiography is the primary diagnostic modality for congenital heart disease patients. The written report is used to communicate with the care team and organization is often divided into the body with detailed findings and the conclusions with important findings summarized. Strategies to increase workflow efficiency include batch writing of reports after performance of multiple echocardiograms and the use of report templates which may contribute to discrepancies within report leading to potential downstream medical errors. The aim of this project was to measure the rate of inconsistencies in the echocardiogram reports and through an iterative series of process improvement decrease this rate while maintaining sonographer efficiency and diagnostic accuracy. The discrepancy rate, diagnostic error rate, and sonographer productivity were collected one-year prior and during the iterative quality improvement process. The primary outcome and discrepancies in reports were determined by two reviewers: an experienced pediatric echocardiographic cardiologist and a senior sonographer. Minor discrepancies were defined as contradictions between the body and the conclusion of the report that were unlikely to affect patient care. Major discrepancies were defined as discrepancies between the body and the conclusion that had significant potential to affect patient care. Sonographer productivity was measured as studies per sonographer per month. Our primary intervention was to initiate a quarterly QI meeting and to decrease the batch writing of preliminary echocardiogram reports. No major discrepancies were identified pre- or post-intervention. The minor discrepancies decreased from 40.7 to 6%. Sonographer productivity was not significantly changed with a slight increase from 100 studies/sonographer/month during the baseline to 101 studies/sonographer/month during the intervention. There was no change in major or minor diagnostic error rate. Our quality improvement intervention increased the value of our reports by significantly decreasing minor discrepancies without negatively impacting sonographer productivity or diagnostic accuracy.
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Competência Clínica/normas , Ecocardiografia/normas , Cardiopatias Congênitas/diagnóstico por imagem , Melhoria de Qualidade , Erros de Diagnóstico/estatística & dados numéricos , Ecocardiografia/métodos , Humanos , Projetos Piloto , Qualidade da Assistência à Saúde/normasRESUMO
Transplant coronary artery vasculopathy (TCAV) following orthotopic heart transplantation (OHT) continues to be the primary reason for late graft failure in children. The current gold standard of diagnosis of TCAV is coronary angiography with or without intravascular ultrasound. This study investigates the longitudinal use of speckle-tracking echocardiographic strain imaging as an early non-invasive marker to screen for development of TCAV. Echocardiograms from patients who underwent OHT between 2006 and 2010 at Children's Hospital Colorado (n = 50) were retrospectively assessed. Studies were evaluated at baseline (within a month of transplant), then at each annual clinical follow-up for peak longitudinal (LS) and circumferential (CS) strain, systolic strain rate, and diastolic strain rate using Siemens Velocity Vector Imaging software. Comparisons were made between subjects who did and did not develop TCAV. Mean time to TCAV diagnosis following OHT was 3.2 years (range 1-5.1 years). One year after transplant, significant differences were seen between groups in LS (non-TCAV mean -19.6%, TCAV mean -17.3%, p = 0.03) and longitudinal strain rate (non-TCAV mean -1.7%/s, TCAV mean -1.4%/s, p = 0.04). These differences persisted in subsequent years. Differences in LS preceded the catheterization-based diagnosis of TCAV in pediatric heart recipients and were noted as early as one year post transplant. Additionally, within-subject LS changes may have utility as a non-invasive screening tool to predict those patients at increased risk for development of TCAV.
Assuntos
Cateterismo Cardíaco/métodos , Doença da Artéria Coronariana/diagnóstico por imagem , Vasos Coronários/patologia , Ecocardiografia/métodos , Transplante de Coração/efeitos adversos , Adolescente , Criança , Pré-Escolar , Colorado , Doença da Artéria Coronariana/etiologia , Vasos Coronários/diagnóstico por imagem , Feminino , Humanos , Estudos Longitudinais , Masculino , Programas de Rastreamento/métodos , Variações Dependentes do Observador , Curva ROC , Estudos RetrospectivosRESUMO
Transplant coronary artery vasculopathy (TCAV) is the primary cause of late graft loss in pediatric heart transplant recipients. TCAV is diagnosed using angiography or intravascular ultrasound; however, noninvasive methods remain elusive. We sought to define patterns of myocardial mechanics in patients with TCAV and to determine whether this can detect TCAV before invasive methods. In this retrospective study, we queried our heart transplant database to identify all recipients with TCAV since 2006 (n = 41). Echoes were reviewed from the last normal catheterization and at TCAV diagnosis, and from time-matched transplant controls (n = 33) without TCAV. Peak global circumferential and longitudinal strain and systolic and diastolic strain rate (SSR and DSR) of the left ventricle were derived using velocity vector imaging. T tests were used to compare both groups longitudinally and between groups at both time points. Longitudinal strain, SSR, and DSR were diminished in the TCAV group compared to the transplant control group at both time points. No differences were found across time points in either group. Retrospective modeling using a longitudinal strain cutoff of 15 % on echoes 2 years prior to TCAV diagnosis predicted development or exclusion of TCAV with sensitivity of 53 %, specificity of 89 % with an area under the curve of 0.8. Decreases in longitudinal strain measurements demonstrate that alterations in myocardial mechanics occur in patients with TCAV at least 2 years prior to invasive diagnosis. These early changes may be due to microvascular disease. This modality could aid in earlier treatment and intervention for this challenging problem .
Assuntos
Angiografia Coronária , Vasos Coronários/fisiopatologia , Ecocardiografia , Transplante de Coração/efeitos adversos , Doenças Vasculares/diagnóstico por imagem , Função Ventricular Esquerda , Adolescente , Autopsia , Criança , Pré-Escolar , Colorado , Bases de Dados Factuais , Diástole , Feminino , Humanos , Masculino , Estudos Retrospectivos , Volume Sistólico , SístoleRESUMO
In hypoplastic left heart syndrome, thrombosis of the native ascending aorta is rare and often fatal; there are no previously reported cases presenting with acute heart block. We review a case of native ascending aorta thrombosis in a 2-year-old boy with hypoplastic left heart syndrome, presenting with acute heart block. This case highlights the benefit of multi-modality imaging in complex cases.
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Bloqueio Cardíaco/etiologia , Síndrome do Coração Esquerdo Hipoplásico/complicações , Isquemia Miocárdica/etiologia , Doença Aguda , Pré-Escolar , Humanos , MasculinoRESUMO
BACKGROUND: Predicting later outcome around time of diagnosis of acute dilated cardiomyopathy (DCM) is difficult. We hypothesized that strain and strain rate on initial and follow-up echoes were worse in patients with acute DCM from all causes with poor one-year outcomes. METHODS: This was a retrospective study including all patients with DCM aged 0-18 years with left ventricle dilation, low ejection fraction, or low fractional shortening on initial echo. Longitudinal and circumferential strain and systolic and diastolic strain rate were measured on echo at presentation, 1-3 weeks after presentation, and at 1 year. Patients were separated into "Stable" (survivors) and "Progressive" (referred for transplant or died) outcome groups, and results were analyzed to determine whether strain or strain rate at each echo was worse in the "Stable" group compared with the "Progressive" group. RESULTS: The patient population included patients with DCM from idiopathic causes, myocarditis, iron deficiency anemia, lupus, chemotherapy, and LV noncompaction. Longitudinal and circumferential strain and systolic strain rate were significantly better in the "Stable" (n = 7) compared with the "Progressive" (n = 8) outcome group on 1- to 3-week echo. Longitudinal strain more negative than -10% had 87% specificity and 100% sensitivity for predicting "stable" outcome (AUC 0.98), while circumferential strain more negative than -8% had 60% specificity and 100% sensitivity (AUC 0.83). CONCLUSIONS: Longitudinal and circumferential strain and systolic strain rate measured 1-3 weeks after starting therapy are worse in acute dilated cardiomyopathy patients with poor one-year outcomes. Further studies with less heterogeneity and more study subjects are needed.
Assuntos
Cardiomiopatia Dilatada/diagnóstico por imagem , Cardiomiopatia Dilatada/fisiopatologia , Avaliação de Resultados em Cuidados de Saúde/estatística & dados numéricos , Doença Aguda , Adolescente , Cardiomiopatia Dilatada/tratamento farmacológico , Cardiotônicos/uso terapêutico , Criança , Pré-Escolar , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/fisiopatologia , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Ultrassonografia , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/parasitologia , Disfunção Ventricular Esquerda/fisiopatologiaRESUMO
BACKGROUND: Anthracycline chemotherapeutic agents carry the well-recognised risk of cardiotoxicity. Previous methods to evaluate cardiac function are useful, but have significant limitations. We sought to determine the left ventricular strain and strain rate of paediatric cancer patients with normal fractional shortening treated with anthracyclines using the latest ultrasound feature-tracking technology. PATIENTS AND METHODS: Echocardiograms on cancer patients before anthracycline exposure and following completion of treatment were retrospectively analysed using Velocity Vector Imaging software in the circumferential and longitudinal planes. The same analysis was performed on matched controls. Only patients with a fractional shortening ≥28% were included. RESULTS: In all, 71 patients were identified with an age at diagnosis of 10.5 ± 4.7 years. The time from diagnosis to follow-up was 3.9 ± 4.0 years and the cumulative anthracycline dose was 356 ± 106 mg/m². Following anthracycline exposure, paediatric cancer patients had a higher heart rate and a lower longitudinal strain, longitudinal diastolic strain rate, circumferential strain, and circumferential systolic and diastolic strain rate when compared with controls. Diastolic strain rate showed the greatest percent difference following anthracycline exposure versus controls. CONCLUSION: Despite having a normal fractional shortening, children exposed to anthracyclines have subclinical derangement of their left ventricular deformation as measured by decreases in strain and strain rate in both the circumferential and longitudinal axis. In particular, there was a profound decrease in diastolic strain rate following anthracycline exposure compared with controls. Whether the decline of strain or strain rate can predict future risk of developing cardiomyopathy requires further investigation.
Assuntos
Antraciclinas/efeitos adversos , Ecocardiografia/métodos , Ventrículos do Coração/diagnóstico por imagem , Neoplasias/tratamento farmacológico , Volume Sistólico/fisiologia , Disfunção Ventricular Esquerda/induzido quimicamente , Função Ventricular Esquerda/fisiologia , Antraciclinas/uso terapêutico , Antibióticos Antineoplásicos/efeitos adversos , Antibióticos Antineoplásicos/uso terapêutico , Criança , Feminino , Seguimentos , Ventrículos do Coração/efeitos dos fármacos , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Estudos Retrospectivos , Volume Sistólico/efeitos dos fármacos , Fatores de Tempo , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda/efeitos dos fármacosRESUMO
The transplanted heart experiences numerous hemodynamic changes during and after cardiac transplantation. This study sought to evaluate the left ventricular myocardial mechanics in the pediatric heart transplant population using Velocity Vector Imaging (VVI). This study retrospectively evaluated 28 heart transplant recipients by echocardiography 12 months after transplantation. Echocardiograms from 28 age- and gender-matched subjects were used as a control group. Peak global longitudinal and circumferential left ventricular strain, systolic strain rate, and diastolic strain rate were obtained. Student's t tests were used to assess differences between the two groups (defined as p ≤ 0.05). The peak global left ventricular longitudinal strain was lower in the transplant group (17.21%) than in the control group (22.14%). The transplant and control groups did not differ significantly in terms of their peak global circumferential strain (20.28% vs. 20.79%, respectively). Similar results were observed for longitudinal and circumferential systolic and diastolic strain rates. The transplant patients showed statistically significant reductions in all peak global longitudinal measures compared with those of the control subjects. Circumferential myocardial deformation appears to be preserved in transplant recipients. This could suggest evidence of ischemia given the known myocardial fiber arrangement of longitudinal fibers toward the endocardial surface, which is also more distal in the coronary arterioles.
Assuntos
Ecocardiografia/métodos , Transplante de Coração , Função Ventricular Esquerda , Adolescente , Biópsia , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Testes de Função Cardíaca , Hemodinâmica , Humanos , Lactente , Recém-Nascido , Masculino , Estudos RetrospectivosRESUMO
Children with single-ventricle heart disease (SVHD) are at risk for morbidity across multiple organ systems. A single-ventricle multidisciplinary clinic (SVMDC) may address complex health-care needs by providing access to, and coordination among, pediatric subspecialties. However, the patient and family experience of multidisciplinary care for SVHD remains unexplored. We e-mailed a 26-question survey to families after an SVMDC visit, which included evaluation with subspecialists from cardiology, pulmonology, gastroenterology, neuropsychology, and pediatric psychology, as well as social activities during clinic. Responses were anonymized to protect privacy, and data were analyzed quantitatively and qualitatively. Over 3 years, 22% (27/122) of families completed the survey. Overall, families' experiences were positive, with 100% reporting that they would recommend the SVMDC to others. Qualitative themes emerged regarding logistics, multidisciplinary care, key takeaways from clinic, and connection-making with other families. A multidisciplinary clinic demonstrated overall acceptability and perceived benefit to families of children with SVHD. Considerations for mixed experiences regarding financial commitment and connection-making among parents are discussed, as are the benefits of the synergy achieved through multidisciplinary care.
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BACKGROUND: Right ventricular (RV) failure, a determinant of outcomes in pulmonary hypertension (PH), occurs when the right ventricle cannot compensate for increased afterload. The authors showed that RV stroke work (RVSW) can be estimated in children with PH as the product of stroke volume and RV pressure and is related to adverse outcomes. The aim of this study was to test the hypothesis that ventricular-vascular (VV) mismatch (high afterload and low RVSW) is associated with echocardiographic measures of RV performance and adverse outcomes. METHODS: Invasive hemodynamic data and concurrent echocardiograms were reviewed. Fifty subjects with PH were included. Four groups were created by dividing the patients using median RVSW and median pulmonary vascular resistance. For each group, tricuspid annular plane systolic excursion, fractional area change, myocardial performance index, and anterior RV wall thickness were determined. Both major (i.e., death) and minor (i.e., worsening World Health Organization class) clinical outcomes were tabulated. Groups were compared using the Kruskal-Wallis or Fisher exact test. RESULTS: Patients in the high pulmonary vascular resistance/low RVSW cohort (VV mismatch) had the worst RV dysfunction: median tricuspid annular plane systolic excursion, 0.8 cm (interquartile range, 0.7-0.8 cm; P = .0002); median fractional area change, 0.29% (interquartile range, 0.27%-0.30%; P = .004); median myocardial performance index, 0.622 (interquartile range, 0.548-0.789; P = .0004). This group had the highest incidence of adverse outcomes: major events in 40%, minor events in 80%, and syncope in 60%. CONCLUSION: VV mismatch in pediatric PH can be assessed using RVSW and pulmonary vascular resistance and is associated with RV performance and adverse events. RVSW increases in compensated high-afterload states and falls as the right ventricle fails to meet increased load; thus, VV matching status may be a sensitive predictor of outcomes in pediatric PH.
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Ecocardiografia/métodos , Ventrículos do Coração/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Artéria Pulmonar/fisiopatologia , Volume Sistólico/fisiologia , Resistência Vascular/fisiologia , Função Ventricular Direita/fisiologia , Adolescente , Cateterismo Cardíaco/métodos , Criança , Estudos Transversais , Feminino , Seguimentos , Ventrículos do Coração/fisiopatologia , Humanos , Masculino , Artéria Pulmonar/diagnóstico por imagem , Estudos RetrospectivosRESUMO
BACKGROUND: The current management paradigm for children with single ventricle heart disease involves a series of palliative surgeries, culminating in the Fontan operation. This physiology results in a high central venous pressure, and in the setting of single ventricle heart disease, results in hepatic injury and fibrosis over time. Fontan-associated liver disease (FALD) is universally present in this cohort, and the current gold standard for diagnosis remains biopsy. Noninvasive assessments of liver fibrosis, such as ultrasound with elastography or acoustic radiation force impulse (ARFI), has been utilized in this cohort. The effect of poor cardiovascular efficiency, as measured by cardiopulmonary exercise test (CPET), on assessments of liver fibrosis remains poorly understood. METHODS: Retrospective, cross-sectional study. Subjects were evaluated in a multidisciplinary clinic setting for patients who have undergone Fontan operation. CPETs, liver ultrasound with elastography (ARFI), and standard laboratory tests were performed as part of routine clinical care pathway. Statistical analysis included linear correlation. RESULTS: There was a poor correlation between mean ARFI velocity and peak oxygen consumption (VO2max ) in this cohort (r = .20, P = NS). Similarly, there was poor correlation between ARFI and biomarkers of liver injury, time since Fontan operation and Fontan pressure. DISCUSSION: ARFI had poor correlation with functional capacity after Fontan, as measured by VO2max obtained during CPET. While a single measurement of liver elastography was not associated with cardiopulmonary efficiency, longitudinal data may reveal an association.
Assuntos
Técnicas de Imagem por Elasticidade/métodos , Técnica de Fontan , Cardiopatias Congênitas/cirurgia , Cirrose Hepática/diagnóstico , Fígado/diagnóstico por imagem , Complicações Pós-Operatórias/diagnóstico , Criança , Estudos Transversais , Teste de Esforço , Feminino , Humanos , Cirrose Hepática/etiologia , Masculino , Complicações Pós-Operatórias/etiologia , Curva ROC , Reprodutibilidade dos Testes , Estudos RetrospectivosRESUMO
OBJECTIVE: Infants with single ventricle physiology typically undergo cardiac catheterization prior to superior cavopulmonary anastomosis (SCPA) to assess operative suitability. Predictors of poor outcome at sea level include elevated pulmonary artery pressure (mPAP), indexed pulmonary vascular resistance (PVRi), age <3 months, significant atrioventricular valve regurgitation, among others. Increased altitude has vasoconstrictive effects on the pulmonary vasculature, which may affect pre-SCPA hemodynamics and outcomes. The goal of this study was to determine the predictive value of pre-SCPA catheterization data with regard to reaching Fontan palliation at altitude. DESIGN: A retrospective review revealed 150 patients who underwent pre-SCPA catheterization over a 10-year period. Subjects were grouped by progression to Fontan vs aborted palliation, heart transplant or death. Statistics included t-tests, logistic regression and receiver operator characteristic (ROC) curve analysis. RESULTS: Independent predictors of failure to achieve Fontan operation at increased altitude include decreased ventricular function, increased mPAP, increased PVRi, and prolonged hospitalization, after adjusting for ventricular morphology and sex. CONCLUSIONS: Our data indicate that decreased ventricular function most strongly predicts failure to reach Fontan palliation. Additionally, mPAP and PVRi play an important role in determining outcomes at increased altitude. Prolonged hospitalization is likely a marker of increased medical complexity or more problematic physiology.
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Altitude , Cateterismo Cardíaco/métodos , Previsões , Derivação Cardíaca Direita/métodos , Cardiopatias Congênitas/cirurgia , Medição de Risco , Estudos de Casos e Controles , Colorado/epidemiologia , Feminino , Seguimentos , Cardiopatias Congênitas/mortalidade , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Mortalidade Infantil/tendências , Recém-Nascido , Masculino , Cuidados Paliativos , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Fatores de Tempo , Resultado do TratamentoRESUMO
BACKGROUND: Through ventricular interdependence, pulmonary hypertension (PH) induces left ventricular (LV) dysfunction. We hypothesized that pediatric PH patients have LV diastolic dysfunction, related to adverse pulmonary hemodynamics, leftward septal shift, and prolonged right ventricular systole. METHODS AND RESULTS: Echocardiography was prospectively performed at 2 institutions in 54 pediatric PH patients during cardiac catheterization and in 54 matched controls. Diastolic LV measures including myocardial deformation were assessed by echocardiography. PH patients had evidence of LV diastolic dysfunction, most consistent with impaired LV relaxation, though some features of reduced ventricular compliance were present. PH patients demonstrated the following: reduced mitral E velocity and inflow duration, mitral E' and E'/A', septal E' and A', pulmonary vein S and D wave velocities, and LV basal global early diastolic circumferential strain rate and increased mitral E deceleration time, LV isovolumic relaxation time, mitral E/E', and pulmonary vein A wave duration. PH patients demonstrated leftward septal shift and prolonged right ventricular systole, both known to affect LV diastole. These changes were exacerbated in severe PH. There were no statistically significant differences in diastolic measures between patients with and without a shunt and minimal differences between patients with and without congenital heart disease. Multiple echocardiographic LV diastolic parameters demonstrated weak-to-moderate correlations with invasively determined PH severity, leftward septal shift, and prolonged right ventricular systole. CONCLUSIONS: Pediatric PH patients exhibit LV diastolic dysfunction most consistent with impaired relaxation and reduced myocardial deformation, related to invasive hemodynamics, leftward septal shift, and prolonged right ventricular systole.
Assuntos
Hemodinâmica , Hipertensão Pulmonar/complicações , Circulação Pulmonar , Veias Pulmonares/fisiopatologia , Disfunção Ventricular Esquerda/etiologia , Função Ventricular Esquerda , Adolescente , Fatores Etários , Cateterismo Cardíaco , Estudos de Casos e Controles , Criança , Pré-Escolar , Colorado , Estudos Transversais , Diástole , Ecocardiografia Doppler de Pulso , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/fisiopatologia , Lactente , Recém-Nascido , Masculino , Valva Mitral/fisiopatologia , Ontário , Valor Preditivo dos Testes , Estudos Prospectivos , Sístole , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/fisiopatologia , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/fisiopatologia , Função Ventricular Direita , Adulto JovemAssuntos
Artéria Pulmonar/anormalidades , Artéria Pulmonar/diagnóstico por imagem , Persistência do Tronco Arterial/diagnóstico por imagem , Persistência do Tronco Arterial/patologia , Adulto , Angiografia , Ecocardiografia , Feminino , Humanos , Recém-Nascido , Masculino , Ultrassonografia Pré-NatalRESUMO
BACKGROUND: Through ventricular interdependence, pulmonary hypertension (PH) induces left ventricular (LV) dysfunction. We hypothesized that LV strain/strain rate, surrogate measures of myocardial contractility, are reduced in pediatric PH and relate to invasive hemodynamics, right ventricular strain, and functional measures of PH. METHODS AND RESULTS: At 2 institutions, echocardiography was prospectively performed in 54 pediatric PH patients during cardiac catheterization, and in 54 matched controls. Patients with PH had reduced LV global longitudinal strain (LS; -18.8 [-17.3 to -20.4]% versus -20.2 [-19.0 to -20.9]%; P=0.0046) predominantly because of reduced basal (-12.9 [-10.8 to -16.3]% versus -17.9 [-14.5 to -20.7]%; P<0.0001) and mid (-17.5 [-15.5 to -19.0]% versus -21.1 [-19.1 to -23.0]%; P<0.0001) septal strain. Basal global circumferential strain was reduced (-18.7 [-15.7 to -22.1]% versus -20.6 [-19.0 to -22.5]%; P=0.0098), as were septal and free-wall segments. Mid circumferential strain was reduced within the free-wall. Strain rates were reduced in similar patterns. Basal septum LS, the combined average LS of basal and mid interventricular septal segments, correlated strongly with degree of PH (r=0.66; P<0.0001), pulmonary vascular resistance (r=0.60; P<0.0001), and right ventricular free-wall LS (r=0.64; P<0.0001). Brain natriuretic peptide levels correlated moderately with septal LS (r=0.48; P=0.0038). PH functional class correlated moderately with LV free-wall LS (r=-0.48; P=0.0051). The septum, shared between ventricles and affected by septal shift, was the most affected LV region in PH. CONCLUSIONS: Pediatric PH patients demonstrate reduced LV strain/strain rate, predominantly within the septum, with relationships to invasive hemodynamics, right ventricular strain, and functional PH measures.
Assuntos
Hemodinâmica , Hipertensão Pulmonar/fisiopatologia , Disfunção Ventricular Esquerda/fisiopatologia , Disfunção Ventricular Direita/fisiopatologia , Função Ventricular Esquerda , Função Ventricular Direita , Adolescente , Fatores Etários , Fenômenos Biomecânicos , Cateterismo Cardíaco , Estudos de Casos e Controles , Criança , Pré-Escolar , Ecocardiografia Doppler em Cores , Feminino , Humanos , Hipertensão Pulmonar/complicações , Hipertensão Pulmonar/diagnóstico por imagem , Lactente , Recém-Nascido , Masculino , Contração Miocárdica , Ontário , Valor Preditivo dos Testes , Estudos Prospectivos , Reprodutibilidade dos Testes , Índice de Gravidade de Doença , Estresse Mecânico , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/etiologia , Septo Interventricular/fisiopatologia , Adulto JovemRESUMO
BACKGROUND: Right ventricular (RV) failure is a key determinant of mortality in children with pulmonary arterial hypertension (PAH). RV stroke work (RVSW) can be estimated as the product of RV systolic pressure and stroke volume. The authors have shown that RVSW predicts adverse outcomes in this population when derived from hemodynamic data; noninvasive assessment of RVSW may be advantageous but has not been assessed. There are few data validating noninvasive versus invasive measurements in children with PAH. The aim of this study was to compare echocardiographically derived RVSW with RVSW determined from hemodynamic data. METHODS: This was a retrospective study, including subjects with idiopathic PAH and minor or repaired congenital heart disease. Forty-nine subjects were included, in whom cardiac catheterization and echocardiography were performed within 1 month. Fourteen additional patients were included in a separate cohort, in whom catheterization and echocardiography were performed simultaneously. Catheterization-derived RVSW was calculated as RV systolic pressure × (cardiac output/heart rate). Echocardiographically derived RVSW was calculated as 4 × (peak tricuspid regurgitant jet velocity)(2) × (pulmonary valve area × velocity-time integral). Statistics included the intraclass correlation coefficient and Bland-Altman analysis. RESULTS: Echocardiographically derived RVSW was linearly correlated with invasively derived RVSW (r = 0.74, P < .0001, intraclass correlation coefficient = 0.76). Bland-Altman analysis showed adequate agreement. Echocardiographically derived RV work was related to indexed pulmonary vascular resistance (r = 0.43, P = .002), tricuspid annular plane systolic excursion (r = 0.41, P = .004), and RV wall thickness (r = 0.62, P < .0001). CONCLUSIONS: The authors demonstrate that RV work, a potential novel index of RV function, can be estimated noninvasively and is related to pulmonary hemodynamics and other indices of RV performance.