RESUMO
This scientific statement from the American Heart Association focuses on treatment strategies and modalities for cardiomyopathy (heart muscle disease) in children and serves as a companion scientific statement for the recent statement on the classification and diagnosis of cardiomyopathy in children. We propose that the foundation of treatment of pediatric cardiomyopathies is based on these principles applied as personalized therapy for children with cardiomyopathy: (1) identification of the specific cardiac pathophysiology; (2) determination of the root cause of the cardiomyopathy so that, if applicable, cause-specific treatment can occur (precision medicine); and (3) application of therapies based on the associated clinical milieu of the patient. These clinical milieus include patients at risk for developing cardiomyopathy (cardiomyopathy phenotype negative), asymptomatic patients with cardiomyopathy (phenotype positive), patients with symptomatic cardiomyopathy, and patients with end-stage cardiomyopathy. This scientific statement focuses primarily on the most frequent phenotypes, dilated and hypertrophic, that occur in children. Other less frequent cardiomyopathies, including left ventricular noncompaction, restrictive cardiomyopathy, and arrhythmogenic cardiomyopathy, are discussed in less detail. Suggestions are based on previous clinical and investigational experience, extrapolating therapies for cardiomyopathies in adults to children and noting the problems and challenges that have arisen in this experience. These likely underscore the increasingly apparent differences in pathogenesis and even pathophysiology in childhood cardiomyopathies compared with adult disease. These differences will likely affect the utility of some adult therapy strategies. Therefore, special emphasis has been placed on cause-specific therapies in children for prevention and attenuation of their cardiomyopathy in addition to symptomatic treatments. Current investigational strategies and treatments not in wide clinical practice, including future direction for investigational management strategies, trial designs, and collaborative networks, are also discussed because they have the potential to further refine and improve the health and outcomes of children with cardiomyopathy in the future.
Assuntos
Cardiomiopatias , Cardiomiopatia Restritiva , Cardiopatias , Humanos , American Heart Association , Cardiomiopatias/diagnóstico , Cardiomiopatias/terapia , Cardiomiopatias/etiologia , Cardiopatias/complicações , Fenótipo , CriançaRESUMO
BACKGROUND: Continuous-flow ventricular assist devices (CF-VADs) are used increasingly in pediatric end-stage heart failure (ESHF) patients. Alongside common risk factors like oxidant injury from hemolysis, non-pulsatile flow constitutes a unique circulatory stress on kidneys. Post-implantation recovery after acute kidney injury (AKI) is commonly reported, but long-term kidney outcomes or factors implicated in the evolution of chronic kidney disease (CKD) with prolonged CF-VAD support are unknown. METHODS: We studied ESHF patients supported > 90 days on CF-VAD from 2008 to 2018. The primary outcome was CKD (per Kidney Disease Improving Global Outcomes (KDIGO) criteria). Secondary outcomes included AKI incidence post-implantation and CKD evolution in the 6-12 months of CF-VAD support. RESULTS: We enrolled 134 patients; 84/134 (63%) were male, median age was 13 [IQR 9.9, 15.9] years, 72/134 (54%) had preexisting CKD at implantation, and 85/134 (63%) had AKI. At 3 months, of the 91/134 (68%) still on a CF-VAD, 34/91 (37%) never had CKD, 13/91 (14%) developed de novo CKD, while CKD persisted or worsened in 49% (44/91). Etiology of heart failure, extracorporeal membrane oxygenation use, duration of CF-VAD, AKI history, and kidney replacement therapy were not associated with different CKD outcomes. Mortality was higher in those with AKI or preexisting CKD. CONCLUSIONS: In the first multicenter study to focus on kidney outcomes for pediatric long-term CF-VAD patients, preimplantation CKD and peri-implantation AKI were common. Both de novo CKD and worsening CKD can happen on prolonged CF-VAD support. Proactive kidney function monitoring and targeted follow-up are important to optimize outcomes.
Assuntos
Injúria Renal Aguda , Insuficiência Cardíaca , Coração Auxiliar , Insuficiência Renal Crônica , Criança , Humanos , Masculino , Adolescente , Feminino , Coração Auxiliar/efeitos adversos , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/terapia , Injúria Renal Aguda/epidemiologia , Injúria Renal Aguda/etiologia , Injúria Renal Aguda/terapia , Insuficiência Renal Crônica/terapia , Insuficiência Renal Crônica/complicações , Rim , Estudos Retrospectivos , Resultado do TratamentoRESUMO
BACKGROUND: Although ventricular failure is a late finding in adults with AC, we hypothesize that this is a presenting symptom in pediatric heart failure patients who undergo HT and that their ventricular arrhythmia burden could differentiate AC from other cardiomyopathies. METHODS: We performed a single-center retrospective cohort study reviewing 457 consecutive pediatric (≤18 years) HT recipients at our institution. Explanted hearts were examined to establish the primary diagnosis, based on pathologic findings. Demographic and clinical variables were compared between AC versus non-HCM cardiomyopathy cases. RESULTS: Forty-five percent (n = 205/457) had non-HCM cardiomyopathies as the underlying primary diagnosis. Ten cases (10/205 = 4.9%) were diagnosed with AC. All 10 had biventricular disease. In 8/10 patients (80%), AC diagnosis was unrecognized pre-HT. Compared with non-AC cardiomyopathies, the AC group was older at diagnosis (9.3 years vs. 4.3 years, p = .012) and transplant (11.1 years vs. 6.5 years, p = .010), had more ventricular arrhythmias (80.0% vs 32.8%, p = .003), and required more anti-arrhythmic use (80.0% vs 32.3%, p = .001). Genetic testing yielded causative pathogenic variants in all tested individuals (n = 5/5, 100%). CONCLUSION: AC is often an unrecognized cardiomyopathy pretransplant in children who undergo HT. Pediatric non-HCM phenotypes with heart failure who have a significant ventricular arrhythmia burden should be investigated for AC.
Assuntos
Cardiomiopatias , Insuficiência Cardíaca , Humanos , Estudos Retrospectivos , Cardiomiopatias/complicações , Cardiomiopatias/diagnóstico , Cardiomiopatias/patologia , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/cirurgia , Arritmias Cardíacas/complicações , Arritmias Cardíacas/diagnóstico , AntiarrítmicosRESUMO
BACKGROUND: Valvular disease in pediatric and young adult donor hearts may be a relative contraindication to graft use. Outcomes following the use of donor hearts with bicuspid aortic valve (BAV) have not been previously reported in children. We describe 4 cases of pediatric heart transplantation (HTx) utilizing a donor heart with a BAV. CASE SERIES: Of the 469 HTx included in this study, 4 utilized a donor heart with a BAV. All recipients were female; median age was 11 years (range 0.3 to 19 years). In all cases, the BAV was not discovered until after HTx. All donors were less than 30 years old. The patients were followed for a median of 6 years (range 2 to 9 years) with all patients alive at last follow-up. Two patients have transitioned to adult care, and 2 patients continue to follow in our clinic. In follow-up, no patient has required an aortic valve intervention or had infective endocarditis. At last review, no patient had greater than mild aortic insufficiency or more than mild aortic stenosis. Three patients developed mild-to-moderate left ventricular hypertrophy in the first year post-transplant that improved over time. One patient experienced a peri-operative embolic stroke at time of transplant unrelated to the BAV. CONCLUSION: On short- and intermediate-term follow-up, pediatric and young adult donor hearts with BAV demonstrated acceptable graft longevity and valvular function. A functionally normal BAV in a pediatric heart transplant donor should not be a contraindication to organ acceptance.
Assuntos
Doença da Válvula Aórtica Bicúspide , Transplante de Coração , Doenças das Valvas Cardíacas , Adolescente , Adulto , Valva Aórtica/cirurgia , Criança , Pré-Escolar , Feminino , Doenças das Valvas Cardíacas/complicações , Doenças das Valvas Cardíacas/cirurgia , Humanos , Lactente , Masculino , Estudos Retrospectivos , Doadores de Tecidos , Adulto JovemRESUMO
BACKGROUND: Pediatric sHKTx remains uncommon in the US. We examined outcomes of pediatric sHKTx compared to PHTx alone. Our objective was to identify a threshold eGFR that justified pediatric sHKTx. METHODS: Data from the SRTR heart and kidney databases were used to identify 9245 PHTx, and 63 pediatric sHKTx performed between 1992 and 2017 (age ≤21 years). RESULTS: The median age for sHKTx was 16 years, and included 31 males (31/63 = 49%). Over half of sHKTx (36/63 = 57%) were performed in cases where pretransplant dialysis was initiated. Among patients who required pretransplant dialysis, the risk of death in sHKTx recipients was significantly lower than PHTx alone (sHKTx vs. PHTx: HR 0.4, 95% CI [0.2, 0.9], p = .01). In those without pretransplant dialysis, there was no improvement in survival between sHKTx and PHTx (p = .2). When stratified by eGFR, PHTx alone recipients had worse survival than sHKTx in the group with eGFR ≤35 ml/min/1.73 m2 (p = .04). The 1- and 5-year actuarial survival rates in pediatric sHKTx recipients were 87% and 81.5% respectively and was similar to isolated PHTx (p = .5). One-year rates of treated heart (11%) and kidney (7.9%) rejection were similar in sHKTx compared to PHTx alone (p = .7) and pediatric kidney transplant alone (p = .5) respectively. CONCLUSION: Pediatric sHKTx should be considered in HTx candidates with kidney failure requiring dialysis or eGFR ≤35 ml/min/1.73 m2 . The utility of sHKTx in cases of kidney failure not requiring dialysis warrants further study.
Assuntos
Insuficiência Cardíaca/cirurgia , Transplante de Coração/métodos , Falência Renal Crônica/cirurgia , Transplante de Rim/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Taxa de Filtração Glomerular , Sobrevivência de Enxerto , Insuficiência Cardíaca/complicações , Transplante de Coração/mortalidade , Humanos , Lactente , Estimativa de Kaplan-Meier , Falência Renal Crônica/complicações , Falência Renal Crônica/mortalidade , Falência Renal Crônica/terapia , Transplante de Rim/mortalidade , Modelos Logísticos , Masculino , Diálise Renal , Estudos Retrospectivos , Resultado do TratamentoRESUMO
BACKGROUND: Guidance and data on ventricular assist device (VAD) support for children with chemotherapy-induced cardiomyopathy, particularly within the first 2 years after chemotherapy, are limited. METHODS: We performed a single-center retrospective case series, reviewing medical records of children <18 years of age with chemotherapy-induced cardiomyopathy and advanced heart failure (HF) who received durable VAD support. RESULTS: Six patients met inclusion criteria-5 HeartWare™ HVAD, 1 Berlin Heart EXCOR® . Median age at cancer diagnosis was 6 years (IQR 4.5-10 years). Median dose of anthracycline received was 540 mg/m2 (IQR 450-630 mg/m2 ). All patients developed HF within 1 year after initiation of cancer treatment (median 8 months, IQR 6-11.5 months) and were initiated on durable VAD support at a median of 8 months after completion of cancer treatment (IQR 3.3-43.5 months). Four patients had significant right ventricular dysfunction needing oral pulmonary vasodilator therapy, one patient had a major bleeding complication, and two patients had thromboembolic strokes while on VAD support. Median duration of VAD support was 7.5 months (IQR 3-11.3 months). Two patients underwent VAD explant due to recovery of LV function, one died due to cancer progression, and three underwent heart transplantation. CONCLUSIONS: Durable VAD support should be considered as a therapeutic option for children who have advanced HF due to chemotherapy-induced cardiomyopathy, even within 2 years of completing cancer treatment. A multi-disciplinary approach is essential for appropriate patient selection prior to implant and to ensure comprehensive care throughout the duration of VAD support.
Assuntos
Antineoplásicos , Cardiomiopatias , Insuficiência Cardíaca , Transplante de Coração , Coração Auxiliar , Antineoplásicos/efeitos adversos , Cardiomiopatias/induzido quimicamente , Cardiomiopatias/terapia , Criança , Insuficiência Cardíaca/etiologia , Transplante de Coração/efeitos adversos , Coração Auxiliar/efeitos adversos , Humanos , Estudos Retrospectivos , Resultado do TratamentoRESUMO
OBJECTIVES: Bivalirudin is a direct thrombin inhibitor that is being increasingly used for anticoagulation in children after ventricular assist device (VAD) implantation. While the data on bivalirudin use in pulsatile flow VADs are growing, reports on its use in patients on continuous flow (CF) VAD as well as comparisons of associated outcomes with unfractionated heparin (UFH) remain limited. DESIGN: Retrospective cohort study. SETTING: Single tertiary-quaternary referral center. PATIENTS: All patients less than 21 years old on CF-VAD support who received bivalirudin or UFH for anticoagulation between the years 2016 and 2020. INTERVENTIONS: Not applicable. MEASUREMENTS AND MAIN RESULTS: Clinical characteristics compared between the cohorts included time to target range of anticoagulation, markers of hemolysis, and prevalence of hemocompatibility-related adverse events such as major hemorrhagic complications, ischemic stroke, and pump thrombosis. In 42 unique patients (41 HeartWare HVAD [Medtronic, Minneapolis, MN], one HeartMate 3 LVAD [Abbott Laboratories, Abbott Park, IL]) during the study period, a total of 67 encounters of IV anticoagulation infusions (29 UFH and 38 bivalirudin) were retrospectively reviewed. In comparison with use of UFH, bivalirudin was associated with lesser odds of major bleeding complications (odds ratio [OR], 0.29; 95% CI, 0.09-0.97; p = 0.038). We failed to identify any difference in odds of major thrombotic complications (OR, 2.53; 95% CI, 0.47-13.59; p = 0.450). Eight of the patients (28%) on UFH were switched to bivalirudin due to hemorrhagic or thrombotic complications or inability to achieve therapeutic anticoagulation, while two of the patients (5%) on bivalirudin were switched to UFH due to hemorrhagic complications. Bivalirudin was used for a "washout" in eight cases with concern for pump thrombosis-six had resolution of the pump thrombosis, while two needed pump exchange. CONCLUSIONS: Use of bivalirudin for anticoagulation in patients on CF-VAD support was associated with lesser odds of hemorrhagic complications compared with use of UFH. Bivalirudin "washout" was successful in medical management of six of eight cases of possible pump thrombosis.
Assuntos
Coração Auxiliar , Trombose , Adulto , Anticoagulantes/efeitos adversos , Antitrombinas/efeitos adversos , Criança , Coração Auxiliar/efeitos adversos , Hemorragia/induzido quimicamente , Hemorragia/epidemiologia , Heparina/efeitos adversos , Hirudinas/efeitos adversos , Humanos , Fragmentos de Peptídeos/efeitos adversos , Proteínas Recombinantes/efeitos adversos , Estudos Retrospectivos , Trombose/epidemiologia , Trombose/etiologia , Trombose/prevenção & controle , Resultado do Tratamento , Adulto JovemRESUMO
INTRODUCTION: Iron deficiency is associated with worse outcomes in children and adults with systolic heart failure. While oral iron replacement has been shown to be ineffective in adults with heart failure, its efficacy in children with heart failure is unknown. We hypothesised that oral iron would be ineffective in replenishing iron stores in ≥50% of children with heart failure. METHODS: We performed a single-centre retrospective cohort study of patients aged ≤21 years with systolic heart failure and iron deficiency who received oral iron between 01/2013 and 04/2019. Iron deficiency was defined as ≥2 of the following: serum iron <50 mcg/dL, serum ferritin <20 ng/mL, transferrin >300 ng/mL, transferrin saturation <15%. Iron studies and haematologic indices pre- and post-iron therapy were compared using paired-samples Wilcoxon test. RESULTS: Fifty-one children with systolic heart failure and iron deficiency (median age 11 years, 49% female) met inclusion criteria. Heart failure aetiologies included cardiomyopathy (51%), congenital heart disease (37%), and history of heart transplantation with graft dysfunction (12%). Median dose of oral iron therapy was 2.9 mg/kg/day of elemental iron, prescribed for a median duration of 96 days. Follow-up iron testing was available for 20 patients, of whom 55% (11/20) remained iron deficient despite oral iron therapy. CONCLUSIONS: This is the first report on the efficacy of oral iron therapy in children with heart failure. Over half of the children with heart failure did not respond to oral iron and remained iron deficient.
Assuntos
Anemia Ferropriva , Insuficiência Cardíaca Sistólica , Insuficiência Cardíaca , Deficiências de Ferro , Adulto , Anemia Ferropriva/tratamento farmacológico , Anemia Ferropriva/etiologia , Criança , Feminino , Insuficiência Cardíaca/complicações , Insuficiência Cardíaca/tratamento farmacológico , Insuficiência Cardíaca Sistólica/complicações , Insuficiência Cardíaca Sistólica/tratamento farmacológico , Humanos , Ferro/uso terapêutico , Masculino , Estudos Retrospectivos , Transferrina/uso terapêuticoRESUMO
BACKGROUND: Pediatric HLT remains uncommon in the United States and criteria for HLT are unclear. The objectives of this study were to review the indications, and outcomes of pediatric HLT. METHODS: Data from the Scientific Registry of Transplant Recipients heart and liver databases were used to identify 9245 pediatric isolated heart transplants (PHT), 14 134 pediatric isolated liver transplant (PLT), and 20 pediatric HLT (16 patients underwent sHLT [same organ donor] and four patients with a history of PHT followed by PLT [different organ donors]; age ≤21 years) between 1992 and 2017. Outcomes included patient survival, and 1-year rates of acute heart and liver rejection. RESULTS: The median age for pediatric HLT was 15.6 (IQR: 10.5, 17.9) years, and included 12 males (12/20 = 60%). In the HLT group, the most common indication for HT was CHD (12/20 = 60%), and the most common indication for liver transplant was cirrhosis (9/20 = 45%). The 1, 3, and 5 year actuarial survival rates in pediatric simultaneous HLT recipients (n = 16) were 93%, 93%, and 93%, respectively, and was similar to isolated PHT alone (88%, 81%, and 75.5%, respectively and isolated PLT alone (84%, 82%, and 80%), respectively. There was no heart or liver rejection reported in the HLT group versus 9.9% in heart and 10.6% in liver transplant-only groups, respectively. CONCLUSION: Pediatric HLT is an uncommon but acceptable option for recipients with combined end-organ failure, with intermediate survival outcomes comparable to those of single-organ recipients.
Assuntos
Transplante de Coração , Transplante de Fígado , Avaliação de Processos e Resultados em Cuidados de Saúde , Adolescente , Criança , Estudos de Coortes , Feminino , Rejeição de Enxerto , Sobrevivência de Enxerto , Humanos , Masculino , Sistema de Registros , Taxa de Sobrevida , Estados UnidosRESUMO
BACKGROUND: Pre-mature birth impacts left ventricular development, predisposing this population to long-term cardiovascular risk. The aims of this study were to investigate maturational changes in rotational properties from the neonatal period through 1 year of age and to discern the impact of cardiopulmonary complications of pre-maturity on these measures. METHODS: Pre-term infants (<29 weeks at birth, n = 117) were prospectively enrolled and followed to 1-year corrected age. Left ventricular basal and apical rotation, twist, and torsion were measured by two-dimensional speckle-tracking echocardiography and analysed at 32 and 36 weeks post-menstrual age and 1-year corrected age. A mixed random effects model with repeated measures analysis was used to compare rotational mechanics over time. Torsion was compared in infants with and without complications of cardiopulmonary diseases of pre-maturity, specifically bronchopulmonary dysplasia, pulmonary hypertension, and patent ductus arteriosus. RESULTS: Torsion decreased from 32 weeks post-menstrual age to 1-year corrected age in all pre-term infants (p < 0.001). The decline from 32 to 36 weeks post-menstrual age was more pronounced in infants with cardiopulmonary complications, but was similar to healthy pre-term infants from 36 weeks post-menstrual age to 1-year corrected age. The decline was due to directional and magnitude changes in apical rotation over time (p < 0.05). CONCLUSION: This study tracks maturational patterns of rotational mechanics in pre-term infants and reveals torsion declines from the neonatal period through 1 year. Cardiopulmonary diseases of pre-maturity may negatively impact rotational mechanics during the neonatal period, but the myocardium recovers by 1-year corrected age.
Assuntos
Displasia Broncopulmonar , Permeabilidade do Canal Arterial , Ventrículos do Coração , Hipertensão Pulmonar , Displasia Broncopulmonar/diagnóstico por imagem , Permeabilidade do Canal Arterial/diagnóstico por imagem , Ecocardiografia , Ventrículos do Coração/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido , Função Ventricular EsquerdaRESUMO
BACKGROUND: Loop diuretics are considered first-line therapy for congestion in children with heart failure, although some patients remain volume overloaded during treatment. We sought to characterize loop diuretic responsiveness (DR) in children hospitalized with acute decompensated failure and to determine whether a decreased response was associated with worse outcomes. METHODS AND RESULTS: DR was calculated for 108 consecutive children Ë21 years of age who were hospitalized with acute decompensated heart failure. DR was defined as net fluid (mL) output per 1 mg of furosemide equivalents during the first 72 hours of treatment with a loop diuretic. The primary outcome was the composite end point of inpatient death or use of mechanical circulatory support. The median DR was 6.0 mL/mg (interquartile range -2.4 to 15.7 mL/mg). Thirty-two percent of patients remained in a positive fluid balance after 72 hours of treatment with a loop diuretic. Death or use of mechanical circulatory support occurred in 29 patients (27%). Low DR was associated with the composite end point, even after adjusting for net urine output and loop diuretic dose indexed to weight (odds ratio 5.3; Pâ¯=â¯.003). Patients with low DR also experienced longer length of hospital stay than patients with greater DR (median 33 days vs 11 days; Pâ¯=â¯.002). CONCLUSION: In children hospitalized with acute decompensated heart failure, early diminished loop DR during decongestion therapy is common and portends a poor prognosis.
Assuntos
Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/tratamento farmacológico , Inibidores de Simportadores de Cloreto de Sódio e Potássio/uso terapêutico , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Insuficiência Cardíaca/fisiopatologia , Humanos , Lactente , Masculino , Prognóstico , Estudos Retrospectivos , Resultado do TratamentoRESUMO
INTRODUCTION: Prematurity impacts myocardial development and may determine long-term outcomes. The objective of this study was to test the hypothesis that preterm neonates develop right ventricle dysfunction and adaptive remodelling by 32 weeks post-menstrual age that persists through 1 year corrected age. MATERIALS AND METHODS: A subset of 80 preterm infants (born <29 weeks) was selected retrospectively from a prospectively enrolled cohort and measures of right ventricle systolic function and morphology by two-dimensional echocardiography were assessed at 32 weeks post-menstrual age and at 1 year of corrected age. Comparisons were made to 50 term infants at 1 month and 1 year of age. Sub-analyses were performed in preterm-born infants with bronchopulmonary dysplasia and/or pulmonary hypertension. RESULT: In both term and preterm infants, right ventricle function and morphology increased over the first year (p < 0.01). The magnitudes of right ventricle function measures were lower in preterm-born infants at each time period (p < 0.01 for all) and right ventricle morphology indices were wider in all preterm infants by 1 year corrected age, irrespective of lung disease. Measures of a) right ventricle function were further decreased and b) morphology increased through 1 year in preterm infants with bronchopulmonary dysplasia and/or pulmonary hypertension (p < 0.01). CONCLUSION: Preterm infants exhibit abnormal right ventricle performance with remodelling at 32 weeks post-menstrual age that persists through 1 year corrected age, suggesting a less developed intrinsic myocardial function response following preterm birth. The development of bronchopulmonary dysplasia and pulmonary hypertension leave a further negative impact on right ventricle mechanics over the first year of age.
Assuntos
Displasia Broncopulmonar/complicações , Hipertensão Pulmonar/complicações , Doenças do Prematuro/patologia , Disfunção Ventricular Direita/etiologia , Disfunção Ventricular Direita/patologia , Remodelação Ventricular , Displasia Broncopulmonar/patologia , Ecocardiografia , Feminino , Humanos , Hipertensão Pulmonar/patologia , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Doenças do Prematuro/diagnóstico por imagem , Doenças do Prematuro/etiologia , Masculino , Estudos Retrospectivos , Disfunção Ventricular Direita/diagnóstico por imagemRESUMO
OBJECTIVE: To test the hypothesis that echocardiographic markers of pulmonary vascular disease (PVD) exist in asymptomatic infants born preterm at 1-year corrected age. STUDY DESIGN: We conducted a prospective cohort study of 80 infants born preterm (<29 weeks of gestation) and 100 age- and weight-matched infants born at term and compared broad-based conventional and quantitative echocardiographic measures of pulmonary hemodynamics at 1-year corrected age. Pulmonary artery acceleration time (PAAT), a validated index of pulmonary vascular resistance, arterial pressure, and compliance, was used to assess pulmonary hemodynamics. Lower PAAT is indicative of PVD. Subanalyses were performed in infants with bronchopulmonary dysplasia (BPD, n = 48, 59%) and/or late-onset pulmonary hypertension (n = 12, 15%). RESULTS: At 1 year, there were no differences between conventional measures of pulmonary hypertension in the infants born at term and preterm. All infants born preterm had significantly lower values of PAAT than infants born at term (73 ± 8 milliseconds vs 98 ± 5 milliseconds, P < .001). Infants born preterm with BPD had even lower PAAT than those without BPD (69 ± 5 milliseconds vs 79 ± 4 milliseconds, P < .01). The degree of PVD at 1-year corrected age was inversely related to gestation in all infants born preterm. Data analysis included adjustment for ventricular function and other confounding factors. CONCLUSIONS: In comparison with infants born at term, infants born preterm exhibit abnormal PAAT at 1-year corrected age irrespective of neonatal lung disease status, suggesting the existence of PVD beyond infancy. PAAT measurements offer a reliable, noninvasive tool for screening and longitudinal monitoring of pulmonary hemodynamics in infants.
Assuntos
Displasia Broncopulmonar/complicações , Ecocardiografia/métodos , Hipertensão Pulmonar/diagnóstico por imagem , Artéria Pulmonar/diagnóstico por imagem , Resistência Vascular/fisiologia , Biomarcadores , Estudos de Coortes , Feminino , Hemodinâmica/fisiologia , Humanos , Hipertensão Pulmonar/fisiopatologia , Recém-Nascido , Recém-Nascido Prematuro , Estudos Longitudinais , Masculino , Estudos Prospectivos , Artéria Pulmonar/fisiopatologia , Circulação Pulmonar/fisiologiaRESUMO
BackgroundTo test the hypothesis that infants born to obese women with pre-gestational type 2 diabetes mellitus (IBDMs) have ventricular dysfunction at 1 month that is associated with markers of maternal lipid and glucose metabolism.MethodsIn a prospective observational study of IBDMs (OB+DM, n=25), echocardiographic measures of septal, left (LV) and right ventricular (RV) function, and structure were compared at 1 month of age with those in infants born to OB mothers without DM (OB, n=24) and to infants born to non-OB mothers without DM (Lean, n=23). Basal maternal lipid and glucose kinetics and maternal plasma and infant (cord) plasma were collected for hormone and cytokine analyses.ResultsRV, LV, and septal strain measures were lower in the OB+DM infants compared with those in other groups, without evidence of septal hypertrophy. Maternal hepatic insulin sensitivity, maternal plasma free-fatty-acid concentration, and cord plasma insulin and leptin most strongly predicted decreased septal strain in OB+DM infants.ConclusionIBDMs have reduced septal function at 1 month in the absence of septal hypertrophy, which is associated with altered maternal and infant lipid and glucose metabolism. These findings suggest that maternal obesity and DM may have a prolonged impact on the cardiovascular health of their offspring, despite the resolution of cardiac hypertrophy.
Assuntos
Glicemia/metabolismo , Diabetes Mellitus Tipo 2/complicações , Lipídeos/sangue , Obesidade/complicações , Gravidez em Diabéticas , Efeitos Tardios da Exposição Pré-Natal , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Direita/etiologia , Função Ventricular Esquerda , Função Ventricular Direita , Adulto , Biomarcadores/sangue , Diabetes Mellitus Tipo 2/sangue , Diabetes Mellitus Tipo 2/diagnóstico , Ecocardiografia , Feminino , Sangue Fetal/metabolismo , Humanos , Lactente , Recém-Nascido , Obesidade/sangue , Obesidade/diagnóstico , Gravidez , Estudos Prospectivos , Fatores de Risco , Fatores de Tempo , Disfunção Ventricular Esquerda/sangue , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/fisiopatologia , Disfunção Ventricular Direita/sangue , Disfunção Ventricular Direita/diagnóstico por imagem , Disfunção Ventricular Direita/fisiopatologia , Adulto JovemRESUMO
BACKGROUND: Carotid artery access in infants with congenital heart disease undergoing cardiac catheterization via a surgical cut down has been well described. There is a paucity of information regarding percutaneous carotid artery (CA) access in infants <3 months. METHODS: A retrospective review of infants <3 months of age undergoing cardiac catheterization via percutaneous CA approach was performed after IRB approval. Between January 2012 and May 2015, 18 patients underwent 20 procedures; median age 13 days (2-77); median weight 3.3 kg (1.6-5). Procedures performed were patent ductus arteriosus (PDA) stenting (8), modified blalock taussig (BT) shunt stenting (3), balloon aortic valvuloplasty (6), and balloon angioplasty of coarctation (3). RESULTS: Percutaneous access was obtained with a Doppler needle under ultrasound guidance into the right (16) and left CA (4). Sheath size used was 4 Fr (17), 5 Fr (2), and 6 Fr (1). Median time to sheath insertion was 6.5 min (2-20). Percutaneous access was obtained successfully in all cases. There were no major procedural complications. There were two minor complications; hypotension, and ductal spasm. Hemostasis was achieved by manual compression; median time was 14.5 min (8-36). There were two post-procedural complications involving development of CA pseudo aneurysms that were repaired surgically. Post-procedure CA patency was documented by angiography (3), MRA (3), or vascular ultrasound (14). There were no documented arterial occlusions. CONCLUSION: Our experience suggests that percutaneous CA access in infants <3 months of age is safe and feasible with preserved vascular patency and no neurological adverse events.
Assuntos
Cateterismo Cardíaco/métodos , Artérias Carótidas , Cateterismo Periférico/métodos , Cardiopatias Congênitas/terapia , Fatores Etários , Angioplastia com Balão , Valvuloplastia com Balão , Procedimento de Blalock-Taussig , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/instrumentação , Cateteres Cardíacos , Artérias Carótidas/diagnóstico por imagem , Artérias Carótidas/fisiopatologia , Cateterismo Periférico/efeitos adversos , Cateterismo Periférico/instrumentação , Feminino , Cardiopatias Congênitas/diagnóstico , Humanos , Lactente , Recém-Nascido , Masculino , Punções , Estudos Retrospectivos , Stents , Resultado do Tratamento , Grau de Desobstrução VascularRESUMO
Background: Traditionally, low cardiac output has been considered the primary hemodynamic driver of renal function and injury. Adult data suggest that central venous pressure (CVP) is a more important factor. Objectives: The authors hypothesized that in children with cardiovascular disease, higher CVP predicts lower estimated glomerular filtration rate (eGFR) and worsening renal function (WRF). Methods: We performed a single-center cohort study of patients aged 3 months to 21 years with biventricular circulation undergoing cardiac catheterization. Pearson's correlation and linear and Cox regression analyses were performed to determine associations with eGFR at the time of catheterization and WFR within 180 days after catheterization. Results: 312 patients had median age 7.9 years (IQR: 2.3 to 14.5 years), median eGFR 97 mL/min/1.73 m2 (IQR: 81-118 mL/min/1.73 m2), median CVP 7 mm Hg (IQR: 5-9 mm Hg), and median cardiac index 3.7 mL/min/m2 (IQR: 2.9-4.6 mL/min/m2). Nearly half (48%) were transplant recipients. In multivariable analysis, CVP was independently associated with eGFR (ß = -2.65; 95% CI: -4.02, -1.28; P < 0.001), as was being a transplant recipient (ß = -10.20; 95% CI: -17.74, -2.65; P = 0.008), while cardiac index was not. Fifty-one patients (16%) developed WRF. In a proportional hazards model adjusting for cardiac index, only higher CVP (HR: 1.10; 95% CI: 1.04-1.17; P = 0.002) and greater contrast volume by weight (HR: 1.05; 95% CI: 1.01-1.10; P = 0.021) predicted WRF. CVP ≥7 mm Hg likewise predicted WRF (HR: 2.57; 95% CI: 1.29-5.12; P = 0.007). Conclusions: Among children with a spectrum of cardiovascular disease, higher CVP is associated with lower eGFR and development of WRF, independent of cardiac index.
RESUMO
BACKGROUND: Despite growing cardiogenic shock (CS) research in adults, the epidemiology, clinical features, and outcomes of children with CS are lacking. OBJECTIVES: This study sought to describe the epidemiology, clinical presentation, hospital course, risk factors, and outcomes of CS among children hospitalized for acute decompensated heart failure (ADHF). METHODS: We examined consecutive ADHF hospitalizations (<21 years of age) from a large single-center retrospective cohort. Patients with CS at presentation were analyzed and risk factors for CS and for the primary outcome of in-hospital mortality were identified. A modified Society for Cardiovascular Angiography and Interventions shock classification was created and patients were staged accordingly. RESULTS: A total of 803 hospitalizations for ADHF were identified in 591 unique patients (median age 7.6 years). CS occurred in 207 (26%) hospitalizations. ADHF hospitalizations with CS were characterized by worse systolic function (P = 0.040), higher B-type natriuretic peptide concentration (P = 0.032), and more frequent early severe renal (P = 0.023) and liver (P < 0.001) injury than those without CS. Children presenting in CS received mechanical ventilation (87% vs 26%) and mechanical circulatory support (45% vs 16%) more frequently (both P < 0.001). Analyzing only the most recent ADHF hospitalization, children with CS were at increased risk of in-hospital mortality compared with children without CS (28% vs 11%; OR: 1.91; 95% CI: 1.05-3.45; P = 0.033). Each higher CS stage was associated with greater inpatient mortality (OR: 2.40-8.90; all P < 0.001). CONCLUSIONS: CS occurs in 26% of pediatric hospitalizations for ADHF and is independently associated with hospital mortality. A modified Society for Cardiovascular Angiography and Interventions classification for CS severity showed robust association with increasing mortality.
Assuntos
Insuficiência Cardíaca , Choque Cardiogênico , Adulto , Humanos , Criança , Choque Cardiogênico/epidemiologia , Choque Cardiogênico/terapia , Choque Cardiogênico/etiologia , Estudos Retrospectivos , Insuficiência Cardíaca/epidemiologia , Hospitalização , Fatores de Risco , Mortalidade HospitalarRESUMO
BACKGROUND: The significance of atypical infiltrates (eosinophils or plasma cells) on endomyocardial biopsy (EMB) after pediatric heart transplant (HTx) is not known. We hypothesized that atypical infiltrates are associated with worse post-HTx outcomes. METHODS: We performed a retrospective cohort study of consecutive patients <21 years old who underwent primary HTx between 2013 and 2017. Multiorgan transplants were excluded. The presence of atypical infiltrates and burden of atypical infiltrates (rare vs predominant) on EMB were recorded. Primary outcome was a composite of cardiac allograft vasculopathy, graft failure (relisting or retransplant), or death. Presence of atypical infiltrates was evaluated: (1) overall using Cox regression with time-dependent covariates and (2) if present by 1 year post-HTx using Kaplan-Meier analysis. RESULTS: Atypical infiltrates were present in 24 out of 95 patients (25%) and were associated with a higher likelihood of reaching the composite outcome (hazard ratio (HR) 6.22, 95% confidence interval (CI) 2.60-14.89, p < 0.0001). This persisted when controlling for rejection in multivariable analysis. There was also a greater risk of the composite outcome if ≥2 nonconsecutive EMBs had atypical infiltrates (HR 11.80, 95%CI 3.17-43.84, p = 0.0002) or if atypical infiltrates were the predominant feature on EMB (HR 30.58, 95%CI 9.34-100.06, p < 0.0001). Patients with atypical infiltrates by 1-year post-HTx had a 5-year freedom from the composite outcome of 48%, compared to 90% if no atypical infiltrates had been present by this timepoint (log rank p = 0.002). CONCLUSIONS: The presence of atypical infiltrates on EMB is associated with significantly worse outcomes in children following HTx. These patients require closer follow-up to assess for developing graft dysfunction.