Association between tricuspid regurgitant pressure and heart failure severity in children with acyanotic congenital heart disease receiving sildenafil therapy

ObjectiveTo investigate the prevalence, profile and predictors of severe malnutrition in children with congenital heart defects (CHDs).DesignCase–control, observational study.SettingTertiary teaching hospital in Lagos, Nigeria (March 2006 to March 2008).ParticipantsChildren aged...

Why is heart failure in children important? If we just consider the number of individuals affected, adult heart failure is clearly a more compelling public health problem. However, the relatively small numbers belie the overall economic and social impact of pediatric heart failure. When a child is admitted to the hospital for heart failure, the costs are considerably higher for children than adults because of the frequent need for surgical or catheter-based intervention. The demands of medical care can fray the family structure and adversely affect parental economic productivity. When a child dies of heart failure, the economic impact is magnified enormously because of the number of potentially productive years lost per death. For these and other reasons, heart failure in children is a serious public health concern. In addition, growing numbers of children with heart failure are reaching adulthood because of the successful application of medical and surgical heart failure therapies and the improved outcomes of congenital heart surgery. A greater understanding of the pathophysiology of heart failure in childhood may help inform therapeutic strategies once these children become adults. Furthermore, given the recent explosion of research in the impact of cardiac development and cardiogenetics on pediatric cardiovascular disease, it is not outside the realm of possibility for a pediatric discovery to be made that will also benefit adults with heart failure. We may not yet be able to agree on a definition of heart failure, but the cardinal symptoms, dyspnea, anasarca (“dropsy”), and cachexia, were well recognized in antiquity.1 These symptoms were not specific for cardiac pathology, and it was not until the 17th century, when William Harvey definitively identified the heart as an organ that pumped blood rather than generating heat, that the heart could begin to be understood as a source of dyspnea, edema, and …

To improve the accuracy of diagnosis of heart failure (HF) has been the focus of research for a long time. The diagnosis for HF with congenital heart disease, however, is more difficult. The aim of the study was to evaluate the diagnostic criteria for HF in children and examine the value of plasma brain natriuretic peptide (BNP) and NT-proBNP for diagnosing HF in pediatric patients with congenital heart disease, and to look for the most valuable index for the diagnosis according to the multifactor analysis. Totally 118 children with congenital heart disease were enrolled. They were diagnosed using modified Ross score, Qingdao criteria, NYU PHFI, and plasma BNP and NT-proBNP. According to modified Ross score as the referent criteria, other diagnostic criteria and plasma BNP and NT-proBNP were studied. The sensitivity, specificity and area of the ROC curve were examined. Logistic regression analysis was used to select the valuable index for diagnosing HF. (1) The value of each clinical criteria: 1 The sensitivity of Qingdao criteria for diagnosing HF was 47.9%. The specificity was 100% and the accuracy was 57.6%. 2 There were 52 patients younger than six months in whom 27 (51.9%) were breast fed. Only 25 children were measured with Ross score. The Ross score was positively correlated with the modified Ross score (r = 0.948). The area under the ROC curve of Ross score diagnosing HF was 0.985, and the sensitivity was 88%, while the specificity was 100%. 3 NYU PHFI score was positively correlated with the modified Ross score. The area under the ROC curve of the NYU PHFI diagnosing HF was 0.964, and the sum of sensitivity and specificity was favorite when > or = 8 was set as the cut-off point. If > 2 was set as cut-off point, it had a high sensitivity but a low specificity. The sensitivity of NYU PHFI was 100% > was set 2 as cut-point for diagnosing HF, but the specificity was 4.5%. (2) Plasma BNP and NT-proBNP were positively correlated with the modified Ross score, and increased with the severity of congestive HF. The area under the ROC curve of BNP was 0.880, and the cut-off line was > or = 349 pg/ml. The area under the ROC curve of NT-proBNP was 0.981, and the cut-off line was > or = 499 fmol/ml. (3) Logistic regression analysis showed that in multifactor analysis, only plasma concentration of NT-proBNP, dyspnea, tachycardia, tachypnea, failure to thrive were the independent predictors for diagnosing HF. (4) Plasma concentration of NT-proBNP incorporated with clinical criteria would improve its accuracy. All the clinical criteria commonly used were valuable for diagnosing HF in children with congenital heart disease, but each has its own limits, such as the low sensitivity of Qingdao, the low adaptation of Ross score because of the high breast-feeding rate in our country and the low specificity of NYU PHFI when > 2 was set as the cut-off point. Plasma concentrations of BNP and NT-proBNP were valuable for diagnosing HF in children with congenital heart disease, and NT-proBNP was the independent predictor for HF.

Heart failure (HF) represents an important cause of morbidity and mortality in children. It is mostly caused by congenital heart disease (CHD) and cardiomyopathy. The Ross HF classification was developed to assess severity in infants and has subsequently been modified to apply to all pediatric ages. The modified Ross classification for children provides a numeric score comparable with the New York Heart Association (NYHA) HF classification for adults. The aim of this work is to investigate the role of modified Ross score in the evaluation of children with severe lower respiratory tract infection admitted to the pediatric intensive care unit (PICU). One hundred and sixty-four children with severe LRTI admitted to the PICU were enrolled in this prospective cohort study, which was carried out at Assiut University Children Hospital, from the start of July 2021 up to the end of December 2021. Sixty patients (36.6%) of studied cases with severe LRTI admitted to PICU had HF. Out of these, 37 (61.7%) had mild HF; 17 (28.3%) had moderate HF, while six cases (10%) had severe HF according to the modified Ross score. The value of modified Ross score was significantly higher in children with heart failure with sensitivity and specificity 100% with cutoff value of 2. Admission to NICU, history of previous ventilation, and prematurity were higher in patients who developed HF. Patients with pulmonary hypertension (PH) and those with raised neutrophil lymphocyte ratio were significantly higher in the group of patients with moderate and severe degree of HF. Conclusion: Modified Ross score is a simple clinical score which may help in assessing and predicting children with severe LRTI.What is Known:• Hear failure is common complication to lower respiratory tract infection.• Modified Ross score was used to predict and classify heart failure in adult with lower respiratory infection.What is New:• Modified Ross score found to be of value in prediction of heart failure in children with lower respiratory tract infection.

International Society for Heart and Lung Transplantation: Practice guidelines for management of heart failure in children

This is the report of the American College of Veterinary Internal Medicine (ACVIM) Specialty of Cardiology consensus panel convened to formulate guidelines for the diagnosis and treatment of chronic valvular heart disease (CVHD, also known as endocardiosis and myxomatous valve degeneration) in dogs. It is estimated that approximately 10% of dogs presented to primary care veterinary practices have heart disease, and CVHD is the most common heart disease of dogs in many parts of the world, accounting for approximately 75% of canine cases of heart disease cases seen by veterinary practices in North America. CVHD most commonly affects the left atrioventricular or mitral valve, although in approximately 30% of cases the right atrioventricular (tricuspid) valve also is involved. The disease is approximately 1.5 times more common in males than in females. Its prevalence is also higher in smaller (<20 kg) dogs, although large breeds occasionally are affected.1 In small breed dogs, the disease generally is slowly but somewhat unpredictably progressive, with most dogs experiencing the onset of a recognizable murmur of mitral valve regurgitation years before the clinical onset of heart failure. When large breed dogs are affected by CVHD, the progression of the disease appears to be more rapid than that observed in small breed dogs.2 Cavalier King Charles Spaniels are predisposed to developing CVHD at a relatively young age, but the time course of their disease progression to heart failure does not appear to be markedly different from that of other small breed dogs except for the early age of onset.3,4 The cause of CVHD is unknown, but the disease appears to have an inherited component in some breeds studied.5,6 CVHD is characterized by changes in the cellular constituents as well as the intercellular matrix of the valve apparatus (including the valve leaflets and chordae tendineae).7,8 These changes involve both the collagen content and the alignment of collagen fibrils within the valve.9,10 Endothelial cell changes and subendothelial thickening also occur,11 although affected dogs do not appear to be at increased risk for arterial thromboembolism or infective endocarditis. Mitral valve prolapse is a common complication of myxomatous valve degeneration and represents a prominent feature of CVHD in some breeds.6,12 Progressive deformation of the valve structure eventually prevents effective coaptation and causes regurgitation (valve leakage). Progressive valvular regurgitation increases cardiac work, leading to ventricular remodeling (eccentric hypertrophy and intercellular matrix changes) and ventricular dysfunction. Abnormal numbers or types of mitogen receptors (eg, any of the subtypes of serotonin, endothelin, or angiotensin receptors) on fibroblast cell membranes in the valves of affected dogs may play a role in the pathophysiology of the valvular lesions.13 Systemic or local metabolic, neurohormonal or inflammatory mediators (eg, endogenous catecholamines and inflammatory cytokines) also may influence progression of the valve lesion or the subsequent myocardial remodeling and ventricular dysfunction that accompany long-standing, hemodynamically significant valvular regurgitation. However, these factors are poorly understood at this time.14 The prevalence of CVHD increases markedly with age in small breed dogs (with up to 85% showing some evidence of the lesion at necropsy by 13 years of age), but the presence of the pathologic lesion does not necessarily indicate that a dog will develop clinical signs of heart failure. Like the underlying cause of the disease, the factors that determine the progression of the lesion remain unknown, although age, left atrial size, and heart rate have been shown to predict outcomes.15,16 Heart failure is a general term that describes a clinical syndrome that can be caused by a variety of specific heart diseases, including CVHD. Heart failure from any cause is characterized by cardiac, hemodynamic, renal, neurohormonal, and cytokine abnormalities. The classification systems for heart failure most familiar to veterinarians are the modified New York Heart Association (NYHA)17 and International Small Animal Cardiac Health Council18 functional classification systems, both of which were designed to provide a framework for discussing and comparing the clinical signs of patients in heart failure. These functional classification systems vary in their details, but both serve as semiquantitative schemes for judging the severity of a patient's clinical signs. Such categorization aids in teaching therapeutic protocols and constitutes a basis for stratification of subjects in clinical trials. The modified NYHA functional classification of heart failure can be summarized as follows: Class I describes patients with asymptomatic heart disease (eg, CVHD is present, but no clinical signs are evident even with exercise). Class II describes patients with heart disease that causes clinical signs only during strenuous exercise. Class III describes patients with heart disease that causes clinical signs with routine daily activities or mild exercise. Class IV describes patients with heart disease that causes severe clinical signs even at rest. Functional classification systems share a common problem in that they are based on relatively subjective assessments of clinical signs that can change frequently and dramatically over short periods of time. Furthermore, treatments may not differ substantially across the functional classes. A newer classification system that might more objectively categorize patients in the course of their heart disease has been developed, and this scheme was used by the panel for consensus recommendations. The goal was to link severity of signs to appropriate treatments at each stage of illness. In formulating these guidelines, the consensus panel adapted the 2001 American College of Cardiology/American Heart Association classification system for the treatment of heart disease and failure in human patients to the management of canine CVHD.19 In this approach, patients are expected to advance from 1 stage to the next unless progression of the disease is altered by treatment. The classification system presented below and used in these guidelines is meant to complement, not replace, functional classification systems. The new system describes 4 basic stages of heart disease and failure: Stage A identifies patients at high risk for developing heart disease but that currently have no identifiable structural disorder of the heart (eg, every Cavalier King Charles Spaniel without a heart murmur). Stage B identifies patients with structural heart disease (eg, the typical murmur of mitral valve regurgitation is present), but that have never developed clinical signs caused by heart failure. Because of important clinical implications for prognosis and treatment, the panel further subdivided Stage B into Stage B1 and B2. Stage B1 refers to asymptomatic patients that have no radiographic or echocardiographic evidence of cardiac remodeling in response to CVHD. Stage B2 refers to asymptomatic patients that have hemodynamically significant valve regurgitation, as evidenced by radiographic or echocardiographic findings of left-sided heart enlargement. Stage C denotes patients with past or current clinical signs of heart failure associated with structural heart disease. Because of important treatment differences between dogs with acute heart failure requiring hospital care and those with heart failure that can be treated on an outpatient basis, these issues have been addressed separately by the panel. Some animals presenting with heart failure for the 1st time may have severe clinical signs requiring aggressive therapy (eg, with additional afterload reducers or temporary ventilatory assistance) that more typically would be reserved for those with refractory disease (see Stage D). Stage D refers to patients with end-stage disease with clinical signs of heart failure caused by CVHD that are refractory to "standard therapy" (defined later in this document). Such patients require advanced or specialized treatment strategies in order to remain clinically comfortable with their disease. As with Stage C, the panel has distinguished between animals in Stage D that require acute, hospital-based therapy and those that can be managed as outpatients. This classification system emphasizes that there are risk factors and structural prerequisites for the development of heart failure in CVHD. The use of this classification system is meant to encourage veterinary clinicians to think about heart disease in a way analogous to the current clinical approach to cancer. This classification system is designed to aid in: Developing screening programs for the presence of CVHD in dogs known to be at risk. Identifying interventions that may (now or in the future) decrease the risk of disease development. Identifying asymptomatic dogs with CVHD early in the course of their disease, comparable to "in situ" cancer, so that they can perhaps be treated more effectively. Identifying symptomatic dogs with CVHD so that these patients can be treated medically and either potentially cured (interventionally or surgically) or managed with their chronic disease. Identify symptomatic dogs with advanced heart failure from CVHD and refractory to conventional therapy—these patients require aggressive or new treatment strategies or potentially hospice-type end-of-life care. In classifying dogs with CVHD according to their disease stage and clinical status and matching them with diagnostic, pharmacologic, and dietary treatment recommendations, the consensus panel considered both the quantity and quality of evidence available to inform the diagnostic and therapeutic decisions made in these patients. The heading "Consensus recommendation" preceding a diagnostic, therapeutic, or dietary recommendation indicates that the panelists were unanimous in their opinion that the combination of available clinical trial evidence, other published experimental or anecdotal evidence, clinical experience, and expert opinion indicate that the potential benefit of the approach under discussion clearly outweighs the potential risks to the patient and minimizes financial impact on the client. In situations in which the available evidence regarding the efficacy of a diagnostic or therapeutic maneuver was conflicting, weak, or absent and no consensus on a recommended course of action could be reached by the panelists based on the available evidence and their collective clinical experience, the panel's opinions and reasoning on clinically important issues are briefly summarized. These bulleted summary statements are grouped together and summarized under the heading "No consensus." The panel recognized that there is considerable variation in the scientific quality of the evidence available to support clinical decision making, and sought to include topically relevant references. Whereas the status of a particular recommendation (consensus versus no consensus) reflects the collective judgment of the panel on each question addressed, no attempt was made to assign a specific scientific grade or value to each included citation. Stage A—Dogs at high risk for development of heart failure, but without apparent structural abnormality (no heart murmur is heard) at the time of examination. Consensus recommendations: Small breed dogs, including breeds with known predisposition to develop CVHD (eg, Cavalier King Charles Spaniels, Dachshunds, Miniature and Toy Poodles) should undergo regular evaluations (yearly auscultation by the family veterinarian) as part of routine health care. Owners of breeding dogs or those at especially high risk, such as Cavalier King Charles Spaniels, may choose to participate in yearly screening events at dog shows or other events sponsored by their breed association or kennel club and conducted by board-certified cardiologists participating in an ACVIM-approved disease registry. Consensus recommendations: No drug therapy is recommended for any patient. No dietary therapy is recommended for any patient. Potential breeding stock should no longer be bred if mitral regurgitation (MR) is identified early, during their normal breeding age of <6–8 years. Stage B—These patients have a structural abnormality indicating the presence of CVHD, but have never had clinical signs of heart failure. These patients are generally recognized during a screening or routine health examination with a heart murmur typical of mitral valve insufficiency. Consensus recommendations: Thoracic radiography is recommended in all patients to assess the hemodynamic significance of the murmur and also to obtain baseline thoracic radiographs at a time when the patient is asymptomatic for CVHD. Blood pressure measurement is recommended for all patients. In small breed dogs with typical murmurs, echocardiography is recommended to answer specific questions regarding either cardiac chamber enlargement or the cause of the murmur if those questions are not answered adequately by auscultation and thoracic radiography. Echocardiography generally is indicated in larger breed dogs because the murmur of MR is more likely to be related to other causes (eg, dilated cardiomyopathy). Basic laboratory work (a minimum of hematocrit, total protein concentration, serum creatinine concentration, and urinalysis) is indicated in all patients. Because their prognosis and therapy may differ substantially, asymptomatic patients with murmurs of mitral valve insufficiency are further subcategorized into 2 groups based on the results of the above evaluation: Stage B1: Hemodynamically insignificant MR (defined as radiographically or echocardiographically normal or equivocally enlarged LA, LV, or both, with normal LV systolic function; normal vertebral heart score on radiography; normotensive, normal laboratory results). Therapy for Stage B1 (both pharmacologic and dietary) is identical for both small and large breed dogs. Consensus recommendations: Small and large breed dogs: No drug or dietary therapy is recommended. Re-evaluation is suggested by either radiography or echocardiography with Doppler studies in approximately 12 months (some panelists recommend more frequent follow-up in large dogs). Stage B2: Hemodynamically significant MR with cardiac remodeling (defined as clearly enlarged LA, LV, or both); normotensive. Therapy for Stage B2 (both pharmacologic and dietary) is controversial, and no consensus could be reached with currently available evidence. No consensus: Small breed dogs: Angiogensin converting enzyme inhibitor (ACEI): For patients with clinically relevant left atrial enlargement on either initial examination, or those in which the left atrium has increased in size dramatically on successive monitoring examinations, a majority of the panel members recommend initiation of therapy with an ACEI. Clinical trials addressing the efficacy of ACEI for the treatment of dogs in Stage B2 have had mixed results—either no effect or a small positive effect delaying the onset of congestive heart failure.20–22 A minority of the panel members recommend no therapy for asymptomatic animals pending further clinical trials to examine the efficacy of therapy in this setting. β blockers: For patients with clinically relevant left atrial enlargement on either initial examination, or when the left atrium has increased in size dramatically on successive monitoring examinations, a minority of the panel members recommend initiation of therapy with a low dosage of a β blocker, titrating to the highest tolerated dose over a period of approximately 1–2 months depending on the specific medication recommended. A majority of the panel members recommend no β-blocker therapy for asymptomatic animals pending further clinical trials to examine the efficacy of therapy in this setting. Clinical trials addressing the efficacy of β blockers for the treatment of dogs in Stage B2 are in progress. No other pharmacologic treatments were recommended in Stage B2 by a majority of A panelists considered the use of the for patients in Stage B2 under specific and The panel in general that these treatment strategies additional into their efficacy and in this patient before a consensus recommendation could be treatment was recommended by a majority of panelists in Stage a minority of the panel recommended no dietary dietary treatment at this stage include mild dietary and of a with protein and for breed dogs: panelists recommended treatment in smaller breed dogs their the use of both ACEI and β blockers in larger breed dogs in Stage B2. treatment for larger breed dogs were the as those for small mild and protein and if changes were recommended. Stage have a structural abnormality and current or clinical signs of heart failure caused by CVHD. Stage C all patients that have had an of clinical heart failure. Such patients in this stage of their clinical signs with therapy if their clinical signs for are for both management of heart failure and for care management of heart failure, as well as for chronic dietary Some patients that in Stage C may have clinical and require more acute therapy than is considered These acute care patients may share some management strategies with dogs that have to Stage D heart failure, In Stage C, heart failure to CVHD, the panel not clinically relevant therapeutic between small and larger breed dogs for either acute or chronic For both C and D patients with symptomatic heart the acute care of heart failure is on the patient's hemodynamic status by monitoring well as under clinical and heart and to cardiac decrease the of mitral valve regurgitation if and clinical signs associated with either low cardiac or increased The of chronic management are on these hemodynamic to the additional treatments at clinical signs of congestive heart failure, and quality Consensus recommendations: Because of the relatively high prevalence of chronic disease in the at risk for CVHD, the presence of a typical left murmur in a dog does not necessarily that the clinical signs are the of CVHD. A clinical (including radiographs and an and basic laboratory be and to determine the cause of clinical signs in animals with CVHD. should in the cause of clinical signs in dogs with CVHD. there is no as a dogs with clinical signs caused by heart failure have higher serum than those with clinical signs caused by primary disease, the positive value of any concentration, by a available has not been adequately characterized at the time of this to a consensus recommendation with to The and examination can be in the of heart failure as a cause of clinical signs in patients with CVHD. For dogs with no of are likely to be in heart failure to dogs with and relatively heart also are likely to have clinical signs to CVHD. of these dogs are or and is to the with a serum and especially if therapy for is Consensus recommendations: specific of in a dog with should be related to the severity of clinical signs and the response to initial or higher (eg, may be appropriate in specific IV or a rate IV may be indicated for poorly dogs. For of associated with severe on thoracic initial response to with failure of and rate to over 2 is as a rate at a dose of 1 the initial patient to has the clinical trial evidence the chronic use of in the management of Stage C heart failure from CVHD is than for the acute the recommendation to use in acute heart failure therapy is by hemodynamic and experimental as well as the anecdotal of the if can be a and or or a treatments (eg, and are recommended to to or cause including of an appropriate and in the on and of patients in associated with should be or a with an are most used by or IV was the most for this of and or as well as other including and also have been of for up to is for poorly to Class D below for specific No consensus was reached on the acute care Stage C be to the pressure and response to and in the of acute heart failure. No specific treatment or dosage was used by all ACEI (eg, treatment with ACEI is a consensus recommendation for chronic Stage C heart failure and a majority of panelists also acute heart failure with the evidence ACEI efficacy and in acute therapy when with and is evidence that the acute of in acute heart failure results in in pressure when with the of approximately for Some panelists recommend the in (eg, 12 12 panelists do not use in this setting. Consensus recommendations: to commonly at a dosage of 2 The daily dosage for dogs with is and can be as low as 1–2 to The dosage be to patient and with to on and to patient in the of appropriate therapy indicates disease progression to Stage or ACEI (eg, or dose of ACEI if for The dosage of is most panelists at the of this of serum creatinine and an ACEI is recommended for animals with Stage C heart failure. recommend a β in the of clinical signs of heart failure (eg, caused by CVHD. of the panelists use in the chronic treatment of Stage C heart failure. in a care to and heart rate monitoring support to medication and dosage in patients with heart failure is No consensus was reached regarding the treatment strategies in Stage was recommended by a majority of panelists as an for the chronic therapy of dogs in Stage C heart failure. The primary of in this is to be No clinically relevant effect should be This treatment is in at a dosage of 2 with of For the chronic management of Stage C heart failure, a majority of panelists recommended the of in cases by atrial to the ventricular response Some panelists also at this dosage for patients in Stage C heart failure in the of as as no to is evident (eg, increased serum creatinine concentration, ventricular over chronic disease in frequent or of or heart failure signs have a medication has been and the patient is and a minority of panelists recommend a low of a β is no clinical trial evidence in dogs to support this there is no consensus regarding which specific β to use or is the most frequently The of β in this is related to potential on myocardial and These have been in some experimental and in with heart failure, but not in clinical trials. The presence of atrial the for β the ventricular response to atrial for those panelists recommended a β In patients a β before the onset of Stage C heart failure, the majority of panelists β some panelists would dosage if clinically because of clinical signs of low cardiac or Some panelists of are some for chronic heart rate in atrial Some panelists in patients in Stage C heart failure from CVHD. Some panelists in patients in Stage C heart failure from CVHD. Cardiac is as the of of the patient's not including associated with the of or the of has and is to that to Consensus recommendations: in Stage C should provide approximately to that in and about the of and to any or other identifiable causes of that the of the patient at every and the cause of or protein and designed to chronic disease, unless severe failure is into from all dietary (including dog and used to and any or other serum and the with from either or if is is relatively in patients treated for heart failure with even in those an ACEI in combination with and with high content should be when has been No consensus was reached on the dietary therapy for Stage monitoring serum especially as and in animals with with in cases in which is with especially in dogs with or Stage have clinical signs of failure refractory to treatment for Stage C heart failure from CVHD, as Stage D heart failure patients should be the recommended dosage of an and as in the Stage C guidelines indicated and tolerated to or the ventricular response to atrial in a heart rate of also should be used before a patient is considered refractory to there have been clinical trials addressing drug efficacy and in this patient This cardiologists patients with heart failure refractory to conventional therapy with a variety of treatment Because of the of clinical trial evidence and the clinical of patients with end-stage heart failure, development of consensus guidelines regarding the and of pharmacologic and dietary treatment strategies for Stage D patients As with Stage C, guidelines for drug treatment are for both and for care management of heart failure, and for chronic dietary therapy are also Because Stage D heart failure patients by refractory to the treatments for Stage C refractory congestive heart failure the diagnostic for Stage C the of failure to to treatments in the Stage C Consensus recommendations: In the of severe insufficiency serum creatinine additional is IV as a at a dosage of 2 by either additional or a at a dosage of 1 and has or for a of 4 As indicated the dosage or is a and higher or may be appropriate for a to patient to has (eg, as to or In to as in Stage C ventilatory may be to the patient more to time for to have an and to provide time for left atrial to increases in mitral valve in patients with acute of CVHD (eg, chordae with severe and failure. afterload in patients that can arterial potentially include at or should be at a low dosage and clinical by a decrease of approximately in systolic pressure is These are recommended in to an ACEI and The should be that any in pressure will also on specific For are rapid onset with but with is to pressure and systolic arterial pressure or arterial pressure creatinine should be before and of these in Stage D have heart failure, and a trial of additional afterload is The panel that because afterload may cardiac substantially in the of severe MR and heart failure, of an arterial in this does not necessarily decrease No consensus was reached regarding the acute care Stage D recommendations: dosage may be increased to include a daily Some panelists an additional dose of on of Stage D patients with acute Because this dosage recommendation is of the for this use of the drug should be to and by the client. In animals to be to for the of afterload or support (eg, with or without or afterload in or support of the be by can be at of and every to a of approximately These either separately or in can be used for to hemodynamic status and refractory and pressure monitoring is recommended to the potential risks of this is used by a minority of panelists to acute of Stage D heart failure caused by CVHD, even in the of are recommended as an therapy in in patients by a minority of Consensus recommendations: dosage should be increased as to decrease or if use is not by dysfunction generally should be dosage The specific and of dosage (eg, dose increased to times versus 2 higher 1 dose for a dose or dose based on or the if not in Stage C, is indicated for chronic treatment of Stage D patients. β generally should not be at this stage unless clinical signs of heart failure can be as in Stage No consensus was reached regarding the chronic Stage D therapeutic recommendations: was recommended by panelists as therapy with (including only use every Some panelists of the risk of acute failure and based on dosage is increased by some panelists to include a daily dose and as for at the recommended by some panelists for Stage C heart failure, was recommended for treatment of atrial for patients in Stage with the in Stage C at the recommended by some panelists for Stage C heart failure, also was recommended by a minority of panelists for all patients in Stage D in no was is used by some panelists to Stage D heart failure caused by CVHD or to advanced CVHD by The majority of panelists that β at an stage of heart failure in CVHD should not be but that dose may be if of could not be by the of other or if or both were β may be to decrease the ventricular response rate in atrial and are recommended by a minority of panelists to in Stage D patients care. are recommended by a minority of panelists to in Stage D patients Consensus recommendations: of the dietary for Stage C In patients with refractory should be made to further decrease dietary if can be without or

Introduction: Patient reported outcome (PRO) measures correlate with heart failure (HF) severity among adults and are widely used as adjunct tools in the assessment of adult HF. However, limited data exist in pediatric HF. PROs may be valuable in monitoring heart failure (HF) status in children. Hypothesis: Patient Reported Outcome Measurement Information System (PROMIS) fatigue score correlates with HF severity in children. Methods: In this prospective cohort study, English and Spanish speaking patients were recruited from 4 pediatric HF centers 09/2019 - 02/2023. Children 8.0 to 18.0 years old with systolic and/or diastolic HF were enrolled while receiving inpatient HF treatment. Pediatric self-report PROMIS measures were administered during inpatient treatment and at additional outpatient follow-up time point(s) within 6 months post-discharge. Measures included mobility, fatigue, anxiety, depressive symptoms, and peer relationships. NYU HF Index scores were calculated at each time point. Pearson’s correlation with a 95% confidence interval (CI) compared PROMIS Fatigue T-score and NYU HF Index score at the inpatient time point, and a linear mixed effects model determined their association over time. Results: We enrolled 41 patients with median age of 13.5 ±4.2 years. Patient diagnoses included cardiomyopathy (48.8%), congenital heart disease (41.5%) and other (9.7%). Twenty patients (48.8%) were discharged from hospital alive and without a VAD or heart transplant, of which 18 completed both inpatient and outpatient follow-up assessment. Median PROMIS Fatigue T-scores and NYU HF Index scores improved from inpatient to outpatient assessment. NYU Index scores improved from 12.0 (IQR 11.2, 15.0) to 7.5 (IQR 5.8,10.2); p&lt;0.05. PROMIS fatigue scores improved from 58.5 (IQR 54.6, 64.9) to 50.2 (IQR 33.5, 59.2); p&lt;0.05. The correlation between PROMIS Fatigue T-score and NYU HF Index score at the inpatient time point was 0.5 (95% CI 0.2, 0.7), and their correlation over time was 0.5 (95% CI 0.3, 0.6). Conclusions: PROMIS Fatigue T-score moderately correlates with HF severity in children. Larger scale application of PROMIS could aid in longitudinal symptom management of pediatric HF patients and should be further tested in routine clinical care.

Growth differentiation factor 15 as a useful biomarker of heart failure in young patients with unrepaired congenital heart disease of left to right shunt

Task Force 7: Pediatric Cardiology Fellowship Training in Pulmonary Hypertension, Advanced Heart Failure, and Transplantation. SPCTPD/ACC/AAP/AHA.

Heart failure is a deadly disease that has reached epidemic proportions in industrialized countries. Patients living with heart failure carry a heavy burden in terms of morbidity. Many patients require repeated hospitalizations for cardiovascular problems, especially for episodes of worsening heart failure. In fact, heart failure is one of the most important causes of hospital admissions in the United States, accounting for over 2.5 million admissions per year. Once hospitalized, patients with heart failure have an increased risk of recurrent hospitalizations and death. Approximately 30% to 40% of patients are readmitted within 6 months of an index hospitalization. Angiotensin-converting enzyme (ACE) inhibitors, digitalis, and spironolactone decrease the risk of hospitalization in heart failure patients; however, the annual rate of hospital admission for worsening heart failure has remained high.1–3⇓⇓ See p 2194 Given these challenges, clinical trials conducted in the mid 1990s that demonstrated that β-blocker therapy in addition to ACE inhibitors and digitalis reduces the risk of hospitalization in heart failure patients by about 20% to 30% represented remarkable progress. These beneficial effects of β-blocking agents on morbidity were recognized well before favorable effects on survival were unequivocally established (Table). In some, but not all, trials, the clinical benefits of β-blocker treatment included improved heart failure symptoms as assessed by physicians and patients. View this table: Large-Scale Clinical Trials Reporting β-Blocker Effects on Heart Failure Morbidity Previous trials addressing the effects of β-blockers on morbidity have been conducted in patients …

The value of plasma brain natriuretic peptide (BNP) and N-terminal proBNP (NT-proBNP) can reflect cardiac function and therefore can be used for diagnosing congestive heart failure (CHF) and evaluating cardiac function. There are few reports, however, on the value of BNP and NT-proBNP in pediatric cases of congenital heart defect. The aim of this study was to assess the value of plasma NT-proBNP in the diagnosis of CHF and evaluation of cardiac function in pediatric patients with ventricular septal defect (VSD). Fifty-one patients with VSD aged from 2 months to 2 years old (mean 7.9 months) were enrolled. According to the modified Ross Score, the patients were divided into three groups, no CHF group (20 patients), mild CHF group (18 patients) and moderate to severe CHF group (13 patients). Fifteen age-matched normal children were used as controls. Plasma NT-proBNP was measured using enzyme immunoassay. All patients had complete echocardiographic study, including measurement of left ventricular end diastolic volume index (LVEDVI), left ventricular end systolic wall stress (LVSEWS), heart rate corrected mean velocity of circumferential fiber shortening (mVcFc), left ventricular ejection fraction (LVEF), left ventricular fractional shortening (LVFS), and contractility index (Con). The correlation between plasma NT-proBNP level and modified Ross Score and echocardiographic cardiac functional indexes was determined. The sensitivity, specificity and ROC curve of plasma NT-proBNP for diagnosing CHF was studied. Plasma NT-proBNP was positively correlated with modified Ross Score (r = 0.75, P < 0.01). Plasma NT-proBNP concentration in moderate to severe CHF group (2061 +/- 908) fmol/ml was significantly higher than that of mild CHF group (810 +/- 335) fmol/ml, and Plasma NT-proBNP concentration in mild CHF group was higher than that in no CHF group (309 +/- 68) fmol/ml. 97.14% of normal controls and subjects in no CHF group had their plasma NT-proBNP below 400 fmol/ml. 83.3% of children in mild CHF group had their plasma NT-proBNP between (400-1400) fmol/ml while in moderate and severe CHF group 84.6% of children had their plasma NT-proBNP beyond 1400 fmol/ml. Plasma NT-proBNP was also positively correlated with LVEDVI and LVSEWS. There was no correlation among mVcFc, LVEF, LVFS, Con and plasma NT-proBNP concentration. Using plasma NT-proBNP concentration > or = 400 fmol/ml as cut-point for diagnosing CHF, the sensitivity was 89.3%, the specificity was 91.2%, and the area under the ROC curve was 0.944. Plasma NT-proBNP level could be used to assess cardiac function and diagnose CHF in pediatric patients with VSD.

B lines in lung ultrasound are useful to diagnose heart failure in adults. This study was to done to correlate lung ultrasound score with heart failure severity in CHD. Fifty infants with left to right shunt underwent Ross scoring, measurement of NT pro BNP and lung ultrasound. Lung was divided into 14 zones (7 on each side); each zone was scored as: No B lines = 0, less than 3 B lines = 1, 3-7 B lines = 2, > 7 /or confluent B lines = 3. Total score was calculated by adding all scores. A modified ultrasound score was calculated by excluding scores of 1. Relationship between ultrasound scores and heart failure was assessed. Receiver operator curve (ROC) was generated to find a cut-off of ultrasound score to detect moderate or severe heart failure. No correlation was found between either total or modified lung ultrasound score with the modified Ross score or NT pro BNP. A cut-off of total lung ultrasound score of 11 gave an accuracy of 68% with area under the curve (AUC) of 0.64 to detect moderate or severe heart failure. Utility of lung ultrasound in detecting severity of heart failure in infants with left to right shunts is limited at present due to its low accuracy and lack of standardised method of scoring.

Background Over the past two decades, heart failure in children has increased in terms of symptom recognition and prevalence. The initial clinical manifestations of heart failure in children are non-specific. Therefore, diagnosis requires the support of echocardiography. The symptomatic severity of heart failure in children can be classified through a simple scoring system such as Ross score. The duration of heart disease, duration of therapy, and cardiac remodeling status may have clinical and anatomical effects on the disease. Objective To analyze for a possible correlation between modified Ross score and echocardiographic score by subgroup analysis consisting of duration of heart disease, duration of therapy, and cardiac remodeling. Methods This cross-sectional study included children aged 1 month - 18 years with heart failure who sought treatment at Prof.Dr. I.G.N.G Ngoerah Hospital, Denpasar from June 2019 to February 2020. Cardiac remodeling was defined as &gt;20% increase in left ventricle internal end diastolic dimension (LVIDd) compared to normal values, ??based on body surface area. Spearman’s correlation test was used for statistical analysis. Results A total of 30 subjects were analyzed in this study. The median modified Ross score and echocardiography score were 3 points (range 2-11) and 4 (range 2-6), respectively. The median durations of heart disease and preventive heart failure therapy were 2 years (range 7 days-15 years) and 1 year (range 7 days-15 years), respectively. The mean LVIDd was 4.3 (SD 1.4) cm. Twenty-one out of 30 subjects experienced a ? 20% increase of LVIDd from baseline. The modified Ross score and echocardiographic score had no significant correlation (r=0.18; P=0.33). However, the modified Ross score had significant correlations with duration of heart disease (r=-0.632; P&lt;0.001) as well as duration of therapy (r=-0.584; P=0.001). In addition, no correlation was found between echocardiographic score with heart disease and therapy duration (P&gt;0.05). Echocardiography score and remodelling process was significantly correlated (r=0.64; P&lt;0.001). Conclusion There is no correlation between modified Ross score and echocardiographic score. Duration of heart disease and duration of therapy are significantly negatively correlated with modified Ross scores. The remodelling process is positively correlated with echocardiographic score. Further research on acute symptomatic and validated echocardiographic scores are needed.

Heart failure, N-terminal pro-BNP

Cardiopulmonary bypass (CPB) reduces coagulation factor levels through hemodilution and consumption. Differences in CPB-induced alterations of factor XIII (FXIII) levels in children with cyanotic and acyanotic congenital heart defects (CHDs) are not well characterized. FXIII activity (determined by Berichrom assay), prothrombin index, activated partial thromboplastin time, and fibrinogen were measured before open heart surgery with CPB and 5 days postoperatively for children older than 3 years with acyanotic (n = 30) and cyanotic (n = 30) CHDs. The preoperative FXIII levels did not differ significantly among the children of the compared groups. The cyanotic patients showed a significantly longer duration of CPB (111.4 ± 45.8 vs 71.5 ± 33.6 min; p = 0.026) and aortic cross-clamp (68.0 ± 27.1 vs 45.4 ± 31.4 min; p = 0.034). The drop in FXIII levels after termination of CPB was more profound for the children with cyanotic CHDs (87.1 ± 13.4 to 49.1 ± 13.2 vs 81.5 ± 12.6 to 58.6 ± 11.1 %, respectively; p = 0.018). The cyanotc patients also were restored to their baseline FXIII levels later than the children with acyanotic CHDs (at 48 vs 24 h). The post-CPB dynamics of the majority of the other coagulation parameters in the compared groups of patients were similar. The cyanotic patients experienced significantly greater postoperative blood loss than the acyanotic patients (12.6 ± 4.9 vs 5.0 ± 2.1 mL/kg; p < 0.001) and were transfused with larger volumes of red blood cells (10.4 ± 6.5 vs 4.2 ± 2.5 mL/kg; p = 0.007). The decrease in FXIII levels after CPB is more profound and lasts longer in children with cyanotic CHDs than in acyanotic patients. The rational strategy of postoperative FXIII replacement therapy for these categories of patients needs to be determined.