Diastolic heart failure (DHF) currently accounts for more than 50% of all heart failure patients. DHF is also referred to as heart failure with normal left ventricular (LV) ejection fraction (HFNEF) to indicate that HFNEF could be a precursor of heart failure with reduced LVEF. Because of improved cardiac imaging and because of widespread clinical use of plasma levels of natriuretic peptides, diagnostic criteria for HFNEF needed to be updated. The diagnosis of HFNEF requires the following conditions to be satisfied: (i) signs or symptoms of heart failure; (ii) normal or mildly abnormal systolic LV function; (iii) evidence of diastolic LV dysfunction. Normal or mildly abnormal systolic LV function implies both an LVEF > 50% and an LV end-diastolic volume index (LVEDVI) 16 mmHg or mean pulmonary capillary wedge pressure >12 mmHg) or non-invasively by tissue Doppler (TD) (E/E' > 15). If TD yields an E/E' ratio suggestive of diastolic LV dysfunction (15 > E/E' > 8), additional non-invasive investigations are required for diagnostic evidence of diastolic LV dysfunction. These can consist of blood flow Doppler of mitral valve or pulmonary veins, echo measures of LV mass index or left atrial volume index, electrocardiographic evidence of atrial fibrillation, or plasma levels of natriuretic peptides. If plasma levels of natriuretic peptides are elevated, diagnostic evidence of diastolic LV dysfunction also requires additional non-invasive investigations such as TD, blood flow Doppler of mitral valve or pulmonary veins, echo measures of LV mass index or left atrial volume index, or electrocardiographic evidence of atrial fibrillation. A similar strategy with focus on a high negative predictive value of successive investigations is proposed for the exclusion of HFNEF in patients with breathlessness and no signs of congestion. The updated strategies for the diagnosis and exclusion of HFNEF are useful not only for individual patient management but also for patient recruitment in future clinical trials exploring therapies for HFNEF.

http://www.carsten-tschoepe.de/usr_files/6_Paper%2010.pdf

How to diagnose diastolic heart failure: a consensus statement on the diagnosis of heart failure with normal left ventricular ejection fraction by the Heart Failure and Echocardiography Associations of the European Society of Cardiology

Background: Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acqui...

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association.

Guidelines summarize and evaluate all currently available evidence on a particular issue with the aim of assisting physicians in selecting the best management strategies for an individual patient, suffering from a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular diagnostic or therapeutic means. Guidelines are no substitutes for textbooks, and their legal implications have been discussed previously. Guidelines and recommendations should help physicians to make decisions in their daily practice. However, the ultimate judgement regarding the care of an individual patient must be made by his/her responsible physician(s).

A large number of Guidelines have been issued in recent years by the European Society of Cardiology (ESC) as well as by other societies and organizations. Because of the impact on clinical practice, quality criteria for the development of guidelines have been established in order to make all decisions transparent to the user. The recommendations for formulating and issuing ESC Guidelines can be found on the ESC Web Site (http://www.escardio.org/guidelines/rules).

Members of this Task Force were selected by the ESC to represent all physicians involved with the medical care of patients in this pathology. In brief, experts in the field are selected and undertake a comprehensive review of the published evidence for management and/or prevention of a given condition. A critical evaluation of diagnostic and therapeutic procedures is performed, including assessment of the risk–benefit ratio. Estimates of expected health outcomes for larger populations are included, where data exist. The level of evidence and the strength of recommendation of particular treatment options are weighed and graded according to pre-defined scales, as outlined in Tables 1 and 2 .

View this table:

Table 1 
Classes of recommendations



View this table:

Table 2 
Levels of evidence



The experts of the writing and reviewing panels have provided disclosure statements of all relationships they may have which …

/pdf/esc-guidelines-for-the-management-of-grown-up-congenital-542gk4n515.pdf

ESC Guidelines for the management of grown-up congenital heart disease (new version 2010).

Background—Kawasaki disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in 15% to 25% of untreated children and may lead to ischemic heart disease or sudden death. Methods and Results—A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for 5 days and 4 classic criteria should undergo echocardiography, receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor- antagonists, and abciximab. Long-term management of patients with Kawasaki disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac disease are classified according to risk strata. Conclusions—Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients. (Circulation. 2004;110:2747-2771.)

/pdf/diagnosis-treatment-and-long-term-management-of-kawasaki-3zokgy3fx8.pdf

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease A Statement for Health Professionals From the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association

Background. Kawasaki disease is an acute self-limited vasculitis of childhood that is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Coronary artery aneurysms or ectasia develop in ∼15% to 25% of untreated children and may lead to ischemic heart disease or sudden death.
 Methods and Results. A multidisciplinary committee of experts was convened to revise the American Heart Association recommendations for diagnosis, treatment, and long-term management of Kawasaki disease. The writing group proposes a new algorithm to aid clinicians in deciding which children with fever for ≥5 days and ≤4 classic criteria should undergo electrocardiography, receive intravenous gamma globulin (IVIG) treatment, or both for Kawasaki disease. The writing group reviews the available data regarding the initial treatment for children with acute Kawasaki disease, as well for those who have persistent or recrudescent fever despite initial therapy with IVIG, including IVIG retreatment and treatment with corticosteroids, tumor necrosis factor-α antagonists, and abciximab. Long-term management of patients with Kawasaki disease is tailored to the degree of coronary involvement; recommendations regarding antiplatelet and anticoagulant therapy, physical activity, follow-up assessment, and the appropriate diagnostic procedures to evaluate cardiac disease are classified according to risk strata.
 Conclusions. Recommendations for the initial evaluation, treatment in the acute phase, and long-term management of patients with Kawasaki disease are intended to assist physicians in understanding the range of acceptable approaches for caring for patients with Kawasaki disease. The ultimate decisions for case management must be made by physicians in light of the particular conditions presented by individual patients.

Diagnosis, treatment, and long-term management of Kawasaki disease: A statement for health professionals from the committee on rheumatic fever, endocarditis, and Kawasaki disease, council on cardiovascular disease in the young, American Heart Association

Objective. Varying observations have been made concerning the use of aspirin (ASA) and/or intravenous immunoglobulin (IVIG) in the prevention of coronary artery aneurysm (CAA) in children with Kawasaki disease. A meta-analysis of published articles on the subject was conducted to evaluate the reported efficacy of these therapies. Methods. All published studies in all languages from 1967 through 1993 obtained from MEDLINE and EMBASE were considered, and a defined set of inclusion and exclusion criteria selected the studies for analysis. These studies were grouped based on whether the children in the studies received: (1) ASA alone, (2) low IVIG (≤1 g/kg) and ASA, (3) high IVIG (>1 g/kg) and ASA, (4) single IVIG (>1 g/kg) and ASA, (5) high IVIG and low ASA (≤80 mg/kg), or (6) high IVIG and high ASA (> 80mg/kg). Studies that satisfied the test for homogeneity were subjected to further analysis. The best estimate of the true proportion of CAA as well as the 95% confidence interval for each group were calculated at 30 and 60 days. Hypothesis testing was conducted to determine the statistical significance of the calculated difference in each compared treatment group. Results. The best estimate of true proportion of CAA and the 95% confidence interval in each group at 30 and 60 days were: (1) ASA group, 30 days, 22.8% (20.6%, 25%); 60 days, 17.1% (13.6%, 20.7%); (2) low-IVIG group, 30 days, 17.3% (14.3%, 20.2%); 60 days, 11.1% (8.7%, 13.6%); (3) high-IVIG group, 30 days, 10.3% (8.3%, 12.3%); 60 days, 4.4% (2.8%, 6%); (4) single-IVIG group, 30 days, 2.3% (0.5%, 4.2%); 60 days, 2.4% (0.5%, 4.2%); (5) high-IVIG-low-ASA group, 30 days, 13% (9%, 17%); 60 days, 4.8% (2.3%, 7.4%); and (6) high-IVIG-high-ASA group, 30 days, 9.1% (6.9%, 11.4%); 60 days, 4% (2%, 6.1%). Conclusion. The incidence of CAA both at 30 and 60 days was significantly lower in low-IVIG than in ASA and in high-IVIG than in low-IVIG groups. Also, the incidence was lower in the single-IVIG than in the high-IVIG group, but this was noted at 30 days and not at 60 days. There was no statistically significant difference in the incidence of CAA both at 30 and 60 days between the high-IWIG-low-ASA and high-IVIG-high-ASA groups.

The prevention of coronary artery aneurysm in Kawasaki disease: a meta-analysis on the efficacy of aspirin and immunoglobulin treatment.

Several cases of Kawasaki disease (KD) were unresponsive to the initial treatment with intravenous immunoglobulin (IVIG). We retrospectively analyzed all children admitted with KD to determine the occurrence and variables associated with the initial IVIG treatment failure. All patients who fulfilled the criteria for KD and were treated with a single dose (2 g/kg) of IVIG between January 1995 and August 2001 were enrolled. An analysis of the patients who had initially failed to respond to IVIG was performed. A total of 120 patients were enrolled during the study period. There were 68 boys (56.7%). Fourteen patients (11.6%) were found to be unresponsive to initial IVIG treatment. Patients who were anemic (Hb 75%), a high band count, and low albumin were at risk of failure to respond to a single dose of IVIG. We found no correlation among age, gender, days since starting IVIG treatment, and erythrocyte sedimentation rate (ESR) with failure of the initial IVIG treatment. There were 12 patients (10%) who developed coronary artery aneurysms. The failure of a single dose of IVIG treatment occured in up to 11.6% of our Kawasaki patients. We found that low hemoglobin ( 75%), high band count, and a low albumin were associated with the requirement for retreatment with a second dose of IVIG.

Immunoglobulin Failure and Retreatment in Kawasaki Disease

Background—The objectives of our study were to determine the frequency of supraventricular tachyarrhythmias (SVTAs) among modifications of the Fontan operation and identify risk factors for developing SVTA. Methods and Results—The population consisted of all patients who had any modification of the Fontan operation at the Mayo Clinic between 1985 and 1993. Clinically significant SVTAs were those requiring initiation or change of antiarrhythmic treatment, and they were divided into early SVTAs (<30 days after the operation) and late SVTAs (≥30 days after the operation). Clinical histories were reviewed, and health status questionnaires were sent. Four hundred ninety-nine patients had various modifications of the Fontan operation. Frequency of early SVTA was 15%. Risk factors identified by multivariate analysis for early SVTA were AV valve regurgitation, abnormal AV valve, and preoperative SVTA. Frequency of late SVTA was 6% by 1 year, 12% by 3 years, and 17% by 5 years. Risk factors for late SVTA were age ...

Predictors of Early- and Late-Onset Supraventricular Tachyarrhythmias After Fontan Operation

Objective To extend noninvasive assessment. of diastolic cardiac function into the pediatric age-group. Design This study was divided into two phases, the first of which was designed to provide an age-appropriate set of normal diastolic Doppler echocardiographic data for children and adolescents and the second of which was to determine whether these Doppler techniques could be used to identify children with increased ventricular end-diastolic pressure (EDP). Material and Methods Complete echocardiographic studies focusing on Doppler variables of diastolic ventricular function were performed on 223 normal children. Values observed were analyzed for dependence on age, heart rate, and gender. Results from the normal group were then compared with Doppler values observed in a group of 24 children with catheterization-substantiated increases in ventricular EDP. Results Normal values for the Doppler factors studied vary with both age and heart rate. The variables that most confidently distinguished children with increased EDP from normal subjects were the ratio of and the difference between the durations of pulmonary vein atrial reversal and the mitral A wave. A ratio of 1.2 or more or a difference of 29 ms or more identified those children with increased EDP with sensitivities of 88 and 90% and specificities of 86 and 86%, respectively. Conclusion Use of the normal data and the Doppler techniques described in this study will allow confident assessment of diastolic function in children as well as in adults.

Diastolic ventricular function in children: a Doppler echocardiographic study establishing normal values and predictors of increased ventricular end-diastolic pressure

Kritvikrom Durongpisitkul

Papers

The prevention of coronary artery aneurysm in Kawasaki disease: a meta-analysis on the efficacy of aspirin and immunoglobulin treatment.

Immunoglobulin Failure and Retreatment in Kawasaki Disease

Predictors of Early- and Late-Onset Supraventricular Tachyarrhythmias After Fontan Operation

Diastolic ventricular function in children: a Doppler echocardiographic study establishing normal values and predictors of increased ventricular end-diastolic pressure

Cardiorespiratory Response to Exercise After Modified Fontan Operation: Determinants of Performance