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Background: Pulmonary hypertension (PH) is increasingly recognized in premature infants with bronchopulmonary dysplasia. Management of PH is more difficult in the presence of pulmonary vein stenosis (PVS) and significant congenital heart disease (CHD). Objectives: To describe the characteristics of premature infants with BPD and PH who underwent cardiac catheterization and evaluate the effect of PVS and CHD on mortality at follow-up. Methods: This was a single-centered retrospective study to describe premature infants born at 1.5) and structural …

Fenestration closure is considered to remove the persistent right-to-left shunt after the Fontan operation. However, the criteria for effective transcatheter closure of fenestration to avoid both acute and chronic Fontan failure have not been clarified. The objective of this study was to describe the hemodynamic data with test occlusion of the Fontan fenestration for patients who underwent a closure and those who did not, along with subsequent development of Fontan-associated diseases (FAD) at follow-up. This was a retrospective study conducted to assess the outcome of Fontan fenestration closure at Children’s Hospital of Michigan over 27 years (1993–2019). The inclusion criteria were patients undergoing cardiac catheterization indicated for Fontan fenestration closure. Data were compared between the two groups: closure and non-closure patients. Baseline characteristics and hemodynamic variables with the fenestration occlusion test were analyzed. The primary outcome was the development of composite events of death/transplant, deteriorated New York Heart Association class symptoms, or FAD. Among the 38 patients who were brought to the catheterization laboratory, 33 received fenestration closure and 5 did not. On a median follow-up of 3.4 years (range, 1 month–12.6 years), the incidence of primary adverse outcomes was 13% (5/38). The incidence of primary outcome was significantly higher in the non-closure group (60% vs. 6%, p

Background: Blood pressure (BP) response to the treadmill cardiopulmonary exercise test (CPET) has yet been studied with no standard criteria for abnormal BP responses in children. Our hypothesis was that the reference values of BP responses to the treadmill CPET should be defined by gender and age in children. Objectives: The objective of our study was to describe the BP responses to the treadmill CPET and propose the criteria for abnormal BP responses in children. Methods: The treadmill CPET data were retrospectively analyzed to evaluate the BP responses to exercise during the period of 8 years (2011-2018). Children aged Results: Our cohort consisted of 1,085 children (males 59%) aged 7-17 years. The analysis of variance for Systolic BP at peak exercise (peak SBP) and change (Δ) of SBP showed significant age and gender effects and age-gender interaction effect ( p p 90% confidence interval upper limit of reference values based on age and gender. Blunted BP response was defined by 1) ΔSBP Conclusion: Blood Pressure response to the treadmill CPET was dependent on age and gender in children with normal hearts. The proposed criteria for abnormal BP responses in children should prove useful in interpreting BP responses in children during the CPET.

Objectives The aim of this study was to evaluate the diagnostic utility of cardiac catheterization and the efficacy of transcatheter intervention in patients with hemoptysis. Background Cardiac catheterization may play a role in identifying the etiologies of hemoptysis with the potential for transcatheter intervention. Methods This was a retrospective study of all the patients who were brought to the pediatric cardiac catheterization laboratory for the indication of hemoptysis over a 15-year period (2006–2020). Results Twenty-one patients underwent 28 cardiac catheterizations. The median age was 17.4 years (range 0.3–60.0 years), and the underlying cardiac diagnoses were normal heart n = 3, pulmonary hypertension 1, heart transplant 1, pulmonary arteriovenous malformation 1, pulmonary vein disease 3, biventricular congenital heart diseases 5, and single ventricles 7. The diagnostic utility of catheterization was 81% (17/21). At two-thirds (18/28) of catheterizations, transcatheter interventions were performed in 14/21 (67%) patients: aortopulmonary collateral embolization 14, aortopulmonary and veno-venous collateral embolization 1, and pulmonary arteriovenous malformation embolization 3. Although recurrent hemoptysis was frequent (50%) post-intervention, the final effectiveness of transcatheter interventions was 79% (11/14 patients). Overall mortality was 19% (4/21), all in those presenting with massive hemoptysis. Conclusions Cardiac catheterization was shown to have good diagnostic utility for hemoptysis especially in patients with underlying congenital heart disease. Despite the high mortality and recurrent hemoptysis rate, transcatheter interventions were effective in our cohort.

INTRODUCTION Reduction of radiation dosage in the pediatric cardiac catheterization laboratory (PCL) is important to reduce the risk of its stochastic effect in children with congenital heart disease. Lowering the frame rate would reduce radiation dosage possibly at the expense of image quality, potentially resulting in higher fluoroscopic time and procedural complication rate. METHODS The data were retrospectively analyzed in three eras: era 1 (n = 234), cineangiography 30 frames/sec (f/s) and fluoroscopy 15 pulse/sec (p/s); era 2 (n = 381), cineangiography 30 f/s and fluoroscopy 6 p/s; and era 3 (n = 328), cineangiography 15 f/s and fluoroscopy 6 p/s. Also, three operators blinded to the frame rate setting evaluated cineangiography image quality. In this study, the impact of lowering the default frame rates on radiation dosage, fluoroscopic time, contrast volume, diagnostic image quality, and complication rates in the PCL was assessed. RESULTS Overall radiation dosage progressively declined during these eras (70.0 vs 64.1 vs 36.6 µGym2 /kg, P

Background Patients with cardiomyopathy (CM) are at increased risk for pulmonary hypertension (PH). Data are lacking on the use of noninvasive PH measures by echocardiography in patients with CM. The aim of this study was to evaluate the correlation between Doppler-derived echocardiographic indices and catheterization-based measurement of pulmonary vascular resistance (PVR) in children and young adults with CM. Methods Imaging studies were retrospectively reviewed from pediatric patients with CM who underwent both echocardiography and cardiac catheterization within a 72-hour period. The ratio of peak tricuspid regurgitation velocity to right ventricular outflow tract velocity-time integral, the S/D ratio, and right ventricular myocardial performance index were correlated with invasive PVR. Receiver operating characteristic curves were developed to determine cutoffs for detecting PVR ≥ 6 indexed Wood units, a value associated with higher heart transplantation risk. Results Twenty-three patients with CM (median age, 11.7 years; range, 0.5–21 years) met the criteria for analysis, the majority ( n = 17 [74%]) of whom had dilated CM. Linear regression showed significant correlations between echocardiography-based ratio of peak tricuspid regurgitation velocity to right ventricular outflow tract velocity-time integral, S/D ratio, and right ventricular myocardial performance index versus invasive PVR ( r = 0.84, r = 0.72, and r = 0.72, respectively, P Conclusions Right-sided Doppler-derived echocardiographic indices correlate with PVR measured by cardiac catheterization in children and young adults with CM. These parameters may serve as useful adjuncts in serial assessment of right ventricular hemodynamics in this population.

Pseudoaneurysm (PSA) is a known but rare complication of the right ventricle to pulmonary artery (RV-PA) conduits. The patient's clinical presentation can be variable ranging from asymptomatic to potential rupture. We describe an unusual case of a massive PSA in an infant who underwent RV-PA pulmonary homograft placement after relief of right ventricular outflow tract obstruction.

To describe blood pressure (BP) responses during cardiopulmonary exercise testing (CPET) and propose criteria for abnormal BP responses in children with normal hearts.Treadmill CPET data of children18 years with normal hearts were retrospectively analyzed to evaluate BP responses to exercise at the Children's Hospital of Michigan over an 8-year period (2011-2018).Our cohort included 1085 children (boys, 59%) aged 7-17 years. Analysis of variance for systolic BP at peak exercise (peak SBP) and SBP change (Δ) showed significant age and sex effects and age-sex interaction effects (P .01). In the multiple linear regression model (P .001, R square 0.298, standard error of the estimate 17.15), peak SBP (mm Hg) was predicted as 132.27 + 1.37 × age (years) + (3.31 × age (years) - 31.88) × sex [boys 1, girls 0]. The following criteria for abnormal BP responses in children were proposed: hypertensive BP response was defined by peak SBP with a 90% CI upper limit of reference values based on age and sex, and blunted BP response as ΔSBP10 mm Hg for aged 7-11 years,20 mm Hg for boys aged 12-17 years, and ΔSBP10 mm Hg in girls.BP responses to treadmill CPET depend on age and sex in children with normal hearts. The proposed criteria for abnormal BP responses in children during CPET will need to be verified in a large pediatric cohort.

We report a critically ill premature infant with severe mitral valve regurgitation associated with pulmonary hypertension and a severely dilated left atrium from a large patent ductus arteriosus. The mitral valve regurgitation improved significantly with normalisation of left atrial size 4 weeks after percutaneous closure of the patent ductus arteriosus. This case highlights the potential reversibility of severe mitral valve regurgitation with treatment of an underlying cardiac shunt.

Infantile hemangioma is a benign vascular neoplasm that spontaneously involutes over time. Management, when needed, consists of medications, laser treatment and surgical excision. We describe a 3-year-old girl who presented shortly after birth with diffuse cutaneous hemangiomas, hepatosplenomegaly with liver lesions, anemia, and acute heart failure. She was diagnosed with hepatic and cutaneous infantile hemangioma based on skin biopsy. She developed progressive pulmonary hypertension with numerous pulmonary nodules suspicious for pulmonary arteriovenous malformations. She was started on sirolimus and had significant improvement in her pulmonary hypertension and liver lesions. This report supports prior studies that sirolimus is effective for vascular anomalies including IH refractory to conventional therapy.

AIM To evaluate predictors of morbidity and mortality in pediatric patients with pulmonary hypertension (PH), laboratory and echocardiographic measures of PH were analyzed. METHODS A retrospective review of all infants and children

The three most common reasons for referral to the cardiologist’s office for new patients are chest pain, syncope, and a heart murmur. These same three complaints or findings are frequent reasons for children to be brought to an emergency room or urgent care facility. In this chapter, typical cases for each are presented followed by a guide to the workup and management of each. As most of these will be non-cardiac in origin, it is important to distinguish the common non-cardiac causes from the uncommon true cardiac etiologies and to know when to intervene or contact cardiology urgently for the latter. Algorithms for the evaluation and management for each are provided.

Pulmonary hypertension (PH) is an uncommon but potentially life-threatening condition in children, and improved survival makes it more likely that acute care practitioners will encounter children with PH. This is due in part to better outcomes of extremely preterm infants who develop PH from chronic lung disease and children with congenital heart and lung defects who survive surgery but have residual PH, increased diagnosis of PH due to expanded screening indications, and better diagnostic modalities. This chapter provides an overview of the types of pediatric PH likely to be encountered in the acute care setting and discusses the recognition and initial management of this disease. Specific clinical cases are presented to illustrate various pulmonary hypertensive emergencies in the five categories of PH as classified by the World Health Organization. These consist of (1) pulmonary arterial hypertension, (2) PH with left heart disease, (3) PH associated with lung diseases and/or hypoxemia, (4) PH due to chronic thrombotic and/or embolic disease, and (5) miscellaneous.

The ability to accurately describe and classify the signs and symptoms of heart failure is critical to the study of and communication about the condition. This is particularly challenging in children in whom these variables change dramatically from infancy through adolescence. The ideal classification system would take into account these age-related changes as well as be reproducible and accurately reflect improvement with or without therapy or clinical worsening. It would be most useful if the system is correlated with risks and outcomes so that treatment could be tailored to the class and risk category. This chapter reviews the various systems used and proposed for achieving these goals.

Heart failure remains the main cause of death in children with heart disease. In USA and Europe hospital mortality of children with heart failure is about 7% of children, nearly twice as high as in adults. In this review a group of authors report about their experience with beta-blockers in childhood heart failure. Most of them start to treat children with severe heart failure at a time - 20 years ago - when beta blockers seem to be contraindicated in this situation. The physicians and their patients and/or parents all are aware of the risk of this decision. However, unproven medical therapies for heart failure are the most important therapeutical dilemma in pediatric cardiology. The authors carefully observed a highly selected group of patients with the highest risk to die and had the patience to wait for the longtime follow up. Today - based upon this experience -we know that beta blockers are safe and may save the lives of many children with heart disease all over the world. Together with young colleagues who enthusiastically support this idea the authors now intend to break down the "wall of ignorance" for this promising therapy in pediatric cardiology.

N-terminal pro-brain type natriuretic peptide (NT-proBNP) has been reported to have an association with increased morbidity and mortality in adults with septic shock. The relationship between NT-proBNP levels and morbidity/mortality in children with systemic inflammatory response syndrome (SIRS) has not been studied. This was a retrospective study. The inclusion criteria were children (

Down syndrome (DS) patients are prone to pulmonary hypertension (PHTN) due to various cardiopulmonary causes. However, the association of DS with pulmonary vein stenosis (PVS) is not adequately described. We illustrate three cases from our center and an additional 13 cases from an extensive review of the literature of DS patients with PHTN and PVS. In DS patients PVS is rare, they were diagnosed at a young age (

Since the 2005 publication of the first “Training Guidelines for Pediatric Cardiology Fellowship Programs,”1 the field of pediatric cardiology has undergone significant growth and change, and thus, the Society of Pediatric Cardiology Training Program Directors (SPCTPD) in conjunction with the Joint Council of Congenital Heart Disease recommended the guidelines be revised accordingly. The SPCTPD board assembled a Steering Committee that nominated 2 chairs for each of the 8 Task Forces (7 as in the original document plus 1 for “advanced medical therapies,” [ie, heart failure, pulmonary hypertension, and cardiac transplantation]). Six to 8 members were selected from a list of potential committee members representing a wide range of program sizes, geographic regions, and subspecialty focuses. Representatives from the American College of Cardiology, American Academy of Pediatrics, and American Heart Association participated. These participants, along with 1 Steering Committee member, comprised each Task Force. A Steering Committee member was added to provide perspective to each Task Force as a “nonexpert” in that field. The authors developed the Task Force reports under guidance from the Task Force chairs, approved them for review by individuals selected by the participating organizations, and addressed the 258 comments submitted. The peer reviewers for each report are listed in an appendix in each Task Force report along with their employment information and affiliation in the review process. The final, complete document was approved by the Society of Pediatric Cardiology Training Program Directors, American Academy of Pediatrics, and the American Heart Association in February 2015 and by the American College of Cardiology in March 2015, and individual Task Force reports were endorsed by the organizations noted in each report. During the process of updating the guidelines, a paradigm shift in medical education occurred. The change to competency-based training now requires trainees to achieve an expected level of …

1.1 Document Development Process The Society of Pediatric Cardiology Training Program Directors (SPCTPD) board assembled a Steering Committee that nominated 2 chairs, 1 SPCTPD Steering Committee member, and 4 additional experts from a wide range of program sizes, geographic regions, and

1.1. Document Development Process The Society of Pediatric Cardiology Training Program Directors (SPCTPD) board assembled a Steering Committee that nominated 2 chairs, 1 SPCTPD Steering Committee member, and 6 additional experts from a wide range of program sizes, geographic regions, and subspecialty focuses. Representatives from the American College of Cardiology (ACC), American Academy of Pediatrics (AAP), and American Heart Association (AHA) participated. The Steering Committee member was added to provide perspective to each Task Force as a “nonexpert” in that field. Relationships with industry and other entities were not deemed relevant to the creation of a general cardiology training statement; however, employment and affiliation information for authors and peer reviewers are provided in Appendixes 1 and 2, respectively, along with disclosure reporting categories. Comprehensive disclosure information for all authors, including relationships with industry and other entities, is available as an online supplement to this document. The writing committee developed the document, approved it for review by individuals selected by the participating organizations (Appendix 2), and addressed their comments. The final document was approved by the SPCTPD, AAP, and AHA in February 2015 and approved by the ACC in March 2015. This document is considered current until the SPCTPD revises or withdraws it. ### 1.2. Background and Scope There has been substantial scientific progress relevant to pediatric cardiology in the 10 years since the last training guidelines for research were published.1 The prior guidelines and a National Institutes of Health (NIH) expert panel stressed that there remains a critical need for advancement and application of new knowledge in a breadth of disciplines relevant to the field.2 These guidelines begin with the principle that there is a compelling need to train pediatric cardiology fellows to develop new knowledge and to translate research findings into practice. Because research skills are relevant to all those trained in our field, it …

Background:Cardiac remodelling is now recognised as an important aspect of cardiovascular disease progression and is, therefore, emerging as a therapeutic target in cardiac failure due to different etiologies. Little is known about the influence of different therapies for cardiac failure on the remodelling seen in infants with congenital cardiac disease.Methods:During follow-up of a prospective and randomized trial, we investigated therapeutic effects on neurohormonal activation, ventricular function, and myocardial gene expression. We compared the data from 8 infants with severe congestive heart failure due to left-to-right shunts, who received digoxin and diuretics alone, to 9 infants who received additional treatment with propranolol.Results:In these infants, β-adrenergic blockade significantly reduced highly elevated levels of renin, from 284 ± 319 μU/ml compared to 1061 ± 769 μU/ml. Systolic ventricular function was normal in both groups, but diastolic ventricular function was improved in those receiving propranolol, indicated by significantly lower left atrial pressures, lower end-diastolic pressures, and less pronounced ventricular hypertrophy, the latter estimated by lower ratios of myocardial wall to ventricular cavity areas on average of 42%. Further hemodynamic parameters showed no significant differences between the groups, except for the lower heart rate in infants treated with propranolol. In those treated with digoxin and diuretics, there was a significant downregulation of β2-receptor and angiotensin-2 receptor genes, and up-regulation of endothelin A receptor and connective tissue growth factor genes, that were partially prevented by additional treatment with propranolol.Conclusions:β-blockade is a new therapeutic approach for congestive heart failure in infants with congenital cardiac disease, producing with significant benefits on neurohormonal activation, diastolic ventricular function, and cardiac remodelling.

Heart failure remains the main cause of death in children with heart disease. In USA and Europe hospital mortality of children with heart failure is about 7% of children, nearly twice as high as in adults. In this review a group of authors report about their experience with beta-blockers in childhood heart failure. Most of them start to treat children with severe heart failure at a time - 20 years ago - when beta blockers seem to be contraindicated in this situation. The physicians and their patients and/or parents all are aware of the risk of this decision. However, unproven medical therapies for heart failure are the most important therapeutical dilemma in pediatric cardiology. The authors carefully observed a highly selected group of patients with the highest risk to die and had the patience to wait for the longtime follow up. Today - based upon this experience -we know that beta blockers are safe and may save the lives of many children with heart disease all over the world. Together with young colleagues who enthusiastically support this idea the authors now intend to break down the "wall of ignorance" for this promising therapy in pediatric cardiology.

We studied neurohormonal, clinical and invasively measured hemodynamic data of 47 infants with left-to-right shunts and varying degrees of congestive failure. When referred to a clinical heart failure score, plasma renin activities (r=0.71) and norepinephrine levels (r=0.43) are significantly increased. Arterial hypotension seems to be the hemodynamic trigger of renin release (r=-0.72), but not decreased systemic cardiac index (r=-0.43), the magnitude of the left-to-right shunt (r=0.33) or a reduced ejection fraction (r=0.12). These data indicate neurohormonal activation in infants with left-to-right shunts with preserved myocardial function is similar to the activation in adults with heart failure secondary to myocardial pump failure. These findings have to be considered for optimal medical treatment of these infants with angiotensin-converting enzyme inhibitors or beta-blockers.

To evaluate the therapeutical effects of the angiotensin converting enzyme inhibitor Captopril to the beta-blocker Propranolol in infants with congestive failure due to pulmonary overcirculation, we retrospectively analysed clinical, neurohormonal and hemodynamic data in 22 infants, 11 of whom were treated with Captopril (Group 1), 11 with Propranolol (Group 2). Age, weight, number of palliative operations, plasma renin activities and pulmonary to systemic flow ratios (3.5 vs. 3.5) were not significantly different prior to Captopril or Propranolol therapy. If treatment with digoxin and diuretics did not succeed, the infants were additionally treated with Captopril (1 mg/kg) for a mean of 7.4 months, or with 1.9 mg/kg Propranolol for 9.2 months. Results: 1 mg/kg Captopril did not effectively suppress angiotensin converting enzyme in the steady state at trough level (92±52 vs. 87±50 nmol/min/ml). In the Propranolol group, the clinical heart failure score (2.6±1.5 vs. 7.4±2.5) and plasma renin activities (14±10 vs. 101±70 ng/ml/h) were significantly lower, compared to the Captopril group. Length of hospital stay (23±9 vs. 52±24 days) was lower and weight gain (126±38 vs. 86±84 g/week) was higher within 3 months after starting Propranolol therapy. Significantly lower left atrial pressures (6.2±2.2 vs. 13.4±9.2 mmHg) and lower endiastolic ventricular pressures (7.6±2.5 vs. 12.6±4.0 mmHg) during pre-operative cardiac catheterization indicated a better diastolic ventricular function under chronic Propranolol treatment. Conclusion: Although high dose Captopril was not evaluated in this study, when compared to patients on low Captopril dosages, infants who received Propranolol treatment showed improvement in heart failure scores, shorter lengths of hospital stay, lower plasma renin activities and better diastolic ventricular functions.

The original Palmaz balloon expandable stent has been used extensively for the treatment of vascular stenoses in older children and young adults. Placement of the Palmaz stent in infants and small children, however, is limited by stent inflexibility, large delivery sheath size, and concerns about creating fixed obstructions after the placement of small diameter stents in growing patients. New Palmaz Corinthian stents were placed through 6 French sheaths in four high-risk patients with postoperative right ventricular outflow obstruction. Patients were not considered candidates for surgical repair. Median patient age and weight were 17 months (range 5‐32 months) and 7.7 kg (range 4.6 ‐11.1 kg), respectively. Median fluoroscopy time was 58.2 min (range 55.2‐172 min). No complications were encountered. In each case, successful stent placement was achieved, and surgery with cardiopulmonary bypass was avoided. Palmaz Corinthian stents are more flexible, require a smaller delivery sheath, have equal or increased radial strength, and can be maximally expanded to a greater cross sectional area when compared to the original Palmaz stent. These characteristics make the Palmaz Corinthian stent a reasonable alternative for use in a select group of infants and small children who are not candidates for surgical repair of postoperative right ventricular outflow obstruction. Cathet. Cardiovasc. Intervent. 51:444 ‐ 449, 2000. © 2000 Wiley-Liss, Inc.

Reason for Withdrawal Authors have made no progress with this protocol. No new authors are being sought to take over this protocol as the Heart Group considers this topic as of not sufficiently high priority. To view the published versions of this article, please click the 'Other versions' tab.

Clinical outcomes among children with idiopathic dilated cardiomyopathy (IDC) are diverse, which makes the decision as to when a patient should be listed for a cardiac transplantation challenging. This study aimed to determine echocardiographic and clinical variables that can help clinicians identify those at highest risk for death or cardiac transplantation. The study was a single-center, retrospective chart review of children with IDC. Patients younger than 18 years with a diagnosis of IDC, as defined by a left ventricular end-diastolic dimension (LVEDD) z-score higher than 2, and fractional shortening of less than 28 % on the initial echocardiogram, were included in the study. Echocardiographic parameters including mitral regurgitation (MR) grade and certain clinical parameters at the time of presentation were assessed. A follow-up echocardiogram was similarly studied. The study included 49 children with IDC. Those who died or underwent cardiac transplantation were grouped as “nonsurvivors” (n = 26). The remaining children who either completely recovered or experienced chronic dilated cardiomyopathy were grouped as “survivors” (n = 23). The median age overall was 1.25 years (range 0.1–17 years). The follow-up echocardiograms of the survivors showed significant improvement in left ventricle size, systolic function, left atrial volume, and MR grade, whereas these parameters did not change in the nonsurvivor group. The use of inotropic medications at initial presentation was an independent predictor of death or cardiac transplantation (p < 0.05). The presence of moderate to severe MR at diagnosis also was predictive of a worse outcome.

Background. Atresia of the coronary sinus orifice with a persistent left superior vena cava is an intrinsically benign cardiac anomaly with important surgical implications. Methods. The medical records of 5 patients with atresia of the coronary sinus orifice with a persistent left superior vena cava were reviewed retrospectively, and a computer search of the world literature describing this cardiac malformation was undertaken. Results. The 5 patients ranged in age from 9 months to 5 years. In 2, the diagnosis was made preoperatively by angiocardiography, and in 3, the abnormality was found incidentally at the time of cardiotomy for repair of associated congenital heart disease. Four of the 5 patients underwent repair of associated cardiac lesions. During operation, care was taken to avoid disruption of left superior vena cava flow to prevent coronary venous obstruction. All patients survived and are doing well at follow-up. Conclusions. Atresia of the coronary sinus orifice with persistent left superior vena cava is, in itself, a benign anomaly without physiologic consequence. However, the recognition of this lesion during repair of associated cardiac lesions is of vital importance to the cardiac surgeon. Interruption of this sole route of coronary venous drainage can potentially lead to myocardial ischemia and necrosis.

Pediatric heart failure (HF) is an important cause of morbidity and mortality in childhood. This article presents guidelines for the recognition, diagnosis, and early medical management of HF in infancy, childhood, and adolescence. The guidelines are intended to assist practitioners in office-based or emergency room practice, who encounter children with undiagnosed heart disease and symptoms of possible HF, rather than those who have already received surgical palliation. The guidelines have been developed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, and are accompanied by practical Recommendations for their application in the clinical setting, supplemented by online material. This work does not include Recommendations for advanced management involving ventricular assist devices, or other device therapies.

Accurate grading of the presence and severity of heart failure (HF) signs and symptoms in infants and children remains challenging. It has been 25 years since the Ross classification was first used for this purpose. Since then, several modifications of the system have been used and others proposed. New evidence has shown that in addition to signs and symptoms, data from echocardiography, exercise testing, and biomarkers such as N-terminal pro-brain natriuretic peptide (NT-proBNP) all are useful in stratifying outcomes for children with HF. It also is apparent that grading of signs and symptoms in children is dependent on age because infants manifest HF differently than toddlers and older children. This review culminates in a proposed new age-based Ross classification for HF in children that incorporates the most useful data from the last two decades. Testing of this new system will be important to determine whether an age-stratified scoring system can unify the way communication of HF severity and research on HF in children is performed in the future.

This study provided an assessment of the effects of lead on early growth in rats based on structural equation modeling and nested analysis of variance (ANOVA). Structural equation modeling showed that lead in drinking water (250, 500, or 1000 ppm) had a direct negative effect on body weight and tail length (i.e., growth) in female rats during the first week of exposure. During the following 2 weeks of exposure, high correlation between growth measurements taken over time resulted in reduced early postnatal growth. By the fourth week of exposure, reduced growth was not evident. Mating began after 8 weeks of exposure, and exposure continued during gestation. Decreased fetal body weight was detected when the effects of litter size, intrauterine position, and sex were controlled in a nested ANOVA. Lead exposure did not appear to affect fetal skeletal development, possibly because lead did not alter maternal serum calcium and phosphorus levels. The effect of lead on individual fetal body weight suggests that additional studies are needed to examine the effect of maternal lead exposure on fetal development and early postnatal growth.

Endothelin-1 (ET), a potent vasoconstrictor peptide, has been found to be elevated in children with pulmonary hypertension associated with congenital heart defects. To evaluate the effect of pulmonary blood flow on ET concentrations, 5 ml blood samples were obtained peripherally at cardiac catheterization from 35 patients, ages 0.13 to 17 years (median 2). Plasma was extracted and ET measured by radioimmunoassay. Patients were classified into 2 groups based on the presence (group A) or absence (group B) of increased pulmonary blood flow defined as a Qp Qs ≥1.5 . When the 13 patients (37%) in group A were compared with the 22 patients (63%) in group B there were no significant differences in age, cardiac index, or pulmonary and systemic resistances. ET concentrations were significantly higher in group A patients (median 3.25, range 0 to 16.5 vs median 0, range 0 to 6.35 pg/ml; p ≤ 0.05). Pulmonary Mood flow and pulmonary artery pressure were also higher in group A patients (p ≤ 0.01). When patients within group A were subdivided into those with and without pulmonary hypertension, no difference was present in their ET concentrations ( mean/SD : 4.4 4.3 vs 4.0 6.4 pg/ml, p = NS ). Thus, ET is elevated in patients with congenital heart disease associated with left-to-right shunts and it appears that this increase is related to increased pulmonary blood flow independent of pulmonary artery pressure.

To determine which variables most accurately define congestive heart failure (CHF) in infants, 41 patients (median age 2.5 months) were graded by four pediatric cardiologists for the presence and severity of CHF based on the following variables: amount of formula consumed per feeding, feeding time, history of diaphoresis or tachypnea, growth parameters, respiratory and heart rates, respiratory pattern, perfusion, presence of edema, diastolic filling sounds, and hepatomegaly. There were 19 patients graded as having no CHF, nine as mild, seven moderate, and six severe CHF. The most sensitive and specific variables (p less than 0.0001) for the presence of CHF were a history of less than 3.5 oz/feed, respiratory rate greater than 50/min, an abnormal respiratory pattern, diastolic filling sounds, and hepatomegaly. Moderate to severe CHF was present when patients took less than 3 oz/feed or greater than 40 min/feed, had an abnormal respiratory pattern with a resting respiratory rate greater than 60/min, and had a diastolic filling sound and moderate hepatomegaly. Severe CHF was accompanied by a heart rate greater than 170/min, decreased perfusion, and severe hepatomegaly. Thus, the grading of the severity of CHF in infants should include an accurate description of these historical and clinical variables.