Carissa M. Baker-Smith

Clinical Practice Guideline for Screening and Management of High Blood Pressure in Children and Adolescents.

These pediatric hypertension guidelines are an update to the 2004 “Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents.” Significant changes in these guidelines include (1) the replacement of the term “prehypertension” with the term “elevated blood pressure,” (2) new normative pediatric blood pressure (BP) tables based on normal-weight children, (3) a simplified screening table for identifying BPs needing further evaluation, (4) a simplified BP classification in adolescents ≥13 years of age that aligns with the forthcoming American Heart Association and American College of Cardiology adult BP guidelines, (5) a more limited recommendation to perform screening BP measurements only at preventive care visits, (6) streamlined recommendations on the initial evaluation and management of abnormal BPs, (7) an expanded role for ambulatory BP monitoring in the diagnosis and management of pediatric hypertension, and (8) revised recommendations on when to perform echocardiography in the evaluation of newly diagnosed hypertensive pediatric patients (generally only before medication initiation), along with a revised definition of left ventricular hypertrophy. These guidelines include 30 Key Action Statements and 27 additional recommendations derived from a comprehensive review of almost 15 000 published articles between January 2004 and July 2016. Each Key Action Statement includes level of evidence, benefit-harm relationship, and strength of recommendation. This clinical practice guideline, endorsed by the American Heart Association, is intended to foster a patient- and family-centered approach to care, reduce unnecessary and costly medical interventions, improve patient diagnoses and outcomes, support implementation, and provide direction for future research.

Variation in Postoperative Care following Stage I Palliation for Single-ventricle Patients: A Report from the Joint Council on Congenital Heart Disease National Quality Improvement Collaborative

BACKGROUND AND METHODS The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) has established a national data registry for patients with hypoplastic left heart syndrome and its variants undergoing staged palliation. The goal of this collaborative is to better understand current care practices and to improve outcomes in children with these severe and complex forms of congenital heart disease. In this study, we describe the postoperative intensive care course, and its variations, for the first 100 patients enrolled into the registry. RESULTS Patients were enrolled from 21 contributing sites and were discharged home after stage I palliation between July 2008 and February 2010. Following stage I palliation, enrolled participants remained in the intensive care unit for a median duration of 11 days (range: 3-68 days). Duration of intensive care unit stay varied and was greatest for those patients with aortic atresia versus aortic hypoplasia (P= 0.04) and for those who underwent a modified Blalock-Taussig shunt as part of their palliation. The duration of intensive care unit stay also varied by contributing site (medians ranged from 8 to 18 days). Participants requiring reoperation had significantly prolonged lengths of stay (P= .0003). Inotropic agent use among univentricular registry participants also varied by site. The majority of recipients received milrinone (87%), dopamine (64%), and epinephrine (62%). Cardiac catheterization following surgery occurred in 20 patients. Fifteen percent of participants underwent an interventional procedure. Complication following stage I palliation was also fairly common. CONCLUSIONS Considerable variation exists in the postoperative course and management of univentricular patients following stage I palliation. Variation in length of intensive care unit stay, inotropic agent use, need for reoperation or cardiac catheterization, and postoperative complications are described. Further studies to determine etiologies for observed variation may result in improved standards of care and better outcomes during the interstage period.

Predictors of Prolonged Hospital Length of Stay Following Stage II Palliation of Hypoplastic Left Heart Syndrome (and Variants): Analysis of the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) Database

The objective of this study is to identify predictors of prolonged hospital length of stay (LOS) for single ventricle patients following stage 2 palliation (S2P), excluding patients who underwent a hybrid procedure. We explore the impact of demographic features, stage 1 palliation (S1P), interstage I (IS1) management, S2P, and post-surgical care on hospital LOS following S2P. We conducted a retrospective analysis of the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) database. The NPC-QIC database is an established registry of patients with hypoplastic left heart syndrome (HLHS) and its variants. It contains detailed information regarding the demographic features, S1P, IS1, S2P, and interstage 2 (IS2) management of children with HLHS and related single ventricle cardiac malformations. Between 2008 and 2012, there were 477 participants with recorded LOS data in the NPC-QIC registry. Excluding the 29 patients who underwent hybrid procedure, there were 448 participants who underwent a Norwood (or Norwood-variant procedure) as S1P. In order to be included in the NPC-QIC database, participants were discharged to home following S1P and prior to S2P. We found that postoperative LOS among the 448 S2P procedure recipients is most strongly influenced by the need for reoperation following S2P, the need for an additional cardiac catheterization procedure following S2P, the use of non-oral methods of nutrition (e.g., nasogastric tube, total parental nutrition, gastrostomy tube), and the development of postoperative complications. Factors such as age at the time of S2P, the presence of a major non-cardiac anomaly, site participant volume, IS1 course, the type and number of vasoactive agents used following S2P, and the need for more than 1 intensive care unit (ICU) hospitalization (following discharge to the ward but prior to discharge to home) were significant predictors by univariate analysis but not by multivariate analysis. We excluded participants undergoing the hybrid procedure as S1P from this analysis given that the S2P following the initial hybrid is typically a more complicated procedure. Hospital LOS following S2P among children undergoing the Norwood or Norwood-variant procedure as S1P is most strongly influenced by events following S2P and not demographic or S1P factors. Factors most predictive of prolonged LOS include the need for reoperation, the need for an additional cardiac catheterization procedure following S2P, the need for non-oral methods of nutrition, and the development of postoperative complications.

Development of Quality Metrics in Ambulatory Pediatric Cardiology

The American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Section had attempted to create quality metrics (QM) for ambulatory pediatric practice, but limited evidence made the process difficult. The ACPC sought to develop QMs for ambulatory pediatric cardiology practice. Five areas of interest were identified, and QMs were developed in a 2-step review process. In the first step, an expert panel, using the modified RAND-UCLA methodology, rated each QM for feasibility and validity. The second step sought input from ACPC Section members; final approval was by a vote of the ACPC Council. Work groups proposed a total of 44 QMs. Thirty-one metrics passed the RAND process and, after the open comment period, the ACPC council approved 18 metrics. The project resulted in successful development of QMs in ambulatory pediatric cardiology for a range of ambulatory domains.

Safe and Effective Use of Pharmacologic and Device Therapy for Peripartum Cardiomyopathy.

Peripartum cardiomyopathy (PPCM) is an uncommon, idiopathic complication of pregnancy associated with significant (10–30%) mortality. The disease occurs late in pregnancy or in the months following delivery, resulting in reduced systolic function and heart failure (HF) symptoms. Limited direction is provided for the management of PPCM, and the safe and effective use of medications in pregnant and breastfeeding women with PPCM presents a unique challenge. Although several HF therapies in pregnant and lactating women are supported by robust evidence, evidence to support the use of other therapies is significantly lacking. Current guidelines recommend treatment as in other forms of HF with reduced left ventricular ejection fraction (LVEF) but with consideration for the important nuances in this population as well as the unique and potential teratogenic effects of these therapies. Since most patients with PPCM recover their LVEF, the duration of therapy is currently unknown and warrants further study. We review the available literature surrounding pharmacologic and device therapy for PPCM and provide insight into managing patients with the condition.

Cardiac Arrest and Sudden Cardiac Death in the Pediatric Population

Sudden cardiac arrest in children is a rare event. However, sudden cardiac arrest of a child is a devastating event. Clinicians should be aware of conditions associated with sudden cardiac arrest in the pediatric population. This article reviews the most common causes of sudden cardiac arrest in the pediatric population. We review recommendations for screening and diagnosis. We also briefly discuss current management recommendations for the various causes of sudden cardiac arrest in the pediatric population.

Diagnosis, Evaluation, and Management of High Blood Pressure in Children and Adolescents

Systemic hypertension is a major cause of morbidity and mortality in adulthood. High blood pressure (HBP) and repeated measures of HBP, hypertension (HTN), begin in youth. Knowledge of how best to diagnose, manage, and treat systemic HTN in children and adolescents is important for primary and subspecialty care providers. OBJECTIVES: To provide a technical summary of the methodology used to generate the 2017 “Clinical Practice Guideline for Screening and Management of High Blood Pressure in Children and Adolescents,” an update to the 2004 “Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents.” DATA SOURCES: Medline, Cochrane Central Register of Controlled Trials, and Excerpta Medica Database references published between January 2003 and July 2015 followed by an additional search between August 2015 and July 2016. STUDY SELECTION: English-language observational studies and randomized trials. METHODS: Key action statements (KASs) and additional recommendations regarding the diagnosis, management, and treatment of HBP in youth were the product of a detailed systematic review of the literature. A content outline establishing the breadth and depth was followed by the generation of 4 patient, intervention, comparison, outcome, time questions. Key questions addressed: (1) diagnosis of systemic HTN, (2) recommended work-up of systemic HTN, (3) optimal blood pressure (BP) goals, and (4) impact of high BP on indirect markers of cardiovascular disease in youth. Once selected, references were subjected to a 2-person review of the abstract and title followed by a separate 2-person full-text review. Full citation information, population data, findings, benefits and harms of the findings, as well as other key reference information were archived. Selected primary references were then used for KAS generation. Level of evidence (LOE) scoring was assigned for each reference and then in aggregate. Appropriate language was used to generate each KAS based on the LOE and the balance of benefit versus harm of the findings. Topics that could not be researched via the stated approach were (1) definition of HTN in youth, and (2) definition of left ventricular hypertrophy. KASs related to these stated topics were generated via expert opinion. RESULTS: Nearly 15 000 references were identified during an initial literature search. After a deduplication process, 14 382 references were available for title and abstract review, and 1379 underwent full text review. One hundred twenty-four experimental and observational studies published between 2003 and 2016 were selected as primary references for KAS generation, followed by an additional 269 primary references selected between August 2015 and July 2016. The LOE for the majority of references was C. In total, 30 KASs and 27 additional recommendations were generated; 12 were related to the diagnosis of HTN, 13 were related to management and additional diagnostic testing, 3 to treatment goals, and 2 to treatment options. Finally, special additions to the clinical practice guideline included creation of new BP tables based on BP values obtained solely from children with normal weight, creation of a simplified table to enhance screening and recognition of abnormal BP, and a revision of the criteria for diagnosing left ventricular hypertrophy. CONCLUSIONS: An extensive and detailed systematic approach was used to generate evidence-based guidelines for the diagnosis, management, and treatment of youth with systemic HTN.

Description of Antihypertensive Medication Use in a Pediatric Practice: Single and Multiple Antihypertensive Medication Therapy

Prescription of multiple antihypertensive medications for the treatment of essential hypertension (HTN) has been well described in adults but not in children and adolescents. The authors describe the frequency with which children with essential HTN are prescribed a single vs two or more concomitantly administered antihypertensive medications. They also describe demographic features and comorbidities associated with the prescription of a single vs multiple antihypertensive medications. Multiple antihypertensive medication use in the management of pediatric HTN, as in the management of adult HTN, is not uncommon. In this single‐center, retrospective study of 113 children with essential HTN, 28% of children were concomitantly prescribed two or more antihypertensive medications for poorly controlled blood pressure following prescription of a single medication. Demographic and comorbid conditions associated with the prescription of more than one antihypertensive medication include advanced hypertensive stage, race, and a family history of HTN.