Assessment of Exercise Function in Children and Young Adults with Hypertrophic Cardiomyopathy and Correlation with Transthoracic Echocardiographic Parameters.

Exercise function is well characterized in adults with hypertrophic cardiomyopathy (HCM); however, there is a paucity of data in children and young adults with HCM. Here we sought to characterize exercise function in young people with HCM, understand limitations in exercise function by correlating exercise function parameters with echocardiogram parameters and identify prognostic value of exercise parameters. We performed a retrospective, single-center cohort study characterizing exercise function in patients < 26 years old with HCM undergoing cardiopulmonary exercise testing (CPET). Patients with syndromic HCM or submaximal effort were excluded. We compared exercise function in this cohort to population normal values and measured changes in exercise function over time. We correlated exercise function parameters with echocardiographic parameters and investigated the relationship between exercise test parameters and a clinical composite outcome comprised of significant ventricular arrhythmia, death, or heart transplantation. We identified 229 CPETs performed by 117 patients (mean age at time of first CPET 15.6 ± 3.2 years). Mean %-predicted peak VO2, O2 pulse, and peak heart rate were statistically significantly depressed compared to population normal values and exercise function gradually worsened over time. Abnormal exercise testing correlated closely with echocardiographic indices of diastolic dysfunction. There was a trend toward increased incidence of poor clinical outcome in patients with abnormal exercise function. While adverse clinical outcomes were rare, normal exercise function appears to be a marker of low risk for adverse clinical outcomes in this population.

Increasing Utilization of the Preparticipation Physical Evaluation.

OBJECTIVE: The American Heart Association and American Academy of Pediatrics endorse the preparticipation physical evaluation (PPE) to screen student athletes for the risk of sudden cardiac arrest. We sought to identify barriers precluding its use and improve utilization. METHODS: We analyzed documentation of PPE elements during well-care visits of patients aged 12 to 18 years from 5 primary care practices. Employing quality improvement (QI) methodology, we focused on improving PPE utilization in 1 practice by assessing the number of PPE elements addressed per chart. We expanded our QI project to 4 additional practices by using the same interventions but assessing the percentage of charts that had a complete PPE documented. RESULTS: A baseline analysis of 5 targeted practices revealed an average of 3.5 of 14 PPE elements documented. Using plan-do-study-act cycles, PPE elements addressed increased from 2.5 to 14 over an 18-month period in the initial practice. By spreading successful interventions to 4 other practices, complete PPE utilization increased from a median baseline of 10.0% to a median of 70.0% over a 12-month period. Postintervention, 12 of 16 patients (75%) required additional follow-up with pediatric cardiology beyond the initial consultation, as compared with 2 of 14 patients (14%) preintervention. CONCLUSION: The PPE is an underutilized but effective tool in screening student athletes for sudden cardiac arrest. QI methodology was helpful in increasing the use of PPE in the primary care setting.

Cardiopulmonary stress testing in children and adults with congenital heart disease.

Cardiopulmonary exercise stress testing (CPET) is a vital tool used to assess patients with a history of congenital heart disease. There are several tests in the cardiologist's armamentarium that allow for assessment of cardiac anatomy and function. The majority of these tests are only performed with the body at rest and some even require sedation. Exercise stress testing is unique in allowing assessment of the hemodynamic status of a patient in motion. In addition to providing all the information obtained during an exercise stress test, such as heart rate, rhythm, ST-segment analysis, and blood pressure, the CPET provides critical metabolic information. Parameters such as VO2, oxygen pulse, and VE/VCO2 slope help to detail the patient's physiology in a dynamic state. Decisions can then be better made regarding follow-up plans, acceptable exercise recommendations, and future interventions, if necessary. It allows insight into the patient's exercise capacity and quality of life. Norms for both children and adults with many forms of congenital heart disease are now available allowing appropriate comparisons to be made. This review will discuss in detail the CPET and its application in congenital heart disease.