The Role of Exercise Prescription in Pediatric Preventive Cardiology Programs.

Lifestyle changes such as exercise and dietary change are recommended first-line therapy for children with dyslipidemia, hypertension, and obesity. Although most clinicians recommend exercise, specific exercise prescriptions are not usually provided. The optimal type, duration, and intensity of activity to achieve a meaningful outcome is not definitively defined. As the pediatric population becomes more sedentary and morbidity accumulates from this lifestyle, understanding how targeted exercise prescriptions can benefit patients will be critical to effectively manage this group of patients. This review focuses on the role of exercise for the treatment of pediatric patients with dyslipidemia, hypertension, and obesity and describes additional factors that require future research to achieve desired outcomes in this at-risk population. [Pediatr Ann. 2018;47(12):e494-e498.].

Heart rate recovery is impaired after maximal exercise testing in children with sickle cell anemia.

OBJECTIVE: To examine heart rate recovery (HRR) as an indicator of autonomic nervous system dysfunction after maximal exercise testing in children and young adults with sickle cell anemia (SCA). STUDY DESIGN: Recovery phase heart rate (HR) in the first 5 minutes after maximal exercise testing in 60 subjects with SCA and 30 matched controls without SCA was assessed. The difference between peak HR and HR at both 1-minute (ΔHR1min) and 2-minutes recovery was our primary outcome. RESULTS: Compared with controls, subjects with SCA demonstrated significantly smaller mean ΔHR1min (23 beats per minute [bpm], 95% CI 20-26 vs 32 bpm, 95% CI 26-37, P = .006) and the difference between maximal HR and HR at 2 minutes (39 bpm, 95% CI 36-43 vs 48 bpm, 95% CI 42-53, P = .011). Subjects with SCA also showed smaller mean changes in HR from peak HR to 1 minute, from 1 minute to 2 minutes, and from 2 through 5 minutes of recovery by repeated-measures testing. In a multivariable regression model, older age was independently associated with smaller ΔHR1min in subjects with SCA. Cardiopulmonary fitness and hydroxyurea use, however, were not independent predictors of ΔHR1min. CONCLUSIONS: Children with SCA demonstrate impaired HRR after maximal exercise. Reduced postexercise HRR in SCA suggests impaired parasympathetic function, which may become progressively worse with age, in this population.

Initiation and use of propranolol for infantile hemangioma: report of a consensus conference.

Infantile hemangiomas (IHs) are common neoplasms composed of proliferating endothelial-like cells. Despite the relative frequency of IH and the potential severity of complications, there are currently no uniform guidelines for treatment. Although propranolol has rapidly been adopted, there is significant uncertainty and divergence of opinion regarding safety monitoring, dose escalation, and its use in PHACE syndrome (PHACE = posterior fossa, hemangioma, arterial lesions, cardiac abnormalities, eye abnormalities; a cutaneous neurovascular syndrome characterized by large, segmental hemangiomas of the head and neck along with congenital anomalies of the brain, heart, eyes and/or chest wall). A consensus conference was held on December 9, 2011. The multidisciplinary team reviewed existing data on the pharmacologic properties of propranolol and all published reports pertaining to the use of propranolol in pediatric patients. Workgroups were assigned specific topics to propose protocols on the following subjects: contraindications, special populations, pretreatment evaluation, dose escalation, and monitoring. Consensus protocols were recorded during the meeting and refined after the meeting. When appropriate, protocol clarifications and revision were made and agreed upon by the group via teleconference. Because of the absence of high-quality clinical research data, evidence-based recommendations are not possible at present. However, the team agreed on a number of recommendations that arose from a review of existing evidence, including when to treat complicated IH; contraindications and pretreatment evaluation protocols; propranolol use in PHACE syndrome; formulation, target dose, and frequency of propranolol; initiation of propranolol in infants; cardiovascular monitoring; ongoing monitoring; and prevention of hypoglycemia. Where there was considerable controversy, the more conservative approach was selected. We acknowledge that the recommendations are conservative in nature and anticipate that they will be revised as more data are made available.

J. Maxwell Chamberlain Memorial Paper for congenital heart surgery. 111 Fontan conversions with arrhythmia surgery: surgical lessons and outcomes.

BACKGROUND: The evolving operative strategy and course of 111 consecutive Fontan conversions with arrhythmia surgery and pacemaker therapy were reviewed to identify risk factors for poor outcome. METHODS: Since 1994, 111 patients (mean age 22.5 +/- 7.9 years) underwent Fontan conversion with arrhythmia surgery. The series was divided into three time periods: (1) 1994 to 1996 (initial isthmus ablation, n = 9, group I); (2) 1996 to 2003 (early modified right atrial maze and Cox-maze III, n = 51, group II); and (3) 2003 to 2006 (recent modifications of the modified right atrial maze and left atrial Cox-maze III for both atrial fibrillation and left atrial reentry tachycardia, n = 51, group III). RESULTS: There were one early (0.9%) and six late deaths (5.4%); six patients required cardiac transplantation (5.4%). Two late deaths occurred after transplantation. Renal failure requiring dialysis occurred in four patients (3.6%). Mean hospital stay was 13.7 +/- 12.1 days. Mean cross-clamp time was 70.8 +/- 41.6 minutes. Four risk factors for death or transplantation were identified: presence of a right or ambiguous ventricle, preoperative protein-losing enteropathy, preoperative moderate-to-severe atrioventricular valve regurgitation, and long (>239 minutes) cardiopulmonary bypass time. In intergroup comparisons (groups I and II versus group III), three trends were noted: increased incidence of concomitant surgical repairs (p = 0.03), older patients (p = 0.01), and increased incidence of left atrial reentry tachycardia and atrial fibrillation (p = 0.04). Late recurrence of atrial tachycardia ensued in 15 of 111 (13.5%); 8 of 51 in group II (15.7%) and 4 of 51 in group III (7.8) (p = 0.3). CONCLUSIONS: Fontan conversion with arrhythmia surgery is safe and efficacious. Based on improved results and evolving surgical techniques, selection criteria can be more clearly defined.

Pediatric heart transplantation for anthracycline cardiomyopathy: cancer recurrence is rare.

BACKGROUND: Although anthracycline therapy is invaluable for treating neoplastic disorders, morbidity includes severe cardiomyopathy that leads to heart transplantation. This multicenter study describes the course of children who experienced anthracycline cardiomyopathy (ACM) and who subsequently required heart transplantation. METHODS: We reviewed transplant databases/registries at 4 pediatric heart transplant centers to identify children with ACM who were listed for heart transplantation. We reviewed medical records to determine cancer therapy, clinical course, and outcome. RESULTS: Eighteen patients were listed, and 17 underwent transplantation. Mean age at cancer diagnosis was 6.0 years (SD, 3.7). The mean anthracycline dose was 361 mg/m2 (SD, 110). The median time from cancer diagnosis to listing for heart transplantation was 9.2 years (range, 0.4-15.2 years). Six transplantations were performed in patients who had disease-free intervals of <5 years. Two patients were lost to follow-up, and 8 are alive at 4.9 years (SD, 2.0; range, 1.3-7.4 years) after transplantation. Seven patients died at 4.7 years (SD, 2.0; range, 1.2-7.1 years) after transplantation. One patient had recurrent cancer. One-, 2- and 5-year survivals were 100%, 92%, and 60%, respectively. CONCLUSIONS: Cardiomyopathy that progresses to the need for heart transplantation occurs in patients receiving a wide range of cumulative anthracycline doses. The time from chemotherapy to ACM varies. Outcomes after transplantation are acceptable, and cancer recurrence is rare. Reconsideration of the 5-year disease-free wait period is warranted.