Timothy Cotts

Relation of Right Ventricular Dilation, Age of Repair, and Restrictive Right Ventricular Physiology With Patient-Reported Quality of Life in Adolescents and Adults With Repaired Tetralogy of Fallot

The present study aimed to determine the predictors of patient-reported quality of life and restrictive right ventricular (RV) physiology in adolescents and adults with repaired tetralogy of Fallot. A total of 62 patients (median age 28.5 years, range 14 to 69) undergoing cardiovascular magnetic resonance imaging completed the Short Form 36-item questionnaire, version 2, a validated quality of life assessment. RV inflow curves were generated from the sum of tricuspid inflow and pulmonary insufficiency. The patient-reported quality of life was comparable to population norms. Patients repaired after 1 year of age showed a strong trend toward a greater likelihood of physical component summary age-adjusted z-score ≤-1 (odds ratio 7.50, 95% confidence interval 0.90 to 62.3, p = 0.06). Patients with a RV ejection fraction of <45% reported decreased physical component summary (p = 0.02) and physical functioning (p = 0.02) scores. The RV end-diastolic volume, pulmonary regurgitation, and diastolic indexes did not predict the quality of life. The indexed RV end-diastolic volume was related to diastolic abnormalities, correlating with a greater peak early filling rate (r = 0.71, p <0.0001), ratio of peak early to atrial filling rates (r = 0.45, p = 0.006), and showing a strong trend with the end-diastolic forward flow in the pulmonary trunk (odds ratio 2.67 for moderate dilation and 3.50 for severe dilation, p = 0.06). Patients who underwent repair before 1 year old were more likely to have end-diastolic forward flow (15 of 17 vs 25 of 42, p = 0.03). In conclusion, the RV ejection fraction and age of repair were the best predictors of quality of life in this population, in whom end-diastolic forward flow and associated diastolic parameters appeared to reflect an overdistended ventricle, which might suggest a role for early pulmonary valve replacement.

Tracheostomy after pediatric cardiac surgery: Frequency, indications, and outcomes

OBJECTIVES This study was designed to review baseline characteristics and outcomes of children requiring tracheostomy after cardiac surgery. METHODS A retrospective review of children under age 2 requiring tracheostomy after cardiac surgery between January 1999 and December 2005 was performed. Indications for tracheostomy, survival, and completion of staged palliation were documented. RESULTS After cardiac surgery, 59 (1.3%) of 4503 patients with a median age at surgery of 15 days and weight of 3.5 kg required tracheostomy. Median duration from surgery to tracheostomy was 36 days (range 10-145 days). Genetic syndromes or major noncardiac comorbidities were present in 40% of patients. Biventricular repair was performed in 34 patients and univentricular repair in 25. Tetralogy of Fallot variants (29%) and coarctation±ventricular septal defect (21%) constituted the majority of biventricular lesions associated with tracheostomy, whereas unbalanced atrioventricular septal defect and hypoplastic left heart syndrome with highly restrictive atrial septal defect accounted for 52% of the single ventricle group. Indications for tracheostomy included the following: multifactorial (37%), tracheobronchomalacia, (24%), cardiac (12%), bilateral vocal cord paralysis (10%), bilateral diaphragm paralysis (2%), and other airway issues (15%). Hospital survival was 75% with intermediate-term (median, 25.5 months; range, 1-122 months) survival of 53%. Of 25 single ventricle patients, 6 (24%) had successful completion of the Fontan procedure. Of 12 patients with single ventricle who were ventilator-dependent after initial repair, 10 died, 1 remains at hemi-Fontan, and 1 has undergone completion of the Fontan procedure. CONCLUSIONS Requirement for tracheostomy in pediatric patients after cardiac surgery was associated with significant mortality. Patients with single ventricle have the highest late death rate and those with chronic ventilator dependency were unlikely to undergo successful Fontan completion.

Clinical research priorities in adult congenital heart disease

BACKGROUND Adult congenital heart disease (ACHD) clinicians are hampered by the paucity of data to inform clinical decision-making. The objective of this study was to identify priorities for clinical research in ACHD. METHODS A list of 45 research questions was developed by the Alliance for Adult Research in Congenital Cardiology (AARCC), compiled into a survey, and administered to ACHD providers. Patient input was sought via the Adult Congenital Heart Association at community meetings and online forums. The 25 top questions were sent to ACHD providers worldwide via an online survey. Each question was ranked based on perceived priority and weighted based on time spent in ACHD care. The top 10 topics identified are presented and discussed. RESULTS The final online survey yielded 139 responses. Top priority questions related to tetralogy of Fallot (timing of pulmonary valve replacement and criteria for primary prevention ICDs), patients with systemic right ventricles (determining the optimal echocardiographic techniques for measuring right ventricular function, and indications for tricuspid valve replacement and primary prevention ICDs), and single ventricle/Fontan patients (role of pulmonary vasodilators, optimal anticoagulation, medical therapy for preservation of ventricular function, treatment for protein losing enteropathy). In addition, establishing criteria to refer ACHD patients for cardiac transplantation was deemed a priority. CONCLUSIONS The ACHD field is in need of prospective research to address fundamental clinical questions. It is hoped that this methodical consultation process will inform researchers and funding organizations about clinical research topics deemed to be of high priority.

A Middle School Intervention to Improve Health Behaviors and Reduce Cardiac Risk Factors

OBJECTIVE This study aimed to measure the impact of a school-based multidisciplinary intervention program on risk factors for atherosclerosis in sixth-grade middle school students. We also measured health behaviors before and after the intervention using a validated questionnaire. METHODS A prospective study was performed in which students served as historical controls. Sixth-grade students from 23 middle schools in 12 cities with varying populations were exposed to a program promoting healthful activity and nutrition habits through educational and environmental change. Along with a modified School-Based Nutrition Monitoring behavioral questionnaire, physiologic risk factors were studied, including body mass index, systolic and diastolic blood pressures, cholesterol panel, and random blood glucose, which were measured before the 10-week program and again 1 to 3 months after program completion. RESULTS Of 4021 sixth graders (male, 49%) at 23 middle schools completing a before and after behavioral survey, 2118 students, aged 11.56 ± 0.47 years, consented to participate in the screening. The mean total cholesterol value decreased from 161.64 ± 28.99 mg/dL to 154.77 ± 27.26 mg/dL (P < .001). The low-density lipoprotein value decreased from 89.37 ± 25.08 mg/dL to 87.14 ± 24.25 mg/dL (P < .001). The high-density lipoprotein value decreased from 52.15 ± 13.35 md/dL to 49.95 ± 13.28 mg/dL (P < .001). The measure of triglycerides decreased from 113.34 ± 73.19 mg/dL to 101.22 ± 63.93 mg/dL (P < .001). The random glucose value decreased from 97.51 ± 16.00 to 94.94 ± 16.62 (P < .001). The mean systolic blood pressure decreased from 109.47 ± 15.26 mm Hg to 107.76 ± 10.87 mm Hg (P < .001), and the mean diastolic blood pressure decreased from 64.78 ± 8.57 mm Hg to 63.35 ± 7.81 mm Hg (P < .001). These changes in physiologic measures seemed to correlate with self-reported increases in vegetable and fruit consumption, increases in physical activity, and less screen time. CONCLUSIONS Project Healthy Schools, a middle school intervention to improve childhood cardiovascular risk factors, is feasible and seems to be effective. The results showed significant improvements in risk factors associated with early atherosclerosis among sixth-grade students, including total cholesterol, low-density lipoprotein cholesterol, triglycerides, and systolic and diastolic blood pressures. Further study with a larger group of students and a longer follow-up period would be valuable.

Assessment of Transition Readiness in Adolescents and Young Adults with Heart Disease.

OBJECTIVES To evaluate transition readiness, including perceived knowledge deficits, self-efficacy, and self-management behaviors, in 13- to 25-year-olds with congenital heart disease or heart transplant and to examine the relationships between transition readiness assessment, information seeking behavior, and quality of life (QOL). STUDY DESIGN In this cross-sectional study, patients (n = 164) completed the Transition Readiness Assessment and the Pediatric Quality of Life Inventory using an e-tablet, web-based format at a routine clinic visit. RESULTS Median patient age was 18.1 years (range 13.0-25.5). Average perceived knowledge deficit score (% of items with no knowledge) was 25.7% (range 0%-75%). On a 100-point scale, the mean score was 72.0 ± 17.2 for self-efficacy and 49.7 ± 17.5 for self-management. Knowledge deficits were negatively correlated with self-efficacy (r = -0.45, P < .0001) and self-management (r = -0.36, P < .0001). Overall, 66% of patients requested information (73% ≥ 18 years old). Higher psychosocial QOL scores were correlated with lower knowledge deficit scores (r = -0.21, P = .01) and higher perceived self-efficacy scores (r = 0.41, P < .0001). CONCLUSIONS Transition knowledge deficits are common and associated with decreased self-efficacy and self-management skills in adolescents and young adults with heart disease. Greater transition knowledge and perceived self-efficacy are associated with better psychosocial QOL. Routine assessment of transition readiness is recommended to facilitate recognition of deficits and interventions to promote successful transition outcomes.

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.

The Patient Protection and Affordable Care Act: Impact on care to patients with paediatric and congenital cardiac disease

Department of Pediatrics and Communicable Diseases, University of Michigan, C.S. Mott Children’s Hospital, Ann Arbor, Michigan; Department of Surgery, University of South Florida, Johns Hopkins Children’s Heart Surgery, All Children’s Hospital, St. Petersburg, Florida; Department of Pediatrics and Communicable Diseases, Gerald R. Ford School of Public Policy, Division of General Medicine, Child Health Evaluation and Research (CHEAR) Unit, Institute for Healthcare Policy and Innovation, University of Michigan, Ann Arbor, Michigan, United States of America

Late Migration of a Sideris Buttoned Device for Occlusion of Atrial Septal Defect

We describe a 17‐year‐old patient with an atrial septal defect who underwent device closure with a second generation Sideris buttoned device at 4 years of age. She presented 13 years after the procedure with the acute onset of chest discomfort, at which time a chest radiograph showed migration of the wire of the right atrial counter‐occluder to the lateral aspect of the right atrial wall, with an associated right atrial perforation. The patient underwent uneventful surgical device retrieval and atrial septal defect closure. Late migration of a portion of the Sideris buttoned device can occur, suggesting the need for continued follow up, and a high index of suspicion for device failure should a patient become symptomatic.

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Right pulmonary artery to left atrial fistula: a rare presentation.

Right pulmonary artery to left atrial fistula is a rare congenital vascular anomaly which usually presents with cyanosis, clubbing, dyspnea, and signs and symptoms of a right to left shunt. Paradoxical embolism to the brain resulting in cerebral abscess formation and death is a rare and ominous complication that has been described. We describe an unusual presentation with abdominal pain resulting from splenic infarction.