Preparticipation Cardiac Evaluation from the Pediatric Perspective.

Each year millions of children and adolescents undergo sports preparticipation evaluations (PPEs) before participating in organized sports. A primary aim of the PPE is to screen for risk factors associated with sudden cardiac death. This article is designed to summarize the current thoughts on the PPE with a specific slant toward the pediatric and early adolescent evaluation and how these evaluations may differ from those in adults.

Impact of Sex on Cardiovascular Adaptations to Exercise: JACC Review Topic of the Week.

Routine exercise leads to cardiovascular adaptations that differ based on sex. Use of cardiac testing to screen athletes has driven research to define how these sex-based adaptations manifest on the electrocardiogram and cardiac imaging. Importantly, sex-based differences in cardiovascular structure and outcomes in athletes often parallel findings in the general population, underscoring the importance of understanding their mechanisms. Substantial gaps exist in the understanding of why cardiovascular adaptations and outcomes related to exercise differ by sex because of underrepresentation of female participants in research. As female sports participation rates have increased dramatically over several decades, it also remains unknown if differences observed in older athletes reflect biological mechanisms vs less lifetime access to sports in females. In this review, we will assess the effect of sex on cardiovascular adaptations and outcomes related to exercise, identify the impact of sex hormones on exercise performance, and highlight key areas for future research.

Standardizing Prostaglandin Initiation in Prenatally Diagnosed Ductal-Dependent Neonates; A Quality Initiative.

Prostaglandin E1 (PGE) is used in patients with ductal-dependent congenital heart disease (CHD). Side effects of apnea and fever are often dose dependent and occur within 48 h after initiation. We initiated a standardized approach to PGE initiation after our institution recognized a high incidence of side effects and a wide variety of starting doses of PGE. Neonates with prenatally diagnosed ductal-dependent CHD were identified, started on a standardized protocol that started PGE at 0.01 mcg/kg/min, and evaluated for PGE related side effects. Compliance, outcomes and dose adjustments during the first 48 h post-PGE initiation were evaluated. Fifty patients were identified (25 pre-intervention; 25 post-intervention). After intervention, compliance with the protocol was 96%, and apnea or fever occurred in 28% (compared to 63% pre-intervention, p = 0.015). Dose adjustments (either increase or decrease) prior to cardiac surgery were similar in both cohorts (60%, 52%, p = 0.569). There were no mortalities or emergent procedures performed due to ductus arteriosus closure. Standardizing a protocol for initiating PGE in prenatally diagnosed ductal-dependent CHD was successful and reduced the incidence of apnea, fever, and sepsis evaluations. A starting dose of 0.01 mcg/kg/min did not cause increased adverse effects.

Preparticipation Cardiac Evaluation from the Pediatric Perspective.

Each year millions of children and adolescents undergo sports preparticipation evaluations (PPEs) before participating in organized sports. A primary aim of the PPE is to screen for risk factors associated with sudden cardiac death. This article is designed to summarize the current thoughts on the PPE with a specific slant toward the pediatric and early adolescent evaluation and how these evaluations may differ from those in adults.

The Importance of Surrounding the Athlete's Heart with a Team.

Providing medical care for an athlete can be challenging in many aspects. One specific aspect is the athlete's cardiovascular system. Athletic training and physical activity certainly can improve cardiovascular health, but it can also cause cardiac adaptations and place athletes at risk for sudden cardiac arrest. When an athlete has cardiac symptoms, a concerning family history, abnormal cardiac testing, or an underlying cardiac condition, a wide range of professionals are needed to appropriately care for the athlete under evaluation.

Hospital Survival After Surgical Repair of Truncus Arteriosus with Interrupted Aortic Arch: Results from a Multi-institutional Database.

Truncus arteriosus (TA) is a major congenital cardiac malformation that requires surgical repair in the first few weeks of life. Interrupted aortic arch (IAA) is an associated malformation that significantly impacts the complexity of the TA operation. The aim of this study was to (1) define the comorbid conditions associated with TA and (2) determine the hospital survival and morbidity of patients with TA with and without an IAA. Data was collected from the Vizient Clinical Database/Resource Manager, formerly University HealthSystem Consortium, which encompasses more than 160 academic medical centers in the United States. The database was queried for patients admitted from 2002 to 2016 who were ≤ 4 months of age at initial admission, diagnosed with TA, and underwent complete surgical repair during that hospitalization. Of the 645 patients with TA who underwent surgery, 98 (15%) had TA with an interrupted aortic arch (TA-IAA). Both TA and TA-IAA were associated with a high prevalence of comorbidities, including DiGeorge syndrome, prematurity, and other congenital malformations. There was no difference in mortality between TA and TA-IAA (13.7-18.4%, p value = 0.227). No comorbid conditions were associated with an increased mortality in either group. However, patients with TA-IAA had a longer post-operative length of stay (LOS) compared to those without IAA (30 versus 40.3 days, p value = 0.001) and this effect was additive with each additional comorbid condition. In conclusion, the addition of IAA to TA is associated with an increased post-operative LOS, but does not increase in-hospital mortality.

Athlete Cardiovascular Concerns in the Training Room: What Do I Do If ?

Cardiac disease can present in the training room through three portals: the preparticipation history and physical may identify concerns, the athlete may present with symptoms, or screening modalities may demonstrate abnormal findings. Training-related cardiovascular remodeling can mimic real disease, therefore providers must be able to separate the two. Sports medicine providers must be knowledgeable in how these present and how to care for these concerns to ensure proper care and avoid unnecessary restrictions of athletes. This article discusses 10 common cardiac concerns that can arise in the training room.

Trajectory of right ventricular indices is an early predictor of outcomes in hypoplastic left heart syndrome.

BACKGROUND: Children with hypoplastic left heart syndrome (HLHS) have risk for mortality and/or transplantation. Previous studies have associated right ventricular (RV) indices in a single echocardiogram with survival, but none have related serial measurements to outcomes. This study sought to determine whether the trajectory of RV indices in the first year of life was associated with transplant-free survival to stage 3 palliation (S3P). METHODS: HLHS patients at a single center who underwent stage 1 palliation (S1P) between 2000 and 2015 were reviewed. Echocardiographic indices of RV size and function were obtained before and following S1P and stage 2 palliation (S2P). The association between these indices and transplant-free survival to S3P was examined. RESULTS: There were 61 patients enrolled in the study with 51 undergoing S2P, 20 S3P, and 18 awaiting S3P. In the stage 1 perioperative period, indexed RV end-systolic area increased in patients who died or needed transplant following S2P, and changed little in those surviving to S3P (3.37 vs -0.04 cm2 /m2 , P = .017). Increased indexed RV end-systolic area was associated with worse transplant-free survival. (OR = 0.815, P = .042). In the interstage period, indexed RV end-diastolic area increased less in those surviving to S3P (3.6 vs 9.2, P = .03). CONCLUSION: Change in indexed RV end-systolic area through the stage 1 perioperative period was associated with transplant-free survival to S3P. Neither the prestage nor poststage 1 indexed RV end-systolic area was associated with transplant-free survival to S3P. Patients with death or transplant before S3P had a greater increase in indexed RV end-diastolic area during the interstage period. This suggests earlier serial changes in RV size which may provide prognostic information beyond RV indices in a single study.

Malrotation is not associated with adverse outcomes after cardiac surgery in patients with heterotaxy syndrome.

BACKGROUND: Patients with heterotaxy syndrome (HS) often have asymptomatic malrotation. There is a lack of consensus regarding the management of these patients, particularly in patients with complex congenital heart disease (CHD). We sought to describe the prevalence of malrotation and incidence of volvulus in a population of patients with complex CHD and to identify the impact of malrotation on morbidity and mortality following cardiac surgery. METHODS: We performed a retrospective review of all patients with HS and complex CHD who required cardiac surgery in the first year of life at a single center between October 1995 and September 2015. Malrotation was diagnosed by abdominal imaging or by direct inspection during abdominal surgery. Demographic data was collected along with details of hospitalization following cardiac and GI surgeries. Descriptive analysis along with appropriate hypothesis testing was conducted to evaluate the results. RESULTS: We identified 49 patients with HS, 42 with single ventricle anatomy and 7 with biventricular anatomy. Of the 49 patients, 29 (59%) were diagnosed with malrotation, 6 (12%) had normal intestinal rotation, and 14 (29%) had no evaluation of intestinal rotation. The prevalence of malrotation in the population who underwent abdominal imaging was 29 out of 35 (83%). There was no difference in survival following cardiac surgery between patients with malrotation and those with unknown or normal intestinal anatomy. Comparing patients with malrotation and patients with normal or unknown intestinal rotation, there was also no difference in surrogate markers of morbidity. Of the 29 patients with known malrotation, only 2 patients (7%) underwent therapeutic Ladd procedures and 19 (65%) underwent prophylactic Ladd procedures. CONCLUSIONS: We conclude that the outcomes following cardiac surgery for patients with HS are not impacted by the presence of malrotation. Furthermore, we also found that the incidence of volvulus in the studied group is low. Given these findings, and the understanding that patients with HS and significant CHD are frequently tenuous and high risk surgical candidates, we do not believe performing prophylactic Ladd procedures is warranted. LEVEL OF EVIDENCE: III.

Coronary Artery Evaluation by Screening Echocardiogram in Intercollegiate Athletes.

INTRODUCTION: Coronary artery anomalies are the second most common congenital cause of sudden cardiac death in young athletes. Some centers have advocated for limited transthoracic echocardiogram (TTE) protocols in the screening of college athletes, which may miss important causes of sudden cardiac death. PURPOSE: The purpose of this study was to evaluate the ability of screening TTE to determine the origin and proximal course of the coronary arteries in intercollegiate athletes. METHODS: An institutional review board-approved retrospective review of all incoming National Collegiate Athletic Association Division-I athletes at a single institution for the 2013-2014 academic year was performed. Two pediatric cardiologists independently reviewed all TTE studies for right coronary artery (RCA) and left coronary artery (LCA) ostia, proximal course, and measurable course. Proximal course was defined as a measurable course >1 cm for the RCA and a measurable course >1 cm or observation of the bifurcation for the LCA. Measurable course was defined as the maximum continuously observable distance of coronary artery from a clearly delineable ostium. Results were compared among athlete characteristics, and interobserver reliability was evaluated. RESULTS: Chart review identified 146 athletes, representing 22 sports and 6 ethnicities. No coronary anomalies were found. The average measurable course was 2.1 cm for the RCA and 1.5 cm for the LCA. Both readers observed the RCA ostium in 143 athletes (98%), LCA ostium in 143 athletes (98%), RCA proximal course in 119 athletes (82%), and LCA proximal course in 118 athletes (81%). Statistical analysis showed good interobserver reliability for observation of the ostia and proximal course. CONCLUSIONS: The origin and proximal course of the coronary arteries were readily and reliably observed in the majority of athletes, suggesting coronary artery evaluation should be included in athlete screening TTE protocols.

Congenital Heart Disease and the Athlete: What We Know and What We Do Not Know.

Since outcomes for patients with congenital heart disease (CHD) have greatly improved, most patients with CHD are surviving into adulthood and creating dilemmas for practitioners with regard to competitive sports participation. Much time, effort, and expertise have gone into developing the new American Heart Association/American College of Cardiology's guidelines and the European Society of Cardiology's guidelines. Practitioners should consult the guidelines but also be aware of gaps in the literature and should individualize recommendations for each patient. Both participation and restriction decisions should be made thoughtfully and collaboratively with athletes as either decision carries important consequences."

Endurance Training on Congenital Valvular Regurgitation: An Athlete Case Series.

BACKGROUND: Both intense endurance training and valvular regurgitation place a volume load on the right and left ventricles, potentially leading to dilation, but their effects in combination are not well-known. PURPOSE: The purpose of this case series is to describe the combined volume load of intense endurance athletic training and regurgitant valvular disease as well as the challenging assessment of each component's cardiovascular effect. METHODS: In this article, the clinical course of three elite endurance athletes with congenital valvular disease were reviewed. RESULTS: A swimmer with aortic regurgitation, a cyclist with aortic regurgitation, and a cyclist with pulmonary regurgitation were found to have severe dilation of the associated ventricles despite continuing to train at an elite level without symptoms. CONCLUSIONS: Because of the cumulative effects of endurance training and valvular regurgitation, each athlete manifested ventricular dilation out of proportion to their valvular disease and symptoms. Although the effects of congenital valvular disease and athletic remodeling on ventricular dilation have been thoroughly studied individually, their cumulative effect is not well understood. This complicates the assessment of athletes with valvular regurgitation and underscores the need for athlete-specific recommendations for valve replacement.

The Historical Perspective of Athletic Sudden Death.

Since antiquity, the athlete has been elevated to a heroic status both within small communities and at the international level. Although numerous population studies have estimated athletic sudden death to be a rare event, the consequences resonate far beyond those directly affected. Sports cardiology has evolved as a result of these tragedies, which highlighted a need for safer play and more programmatic protection of the athlete in play. In this article, athletic sudden death is analyzed from a historical and literary perspective and the development of modern initiatives to protect athletes from sudden death is reviewed.

Sports participation and quality of life in adolescents and young adults with congenital heart disease.

BACKGROUND: Adolescents and young adults with congenital heart disease (CHD) are often restricted from physical activity and sports participation, which may have adverse effects. OBJECTIVES: To determine the amount of physical activity, type of sports participation, and reasons for sports restrictions, and to evaluate the effect of sports participation on quality of life (QoL) in a cohort of patients with CHD. METHODS: Individuals with CHD aged 13-30 years were recruited at outpatient visits or via mailings. They completed a questionnaire addressing physical activity, sports participation, sports restrictions, and QoL (Pediatric Quality of Life Inventory). We also reviewed the patient's medical record. RESULTS: Of the 177 patients who responded (mean age 20 years), 31% have mild CHD, 40% have moderate CHD, and 29% have severe CHD. In the cohort, 52% participate in competitive sports, 25% recreational sports, and 23% no sports. Among patients with severe CHD, 29% participate in competitive sports that would be restricted by published guidelines (36th Bethesda Conference). After controlling for age, sex, CHD severity, residual hemodynamic disease, and comorbidities, participation in competitive sports and increased frequency of physical activity are independently associated with a higher QoL (P = .003 and P = .001, respectively). In an identical model, competitive sports participation and frequency of physical activity are associated with higher maximum predicted oxygen consumption (VO2 ) (n = 40; P = .002 and .02) and slightly lower body mass index (BMI) (P = .02 and .01). All findings were similar when analyses were stratified by recruitment method. CONCLUSIONS: Patients with CHD commonly participate in competitive sports, and such participation is associated with higher QoL, improved exercise capacity, and lower BMI.

Effects of race, ethnicity, and gender on surgical mortality in hypoplastic left heart syndrome.

Information is limited regarding the effect of race, ethnicity, and gender on the outcomes of the three palliative procedures for hypoplastic left heart syndrome (HLHS). This study examined the effects of race, ethnicity, gender, type of admission, and surgical volume on in-hospital mortality associated with palliative procedures for HLHS between 1998 and 2007 using data from the University HealthSystem Consortium. According to the data, 1,949 patients underwent stage 1 palliation (S1P) with a mortality rate of 29 %, 1,279 patients underwent stage 2 palliations (S2P) with a mortality rate of 5.4 %, and 1,084 patients underwent stage 3 palliation (S3P) with a mortality rate of 4.1 %. The risk factors for increased mortality with S1P were black and "other" race, smaller surgical volume, and early surgical era. The only risk factors for increased mortality with S2P were black race (11 % mortality; odds ratio [OR], 3.19; 95 % confidence interval [CI] 1.69-6.02) and Hispanic ethnicity (11 % mortality; OR 3.30; 95 % CI 1.64-6.64). For S2P, no racial differences were seen in the top five surgical volume institutions, but racial differences were seen in the non-top-five surgical volume institutions. Mortality with S1P was significantly higher for patients discharged after birth (37 vs 24 %; p = 0.004), and blacks were more likely to be discharged after birth (12 vs 5 % for all other races; p < 0.001). No racial differences with S3P were observed. The risk factors for increased mortality at S1P were black and "other" race, smaller surgical volume, and early surgical era. The risk factors for increased in-hospital mortality with S2P were black race and Hispanic ethnicity.

Stent angioplasty to relieve left pulmonary artery obstruction caused by patent ductus arteriosus device occlusion: bipartisan teamwork by two interventional devices.

A 7-month-old patient in congestive heart failure due to a moderate sized patent ductus arteriosus (PDA) underwent uncomplicated implantation of an Amplatzer Ductal Occluder (ADO1). Two months after percutaneous device PDA closure, left pulmonary artery (LPA) stenosis was discovered. Rather than spontaneous improvement as reported in previous cases, our patient's LPA stenosis progressed in severity 7 months after ADO1 placement. A catheterization demonstrated a 32 mm Hg peak gradient from her main pulmonary artery to her LPA. She underwent successful stent angioplasty of her LPA with an excellent result and preserved PDA closure. This case demonstrates that stent angioplasty is a feasible an effective method of relieving LPA obstruction caused by a PDA occluder device. Additionally, despite slight deflection by the stent, the ADO1 device continued to provide complete ductal closure. Stent angioplasty should be considered in patients who have LPA stenosis caused by ADO1 occluder device that does not improve over time.

Cryoablation and angiographic evidence of a concealed right atrial appendage to right ventricle accessory pathway in an infant.

We report a case of successful cryoablation of a concealed accessory pathway from the right atrial appendage to the right ventricle in an infant. A 4-month-old female who had previously undergone successful ablation of a left-sided accessory pathway was taken to the electrophysiology lab due to recurrent supraventricular tachycardia. While in this second supraventricular tachycardia, we found earliest atrial activation in the distal right atrial appendage. Angiography confirmed a connection between right atrial appendage and the right ventricle. Cryoablation at this location promptly terminated the supraventricular tachycardia. Following ablation, there were no further inducible arrhythmias and ventriculo-atrial dissociation was appreciated.

Inpatient costs and charges for surgical treatment of hypoplastic left heart syndrome.

OBJECTIVE: Hypoplastic left heart syndrome (HLHS) is one of the most serious congenital cardiac anomalies. Typically, it is managed with a series of 3 palliative operations or cardiac transplantation. Our goal was to quantify the inpatient resource burden of HLHS across multiple academic medical centers. METHODS: The University HealthSystem Consortium is an alliance of 101 academic medical centers and 178 affiliated hospitals that share diagnostic, procedural, and financial data on all discharges. We examined inpatient resource use by patients with HLHS who underwent a staged palliative procedure or cardiac transplantation between 1998 and 2007. RESULTS: Among 1941 neonates, stage 1 palliation (Norwood or Sano procedure) had a median length of stay (LOS) of 25 days and charges of $214,680. Stage 2 and stage 3 palliation (Glenn and Fontan procedures, respectively) had median LOS and charges of 8 days and $82,174 and 11 days and $79,549, respectively. Primary neonatal transplantation had an LOS of 87 days and charges of $582,920, and rescue transplantation required 36 days and $411,121. The median inpatient wait time for primary and rescue transplants was 42 and 6 days, respectively. Between 1998 and 2007, the LOS for stage 1 palliation increased from 16 to 28 days and inflation-adjusted charges increased from $122,309 to $280,909, largely because of increasing survival rates (57% in 1998 and 83% in 2007). CONCLUSIONS: Patients with HLHS demand considerable inpatient resources, whether treated with the Norwood-Glenn-Fontan procedure pathway or cardiac transplantation. Improved survival rates have led to increased hospital stays and costs.