Pacing in Pediatric Patients with Postoperative Atrioventricular Block.

Surgery for congenital heart disease may compromise atrioventricular (AV) nodal conduction, potentially resulting in postoperative AV block. In the majority of cases, AV nodal function recovers during the early postoperative period and may only require short-term pacing support, typically provided via temporary epicardial wires. Permanent pacing is indicated when the postoperative AV block persists for more than 7 to 10 days due to the risk of mortality if a pacemaker is not implanted. Although there is a subset of patients who may have late recovery of AV nodal function, those with continued postoperative AV block will need lifelong pacing therapy.

Preparticipation State Cardiac Screening Forms for Athletes.

BACKGROUND AND OBJECTIVES: Annual preparticipation physical evaluation (PPE) is used in the United States to screen adolescents for potential causes of sudden cardiac death. The American Heart Association recommends 14 screening elements of history and physical examination. This study sought to define the utilization of these screening elements by each of the 50 states before high school athletics. METHODS: PPE forms were obtained from the public website of the high school athletics governing body in every state. Form content was analyzed to identify which of the 14 screening elements were explicitly fulfilled. Additional PPE forms provided by private/parochial schools, other professional societies, or independent groups were excluded from this study. RESULTS: A total of 48 states (96%) had PPE forms posted online. The remaining 2 states (4%) deferred the specific method of PPE documentation to individual school districts and provided no standardized form. Of the 48 states providing PPE forms, 13 (27%) included all 14 American Heart Association screening elements. The median criteria included by each state was 11 (range 3-14). The 3 criteria most commonly absent were (1) the examination of femoral pulses to exclude coarctation (58%), (2) a family history of specific inherited cardiac disease (31%), and (3) personal history of hypertension (27%). CONCLUSIONS: Annual preparticipation forms are important screening tools. Only a minority of states include all 14 cardiac screening elements recommended by the American Heart Association.

Interdisciplinary care of children with trisomy 13 and 18.

Children with trisomy 13 and 18 (previously deemed "incompatible with life") are living longer, warranting a comprehensive overview of their unique comorbidities and complex care needs. This Review Article provides a summation of the recent literature, informed by the study team's Interdisciplinary Trisomy Translational Program consisting of representatives from: cardiology, cardiothoracic surgery, neonatology, otolaryngology, intensive care, neurology, social work, chaplaincy, nursing, and palliative care. Medical interventions are discussed in the context of decisional-paradigms and whole-family considerations. The communication format, educational endeavors, and lessons learned from the study team's interdisciplinary care processes are shared with recognition of the potential for replication and implementation in other care settings.

Williams Syndrome and Neonatal Cardiac Surgery for Congenital Single Ventricle.

Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe double-outlet right ventricle with mitral atresia and aortic arch hypoplasia in an infant with WS. This case demonstrates the difficulty in managing patients with WS with complex cardiac defects. To our knowledge, this is the first reported single-ventricle physiology in a patient with WS. (Level of Difficulty: Advanced.).

Tilt table testing to diagnose pseudosyncope in the pediatric population.

OBJECTIVE: Pseudosyncope can be difficult to distinguish from true syncope. Often, pediatric patients with pseudosyncope undergo multiple tests and referrals before the appropriate diagnosis is reached. The purpose is to describe the utility of the head-up tilt table test to elicit the diagnosis of pseudosyncope in the pediatric population. DESIGN: Retrospective chart review from November 2012 to December 2015 of patients age ≤23 years referred for 30-minute, 80-degree tilt table test. Pretest probability for pseudosyncope was high if there was no response to traditional management, atypical episodes, occurrence during undesirable exercise, or prolonged episode duration. Inductive techniques were utilized to persuade patients of the likelihood of experiencing an episode during the procedure. Pseudosyncope was confirmed when a patient had normal vital signs during their event and had reflex responses to disruptive maneuvers. RESULTS: Tilt table testing was performed on 89 patients [median age 16 years (5-23); 26% male] with the majority (60%) being negative for pseudosyncope, including 51 true negatives and 2 false-negatives. Of the 36 patients with syncope during tilt table testing, 28 were diagnosed with vasovagal syncope and 8 with pseudosyncope [median age 16 years (15-21); 38% male]. Pseudosyncope episodes were observed immediately in 2 patients. All patients with late-onset pseudosyncope required inductive techniques prior to the recorded episode. CONCLUSIONS: Pseudosyncope can be identified during tilt table testing if inductive techniques are utilized in patients with a high index of suspicion. Disruptive maneuvers are excellent adjunctive methods to confirm the diagnosis. Tilt table testing is an effective means to identify pseudosyncope and allow appropriate diagnosis and treatment.

Adolescent Presents With Altered Mental Status and Elevated Anion Gap After Suicide Attempt by Ethylene Glycol Ingestion.

We report the case of a 16-year-old healthy adolescent male who presented to the local emergency department with altered mental status. En route to a tertiary care facility, he began to decompensate and was found to be markedly acidotic. Further investigation revealed an elevated anion gap, and physical examination showed only abdominal pain and decreased level of consciousness. A broad differential diagnosis was considered at the time of the patient's presentation at the tertiary care center including ingestion of a volatile alcohol, sepsis, and an abdominal catastrophe. Although fomepizole and emergent dialysis were being initiated, laboratory tests confirmed ethylene glycol poisoning. This case demonstrates the importance of early recognition of potential ingestions in patients with altered mental status and supportive laboratory findings.

Duration and magnitude of vasopressor support predicts poor outcome after infant cardiac operations.

BACKGROUND: The vasoactive inotrope score (VIS) is a sum of the total vasopressor dose at a single point in time. Incorporating duration and magnitude of vasopressor requirements during the postcardiac surgical period could improve VIS sensitivity for predicting poor outcome. METHODS: This is a retrospective review of 244 infants (aged ≤365 days) who underwent cardiopulmonary bypass during congenital cardiac operations from 2002 to 2011. The VIS was calculated hourly for the first 72 hours. Poor outcome was defined as prolonged mechanical ventilation (≥6 days) or intensive care length of stay (≥12 days). First, the association between the maximum VIS (maxVIS) in the first 48 postoperative hours and poor outcome was confirmed for our study population. Next, postoperative intervals and VIS values that were significantly associated with poor outcome were identified and incorporated into a formula, termed the VISindex, which was compared with the traditional maxVIS. RESULTS: The VISindex demonstrated improved sensitivity for predicting prolonged mechanical ventilation (VISindex: area under the curve [AUC], 0.85; 95% confidence interval [CI], 0.79 to 0.90; maxVIS: AUC, 0.80; 95% CI, 0.75 to 0.86) and intensive care unit length of stay (VISindex: AUC, 0.84; 95% CI, 0.79 to 0.89; maxVIS: AUC, 0.77; 95% CI, 0.71 to 0.83) after cardiac operations in infants. CONCLUSIONS: Incorporating magnitude and duration of postoperative vasopressor support into the VIS improves its sensitivity for predicting poor outcome.

Cardiac and multiorgan transplantation for end-stage congenital heart disease.

OBJECTIVE: To report our single-center experience with patients who had cardiac and multiorgan transplantation for end-stage congenital heart disease (CHD). PATIENTS AND METHODS: We reviewed records for all patients with CHD who had undergone heart transplantation at Mayo Clinic, Rochester, Minnesota, from November 1, 1990, through June 30, 2012. Patients with cardiomyopathy were excluded, unless CHD was present. RESULTS: Overall, 45 patients had cardiac transplantation for end-stage CHD (mean age, 26.1±18.4 years; range, 1 month to 65 years). Two patients (4%) had combined heart/liver transplantation; 1 (2%) had heart/kidney transplantation. Six patients (13%) had no previous cardiac operation; the remaining 39 patients had a mean of 3 (range, 1-8) previous cardiac operations. Patient survival (95% CI) at 1, 5, and 10 years was 89% (80%-98%), 89% (80%-98%), and 72% (56%-87%), respectively, while graft survival at 1, 5, and 10 years was 89% (80%-98%), 89% (80%-98%), and 61% (44%-78%), respectively. During the same era, the International Society for Heart & Lung Transplantation reported that survival in patients undergoing transplant for non-congenital diagnoses was 85%, 72%, and 56%, respectively. Over a mean follow-up of 8.7±6.2 years, rejection requiring treatment was documented in 35 patients (78%). Eleven patients (24%) have been diagnosed with neoplasia (8 skin, 1 blood, 1 lymph, and 1 other), and 3 patients (7%) have required retransplantation. Four patients (9%) have developed significant coronary vasculopathy; 1 successfully underwent retransplantation, and 3 died 6, 8, and 14 years after transplantation. CONCLUSION: With appropriate patient selection and posttransplant monitoring, survival has improved for patients with complex end-stage CHD. Multiorgan transplantation is an option for selected patients with CHD.

Pitfalls in diagnosis: suspected anomalous origin of the right coronary artery from the pulmonary artery.

Anomalous coronary arteries are rare in the general population. We report the case of a term neonate who underwent an echocardiogram to evaluate a possible patent ductus arteriosus. Unexpectedly, an apparent anomalous origin of the right coronary artery from the main pulmonary artery was detected by surface 2-dimensional transthoracic echocardiography and color-flow Doppler imaging. Because ventricular size and function were normal, the patient ultimately underwent cardiac catheterization to verify the anatomy before proposed surgery. Angiograms showed that the right coronary artery arose from the left anterolateral portion of the mid-ascending aorta. The patient did not require surgery. This case report illustrates pitfalls that can occur in the diagnosis of coronary artery anomalies.