Samuel Hanke

Left Ventricular Noncompaction Cardiomyopathy in Barth Syndrome: An Example of an Undulating Cardiac Phenotype Necessitating Mechanical Circulatory Support as a Bridge to Transplantation

Barth syndrome (BTHS) is associated with myocardial disease, frequently left ventricular noncompaction cardiomyopathy, which may necessitate cardiac transplantation or lead to death in some patients. We report a child with BTHS who had an “undulating cardiac phenotype” and ultimately developed decompensated heart failure requiring mechanical circulatory support with a ventricular assist device as a bridge to transplantation. His course was complicated by acute lung injury requiring placement of an in-line oxygenator to maintain end-organ function. Not only was his course complicated by cardiac and respiratory failure but his BTHS associated comorbidities complicated the management of his therapy using mechanical assist device support. He was successfully supported and subsequently was transplanted. Here we discuss the management of a child with BTHS using mechanical circulatory support and describe the use of an in-line oxygenator, Quadrox, with the Berlin Excor device.

Factors associated with long-term mortality after Fontan procedures: a systematic review.

Background Despite an ageing Fontan population, data on late outcomes are still scarce. Reported outcome measures and determinants vary greatly between studies making comprehensive appraisal of mortality hazard challenging. Methods We conducted a systematic review to evaluate causes and factors associated with late mortality in patients with Fontan circulation. Late mortality was defined as mortality beyond the first postoperative year. Studies were included if they had ≥90 patients or ≥20 late mortalities and/or transplants. Studies with overlapping patients were rationalised to include only the most recent studies to avoid duplication. Results From 28 studies, a total of 6707 patients with an average follow-up time of 8.23±5.42 years was identified. There were 1000 deaths. Causes of late death were reported in 697 cases. The five most common causes were heart/Fontan failure (22%), arrhythmia (16%), respiratory failure (15%), renal disease (12%) and thrombosis/bleeding (10%). Factors associated with late mortality were evaluated and classified into 9 categories. Conclusions Causes and factors associated with late mortality after the Fontan operation are summarised in this study. The presented information will aid in identifying patients at highest risk for mortality and guide our risk stratification efforts in this patient population.

Digoxin Use Is Associated With Reduced Interstage Mortality in Patients With No History of Arrhythmia After Stage I Palliation for Single Ventricle Heart Disease

Background Interstage mortality (IM) remains significant after stage 1 palliation (S1P) for single‐ventricle heart disease (SVD), with many deaths sudden and unexpected. We sought to determine whether digoxin use post‐S1P is associated with reduced IM, utilizing the multicenter database of the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC). Methods and Results From June 2008 to July 2013, 816 infants discharged after S1P from 50 surgical sites completed the interstage to stage II palliation, transplant, or IM. Arrhythmia during S1P hospitalization or discharge on antiarrhythmic medications were exclusions (n=270); 2 patients were lost to follow‐up. Two analyses were performed: (1) propensity‐score adjusted logistic regression with IM as outcome and (2) retrospective cohort analysis for patients discharged on digoxin versus not, matched for surgical site and other established IM risk factors. Of 544 study patients, 119 (21.9%) were discharged on digoxin. Logistic regression analysis with propensity score, site‐size group, and digoxin use as predictor variables showed an increased risk of IM in those not discharged on digoxin (odds ratio, 8.6; lower confidence limit, 1.9; upper confidence limit, 38.3; P<0.01). The retrospective cohort analysis for 60 patients on digoxin (matched for site of care, type of S1P, post‐S1P ECMO use, genetic syndrome, discharge feeding route, ventricular function, tricuspid regurgitation, and aortic arch gradient) showed 0% IM in the digoxin at discharge group and an estimated IM difference between the 2 groups of 9% (P=0.04). Conclusions Among SVD infants in the NPCQIC database discharged post‐S1P with no history of arrhythmia, use of digoxin at discharge was associated with reduced IM.

Predicting long-term mortality after Fontan procedures: A risk score based on 6707 patients from 28 studies

BACKGROUND Reported long-term outcome measures vary greatly between studies in Fontan patients making comprehensive appraisal of mortality hazard challenging. We sought to create a clinical risk score to assist monitoring of Fontan patients in the outpatient setting. METHODS A systematic review was conducted to evaluate risk factors for long-term (beyond the first postoperative year) mortality in Fontan patients. Studies were eligible for inclusion if ≥90 patients were included or ≥20 long-term mortalities we reported. Risk factors for long-term mortality were determined. The pooled hazard ratios were used to create components of a clinical score for long-term mortality using meta-analysis techniques. RESULTS Twenty-eight studies were included. The total number of patients was 6707 with an average follow-up of 8.23 ± 5.42 years. There were 1000 deaths. Thirty-five risk factors for late mortality were identified and classified into 9 categories and their relative hazards were used to derive the initial components of a weighted, practical and clinically based Fontan risk score (ranging from 0 to 100). The final score included 8 risk factors: anatomic risk factors, elevated preoperative pulmonary artery pressure, atriopulmonary Fontan, heart failure symptoms, arrhythmia, moderate/severe ventricular dysfunction or atrioventricular valve regurgitation, protein losing enteropathy, and end organ disease (cirrhosis or renal insufficiency). CONCLUSION In patients with Fontan circulation, the influence of readily available risk factors can be quantified in an integer score to predict long-term mortality. Prospective validation and refinement of this risk score will be undertaken.

A 14-Year-Old Boy with Unusual Presentation of Respiratory Distress

There are multiple cardiac etiologies for wheezing and respiratory distress which require a high degree of suspicion for the pediatrician to diagnose. We present a case of a patient with a history of long-standing mild persistent asthma with minimal improvement on controller and bronchodilator therapies who presented to the emergency room with acute respiratory distress. When he demonstrated a lack of improvement with traditional respiratory therapies, additional etiologies of respiratory distress were considered. Ultimately an echocardiogram was performed, which revealed the diagnosis of cor triatriatum. He underwent surgical resection of his accessory membrane and has had no additional symptoms of asthma since repair.

Chapter 36 – Quality Metrics and Quality Improvement in Pediatric Heart Failure

Abstract In the 2000 report, Crossing the Quality Chasm, the Institute of Medicine broadly outlined the changes needed to reinvent the delivery of health care in the United States. This report highlighted the limitations of traditional scientific discovery methods to translate knowledge into consistent, high-quality medical care. Since the release of this report, the health care industry has seen an increased application of quality improvement methods developed by Dr. Walter Shewhart and implemented with great success by W. Edwards Deming in the manufacturing industries. This systems-based approach is predicated on the concept of learning from sequential changes and establishing objective measures to assess if those changes result in improvement (Langley, 2009).

STANDARDIZING THE CARE OF OUTPATIENT PEDIATRIC SYNCOPE AND DIZZINESS: A LESSON IN UTILIZATION AND CHARGE REDUCTION

Background: Variability in medical practice is associated with higher cost without improved outcome. Our aim was to standardize the evaluation and treatment of dizziness/syncope and track resultant cost savings. Methods: A multidisciplinary team developed a care algorithm using best evidence and

Risk Factors for Unanticipated Readmissions During the Interstage: A Report From the National Pediatric Cardiology Quality Improvement Collaborative

This study describes unanticipated interstage readmissions in patients with hypoplastic left heart syndrome, identifies independent risk factors for unanticipated interstage readmissions, and evaluates variation in unanticipated readmission rates among collaborative centers. Retrospective data of patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from July 2008 to July 2013 were analyzed. Risk factors present at the beginning of the interstage were captured. Competing risks time to event analyses determined the association between these factors and unanticipated interstage readmission. Readmission center variation was examined using funnel plots. Unanticipated interstage readmissions occurred in 66% of 815 patients at 50 centers. The median readmission length of stay was 2 days (interquartile range: 0-6) and median time to first readmission was 29 days (interquartile range: 9-63). Most readmissions were prompted by minor changes in clinical status (64%), whereas only 6% were major adverse event readmissions. Independent readmission risk factors included genetic syndrome (HR = 1.40, 95% CI: 1.05-1.88), center volume (small vs large HR = 1.32, CI: 1.04-1.66, medium vs large HR = 1.35, CI: 1.09-1.68), preoperative ventricular dysfunction (HR = 2.02, CI: 1.31-3.10), tricuspid regurgitation (HR = 1.36, CI: 1.08-1.72), duration of circulatory arrest (HR = 0.99, CI: 0.989-0.998), and undergoing Hybrid procedure relative to Norwood/right ventricle to pulmonary artery conduit (HR = 1.40, CI: 1.02-1.93). There was significant center variation in the number of readmissions and duration of readmissions. Unanticipated readmissions are common during the interstage period with notable center variation. However, these readmissions are short and are rarely in response to major adverse events.