These posters have been presented at meetings in Children's Mercy and around the world. They represent research that was done at the time they were created, and may not represent medical knowledge or practice as it exists at the time viewers access these posters.
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Knowledge Base of Adolescents with Congenital Heart Disease
Mark Gelatt, Julie Martin, Jennifer A. Marshall, Jennifer Panuco, Jenea Schmidt, and Pamela Finn
Introduction:
Most congenital heart disease [CHD] is diagnosed and treated in early childhood with parents responsible for decision-making. The adolescent assumes this role in preparation for transition to an adult congenital heart program. We studied the knowledge base of our adolescent CHD patients and their parents.
Methods:
Established CHD patients, >11 y.o. and their parents, were independently surveyed in the outpatient clinic. Participation was voluntary. Cardiomyopathy, electrophysiology and transplant patients were excluded. Scores were assessed as full, partial or incomplete.
Results:
Most (98% parents; 83% adolescents) reported that their cardiologist had provided education. Adolescents provided a full (49%) and partial (15%) medical name and full (32%) and partial (35%) description of their condition. Older adolescents performed better (p<0.005). Parents scored better, regardless of patient age (p<0.005). Presence of prior surgery/intervention made no difference. Patients undergoing last surgery >11 y.o. scored better than those with surgery only when younger or without surgery (p<0.05) [data not shown]. Patients with mild unrepaired or surgically repaired shunt lesions (ASD, VSD, PDA), and their parents, were much less able to name their lesion than those with left heart lesions (aortic stenosis, coarctation, mitral valve disease), tetralogy of Fallot, TGA/TAPVR or palliated single ventricles, but similar in ability to at least partially describe the defect (p<0.005). In this more affected population, the ability to fully name the defect (81/134; 60%) far exceeded the ability to fully describe it (37/134; 28%; p<0.005).
Conclusion:
Only one-half of adolescents are able to name their cardiac defect, and one-third are able to adequately describe the anatomy. Older adolescents, those with a more recent history of surgery and those with more complex defects perform better. Parents are much more knowledgeable than their children. A strong emphasis should be made on educating adolescents with CHD as they transition to adulthood.
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Knowledge Gap in Adolescent Heart Transplant Recipients
Pamela Finn, Brian Birnbaum, Audrey R. Kennedy, Jennifer A. Marshall, and Mark Gelatt
Background:
Studies have reported a knowledge gap Studies have reported a knowledge gap among adolescent and young adult heart heart transplant patients upon transitioning to adult cardiology practices.
This population is vulnerable to life-threatening complications as they progress to adult care.
Framework/Methods:
We assess the current knowledge deficit of our adolescent and young adult patients and their caregivers in order to inform pediatric providers of their unique needs as they prepare to transition.
Heart transplant patients >10 yrs and their caregivers completed a written questionnaire prior to a clinic visit. 17 questions were asked including the need for transplant, medication names, purposes and adverse effects and the effects of living with a transplanted heart on exercise and work choices.
Patients were all transplanted in another facility and initial education occurred at that facility.
Conclusions:
Genetic factors and exercise/work limitations were the most misunderstood.
Our patients and caregivers also lacked a clear understanding of medications and common and/or serious adverse effects.
Areas that were well understood included: why the transplant was needed, meaning of immunosuppression, and the need for lifelong follow-up.
Recently our facility was granted permission to perform heart transplants and it will be our responsibility to initially and fully educate the patient and caregivers. A comprehensive team including a pharmacist, dietician, genetic counselor, and psychologist, in addition to nurses and physicians has been formed and they will be knowledgeable of ongoing needs with the intent to close the knowledge gap.
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B-Type Natriuretic Peptide (BNP) Levels During Extracorporeal Membrane Oxygenation (Ecmo) Weaning May Predict Survival To Hospital Discharge
John Graham, Jennifer Flint, and Erica Molitor-Kirsch
Background: Despite apparent clinical and echocardiographic evidence of adequate myocardial function and successful weaning towards decannulation, up to 33% of children who separate from cardiac ECMO do not survive to hospital discharge. Brain natriuretic peptide (BNP) is used as a marker of myocardial dysfunction in multiple clinical settings. We have anecdotally observed that during ECMO weaning, BNP levels remain stable or decreased in patients who survive after decannulation, but are elevated or rise in other children who die following decannulation.
Hypothesis: We hypothesized that trends in BNP levels during weaning from V-A ECMO may be useful to predict survival after decannulation from ECMO.
Methods Design: Retrospective cohort study Participants: Patients requiring V-A ECMO for cardiac failure admitted to Children’s Mercy Hospital PICU from May 2011-Feb 2014 who had BNP levels while on ECMO were included Measurements: The BNP level on full ECMO support and the BNP level on the lowest level of ECMO support prior to decannulation were recorded. Mann-Whitney U was used to compare BNP levels between those who died within 48 hours of decannulation, those who survived >48 hours off ECMO, and those who survived to hospital discharge.
Results: Twelve patients met inclusion criteria. Eleven had congenital heart disease, 7 had single ventricle physiology. Median age was 57.5 days (range 10-2237). Median weight was 3.85kg (range 2.7-28). Four patients survived >48hrs after decannulation (33.3%) and 2 patients survived to hospital discharge (16.7%). Among non survivors, median time to death after decannulation was 2.13 hours (range 0.13-1601). Survivors had a decrease or no change in their BNP level with weaning of ECMO support. Of non survivors, 57% had an increase BNP level or a BNP level that remained >5,000 pg/mL with ECMO weaning. At the time of minimal ECMO support, there was a trend towards lower median BNP levels in those who survived greater than 48 hours (314, 153-655) vs. those who died within 48 hours of decannulation (2671, 199-5001), p=0.088. There was a stronger trend towards lower BNP levels at the time of minimal ECMO support in those who survived to hospital discharge (190, 153-227) compared to all those who died (1174, 199-5001), p=0.059. STUDY LIMITATIONS: Our study was limited by the retrospective study design, small sample size, and the inability to quantify BNP levels >5,000 pg/mL.
Conclusion: BNP may be a useful biomarker to assess myocardial function while weaning ECMO support and warrants further study.
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Do the Best Hospitals Have Librarians?
Brenda R. Pfannenstiel
Objective: Determine how the presence of medical librarians correlates with hospital ranking on the U.S. News & World Report best hospitals list.
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Ahead of Their Time: The Story of Alice Berry Graham and Katharine Berry Richardson, the Founders of Children's Mercy Hospital in Kansas City
Jane F. Knapp MD and Robert Schremmer
Describes the founding of Children's Mercy Hospital through the story of its founders, Katharine Berry Richardson and Alice Berry Graham.
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The Orphan Train Movement and its Influence on Child Welfare Policy in Kansas
Robert Schremmer and Jane F. Knapp MD
The Orphan Train Movement was responsible for relocating thousands of children from large eastern cities to rural areas and can be seen as the forerunner to today's foster care system.
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B-type Natriuretic Peptide (BNP): A Potential Biomarker for Extubation Failure in Infants Following Cardiac Surgery
Jennifer Flint, Lori Erickson, Dawn Tucker, and Erica Molitor-Kirsch
Background: BNP is a hormone released from the cardiac ventricles in response to increased pressure and volume overload1 and is an important biomarker in heart failure. Following congenital heart surgery, elevated BNP levels correlate with longer duration of mechanical ventilation, low cardiac output syndrome, and increased ICU length of stay2-4 Mechanical ventilation (MV) has an exaggerated impact on cardiopulmonary interactions in children with myocardial dysfunction, and extubation readiness can be difficult to determine post-operatively following congenital heart surgery.
Hypothesis: An increase in post-extubation BNP levels can predict extubation failure and the need for reintubation within 48 hours.
Methods:
Design: prospective, observational, blinded pilot Participants: Infants ≤ 30 days of age with RACHS-15 score ≥3 admitted to the PICU following congenital heart surgery
Measurements: BNP levels were obtained on full MV just prior to weaning per standardized weaning protocol, one hour following a pressure support trial (PST), and at 2, 6, and 12 hours following extubation. Inotropic scores6 were calculated at each interval.
Conclusions:
1.Patients who failed extubation had a trend towards higher BNP levels compared to those who did not fail extubation
2.BNP levels increased in all patients with MV weaning and following extubation
3.Inotropic scores did not correlate with BNP values
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B-type Natriuretic Peptide (BNP): A Potential Biomarker for Extubation Failure in Infants Following Cardiac Surgery
Jennifer Flint, Lori Erickson, Dawn Tucker, and Erica Molitor-Kirsch
Background: BNP is a hormone released from the cardiac ventricles in response to increased pressure and volume overload1 and is an important biomarker in heart failure. Following congenital heart surgery, elevated BNP levels correlate with longer duration of mechanical ventilation, low cardiac output syndrome, and increased ICU length of stay2-4 Mechanical ventilation (MV) has an exaggerated impact on cardiopulmonary interactions in children with myocardial dysfunction, and extubation readiness can be difficult to determine post-operatively following congenital heart surgery.
Hypothesis: An increase in post-extubation BNP levels can predict extubation failure and the need for reintubation within 48 hours.
Methods:
Design: prospective, observational, blinded pilot Participants: Infants ≤ 30 days of age with RACHS- 15 score ≥3 admitted to the PICU following congenital heart surgery
Measurements: BNP levels were obtained on full MV just prior to weaning per standardized weaning protocol, one hour following a pressure support trial (PST), and at 2, 6, and 12 hours following extubation
Conclusions:
1. Patients who failed extubation had a trend towards higher BNP levels compared to those who did not fail extubation
2. BNP levels increased in all patients with MV weaning and following extubation
3. Single ventricle patients had higher BNP
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Librarians Collaborate to Touch the Lives of Patients through Community Pediatricians
Keri Swaggart and Nancy Allen
MCMLA 2012 Annual Meeting Presentation
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Symptomatic Hypocalcemia During Urinary Alkalinization for Acute Aspirin Toxicity
Jennifer Flint and Bruce Banwart
A16y/o ingested 165 tablets of 325 mg ASA(1000 mg/kg). He presented to an outside facility 8 hours after ingestion alert with stable vital signs and ASA level 78 mg/dL. Initial lab values showed an arterial blood gas (ABG) pH 7.46, pCO2 26 mmHg, HCO3 18 mmol/L, ionized calcium 1.09 mmol/L, serum calcium 10 mg/dL, albumin 4.5 gm/dL, magnesium 2.1 mg/dL. Urinary alkalinization was initiated with a sodium bicarbonate infusion (NaHCO3) and he arrived to our facility 12 hours after ingestion with Kussmaul respirations and altered mentation. Repeat ASA 116mg/dL, ionized calcium 0.88 mmol/L and serum calcium 8.8 mg/dL. He became encephalopathic with marked hyperpnea and diaphoresis. He was not intubated due to the risk of impairing his respiratory drive and lowering his pH. Jaw thrust maneuvers were provided to maintain his airway. A dialysis catheter was placed with out sedation and dialysis was initiated. He developed bradycardia, hypotension, ST segment depression, and ventricular dysrhythmias. He required 5 liters of fluid, 3.5 gms calcium chloride, 175 mcg (3.4mcg/kg) intermittent bolus epinephrine and continuous infusions of dopamine (10 mcg/kg/min), epinephrine (0.12 mcg/kg/min), and norepinephrine (0.05 mcg/kg/min). After 3 hours of dialysis, ASA level 49 mg/dL, vital sign sstabilized, ionized calcium normalized, vasopressors were weaned off, and neurological status returned to baseline. To our knowledge, this is the first case report of urinary alkalinization leading to low ionized calcium levels with associated hemodynamic instability and dysrhythmias. Altering the serum pH during urinary alkalinization can alter the availability of ionized calcium. Urinary calcium loss may be enhanced by the excretion of the sodium load from a NaHCO3 infusion due to inhibition of calcium reabsorption in the proximal and late distal tubule, contributing to total calcium losses. The degree of insensible losses and volume depletion can be under appreciated in the setting of acute aspirintoxicity. Hypocalcemia in combination with volume depletion and rapid volume shifts seen with initiation of dialysis can lead to significant hemodynamic instability.
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The Life and Times of a Kansas Horse and Buggy Doctor and His Recollections on the Care of Children
Robert D. Schremmer MD and Jane F. Knapp MD
Describes the career of Arthur Emmanuel Hertzler, MD, 1870-1946, who practiced in Halstead, Kansas.
