PDRC: medical — case example
This case was reviewed by the PDRC – medical in 2018 and illustrates the difficulties and challenges that can arise for health care practitioners when caring for paediatric patients.
File: 2017-3272 (PDRC 2018-03)
Age: 14 months
- Coroner’s Investigation Statement
- Medical records from:
- Primary pediatrician
- Hospital A
- Hospital B – from Health System A
- Hospital C – from Health System A
- Report of Post Mortem Examination
- Cardiovascular Pathology Report
- Report of Pediatric Cardiovascular Pathology Consultation
Past medical history
The decedent was a 14-month-old male child who was born with an antenatal diagnosis of borderline left ventricle with critical aortic stenosis and endocardial fibroelastosis (EFE). He underwent balloon dilation of the aortic valve three times and was then palliated with a hybrid procedure (i.e. banding of his pulmonary arteries and stenting of his ductus arteriosus) in December 2015. He was readmitted to a quaternary children cardiac care hospital in June 2016 to complete a biventricular repair consisting of debanding and patch augmentation of his branch pulmonary arteries, excision of the ductal stent, coarctation repair with end-to-end anastomosis, and EFE resection. An adjustable atrial septal defect was left open. His post-operative course was complicated by atrial ectopic tachycardia treated with propranolol, diuretic dependence, vocal cord palsy, and feeding intolerance with gastro-esophageal reflux.
Upon discharge in August 2016, his echocardiogram reported mildly reduced left ventricular function, good right ventricular function, mild tricuspid and mitral regurgitation, and no outflow tract obstruction. His discharge medication included domperidone, omeprazole, ranitidine, furosemide (twice daily), and propranolol. A follow-up appointment with his quaternary care cardiologist was arranged for two months with a sedated echocardiogram. A children’s cardiac care centre (Hospital A - closer geographically to the family) pediatrician was recorded as the primary physician on the discharge note provided to the parents.
Following hospital discharge, the child’s course was marked by recurrent community hospital visits and admission. He was first seen on August 22, 2016 after experiencing an apnea associated with a coughing/choking spell. The parents suctioned thick secretions and the child was near baseline by the time he was seen in the emergency department.
During this presentation, a referral was made to a local pediatrician and all ongoing medical follow-up and coordination of care was transitioned on September 2, 2016. There was no information to indicate that the child’s prior medical records were transferred from his previous providers.
On October 4, 2016, the child was readmitted to the quaternary children’s hospital for a G-tube insertion. A follow-up echocardiogram revealed a dilated left ventricle with mild-moderate dysfunction (ejection fraction = 38-45%), an aortic valve gradient of 28mmHg (peak) and 15mmHg (mean), moderate aortic insufficiency, and mild dilation of the ascending aorta and transverse arch. The child was seen during the hospitalization by the quaternary care cardiologist and one-year follow-up was arranged. Discharge medications remain unchanged and included regular Lasix. The discharge summary was sent only to the child’s Hospital A pediatrician.
Over the subsequent three months, the child had multiple pediatric clinic, Community Care Access Centre (CCAC), and hospital visits/admissions for respiratory distress and wheezing, often associated with upper respiratory tract infection symptoms. Isolated nasal pharyngeal swabs were negative. Symptoms were attributed to bronchiolitis and possibly aspiration syndrome. Treatment included routine use of salbutamol (held sporadically by parents during short periods in which he was asymptomatic) inhaled and systemic corticosteroids, and short courses of oral antibiotics for presumed episodes of pneumonia. Oral feeds were held due to concern of aspiration and ENT consultation (for vocal cord re-assessment) and feeding studies was sought.
Swallowing assessment by Hospital A’s gastro-intestinal and swallowing team recorded no further episodes of spit ups on history and revealed no evidence of penetration or aspiration. The team recommended that it was safe to start oral purees. Medication reconciliation by CCAC and the child’s pediatrician no longer listed furosemide, but it is unclear when it was discontinued.
The child’s final hospital admission was on December 28, 2016 with a diagnosis of bronchiolitis and pneumonia. He was last seen in a pediatric clinic and CCAC nursing on January 23, 2017 with ongoing wheezing.
On January 29, 2017 at 0901 hours, the child was brought to Hospital B with a two-day history of cold symptoms, runny nose and vomiting; fever was not noted. The child was irritable, pale and grey (according to notes from the triage nurse) and had difficulty breathing (not relieved with Ventolin). Initial vitals included tachycardia (heart rate 178), blood pressure of 111/90, respiratory rate of 32, and oxygen saturation of 92-97% in room air. The child’s breathing was distressed with a decrease air entry to the left base. A murmur was audible and capillary refill time was less than two seconds. A left lung consolidation and stable cardiomegaly were seen on chest X-ray. Treatment included Ceftriaxone, Ventolin (x3) nebulizations, epinephrine nebulization, ipratroprium bromide nebulization and dexamethasone. A normal saline intravenous bolus of 20 mL/kg was given. The child remained tachycardic (heart rate 170-180) and had respiratory rates in 40’s. The child was transferred to Hospital A by emergency medical services (EMS).
At 1155 hours, upon arrival at Hospital A, the child was described as being alert with grunting respiration. He was febrile (39.2C) with similar vital signs (heart rate of 192, blood pressure 90/40, and oxygen saturation of 95%). He was given a diagnosis of bronchiolitis/reactive airway disease and given Salbutamol (x2), Atrovent (x2), methylprednisolone, acetaminophen, and magnesium sulfate. Ventolin was repeated every 20 minutes and a 10 mL/kg normal saline bolus was administered.
By 1312 hours, the child was noted to have increased irritability which prompted the parents to request a medical re-evaluation.
At 1354 hours, it was noted that the child was “looking unwell.”
At 1404 hours, blood work revealed a lactate level of 6 and base excess of –9. The child had gasping respirations and poor systemic perfusion. A chest X-ray showed increasing cardiomegaly, vascular congestion, and patchy airspace disease. Cardiology and Intensive Care Unit (ICU) teams were contacted for consultation.
Short, self-limited runs of ventricular tachycardia were seen and a point-of-care ultrasound showed poor ventricular function. The child was transferred to the paediatric ICU where he was started on BiPAP, milrinone 0.3 mcg/kg/min, furosemide, sodium bicarbonate, and calcium. A formal echocardiogram revealed severely dilated and dysfunctional left ventricle (with an ejection fraction of 19%), mild mitral regurgitation, mild aortic stenosis and moderate aortic insufficiency. He had good right ventricular function and an unobstructed aorta arch. Transfer to the Cardiac Critical Care Unit at the quaternary children’s hospital was initiated. Amiodarone (2.5 mg/kg) was given for runs of ventricular tachycardia.
Prior to transfer, the child developed intractable ventricular tachycardia followed by pulseless electrical activity and asystole. Resuscitation attempts were made for 90 minutes but were unsuccessful. The child died at 1829 hours.
Post mortem examination findings
Autopsy results indicated:
- Weight (75th percentile), length (<3rd percentile)
- Pulmonary congestion - bilateral, right greater than left
- Bilateral serous pleural effusions
- Cardiomegaly (weight 157.3 grams – expected 45 grams)
- Aortic stenosis with fusion of right and left coronary cusps
- Left ventricular hypertrophy
- Endocardial fibroelastosis of the left ventricle
- Dilated ascending aorta (2.5 centimetres)
- Dilated main pulmonary artery
- Atrial septal defect (2 x 3 millimetres)
- Double lumen aortic arch. Superior lumen is blind-ended and gives rise to all head and neck vessels. Inferior lumen in continuity with descending aorta with evidence of surgical repair at junction.
- Congestion of gastric mucosa
- Distension of jejunum and large intestine
Pediatric cardiovascular pathology consultation:
Review of the cardiovascular pathology confirmed congenital aortic stenosis with a bicuspid aortic valve and mild residual stenosis. Previous repair showed an unobstructed aortic arch with double lumen configuration. Marked cardiomegaly, extensive endocardial fibroelastosis of the left ventricle, and stigmata of congestive heart failure (pulmonary oedema, pulmonary and hepatic congestion) was also found. Evidence of acute bronchopneumonia of the left lung and right upper lobe was noted.
The cause of death was determined to be acute bronchopneumonia complicating congestive heart failure due to congenital heart disease with marked EFE, post-cardiovascular surgery (remote).
Cause of death:
The decedent was a 14-month-old male child born with complex congenital heart disease and had a clinical course complicated by numerous comorbidities associated with this condition and the resulting surgical interventions. Although he died from bronchopneumonia in the setting of end-stage heart failure, the severity of his evolving heart dysfunction went unrecognized as his symptoms were attributed to upper respiratory tract infections and his other medical conditions (i.e. gastroesophageal reflux disease and vocal cord palsy).
A number of systemic issues were identified that impacted communication and may have influenced this child’s medical course.
At 9.5 months of age, primary care was assumed by a new pediatrician after the child presented with an acute aspiration event. The new pediatrician was intended to provide specialized pediatric care within the child’s immediate community. There had been no prior relationship with this pediatrician as earlier care was provided by a pediatrician from another community, including through to the child’s final, and definitive, heart surgery.
Following his initial consultation, the new pediatrician became the child’s primary medical contact, managing acute issues and coordinating care with numerous specialist referrals and community resources, including CCAC nursing. However, despite the child’s extensive pre-existing medical history, there was no evidence of an official transfer or handover of care; the charts available for review from the new pediatrician contained no prior records. The change in primary provider was also not updated at the quaternary care children’s hospital, as all medical communication from his final visit was sent to the Hospital A pediatrician; only a copy of the parent summary for his last hospitalizations at the children’s hospital was found. As a result, the new pediatrician may not have appreciated the complete historical and clinical context for the child. Updates from the children’s hospital were based on parent communication and, possibly through the electronic child health network. These levels of separation increased the potential for errors, loss of data, and may have compromised opportunities for effective communication and sharing of information.
Following his G-tube insertion, the child presented with persistent increased work of breathing and wheezing. Intercurrent illness, pneumonia, and recurrent aspiration were considered the likely etiologies, in setting of higher risk of reflux and vocal cord palsy, ancillary testing (including nasal pharyngeal swabs and swallowing assessments), were negative.
Despite a long history of known comorbidities, the respiratory symptoms were not seen previously and appeared to be poorly responsive to the medical therapies introduced. It is important to note that Furosemide was no longer included on his medication list following his G-tube insertion, but it is unclear when this was discontinued. When last re-evaluated, his echocardiogram showed mild-to-moderate left ventricular dysfunction, which was considered reassuring, and a one-year follow-up with cardiology was arranged. It is likely, however, that the child’s cardiac status and evolution contributed, at least in part, to his symptoms.
The child was at risk for poor relaxation of his heart muscle such that even if his contractility was adequate, poor relaxation could have made him sensitive to fluid balances and poorly tolerant of discontinuation of his diuretics. A cardiac cause of wheezing, however, was not mentioned in the medical record and therefore, likely not considered or appreciated. Earlier communication with, and reassessment by cardiology may have identified progressive deterioration of his heart function and provided an earlier opportunity for intervention.
The child presented to Hospital B in poor condition and transfer was rapidly initiated to the regional tertiary care center. His transfer was arranged directly through the emergency medical services and he was taken to the emergency department.
Consideration for critical care transport, through Criticall Ontario, would have been fitting for this situation. His early treatment, both at Hospital B and Hospital A centered on diagnosis of reactive airway disease/bronchiolitis; the possibility of cardiac origin for his condition was raised only later by the consulting ICU team. He deteriorated despite institution of appropriate therapy. It is unclear if earlier recognition and transfer to quaternary care center would have impacted his outcome.
It was also noted that the cardiology team of Hospital A, despite being the regional provider, were not previously aware of, or provided care to the child prior to his final decompensation. This did not impact the child’s care or his final outcome.
To Hospitals A and B:
- Hospital A and Hospital B emergency departments should conduct a lessons-learned case review of the care and management of the child. The review should include the Chiefs of Emergency Department in each hospital, physicians and nursing staff involved in the care, and the primary physician at Hospital B who was providing ongoing care. The review should focus on:
- Wheezing in a child with congenital heart disease
- Use of diuretics in child with congenital heart disease
- Role for Criticall for the transport of acutely ill children
To the regional chair and chief of pediatrics for Health System A (where Hospitals A and B are located):
- The Regional Chief for the area where Hospitals A and B are geographically located, should conduct a lessons-learned case review with all pediatric physicians in the region to discuss:
- Understanding the role of medical handover and transfer of care between physicians
- Wheezing in a child with heart disease
To the children’s hospital involved:
- The children’s hospital should review their practice of updating receiving physician information for all patients upon hospital admission and discharge.
To the children’s hospital and Hospital A:
- Consideration should be given for the establishment of a framework between the quaternary children’s hospital and regional tertiary care pediatric centers for sharing information relating to local, medically complex children that are receiving primary care from the quaternary children’s hospital.