4-2-18 visit with Pediatrician, cx of voice changes occurring gradually in last 6 weeks. Pt referred to ENT.
6-12-18 cx cough (call from parents) OV – 15 y/o females cx cough. Onset gradual. Constant, dry, plus voice changes.. ENT was negative. Lungs normal clear to auscultate
9-19-18 Ped Pulmonologist - SOB has diff walking up one flight of stairs, no associated cough or wheezing, can run for 3-5 min before developing symptoms, Symptoms take 20 min to resolve. Says she feels like having trouble getting air in.. Unable to make loud noises. PE: aeration good, equal, chest clear. Heart RRR, PFT not exhale long enough to meet requirements for testing. Sees Allergist rec HH and possible cardiac w/u… Dx vocal cord dysfunction, continue asthma meds and if not improving workup. to include echo, refer to speech therapy. Continue Flovent. If not improved will order echo, refer to speech therapy.
10-9-18 Ped Pulmonologist - since last visit has improved, less difficulty with exertion.
Able to climb stairs now. DX ACT score = 15 asthma not well controlled.. If she continues to have difficulty with voice see ENT again or start trial of reflux meds.
1-18-19 - Pediatrician appointment - went to Disney recently and she became SOB often and was having a hard time breathing on long walks (wants a new specialist referral not happy) HX; SOB began last spring, Pt could hardly walk for 200-400 feet without getting SOB even with inhaler. C/O tightness of chest at Disney this past weekend. Burping a lot. PE, normal RR, Lungs Clear to auscultate, 10 Lb weight loss. Dx Asthma
2-28-19 Call cx vomiting abd pain, pt seen in office, episodes of random vomiting, gained 3 lbs… start on zantac
3-7-19 Pediatric Hospital. ED, vomiting etc.. Pt presents with SOB, 3 week hx abdominal pain and vomiting, Labs PC02 28.8 (L) Hco3 17.4 (L), base excess -6 low, Tc02 18 (L). CXR- Lingula and LLL pneumonia, Heart enlarged, Suspicious Mid R lung pericardial effusion. CT – very large pulmonary artery suggesting pulmonary hypertension, multiple cavity lesions throughout lungs: Consult 16 y/o with chronic pulmonary Hypertension of unclear etiology. Echo: Severe RA dilation, Dilated IVC and hepatic veins, Mod to severe TR with RVp 112 consist with severe pulmonary hypertension.
3-10-19 Transferred to Mayo Clinic – Pt pre-lung transplant candidate. Awaiting DX of cause of pulmonary hypertension.
3-19-19 Death. Cause of death on Death Certificate: (1) Pulmonary Edema; (2) Pulmonary Arterial Hypertension; and (3) Acute Respiratory Failure with Hypoxia
Files:
Q: was there an autopsy?
A: No autopsy was performed.
Q: any significant PMH or travel history
A: No significant PMH and travel history included a trip to Ireland in July 2018 and trip to Disney in Orlando, FL in January 2019.
Q: CXR findings before the final admission?
A: 3-7-19 CRX - Left upper lobe patchy opacity with consolidation. Suspicious right mid lung nodular opacity. Differential includes pneumonia versus neoplastic process. Suspicious cardiomegaly and pericardia! effusion is in the differential.
Do you believe there might have been medical error?
A chest x-ray should have been ordered in September/October by the pediatric pulmonologist. This would have helped to diagnose the patient many months earlier. An ECHO of the heart would have been done and treatment for pulmonary hypertension would have started.
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
If patient was diagnosed in September/October 2018 treatment would have started earlier. Pulmonary hypertension is a progressive disease and initiation of early treatment is life saving. Patient might not have needed lung transplantation or at least would have plenty more time to wait on the list and could have been alive now. Delay in diagnosis is DIRECTLY related to patient's death.
What makes you a good expert for this case?
I have many years of experience in cases like this. I treated many kids with pulmonary hypertension and also involved in their lung transplantation. I served as the medical director of one of the largest pediatric lung and heart/lung transplant program for years. I also have plenty of experience as an expert witness.
How often do you encounter cases similar to this one in your practice?
Not very often since this is a rare disease but I would say around 2-3 per year.
Do you believe there might have been medical error?
The visit on 1/18/19 is very worrisome. She could hardly walk 200-400 feet and she had a 10lb weight loss. The very severe impairment in her ability to walk and a 10 lb weight loss is unusual even a child with severe asthma. Additional investigation was warranted at that time including a CXR, HRCT and and ECHO. There was no notation that her asthma therapy was escalated at that point.
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
That is a difficult question to answer because we don't have a final diagnosis. I would say that if a w/u had been done earlier I am confident that the pulmonary hypertension would have been picked up sooner and more likely than not more workup could have been done and she could have been listed for a transplant sooner.
What makes you a good expert for this case?
I have 40 years experience taking care of children with chronic lung diseases including asthma and a whole variety of pulmonary diseases in children. My experience as a PICU attending gives me additional insight into the pathogenesis of pulmonary diseases complicated by pulmonary hypertension. For the last 6 years I have worked in a large children's hospital. I am board certified in Pediatric Pulmonary and Pediatric Critical Care Medicine. I have had experience as an expert witness giving many depositions and some trail testimony.
How often do you encounter cases similar to this one in your practice?
This is an unusual case so I would say not very often.
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