37 wk, GA infant born via SVD with APGARS of 8, 9, with an initial bilirubin of 10.55, who upon auscultation, was found to have S1, 52 with harsh, blowing systolic Grade 3/6 murmur heard throughout. Echo the following day was abnormal with AoV mean peak gradient at 21.2.. The child was discharged with instruction to follow up with his pediatrician and ped cardiologist in a weeks time. Upon follow up, a second echo was performed. The second echo was substantially changed with a peak gradient of 104 with other significant changes.
The child was immediately admitted and a full cardiac workup was begun. Days after admission the child underwent a right/left heart cath with balloon valvuloplasty with a post gradient of 5 mm/hg. he had no complications. Post procedure lactate was mid 4's and it continued to rise. Fluid resuscitation and PRBC's were given. The next day the child had hematuria with evidence of hemolysis. along with hyper bilirubinemia. His cardiac function remained "normal" .
There after he developed abdominal compartment syndrome, and after a difficult course and heroic measures the parents request comfort care and the child expired.
The D/C summary and other documents are attached for more detailed information.
Is it possible a delay or mis-diagnosis of severity of cardiac congenital disease caused systemic decreased perfusion over a long period, leading to end organ damage and failure and death?
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Do you believe there might have been medical error?
This patient was admitted to the hospital at 1 week of life with clinical and echocardiographic evidence of severe aortic valve stenosis. Urgent/emergent balloon valvuloplasty is indicated in this case. It does not appear that lactate level was monitored prior to the procedure- given the clinical status of the patient on admission, there was likely to be diminished perfusion at that time.
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
The delay of treatment (balloon valvuloplasty) likely resulted in a prolonged period of diminished systemic perfusion
What makes you a good expert for this case?
I am a board certified academic pediatric cardiologist with extensive experience in both inpatient/critical care and outpatient settings. This case relies upon echocardiographic data, which is a my clinical specialty.
How often do you encounter cases similar to this one in your practice?
I see severe/critical aortic valve stenosis 5-6 times per year
Do you believe there might have been medical error?
I would have managed the patient similarly. The only question is if there was an event in the cath lab that could explain the clinical worsening. A repeat echo following the balloon valvuloplasty would also be informative.
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
See above. No clear error in management from my perspective, despite the unfortunate outcome.
What makes you a good expert for this case?
I am a pediatric cardiologist with expertise in management of congenital heart disease
How often do you encounter cases similar to this one in your practice?
A few times per year we encounter aortic stenosis in the neonatal period
Do you believe there might have been medical error?
The diagnosis of aortic valve stenosis appeared timely and the cardiac catheterization was uneventful. It is not uncommon where the gradient across the valve initially was in mild range then a week later the gradient is increased. Cardiac systolic function appeared preserved. There was mild aortic regurgitation following the cardiac cath which is not uncommon. The patient developed decreased perfusion to lower extremity which is also not uncommon. The unusual complication in the case was the low ADAMTS13 level which indicates TTP (either acquired or hereditary). The patient had hemolysis, thrombocytopenia and microangiopathy. Endothelial injury may trigger low ADAMTS13 levels. Unfortunately, the patient suffered severe complications including renal failure, seizures, abdominal compartment syndrome and expired. Patients with TTP can usually respond to plasma infusions or exchange.
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
It sounds like TTP was diagnosed fairly quickly however the patient had already experienced a number of complications including renal failure. Patient was not tolerating CRRT. Perhaps plasma infusion/exchange was not effective given severity of critical illness.
What makes you a good expert for this case?
I am a pediatric cardiologist with ~24 years experience in academic and private setting.
How often do you encounter cases similar to this one in your practice?
We see patients with aortic valve stenosis fairly commonly. We are very familiar with issues that can occur following cardiac catheterization. TTP is fairly uncommon but we are very familiar with secondary thrombotic microangiopathy (TMA) in bone marrow transplant population. You may want to engage a pediatric hematologist to review the case as well.
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