The decedent is a 15-month-old male born at 37 weeks and 3 days with congenital heart disease, polydactyly of left foot (status post removal of postaxial toe), failed hearing test, abnormal involuntary movements, central hypotonia, gross motor defects, bilateral undescended testes, and phimosis. Pregnancy was complicated by polyhydramnios, gestational hypertension, and intrauterine growth restriction. Genetic analysis identified him as having SETD5-related neurodevelopmental disorder. Was dx with AVCD prenatally and confirmed after birth.
He followed with pediatric cardiology in an outpatient setting, and echocardiogram studies showed a transitional atrioventricular canal defect, large primum and small secundum atrial septal defects, right atrium dilation, mild right atrioventricular valve insufficiency, and mild left atrioventricular valve regurgitation due to a cleft directed to the right atrium. Due to worsening right heart dilation and left to right shunting, he underwent surgery for repair of the transitional atrioventricular canal defect, left atrioventricular valve cleft closure, and patch closure of atrial septal defect. Post-repair transesophageal echocardiogram was reassuring and he was extubated. Overnight he developed lactic acidosis with hypotension and repeat echocardiogram showed new severely depressed biventricular function with evidence of pulmonary hypertension.
He additionally developed clinlcal signs of infection, and was placed on VA-ECMO three days following his operation in order to recover lung and cardiac function.
His ventricular and pulmonary function improved, and he was decannulated from ECMO approximately five days following. However, shortly after this he developed intermittent fevers, elevated C-reactive protein, elevated white blood cell count, and acute desaturation requiring intubation. Chest x-ray showed a completely consolidated right lung. Bronchoscopy was attempted to inflate the right lung but was unsuccessful and distal tracheomalacia and mild right bronchomalacia were noted. His blood cultures showed no growth, and respiratory viral panel was negative. He was given broad-spectrum empiric antibiotics. He developed acute hypercarbic respiratory failure; chest x-ray showed pulmonary edema. He underwent cardiac catheterization, which showed restrictive pathology, although coronaries were unremarkable.
Approximately two weeks following his procedure, he was transferred to different pediatric specialty facility for further management and heart transplant evaluation. Karius testing identified Achromobacter xylosoxidans and he was treated with multiple antibiotics. He had abrupt significant worsening of pulmonary edema, with only transient improvement with increased diuresis. Subsequently, he acutely desaturated, became bradycardic, and developed pulseless electrical activity. Acute cardiovascular life support was conducted with return of spontaneous circulation after 23 minutes.
Despite maximal vasopressor support, he developed multiorgan system failure. The decedent passed shortly after switching to comfort care.
Significant findings at autopsy included an enlarged heart with evidence of prior procedure with an intact patch and evidence of healing on histology. The pericardium was roughened, and fibrin deposition without significant inflammation was identified on the pericardium and epicardial surfaces. Bilateral lungs showed evidence of diffuse alveolar damage and parenchymal hemorrhage. The liver showed diffuse near bridging centrilobular necrosis, and the kidneys showed evidence of acute kidney injury. The brain showed hypoxic injury.
Looking for a pediatric cardiologist familiar with this condition to determine if the course of events and treatment plan was reasonable/within SOC or if there were possible opportunities for deviations.
Attached you will find updated progress notes from the last facility the IP was admitted to for reference.
Please notify of any questions. Thank you in advance.
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Do you believe there might have been medical error?
The pt developed heart failure first post op night. This is either related to sepsis or potentially post bypass SIRS. The surgical repair appears intact on TEE and autopsy. Karius testing revealed Achromobacter xylosoxidans which is a very unusual organism (nosocomial) -usually multi drug resistant(MDR). Restrictive cardiac physiology likely related to multiple cardiac insults/hypoxia/ECMO etc. It is unclear the role of the underlying genetic condition (probably another risk factor).
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
Perhaps the patient should not have been extubated so soon after surgery. It does not say was the patient on inotropic support? Aside from lactic acidosis, were there others signs of low cardiac output until it became clear that the pt was in profound heart failure. Achromobacter xylosoxidans is a very unusual organism (nosocomial) and MDR.
What makes you a good expert for this case?
More than 25 years in pediatric cardiology in academic and private setting. I primarily work in pediatric echocardiography and TEE.
How often do you encounter cases similar to this one in your practice?
We have an active full service CVICU with many similar pts.
Do you believe there might have been medical error?
From a peds cardiology and cardiothoracic surgical standpoint closure of a primum ASD and clef mitral valve is a standard low risk procedure in most centers assuming there was no evidence of pulmonary artery hypertension prior to surgical intervention which is also unlikely. I assume this patient did not have trisomy 21 and I would have to review the preoperative echocardiogram and postoperative studies to see if there was any deviation in care surrounding the surgical intervention.. I am suspicious for an infectious process developing immediately after the cardiac surgery based on what transpired less than 24 hrs postoperatively.
Do you believe there might have been causation (i.e. the medical error resulted in an injury)?
There is not sufficient information to say for certain that the surgical procedure was performed below the standard of care especially if the patient was hemodynamically stable coming off CPB and within the first 24 hrs postoperatively in the pediatric ICU.. A more intense review of the medical records would be necessary to render any opinion regarding a deviation in care postoperatively.
What makes you a good expert for this case?
I have been practicing pediatric cardiology and managing patients pre and post operatively for 35 years at a children's hospital with a moderate size pediatric cardiology and peds cardiothoracic surgical program. I have seen many cases of children with primum ASD's undergo this type of surgery
How often do you encounter cases similar to this one in your practice?
This would be considered an unusual postoperative course for a child with an isolated primum ASD and cleft mitral valve who is otherwise asymptomatic
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