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Pediatrics Vital Signs
ProAssurance Risk ManagementNovember 20235 min read

Undiagnosed Cardiomyopathy Leads to Injury and Heart Transplant


Failure to diagnose cardiomyopathy in a 19 MOM resulting in cardiac arrest, permanent hypoxic injury, and need for heart transplant.

Case Details

The child, a 12 MOM, was an established patient and seen by an insured pediatric group pediatric nurse practitioner (PNP) with complaints of congestion, raspy cough, and runny nose. PE found afebrile, clear drainage, lung sounds with bilateral crackles, and a negative RSV. A CXR demonstrated normal heart size and peribronchial prominence. Impression was pneumonitis-bronchiolitis. Treatment included albuterol nebulizer, azithromycin, montelukast, and cetirizine. Three days later the patient presented with a fever, positive RSV, and crackles in lung fields without wheezing. Treatment included fluids, saline mist, and budesonide inhaler. Over the next seven months, the child was evaluated and treated by two insured pediatricians and the PNP five times for similar respiratory complaints including fever. At least one of those visits to the PNP was signed off on by a pediatrician. Additionally, the child was seen once by an emergency department in this timeframe for similar symptoms. Two subsequent CXR’s during this course of ongoing care found a normal heart size.

Eight months following the initial visit in this ongoing course of care, the now 19 MOM presented to the practice again with cough, wheezing, and a runny nose and was seen by the PNP. Exam noted a negative RSV and a CXR reported mild bilateral interstitial densities at the bilateral lungs and an enlarged appearing cardiac size which may relate to artifact of projection but recommended clinical correlation to rule out cardiac disease. The PNP reviewed and initialed the CXR and did not report the findings to supervising pediatricians as she did not believe the result was abnormal. Treatment included cefdinir, albuterol, budesonide, push fluids, saline mist, elevate head of bed, and follow up in ten days for immunizations.

Seven weeks later the child returned to the pediatric practice with complaints of vomiting and a cough and saw a third pediatrician. An examination revealed moderate respiratory distress and bilateral wheezing with intercostal and subcostal retraction. RSV and influenza A and B were negative. A CRX found cardiomegaly and diffuse lung opacities suggestive of pulmonary edema versus bilateral pneumonia, and possible left pleural effusion. Diagnosis of acute exacerbation of asthma, cardiomegaly, and respiratory distress. Following discussion with a practice partner physician, a decision was made to admit the child to the hospital.

Later the same day the child was transferred from the regional medical center to a children’s hospital and shortly after arrival he arrested. He was resuscitated for 27 minutes and treated with ECMO due to severe heart failure associated with a dilated cardiomyopathy. A head CT six days after admission showed a basilar subarachnoid hemorrhage and intraventricular hemorrhage resulting in hydrocephalus and subacute infarcts. Pediatric neurologists charted abnormal movement and decreased tone in the arms and legs. The diagnosis was encephalopathy, with concerns for ischemic injury, basilar subacute hemorrhage, and intraventricular hemorrhage causing obstructive hydrocephalus and cardiovascular collapse of unknown etiology. The child received a video electroencephalogram which showed moderately slow and poorly reactive patterns consistent with moderate generalized encephalopathy. However, the possibility existed that this would be temporary and reversible and related to sedating medications.

Twelve days later the child transitioned to a BiVAD after an inability to wean from ECMO. Ten weeks later the child received a heart transplant but encountered a difficult recovery due to initial diastolic dysfunction and a viral infection. Postoperatively, the patient experienced low cardiac output causing acute kidney injury. Pathology of the explanted heart showed dilated cardiomyopathy with endocardial fibroelastosis.

Seven weeks post-transplant the child was discharged to receive outpatient speech, physical, and occupational therapy. Almost three years post-transplant, neurology indicated the child would start kindergarten the next year despite suffering some motor deficits that could impede functions such as handwriting. The child’s lower extremities demonstrated increased tone in the hamstrings, good range of motion, and increased tone in the ankle with ability to dorsiflex five degrees past neutral. The child was able to run in a coordinated manner with a brace. Neurology’s final assessment was right monoplegic cerebral palsy secondary to an ischemic stroke.

Expert Testimony

Experts on either side of this matter disagreed about the care that this pediatric patient received. Plaintiff’s experts argued that the practice breached standards of care by failing to appropriately supervise the PNP and by failing to follow up on changes in and obtain specialist consultation for the CXR’s the child received. Plaintiffs argued that had the child been seen by a cardiologist, the cardiomyopathy could have been diagnosed and treated, negating the subsequent complications the child suffered.

Defense experts, on the other hand, offered support for the care that the child received. More than one expert supported the PNP opinion that there was no evidence of a cardiac problem in the CXR she reviewed and therefore no need for her to consult with physicians about the patient. Further, they opined that the CRX’s were normal all the way up to the hospital admission exam. One also testified the child being seen by a cardiologist likely would not have altered the outcome as to the transplant. He additionally testified that the embolic strokes caused brain damage because of the ECMO, which the patient needed to prevent another arrest due to left ventricular non-compaction seen in the pathology of the explanted heart. Whether the Collaborative Practice Agreement for the PNP was completed and appropriately followed also factored into the resolution of the case.


The case settled prior to trial.

Risk Reduction Strategies

  • Know the requirements in your state for Collaborative Practice Agreements (CPAs) for advanced practice professionals (APP) such as nurse practitioners and physician’s assistants.
  • Review and revise CPAs pursuant to state guidelines and APP education, training, and experience to support compliance with delegation and supervision.
  • Understand how vicarious liability and direct liability can affect your practice and seek to minimize exposure through good policies and procedures, thorough documentation, and appropriate hiring and supervision of employees.
  • Document completely the reasoning behind ordering or not ordering additional tests or treatment, or referrals to specialists.


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