This case involves a one-year-old child who presented to the ER with cyanosis and clubbed fingers. His parents expressed that the child suddenly collapsed while crawling at home. The boy had no medical conditions except for being born with a heart condition known as patent ductus arteriosus (PDA). At birth, the parents were told by a pediatrician that the defect was small enough to resolve on its own. Follow-up with the hospital was recommended but an appointment was never made. The emergency medicine team ordered an echocardiogram, which revealed a condition known as Eisenmenger’s syndrome. The ER physician told the parents that the child’s defect from birth had transformed into a new heart defect. The physician also indicated that the child had developed pulmonary hypertension, requiring heart surgery and lung transplantation. The child underwent successful heart surgery. A few months later, the child received a lung transplant. Unfortunately, the child experienced organ rejection and subsequently died.
Question(s) For Expert Witness
1. Did the pediatrician properly manage the health of this child after birth, and what should have been done to prevent the development of Eisenmenger’s syndrome?
Expert Witness Response
The pediatrician who cared for this child after birth did not properly manage the patent ductus arteriosus (PDA). The PDA should have been treated with indomethacin or percutaneous intervention if needed. PDA is a heart condition characterized by left-to-right shunting of blood through the heart. If left untreated, this shunt can reverse in a right-to-left fashion resulting in pulmonary hypertension. Due to the fact that the original heart defect was neglected, this boy developed this reversal known as Eisenmenger’s syndrome. Eisenmenger’s syndrome will present with cyanosis, a high red blood cell count, finger clubbing, fainting, heart failure, arrhythmia, bleeding disorders, hemoptysis, and iron deficiency. This diagnosis of Eisenmenger’s syndrome can be made with an echocardiogram which will show right-to-left shunting of blood. If a patient does not develop pulmonary hypertension, this condition can be successfully repaired with surgery. If one does develop pulmonary hypertension, both cardiac surgery and lung transplantation are required to improve the quality of life. Unfortunately, a prognosis is poor in these patients. This particular patient’s immune system mounted an adverse reaction to the transplant. After congenital heart defects are diagnosed, it is important to treat them so they do not progress to Eisenmenger’s syndrome. These congenital heart defects include atrial septal defects (ASD), patent ductus arteriosus (PDA), and ventricular septal defects (VSD).
About the author
Jason Cohn
Jason is a 4th year medical student pursuing a career in Otolaryngology/Facial Plastic Surgery. His Interest include sports, fitness, chemistry, otolaryngology, plastic surgery, allergy/immunology, surgical oncology, human genetics, public health, preventative medicine, and rheumatology.
