Abstract The use of extracorporeal membrane oxygenation in infants and children with cardiac disease who develop refractory cardiogenic shock, cyanosis, or cardiac arrest is increasing. Early mortality in children with cardiac disease who require extracorporeal membrane oxygenation remains an important issue, as only 40% of cannulated patients survive to discharge from the hospital. However, it is encouraging that 90% children who are discharged alive from the hospital after extracorporeal membrane oxygenation are still alive at intermediate-term follow-up. Surviving patients are at risk for long-term dysfunction of multiple organ systems related to their underlying cardiac disease, non-cardiac comorbidities, treatment-related complications, and exposure to extracorporeal membrane oxygenation. Among the most important acute complications related to support with extracorporeal membrane oxygenation is injury to the central nervous system, which may contribute to adverse neurodevelopmental outcomes. All of these factors, in turn, influence quality of life. Many survivors remain medically complex related to their underlying cardiac disease, comorbidities, and sequelae of complications acquired over their lifetime. Neurological morbidity clearly plays an important role in approximately one-third of survivors, with significant deficits in approximately 10%. The limited data about quality of life data that are available for survivors of cardiac extracorporeal membrane oxygenation suggests that approximately 15–30% of survivors have at least moderately decreased quality of life. Overall, published data support the ongoing use of support with extracorporeal membrane oxygenation in children with acute cardiac failure, most of whom would die without it. However, programmatic efforts to improve the selection of patients and the preservation of the function of end organs during extracorporeal membrane oxygenation are clearly needed in order to improve long-term outcomes.
Purpose: The CardioMEMS™ HF implantable pulmonary artery (PA) pressure monitor reduces heart failure hospitalizations in adults and is being explored in pediatric patients with congenital heart disease. We report novel optimization of the CardioMEMS™ HF in a pediatric patient with Fontan circulation listed for heart transplant. Improved monitoring in this continuous flow environment allows for more refined heart failure medication regimens and enables weaning of pulmonary vasodilator therapy to decrease vasoplegia at transplant. This platform could also be extrapolated for use in pediatric ventricular assist devices. Methods: A 16 y/o male with hypoplastic left heart syndrome status post Fontan with protein losing enteropathy and chylous ascites was referred for heart transplant listing. Cardiac catheterization showed Fontan pressure of 14 mmHg. The CardioMEMS™ HF device was implanted and appropriately calibrated with mean PA pressure of 15 mmHg. Input from the patient/parents regarding signal malfunction and error messages were obtained. Adjustments were made to decrease the range of pulsatility required by the device to sense appropriate pressure measurements. Further suspect readings were reviewed by the device coordinator and when appropriate were accepted to encourage feedback to the device. Results: Adjustments related to the range of pulsatility resulted in improvement in signal monitoring and in overall ease of use reported by the patient family. The result was increased compliance with more frequent measurements and less erroneous readings. Summary: CardioMEMS™ HF can be optimized in continuous flow through real-time feedback adjustments to improve patient compliance and enable medication titration. These adjustments may further enhance monitoring in continuous flow ventricular assist device placement with the CardioMEMS™ HF.
A 10-year-old girl with pulmonary atresia status post transannular patch repair and secundum atrial septal defect (ASD) underwent percutaneous ASD closure 5 years earlier. There was improvement in arterial saturation from 86% to 98% with minimal residual right-to-left shunt. Despite subsequent medical therapy, she developed recurrent migraines, which led us to pursue percutaneous closure of the shunt.
