Extracorporeal Membrane Oxygenation (ECMO) for suspected neonatal genetic diagnoses with cardiorespiratory failure.

Recent data describe an increasing use of extracorporeal membrane oxygenation (ECMO) in neonates with various clinical conditions besides primary respiratory or cardiac diagnoses. Infants with underlying genetic disorders characterized by cardiopulmonary failure pose unique management challenges. When pathognomonic dysmorphic features for common genetic diagnoses are not present, the prognosis is uncertain at best when determining ECMO candidacy. Lengthy turnaround times of genetic testing often delay definitive diagnosis during the ECMO course. Clinical management pathways to guide practice and evidence to support the use of ECMO in rare genetic conditions are lacking. The decision to initiate ECMO is daunting but may be of benefit if the subsequent genetic diagnosis is non-lethal. In lethal genetic cases warranting discontinuation of care, the time spent on ECMO may still be advantageous as a bridge to diagnosis while allowing for parental bonding with the terminally ill infant. Diagnostic confirmation may also facilitate the attainment of closure for these parents. Here, we report our experience providing ECMO to three neonates presenting with cardiorespiratory failure and later diagnosed with rare genetic syndromes. We share the challenges faced, lessons learned, and outcomes of these critically ill neonates.

Management Strategies during a VA ECMO Run in a Neonate with E. Coli Septic Shock Masquerading as Hypoxic Ischemic Encephalopathy.

Advances in neonatal care for hypoxic respiratory failure, with high-frequency ventilation and inhaled nitric oxide, have led to a decreased need for extracorporeal membrane oxygenation (ECMO). However, neonates resistant to such therapies are more complex and at higher risk of mortality. One such population includes those with hypoxic ischemic encephalopathy (HIE) undergoing controlled hypothermia (CH). We present a challenging case of a full-term neonate with inotrope-resistant Escherichia coli septic shock, profound coagulopathy, hypoxic respiratory failure, and HIE requiring CH and venoarterial (VA) ECMO. We illustrate that family-centered decision-making, ECMO, primary team, and subspecialist support is critical to success. In addition, we share the strategic medical interventions concomitantly used with VA ECMO to aid in the survival of this high-risk infant such as continuous veno-venous hemofiltration with AN69 membrane for cytokine and fluid removal, prostaglandin use to relieve right ventricular strain in malignant pulmonary hypertension, and cautious use of bronchoscopy to assist in lung recruitment.