Extracorporeal Life Support as a Rescue Measure for Managing Life-Threatening Arrythmia and Brugada Syndrome

We describe the use of extracorporeal cardiopulmonary resuscitation (E-CPR) to transiently stabilize a 3-month-old patient who presented with ventricular tachyarrhythmias leading to spontaneous cardiac arrest. The patient required 4 days of extracorporeal life support (ECLS) where he was diagnosed with probable Brugada syndrome (BS). The patient was discharged home in stable condition after implantable cardioverter defibrillator placement. This case highlights the importance of early transfer to extracorporeal membrane oxygenation (ECMO) center in the setting of unexplained cardiac arrhythmia in a pediatric patient. BS is an autosomal dominant genetic disorder with variable expression characterized by abnormal findings on electrocardiogram (ECG) in conjunction with an increased risk of ventricular tachyarrhythmias and sudden cardiac arrest (SCA). Early management is critical and early consideration to transfer to an institution where extracorporeal life support (ECLS/ECMO) is present to support the patient while further diagnostic work up is in progress is lifesaving

Early Pulmonary Vascular Disease in Young Adults Born Preterm

Rationale: Premature birth affects 10% of live births in the United States and is associated with alveolar simplification and altered pulmonary microvascular development. However, little is known about the long-term impact prematurity has on the pulmonary vasculature.Objectives: Determine the long-term effects of prematurity on right ventricular and pulmonary vascular hemodynamics.Methods: Preterm subjects (n = 11) were recruited from the Newborn Lung Project, a prospectively followed cohort at the University of Wisconsin-Madison, born preterm with very low birth weight (≤1,500 g; average gestational age, 28 wk) between 1988 and 1991. Control subjects (n = 10) from the same birth years were recruited from the general population. All subjects had no known adult cardiopulmonary disease. Right heart catheterization was performed to assess right ventricular and pulmonary vascular hemodynamics at rest and during hypoxic and exercise stress.Measurements and Main Results: Preterm subjects had higher mean pulmonary arterial pressures (mPAPs), with 27% (3 of 11) meeting criteria for borderline pulmonary hypertension (mPAP, 19-24 mm Hg) and 18% (2 of 11) meeting criteria for overt pulmonary hypertension (mPAP ≥ 25 mm Hg). Pulmonary vascular resistance and elastance were higher at rest and during exercise, suggesting a stiffer vascular bed. Preterm subjects were significantly less able to augment cardiac index or right ventricular stroke work during exercise. Among neonatal characteristics, total ventilatory support days was the strongest predictor of adult pulmonary pressure.Conclusions: Young adults born preterm demonstrate early pulmonary vascular disease, characterized by elevated pulmonary pressures, a stiffer pulmonary vascular bed, and right ventricular dysfunction, consistent with an increased risk of developing pulmonary hypertension