Resuscitation and Perioperative Management of the High-risk Single Ventricle Patient: First-stage Palliation

Abstract

Infants born with hypoplastic left heart syndrome or other lesions resulting in a single right ventricle face the highest risk of mortality among all forms of congenital heart disease. Before the modern era of surgical palliation, these conditions were universally lethal; recent refinements in surgical technique and perioperative management have translated into dramatic improvements in survival. Nonetheless, these infants remain at a high risk of morbidity and mortality, and an appreciation of single ventricle physiology is fundamental to the care of these high-risk patients. Herein, resuscitation and perioperative management of infants with hypoplastic left heart syndrome are reviewed. Basic neonatal and pediatric life support recommendations are summarized, and perioperative first-stage clinical management strategies are reviewed.

Percutaneous intraluminal downsizing of systemic‐to‐pulmonary artery shunts: a novel application of the Diabolo stent technique—Case series and description of the technique

“…Pulmonary overcirculation in patients with single‐ventricle physiology, especially in those with a systemic‐to‐pulmonary artery shunt, is a high‐risk clinical condition characterized by impaired systemic oxygen delivery that requires intensive medical and surgical interventions . Here, we present our first series of patients with pulmonary overcirculation who successfully underwent intraluminal downsizing of a systemic‐to‐pulmonary artery shunt, describing a novel application of the Diabolo stent technique.…”

“…An adequate balance between systemic and pulmonary circulations represents an important protective factor for survival in patients with single‐ventricle physiology . Although insufficient pulmonary blood flow can result in hypoxemia with subsequent metabolic instability and myocardial dysfunction, excessive pulmonary blood flow can lead to systemic hypoperfusion, coronary insufficiency, metabolic acidosis, and end‐organ dysfunction .…”

“…An adequate balance between systemic and pulmonary circulations represents an important protective factor for survival in patients with single‐ventricle physiology . Although insufficient pulmonary blood flow can result in hypoxemia with subsequent metabolic instability and myocardial dysfunction, excessive pulmonary blood flow can lead to systemic hypoperfusion, coronary insufficiency, metabolic acidosis, and end‐organ dysfunction . The difficulties in balancing pulmonary and systemic blood flow (Qp:Qs) in these patients are reflected in the high rates of complications, including hemodynamic instability, cardiac arrest, and mortality reported across institutions …”

Nurse‐driven analgesia and sedation in pediatric patients with univentricular hearts requiring extracorporeal life support after first‐stage palliation surgery: A pilot study

“…Despite major advantages in surgical technique and perioperative treatment, these procedures still carry a high operative risk and perioperative morbidity. Approximately 10% of these children require postoperative extracorporeal life support following first‐stage palliation . Analgesia and sedation in these children is demanding since international guidelines for analgesia and sedation of children requiring extracorporeal life support are lacking.…”

Improving Outcomes for Infants with Single Ventricle Physiology through Standardized Feeding during the Interstage

“…Recent advances in these staged palliative procedures have improved survival and according to Lowry [ 3 ] seventy percent of these infants are expected to reach adulthood. Although the majority of these children now reach adulthood, many have neurodevelopmental delays attributed to a variety of factors including poor nutrition [ 4 ].…”