Perioperative Considerations for the Fontan Patient Requiring Noncardiac Surgery.

Children and adults with congenital heart disease undergoing noncardiac surgery are at higher risk of perioperative adverse events. Patients have significant comorbidities and syndromic associations that increase perioperative risk further. The complexity of congenital heart disease requires a thorough understanding of lesion-specific pathophysiology in order to provide safe care.

A neonate with long QT syndrome, refractory ventricular arrhythmias, and lidocaine toxicity.

Long QT syndrome is characterized by electrocardiographic appearance of long QT intervals and propensity to polymorphic ventricular tachycardia. Aggressive anticipatory clinical management is required for a good outcome, especially in the symptomatic neonate. We present a neonate with a compound mutation with refractory ventricular tachycardia that necessitated multimodal pharmacotherapy with lidocaine, esmolol, and amiodarone along with ventricular pacing.

Implications of anesthesia in children with long QT syndrome.

Background: Patients with congenital long QT syndrome (LQTS) are susceptible to an episodic malignant ventricular tachyarrhythmia known as torsade de pointes, which can result in a cardiac arrest and death. Patients can suffer severe cardiac events resulting in syncope, seizures, and sudden cardiac death during times of physical and emotional stress and when exposed to certain drugs including anesthetics. We describe the occurrence of perioperative adverse events (AEs) related to arrhythmias in children with congenital LQTS exposed to volatile general anesthesia and describe associated risk factors.

Assessment of parental decision-making in neonatal cardiac research: a pilot study.

Objective: To assess parental permission for a neonate's research participation using the MacArthur competence assessment tool for clinical research (MacCAT-CR), specifically testing the components of understanding, appreciation, reasoning and choice.

Study Design: Quantitative interviews using study-specific MacCAT-CR tools.

Hypothesis: Parents of critically ill newborns would produce comparable MacCAT-CR scores to healthy adult controls despite the emotional stress of an infant with critical heart disease or the urgency of surgery.

Novel use of dexmedetomidine in a patient with pulmonary hypertension.

The presence of pulmonary arterial hypertension (PAH) is a significant predictor of major perioperative cardiovascular complications in patients undergoing cardiac diagnostic or interventional procedure or non cardiac surgery under sedation and/or anesthesia. Factors that precipitate a pulmonary hypertensive crisis include hypoxia, hypercarbia, acidosis, hypothermia, pain and airway manipulations. Pain management is challenging in patients with significant PAH.