Unusual sudden cardiac death from an anomalous left coronary artery from the right sinus of Valsalva.

A left coronary artery arising from the right sinus of Valsalva is a rare congenital coronary anomaly. We report a case of a 5-year-old boy with an anomalous left coronary artery from the right sinus of Valsalva whose presenting sign was cardiac arrest. There is no reported instance of a child <9 years of age without other congenital cardiac defects having died suddenly with this coronary anomaly. The transthoracic echocardiogram demonstrated normal origins of the coronary arteries, but on autopsy, an anomalous origin of the left main coronary artery from the right sinus of Valsalva was found.

A survey of perioperative management of sickle cell disease in North America.

BACKGROUND: Children with sickle cell disease frequently undergo surgical procedures that are associated with acute exacerbations of the disease. Current perioperative management practices are unclear. OBJECTIVES: We aimed at describing the current management. METHODS: We conducted an electronic survey of North American members of the Society for Pediatric Anesthesia, in which we asked about their perioperative management of sickle cell disease. RESULTS: The response rate to valid addresses was 25% (n=510/2006). In four scenarios, (a patient with mild disease undergoing a minor procedure; a patient with mild disease undergoing a more invasive procedure; a patient with severe disease undergoing a minor procedure; and a patient with severe disease undergoing a more invasive procedure) 80%, 38%, 27%, and 16% of respondents, respectively, would rely on oral fluids to hydrate patients during the preoperative fast, while 13%, 34%, 44%, and 59%, respectively, would use intravenous fluid. For the same four scenarios, 64%, 28%, 33%, and 10%, respectively, would not transfuse patients in an attempt to prevent sickle cell exacerbations, while 17%, 49%, 36%, and 51%, respectively, would transfuse to a hemoglobin concentration of 10 g·dl(-1). The tendencies to administer preoperative intravenous fluid and to transfuse blood increased with disease severity and procedure invasiveness (P<0.001). Although 89% felt comfortable managing patients with sickle cell disease, 73% thought an advisory statement on optimal perioperative management was needed. CONCLUSIONS: There is a wide variation in the management of children with sickle cell disease. Clinicians differentiate management based on disease severity and procedure type.

Outcomes of children with congenital heart disease implanted with ventricular assist devices: An analysis of the Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs).

BACKGROUND: The reported ventricular assist device (VAD) experience in the pediatric congenital heart disease (CHD) population is limited. We sought to describe contemporary use and outcomes of VADs in children with CHD and compare these outcomes to those of non-CHD children. METHODS: Patients enrolled in the Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) between September 19, 2012 through June 30, 2017 were included. CHD was classified as biventricular vs single ventricle (Stages 1, 2, or 3). Outcomes were compared between groups and multivariable analysis was used to identify factors associated with mortality on the device. RESULTS: Among the 471 patients enrolled, 108 (24%) had CHD (45 biventricular and 63 single ventricle). CHD patients were younger (5.7 ± 5.7 years vs 9.8 ± 6.5 years; p < 0.0001) and smaller (0.8 ± 0.5 m2 vs 1.2 ± 0.7 m2; p < 0.0001) compared with non-CHD patients. CHD patients were more likely to receive a paracorporeal continuous-flow VAD (36.1% vs 12.9%; p < 0.0001) and less likely to receive an implantable continuous-flow VAD (27.8% vs 55.0%; p < 0.0001) compared with non-CHD patients. After 6 months on a VAD, CHD patients had higher mortality (36.4% vs 12.1%) and a lower transplantation rate (29.1% vs 59.9%) than non-CHD patients (p < 0.0001). In the multivariable analysis, CHD was the factor most strongly associated with mortality on VAD (hazard ratio [HR] = 2.9; p < 0.0001), whereas the factors implantable continuous-flow device and high-volume center were protective (HR = 0.3, p < 0.0001, and HR = 0.6, respectively; p = 0.02). CONCLUSIONS: VAD use in children with CHD is associated with increased mortality and decreased transplant rates compared to children without CHD. For the subgroup of children with CHD who received implantable continuous-flow VADs, survival rates were higher and comparable to those of children without CHD. Increased experience correlated with better survival in pediatric VADs.

A novel approach to life support training using "action-linked phrases".

BACKGROUND: Observations of cardiopulmonary arrests (CPAs) reveal concerning patterns when clinicians identify a problem, (e.g. loss of pulse) but do not immediately initiate appropriate therapy (e.g. compressions) resulting in delays in life saving therapy. METHODS: We hypothesized that when individuals utilized specific, short, easy-to-state action phrases stating an observation followed by an associated intervention, there would be a higher likelihood that appropriate action would immediately occur. Phase I: A retrospective analysis of residents in simulated CPAs measured what proportion verbalized "There's no pulse", statements and/or actions that followed and whether "Action-Linked Phrases" were associated with faster initiation of compressions. Phase II: Two prospective, quasi-experimental studies evaluated if teaching three Action-Linked Phrases for Basic Life Support (BLS) courses or six Action-Linked Phrases for Advanced Cardiovascular Life Support (ACLS) courses was associated with increased use of these phrases. RESULTS: Phase I: 62% (42/68) of residents verbalized "there's no pulse" during initial assessment of a pulseless patient, and only 16/42 (38%) followed that by stating "start compressions". Residents verbalizing this Action-Linked Phrase started compressions sooner than others: (30s [IQR:19-42] vs. 150 [IQR:51-242], p < 0.001). Phase II: In BLS courses, the three Action-Linked Phrases were used more frequently in the intervention group: (226/270 [84%] vs. 14/195 [7%]; p < 0.001). In ACLS courses, the six Action-Linked Phrases were uttered more often in the intervention group: (43% [157/368] vs. 23% [46/201], p < 0.001). CONCLUSIONS: Action-Linked Phrases innately used by residents in simulated CPAs were associated with faster initiation of compressions. Action-Linked Phrases were verbalized more frequently if taught as part of a regular BLS or ACLS course. This simple, easy to teach, and easy to implement technique holds promise for impacting cardiac arrest teams' performance of key actions.