Retrograde venography and three-dimensional mapping of a great cardiac vein with separate drainage into the high right atrium in a patient with Wolf-Parkinson-White syndrome.

A 13-year-old boy presenting with palpitations was diagnosed with Wolf-Parkinson-White syndrome. We elected to bring him to the electrophysiology lab for evaluation of the accessory pathway and potential ablation. Structural assessment of the coronary sinus with electro-anatomical mapping and retrograde venography led to the discovery of a great cardiac vein having a separate insertion from the coronary sinus into the high right atrium. The accessory pathway was localized to the left lateral portion of the mitral annulus and treated successfully with radiofrequency ablation. Such venous anatomical variant has been seldom reported, and its association with an accessory pathway is described here for the first time. Its recognition can have important implications when performing procedures that require access into the coronary venous system.

Effect of dexmedetomidine on pulmonary artery pressure after congenital cardiac surgery: A pilot study.

OBJECTIVE: To characterize the effects of dexmedetomidine on the pulmonary artery pressure in patients after congenital cardiac surgery. DESIGN: Prospective observational pilot study. SETTING: Pediatric cardiac intensive care unit at a university hospital. PATIENTS: Twenty-two patients who received dexmedetomidine after cardiothoracic surgery. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: An echocardiogram was performed at three time points: 1) baseline (T0); 2) 6 mins after dexmedetomidine loading (T1); and 3) 1 hr after initiation of dexmedetomidine infusion (T2). Transthoracic echocardiography was used to estimate pulmonary artery pressure based on tricuspid regurgitant velocity (4 x Velocity2) plus central venous pressure. Twenty-two patients aged 0.9 yrs old (interquartile range, 7.9) were enrolled at a median of 1 hr (1.5) after surgery. Dexmedetomidine loading, 0.62 microg/kg (0.5), was given in all patients followed by 0.5 microg/kg/hr (0.6) at T1 and 0.65 microg/kg/hr (0.5) at T2. None of the patients had any increase in pulmonary artery pressure. Overall, the pulmonary artery pressure decreased from 30 mm Hg (13) at T0 to 24 mm Hg (10) at T1 and 26 mm Hg (8) at T2 (p < .001). The pulmonary artery pressure/systemic systolic blood pressure ratio decreased from 33% (12) at T0 to 23% (15) at T1 and 25% (13) at T2 (p = .002). There was no difference in the left ventricular function, Fio2, oxygen %, Po2, CO2, and vasoactive agents. CONCLUSIONS: Administration of dexmedetomidine after congenital cardiac surgery was not associated with any increase in pulmonary artery pressure.

Minimal sedation second dose strategy with intranasal midazolam in an outpatient pediatric echocardiographic setting.

BACKGROUND: Anxiety and movement in children during transthoracic echocardiography (TTE) can compromise study quality and reliability. Minimal sedation is often required. Intranasal midazolam (INM), used in various procedures, is an excellent sedative. Optimal INM dosing strategies for uncooperative children undergoing TTE are largely unknown, including second-dose INM strategies, introduced to maximize the potential for successful sedation and minimize risk. The purpose of this retrospective review was to evaluate the effectiveness of a second-dose INM minimal sedation strategy recently adopted at the Children's Hospital of Pittsburgh. METHODS: The strategy incorporates a second dose of INM if needed (10-15 minutes after the first dose) to obtain the desired level of anxiolysis. The effectiveness of this strategy was assessed in 100 consecutive patients (age range, 1-59 months). RESULTS: There were no reported complications, minimal untoward side reactions, and no delays in discharge. Eighty patients attained satisfactory minimal sedation levels. CONCLUSION: A second-dose INM strategy was effective in achieving satisfactory minimal sedation in children undergoing TTE. The results of this study also suggest that only a small proportion of patients would benefit from a one-dose INM strategy.

10-year case review of nutritional rickets in Children's Hospital of Michigan.

Nutritional rickets has been on the rise in the United States. A chart review of patients with nutritional rickets from April 1995 to May 2005 was performed. Fifty-eight subjects were studied (62% males, 38% females, with an age range between 2 and 132 months). Of the subjects, 81% were African Americans and 14% were Arabic; 33% were Christians and 19% were Muslims. An increasing number of cases of nutritional rickets have been noted since 2000. Seventy-nine percent of patients with nutritional rickets presented at the emergency department, and in 69% of the cases, rickets was an incidental finding; 96% of patients were exclusively breast-fed, and none received multivitamin supplements. 25-OH vitamin D levels were below 5 ng/mL in 42% of the patients, all of whom were African Americans. We could document complete resolution of nutritional rickets in only 8 patients, and 3 of these patients showed sequelae of rickets.