Extracorporeal Life Support in Multisystem Smooth Muscle Dysfunction Syndrome.

We describe an infant with congenital mydriasis, patent ductus arteriosus (PDA), pulmonary hypertension, and cystic lung disease. She had all the major components of multisystemic smooth muscle dysfunction syndrome. Due to progressive respiratory deterioration, she required surgical PDA interruption, extracorporeal life support, and subsequent prolonged respiratory support. Genetic testing revealed ACTA2 R179H mutation and cystic lung disease on biopsy.

Collaborative DTM reduces hospitalization and healthcare costs in patients with diabetes treated with polypharmacy.

OBJECTIVES: To evaluate the effects of a collaborative pharmacy benefits manager (PBM)/ health plan-administered drug therapy management (DTM) program on healthcare utilization and costs in patients with diabetes treated with polypharmacy. STUDY DESIGN: Retrospective quasi-experimental design with comparison group. METHODS: This DTM program was a collaborative effort between the PBM, PerformRx, and the care management departments of Keystone First (KF) and AmeriHealth Caritas Pennsylvania (ACP) care management departments, targeting patients with diabetes using >15 medications. Pharmacists reviewed member profiles and made evidencebased prescriber and patient interventions, working directly with prescribers and indirectly with members, via care managers. Care managers provided additional services not otherwise within the scope of DTM. The study group consisted of 954 DTM participants reviewed by a pharmacist between November 1, 2010, and July 31, 2011. The control group consisted of 810 matched DTM participants not reviewed by a pharmacist. RESULTS: Intervention acceptance rates for KF and ACP were 33% and 26%, respectively. The study group demonstrated lower inpatient admissions and emergency department utilization rates, although only the KF study group inpatient admission rate achieved statistical significance (76.4%; P = .0002). The study groups realized statistically significant total cost savings (pharmacy + medical) compared with their corresponding control groups (47.8% KF, P = .0039; 50.7% ACP, P = .0497) despite non-statistically significant increases in pharmacy costs. CONCLUSIONS: A collaborative pharmacist-driven DTM program with a care manager-executed patient outreach component results in reduced hospital utilization and significant healthcare cost savings.

Commotio cordis and ventricular pseudoaneurysm.

A case of an eight-year-old boy who experienced commotio cordis with the development of myocardial infarction and a ventricular pseudoaneurysm is described. Progressive enlargement of the aneurysm resulted in distortion and compression of the overlying coronary arteries, causing myocardial ischemia.

The effectiveness of transtelephonic monitoring of pacemaker function in pediatric patients.

BACKGROUND: To determine the sensitivity and specificity, rate of compliance, and predictors of failure of telephone transmission of pacemaker function in a pediatric population. METHODS: A total of 2,638 pacemaker transmission records were reviewed retrospectively. Standard calculations of sensitivity, specificity, and positive and negative predictive values were performed. Longitudinal data analysis was used to detect factors influencing the effectiveness of transtelephonic monitoring. The proportion of missed transmissions was calculated, thus enabling assessment of compliance. Logistic regression was performed to determine predictors of poor compliance. RESULTS: Telephone transmission of pacemaker function, as a diagnostic tool, had a sensitivity of 94.8%, specificity of 99.2%, positive predictive value of 82.1%, and negative predictive value of 99.9%. Longitudinal analysis failed to show any significant predictors of transmission failure. Compliance with a prescribed transmission reached 84.5% in our patient population. Logistic regression analysis failed to identify any predictors of noncompliance. CONCLUSION: Values for sensitivity and specificity indicate that telephone transmission is a useful diagnostic tool for assessing pacemaker function at a distance. Negative predictive value is 99.9%, indicating that normal telephone transmissions are very reassuring of normal pacemaker function. Telephone transmission is equally successful in all age groups, genders, distances from a tertiary referral center, underlying diagnoses, pacing modes, and pacemaker models. Compliance with telephone transmission follow-up was higher in our population than in previous studies.

Successful use of an intravenous infusion of flecainide and amiodarone for a refractory combination of postoperative junctional and ectopic tachycardias.

After repair of an atrioventricular septal defect with common atrioventricular junction in a 2-month-old girl, rapid atrial tachycardia, in combination with junctional ectopic tachycardia, led to severe postoperative cardiovascular compromise. Intercurrent runs of ectopic atrial tachycardia made atrial pacing impossible, despite high doses of intravenous amiodarone. Following the addition of flecainide to the infusion, we were able to control the rhythm, and when combined with atrial pacing, this led to an immediate haemodynamic improvement. Treatment of refractory supraventricular tachycardias with amiodarone combined with flecainide can be very effective in the setting of postoperative cardiac intensive care.