Relation of brain natriuretic peptide to myocardial performance index in adults with congenital heart disease.

Myocardial performance index (MPI) is an echocardiographic Doppler-derived measure of ventricular function previously validated in patients with congenital heart disease. It may be preferred over conventional noninvasive measures of ventricular function in patients with complex anatomy because it is dependent on neither geometric shape nor heart rate. Brain natriuretic peptide (BNP) is a predictor of systolic and diastolic dysfunction in anatomically correct hearts. The correlation of BNP to MPI in patients with congenital heart disease was determined. Fifty-four adults with congenital heart disease were evaluated. BNP was measured using standardized assays. Doppler echocardiography was performed within 6 months of BNP assay. There were no changes in clinical status during this interval. An experienced observer was blinded and evaluated all echocardiographic images, and MPI and ejection fraction (EF) were determined. Left ventricular (LV) or univentricular MPI was calculated in 34 patients and right ventricular (RV) MPI was calculated in 23 patients. Pearson's correlation coefficient test showed that BNP significantly correlated with LV/univentricular MPI (r = 0.461, p = 0.006) and RV MPI (r = 0.748, p <0.0001), whereas LV/univentricular EF and RVEF had no significant correlation with BNP (r = -0.189, p = 0.172; r = 0.066, p = 0.729, respectively). In patients with congenital heart disease, BNP correlated significantly with MPI, but not with LV, RV, or univentricular EF. This is particularly true in patients with geometrically variable right ventricles in which EF may be more difficult to assess. In conclusion, these findings emphasize the unique ability of both BNP and MPI to assess global ventricular function in geometrically complex hearts.

The impact of patient assistance programs and the 340B Drug Pricing Program on medication cost.

OBJECTIVES: Patient assistance programs and the 340B Drug Pricing Program promise to improve the financial stability, better serve vulnerable patients, and decrease the burden of cost for uninsured patients. Our objective is to examine the financial impact that PAPs and the 340B Program have on improving medication cost. STUDY DESIGN: Retrospective analysis of medication dispensary data. METHODS: Dispensary data for uninsured patients obtaining medications at 2 community health centers were collected from February 1 to February 29, 2012. Uninsured patients were divided into 2 samples: (1) patients receiving PAP medications and (2) patients receiving 340B medications. The main outcome measured was the patient's cost savings. Cost savings were calculated based on the amount a medication would have cost had it been purchased by patients at prices found on Epocrates software (drugstore.com). A paired sample t test model using continuous variables was utilized to calculate confidence intervals. RESULTS: A total of 1420 PAP and 2772 340B individual medications were dispensed to uninsured patients in February 2012. For patients receiving PAP medications the mean ± standard deviation (SD) for age = 52 ± 10. Average cost was $0.11 (95% CI, $0.04-$0.17) and average savings was $617.36 (95% Cl, $581.32-$653.40). For patients receiving 340B medications the mean ±SD for age = 50 ± 14. Average cost was $11.50 (95% CI, $10.55-$12.45). Average saving was $62.31 (95% CI, $57.99-$66.63). CONCLUSIONS: PAPs and 340B provide significant medication savings for uninsured patient. More research is needed to establish "best practices" for the successful integration of PAPs.

Regression of pulmonary arteriovenous malformations after transcatheter reconnection of the pulmonary arteries in patients with unidirectional Fontan.

OBJECTIVE: Pulmonary arteriovenous malformations (PAVM) develop in patients with a lung deprived of hepatic blood flow. Unidirectional Fontan patients have superior vena cava (SVC) blood directed to the left or right pulmonary artery (PA), and inferior vena cava (IVC) and hepatic blood baffled to the contra-lateral PA. Cyanosis often develops due to PAVM in the lung deprived of hepatic blood. We report experiences with transcatheter PA reconnection in patients with unidirectional Fontan and PAVM. DESIGN: Patients with unidirectional Fontan, cyanosis, and PAVM underwent transcatheter PA reconnection. Following simultaneous injection of contrast into PAs, a transseptal needle was used to puncture from one PA to the other. A balloon-mounted, uncovered stent was positioned bridging the pulmonary arteries. The procedure provided a nonrestrictive, permanent communication between the PAs. Results were retrospectively reviewed. Catheterization data and oxygen saturations before and after procedure were analyzed. RESULTS: Six patients with a median age of 14 years (range 13-35 years) were identified. All patients had functional single ventricle with unidirectional Fontan and PAVM in the lung supplied solely by SVC flow. The reconnection procedure was performed successfully without complications in all cases. The median follow-up time for all 6 patients was 12.8 months (range 5.8-19.3 months). Median oxygen saturation was 0.86 (range 0.81-0.92) prior to the procedure, and 0.95 (range 0.92-0.98) on follow-up, with a median increase of 9% (range 8-11%). CONCLUSION: Patients with unidirectional Fontan and PAVM demonstrate increased oxygen saturations following reconnection of PAs, suggesting regression of PAVM. This procedure can be performed safely using uncovered stents, and it is effective in improving systemic oxygen saturations.

Fontan operation and the single ventricle.

The Fontan operation has gone through multiple incarnations since Fontan and Baudet's initial description in 1971. Through the medical dossier of a patient with a single ventricle, we plot the history of medical, surgical, and percutaneous interventions over the past 40 years, specifically focusing on the Fontan procedure, its development, indications, sequelae, and complications. Cardiac computed tomography with angiography is highlighted as a noninvasive imaging tool for the evaluation of the complex Fontan circulation.