Accessory Left Ventricle: A Mistaken Case of Ventricular Septal Defect!

Physical examination is becoming a lost art. We describe a case of a patient who was referred to us with an initial diagnosis of ventricular septal defect. Discordance between imaging findings and the physical examination led to a diagnosis of an accessory left ventricle, a rare but benign congenital cardiac condition. (Level of Difficulty: Beginner.).

Age at Fontan procedure impacts exercise performance in adolescents: results from the Pediatric Heart Network Multicenter study.

BACKGROUND: Most single ventricle patients undergo Fontan procedure in a staged manner. However, optimal timing of Fontan completion after an intermediate staging surgery is controversial. Therefore, we investigated the long-term impact of age at Fontan completion on the exercise performance in adolescents. METHODS: We analyzed National Institutes of Health/National Heart, Lung and Blood Institute Pediatric Heart Network Fontan Cross-Sectional Study dataset consisting of children and adolescents 6 to 18 years of age recruited in 2003 to 2004. Multivariate linear regression techniques were used to evaluate association of age at Fontan procedure with percent predicted VO2 maximum, percent predicted maximum O2 pulse, and heart rate reserve in patients who achieved ventilatory anaerobic threshold (VAT). RESULTS: Of the 405 patients who had undergone only one Fontan operation and ramp cycle ergometry, 72% had prior intermediate surgery. Mean age at Fontan completion and exercise testing was 3.4±2 and 12.4±3.2 years. Three hundred twelve patients reached VAT suggesting adequate cardiopulmonary effort. In patients who reached VAT, each year increase in age at Fontan completion was associated with a decline of 1.5 (95% CI -2.5 to -0.5) points in percent-predicted VO2 maximum and a decline of 4.1 (95% CI -6.0 to -2.1) beat/min in heart rate reserve after adjusting for all pertinent variables. CONCLUSIONS: Fontan completion at a younger age is associated with better exercise performance in adolescents.

Risk-prediction models for mortality after coronary artery bypass surgery: application to individual patients.

INTRODUCTION: We derived a risk-assessment model for predicting mortality after coronary artery bypass surgery from patient data from the 1990s and examined the model's accuracy in predicting early mortality in more contemporary patients. We also examined the accuracy of a completely new model and an older model recalibrated with newer data. MATERIALS AND METHODS: Three mortality-prediction models were derived: an "old" model from 8959 patients treated during 1993-1999, a "new" model from 5281 patients treated during 2000-2004, and a version of the old model "recalibrated" with the 2000-2004 data. Each model's discriminatory ability was assessed by computing area under receiver-operator characteristic (ROC) curves, and precision was estimated by comparing observed and predicted mortality rates. To test the temporal applicability of the old model, we applied it to the 2000-2004 data and to data from 1879 patients operated on during 2005-2007. To compare the recalibration and remodeling strategies, the new and recalibrated models were applied to the 2005-2007 data. RESULTS: The old model showed good discrimination (ROC, 0.80) and concordance between observed and predicted mortality for the 2000-2004 patients but overpredicted mortality for the 2005-2007 patients. The new and recalibrated models had good discriminatory ability (ROC, 0.81 and 0.79) and showed similarly good concordance between observed and predicted mortality when applied to the 2005-2007 data. CONCLUSIONS: Predictive models for mortality after cardiac surgery lose precision within a few years after development. Recalibrating old models and creating new models (i.e., remodeling) are equally good strategies for predicting outcomes in contemporary patient cohorts.

Pediatric en bloc kidney transplantation to adult recipients: more than suboptimal?

BACKGROUND: To optimize available organs, kidneys from young donors traditionally believed to be suboptimal are transplanted to adults. The purpose of this study is to compare graft survival (GS) of en bloc kidney (EBK) from young pediatric donors to other deceased donor transplants in adult recipients. METHODS: We analyzed United Network of Organ Sharing/STAR data on primary deceased donor kidney transplants to adult recipients (1988-2006). EBK (age younger than 5 years, n=1696) was compared with solitary pediatric (SP; age younger than 5 years) kidneys (n=1502), and matched standard adult donors (age 18-59 years, n=9594) and expanded criteria donor (ECD; n=6396). The adjusted GS was obtained using Cox proportional hazard model and hazard ratios were calculated. RESULTS: EBK had lowest acute rejection rates (6.0%) but similar to standard adult transplants (6.3%), and lower than SP and ECD (9.0% and 8.2%; P<0.0001). Delayed graft function rates were lowest in EBK (17.9%), highest in ECD (34.8%; P<0.0001), and similar among SP and standard adult transplants (24.4% and 24.2%). The estimated glomerular filtration rate (eGFR) was best in EBK and worst in ECD (P<0.0001). The eGFR of EBK and SP transplants continuously improved but the eGFR of standard adult and ECD declined. Graft loss was higher in EBK and SP transplants than adult donor transplants during the first 6 months. Despite the highest thrombosis rates in EBK (5.0%) (SP, 3.3%; standard adult, 1.8%; ECD, 2.0%, P<0.0001), GS of EBK became similar to standard adult donor transplants by 5 years and best at 10 years posttransplant (64.0%) and worst in ECD (39.6%; P<0.0001). CONCLUSION: EBK had the best long-term outcomes among deceased donor transplants and offer unique options for adult kidney transplant recipients.

Predicting major adverse cardiac events after percutaneous coronary intervention: the Texas Heart Institute risk score.

BACKGROUND: Many models have been devised in the past to predict adverse outcomes after PCI, but with rapid advancements in this field, a new risk-prediction model may be needed. The purpose of our study was to identify the clinical and angiographic variables associated with adverse cardiac events after percutaneous coronary intervention (PCI) and to construct a simple bedside tool for risk stratification of PCI patients. METHODS: Using our institution's database, we analyzed data from 9,494 patients who underwent PCI between January 1, 1996, and December 31, 2002 (ie, during the bare-metal stent era). Predictors of major adverse cardiac events--death, myocardial infarction, stroke, and repeat revascularization by emergent coronary artery bypass grafting or PCI--were identified by multivariate logistic regression analysis using baseline clinical, angiographic, and procedural variables. A simple integer score was constructed by multiplying the beta coefficient for each variable by a constant and rounding the result to the nearest integer. The score was validated in 5,545 patients who underwent PCI between January 1, 2003, and December 31, 2006 (ie, during the drug-eluting stent era). RESULTS: Multivariate regression analysis identified emergent procedure, urgent procedure, unstable angina, acute myocardial infarction, renal insufficiency, hypertension, congestive heart failure, peripheral vascular disease, type C lesion, presence of thrombus, and number of stents placed as independent predictors of adverse events after PCI. The model had good overall discrimination (area under the receiver operator characteristic curve 0.701), and the model fitted the validation cohort adequately. CONCLUSIONS: Risk of complications after PCI can be assessed with this simple tool, which may permit comparisons between different operators as well as different hospitals.

Lower gastrointestinal bleeding: association with Sevelamer use.

Stercoral ulceration results from impaction of hard fecal mass on the colonic wall and is a relatively unknown cause of lower gastrointestinal bleeding. In this report, we describe a case of lower gastrointestinal bleeding due to stercoral ulceration resulting from Sevelamer, a drug which is commonly associated with constipation.

Hans Gunther and his disease.

Congenital erythropoietic porphyria (Gunther's disease) is one of the least common porphyrias. This article describes the life and career of Hans Gunther (after whom the disease is named), his contributions to the field of porphyrias and the current understanding of Gunther's disease.

Effect of hemodialysis on cognitive function in ESRD patients.

BACKGROUND: Uremia is associated with impairment of different cognitive functions. However the pathogenesis of this cognitive dysfunction is unknown. OBJECTIVE: In this study, long-latency event related potentials (ERPs) were used to assess changes in cortical function due to hemodialysis treatment. METHODS: In this cross-sectional study, we measured event related potentials in 15 end stage renal disease (ESRD) patients maintained on hemodialysis, two hours before and two hours after they underwent hemodialysis and compared their data with a strictly age and sex matched healthy control group. The P3 was elicited by using standard auditory "odd-ball" paradigm and the data obtained was statistically analyzed (Wilcoxon signed ranks, Mann Whitney). RESULTS: Before hemodialysis, the patients' P3 latency (347.73 +/- 39.47 ms) was significantly increased as compared with that of healthy control group (308.4 +/- 13.73 ms) (p = 0.001). After hemodialysis, P3 latency of the patients showed a significant decrease (347.73 +/- 39.47 ms to 325.20 +/- 37.15 ms, p = 0.001). P3 latency after dialysis was not significantly different from controls. No significant correlation was noted between various biochemical parameters (hemoglobin, blood urea, creatinine, uric acid and calcium) and P3 latency or amplitude. CONCLUSIONS: Removal of uremic toxins by hemodialysis leads to an improvement in cognitive processing.

Cognitive impairment in chronic kidney disease.

BACKGROUND: Although end-stage renal disease (ESRD) has been associated with cognitive impairment, the relation between lesser degrees of chronic kidney disease (CKD) and cognitive impairment is less well understood. The objective of this study was to assess the cognitive function in patients with varying severity of CKD using P3 event-related potentials (P3ERPs). METHODS: In this cross-sectional study, 15 neurologically asymptomatic (Mini Mental State Examination >24) patients each of CKD stage 3, 4 and 5 (undialysed) were enrolled. Besides this, 15 healthy controls were also studied. All groups were age and sex matched. Glomerular filtration rate (GFR) was estimated using the Modification of Diet in Renal Disease equation. The P300 was studied in all subjects by using standard auditory 'odd-ball' paradigm and the data obtained were statistically analysed. RESULTS: We noted significant prolongation of P300 latencies as severity of CKD increased from stage 3 (318.8 +/- 28.98 ms) to stage 4 (357 +/- 24.65 ms) (P < 0.002) and from stage 4 to 5 (388.47 +/- 31.67 ms) (P < 0.01). P300 latency in CKD stage 3 was not found to be significantly different from controls (308.4 +/- 13.73 ms). Significant positive correlation was noted between serum creatinine, blood urea and uric acid with P3 latency. Significant negative correlation was noted between GFR, serum calcium and haemoglobin with P3 latency. CONCLUSIONS: Increasing severity of CKD is associated with progressive cognitive decline and this may have important clinical consequences.