A Review of Selected Adult Congenital Heart Diseases Encountered in Daily Practice.

The advancement in corrective surgical procedures and anaesthesia technology has resulted in the increased survival of patients with Congenital Heart Diseases (CHD). Most of the surviving CHD patients have successfully reached adulthood and those surviving adults now outnumber the infants born with the CHD. Unfortunately, the surviving adults with CHD do not get proper care due to either inconsistent follow-up or not getting care from a specialist in the field of CHD. It is imperative for general practicing clinicians to be aware of the congenital diseases as well as the current clinical recommendations. This manuscript reviews some of the common congenital diseases seen in adults such as cardiac shunts, left heart obstructive lesions, and aortopathies.

The Significance of Preoperative Serum Sodium and Hemoglobin in Outcomes of Upper Tract Urothelial Carcinoma: Multi-Center Analysis Between China and the United States.

PURPOSE: To analyze the effect of preoperative serum sodium and hemoglobin on oncologic outcomes in upper tract urothelial carcinoma (UTUC) based on a multi-center cohort from China and the United States (U.S.). METHODS: We retrospectively reviewed the records of 775 patients with UTUC treated surgically at tertiary care medical facilities in China or the US from 1998 to 2015. We analyzed associations of preoperative serum sodium and hemoglobin with clinicopathological characteristics, overall survival (OS), cancer-specific survival (CSS) and intravesical recurrence free survival (IVRFS). RESULTS: The US patients had comparatively lower serum sodium and similar hemoglobin at baseline. Preoperative low serum sodium value was associated with tumor multifocality, lymph node metastasis (LNM) and lymphovascular invasion (LVI); preoperative anemia was associated with advanced age, tumor multifocality, high tumor grade and LVI. Preoperative low serum sodium was an independent predictor of worse OS in the entire cohort; preoperative anemia was an independent predictor of worse OS and CSS in the US cohort alone, Chinese cohort alone and the combined cohort. We developed a predictive nomogram for OS which exhibited better prognostic value when it included the values of sodium and anemia, and successfully validated it in different cohorts. CONCLUSION: Preoperative low serum sodium and anemia could be informative in predicting worse pathologic and survival outcomes in different UTUC patient ethnic groups.

Predicted versus observed 30-day perioperative outcomes using the ACS NSQIP surgical risk calculator in patients undergoing partial nephrectomy for renal cell carcinoma.

PURPOSE: The purpose of the study was to evaluate the accuracy of the American College of Surgeons NSQIP Surgical Risk Calculator for predicting risk-adjusted 30-day outcomes for patients undergoing partial nephrectomy (PN) for renal cell carcinoma (RCC). METHODS: A single institution, multi-surgeon, prospectively maintained database was queried for patients undergoing PN for RCC from 1998 to 2015. 21 preoperative factors were analyzed for each patient with predicted risk for 30-day complications, mortality, and length of stay (LOS) calculated. Differences between the mean predicted risk and observed rate of surgical outcomes were determined using two-sided one-sample t test with significance at p < 0.05. Subgroup analyses of outcomes stratified by surgical approach were also performed. RESULTS: 470 patients undergoing PN for RCC were analyzed. Comparing NSQIP predicted to observed outcomes, clinically significant underestimations occurred with rates of overall complications (9.16 vs. 16.81%, p < 0.001), surgical site infections [SSI] (1.65 vs. 2.77%, p < 0.001), urinary tract infection [UTI] (1.41 vs. 3.40%, p < 0.001), and LOS (3.25 vs. 3.73 days, p < 0.001). On subgroup analysis, 209 open PN and 261 minimally invasive PN (MIPN) were performed. The NSQIP calculator consistently underestimated overall complications, SSI, UTI, and LOS (p < 0.001) among both surgical approaches, while overestimating MIPN severe complications (p < 0.001). Clinically important differences persisted when stratifying the MIPN group by laparoscopic (N = 111) and robotic (N = 150) approaches. CONCLUSIONS: The ACS NSQIP Surgical Risk Calculator had significant discrepancies among observed and predicted outcomes. Additional analyses confirmed these differences remained significant irrespective of surgical approach. These findings emphasize the need for urologic oncology-specific calculators to better predict surgical outcomes in this complex patient population.

A Multi-Institutional Comparison of Clinicopathological Characteristics and Oncologic Outcomes of Upper Tract Urothelial Carcinoma in China and the United States.

PURPOSE: We evaluated differences in clinicopathological characteristics and oncologic outcomes between patients with upper tract urothelial carcinoma in mainland China and the United States. MATERIALS AND METHODS: We retrospectively compiled clinicopathological and oncologic outcomes data on patients with upper tract urothelial carcinoma treated surgically at tertiary care medical facilities in the United States or China from 1998 to 2015. Baseline demographics, comorbidities and pathological features were evaluated. Oncologic end points, including intravesical recurrence and cancer specific survival, were obtained after excluding patients who received systemic chemotherapy. Multivariable Cox regression was performed to determine predictors of adverse oncologic outcomes for each country. RESULTS: A total of 775 patients with upper tract urothelial carcinoma were identified, including 451 in China and 324 in the United States. Median followup was 42 months. American patients were more frequently male (65% vs 44%) and smokers (79% vs 18%), and had a worse mean ASA® (American Society of Anesthesiologists®) score (2.7 vs 2.2) and prior bladder cancer (41% vs 4%, all p <0.001). Chinese patients more often had preoperative hydronephrosis (56% vs 40%), high grade pathology (98% vs 77%), muscle invasion (64% vs 38%) and nodal metastases (26% vs 6%, all p <0.001). American patients had worse overall survival on Kaplan-Meier analysis (p = 0.049). However, country of origin did not predict local relapse or cancer specific survival. CONCLUSIONS: Patient and disease characteristics of upper tract urothelial carcinoma differed between the Chinese and American cohorts. Chinese patients appeared relatively healthier at presentation but more often exhibited adverse pathological features. While evaluation and management patterns may account for these variations, the pathological findings may reflect a differential underlying pathogenesis of disease. Additional study is warranted to further characterize these differences.

Kidney function outcomes following thermal ablation of small renal masses.

The diagnosis of small renal masses (SRMs) continues to increase likely attributable to widespread use of axial cross-sectional imaging. Many of these SRMs present in elderly patients with abnormal baseline renal function. Such patients are at risk for further decline following therapeutic intervention. Renal thermal ablation presents one approach for management of SRMs whereby tumors are treated in situ without need for global renal ischemia. These treatment characteristics contribute to favorable renal function outcomes following kidney tumor ablation particularly in patients with an anatomic or functional solitary renal unit.

Complications Following Radical Nephroureterectomy.

Radical nephroureterectomy (RNU) is the gold standard treatment strategy for bulky, high-grade, or muscle-invasive upper tract urothelial carcinoma (UTUC). Many patients with UTUC who require RNU are elderly, comorbid, and at risk for perioperative complications. Recognition of likelihood and extent of such complications guides preoperative counseling, decision-making process for major surgery, and perioperative care. A critical review of such data is essential, given the inevitable impact of complications on hospital duration, need for readmission, resource utilization, and costs associated with management.

Surgical management of bladder urothelial carcinoma with squamous differentiation.

BACKGROUND: Urothelial carcinoma (UC) with squamous differentiation (UC w/SD) is the most common variant bladder cancer histology. MAIN FINDINGS: Accurate identification at the time of transurethral resection is critical although current barriers exist, which include tumor heterogeneity, sampling limitation during resection, and pathologic interpretation of specimens. Although many cases of UC w/SD present with muscle-invasive bladder cancer, those cancers that are confirmed to be truly non-muscle invasive can be managed with endoscopic resection, adjuvant intravesical therapies (i.e., Bacillus Calmitte Guerin), and close surveillance. Radical cystectomy series suggest that UC w/SD tends to present at a more advanced stage than pure UC does although survival outcomes are similar when controlling for standard clinicopathologic factors. PRINCIPAL CONCLUSIONS: Future basic science and clinical studies are requisite to better investigate the biology of urothelial carcinoma with squamous differentiation and response to multimodal therapies.

Yield of esophagogastroduodenoscopy and colonoscopy in cancer of unknown primary.

OBJECTIVES: Carcinoma of unknown primary origin (CUP) is heterogeneous group of cancers. Role of gastrointestinal (GI) endoscopy in this entity is under investigated. Aim of this study was to evaluate yield of Colonoscopy and Esophagogastroduodenoscopy (EGD) in localizing primary tumor in patients with CUP. METHODOLOGY: Patients with histopathologically proven CUP who underwent colonoscopy / EGD to find the primary tumor from December 2009 to December 2011 were included in the study. Abdominal symptoms and cytokeratin (CK) 7 and 20 markers were correlated with presence of primary in GI tract. RESULTS: After giving informed consent 86 patients were included in final analysis. All patients underwent colonoscopy while 60(70%) got EGD along with colonoscopy. Mean age was 55.10 +/-11.94 years with 52(60%) male. Abdominal symptoms were present in 50%. CK7+/CK20- in 34(40%); CK7-/CK20+ in 2(2%) while CK7+/20+ in 7(8%) of metastatic tumor samples. Liver was metastatic site in 47(55%), Lymph node 12(14%) and Ascites in 8(9%). Endoscopy detected primary in 6 (7%) patients with 3 each in stomach and colon. No association of abdominal symptoms and cytokeratin markers was found with presence of GI primary site. CONCLUSION: Yield of localizing primary lesion in the GI tract by pan-endoscopy was limited. Abdominal symptoms and cytokeratin markers do not predict presence of gastrointestinal malignancies.

Bilateral endoscopic inguinofemoral lymphadenectomy using simultaneous carbon dioxide insufflation: an initial report of a novel approach.

Inguinal lymphadenectomy plays a critical role in the diagnosis and treatment of several neoplastic diseases. Frequently, bilateral lymphadenectomy is undertaken for staging and/or treatment of genitourinary cancers. Our objective was to determine if bilateral endoscopic lymphadenectomy could be performed simultaneously, in an effort to decrease overall anesthetic and operative time. This was accomplished by utilizing two carbon dioxide insufflators concurrently. This approach requires careful positioning of the patient, surgical team, and instrumentation, as well as special anesthetic considerations necessary to avoid severe hypercarbia. Simultaneous bilateral endoscopic inguinal lymphadenectomy is a technically feasible and efficient surgical approach.

Designing a balanced scorecard for a tertiary care hospital in Pakistan: a modified Delphi group exercise.

Balanced Scorecards (BSC) are being implemented in high income health settings linking organizational strategies with performance data. At this private university hospital in Pakistan an elaborate information system exists. This study aimed to make best use of available data for better performance management. Applying the modified Delphi technique an expert panel of clinicians and hospital managers reduced a long list of indicators to a manageable size. Indicators from existing documents were evaluated for their importance, scientific soundness, appropriateness to hospital's strategic plan, feasibility and modifiability. Panel members individually rated each indicator on a scale of 1-9 for the above criteria. Median scores were assigned. Of an initial set of 50 indicators, 20 were finally selected to be assigned to the four BSC quadrants. These were financial (n = 4), customer or patient (n = 4), internal business or quality of care (n = 7) and innovation/learning or employee perspectives (n = 5). A need for stringent definitions, international benchmarking and standardized measurement methods was identified. BSC compels individual clinicians and managers to jointly work towards improving performance. This scorecard is now ready to be implemented by this hospital as a performance management tool for monitoring indicators, addressing measurement issues and enabling comparisons with hospitals in other settings.

Randomized trial of warfarin, aspirin, and clopidogrel in patients with chronic heart failure: the Warfarin and Antiplatelet Therapy in Chronic Heart Failure (WATCH) trial.

BACKGROUND: Chronic heart failure remains a major cause of mortality and morbidity. The role of antithrombotic therapy in patients with chronic heart failure has long been debated. The objective of this study was to determine the optimal antithrombotic agent for heart failure patients with reduced ejection fractions who are in sinus rhythm. METHODS AND RESULTS: This prospective, randomized clinical trial of open-label warfarin (target international normalized ratio of 2.5 to 3.0) and double-blind treatment with either aspirin (162 mg once daily) or clopidogrel (75 mg once daily) had a 30-month enrollment period and a minimum of 12 months of treatment. We enrolled 1587 men and women >/=18 years of age with symptomatic heart failure for at least 3 months who were in sinus rhythm and had left ventricular ejection fraction of

Endoluminal full-thickness plication and radiofrequency treatments for GERD: an outcomes comparison.

HYPOTHESIS: Endoluminal therapies have emerged as adjuncts for the treatment of gastroesophageal reflux disease (GERD) in select patients. OBJECTIVE: To compare the effectiveness of endoscopic full-thickness plication and endoscopic radiofrequency treatments for patients with GERD. PATIENTS: A total of 126 patients who underwent either endoscopic full-thickness plication (FTP) of the gastric cardia or endoscopic radiofrequency (RF) treatment of the esophagogastric junction during a 4-year period were included (68 underwent RF and 58 underwent FTP). INTERVENTIONS: Follow-up data was obtained for 51% of patients (mean follow-up, 6 months). MAIN OUTCOME MEASURES: Comparison of medication use, symptom scores, and pH values at baseline and follow-up. RESULTS: In the RF group, patients with moderate to severe heartburn decreased from 55% to 22% (P < .01), and proton pump inhibitor (PPI) use decreased from 84% to 50% (P = .01). Decreases were also seen for dysphagia, voice symptoms, and cough. Percentage of time the pH was less than 4 was unchanged. In the FTP group, patients with moderate to severe heartburn decreased from 53% to 43% (P = .3), and PPI use decreased from 95% to 43% (P = .01). Percentage of time the pH was less than 4 decreased from 10.0% to 6.1% (P = .05). Decreases were also seen for regurgitation, voice symptoms, and dysphagia. There was no change in scores for chest pain or asthma in either group. CONCLUSIONS: For patients with GERD, RF and FTP both resulted in a decrease in both PPI use and in scores for voice symptoms and dysphagia. In addition, RF resulted in decreased heartburn and cough, while FTP resulted in the most dramatic reduction in regurgitation. Our experience indicates that both procedures are effective, providing symptomatic relief and reduction in PPI use. For patients whose chief complaint is regurgitation, FTP may be the preferred procedure.

Cardiorespiratory fitness and short-term complications after bariatric surgery.

BACKGROUND: Morbid obesity is associated with reduced functional capacity, multiple comorbidities, and higher overall mortality. The relationship between complications after bariatric surgery and preoperative cardiorespiratory fitness has not been previously studied. METHODS: We evaluated cardiorespiratory fitness in 109 patients with morbid obesity prior to laparoscopic Roux-en-Y gastric bypass surgery. Charts were abstracted using a case report form by reviewers blinded to the cardiorespiratory evaluation results. RESULTS: The mean age (+/- SD) was 46.0 +/- 10.4 years, and 82 patients (75.2%) were female. The mean body mass index (BMI) was 48.7 +/- 7.2 (range, 36.0 to 90.0 kg/m(2)). The composite complication rate, defined as death, unstable angina, myocardial infarction, venous thromboembolism, renal failure, or stroke, occurred in 6 of 37 patients (16.6%) and 2 of 72 patients (2.8%) with peak oxygen consumption (Vo(2)) levels < 15.8 mL/kg/min or > 15.8 mL/kg/min (lowest tertile), respectively (p = 0.02). Hospital lengths of stay and 30-day readmission rates were highest in the lowest tertile of peak Vo(2) (p = 0.005). There were no complications in those with BMI < 45 kg/m(2) or peak Vo(2) > or= 15.8 mL/kg/min. Multivariate analysis adjusting for age and gender found peak Vo(2) was a significant predictor of complications: odds ratio, 1.61 (per unit decrease); 95% confidence interval, 1.19 to 2.18 (p = 0.002). CONCLUSIONS: Reduced cardiorespiratory fitness levels were associated with increased, short-term complications after bariatric surgery. Cardiorespiratory fitness should be optimized prior to bariatric surgery to potentially reduce postoperative complications.

The effects of beta blockers on morbidity and mortality in heart failure.

Beta-adrenergic receptor blockers are effective in reducing morbidity and mortality in heart failure. These drugs slow the progression of heart failure by modifying the remodeling process, prevent or delay need for hospital admission for heart failure. They reduce both pump failure and sudden arrhythmic death. There are many patients that do not receive beta blocker therapy in the heart failure population. Patients are often treated with beta blockers not tested in clinical trials to demonstrate efficacy in this patient population. Results from the recently published trials are summarized to emphasize the proper selection and dosage of beta blocker therapy to optimize the care of this high risk population.

The Warfarin and Antiplatelet Therapy in Heart Failure trial (WATCH): rationale, design, and baseline patient characteristics.

BACKGROUND: The role of anticoagulation in patients with chronic heart failure has long been an area of interest and controversy. Traditionally the goal of anticoagulation has been to prevent embolic events, but recent trials also demonstrated that oral anticoagulation also prevents vascular events in patients with prior myocardial infarction, who constitute the majority of heart failure patients. Although antiplatelet agents also reduce postinfarction vascular events, few data are available in heart failure patients, and some evidence suggests that aspirin may also have the potential to worsen heart failure morbidity and mortality, possibly by interfering with the effects of angiotensin-converting enzyme inhibitors. Methods and results The Warfarin and Antiplatelet Therapy in Chronic Heart Failure (WATCH) trial was undertaken to determine the optimal antithrombotic agent for heart failure patients. WATCH was a prospective-randomized trial in which symptomatic heart failure patients in sinus rhythm with ejection fractions < or =35% taking angiotensin-converting enzyme inhibitors (unless not tolerated) and diuretics were randomized to open-label warfarin (target International Normalized Ratio 2.5-3.0) or double-blind antiplatelet therapy with aspirin 162 mg or clopidogrel 75 mg. Two primary comparisons were specified: anticoagulation with warfarin versus antiplatelet therapy with aspirin and antiplatelet therapy with clopidogrel versus antiplatelet therapy with aspirin. The primary outcome is the composite of death from all causes, nonfatal myocardial infarction, and nonfatal stroke analyzed as time to first event using the intent-to-treat approach. The secondary endpoint was the broader composite of death from all causes, nonfatal myocardial infarction, non-fatal stroke, and hospitalizations for worsening heart failure, unstable angina pectoris, and systemic or pulmonary artery embolic events. Additional prespecified analyses include heart failure events, coronary events, and resource utilization. CONCLUSIONS: Although the trial was designed to enter 4500 patients, it was terminated 18 months prematurely in June 2003 by the VA Cooperative Study Program because of poor enrollment with a resulting reduction of its power to achieve its original objective. This manuscript describes the study rationale, protocol design, and the baseline characteristics of the 1587 patients who were entered into the study. The WATCH trial will help define the optimal approach to antithrombotic therapy in the contemporary management of patients with chronic heart failure resulting from left ventricular systolic dysfunction.

Periprocedural thromboprophylaxis in patients receiving chronic anticoagulation therapy.

Patients receiving chronic anticoagulation therapy pose a clinical challenge when therapy needs to be interrupted for surgical or invasive procedures. Maintaining anticoagulation places them at risk for serious bleeding complications, whereas discontinuing anticoagulation puts them at risk of thromboembolic complications. Most patients can undergo dental procedures, cataract surgery, and diagnostic endoscopy without discontinuing anticoagulation. The main patient groups that may require a periprocedural alternative to oral anticoagulation (periprocedural thromboprophylaxis or bridging) include patients with prosthetic heart valves, atrial fibrillation, and hypercoagulable states and patients with chronic venous thrombosis who are undergoing surgery. Currently, there is little consensus on the appropriate perioperative treatment of patients on long-term warfarin therapy. There are an increasing number of studies that evaluate the benefits of periprocedural bridging with low-molecular-weight heparin (LMWH) in place of unfractionated heparin (UFH). An advantage of LMWH over UFH is that perioperative conversion from warfarin therapy with LMWH can be carried out in the outpatient setting, which is more convenient for patients and is cost effective. As with the use of UFH, there are reports of maternal thromboembolic complications with LMWHs in pregnant women with mechanical heart valves. This review brings together the available data on periprocedural bridging to assess the available options for patients on long-term warfarin therapy who are undergoing surgical procedures. It provides a rationale for using LMWHs while individualizing the risks versus benefits in a given patient population.

Periprocedural management of anticoagulation in patients on extended warfarin therapy.

Patients receiving chronic anticoagulation therapy pose a clinical challenge when therapy needs to be interrupted for surgical or invasive procedures. Maintaining anticoagulation places them at risk of serious bleeding complications, whereas discontinuing anticoagulation puts them at risk of thromboembolic complications. The main patient groups that may require a periprocedural alternative to oral anticoagulation include patients with prosthetic heart valves, atrial fibrillation, and hypercoagulable states and those with chronic venous thrombosis undergoing surgery. Currently, there is little consensus on appropriate perioperative management of patients on long-term warfarin therapy. This article is an attempt to bring together all the available data on periprocedural bridging to assess the available options for patients undergoing surgical procedures and to provide a rationale for using low-molecular-weight heparins (LMWHs) while individualizing the risks versus benefits in a given patient population.