A case report of ventricular septal defect complicating transcatheter aortic valve implant for aortic regurgitation: novel complication and technical considerations.

BACKGROUND: Transcatheter aortic valve implantation (TAVI) has proven efficacy in the treatment of aortic stenosis (AS). Understandably, there is increasing enthusiasm for its use to treat aortic regurgitation (AR). However, there are significant anatomical differences between AS and AR which make TAVI for AR more complex. CASE SUMMARY: We present the case of technically challenging TAVI for severe AR, which was complicated by a traumatic ventricular septal defect (VSD) that required percutaneous closure. To our knowledge, this is the first published case of VSD post-TAVI for AR. DISCUSSION: This unanticipated complication highlights anatomical differences between TAVI use in AS and AR. Lack of aortic valve calcification and excessive annular compliance made stable deployment of a self-expanding valve extremely challenging. Despite device oversizing, repeated embolization of the prosthesis into the left ventricular outflow tract traumatized the interventricular septum.

Relationship between pulmonary hyperinflation and dyspnoea severity during acute exacerbations of cystic fibrosis.

BACKGROUND AND OBJECTIVE: Acute exacerbations of cystic fibrosis (CF) occur frequently throughout the course of the disease. Dyspnoea is the most common and distressing symptom experienced by patients during these episodes. We tested the hypothesis that pulmonary hyperinflation is an important determinant of dyspnoea severity during acute exacerbations. METHODS: We studied patients during an acute exacerbation of CF. Lung volumes, spirometry and dyspnoea scores were measured at Day 0, Day 7, at the end of treatment (EOT) and 14 days following the EOT. RESULTS: At the start of treatment, mean residual volume (RV)/total lung capacity (TLC) was 54.9%, which decreased significantly with treatment, as did vital capacity (VC), inspiratory capacity (IC) and dyspnoea scores. IC was the only independent predictor of dyspnoea severity. CONCLUSION: Our study demonstrates significant improvements in hyperinflation, spirometry and dyspnoea scores with treatment of acute exacerbations of CF. Hyperinflation, rather than airflow limitation, may contribute towards the increased dyspnoea during exacerbations.

LV Apical Rupture Complicating Acute Myocardial Infarction: The Role of CMR.

PURPOSE: This case illustrates an acute myocardial infarction with occlusion of the left anterior descending coronary artery complicated by apical ventricular rupture and apical thrombus. PROCEDURES: An electrocardiogram, transthoracic echocardiogram (TTE), coronary angiography and cardiac magnetic resonance imaging (CMR) guided optimal management of the patient. FINDINGS: Coronary angiography revealed multivessel disease with an ostial occlusion of the LAD. Echocardiography showed apical dilatation of the left ventricle with a large, echogenic mass at the apex. Contrast echocardiography confirmed the presence of a large apical thrombus, separated from the LV cavity by myocardium. A CMR showed a completed LAD infarct and a filling thrombus was noted in the aneurysmal apical region inferring a contained rupture of the LV apex. PRINCIPLE CONCLUSIONS: Accurate and definitive delineation of unusual cardiac anatomy is best provided by complementary multimodality cardiac imaging, echocardiography and CMR. TTE can miss LV thrombi, particularly when they are large, aneurysmal and apical in nature. CMR provides the cardiac surgeon the ability to visualise in 3D the functional and morphological abnormalities, helping guide necessary intervention. Optimal management of patients with ventricular rupture remains controversial both in terms of timing and choice of intervention.

Life expectancy for community-based patients with heart failure from time of diagnosis.

AIMS: Heart failure has been demonstrated in previous studies to have a dismal prognosis. However, the modern-day prognosis of patients with new onset heart failure diagnosed in the community managed within a disease management programme is not known. The purpose of this study is to report on prognosis of patients presenting with new onset heart failure in the community who are subsequently followed in a disease management program. METHODS AND RESULTS: A review of patients referred to a rapid access heart failure diagnostic clinic between 2002 and 2012 was undertaken. Details of diagnosis, demographics, medical history, medications, investigations and mortality data were analysed. A total of 733 patients were seen in Rapid Access Clinic for potential new diagnosis of incident of heart failure. 38.9% (n=285) were diagnosed with heart failure, 40.7% (n=116) with HF-REF and 59.3% (n=169) with HF-PEF. There were 84 (29.5%) deaths in the group of patients diagnosed with heart failure; 41 deaths (35.3%) occurred in patients with HF-REF and 43 deaths (25.4%) occurred in patients with HF-PEF. In patients with heart failure, 52.4% (n=44) died from cardiovascular causes. 63.8% of HF patients were alive after 5 years resulting on average in a month per year loss of life expectancy over that period compared with aged matched simulated population. CONCLUSIONS: In this community-based cohort, the prognosis of heart failure was better than reported in previous studies. This is likely due to the impact of prompt diagnosis, the improvement in therapies and care within a disease management structure.

Dilated cardiomyopathy and progressive familial intrahepatic cholestasis.

This case is of a 29-year-old man with progressive familial intrahepatic cholestasis type 1 also known as Byler's disease. At the age of 21, our patient developed non-ischaemic dilated cardiomyopathy. Cardiac MRI demonstrated global wall thinning, with significant areas of myocardial fibrosis in the mid and epicardial walls from base to apex on postgadolinium late contrast enhanced images. No shared genetic loci between dilated cardiomyopathy and Byler's or cholestatic liver disease have yet been found. This presents the first documented case of non-ischaemic dilated cardiomyopathy, with evidence of mid wall fibrosis, in association with an established diagnosis of progressive familial intrahepatic cholestasis type 1 since childhood.

Imaging focal and interstitial fibrosis with cardiovascular magnetic resonance in athletes with left ventricular hypertrophy: implications for sporting participation.

Long-term high-intensity physical activity is associated with morphological changes, termed as the 'athlete's heart'. The differentiation of physiological cardiac adaptive changes in response to high-level exercise from pathological changes consistent with an inherited cardiomyopathy is imperative. Cardiovascular magnetic resonance (CMR) imaging allows definition of abnormal processes occurring at the tissue level, including, importantly, myocardial fibrosis. It is therefore vital in accurately making this differentiation. In this review, we will review the role of CMR imaging of fibrosis, and detail CMR characterisation of myocardial fibrosis in various cardiomyopathies, and the implications of fibrosis. Additionally, we will outline advances in imaging fibrosis, in particular T1 mapping. Finally we will address the role of CMR in pre-participation screening.

Extramedullary relapse of IgA-lambda myeloma after recent bortezomib therapy: a case report.

Intracranial plasmacytomas are an uncommon presentation of extramedullary relapse of multiple myeloma. The optimal management of extramedullary plasmacytomas remains unclear, with initial reports of bortezomib showing promising clinical results. We describe a case of multiple extracellular, including intracranial, plasmacytoma, with no evidence of marrow involvement, in a patient with relapsed IgA multiple myeloma. To our knowledge, this is the first reported case of a patient with rapid extramedullary relapse of disease despite recent exposure to bortezomib and dexamethasone.

Sentinel node biopsy for the individualization of surgical strategy for cure of early-stage colon cancer.

INTRODUCTION: The requirement for nodal analysis currently confounds the oncological propriety of focused purely endoscopic resection for early-stage colon cancer and complicates the evolution of innovative alternatives such as natural orifice transluminal endoscopic surgery (NOTES) and its hybrids. Adjunctive sentinel node biopsy (SNB) deserves consideration as a means of addressing this shortfall. METHODS: Data from two prospectively maintained databases established for multicentric studies of SNB in colon cancer that employed similar methodologies were pooled to establish technique potency selectively in T1/T2 disease (both overall and under optimized conditions) and to project potential clinical impact. RESULTS: Of 891 patients with T1-4, M0 intraperitoneal colon cancer, 225 had T1/T2 disease. Sentinel nodes were either not found or were falsely negative in 18 patients with T1/T2 cancers (8%) as compared with 17% (112/646) in those with T3/T4 disease (P = 0.001). Negative predictive value (NPV) in the former exceeded 95%, while sensitivity [including immunohistochemistry (IHC)] was 81%. In the 193 patients with T1/T2 disease recruited from those centers contributing >22 patients, sensitivity was 89% and NPV 97%. Thus, in this cohort, SNB could have correctly prompted localized resection (obviating en bloc mesenteric dissection) in 75% (144) of patients, including 59 with T1 lesions potentially amenable to intraluminal resection alone as their definitive treatment. Forty-four patients (23.4%) would still have conventional resection, leaving three patients (1.6% overall) understaged (11% false-negative rate). CONCLUSION: These findings support the further investigation of SNB as oncological augment for localized resective techniques. Specific prospective study should pursue this goal.

Androgens, exercise capacity, and muscle function in cystic fibrosis.

BACKGROUND: It is well established that muscle strength and exercise capacity are impaired in adults with cystic fibrosis (CF). Numerous factors have been implicated in this process including nutritional factors, disuse, and corticosteroid use. It is also known that serum testosterone levels are frequently low in adolescent male patients with CF. As testosterone is known to have an effect on peripheral muscle, we aimed to assess the relationship among circulating androgen levels, muscle strength, and exercise capacity in adults with CF. METHODS: A prospective observational study was performed. Participants underwent maximal exercise testing and peripheral muscle strength assessment (quadriceps, shoulder flexion, handgrip, maximal inspiratory pressure, and maximal expiratory pressure). Venous blood samples were obtained for the measurement of total testosterone, sex hormone-binding globulin, and albumin. The free testosterone (FT) level was calculated. RESULTS: A total of 15 subjects completed the study. The patients who were studied had impaired muscle strength and exercise capacity but normal mean androgen levels. Quadriceps strength, SF, and peak oxygen uptake (Vo(2)max) were 40.1 +/- 9.4%, 59.3 +/- 20.3%, and 77.4 +/- 22.2% predicted, respectively. One patient had a low testosterone level, with low FT levels evident in two subjects. There was no correlation between testosterone or FT level with any of the muscle strengths calculated. Neither testosterone or FT level was correlated with exercise capacity or pulmonary function. CONCLUSIONS: Male CF patients with impaired skeletal muscle strength and exercise capacity have normal testosterone levels. Hypogonadism was rare in this group of adult men with CF. These findings would not support the role of testosterone in muscle dysfunction or impaired exercise capacity in CF patients.

Prediction of calculated future cardiovascular disease by monocyte count in an asymptomatic population.

INTRODUCTION: Although atherogenesis is clearly entwined with systemic inflammation, the risk-predictive relationship between preclinical and overt cardiovascular disease (CVD) and systemic white blood cell (WBC) subtypes remains unclear. Implication of an association would greatly facilitate cardiac risk prediction, assessment and monitoring. METHODS: 1383 asymptomatic individuals (795 men, 588 women) attending for executive health screening were examined clinically as well as with phlebotomy and exercise stress testing to determine their ten-year risk of developing overt cardiovascular disease (as estimated by both Framingham and SCORE calculations). The significance of their association with overall WBC and subtypes were determined using both univariate and multiple regression modeling. RESULTS: OF ALL WBC SUBTYPES, MONOCYTE COUNT WAS FOUND TO HAVE THE STRONGEST, INDEPENDENT RELATIONSHIP WITH OVERALL CVD RISK BY BACKWARDS LINEAR REGRESSION MODELING (FRAMINGHAM: beta = 0.057; p = 0.03; SCORE: beta = 0.128; p = <0.0005). Independent associations with BMI (beta = 5.214; p = <0.0005), waist circumference (beta = 21.866; p = <0.0005), systolic blood pressure (beta = 10.738; p = 0.003), HDL cholesterol (beta = -0.639; p = <0.0005) and triglyceride concentrations (beta = 0.787; p = <0.0005) were also evident. Overall WBC along with neutrophils, lymphocytes and basophil subfractions were variably (but less strongly) associated with such dependents and outcome measures. CONCLUSIONS: In conclusion, monocyte count, a simple inexpensive test, may provide useful predictive cardiovascular risk information in asymptomatic individuals to inform and guide attempts at interrupting CVD development at a preclinical stage.

Simple adaptation of current abdominal aortic aneurysm screening programs may address all-cause cardiovascular mortality: prospective observational cohort study.

BACKGROUND: Population screening for abdominal aortic aneurysm (AAA) can be cost-justified by its impact on the incidence of emergency presentations with rupture. The objective of this prospective, cohort study was to determine whether the proposed framework can be further evolved to address all-cause cardiovascular mortality in the community by integrating full risk factor assessment. METHODS: Male volunteers aged >60 years attending for AAA screening by ultrasound also underwent full cardiovascular assessment via physician-administered health questionnaire, sphygmomanometry, anthromorphometry, and fasting phlebotomy for lipid and glucose profiling. Framingham and SCORE project evaluations were used to calculate 10-year risk of ischemic heart disease and all-cause fatal cardiovascular disease, respectively. RESULTS: A total of 481 men were screened, and 23 (4.8%) had an AAA detected (22% of which were >4 cm). Obesity, smoking, hypertension, impaired glucose metabolism, and hypercholesterolemia were notably frequent in those without an AAA. Mean 10-year cardiovascular risk in the population without AAA (6.89 and 6.74 by SCORE and Framingham estimations, respectively) was similar to those with AAA (7.78 and 7.04, respectively). Supplementary screening increased total costs by 2.5%. Reserving concomitant atherosclerotic testing for those with elevated systolic blood pressure at presentation allowed identification of 96%/95% of those with Framingham/SCORE risks >10%, respectively, thus saving 162 (33%) individuals from additional investigation and conserving the cost increment to 1.8% overall. CONCLUSION: The proven framework of population sifting for AAA provides a unique opportunity to also confront latent cardiovascular malady. Sphygmomanometry during ultrasonographic screening for AAA seems both cost-effective and readily incorporated within the current paradigm.

An examination of the relationship between normal range thyrotropin and cardiovascular risk parameters: a study in healthy women.

OBJECTIVE: To investigate the relationship between thyrotropin concentrations within the accepted reference range and cardiovascular risk. DESIGN: Initially, 728 women aged 45-60 years were enrolled over a 12-month period. All participants underwent full cardiovascular assessment, including detailed health questionnaire, sphygomanometry, body mass index (BMI) calculation, fasting glucose and lipid profiling, and measurement of serum thyrotropin concentrations. Patients whose thyrotropin concentrations were within the reference range (0.5-4.5 mU/L) were divided into quartiles (n = 629). The means of cardiovascular risk parameters between the first (n = 158) and fourth (n = 157) quartile were compared. Subsequently, the relationships between thyrotropin concentration and risk parameters for cardiovascular disease were examined. MAIN OUTCOME: This study demonstrates that, within the reference range, increasing thyrotropin concentrations are associated with increasing risk parameters for the development of cardiovascular disease. Subjects with thyrotropin concentrations within the uppermost quartile of the reference range had significantly increased waist circumference, BMI, glucose, triglyceride, and systolic blood pressure measurements when compared to those in the lowermost quartile. Furthermore, significant relationships between thyrotropin and waist circumference, BMI, and fasting glucose and triglycerides concentrations were demonstrated. Finally, independent relationships between thyrotropin and both fasting glucose and triglyceride concentrations were demonstrated. CONCLUSION: Within the reference range, increasing thyrotropin concentrations are associated with increasing cardiovascular risk parameters. Fasting glucose and triglycerides have been shown to be independently associated with thyrotropin concentration.