Addison's Disease and Dilated Cardiomyopathy: A Case Report and Review of the Literature.

Addison's disease is often accompanied by a number of cardiovascular manifestations. We report the case of a 30-year-old man who presented with a new onset dilated cardiomyopathy due to Addison's disease. The clinical presentation, treatment, and outcomes of this rare hormone mediated cardiac disorder are reviewed.

The Role of TEE in Diagnosing Hepatopulmonary Syndrome and Cryptogenic Cirrhosis.

In the vast majority of cases, ongoing hypoxemia in a cirrhotic patient is usually hepatopulmonary syndrome (HPS) until proven otherwise; in this case, HPS was suspected prior to any known diagnosis of cirrhosis. This is the first reported case in the literature whereby HPS and cirrhosis were diagnosed after the fact, rather than with the preexisting knowledge of liver cirrhosis.

Curriculum Development and Evaluation of a Hemodynamic Critical Care Ultrasound: A Systematic Review of the Literature.

OBJECTIVE: The application of ultrasound to assess a patient's cardiac function and volume status is becoming commonplace in the practice of critical care. These skills have been taught through varying curricula; however, no consensus on the optimal curriculum has been established. The purpose of this systematic review is to evaluate the literature regarding critical care ultrasound curriculum development and evaluation. DATA SOURCES: Studies were identified using MEDLINE, Embase, CINAHL, PsycInfo, the Cochrane Center Register of Controlled Trials, and ERIC according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines through June 2014. STUDY SELECTION: Included studies were limited to those that described adult (age, > 16 yr) cardiac or hemodynamic critical care ultrasound curricula for physicians. Two reviewers independently screened studies based on predetermined exclusion criteria, and disagreements were resolved by a third reviewer. DATA EXTRACTION: Data were abstracted, and quality was assessed by two reviewers using the Newcastle-Ottawa Scale. Data abstracted from the studies included the learner population, examination type, duration, composition, and setting of the curriculum, means of evaluation, and outcomes. DATA SYNTHESIS: The search yielded 654 studies; of which, 15 met inclusion criteria. All curricula used a combination of didactic and hands-on components. The highest agreement between novice and experts, coupled with the most time-efficient application, was achieved when the study was limited to a basic qualitative approach for the assessment of global function or contractility and assessment of inferior vena cava collapsibility. The mode of delivery seemed most efficient when a hybrid method was used, including online instruction. Minimum scanning competency may be achieved with 30 scans although more rigorous study on this element is necessary. CONCLUSIONS: Assessment of cardiac function and volume assessment is becoming an essential skill in critical care medicine. Physicians can be taught bedside echocardiography in a time-effective manner with positive benefit to patients by applying a concise curriculum with limited content.

Functional mitral regurgitation: current understanding and approach to management.

Functional mitral regurgitation (FMR) is a challenging clinical entity that frequently complicates ischemic and nonischemic cardiomyopathy. The underlying pathophysiology of FMR is caused primarily by ventricular and subvalvular apparatus dysfunction which causes failure of proper leaflet coaptation. Echocardiography is the primary modality used in diagnosis and characterization of FMR. Echocardiography allows for assessment of valvular and ventricular structures and their interaction. FMR portends a poor prognosis, because it is frequently associated with increased morbidity and mortality. The optimal management of FMR involves an individualized approach that incorporates medical therapy and consideration of surgical, percutaneous, and resynchronization therapies according to the severity of regurgitation, presence of symptoms, option for revascularization, and the degree of ventricular remodelling.

Cardiovascular risk assessment in the asymptomatic individual: a case-based approach.

Coronary artery disease remains the leading cause of mortality worldwide, and the identification of at-risk individuals represents a significant clinical challenge. In this review, a typical clinical case is presented, followed by a discussion of the cardiovascular risk assessment of such individuals. Specifically, the role of imaging modalities such as carotid intima-media thickness measurements and coronary artery calcium scoring is explored. Case resolution and recommendations are subsequently suggested.

Carotid intima-media thickness measurements are not affected by the ultrasound frequency.

OBJECTIVE: Carotid intima-media thickness (IMT) is a B-mode ultrasound measure of subclinical atherosclerosis predictive of future cardiovascular risk. Carotid IMT measurements were historically obtained at an ultrasound frequency of 8 MHz or lower, but it is unknown whether measurements obtained at higher frequencies using newer, more advanced ultrasound technology allow for valid comparison to the older general population databases that are commonly used for the interpretation of carotid IMT results. METHODS: Carotid IMT studies were conducted in 35 consecutive patients at standard (8 MHz) and high (14 MHz) frequencies and measurements were performed by two independent expert readers. Systematic bias was assessed by using the paired t-test and agreement was analyzed with the Bland-Altman method. RESULTS: The sample mean carotid IMT obtained at 14 MHz was 0.006 mm lower than that obtained at 8 MHz. The 95% confidence interval (CI) for the mean difference between frequencies indicated that the population mean for 14 MHz is unlikely to be more than 0.02 mm lower than for 8 MHz (95% CI -0.017 to 0.004). The 95% reference range for the difference between the two transducer frequencies indicated that the thickness obtained at 14 MHz was within 0.05 mm of that obtained at 8 MHz for 95% of subjects. CONCLUSIONS: Carotid IMT measurements obtained at higher transducer frequencies are similar to those obtained at standard frequency. This finding has important clinical implications because it validates comparison of carotid IMT measurements obtained with newer, more advanced ultrasound technology with the landmark reference carotid IMT studies commonly used for interpretation of carotid IMT results.

Evaluating the effectiveness of rosuvastatin in preventing the progression of diastolic dysfunction in aortic stenosis: A substudy of the aortic stenosis progression observation measuring effects of rosuvastatin (ASTRONOMER) study.

BACKGROUND: Tissue Doppler imaging (TDI) is a noninvasive echocardiographic method for the diagnosis of diastolic dysfunction in patients with varying degrees of aortic stenosis (AS). Little is known however, on the utility of TDI in the serial assessment of diastolic abnormalities in AS. OBJECTIVE: The aim of the current proposal was to examine whether treatment with rosuvastatin was successful in improving diastolic abnormalities in patients enrolled in the Aortic Stenosis Progression Observation Measuring Effects of Rosuvastatin (ASTRONOMER) study. METHODS: Conventional Doppler indices including peak early (E) and late (A) transmitral velocities, and E/A ratio were measured from spectral Doppler. Tissue Doppler measurements including early (E') and late (A') velocities of the lateral annulus were determined, and E/E' was calculated. RESULTS: The study population included 168 patients (56 ± 13 years), whose AS severity was categorized based on peak velocity at baseline (Group I: 2.5-3.0 m/s; Group II: 3.1-3.5 m/s; Group III: 3.6-4.0 m/s). Baseline and follow-up hemodynamics, LV dimensions and diastolic functional parameters were evaluated in all three groups. There was increased diastolic dysfunction from baseline to follow-up in each of the placebo and rosuvastatin groups. In patients with increasing severity of AS in Groups I and II, the lateral E' was lower and the E/E' (as an estimate of increased left ventricular end-diastolic pressure) was higher at baseline (p < 0.05). However, treatment with rosuvastatin did not affect the progression of diastolic dysfunction from baseline to 3.5 year follow-up between patients in any of the three predefined groups. CONCLUSION: In patients with mild to moderate asymptomatic AS, rosuvastatin did not attenuate the progression of diastolic dysfunction.

Idiopathic ventricular fibrillation controlled successfully with phenytoin.

We describe a case of an individual with idiopathic ventricular fibrillation whose arrhythmias were successfully controlled with phenytoin therapy.

Association of Bicuspid aortic valve morphology and aortic root dimensions: a substudy of the aortic stenosis progression observation measuring effects of rosuvastatin (ASTRONOMER) study.

BACKGROUND: Bicuspid aortic valve (BAV) is the leading cause of aortic stenosis in patients younger than the age of 50. A classification scheme of BAVs is based upon leaflet orientation: Type I (fusion of right and left coronary cusps) and Type II (fusion of right and noncoronary cusps). The correlation between BAV leaflet orientation and aortic root pathology however remains ill defined. OBJECTIVE: The objective was to describe a potential relationship between BAV leaflet morphology and aortic root measurements in the ASTRONOMER study, a multicenter study to assess the effect of rosuvastatin on the progression of AS. METHODS: BAV morphology was classified as Type I or Type II orientation based on the parasternal short-axis view. Echo measurements including left ventricular and aortic root dimensions were obtained. RESULTS: The study population included 89 patients (56 +/- 11 years; 44 males). There were 63 patients with Type I and 26 patients with Type II BAV. Baseline demographics, hemodynamics, and left heart dimensions were similar between both groups. Patients with Type I BAV had larger aortic annulus and ascending root dimensions compared to those patients with Type II BAV (P < 0.05). CONCLUSION: In patients with mild to moderate aortic stenosis due to a BAV, the presence of Type I valve orientation was associated with significantly greater aortic root parameters compared to Type II valve orientation.

Myocardial siderosis due to hemochromatosis in an individual with hypertrophic cardiomyopathy.

A patient with hypertrophic cardiomyopathy (HCM) and transfusion-dependent sideroblastic anemia, who presented with decompensated heart failure, is described. The present case demonstrates the usefulness of cardiac magnetic resonance imaging as a noninvasive imaging modality to assess the etiology of new systolic dysfunction in the setting of HCM. Cardiac magnetic resonance imaging is able to differentiate between the dilated 'burned-out' phase of HCM and a concomitant dilated cardiomyopathy secondary to myocarditis or hemosiderosis.

Clinical utility of cardiac magnetic resonance imaging in Churg-Strauss syndrome: case report and review of the literature.

We describe a case of an individual with Churg-Strauss syndrome who presented with a cerebrovascular accident (CVA) secondary to left ventricular intracavitary thrombi. Noninvasive cardiovascular imaging using transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) was used to identify the cardioembolic source of CVA. The clinical utility of CMR in the management of patients with Churg-Strauss syndrome is reviewed.

Chiari network endocarditis: not just an innocent bystander.

A Chiari's network, noted in approximately 2% of the general population, is a congenital remnant of the sinus venosum valve present in the right atrium. We report a case of a 67-year-old male who presented with acute infective endocarditis of the tricuspid valve due to coagulase-negative Staphylococci. Despite appropriate antimicrobial therapy for 2 weeks, the patient remained febrile with septic emboli to the pulmonary vasculature. Repeat transthoracic echocardiography (TTE) revealed persistent vegetations adherent to the tricuspid valve, extending onto the Chiari network, necessitating surgical intervention.

Association of mitral annular calcification and aortic valve morphology: a substudy of the aortic stenosis progression observation measuring effects of rosuvastatin (ASTRONOMER) study.

AIMS: Mitral annular calcification (MAC) is characterized by calcium and lipid deposition in the annular fibrosa of the mitral valve. Although individuals with MAC are at increased risk of cardiovascular events, little is known about the significance of this finding in patients with concurrent aortic stenosis (AS). The aim of this study was to describe the association of baseline MAC and aortic valve morphology in asymptomatic patients enrolled in the ASTRONOMER study, a multicentre study to assess the effect of Rosuvastatin on the progression of AS. METHODS AND RESULTS: At baseline, transthoracic echocardiography was performed with two-dimensional and Doppler imaging following a standardized protocol. Echo measurements including left ventricular (LV) dimensions and aortic root dimensions were obtained according to the ASE recommendations. MAC was identified by bright echoes at the base of the mitral leaflets or annulus on 2D imaging, and aortic valve calcification by visualization of bright echoes on the aortic valve leaflets. The degree of calcification was semi-quantitated from absent to severe. The study population included 219 patients (57 +/- 14 years; 129 males), divided into two pre-specified categories; bicuspid (n = 133) and tricuspid (n = 86) aortic valves. Baseline LV dimensions, aortic valve haemodynamics, and cholesterol profiles were similar between the two groups at baseline. Individuals with tricuspid aortic valves were older, more hypertensive, with higher degrees of MAC and AV calcification (P < 0.001). The higher degree of MAC persisted in patients with tricuspid AV after adjustment for age and systolic blood pressure (P = 0.004). CONCLUSION: In patients with asymptomatic mild to moderate AS, MAC is more prevalent in those individuals with tricuspid AV, independent of age, and systolic blood pressure. Whether the degree of MAC may be a surrogate for atherosclerosis, and predict the subset of patients who will respond to statin therapy in preventing the progression of AS, remains to be determined.

Resolution of protein-losing enteropathy with low-molecular weight heparin in an adult patient with Fontan palliation.

Protein-losing enteropathy is a well-known complication after the Fontan procedure, and unfractionated heparin therapy has been tried with some success as a potential therapy. Low-molecular weight heparin is believed to be ineffective. We now describe a case in which an adult patient with protein-losing enteropathy after Fontan palliation was successfully treated with systemic doses of low-molecular weight heparin, with complete resolution in the 24-hour fecal alpha-1 anti-trypsin level and serum albumin. The patient continues to remain in remission with this therapy. In conclusion, these findings are novel and compel us to re-evaluate our pathophysiologic understanding of this difficult condition.

Reperfusion times for ST elevation myocardial infarction: a prospective audit.

BACKGROUND: New published guidelines recommend treatment of ST elevation myocardial infarction (STEMI) within 30 minutes of first medical contact to thrombolysis and 90 minutes to primary percutaneous coronary intervention (PCI). OBJECTIVES: To determine how a tertiary care center compares to these new guidelines and to evaluate the success of measures directed to shorten delays. METHODS: This was a prospectively designed audit loop using retrospective chart review. Specific time intervals were evaluated: 1) T2 (ER presentation to diagnostic EKG; 2) T ER (ER presentation to reperfusion); and 3) T AHA (first medical contact to reperfusion). Results of the initial 12-month data were conveyed to Emergency Room staff and a dedicated EKG machine was placed in the ER for the subsequent 12 months, and the results were then re-analyzed. RESULTS: In 2003-4, 58 patients with STEMI were identified, with 41 (70.7%) receiving reperfusion. Of those receiving thrombolysis, median T AHA was 54 [37-72] minutes, with 12.0%<30 minutes, while those receiving PCI, median T AHA was 58 [43-78] minutes, with 25.0%<90 minutes. In 2004-5, 52 patients had STEMI, with 40 (76.9%) receiving reperfusion. The percentage of patients meeting the guidelines was 14.3% for the thrombolysis group and 11.1% for the PCI group. Introduction of a dedicated EKG machine led to a strong trend towards improvement in median T2 (22 vs 10 minutes; P=0.07), but other treatment times remained unchanged. CONCLUSIONS: Treatment times are longer than recommended guidelines. More comprehensive strategies and improved coordination of medical services are required to shorten pre-contact and post-contact response times.

Intermittent androgen suppression in prostate cancer: an update of the Vancouver experience.

INTRODUCTION: This report will review the long-term follow-up of a prospective Phase II evaluation of intermittent androgen suppression in the treatment of prostate cancer. Specifically, this analysis will address completed cycle characteristics, the concept of prolonged off-treatment cycles, the time to cancer progression, cancer-specific survival and the association between PSA and bone scan changes. METHODS: A total of 102 patients have been entered into this protocol. Treatment was initiated with combined androgen blockade and continued for 6 months or longer to reach a serum PSA nadir. Medication was then withheld until the serum PSA increased to predetermined trigger points based on initial parameters. Each cycle of treatment and no-treatment was repeated until the regulation of PSA became biochemically androgen independent. RESULTS: One hundred two patients have been commenced on IAS with an average follow-up time of 219 weeks (range: 14.5 to 588). Ninety-one patients have completed at least one therapeutic cycle with a total of 188 completed cycles available for analysis. The average time off therapy (percentage time off therapy) for cycles 1, 2, 3 and 4 was 13 months (53%), 11 months (51%), 10 months (47%) and 8 months (45%), respectively. A prolonged off-treatment time of greater than 72 weeks was observed in 33 (18%) of all completed cycles, and was most common in the men being treated for radiation failure stage C. Progression and survival data was calculated for the entire trial cohort (n=102). The average time to androgen independent progression in the 29 (28%) patients who progressed was 194 weeks. Death from prostate cancer occurred in 19 (18%) patients at an average of 258 weeks following treatment initiation. A review of bone scans revealed 22 events of newly detected lesions, all but 2 of which were preceded by a rise in serum PSA. CONCLUSIONS: Longer duration follow-up of a single cohort continues to support IAS as a viable treatment option for men with prostate cancer. This approach affords an improved quality of life when the patient is off therapy, with reduced toxicity and costs. There is a trend toward extended times to progression and death compared to contemporary studies of continuous androgen suppression. Randomized, prospective protocols are currently underway to determine whether survival is affected in a beneficial or adverse way in men with locally recurrent or metastatic cancer.