John N. Makaryus

Exertional rhabdomyolysis and renal failure in patients with sickle cell trait: is it time to change our approach?

Abstract Based upon numerous reported cases and despite widespread beliefs to the contrary, sickle cell trait (SCT) may be deemed a quantifiable risk factor in certain subsets of patients. As a result of common misconceptions regarding SCT, most individuals with the condition are generally not informed regarding the possible consequences of certain activities such as venturing to high altitudes or participating in overly exertional physical activities. Acute exertional rhabdomyolysis is a potentially serious clinical illness and is caused by skeletal muscle injury resulting in the release of myoglobin and other cellular contents, including creatine kinase, into the circulatory system. Mild to moderate cases of acute exertional rhabdomyolysis can cause metabolic disorders including hypernatremia, hyperkalemia, hyperphosphatemia, hypocalcemia, lactic acidosis and hyperuricemia. Severe cases may result in renal failure and even death. Several case reports have been published since the early 1970s describing significant morbidity and mortality of acute exertional rhabdomyolysis in patients with SCT. We present the case of a 27-year-old male with a past medical history significant only for SCT who presented after a 1.5 mile run with severe exertional rhabdomyolysis and subsequent acute renal failure requiring hemodialysis (HD). In presenting this case, we hope to raise awareness of a possible underlying cause to many cases of exertional rhabdomyolysis and encourage physicians to counsel their patients with SCT in order to avoid the significant morbidity and mortality that may be associated with the condition.

Unilateral neurogenic pulmonary oedema and severe left ventricular dysfunction secondary to acute multiple sclerosis exacerbation.

Neurological conditions such as stroke, subarachnoid haemorrhage and seizure activity, amongst others, have been associated with the development of pulmonary oedema. The development of clinically significant pulmonary oedema and severe myocardial dysfunction in the setting of acute multiple sclerosis (MS) exacerbation is very rare and poorly understood. The association between MS and neurogenic pulmonary oedema is not clear, nor is the correlation between neurological insults and myocardial dysfunction. Neurological conditions most likely result in cardiopulmonary sequelae as a result of an interplay between enhanced sympathetic tone, inflammatory cytokine release and other factors. Whatever the case, neurogenic pulmonary oedema should always be in the differential diagnosis when patients with presumed neurogenic pathology develop cardiopulmonary compromise. Unilateral pulmonary oedema is also a very rare occurrence, and has not, to our knowledge, been reported in acute MS exacerbation in the past. We present the case of a 31-year-old female who developed respiratory distress with unilateral pulmonary oedema and left ventricular (LV) dysfunction in the context of neurological sequelae and diagnostic evaluation consistent with acute MS exacerbation.

Inflammatory bowel disease as a risk factor for premature coronary artery disease.

Background Crohn’s disease and ulcerative colitis are both systemic chronic diseases that alter bowel physiology. The central process in inflammatory bowel disease (IBD) and the associated manifestations are the result of B-cell production of IgG autoantibodies directed against self-antigens in various organ systems including coronary endothelium. Previous studies have demonstrated significant micro-vascular endothelial dysfunction in patients with IBD compared to patients not affected by the disease. We sought to analyze the relation, if any, between IBD and the development of premature coronary artery disease (CAD). Methods We queried our hospital database to find IBD patients admitted to the hospital from January 1, 2007 to December 31, 2008. Patients with traditional cardiovascular (CV) disease risk factors including hypertension, congestive heart failure (CHF), diabetes, age ≥ 65, hyperlipidemia, family history, end-stage renal disease (ESRD), and greater than five pack-year smoking history were excluded from the study cohort. The charts of the remaining 300 patients with diagnosed IBD were then analyzed for the incidence of CV disease events including acute myocardial infarction (MI), unstable angina, positive stress testing, and any cardiac intervention including coronary angioplasty and/or intracoronary stent implantation. Results Of the 300 patients included, only one patient had a CV disease event. This patient had a positive exercise stress thallium test. Otherwise, the remaining 299 patients (99.7%) did not have any reported CV disease events over the 2-year follow-up period. Conclusion Most of the clinical sequelae of CV disease events are the result of inflammatory changes at the vascular level. While IBD is associated with a chronic inflammatory state as reflected by high sedimentation rates, C-reactive protein (CRP), homocysteine levels, etc., our data seem to indicate that chronic inflammation in the absence of traditional risk factors is not associated with an increased risk of premature CV disease events. More wide-scale prospective studies should be performed to elucidate the relationship, if any, between chronic inflammation and CV disease risk.

Acute myocardial infarction following the use of intranasal anesthetic cocaine

The use of cocaine has become largely obsolete in modern medical practice; however, it is still used by otolaryngologists for topical anesthesia in head and neck surgeries. We present the case of a 68-year-old woman who developed a myocardial infarction after the use of topical cocaine during nasal sinus surgery, and review the literature regarding the use of cocaine as a topical anesthetic in otolaryngologic practice. Awareness is raised of a possible complication of myocardial infarction following topical cocaine anesthetic use.

Hypertension Management in the High Cardiovascular Risk Population

The incidence of hypertension is increasing every year. Blood pressure (BP) control is an important therapeutic goal for the slowing of progression as well as for the prevention of Cardiovascular disease. The management of hypertension in the high cardiovascular risk population remains a real challenge as the population continues to age, the incidence of diabetes increases, and more and more people survive acute myocardial infarction. We will review hypertension management in the high cardiovascular risk population: patients with coronary heart disease (CHD) and heart failure (HF) as well as in diabetic patients.

Spodick's Sign

PRESENTATION Unless routinely sought, much of the valuable information present in an electrocardiogram (ECG) might go unused. The frequently overlooked ECG sign described here can be quite useful in establishing the etiology of a particular clinical condition. The patient was a 56-year-old man with hypertension and diabetes mellitus who was admitted to a local community hospital for flu-like symptoms that had begun approximately 1 week prior to presentation. He was diagnosed with acute promyelocytic leukemia and transferred to our institution for management of his newly diagnosed condition. At our institution, the patient had a multiple-gated acquisition scan which showed no significant cardiac abnormalities, no wall motion abnormalities, and a left ventricular ejection fraction of 81%. His baseline ECG demonstrated normal sinus rhythm at 87 bpm, normal axis, and no ST-T wave abnormalities. He was then started on chemotherapy with all-trans-retinoic acid (tretonin), but after 2 days of therapy, he complained of progressively worsening shortness of breath. In addition, his blood urea nitrogen and creatinine levels, which had been normal upon admission, increased significantly to 36 mg/dL and 3.5 mg/dL, respectively.

Persistent second-degree atrioventricular block following adenosine infusion for nuclear stress testing.

The agents used for nuclear stress testing (NST) including adenosine, dobutamine, and dipyridamole, are generally well tolerated and the incidence of serious complications associated with their use in NST is relatively low. Adenosine possesses a potent inhibitory effect on the atrioventricular (AV) node and may induce a transient conduction defect which could result in first-, second-, or third-degree heart block in some patients. The use of the potent AV nodal blocker adenosine for nuclear stress testing in patients with evidence of underlying conduction system disease may result in serious complications. We present the case of a 79-year-old man who had sustained second-degree AV block requiring permanent pacemaker implantation following adenosine infusion for nuclear stress testing. We also review the literature regarding the association between adenosine NST and AV block.

Planning and Guidance of Cardiac Resynchronization Therapy–Lead Implantation by Evaluating Coronary Venous Anatomy Assessed with Multidetector Computed Tomography

Objectives We sought to evaluate the utility of multidetector computed tomography (MDCT) in preoperative planning of cardiac resynchronization therapy (CRT) device implantation. Background Variation in coronary venous anatomy can affect optimal lead placement and may warrant preimplantation visualization prior to CRT lead placement. Methods Prospective randomized enrollment of 29 patients (17 males; mean age at implant 66.7 ± 12.8 years) was undertaken. Patients were randomized to preimplantation MDCT (GE® 64-detector Lightspeed, n = 16) or no MDCT. Implantation was planned based on three-dimensional coronary venous reconstruction as visualized in the CT group. Measurement of coronary sinus (CS) angulation, CS ostial (os) diameter, right atrial (RA) width, volume, and height was undertaken prior to implant. Intraoperative CS lead implantation times (introduction, cannulation, and left ventricular [LV] lead positioning), procedure time, fluoroscopy time, and venogram contrast volume were measured to determine if there was a difference between patients who underwent preimplant CT scan and those who did not. Results CS os diameter (mean = 13.8 ± 2.9 cm) was inversely correlated with total fluoroscopy time (r = -0.57, P = .008), and total procedure time, but this correlation was not statistically significant (r = - 0.36, P = 0.12). RA width (mean = 52.8 ± 9.9 cm) was associated with a shorter total procedure time (r = −0.44, P = .047) and LV lead positioning time (r = −0.33, P = .012). There were no statistically significant differences between the CT group and the non-CT group with respect to total intraoperative and fluoroscopy times or venogram contrast volumes. Total procedure time was longer in the CT group but the difference was not statistically significant (94 ± 27.2 vs. 74.7 ± 26.6; P = .065). Conclusion Noninvasive visualization of the coronary venous anatomy before CRT implantation can be used as a guide for lead placement. While no significant differences were noted between the two groups with respect to intraoperative variables, CS os diameter and RA width inversely correlated to a shorter procedure time and LV lead positioning time, respectively. Further clinical trials regarding the utility of MDCT to visualize coronary venous anatomy prior to CRT implantation for procedural planning and lead placement guidance are warranted.

Implications of Gender Difference in Coronary Calcification as Assessed by CT Coronary Angiography

Background Arterial calcium as measured by 64-slice computed tomography coronary angiography (64-CT) is a reliable predictor of cardiovascular disease risk. Lipid-rich plaques with lower degrees of calcification may pose greater risk for adverse coronary events than more stabilized calcified plaques as a result of the increased risk of plaque rupture, migration, and subsequent acute coronary syndrome. We sought to examine coronary artery calcium scores as measured via 64-CT to assess the extent of calcification and plaque distribution in women compared to men. Methods A total of 138 patients referred for 64-CT were evaluated. Computerized tomographic angiography was performed using the GE LightSpeed VCT. Subgroup analysis comparing male and female data (including demographic data) was performed. All major coronary arteries were analyzed for coronary stenosis/plaque characterization as well as total vessel calcium (Agatston) score quantification. Patient demographics and coronary risk factors were recorded. Results A total of 552 coronary arteries were evaluated in 138 patients (85 men, 53 women). The average age for females was 64.4 ± 10.8 years and for males 60.0 ± 12.8 years. The only demographic/cardiovascular risk factor in which the difference between men and women was significant was smoking history, where 23.5% of men had a history of smoking while only 9.6% of females endorsed having a smoking history (P < 0.044). On comparison of all total vessel calcium scores, males had a higher total mean calcium score than females in each individual vessel. The results were as follows for males versus females, respectively: left main total vessel calcium score 46.49 versus 16.71 (P = 0.167); left anterior descending 265.21 versus 109.6 (P < 0.003); left circumflex 130.5 versus 39.7 (P < 0.004); and right coronary 213.5 versus 73.8 (P < 0.01). The odds of having a total calcium score >100 (versus not) was 3.62 times greater in males relative to females, given that all the other cardiovascular risk factors are adjusted for (95% confidence interval: 1.37-9.54). On average, men had an average of 2.1 ± 1.5 epicardial vessels with a calcium score ≥11 compared to 1.3 ± 1.4 for women (P < 0.005). Conclusion There are clear differences between males and females regarding total vessel calcium scores and therefore risk of future adverse coronary events. Males tended to have higher average calcium scores in each coronary artery than females with a greater tendency to have multiple vessel involvement. Using this information, more large-scale, randomized controlled studies should be performed to correlate differences in the extent of coronary calcification with the observed variance in clinical presentation during coronary events between males and females as a means to potentially establish gender-specific therapeutic regimens.

Fever Associated with Gastrointestinal Shigellosis Unmasks Probable Brugada Syndrome

Since it was first described approximately 15 years ago, the Brugada Syndrome has spurred a significant quantity of interest in its underlying mechanism and physiology. The Brugada electrocardiographic pattern is characterized by right bundle branch block morphology and ST segment elevations in the right precordial leads with an absence of identifiable underlying structural heart disease. The syndrome is clinically significant since these patients are at a higher risk of developing malignant ventricular arrhythmias. One of the mechanisms behind the disorder involves mutations in specific myocardial sodium channels. Furthermore, these electrocardiographic changes appear to be temperature dependent. We present the case of a 35-year-old male who presented with intestinal Shigellosis and was also found to have Brugada-type electrocardiographic changes on ECG. The electrocardiographic changes that were present when the patient was admitted and febrile resolved following antibiotic therapy and defervescence.