Arrhythmogenic right ventricular cardiomyopathy (ARVC): case report and review of literature.

Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited condition characterised by fibrofatty replacement of the right ventricle. It causes sudden death in 30% of young adults and in 5% of those less than 65 years of age. As the presentation is non-specific, ARVD can be a diagnostic challenge leading to delayed treatment. We report a case along with the review of literature, of a 63-year-old man who presented (atypical) with a history of palpitations, dizziness and raised cardiac enzymes associated with ECG changes in the inferior and anterior leads. Further investigation helped in confirming this rare and potentially fatal cardiac condition.

Disease progression and adverse events in patients listed for elective percutaneous coronary intervention.

OBJECTIVE: To record disease progression and the timing of adverse events in patients on a waiting list for elective percutaneous coronary intervention (PCI). DESIGN: Observational prospective study. SETTINGS: A UK tertiary cardiothoracic centre, at a time when waiting lists for PCI were up to 18 months. PATIENTS: 145 patients (116 men, median age 59.5 years) placed on an elective waiting list for PCI between October 1998 and September 1999. MAIN OUTCOME MEASURES: Adverse events recorded were death, myocardial infarction, need for urgent hospital admission because of unstable angina, and need for emergency revascularisation while waiting for PCI. RESULTS: During a median follow up of 10 months (range 1-18 months), nine (6.2%) patients experienced an adverse event. Eight (5.52%) patients were admitted with unstable angina as emergencies. One was admitted with a myocardial infarction. Twenty nine (20.0%) patients had significant disease progression at the time of the repeat angiogram before PCI. In 10 (7%), disease had progressed so that PCI was no longer feasible and patients were referred for coronary artery bypass graft. Sixteen (11%) were removed from the PCI waiting list because of almost complete resolution of their anginal symptoms. CONCLUSION: Adverse coronary events and clinically significant disease progression occur commonly in patients waiting for PCI. Despite the presence of severe coronary lesions, myocardial infarction was rare and no patients died while on the waiting list. Resolution of anginal symptoms was also comparatively common. The pathophysiology of disease progression frequently necessitates a change in the treatment of patients waiting for PCI.

Relation between coronary pressure derived collateral flow, myocardial perfusion grade, and outcome in left ventricular function after rescue percutaneous coronary intervention.

OBJECTIVE: To evaluate the relation between pressure derived coronary collateral flow (PDCF) index and angiographic TIMI (thrombolysis in myocardial infarction) myocardial perfusion (TMP) grade, angiographic collateral grade, and subsequent recovery of left ventricular function after rescue percutaneous coronary intervention (PCI) for failed reperfusion in acute myocardial infarction. METHODS: The pressure wire was used as the guidewire in 38 consecutive patients who underwent rescue PCI between December 2000 and March 2002. Follow up angiography was performed at six months. Baseline and follow up single plane ventriculograms were analysed off line by an automated edge detection technique. A linear model was fitted to assess the relation between 0.1 unit increase in PDCF and change in left ventricular regional wall motion. RESULTS: Patients with TMP 0 grade had significantly higher mean (SD) PDCF than patients with TMP 1-3 (0.30 (0.11) v 0.15 (0.07), p < 0.0001, r = -0.5). A similar relation was observed between TMP grade and coronary wedge pressure (mean (SD) 28 (16) mm Hg with TMP 0 v 9 (7) mm Hg with TMP 1-3, p = 0.001, r = -0.4). Higher PDCF was associated with increased left ventricular end diastolic pressures (0.28 (0.14) with end diastolic pressure > 20 mm Hg v 0.22 (0.09) with end diastolic pressure < 20 mm Hg, p = 0.08, r = 0.2). No correlation was observed between PDCF and Rentrops collateral grade (0.26 (0.13) with grade 0 v 0.25 (0.11) with grades 1-3, p = 0.4, r = -0.06). No linear relation existed between changes in PDCF and changes in left ventricular regional wall motion. CONCLUSION: PDCF in the setting of rescue PCI for failed reperfusion after thrombolysis does not predict improvement in left ventricular function. Increased PDCF and coronary wedge pressure in acute myocardial infarction reflect a dysfunctional microcirculation rather than good collateral protection.

Cervical spine alignment in the immobilized ice hockey player. A computed tomographic analysis of the effects of helmet removal.

To determine if helmet removal causes a significant increase in lordosis of the cervical spine in ice hockey players, we radiographically assessed the position of the cervical spine in subjects immobilized to a standard spine backboard wearing shoulder pads both with and without a helmet. Ten adult male volunteers (ages, 18 to 28 years) with no previous history of cervical spine injuries were fitted with an appropriately sized ice hockey helmet and shoulder pads and immobilized in a supine position to a standard spine backboard. Computerized tomographic lateral scout scans were obtained of the cervical spine for three conditions: 1) no equipment (control), 2) helmet and shoulder pads, and 3) shoulder pads only (helmet removed). With the helmet removed and the shoulder pads remaining, a significant increase in C2 to C7 lordosis was found when compared with the other two conditions. Individual segmental measurements revealed a significant increase in cervical lordosis at the C6-7 level with the helmet removed compared with the helmet and shoulder pads condition. Our results demonstrate that the removal of an ice hockey helmet from a supine player causes a significant increase in lordosis (extension) of the cervical spine. We recommend that ice hockey helmets not be removed from injured players, with rare exceptions, because doing so results in unnecessary motion of the cervical spine.

A prospective randomized multicenter clinical evaluation of an anterior cervical fusion cage.

STUDY DESIGN: A prospective, concurrently controlled, randomized, multicenter trial of an anterior Bagby and Kuslich cervical fusion cage (BAK/C; Sulzer Spine-Tech, Minneapolis, MN) for treatment of degenerative disc disease of the cervical spine. OBJECTIVES: To report clinical results with maximum 24-month follow-up of fusions performed with the BAK/C fusion cage. SUMMARY OF BACKGROUND DATA: Threaded lumbar cages have been used during the past decade as a safe and effective surgical solution for chronic disabling low back pain. Threaded cages have now been developed for use in anterior cervical interbody fusions to obviate the need for allografts or autogenous bone grafting procedures while providing initial stability during the fusion process. METHODS: Patients with symptomatic cervical discogenic radiculopathy were treated with either anterior cervical discectomy with uninstrumented bone-only fusion (ACDF) or BAK/C fusion cage(s). Independent radiographic assessment of fusion was made and patient-based outcome was assessed by visual analog pain scale and a Short Form (SF)-36 Health Status Questionnaire. RESULTS: Data analysis included 344 patients at 1 year and 180 at 2 years. When the two cage groups (hydroxya, patite-coated or noncoated) were compared with the ACDF group, similar outcomes were noted for duration of surgery, hospital stay, improvements in neck pain and radicular pain in the affected limb, improvements in the SF-36 Physical Component subscale and Mental Component subscale, and the patients' perception of overall surgical outcome. Symptom improvements were maintained at 2 years. A greater percentage of patients with ACDF needed an iliac crest bone harvest than did BAK/C patients (67% vs.- 3%). Successful fusion for one-level procedures at 12 months was 97.9% for the BAK/C groups and 89.7% for the ACDF group (P < 0.05). The complication rate for the ACDF group was 20.4% compared with an overall complication rate of 11.8% with BAK/C. There was no difference in complications that necessitated a second operative procedure. CONCLUSIONS: These results demonstrate that outcomes after a cervical fusion procedure with a threaded cage are the same as those of a conventional uninstrumented bone-only anterior discectomy and fusion with a low risk of complications and rare need for autogenous bone graft harvest.

Arrhythmogenic right ventricular dysplasia. An illustrated review highlighting developments in the diagnosis and management of this potentially fatal condition.

Arrhythmogenic right ventricular dysplasia is an inherited, progressive condition. Characterised by fatty infiltration of the right ventricle, it frequently results in life threatening cardiac arrhythmias, and is one of the important causes of sudden cardiac death in the young. There are characteristic electrocardiographic and echocardiographic features that all physicians need to be aware of if we are to reduce these occurrences of premature death. Diagnosis with magnetic resonance imaging is discussed along with current treatment options.

Magnetic resonance evaluation of recurrent disc herniation: is gadolinium necessary?

STUDY DESIGN: Evaluation of magnetic resonance images (MRIs) with surgical reference standard. OBJECTIVES: To determine whether the addition of contrast-enhanced MRI scans increases diagnostic efficacy in the evaluation of recurrent disc herniation. SUMMARY OF BACKGROUND DATA: Many centers now routinely use gadolinium-enhanced examinations in the evaluation of recurrent disc herniation. Others, noting the additional expense of contrast injection, advocate a more limited role for contrast injection and emphasize the importance of T2-weighted axial sequences. METHODS: The study included 165 consecutive patients who were referred to the authors' outpatient imaging center and had a history of previous lumbar discectomy and recurrent back and/or leg pain. The scanning protocol included sagittal and axial T1-weighted spin-echo pre- and postcontrast injection images and sagittal and axial T2-weighted fast spin-echo images. Twenty-eight patients (32 vertebral levels) had subsequent surgical exploration of a disc margin that had previously undergone discectomy. The surgical findings formed the reference standard. Three spine radiologists interpreted the MRI examinations without knowledge of the surgical results. They first interpreted the unenhanced studies, indicated whether they felt contrast injection would be helpful in further evaluation, and then (regardless of this determination) read the postcontrast study. RESULTS: On pre- and post-contrast examinations Reader 1 had a sensitivity of 95% (20/21), a specificity of 100% (10/10), and an accuracy of 97% (30/31). Reader 2 had a sensitivity of 95% (20/21), a specificity of 90% (9/10), and an accuracy of 94% (29/31). Reader 3 had a sensitivity of 90% (19/21), a specificity of 100% (10/10), and an accuracy of 94% on the precontrast examinations. His postcontrast performance demonstrated a sensitivity of 86% (18/21), a specificity of 100% (10/10), and an accuracy of 90% (28/31). In the nine interpretations wherein the readers thought that a contrast-enhanced examination might provide useful additional information, they did not change their interpretations in three cases, improved their interpretations in two, and made their interpretations worse in four on the basis of addition of the enhanced images. CONCLUSIONS: Routine use of contrast-enhanced examinations in patients who have had prior lumbar surgery probably adds little diagnostic value and may be confusing.

The magnetic resonance imaging appearance of individual structures of the posterolateral knee. A prospective study of normal knees and knees with surgically verified grade III injuries.

The purpose of this study was to contrast the magnetic resonance imaging appearance of uninjured components of the posterolateral knee with that of injured structures, and to assess the accuracy of magnetic resonance imaging in identifying posterolateral knee complex injuries. Thin-slice coronal oblique T1-weighted images through the entire fibular head were used to identify the posterolateral structures in seven uninjured knees. The appearance of corresponding grade III injuries to these structures was identified prospectively in 20 patients and verified at the time of surgical reconstruction. The sensitivity, specificity, and accuracy of imaging for the most frequently injured posterolateral knee structures in this series were as follows: iliotibial band-deep layer (91.7%, 100%, and 95%), short head of the biceps femoris-direct arm (81.3%, 100%, and 85%), short head of the biceps femoris-anterior arm (92.9%, 100%, and 95%), midthird lateral capsular ligament-meniscotibial (93.8%, 100%, and 95%), fibular collateral ligament (94.4%, 100%, and 95%), popliteus origin on femur (93.3%, 80%, and 90%), popliteofibular ligament (68.8%, 66.7%, and 68%), and the fabellofibular ligament (85.7%, 85.7%, and 85.7%). Magnetic resonance imaging of the knee was accurate in the identification of these injuries.

Maternal smoking and Down syndrome: the confounding effect of maternal age.

Inconsistent results have been reported from studies evaluating the association of maternal smoking with birth of a Down syndrome child. Control of known risk factors, particularly maternal age, has also varied across studies. By using a population-based case-control design (775 Down syndrome cases and 7,750 normal controls) and Washington State birth record data for 1984-1994, the authors examined this hypothesized association and found a crude odds ratio of 0.80 (95% confidence interval 0.65-0.98). Controlling for broad categories of maternal age (<35 years, > or =35 years), as described in prior studies, resulted in a negative association (odds ratio = 0.87, 95% confidence interval 0.71-1.07). However, controlling for exact year of maternal age in conjunction with race and parity resulted in no association (odds ratio = 1.00, 95% confidence interval 0.82-1.24). In this study, the prevalence of Down syndrome births increased with increasing maternal age, whereas among controls the reported prevalence of smoking during pregnancy decreased with increasing maternal age. There is a substantial potential for residual confounding by maternal age in studies of maternal smoking and Down syndrome. After adequately controlling for maternal age in this study, the authors found no clear relation between maternal smoking and the risk of Down syndrome.

Sclerosing epithelioid fibrosarcoma: short T2 on MR imaging.

A case of sclerosing epithelioid fibrosarcoma and its appearance on MRI is presented. The tumor showed a zonal architecture on MRI with a large central core of very low signal intensity and a peripheral rim of intermediate to high signal intensity on T1- and T2-weighted spin echo pulse sequences. The core showed decreased cellularity with dense collagen deposition on histologic examination, and the peripheral zone increased cellularity with increased nuclear atypia. The presence of a prominent region of very low signal intensity on T1- and T2- weighted images can be seen with neural tumors, giant cell tumor of the tendon sheath, aggressive fibromatosis, and, in rare instances, with soft tissue sarcomas rich in collagen.

Rationale, design and methodology for the Navajo Health and Nutrition Survey.

As recently as 1990, there was no reservation-wide, population-based health status information about Navajo Indians. To remedy this shortcoming, the Navajo Health and Nutrition Survey was conducted from 1991 to 1992 to assess the health and nutritional status of Navajo Reservation residents using a population-based sample. Using a three-stage design, a representative sample of reservation households was selected for inclusion. All members of selected households 12 y of age and older were invited to participate. A total of 985 people in 459 households participated in the study. Survey protocols were modeled on those of previous national surveys and included a standard blood chemistry profile, complete blood count, oral glucose tolerance test, blood pressure, anthropometric measurements, a single 24-h dietary recall and a questionnaire on health behaviors. The findings from this survey, reported in the accompanying papers, inform efforts to prevent and control chronic disease among the Navajo. Lessons learned from this survey may be of interest to those conducting similar surveys in other American Indian and Alaska Native populations.

Weight, body image, and weight control practices of Navajo Indians: findings from the Navajo Health and Nutrition Survey.

Historically, the Navajo exhibited a low prevalence of overweight, but a number of small studies over the past few decades indicate that the prevalence is increasing. In the population-based Navajo Health and Nutrition Survey conducted in 1991-92, overweight was defined as a body mass index (BMI, kg/m2) at or above the 85th percentile (BMI > 27.8 for men, > 27.3 for women) of the Second National Health and Nutrition Examination Survey. One third of men age 20 and 39 and one half of men age 40 and 59, but fewer than 10% of men age 60 and older were overweight. Two thirds or more of women in all age groups were overweight. Nineteen percent of the participants underestimated their weight status (underweight, appropriate, overweight) relative to their BMI category and 17% overestimated their weight status. Women overestimated their weight status more often than men (P < 0.05), and participants age 20-39 overestimated their weight status more often than older participants (P < 0.001). Men and women age 60 and older preferred heavier body shape models as ideals of health more often than younger participants (P < 0.001). Nearly half of the participants, regardless of their weight status, reported that they were trying to lose weight; most reported using diet and exercise. Because overweight is an important risk factor for many chronic diseases, including diabetes mellitus, cardiovascular disease and cancer, primary prevention of overweight and weight management for adults are recommended to prevent an increase in the burden of chronic disease among the Navajo.

Prevalence of hypertension among Navajo Indians: findings from the Navajo Health and Nutrition Survey.

Hypertension and other chronic diseases are becoming increasingly important health problems for many Native American people, including the Navajo. A community-based survey that included three standardized measurements of blood pressures, was conducted during 1991-92 on the Navajo Reservation. Among the 780 adults examined, the overall age-standardized prevalence of hypertension, defined as an elevated systolic (> or = 140 mm Hg) or diastolic (> or = 90 mm Hg) blood pressure, or possession of prescription antihypertensive medications, was 19% (24% among men and 15% among women). The prevalence of hypertension increased with age and relative weight, and among men, was associated with diabetes mellitus. Among women, hypertension was associated with a central distribution of body fat, cigarette smoking, self-reported diabetes mellitus and impaired glucose tolerance. Although only 50% of the persons found to have elevated blood pressure at the examination reported they had been previously told that they had hypertension, persons who had been previously diagnosed with hypertension had a slightly higher rate (approximately 60%) of blood pressure control than that seen in the general U.S. population. On the basis of these results, the prevalence of hypertension among the Navajo appears to have substantially increased since the 1930s. Improved prevention and management of hypertension, especially for overweight and diabetic individuals, may reduce morbidity and mortality from cardiovascular and renal disease.

Imaging of acute injuries to the wrist and hand.

This article reviews the basic characteristics of common fractures and dislocations in the hand and wrist. There is increasing recognition that fractures and dislocations of the hand and wrist can result in long-term pain and dysfunction. This article includes descriptions and, when helpful, classifications for these fractures with attention to those features that are of prognostic and therapeutic significance. With prompt recognition and proper characterization of these injuries, patients can be triaged to the orthopedic surgeon for early and more aggressive treatment.

Large airway obstruction by a chronic dissecting aortic aneurysm in the Marfan syndrome.

We describe a patient with the Marfan syndrome who presented with an acute aortic dissection. She underwent composite graft replacement of the aortic root. She returned two years later with dyspnoea and stridor due to tracheal compression by a large chronic dissection of the thoracic aorta. Marfan patients are at risk of chronic dissection involving the remaining distal aorta and require regular noninvasive assessment following surgery.

Lipoblastoma of the foot.

Lipoblastoma is an uncommon benign lipomatous soft tissue mass of childhood, occurring most commonly in children less than 3 years of age. We present a case of lipoblastoma occurring in the foot of a 14-year-old boy and review the literature. The appearance of the mass on magnetic resonance imaging (MRI) is illustrated and is correlated with the findings at gross and microscopic pathological examination. A lobulated architecture, the presence of adipose tissue, thin nonenhancing septa, peripheral lobules of more immature and therefore less specific tissue, and a peripheral pseudocapsule were evident on MRI and at pathological examination.