Imaging spectrum of sudden athlete cardiac death.

Sudden athlete death (SAD) is a widely publicized and increasingly reported phenomenon. For many, the athlete population epitomize human physical endeavour and achievement and their unexpected death comes with a significant emotional impact on the public. Sudden deaths within this group are often without prior warning. Preceding symptoms of exertional syncope and chest pain do, however, occur and warrant investigation. Similarly, a positive family history of sudden death in a young person or a known family history of a condition associated with SAD necessitates further tests. Screening programmes aimed at detecting those at risk individuals also exist with the aim of reducing fatalities. In this paper we review the topic of SAD and discuss the epidemiology, aetiology, and clinical presentations. We then proceed to discuss each underlying cause, in turn discussing the pathophysiology of each condition. This is followed by a discussion of useful imaging methods with an emphasis on cardiac magnetic resonance and cardiac computed tomography and how these address the various issues raised by the pathophysiology of each entity. We conclude by proposing imaging algorithms for the investigation of patients considered at risk for these conditions and discuss the various issues raised in screening.

The lady with the dragon tattoo.

BACKGROUND: Though the skin is affected in sarcoidosis in about one-third of cases, granulomatous tattoo reactions are an unusual manifestation of the disease. It is important phenomenon to recognize, as it frequently leads to the diagnosis of systemic sarcoidosis. CASE PRESENTATION: A 35-year-old Caucasian female with multiple tattoos presented with a 5-week history of tenderness of the black dye in a tattoo depicting a dragon. She also described a 15-month history of fatigue, polyarthralgia, and mild dyspnea. Skin biopsy demonstrated multiple dermal non-caseating granulomata with associated tattoo ink. Further investigation revealed the presence of systemic sarcoidosis. Her symptoms and skin changes improved with conservative management. CONCLUSION: Sarcoidal tattoo reactions in those without systemic sarcoidosis are a rare occurrence, and their presence should prompt a search for systemic involvement. The accurate identification of skin involvement in sarcoidosis is important, as it tends to occur early in the course of disease, and the skin is a readily accessible site for biopsy, allowing for prompt diagnosis.

Oral bisphosphonates improve Bone Mineral Density in adults with cystic fibrosis.

In adults with Cystic Fibrosis (CF) we sought to establish the effect of oral bisphosphonate therapy. Bone densitometry measured by dual energy X-ray absorptiometry (DXA), and clinical patient data, were reviewed retrospectively. Eighty-one patients (median age 27 years) had baseline and follow-up DXA, with an interval of 19.2 +/- 7.1 months. Thirty-six patients were treated with bisphosphonates (alendronate=23 and risedronate=13). Median follow-up Bone Mineral Density in the bisphosphonate group was 3.7% greater at the lumbar spine (95%CI 1.9 to 5.7%, P<0.0005) and 2.4% greater at the femur (95%CI 0.8 to 3.9%, P<0.005) than the group not treated with bisphosphonates. Oral bisphosphonate therapy had a beneficial effect on BMD in adults with CF.

Disordered breathing during sleep and exercise in idiopathic pulmonary fibrosis and the role of biomarkers.

BACKGROUND AND OBJECTIVE: Idiopathic pulmonary fibrosis (IPF) patients report fatigue, possibly reflecting sleep disturbance, but little is known about sleep-related changes. We compared ventilation and gas exchange during sleep and exercise in a cohort of IPF patients, and evaluated associations with selected biological markers. METHODS: Twenty stable IPF patients (aged 67.9 ± 12.3 [SD]) underwent overnight polysomnography following an acclimatization night. Cardiopulmonary exercise testing was performed and inflammatory markers measured including TNF-α, IL-6, CXCL8, C-C motif ligand 18 (CCL-18) and C-reactive protein (CRP) RESULTS: Nine patients had sleep-disordered breathing (SDB) with an apnea-hypopnea frequency (AHI) ≥ 5/h, but only two had Epworth sleepiness score ≥ 10, thus having an obstructive sleep apnea syndrome. Sleep quality was poor. Transcutaneous carbon dioxide tension (PtcCO2) rose by 2.56 ± 1.59 kPa overnight (P = 0.001), suggesting hypoventilation. Oxygen saturation (SaO2) was lower during sleep than exercise (P < 0.01), and exercise variables correlated with resting pulmonary function. CCL-18 and CRP levels were elevated and correlated with PtcCO2 rise during sleep (P < 0.05). CCL-18 negatively correlated with diffusion capacity of carbon monoxide (DLCO), arterial oxygen (PaO2) and mean arterial carbon dioxide (PaCO2) (P < 0.05) and CRP negatively correlated with DLCO, PaO2, sleep SaO2 and oxygen uptake (VO2) during exercise (P < 0.05). CONCLUSIONS: IPF patients desaturate more during sleep than exercise; thus, nocturnal pulse oxymetry could be included in clinical assessment. CCL-18 and CRP levels correlate with physiological markers of fibrosis.

Effect of respiratory rate on airway deadspace ventilation during exercise in cystic fibrosis.

Gas exchange during exercise in patients with cystic fibrosis (CF) is characterised by an elevated physiological deadspace to tidal volume ratio. While this has been attributed to alveolar ventilation perfusion mismatch, there are other potential causes of the high proportion of wasted ventilation, including factors relating to the volume and the ventilation of the airway deadspace. CF (n = 6, F = 1, FEV1 26-63% pred) and control (n = 6, F = 2) subjects completed steady-state exercise on a cycle ergometer. Gas exchange was measured breath-by-breath and the volume of the airway deadspace (V(Daw)) determined using the equal areas method. Exercise data were interpolated to a CO2 output of 0.7 l/min. V(Daw) was similar in the two groups both at rest and during exercise. However, the airway deadspace ventilation (V(Daw)) (median (inter-quartile range)), patients, 6.8 (5.1-7.1) l/min; controls, 4.9 (3.5-5.6) l/min, P < 0.05) was significantly greater in the CF group due to a greater respiratory frequency. These results indicate that in CF patients, abnormally increased V(Daw) is an important contributor to the total (physiological) deadspace ventilation. Exercise performance in CF might be enhanced by efforts directed at facilitating an increase in exercise tidal volume and therefore the adoption of a more efficient pattern of breathing.

Giant coronary aneurysm in adult Kawasaki disease: angiographic, 64-slice coronary MDCT and cardiac MRI appearances.

BACKGROUND: Kawasaki disease is a small-to-medium-vessel vasculitis that preferentially affects infants and young children. This condition is rare in adults, and therefore the diagnosis can easily be missed in a patient presenting to a primary care clinic. We report an unusual case of a patient who presented with ventricular fibrillation on a background of adult Kawasaki disease. OBJECTIVES: To identify the advantages of using coronary multidetector computed tomography (MDCT) and cardiac magnetic resonance imaging (MRI) in diagnosing adult Kawasaki disease. METHODS: We studied a 52-year-old patient with Kawasaki disease using coronary angiography, cardiac MDCT and MRI. RESULTS: Invasive coronary angiography demonstrated an occluded right coronary artery (RCA) and appearances suspicious for a calcified giant RCA aneurysm. The full extent of the aneurismal RCA was depicted with MDCT. Cardiac MRI revealed a chronic inferior segment myocardial infarction representing an arrhythmia substrate. DISCUSSION: Our case highlights the increasing utility of contrast-enhanced cardiac MRI and MDCT in the diagnosis of this rare condition in adults.

Primary cardiac lymphoma: diagnostic tools and treatment challenges.

Primary cardiac lymphoma (PCL) is a rare malignancy and the optimal treatment strategy remains uncertain. It appears to respond much better to systemic chemotherapy than to surgery and it should be considered in the differential diagnosis of all cardiac tumours before definitive management is undertaken. We report a case of this rare disorder treated successfully with a combination of rituximab and cyclophosphamide, adriamycin, vincristine and prednisolone. The patient developed recurrent unstable ventricular tachycardia (VT) post-chemotherapy secondary to extensive scarring at the tumour site. The tumour as well as the post-treatment scarring is well illustrated by cardiac magnetic resonance imaging highlighting its usefulness in this setting. An implantable cardioverter defibrillator (ICD) was placed. This is only the second case in the literature of PCL to have an ICD placed for recurrent VT. A brief literature review is included.

Honeycomb cysts in idiopathic pulmonary haemosiderosis: high-resolution CT appearances in two adults.

Idiopathic pulmonary haemosiderosis (IPH) is characterized by recurrent episodes of pulmonary haemorrhage. The disease predominates in childhood, with approximately 20% of patients presenting in adulthood. Most patients present with dyspnoea, fatigue and recurrent haemoptysis, resulting in iron deficiency anaemia. High-resolution CT manifestations of IPH include patchy or diffuse ground glass opacity and consolidation resulting from alveolar haemorrhage. We describe a new high-resolution CT finding in two adults with IPH - multiple honeycomb cysts, which were characteristically focal and localized predominantly to the posterior and lateral basal segments. We suggest that the development of honeycombing in patients with IPH is a traction phenomenon resulting from recurrent haemosiderin deposition in the interstitium, which is known to lead to progressive fibrosis. These honeycomb cysts may indicate the sites of the most severe and recurrent alveolar haemorrhage in adults with IPH.

Bronchiolitis obliterans following lung transplantation: early detection using computed tomographic scanning.

BACKGROUND: Computed tomographic (CT) scanning may enable earlier diagnosis of chronic lung allograft dysfunction than forced expiratory volume in 1 second (FEV1). A study was undertaken to determine intra-observer and inter-observer agreement of composite and air trapping CT scores, to examine the association of FEV1 with the composite and air trapping CT score, and to relate the baseline composite CT score to changes in FEV1 and changes in the composite CT score over 1 year. METHODS: Lung function and baseline CT scans following transplantation and at subsequent annual follow ups were analysed in 38 lung transplant recipients. Scans were randomly scored by two observers for bronchiectasis, mucus plugging, airway wall thickening, consolidation, mosaic pattern, and air trapping, and re-scored after 1 month. CT scores were expressed on a scale of 0-100 and correlated with FEV1 as a percentage of the post-transplant baseline value. RESULTS: The mean (SD) interval between baseline and follow up CT scans was 11.2 (4.7) months. Inter-observer and intra-observer agreement was good for both the composite and air trapping CT scores. There was a significant association between FEV1 and the composite CT score, with each unit of worsening in the baseline composite CT score predicting a 1.55% and 1.37% worsening in FEV1 over the following year (p<0.0001) and a 1.25 and 1.12 unit worsening in the composite CT score (p<0.0001) for observers 1 and 2, respectively. CONCLUSION: These findings indicate a potential role for a composite CT scoring system in the early detection of bronchiolitis obliterans.

Diagnosis and treatment of enterovesical fistulae.

OBJECTIVE: Enterovesical fistulae are a recognized complication of a variety of inflammatory and neoplastic conditions. Despite advances in imaging and treatment the diagnosis may be delayed and the management remains diverse. We describe our experience with their diagnosis and management. PATIENTS AND METHODS: This retrospective study encompassed all patients referred over a 10-year period with clinical suspicion of or confirmed enterovesical fistula. Demographics, clinical presentation, aetiology and clinical outcome were evaluated. Mean follow-up was 18 months (range 6-50 months). RESULTS: Thirty patients were studied. The mean age was 63.5 years (range 23-92 years). Fifteen (50%) patients presented with classical urinary symptoms (pneumaturia, faecaluria and recurrent urinary tract infections). The commonest investigations (n, % positive) included CT (15, 80), cystoscopy (16, 87.5), endoscopy (11, 54.5) and barium enema (8, 50). There were 20 inflammatory and 10 neoplastic aetiologies. Five patients were treated conservatively and 25 patients underwent surgery. Surgery resulted in symptomatic cure in the majority of cases (22/25). CONCLUSION: Classical urinary symptoms were only evident in 50% of patients with confirmed fistulae. We advocate CT scanning as the optimum imaging modality before surgical intervention. Surgical treatment in a specialized unit remains the most effective treatment of enterovesical fistulae.

Minimal preparation computed tomography instead of barium enema/colonoscopy for suspected colon cancer in frail elderly patients: an outcome analysis study.

AIM: To evaluate the efficacy of minimal preparation computed tomography (MPCT) in diagnosing clinically significant colonic tumours in frail, elderly patients. MATERIALS AND METHODS: A prospective study was performed in a group of consecutively referred, frail, elderly patients with symptoms or signs of anaemia, pain, rectal bleeding or weight loss. The MPCT protocol consisted of 1.5 l Gastrografin 1% diluted with sterile water administered during the 48 h before the procedure with no bowel preparation or administration of intravenous contrast medium. Eight millimetre contiguous scans through the abdomen and pelvis were performed. The scans were double-reported by two gastrointestinal radiologists as showing definite (>90% certain), probable (50-90% certain), possible (<50% certain) neoplasm or normal. Where observers disagreed the more pessimistic of the two reports was accepted. The gold standard was clinical outcome at 1 year with positive end-points defined as (1) histological confirmation of CRC, (2) clinical presentation consistent with CRC without histological confirmation if the patient was too unwell for biopsy/surgery, and (3) death directly attributable to colorectal carcinoma (CRC) with/without post-mortem confirmation. Negative end-points were defined as patients with no clinical, radiological or post-mortem findings of CRC. Patients were followed for 1 year or until one of the above end-points were met. RESULTS: Seventy-two patients were included (mean age 81; range 62-93). One-year follow-up was completed in 94.4% (n=68). Mortality from all causes was 33% (n=24). Five histologically proven tumours were diagnosed with CT and there were two probable false-negatives. Results were analysed twice: assuming all CT lesions test positive and considering "possible" lesions test negative [brackets] (95% confidence intervals): sensitivity 0.88 (0.47-1.0) [0.75 (0.35-0.97)], specificity 0.47 (0.34-0.6) [0.87 (0.75-0.94)], positive predictive value 0.18 [0.43], negative predictive value 0.97 [0.96], positive likelihood ratio result 1.6 [5.63], negative likelihood ratio result 0.27 [0.29], kappa 0.31 [0.43]. Tumour prevalence was 12%. A graph of conditional probabilities was generated and analysed. A variety of unsuspected pathology was also found in this series of patients. CONCLUSIONS: MPCT should be double-reported, at least initially. "Possible" lesions should be ignored. Analysis of the graph of conditional probability applied to a group of frail, elderly patients with a high mortality from all causes (33% in our study) suggests: (1) if MPCT suggests definite or probable carcinoma, regardless of the pre-test probability, the post-test probability is high enough to warrant further action, (2) frail, elderly patients with a low pre-test probability for CRC and a negative MPCT should not have further investigation, (3) frail, elderly patients with a higher pre-test probability of CRC (such as those presenting with rectal bleeding) and a negative MPCT should have either double contrast barium enema (DCBE) or colonoscopy as further investigations or be followed clinically for 3-6 months. MPCT was acceptable to patients and clinicians and may reveal significant extra-colonic pathology.