Long-term follow-up of patients with idiopathic inflammatory myopathy at Waitemata District Health Board.

BACKGROUND: Idiopathic inflammatory myopathies (IIMs) are an uncommon group of diseases that can be associated with significant morbidity and mortality related to systemic involvement or treatment-related complications. AIM: This study reports the concomitant diseases, extent of organ involvement, immunosuppressive use, treatment-related complications and damage outcome in a cohort of adult IIM. METHODS: All patients with IIM fulfilling at least 3 of the 4 Bohan and Peter criteria were identified. Medical notes were reviewed retrospectively. Data was collected for clinical presentation, autoantibody profile, immunosuppressives received, treatment-/disease-related complications and mortality rate. Systemic involvement was divided into 12 organ systems. Patient damage index was calculated using the Myositis Damage Index (MDI) tool. RESULTS: Twenty patients were identified from a single centre, with median duration of follow-up of 9 years. The majority of patients had polymyositis, with the remaining having dermatomyositis. Osteoporosis and osteopenia were the most common concomitant diseases observed, at 111 per 1,000 patient years, followed by lung involvement with 78 events per 1,000 patient years follow-up. ANA was positive in 6/20(30%) patients with Jo-1 positivity in 10% of patients. Nineteen patients (95%) received steroids. There were significant steroid-related complications, with 85% of patients developing osteopenia/osteoporosis. The mean MDI score was 3.11. The mortality rate was 52/1,000 patient years and infection was the most common cause of death. CONCLUSION: There is significant treatment-related morbidity in adult IIM, with a high incidence of steroid-related complications, in particular, osteoporosis. Infections accounted for the most common cause of death.

Screening for pulmonary arterial hypertension in patients with scleroderma--a New Zealand perspective.

BACKGROUND: Pulmonary arterial hypertension (PAH) in scleroderma (SSc) patients is a devastating complication with high mortality if untreated. Early recognition and specific treatment of PAH may improve outcome. Regular interval screening for PAH is generally recommended in scleroderma patients especially with the availability of emerging new therapies. The aim of this study is to determine the self-reported screening and treatment practices for SSc-PAH amongst rheumatologists in New Zealand (NZ). METHODS: An anonymous online questionnaire survey was emailed to all rheumatologists in New Zealand. RESULTS: Responses were received from 65% (39/60) of rheumatologists. The majority of patients had limited SSc (lcSSc) (57%) versus diffuse SSc (dcSSc) (34%). Twelve percent of patients had PAH. Eighty-two percent of rheumatologists screened for PAH in all SSc patients regardless of symptoms. The most commonly used screening modalities were pulmonary function tests (PFT) (97%) followed by clinical examination (95%) and echocardiogram (TTE) (92%). The majority of rheumatologists performed screening tests on a yearly basis (80% used PFT and 64% used TTE). A right heart catheter was used to confirm PAH in 70% of patients. Sixty-four percent of rheumatologists extend screening interval time if their patients were clinically stable. The most common PAH-specific therapy used was sildenafil (57%) followed by bosentan (19%). Sixty-four percent of rheumatologists supported a national PAH-SSc screening guideline. CONCLUSION: This study has shown a wide variability of how NZ rheumatologists screen for PAH in scleroderma patients. The development of a PAH-SSc guideline for screening and diagnosis may help standardise treatment practices in NZ.

Utility of cardiac magnetic resonance in the evaluation of unselected patients with possible arrhythmogenic right ventricular cardiomyopathy.

INTRODUCTION: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare but important cause of sudden cardiac death. We investigated the role of cardiac magnetic resonance imaging (CMR) in the evaluation of patients with suspected ARVC referred by a general cardiology service. METHODS: Ninety-two patients (mean age 48 ± 15, 49% female), referred for CMR assessment of possible ARVC, were reviewed. CMR included both functional and tissue characteristic imaging. RESULTS: No patients had ARVC based on the 1994 Task Force Criteria (TFC) prior to CMR, but 4 met proposed Modified TFC; 15% met one major (±1 minor) TFC, 71% 1 or 2 minor TFC, and 14% no TFC. Reasons for CMR referral included symptomatic arrhythmia of likely RV origin (28%), Electrocardiogram/Holter abnormalities (28%), echocardiographic features suspicious of ARVC (19%), and family history of ARVC (8%). CMR findings strongly suggestive of ARVC were found in nine patients (10%), although only three were considered typical. Of these patients two met 1 major TFC and seven met 1 or 2 minor TFC. CMR findings included RV thinning, aneurysm, and diastolic out-pouching, but only 1 patient had definite fatty infiltration of the RV. Incidentally, CMR detected important, previously undiagnosed pathology, including anomalous pulmonary venous drainage (2 patients) and non-ischaemic cardiomyopathy (6%). CMR was normal in 63%, with minor abnormalities in 29%. CONCLUSIONS: CMR may play an important diagnostic role in the evaluation of possible ARVC. Patients who do not meet TFC for diagnosis may have CMR features typical of ARVC. Additionally CMR may detect other hitherto undiagnosed structural or functional abnormalities that alter patient management. However the majority of patients referred have a low pretest probability of ARVC, and the rate of normal CMR scans is high.

An uncommon cause of myocardial infarction.

A 73 year-old lady with hypertension and chronic atrial fibrillation (AF) developed chest pain followed by ventricular fibrillation (VF) cardiac arrest. Her electrocardiogram post-cardioversion revealed inferior ST-elevation myocardial infarction (MI). Her coronary arteries were angiographically normal. Contrast-enhanced cardiac magnetic resonance(CE-CMR) demonstrated both an inferior subendocardial infarction and left atrial (LA) appendage thrombus suggesting cardioembolism as the most likely cause of her presentation.

Prevalence and prognostic significance of left ventricular dysfunction in patients presenting acutely with atrial fibrillation.

CONDENSED ABSTRACT: The prevalence and prognostic importance of CM occurring as a consequence of AF is poorly defined. This study investigated the incidence of CM in patients with AF, its clinical features and long-term outcomes. We demonstrated that CM is common in patients presenting acutely with newly diagnosed rapid AF, and carries a worse long-term prognosis. Systolic dysfunction was reversible in an important proportion of patients, suggesting a greater prevalence of rate-related CM in AF than has previously been postulated. This underscores the importance of appropriate rhythm management strategies and repeat imaging studies. BACKGROUND: Atrial fibrillation (AF) may precipitate LV dysfunction, potentially leading to cardiomyopathy (CM). The prevalence and prognostic importance of CM occurring as a consequence of AF is poorly defined. We investigated the incidence of CM in patients with AF, its clinical features and long-term outcomes. METHODS: We reviewed 292 consecutive patients (average age 72 +/- 13yrs) presenting acutely with AF and tachycardia over a 3 year period from June 2004. Clinical details were obtained from medical records. CM was defined as ejection fraction (EF)

Characteristics and prognostic importance of myocardial fibrosis in patients with dilated cardiomyopathy assessed by contrast-enhanced cardiac magnetic resonance imaging.

INTRODUCTION: Dilated cardiomyopathy (DCM) is associated with significant morbidity and mortality. Contrast-enhanced cardiac MRI (CE-CMR) can detect potentially prognostic myocardial fibrosis in DCM. We investigated the role of CE-CMR in New Zealand patients with DCM, both Maori and non-Maori, including the characteristics and prognostic importance of fibrosis. METHODS: One hundred and three patients (mean age 58 ± 13, 78 male) referred for CMR assessment of DCM were followed for 660 ± 346 days. Major adverse cardiac events (MACE) were defined as death, infarction, ventricular arrhythmias or rehospitalisation. CE-CMR used cines for functional analysis, and delayed enhancement to assess fibrosis. RESULTS: Myocardial fibrosis was present in 30% of patients, the majority of which was mid-myocardial (63%). Volumetric parameters were similar in patients with or without fibrosis. At 2 years patients with fibrosis had an increased rate of MACE (HR = 0.77, 95% CI 0.3-2.0). Patients with full thickness or subendocardial fibrosis had the highest MACE, even in the absence of CAD). More Maori had fibrosis on CE-CMR (40% vs. 28% for non-Maori), and the majority (75%) was mid-myocardial. Maori and non-Maori had similar outcomes (25% vs. 24% with events during follow-up). CONCLUSIONS: DCM patients frequently have myocardial fibrosis detected on CE-CMR, the majority of which is mid-myocardial. Fibrosis is associated with worse outcome in the medium term. The information obtained using CE-CMR in DCM may be of incremental clinical benefit.

Bartonella henselae aortic valve endocarditis mimicking systemic vasculitis.

A 28-year-old man with a bicuspid aortic valve presented with facial droop and slurred speech with several months of constitutional symptoms of night sweats, weight loss and productive cough. Examination confirmed aortic regurgitation, palpable spleen and left facial droop. Multiple peripheral blood cultures were negative. Inflammatory markers, cytoplasmic staining antineutrophil cytoplasmic antibodies (cANCA) and anti-PR3 antibody were all elevated. MRI of the brain and CT of the chest and abdomen confirmed embolic infarcts to brain, kidney and spleen. Transoesophageal echocardiogram (ECG) showed valve vegetations and severe aortic regurgitation. Endocardial Wegener's granulomatosis was considered. Aortic valve replacement was performed. Grindings from aortic valve leaflets were analysed for rpoB gene, which confirmed the presence of Bartonella henselae. Serological assays demonstrated B henselae IgM 20 (normal <20) and IgG >2048 (normal < 64). The patient completely recovered after prolonged antibiotic treatment. Culture-negative infective endocarditis may mimic vasculitis and be associated with positive cANCA. Serology and molecular techniques may aid diagnosis.

Pregnancy complicating Wegener's granulomatosis.

Pregnancy associated with Wegener's granulomatosis is rare. Therapeutic options are limited. There is a paucity of published literature to guide clinical decision-making in these complex patients. Two cases are presented. Firstly, a 33-year-old woman with generalized Wegener's in remission and off all medications presented with a flare in the third trimester with haemoptysis, raised C-reactive protein and c-anti-neutrophilic cytoplasmic antibody (c-ANCA) levels. Her care was complicated by florid steroid-induced psychosis. With deteriorating disease control, she was treated with pulsed-intravenous cyclophosphamide with a good response. She delivered a healthy baby at 38 weeks. She had a severe postpartum flare. Secondly, a 37-year-old woman with limited Wegener's in remission for the last two years and off all treatment became pregnant after pre-conception counselling. A normal baby was delivered at term. An exhaustive review of all published literature on Wegener's activity in pregnancy is presented along with therapeutic options and recommendations.

Pericardial effusions with tamponade and visceral constriction in patients with rheumatoid arthritis on tumour necrosis factor (TNF)-inhibitor therapy.

Tumour necrosis factor-inhibitor (TNF-inhibitor) therapy is increasingly used for the treatment of rheumatoid arthritis. While it is effective for the articular manifestations of rheumatoid arthritis we have reason to believe that it is less effective for extra-articular disease. We present two cases of life-threatening cardiac tamponade in two patients with well-controlled rheumatoid arthritis on adalimumab. An extensive literature search was carried out and three other patients were found. We believe that these cases highlight the need for rheumatologists to be vigilant for extra-articular manifestations of rheumatoid arthritis even in the presence of quiescent joint disease while on TNF-inhibitors.

Myocardial scar detected by contrast-enhanced cardiac magnetic resonance imaging is associated with ventricular tachycardia in hypertrophic cardiomyopathy patients.

INTRODUCTION: Hypertrophic cardiomyopathy (HCM) is associated with myocardial scarring and ventricular tachycardia (VT). Contrast-enhanced cardiac magnetic resonance imaging (CE-CMR) can quantify myocardial scar, and scar imaging has been documented in patients with HCM. We investigated the assessment of myocardial scar in HCM patients using CE-CMR, and its correlation with proven VT. METHODS: Twenty-five patients (mean age 54 +/- 8) with HCM who underwent CE-CMR were identified, and clinical data obtained from chart review. Parameters of LV function were calculated from cine imaging, and myocardial scar was assessed using delayed enhancement imaging following gadolinium administration. RESULTS: Myocardial scar was detected in 16 (64%) patients with a mean mass 9 +/- 15 g. Scar was patchy, mid-myocardial and located in the basal anteroseptum, and RV insertion sites. Scar was seen in septal, apical and concentric variants of HCM. Scar mass correlated with both LV Mass (r2 = 0.74) and maximal LV wall thickness (r2 = 0.42). VT occurred in 32% of patients, and was associated with both increased scar mass and wall thickness compared to non-VT patients (21 +/- 22 g vs. 4 +/- 6 g, and 2.4 +/- 0.5 cm vs. 1.8 +/- 0.5 cm, p < 0.05). LV size and function were similar in patients with and without VT. A scar mass of >7 g predicted the presence of VT with a sensitivity of 75% and specificity 82%. CONCLUSIONS: Myocardial scar imaged by CE-CMR is common in patients with HCM, and is predictive of VT. Scar is seen in all HCM variants, and is associated with maximal wall thickness. There may be a role for CE-CMR in improved risk stratification for individual patients with HCM.

Is it possible to distribute a scarce resource equitably? Access to invasive procedures for patients with acute myocardial infarction.

AIMS: To compare waiting times for inpatient cardiac catheterisation between a hospital with on-site cardiac catheterisation facility (Auckland City Hospital, ACH) and one of its referring hospitals (North Shore Hospital, NSH). METHODS: Patients were included if they were admitted ACH or NSH with a myocardial infarction, and subsequently underwent inpatient coronary angiography. RESULTS: 853 patients were identified from NSH and 600 from ACH. Patients from NSH waited significantly longer for coronary angiography (median delay 6 versus 3 days, p<0.0009) and fewer underwent this procedure within 48 hours of admission (11% versus 36%, p<0.0009). Delays in percutaneous coronary intervention were significantly longer for NSH patients (6 versus 3 days, p<0.0009), and fewer NSH patients underwent this procedure within 48 hours (12% versus 41%, p<0.0009). CONCLUSIONS: Inpatients with myocardial infarction waited longer for coronary angiography and percutaneous coronary intervention at a hospital without invasive facility than similar patients at the regional referral hospital with on-site invasive facility.

Detection of myocardial scar by contrast-enhanced cardiac magnetic resonance imaging in patients with troponin-positive chest pain and minimal angiographic coronary artery disease.

Patients who present with chest pain and elevated serum troponin levels may have minimal angiographic coronary artery disease, leading to diagnostic confusion. We investigated patients with troponin-positive acute coronary syndromes and minimal coronary artery disease (CAD) using contrast-enhanced cardiac magnetic resonance imaging to assess for myocardial scar. Twenty-three patients (54 +/- 8 years of age) who presented with chest pain, high troponin I level, and minimal angiographic CAD were included. Patients with a clinical pericarditis/myocarditis, tachyarrhythmia at presentation, previous myocardial infarction, or an alternative explanation for increased troponin I level were excluded. Myocardial scar was assessed with delayed-enhancement inversion-recovery imaging after administration of gadolinium. Hyperenhancement consistent with myocardial scar was seen in 7 of 23 patients (30%) and was located primarily in the right coronary artery distribution. Peak troponin level, clinical characteristics, and volumetric parameters were similar in patients with or without hyperenhancement. One patient had mid-myocardial enhancement that suggested undiagnosed myocarditis. There was a linear relation between the mass of myocardial scar and peak serum troponin level. In follow-up, subsequent cardiac events were more frequent in patients with hyperenhancement (43% vs 12.5%). In conclusion, patients with troponin-positive acute coronary syndromes may have significant myocardial scar even when minimal CAD is found on angiography.