Aortic stenosis and transthyretin cardiac amyloidosis: the chicken or the egg?

BACKGROUND: Aortic stenosis (AS) and transthyretin cardiac amyloidosis (TTR-CA) are both frequent in elderly. The combination of these two diseases has never been investigated. AIMS: To describe patients with concomitant AS and TTR-CA. METHODS: Six cardiologic French centres identified retrospectively cases of patients with severe or moderate AS associated with TTR-CA hospitalized during the last 6 years. RESULTS: Sixteen patients were included. Mean ± SD age was 79 ± 6 years, 81% were men. Sixty per cent were NYHA III-IV, 31% had carpal tunnel syndrome, and 56% had atrial fibrillation. Median (Q1;Q4) NT-proBNP was 4382 (2425;4730) pg/mL and 91% had elevated cardiac troponin level. Eighty-eight per cent had severe AS (n = 14/16), of whom 86% (n = 12) had low-gradient AS. Mean ± SD interventricular septum thickness was 18 ± 4 mm. Mean left ventricular ejection fraction and global LS were 50 ± 13% and -7 ± 4%, respectively. Diagnosis of TTR-CA was histologically proven in 38%, and was based on strong cardiac uptake of the tracer at biphosphonate scintigraphy in the rest. Eighty-one per cent had wild-type TTR-CA (n = 13), one had mutated Val122I and 19% did not had genetic test (n = 3). Valve replacement was surgical in 63% and via transcatheter in 13%. Median follow-up in survivors was 33 (16;65) months. Mortality was of 44% (n = 7) during the whole follow-up period. CONCLUSIONS: Combination of AS and TTR-CA may occur in elderly patients particularly those with a low-flow low-gradient AS pattern and carries bad prognosis. Diagnosis of TTR-CA in AS is relevant to discuss specific treatment and management.

A case of pericardial tuberculoma presenting as a left anterior chest wall mass.

INTRODUCTION: Tuberculosis (TB) can present both in its pulmonary or extra-pulmonary forms. Cardiac tuberculoma previously described only after autopsy is continuously seen with the advent of more advanced imaging modalities. CASE REPORT: A 23-year-old male with a four month history of a progressively increasing left anterior thoracic wall mass of 5 cm in diameter was referred from oncology for clinical re-evaluation and for echocardiography. Systemic examination was essentially normal. Transthoracic and trans-oesophageal echocardiography showed the presence of a pericardial mass around the right atrioventricular junction. Thoracic CT scan showed an anterior mass in left chest wall extending to the pericardium and also the presence of mediastinal lymphadenopathy. Mantoux test was positive and histological examination of tissue biopsy confirmed the presence of TB. However, blood tests and culture of aspirated purulent fluid were unyielding. A diagnosis of pericardial tuberculoma with mediastinal and parietal extension was made and patient was successfully treated with standard anti-TB chemotherapy. DISCUSSION: The possible differential diagnoses for chest wall tumors are varied and a high degree of suspicion is needed to diagnose cardiac tuberculoma especially in endemic regions. Imaging though helpful in morphological description cannot make precise diagnosis. The diagnosis depends on histological and culture studies. There is usually a good evolution with anti-TB treatment. CONCLUSION: In an era of an increasing number of acquired immune-compromised patients, and with increasing number of diagnoses of tuberculosis, a diagnosis of cardiac tuberculoma should be considered in patients presenting with a thoracic wall mass.

Impact of polyvascular disease on baseline characteristics, management and mortality in acute myocardial infarction. The Alliance project.

BACKGROUND: A substantial number of patients with acute myocardial infarction (AMI) have polyvascular disease (PolyVD), defined as cerebrovascular disease (CVD), peripheral arterial disease (PAD) or both. AIM: To investigate the impact of PolyVD on baseline characteristics, management and outcomes. METHODS: The Alliance project is a multicentre, cross-sectional database of patients with myocardial infarction throughout France from 2000 to 2005. A pooled analysis of individual patient data was performed by aggregating data from five registries, representing 9783 patients hospitalized for acute coronary syndromes. Data were collected on history of PAD and CVD and correlated to baseline characteristics, management and hospital outcomes. RESULTS: Eight thousand nine hundred and four patients had full datasets for this analysis (13% with a history of CVD or PAD, 87% without). Patients with PolyVD were older (72 vs 65 years, p<0.0001), had a more frequent history of AMI (26% vs 15%, p<0.0001), percutaneous coronary intervention (PCI), coronary artery bypass graft (CABG), renal insufficiency (12% vs 3%, p<0.0001) and consistently more risk factors for atherosclerosis (hypertension, dyslipidaemia, smoking, diabetes), but less frequently a body mass index>30 kg/m(2) (14.0% vs 20.1%, p<0.0001) compared to patients with coronary artery disease (CAD) alone. Killip class, left-ventricular ejection fraction and GUSTO risk score were all worse among patients with PolyVD. Management of patients with PolyVD was less aggressive (with later admission and less frequent use of in-hospital angiography or evidence-based therapies at discharge). Mortality of patients with PolyVD was consistently higher than in those with CAD alone, regardless of age. Multivariable analysis, adjusting for age, showed that both PAD (odds ratio 1.36 95% confidence interval 1.03-1.79) and history of CVD (odds ratio 1.74, 95% confidence interval 1.27-2.40) were independent predictors of hospital mortality relative to patients with CAD only. CONCLUSION: Patients with PolyVD represented a substantial group among AMI patients, at particularly high risk of death, yet were managed less aggressively than patients with CAD alone. This was associated with markedly higher in-hospital mortality. Further research is warranted to design and test strategies to decrease mortality in this high-risk subset.