Transthyretin amyloid cardiomyopathy (ATTR-CM) is increasingly recognized as a cause of heart failure in the elderly. Although wild-type transthyretin amyloidosis is the most frequent form of ATTR-CM found in the elderly, hereditary transthyretin amyloidosis (ATTRv) can also occur. We sought to determine the prevalence of ATTRv among elderly ATTR-CM patients, identify predictors of ATTRv and evaluate the clinical consequences of positive genetic testing in this population.

PurposeSeveral studies have shown that HIV patients are at higher risk of lung cancer. Our aim is to analyse the prevalence and features of lung cancer in HIV-infected patients. MethodsThe clinical charts of 4,721 HIV-infected patients seen in three hospitals of southeast Spain (study period 1992Á2012) were reviewed, and all patients with a lung cancer were analysed. ResultsThere were 61 lung cancers, giving a prevalence of 1.2%. There was a predominance of men (82.0%), and smokers (96.6%; mean pack-years 35.2), with a median age of 48.0 (41.7Á52.9) years, and their distribution according to risk group for HIV was: intravenous drug use 58.3%, homosexual 20.0%, and heterosexual 16.7%. Thirty-four (56.7%) patients were Aids cases, and 29 (47.5%) had prior pulmonar events: tuberculosis 16, bacterial pneumonia 9, and P. jiroveci pneumonia 4. The median nadir CD4 count was 149/mm 3 (42Á232), the median CD4 count at the time of diagnosis of the lung cancer was 237/mm 3 (85Á397), and 66.1%B350/mm 3 . 66.7% were on ART, and 70% of them had undetectable HIV viral load. The most common histological types of lung cancer were adenocarcinoma and epidermoid, with 24 (40.0%) and 23 (38.3%) cases, respectively. There were 49 (80.3%) cases with advanced stages (III and IV) at diagnosis. The distribution of treatments was: only palliative 23 (39.7%), chemotherapy 14 (24.1%), surgery and chemotherapy 8 (13.8%), radiotherapy 7 (12.1%), surgery 4 (6.9%), and other combined treatments 2 (3.4%). Forty-six (76.7%) patients died, with a median survival time of 3 months. The Kaplan-Meier survival rate at 6 months was 42.7% (at 12 months 28.5%). ConclusionsThe prevalence of lung cancer in this cohort of HIV-patients is high. People affected are mainly men, smokers, with transmission of HIV by intravenous drug use, and around half of them with prior opportunistic pulmonary events. Most patients had low nadir CD4 count, and were immunosuppressed at the time of diagnosis. Adenocarcinoma is the most frequent histological type. The diagnosis is usually made at advanced stages of the neoplasm, and mortality is high.

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This article refers to 'Effect of beta-blocker therapy on the response to mavacamten in patients with symptomatic obstructive hypertrophic cardiomyopathy' by M.T. Wheeler et al., published in this issue on pages 260-270.Nowadays, targeted therapies are emerging in the field of genetic cardiomyopathies. In the coming years, generic treatments and one fits all-based strategies will be hopefully replaced by treatments that focus on pathophysiological bases of disease. [1][2][3] The increasing knowledge of the genetic aetiologies and signalling pathways in cardiomyopathies explain the burst of interest in tailored therapies. Furthermore, thanks to multicentre and collaborative efforts by the medical community, we are reaching aetiological diagnoses more frequently and we are establishing genotype-phenotype correlations that will benefit patients with cardiomyopathies and their families.Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease. Its prevalence has been reported to range from 1:200 to 1:500 individuals and it is predominantly inherited in an autosomal dominant manner. HCM is associated with excessive cardiac myosin-actin cross-bridging 4 that result in hypercontractility, together with left ventricular hypertrophy and reduced left ventricular compliance. Left ventricular outflow obstruction (LVOTO), either at rest or with provocative manoeuvres, is present in almost 70% of patients with HCM. LVOTO is associated with impaired stroke volume and an increased risk of heart failure, as well as poorer survival. 5 The main role of pharmacologic LVOTO therapy is symptom relief, and until recently, obstructive HCM (oHCM) treatment has been limited to beta-blockers or non-dihydropyridine calcium channel blockers (CCB), with disopyramide as a second-line agent.Back in 2020, the double-blind, randomized, placebo-controlled phase 3 EXPLORER-HCM trial 6 highlighted the benefits of a newThe opinions expressed in this article are not necessarily those of the Editors of the European Journal of Heart Failure or of the European Society of Cardiology.

ObjectiveWe sought to investigate prevalence, incidence and prognostic implications of permanent pacemaker (PPM) implantation in patients with cardiac amyloidosis (CA), thereby identifying the predictors of time to PPM implantation.MethodsSeven hundred eighty-seven patients with CA (602 men, median age 74 years, 571 transthyretin amyloidosis (ATTR), 216 light-chain amyloidosis (AL)) evaluated at two European referral centres were retrospectively included. Clinical, laboratory and instrumental data were analysed. The associations between PPM implantation and mortality, heart failure (HF) or a composite endpoint of mortality, cardiac transplantation and HF were analysed.Results81 (10.3%) patients had a PPM before initial evaluation. Over a median follow-up time of 21.7 months (IQR 9.6–45.2), 81 (10.3%) additional patients (18 with AL (22.2%) and 63 with ATTR (77.8%)) underwent PPM implantation with a median time to implantation of 15.6 months (IQR 4.2–40), complete atrioventricular block was the most common indication (49.4%). Independent predictors of PPM implantation were QRS duration (HR 1.03, 95% CI 1.02 to 1.03, p<0.001) and interventricular septum (IVS) thickness (HR 1.1, 95% CI 1.03 to 1.17, p=0.003). The model to estimate the probability of PPM at 12 months and containing both factors showed a C-statistic of 0.71 and a calibration of slope of 0.98.ConclusionsConduction system disease requiring PPM is a common complication in CA that affects up to 20.6% of patients. QRS duration and IVS thickness are independently associated with PPM implantation. A PPM implantation at 12 months model was devised and validated to identify patients with CA at higher risk of requiring a PPM and who require closer follow-up.