Light chain and transthyretin cardiac amyloidosis: Clinical characteristics, natural history and prognostic factors. / Amiloidosis cardíaca por cadenas ligeras y por transtirretina: características clínicas, historia natural y predictores pronósticos.

INTRODUCTION AND OBJECTIVES: Light-chain amyloidosis (AL-CA) and transthyretin amyloidosis (ATTR-CA) are the most common types of cardiac amyloidosis (CA). We sought to study the clinical characteristics and prognosis of both diseases. METHODS: We conducted a single-centre, retrospective review of all patients diagnosed with CA between 1998 and 2018. Clinical characteristics, complementary tests, survival and other adverse clinical events were studied. RESULTS: We identified 105 patients with CA, 65 ATTR-CA and 40 AL-CA. Mean age was 74.4 years; 24.8% were women. In both groups, heart failure was the most frequent clinical presentation (55.2%). The most prevalent electrocardiographic findings were the pseudoinfarct pattern (68.5%) and a Sokolow-Lyon index < 1.5 mV (67.7%), with no differences between the two subtypes of CA. One-year, 3-year, and 5-year survival was 43.3%, 40.4% and 35.4%, respectively, in AC-AL patients, and 85.1%, 57.3% and 31.4% in AC-ATTR patients (p = 0.004). AL-CA subtype (HR 3.41; 95% CI 1.45-8.06; p = 0.005), previous admission for heart failure (HR 4.25; 95% CI 1.63-11.09; p = 0.003) and a NYHA class III-IV (HR 2.76; 95% CI; 1.09-7.03; p = 0.033) were independent predictors of mortality, while beta-blocker therapy was associated with longer survival (HR 0.23; 95% CI 0.09-0.59; p = 0.002). CONCLUSIONS: Differences exist between the clinical presentation of AL-CA and ATTR-CA patients. Both diseases, particularly AL-CA, are associated with poor life prognosis.

Effectiveness and safety beyond 10 years of percutaneous transluminal septal ablation in hypertrophic obstructive cardiomyopathy.

INTRODUCTION AND OBJECTIVES: Percutaneous transluminal septal ablation is an alternative treatment in patients with hypertrophic obstructive cardiomyopathy. However, due to the relatively new introduction of this technique, there is no information on its very long term results (>10 years). METHODS: The present study included consecutive patients treated in 5 centers between 1998 and 2003. We analyzed clinical, hemodynamic, and echocardiographic data at baseline and follow-up. RESULTS: A total of 45 patients were included; there were 31 (69%) women, the mean age was 62.4 (14) years, and 39 patients (86.6%) showed functional class III or IV. Septal thickness was 21.8 (3.5) mm, the peak resting gradient on echocardiography was 77 (39) mmHg, and mitral regurgitation was at least moderate in 22 patients (48.8%). During hospitalization, permanent pacemaker implantation was required in 3 patients and ventricular perforation (by pacing lead) occurred in 1 patient, requiring surgery. After a follow-up of 12.3 years (11.0-13.5 years), 2 patients (4.4%) died from cardiac causes (heart failure and posttransplantation), 3 patients required an implantable cardioverter-defibrillator (1 for primary prevention and 2 due to sustained ventricular tachycardia after cardiac surgery), and 2 underwent cardiac surgery (due to endocarditis and mitral regurgitation). In the last clinical review, functional class was I-II in 39 patients (86.6%) (P<.0001), the peak resting gradient was 16 (23) mmHg (P<.0001), and mitral regurgitation was absent or mild in 34 patients (75.5%) (P<.03). CONCLUSIONS: The results of this study suggest that septal ablation is safe and effective in the very long term. The procedure was not associated with a significant incidence of sudden death or symptomatic ventricular arrhythmias.

Effect of stent thrombosis on the risk-benefit balance of drug-eluting stents and bare metal stents.

INTRODUCTION AND OBJECTIVES: To determine the difference in the risk of stent thrombosis between drug-eluting stents (DES) and bare-metal stents (BMS) and to assess the clinical implications. METHODS: A retrospective analysis of two cohorts of patients treated at our center with either > or =1 paclitaxeleluting stents (PES) (n=430) or > or =1 BMSs (n=1230) during 2003-2004 was carried out using propensity score methods to compare the adjusted risks of stent thrombosis, instent restenosis, cardiovascular death, acute myocardial infarction (AMI), and target-lesion revascularization with the two stent types. RESULTS: After a median follow-up of 46 months, there was a higher risk of stent thrombosis in PESs (hazard ratio [HR]=3; 95% confidence interval [CI], 1.2-7.1] though the risk of in-stent restenosis was lower (HR=0.3; 95% CI, 0.2-0.7]. There was no difference in the risk of cardiovascular death, AMI or target-lesion revascularization. With PESs, the risks of target-lesion revascularization (HR=0.33; 95% CI, 0.2-0.7) and in-stent restenosis (HR=0.32; 95% CI, 0.2-0.7) were reduced during the first year of follow-up. After this time, the risks of target-lesion revascularization (HR=1.8; 95% CI, 1-3.2) and very late stent thrombosis (HR=12.8; 95% CI, 3-55.1) both increased. CONCLUSIONS: Our findings indicate that the balance of risks and benefits of PESs compared with BMSs is different in the early and late periods after stent implantation. The greatly increased risk of very late stent thrombosis in PESs could cancel out the clinical benefits associated with the reduction in in-stent restenosis observed in PESs relative to BMSs.