Canadian Journal of Cardiology最新文献

Background: Transthyretin cardiac amyloidosis (ATTR-CA) is increasingly recognized as a treatable form of heart failure. Atrial fibrillation (AF) is common in patients with ATTR-CA. Whether recent-onset AF can be used as an early marker to identify patients with ATTR-CA has not been elucidated.

Methods: This was a prospective study conducted at 3 Spanish centres. ATTR-CA noninvasive screening was offered to patients ≥ 65 years of age recently diagnosed (< 1 year) with nonvalvular AF and who had ≥ 1 echocardiographic, electrocardiographic, or clinical sign suggestive of ATTR-CA.

Results: A total of 121 patients were included (75% male, mean age 77 ± 7 years). Ten patients (8.3%; 95% confidence interval [CI],4-14.7%), were diagnosed with cardiac amyloidosis (CA): 5 with definite wild-type ATTR-CA (ATTRwt), 4 with likely ATTRwt, and 1 with undetermined CA. Compared with patients without CA, patients with CA were older (84 ± 4 vs 76 ± 7 years; P < 0.001), more frequently men (90% vs 59%; P = 0.047), presented higher median N-terminal pro-B-type natriuretic peptide (NTproBNP) (3800 pg/L, interquartile range [IQR]:1682-6101 vs 1048 pg/mL, IQR: 427-3154; P = 0.017) and higher left ventricular hypertrophy (LVH) (14 mm, IQR: 13-17 vs 12 mm, IQR: 12-13; P = 0.003). Patients with CA also showed higher rate of permanent AF (90% vs 49.5%; P = 0.018) and a greater need for pacemaker implantation during follow-up (30% vs 7.3%; P = 0.049). No differences in mortality were observed between patients with and without CA after a median follow-up of 13 months (IQR: 11-16 months).

Conclusions: Routine DPD scanning in elderly patients with recent-onset AF, LVH and an additional red flag may help to identify patients with ATTR-CA. However, larger studies evaluating this strategy in more diverse clinical settings would be required.

Backgroud: Transcatheter aortic valve replacement (TAVR) is a less invasive treatment option for patients with severe aortic stenosis (AS); however, its economic benefits in patients with low to intermediate surgical risk remain controversial and vary by country. We conducted a systematic review to compare the economic benefits of TAVR versus surgical aortic valve replacement (SAVR).

Methods: We searched six databases, including PubMed, Medline, Scopus, Web of Science, Embase, and Clinical Trials for randomized controlled trials on the economic benefits of TAVR with different valve types and SAVR in symptomatic AS patients with low to intermediate surgical risk, from inception to October 2023. We extracted data on quality-adjusted life years (QALYs), and incremental cost-effectiveness ratio (ICER), with ICER converted to 2023 United States dollars (USD) exchange rates.

Results: Fifteen studies met the inclusion criteria, with the overall quality ranging from intermediate to high. Among these, TAVR was found to be cost-effective in 14 studies, while in one study conducted in a developing country, TAVR was not cost-effective. When adjusted to 2023 USD, the ICER values ranged from $3,669 to $340,038 per QALY gained.

Conclusion: TAVR appears to be a cost-effective alternative to SAVR in patients with low to intermediate AS. In all studies, TAVR was associated with a significant increase in QALYs compared to SAVR. As it is an expensive procedure, the cost-effectiveness of TAVR depends on each country's ICER and willingness-to-pay threshold.

Background: Danon disease (DD) is a rare X-linked dominant lysosomal storage disorder. Studies on DD paediatric patients are limited owing to the small number of cases and challenges in early detection.

Methods: We retrospectively analysed clinical and genetic data of 29 paediatric patients who visited our hospital for treatment of or genetic counselling for DD from July 2014 to December 2023.

Results: The mean age at diagnosis was 7.2 ± 5.9 years for boys (n = 21) and 9.4 ± 5.0 years for girls (n = 8). Asymptomatic elevated liver transaminase or creatine kinase (CK) levels were initial manifestations detected in 10 male patients (48%) and absent in female patients. Hypertrophic cardiomyopathy (HCM) was observed in 20 male patients (95%) and 7 female patients (88%), whereas dilated cardiomyopathy (DCM) was not detected. Ventricular preexcitation (VP) was observed initially in 10 patients (36%) and in 15 (54%) at latest evaluation. Patients with VP had higher left ventricular posterior wall thickness in end-diastole z-scores than those without VP (5.6 ± 2.2 vs 3.5 ± 2.1; P = 0.029). During a median 2.7 years of follow-up, 2 male patients received heart transplants. One boy and 1 girl died of heart failure and sudden cardiac arrest, respectively. Twenty-three pathogenic LAMP2 variants were identified, including 7 novel variants.

Conclusions: A retrospective review of 29 DD cases suggests an underrecognised asymptomatic period in male DD patients, characterised by elevations in serum CK and transaminases. HCM appears to be the only cardiac manifestation in paediatric female patients, unlike a high incidence of DCM in adult female patients. The incidence of VP may increase with disease progression.