Dania Mohty

Current guideline-based classifications of mitral stenosis rely primarily on parameters of valvular narrowing and its associated clinical symptoms. In recent years, novel phenotyping approaches based on the concept of sequential cardiac chamber involvement have been developed for other cardiovascular conditions, including mitral regurgitation, aortic stenosis, and heart failure with preserved ejection fraction. Similarly, the development of an extra-valvular echocardiographic staging system for mitral stenosis, grounded in the extent of cardiac chamber involvement, appears warranted to improve risk stratification and tailor clinical management.

Introduction: To assess how national income level influences global variation in the diagnosis and management of heart failure with preserved ejection fraction (HFpEF). Methods: A web-based survey on HFpEF diagnosis and treatment was distributed worldwide from May to July 2023 through email, scientific societies, and social networks. Respondents provided demographic information and details on diagnostic practices, resource availability, and treatment approaches. Countries were categorized according to the 2023 World Bank income classifications: high-income countries (HICs), upper-middle-income countries (UMICs), lower-middle-income countries (LMICs), and low-income countries (LICs). Results: 1459 physicians from 91 countries completed the survey (median age 42 years; 61% male). Income level influenced the type of clinician managing HFpEF, with cardiologists more frequently involved in UMICs and LMICs/LICs than HICs. Respondents in HICs reported a higher proportion of HFpEF among their HF patients (40% vs 30% elsewhere; P < .001). Use of natriuretic peptides varied significantly across settings, as did the availability of echocardiographic parameters required for HFpEF assessment, which was highest in HICs. Screening for coronary artery disease in new HFpEF cases ranged from 22% in LMICs/LICs to 40% in UMICs. Availability of ACE inhibitors, ARBs, MRAs, and loop diuretics showed clear income-related differences, while SGLT2 inhibitors were widely available across all groups (88%). Multi-disciplinary HF programmes were most common in HICs (62%) and least common in LMICs/LICs (24%; P < .001). Conclusion: National income level is associated with major differences in diagnostic testing, medication access, specialist involvement, and multi-disciplinary care for HFpEF. These disparities highlight the need for scalable, resource-adapted strategies to optimize HFpEF care globally.

Background: Aortic stenosis (AS) and mitral stenosis (MS) are valvular heart diseases that may present concomitantly, particularly in regions where rheumatic heart disease remains prevalent. While each condition has been extensively studied in isolation, there is limited data on the clinical characteristics of patients with combined AS-MS. Methods: We retrospectively identified patients with significant AS and concomitant significant MS from the echocardiography database between 2003 and 2018. Exclusion criteria included left ventricular ejection fraction p < 0.001), had a lower prevalence of hypertension (44% vs. 64%, P = 0.017) and diabetes (22% v. 42%, p = 0.013), and a greater prevalence of atrial fibrillation (17% vs. 5%, p = 0.003). Patients with combined AS-MS had a significantly larger left atrial size (4.79 ± 0.70 cm vs. 3.93 ± 0.73 cm, p < 0.001), higher peak tricuspid velocities (3.14 ± 0.59 m/s vs. 2.72 ± 0.45 m/s, p < 0.001), and greater prevalence of moderate or severe tricuspid regurgitation (15% vs. 1%, p < 0.001). Echocardiographic parameters assessing transvalvular flow rate did not differ significantly between the two groups. After multivariate adjustment for age and gender combined AS-MS was associated with worse 5-year overall survival (HR 2.672, 95% CI 1.060–6.732, p = 0.037). Conclusion: Combined mitral and aortic stenosis is not uncommon (11%) but linked to worse outcomes than isolated AS. Despite expectations, concomitant significant MS did not increase prevalence of paradoxical low-flow, low-gradient AS.

Aims: Cardiac amyloidosis (CA) is a potentially fatal multisystemic disease that remains significantly underdiagnosed, particularly in the Middle East. This study aims to evaluate the prevalence and clinical characteristics of CA in a high-risk population at a tertiary centre in Saudi Arabia. Methods: This cross-sectional, retrospective, single-centre study was conducted at a tertiary hospital in Riyadh, Saudi Arabia. We reviewed the medical records of heart failure patients seen between August 2018 and July 2022 who exhibited red flags for CA and subsequently underwent CA screening. Red flags that prompted the workup included at least two of the following factors: the presence of unilateral or bilateral carpal tunnel syndrome, a family history of transthyretin amyloid (ATTR) amyloidosis and specific electrocardiographic features (relative/absolute low QRS voltage, pseudoinfarct pattern and atrioventricular/interventricular conduction abnormalities). Echocardiographic red flags included mainly increased wall thickness (≥12 mm), significant diastolic dysfunction, reduced left ventricular (LV) longitudinal function, right ventricular (RV) dysfunction and elevated right atrial (RA)/pulmonary artery (PA) pressure. Cardiac magnetic resonance (CMR) red flags included aspects similar to those in an echocardiogram as well as a subendocardial or transmural late gadolinium enhancement (LGE) pattern. These patients were assessed for CA through technetium-99m pyrophosphate ([99mTc]Tc-PYP) bone scintigraphy, serum and urine protein electrophoresis with immunofixation and a serum-free light chain assay. Results: A total of 177 patients were screened, of which 21.0 (11.9%) patients were diagnosed with transthyretin amyloid CA (ATTR-CA) and 13 (7.3%) patients were diagnosed with light chain CA (AL-CA). Compared with patients with negative/equivocal [99mTc]Tc-PYP scans (grades 0–1), patients with positive [99mTc]Tc-PYP scans (grades 2–3) were older (78.0 vs. 68.0 years, P < 0.001), had higher levels of troponin (P = 0.003) and N-terminal pro-brain natriuretic peptide (NT-proBNP) (P < 0.001), had a higher LV mass index (P < 0.001), displayed a more depressed global longitudinal strain (GLS) (P < 0.001) with a greater prevalence of a relative apical sparing pattern (P < 0.001) and demonstrated a higher incidence of first-degree atrioventricular block (P = 0.008) and low voltage patterns on electrocardiography (P < 0.001). Patients with ATTR-CA and AL-CA were more likely to have a subendocardial or transmural LGE pattern on CMR (P < 0.001) and had a significantly lower overall survival (P < 0.001) when compared with other heart failure aetiologies. Conclusions: This is the first study to describe the clinical characteristics and outcomes of CA in the Middle East and Saudi Arabia. The prevalence of CA among screened heart failure patients here aligns with major international studies, suggesting significant underdiagnosis in the region. Therefore, larger multicentric studies and regional screening programmes are urgently needed to accurately characterize the epidemiology and outcomes of CA in the Middle East.

Transthyretin cardiac amyloidosis in Saudi Arabia and the Middle East: insights, projected prevalence and practical applications

Background: Cardiac amyloidosis (CA) is a chronic progressive disease caused by the deposition of amyloid fibrils in cardiac tissues. Diagnosis and management of CA are complicated and have developed over the years.Hypothesis: Middle Eastern countries have significant knowledge disparities in diagnosing, managing, and treating different subtypes of CA.Methods: An online survey was sent to cardiologists in four countries (Saudi Arabia, Lebanon, Egypt, and Iraq) interested in heart failure and practicing for more than a year. The survey questioned the characteristics of the participants and their institutions. It addressed their knowledge and practices in CA specifically diagnostic modalities, treatment options, and interest in education and knowledge exchange.Results: A total of 85 physicians participated in the survey. There was a variation in the participating cardiologists' knowledge, experience level, and readiness of their institutes to manage patients with ATTR‐CM. Most participants believed that a high rate of ATTR‐CM misdiagnosis existed. Participants' knowledge of the diagnostic modalities and “red flags” raising suspicion about ATTR‐CM varied. Another challenge was the availability of essential diagnostic modalities among various cardiology centers. A knowledge gap was also observed regarding updates in ATTR‐CM management. However, there was a high endorsement of the need for more education, physician networking, and knowledge exchange.Conclusions: This survey highlighted the need for increasing awareness levels among cardiologists in the four selected Middle Eastern countries. Cardiologists are most likely to benefit from additional training and knowledge exchange on the latest management advances of this disease. Thus, measures must be taken to focus on the physician's awareness of ATTR‐CM patient journey to achieve a better quality of care and outcome.

Aims: Multimodal imaging has allowed cardiac amyloidosis (CA) to be increasingly recognised as a treatable cause of heart failure with preserved ejection fraction, but its prognosis remains poor due to late diagnosis. To assess the left ventricular diastolic function (LVDF) patterns in a large contemporary CA cohort according to the current recommendations and to identify their determinants. Methods and Results: We conducted a monocentric, observational study on a cohort of CA patients from a tertiary CA referral centre. Diastolic function was analysed using standard echocardiography and clinical, laboratory and survival parameters were collected. Four hundred and sixty-four patients with one of the three main type of CA were included: 41% had grade III diastolic dysfunction (restrictive mitral pattern), 25% had grade II diastolic dysfunction, and 25% had grade I diastolic dysfunction; 9% were unclassified. No difference was found between the main CA types. After multivariate analyses, grades II and III were independently associated with dyspnoea, elevated NT-proBNP, cardiac infiltration and systolic dysfunction (global longitudinal strain). Grade I patients had a better prognosis. Conclusions: All LVDF patterns can be observed in CA. One quarter of CA patients have grade I LVDF, reflecting the emergence of earlier stage-related phenotypes with a better prognosis.