New York Heart Association Research Articles

LEFT VENTRICULAR NON COMPACTION CARDIOMYOPATHY ASSOCIATED WITH MITRAL VALVE PROLAPSE

Left ventricular non compaction (LVNC) is a rare condition characterized by prominent ventricular trabeculations and intertrabecular recesses,Mitral regurgitationwhen present is usually a result of annular dilation, mitral valve prolapse due to chordae tendinae rupture is quite rare.We present the case of a 48 year old male with no medical history who presented with worsening exertional dyspnea classified as NYHA class III. The physical examination found a systolic murmur heard at the apex. Transthoracic echocardiography and cardiac magnetic resonance confirmed the diagnosis of Left ventricular non compaction associated with posterior mitral valve prolapse with severe mitral regurgitation and preserved ventricular function who was successfully operated and found asymptomatic during the follow up.

Effect of Intravenous Iron on Functional Capacity in Patients with Ischemic Cardiomyopathy

Background: Iron deficiency is common in heart failure with reduced ejection fraction. Intravenous (IV) iron supplementation has been found to reduce morbidity in heart failure. However, the effect of IV iron on Bangladeshi patients with heart failure are not known. Objects: The study was done to see the effect of IV iron on functional capacity in Bangladeshi patients with ischaemic cardiomyopathy (ICM) and iron deficiency. Methodology: In this quasi-experimental study, out of 80 hospitalized patients with ICM and iron deficiency, 40 were given IV ferric carboxymaltose (FCM) in addition to standard therapy of heart failure, while another 40 were randomized to standard therapy alone. Primary outcome measure was changes in six-minute walk test (6MWT) distance, while secondary outcome measures were changes in New York Heart Association (NYHA) functional class, left ventricular ejection fraction (LVEF) and plasma NT-proBNP at Weeks 4 and 12. Results: 6MWT distance improved significantly in patients receiving IV iron therapy compared to the patients not receiving IV iron. LVEF improved and plasma NT-proBNP decreased significantly in IV irontreated patients than in patients receiving standard therapy. However, improvement in NYHA functional class was not statistically significant. Conclusion: In Bangladeshi patients with ICM and iron deficiency, IV FCM on top of standard heart failure therapy was associated with improved functional capacity. J Inv Clin Cardiol 2023; 5(2): 32-37

Large-bore Mechanical Thrombectomy Versus Catheter-directed Thrombolysis in the Management of Intermediate-risk Pulmonary Embolism: Primary Results of the PEERLESS Randomized Controlled Trial.

There is a lack of randomized controlled trial (RCT) data comparing outcomes of different catheter-based interventions for intermediate-risk pulmonary embolism (PE). PEERLESS is a prospective, multicenter, RCT that enrolled 550 intermediate-risk PE patients with right ventricular dilatation and additional clinical risk factors randomized 1:1 to treatment with large-bore mechanical thrombectomy (LBMT) or catheter-directed thrombolysis (CDT). The primary endpoint was a hierarchal win ratio (WR) composite of the following: 1) all-cause mortality, 2) intracranial hemorrhage, 3) major bleeding, 4) clinical deterioration and/or escalation to bailout, and 5) postprocedural intensive care unit (ICU) admission and length of stay, assessed at the sooner of hospital discharge or 7 days post-procedure. Assessments at the 24-hour visit included respiratory rate, mMRC dyspnea score, NYHA classification, right ventricle (RV)/left ventricle (LV) ratio reduction, and RV function. Endpoints through 30 days included total hospital stay, all-cause readmission, and all-cause mortality. The primary endpoint occurred significantly less frequently with LBMT vs CDT (WR 5.01 [95% CI: 3.68-6.97]; P<0.001). There were significantly fewer episodes of clinical deterioration and/or bailout (1.8% vs 5.4%; P=0.04) with LBMT vs CDT and less postprocedural ICU utilization (P<0.001), including admissions (41.6% vs 98.6%) and stays >24 hours (19.3% vs 64.5%). There was no significant difference in mortality, intracranial hemorrhage, or major bleeding between strategies, nor in a secondary WR endpoint including the first 4 components (WR 1.34 [95% CI: 0.78-2.35]; P=0.30). At the 24-hour visit, respiratory rate was lower for LBMT patients (18.3±3.3 vs 20.1±5.1; P<0.001) and fewer had moderate to severe mMRC dyspnea scores (13.5% vs 26.4%; P<0.001), NYHA classifications (16.3% vs 27.4%; P=0.002), and RV dysfunction (42.1% vs 57.9%; P=0.004). RV/LV ratio reduction was similar (0.32±0.24 vs 0.30±0.26; P=0.55). LBMT patients had shorter total hospital stays (4.5±2.8 vs 5.3±3.9 overnights; P=0.002) and fewer all-cause readmissions (3.2% vs 7.9%; P=0.03), while 30-day mortality was similar (0.4% vs 0.8%; P=0.62). PEERLESS met its primary endpoint in favor of LBMT vs CDT in treatment of intermediate-risk PE. LBMT had lower rates of clinical deterioration and/or bailout and postprocedural ICU utilization compared with CDT, with no difference in mortality or bleeding.

The impact of heart failure caregiver burden and patient heart failure severity: analysis of the needs assessment tool: advanced heart failure (NAT: PD-HF)

Abstract Background Not only patients with heart failure (HF), but also their caregivers have reported experiencing distress, depression, anxiety, social isolation, and health problems related to caring for a patient. Needs assessment tools have been used in clinical practice to identify patients' problems and needs from an early stage, however, their relationship with caregiver burden and the severity of the HF patients they care for has not been well investigated. Purpose The purpose of this study is to examine the relationship between distress and HF severity in patients and their caregivers using the Needs Assessment Tool: Progressive Disease-Heart Failure (NAT: PD- HF). Methods We enrolled consecutive 106 chronic HF patients who were admitted to a university hospital and assessed needs by the NAT: PD-HF between February 2023 and July 2023. We also enrolled 95 of their caregivers. The NAT: PD-HF is administered by the health care provider in the form of an interview with the patient and their caregiver. The questionnaire consists of an assessment of the patient's well-being and an assessment of the caregiver's ability to care for the patient. The healthcare provider completes the tool by indicating the level of distress (none, mild, significant) for each section. The severity of the New York Heart Association (NYHA) functional classification is examined in each patient. Results The median age was 72 years (interquartile range 59-81), and 41% were in NYHA class III or IV. The most common relationship between patients and their primary caregivers was partners (53%), followed by children (26%), parents (8%), and siblings (5%). There were 100 patients (94%) with more than mild distress, 57 with NYHA I or II and 43 with NYHA III or IV (P = 0.037). There were also 84 (88%) caregivers with more than mild distress, 47 with NYHA I or II, and 37 with NYHA III or IV of the patients they cared for (P = 0.026). Twenty-four patients (22.6%) were in severe distress, of which 10 were NYHA I or II and 14 were NYHA III or IV (P = 0.044). Among caregivers, 18 (18.9%) were in severe distress, of whom 6 with NYHA I or II and 12 with NYHA III or IV for whom they are caring (P = 0.010). Conclusions Regardless of the severity of HF, caregivers were found to experience distress as similar rate as that in the HF patients they cared for. The proportion of patients and caregivers experiencing distress was found to increase with the severity of HF. It is important to introduce palliative care for HF from the early stage of the disease and to support not only the patient but also the caregivers by using screening tools to identify distress.

Utilization and safety of digoxin in patients with chronic heart failure and atrial fibrillation with rapid ventricular rate

Abstract Background Digoxin can reduce symptoms and the risk of tachycardia-induced cardiomyopathy in patients with heart failure (HF) and concomitant atrial fibrillation (AF) with rapid ventricular rate. The effect of digoxin in HF and AF has not been tested in randomized controlled trials, and observational data have shown conflicting results linking digoxin to increased mortality in AF, though the nature of observational data makes it prone to bias and results difficult to interpret. Purpose To assess the utilization of digoxin in patients with HF and AF, and its association with New York Heart Association (NYHA) functional class and survival. Methods We used data from the Norwegian HF registry that collected data from all Norwegian HF clinics between 2013-2022. Data was collected from the first and last visit during optimizization of HF medical therapy. Associations with mortality status from Statistics Norway were assessed in unadjusted time-to-event analysis, and after adjustment for age, sex, body mass index, estimated glomerular filtration rate, aldosterone antagonists, renin-angiotensin-aldosterone system inhibitors, and beta blockers. Results The study included 4,877 HF patients, consisting of 4,067 (83.4%) with ejection fraction (EF) ≤40% and 810 (16.6%) with EF &amp;gt;40%.. The mean age was 67.7±12.0 years; 25.1% were women, and 27.3% had NYHA functional class III/IV. At the first visit, 1,339 (27.5%) patients had AF and 829 (61.9%) of these were treated with ≥50% of the beta-blocker target dose. AF with a ventricular rate ≥100 bpm was present in 277 (20.1%) patients, and 188 (67.9%) of these were on ≥50% beta-blocker target dose. Similarly, 262 (19.6%) of all AF patients and 44 (15.9%) of those with a ventricular rate ≥100 bpm were treated with digoxin. After optimization of HF medical therapy (median duration 140 [Q1–Q3 82–237] days), 1,039 (21.3%) patients had AF. Among these, 227 (21.9%) were treated with digoxin, including 9 (18.0%) patients with ventricular rate ≥100 bpm. Patients with AF who were started on digoxin experienced during the optimization period a larger decline in heart rate compared to those who were not (mean change -23.7±17.2 vs. -5.7±16.7 bpm, p=0.001), and more often an improvement in NYHA functional class (45% vs 30%, p=0.018). During median 779 [Q1–Q3 386–1,334)] days of follow-up after the last optimization visit, digoxin use was associated with lower mortality in HF patients with AF in unadjusted analysis (HR 0.67 [0.46–0.97]). After adjustment for baseline characteristics and other HF medical therapies, the association with mortality was attenuated and no longer significant (HR 0.96 [0.65–1.42]). Conclusion Digoxin was infrequently used in patients with HF and AF with rapid ventricular rate but was associated with a greater decrease in heart rate and improvement in NYHA functional class. The use of digoxin appears safe and was not associated with increased mortality.

Clinical characteristics and burden of non-obstructive hypertrophic cardiomyopathy: a real-world survey in Italy and Spain

Abstract Background Research describing non-obstructive hypertrophic cardiomyopathy (nHCM) and the associated burden of illness is sparse. Purpose This research aimed to describe the clinical characteristics of patients with nHCM and the associated clinical and societal burden of illness in Italy (IT) and Spain (ES). Methods Data were from the Adelphi HCM Disease Specific Programme (DSP), a cross-sectional survey with retrospective data collection in IT and ES (July 2022–January 2023). Ethics approval was obtained from the Pearl Institutional Review Board. Cardiologists provided data on patient demographics, employment, clinical characteristics, symptoms, support needs and treatment for up to ten consecutively consulting adult HCM patients. Patients voluntarily completed a survey containing the Kansas City Cardiomyopathy Questionnaire-23 (KCCQ). Analyses were descriptive and the mean [SD] was summarized for continuous variables. Results Overall, 112 cardiologists provided data for 483 patients with nHCM from IT (243) and ES (240). The mean age [SD] was 54.3 [14.1] and 58.0 [16.0], with 66.3% and 61.7% of patients being male, in IT and ES respectively. Eighty-four percent and 92.1% of patients were classified as New York Heart Association (NYHA) II, 14.8% and 7.1% were NYHA III, and 1.2% and 0.8% were NYHA IV, in IT and ES respectively. Among patients who completed the KCCQ, the mean [SD] KCCQ-clinical summary score was 84.9 [12.9] in IT and 81.8 [20.7] in ES. Regarding work-related impact, 30.8% (IT) and 16.7% (ES) of patients had taken time off work due to nHCM in the last three months, and 41.6% (IT) and 48.4% (ES) were not working full-time, of which 5.6% (IT) and 11.0% (ES) were not working full time due to nHCM. Additionally, 17.6% and 18.2% of patients experienced a delayed diagnosis in IT and ES respectively, with 24.0% (IT) and 25.0% (ES) having multiple delays to diagnosis. The most common symptoms reported by physicians were dyspnoea when active (83.7%), palpitations (29.3%) and fatigue (25.7%). The most prevalent concomitant conditions were hypertension (20.5%), anxiety (18.6%), and atrial fibrillation (18.4%). In IT and ES, respectively, 77.8% and 74.2% of patients were currently prescribed treatment for nHCM. The most frequently prescribed treatments were beta-blockers (83.3%), diuretics (32.1%) and angiotensin converting enzyme inhibitors (20.5%), alone or in combination. In IT and ES respectively, 14.4% and 18.8% of patients required caregiver support, at a mean [SD] of 46.3 [52.4] and 44.2 [47.5] hours per week. Conclusions While the majority of patients with nHCM were classified as NYHA II, a considerable clinical and societal burden is evident. Burden was demonstrated by the high prevalence of symptoms, comorbidities, disruptions to employment and the requirement for caregiver support among these patients, highlighting the need for new treatment and management approaches to enhance health outcomes and reduce societal impact.

Discordance between symptomatic response and changes in cardiac structure and function one year after transcatheter aortic valve implantation

Abstract Background Symptomatic improvement is one of the main goals of transcatheter aortic valve implantation (TAVI) in patients with severe symptomatic aortic stenosis. We hypothesized that the degree of symptomatic response after TAVI is associated with improvement in cardiac structure and function after TAVI. Purpose We aimed to evaluate 1) the change in symptomatic burden measured by New York Heart Association (NYHA) and Canadian Cardiovascular Society (CCS) functional class after TAVI, 2) the change in echocardiographic structural and functional parameters after TAVI, and 3) the association of symptomatic response with echocardiographic response as well as long-term survival. Methods In patients undergoing TAVI, we assessed New York Heart Association (NYHA) and Canadian Cardiovascular Society (CCS) functional class at 12 months. Symptomatic response was defined as NYHA/CCS class I or ≥ 1 improvement in NYHA/CCS class compared to baseline at 12 months. Echocardiographic images at baseline and 6-18 months post-TAVI were analysed using an automated, validated, deep-learning workflow. Logistic regression was used to assess association between change in echocardiographic parameters and symptomatic change at 12 months. The association of symptomatic response and echocardiographic changes in the first 12 months after TAVI with survival beyond 12 months, was evaluated by landmark analysis using Kaplan-Meier analysis. Results Among 868 TAVI patients (median age 80 years, 53% female), the majority (74%) had NYHA/CCS class III at baseline and 77% of patients experienced improvement in symptoms at 12 months post-TAVI (Figure 1). Patients without an improvement in symptoms more often had COPD, atrial fibrillation and more heart failure medications compared to responders. Left ventricular systolic function did not show improvement compared to baseline, whereas a modest improvement in left atrial volume index, left ventricular mass index, E/A ratio, E/e’ ratio, mean e’ and tricuspid regurgitation peak jet velocity was observed. Symptomatic response was not associated with echocardiographic response. Beyond 12 months, patients with symptomatic improvement had lower mortality than those without symptomatic improvement (1.5 vs. 2.4 per 10 patient years; p = .001), whereas changes in echocardiographic parameters were not associated with subsequent mortality (Figure 2). Conclusion Patients undergoing TAVI for symptomatic severe aortic stenosis, showed substantial symptomatic improvement at one year after the procedure, whereas only a modest improvement in echocardiographic parameters was observed. Improvement in symptoms was not associated with improvement in echocardiographic measures of cardiac structure and function. Symptomatic improvement, but not improvement in cardiac structure and function at 12 months, was a strong predictor of subsequent survival.Change in NYHA/CCS class after TAVISymptoms and echo versus mortality

Clinical experience of transapical off-pump mitral valve repair with NeoChord implantation

Abstract Introduction Transapical off-pump mitral valve repair with NeoChord implantation is a novel minimally invasive surgical procedure to treat degenerative mitral valve regurgitation (MR). has gained widespread application for patients presenting with severe mitral regurgitation caused by leaflet prolapse or flail. However, there is a scarcity of data concerning the clinical experience with this device. Purpose This prospective study aims to assess 1-year clinical results of the Neochord Procedure in a consecutive cohort of patients. Methods Between March 2018 and December 2023, 38 patients with severe symptomatic degenerative MR were enrolled in the study. All patients presented with severe MR due to flail of 1 or both leaflets and they all completed postoperative echocardiographic assessment up to 1 year. We identified the primary endpoint as composed of procedural success , freedom from mortality, reintervention, recurrence of severe MR , and decrease of at least 1 New York Heart Association (NYHA) functional class at 1-year follow- up. Results Median age was 59 years (21% female). Acute procedure success (defined as successful placement of at least 3 neochords with reduction of residual MR to less than 2+) was achieved in all patients. The intraoperative and in-hospital mortality rate was 0%. At 1-year overall survival was 97% with the one reported death attributed to other causes. Following the procedure, almost all patients (94%) reported a decrease in NYHA functional class at 1-year follow-up along with a reduction in diuretic usage (18% of patients presented with NYHA Class I, while 76% of them presented with NYHA Class II). Moreover, 18% of patients exhibited mild MR, while 63% presented with moderate MR, and 10% had no detectable mitral leakage. Conclusion Transapical off-pump mitral valve repair with NeoChord implantation seem to be a safe procedure for patients. However, further investigations with a larger sample size and longer follow-up periods are warranted.

Conduction system pacing and atrioventricular node ablation for atrial fibrillation - a systematic review and meta-analysis

Abstract Background/Introduction Biventricular pacing (BVP) has been shown to reduce the risk of heart failure following atrioventricular node ablation (AVNA) for the management of medically refractory atrial fibrillation (AF) when compared with conventional right ventricular pacing.[1] Conduction system pacing (CSP) is an emerging cardiac synchronization alternative to BVP[2], however, its evidence in AVNA for AF is limited. Purpose To evaluate the effect of CSP on left ventricular ejection fraction (LVEF), QRS duration (QRSd), pacing threshold and New York Heart Association (NYHA) class score following AVNA for AF. Methods We conducted a literature review of PUBMED, MEDLINE, EMBASE, Cochrane Library, and Web of Science for randomized clinical trials (RCTs) and observational studies evaluating CSP (His bundle pacing and left bundle branch pacing) post AVNA for AF. Out of 14 studies comprising 1112 patients, we conducted meta-analyses evaluating the impact of CSP on LVEF and QRS. Ten studies were pooled to assess outcomes pre- and post-implantation of CSP following AVNA. Three observational studies and one RCT were included in the meta-analysis comparing outcomes in CSP versus BVP post AVNA. Results CSP resulted in a significant QRSd reduction (-11.01 ms, 95% CI 5.07 - 16.95, p &amp;lt;0.01), improved LVEF (+10.28%, 95% CI 7.03 - 13.52; p &amp;lt;0.01) and improved NYHA score (-1.09; 95% CI -1.41 - -0.78; p &amp;lt;0.01) during follow-up of 6-36 months (Figure 1) from baseline (peri-implantation). There was no significant pacing threshold rise (0.11V; 95% CI -0.14 - 0.36; p= 0.39) observed during the same period. CSP was associated with statistically significant shorter QRSd (-49.81ms; 95% CI, -70.57 - -29.06; p &amp;lt;0.01) when compared to BVP. For LVEF, there was a statistically significant improvement of +8.96% (95% CI 2.55 - 15.37%; p &amp;lt;0.01) with CSP versus BVP. Limited data prohibited comparison of threshold and NYHA outcomes between CSP and BVP. Conclusion Conduction system pacing appears effective in improving baseline LVEF, QRSd and NYHA score in AVNA for AF, and may be a viable alternative to further optimising LVEF and QRSd in comparison to BVP. Further studies are required to evaluate the long-term impact and clinical outcomes of CSP in post AVNA patients.

Angina pectoris after coronary angiography or revascularization in pre- and postmenopausal women with coronary artery disease

Abstract Background Menopause is a known risk factor of coronary artery disease (CAD) in women. Earlier studies indicate that the incidence of angina pectoris (AP) increases with age. Some studies suggest that menopause would be a risk factor for AP in women with history of myocardial infarction (MI). However, the evidence is still incomplete. Purpose The aim of this study was to determine whether pre- or postmenopausal women are more symptomatic after revascularization using Canadian Cardiovascular Society (CCS) grading and New York Heart Association (NYHA) classification. We also wanted to evaluate whether the presence of type two diabetes (T2D) had an impact of the volume of the symptoms of CAD. Methods ARTEMIS Study contains 1,947 subjects with angiographically documented CAD with or without T2D (women 31.8%). Nondiabetic patients were matched with T2D patients for age, sex, prior MI and revascularization. Information of the menopause was from 610 women. Results Total of 584 women were at postmenopausal stage and only 26 were at premenopausal stage. The mean age of the premenopausal women was 57.5±10.8 years (P&amp;lt;0.001) and 69.4±7.5 years in postmenopausal women (P&amp;lt;0.001). Among premenopausal women 46.2% were active or ex-smokers and 26.8% of postmenopausal women still smoked or had smoked in the past (P=0.031). There was not statistically significant difference in other baseline characteristics: BMI, LDL or BNP levels, systolic or diastolic blood pressure, syntax score prior or after the revascularization and prevalence of prior MI. Over half of the postmenopausal women (57.4%, p&amp;lt;0.001) reported to suffer chest pain CCS grade ≥ 2 after the revascularization whereas only one fifth of the premenopausal women (23.1%, P&amp;lt;0.001) reported the same level of AP. T2D seemed to increase the symptoms of CAD as 33.3% of the premenopausal and 61.7% of the postmenopausal women with T2D reported AP with severity of CCS grade ≥ 2 (P=0.050). CCS 2 symptoms were reported by 45.7% of the postmenopausal and 19.2% of the premenopausal women, and CCS 3 symptoms by 11.6% of the postmenopausal women, yet only 3.8% of the premenopausal women (p=0.001 for both, respectively). Three quarter of the premenopausal women reported CCS 1 level of symptoms but only 42.6% of the postmenopausal women (P=0.003). Prevalence of each of CCS grade reported within pre- and postmenopausal women with and without T2D are shown in the Table 1. None of the premenopausal women reported NYHA ≥ 2 dyspnea, and the same level of shortness of breath was reported also only from 4.3 % of the postmenopausal women (p=0.005). Among postmenopausal women with T2D, NYHA ≥ 2 symptoms were more common, though statistical significance was not obtained (9.2%, P=0.196). Conclusion Postmenopausal women seem to experience more CAD-related symptoms after angiography or revascularization compared premenopausal women. Presence of T2D seems to associate with higher CCS grade after revascularization.

MELD score as predictor of mortality in patients with advanced heart failure: a longitudinal evaluation

Abstract Aims Advanced heart failure (AHF) is characterized by recurrent episodes of hemodynamic instability, frequent hospitalizations leading to a progressive decline in quality of life, and high mortality rates. The objective of this study was to assess the clinical associations of the MELD score and its efficacy in predicting mortality among patients with AHF. Methods 102 patients with AHF were enrolled, all patients included in the study were on top of tolerated medical therapy according to guidelines. The MELD score was measured at baseline, and every 6 months during follow-up. All patients underwent echocardiographic assessment, six-minute walking test, and cardiopulmonary testing with evaluation of VO2 max and VE/VCO2. Results The mean age of the study group was 57.8±11.9 years. There were 26 deaths during a follow-up period of 31.9±15.4 months. The mean NYHA class was 2.8±0.6, ejection fraction was 26.5±6.3%, TAPSE was 17.5±4.1 mm, and NT-pro-BNP was 2746.0±2475.9 pg/mL. The mean VO2 max was 11.7±3.5 ml/kg/min, and the distance covered in the six-minute walk test was 270.1±151.5 meters. Data stratified by mortality showed that deceased patients had higher NYHA class (2.7±0.6 vs. 3.2±0.3; p&amp;lt;0.001), NT-pro-BNP levels (4767.2±2888.1 vs. 1884.0±1669.5 pg/mL; p&amp;lt;0.001), and MELD score (19.3±3.8 vs. 11.9±5.1; p&amp;lt;0.001) compared to those who were alive at follow-up. Multiple regression analysis revealed a positive correlation between MELD score and NT-pro-BNP (Beta = 0.300; p=0.010), and a negative correlation between MELD score and TAPSE (Beta = -0.236; p=0.026). Cox regression analysis identified MELD score as a predictor of mortality (HR = 1.113; 95%CI = 1.011-1.225; p=0.029), independently by the effect of age, NYHA class, NT-pro-BNP, ejection fraction, TAPSE, and VO2 max. Changes in MELD score percentage, considered as a dichotomous variable (≤100% and &amp;gt;100%), were found to be predictors of mortality. Receiver operating characteristic (ROC) curves showed an area under the curve (AUC) of 0.897 for MELD score and mortality. Conclusions The MELD score and its longitudinal changes are effective predictors of mortality in patients with AHF, regardless of age, NYHA class, and NT-pro-BNP.

Symptoms trend in patients with obstructive hypertrophic cardiomyopathy treated with disopyramide

Abstract Background In obstructive hypertrophic cardiomyopathy (HOCM) medical intervention becomes necessary when symptoms coexist, whereas invasive septal reduction therapy (SRT) is indicated in presence of NYHA class &amp;gt;II, although optimized medical therapy. Thus, if SRT is avoided by medical therapy patient should be considered responder. However, due to inconsistency on definition of responders, effectiveness of medical therapy among data are conflicting. In addition, only short follow-up (FU) is available for analyses. Purpose To address this, we conducted a retrospective analysis in two HCM centers, both defining responders based on guidelines recommendations. Methods 62 symptomatic HOCM patients (43% women, age 52±14 years) with LVOTG ≥50 mmHg at rest or during provocation, were recruited from two HCM Italian centers. Disopyramide was added as second-line therapy. Responders were those reaching at least NYHA class II, if &amp;gt;II at baseline and those reaching NYHA class I or with stabilization of symptoms if in NYHA II class at baseline. Results At FU, (mean 4.4 years, IQR 1.1-6.6 years), 47 patients (76%) were responders, whereas 15(24%) patients were no-responders. Changes in NYHA class from baseline to follow-up are reported in the Figure. There were no differences at baseline of clinical and echocardiographic data between responders and no-responders, whereas at FU responders showed a significant lower LVOTG than no-responders (43 ±32 vs 66 ±28 mmHg, respectively, p=0.013), with LVOTG &amp;lt;50 mmHg more represented in responders than in no-responders (75% vs 25%, respectively; p=0.004) (Figure). No side effects indicating discontinuation of the therapy were recorded. Conclusion Patients treated with disopyramide as second-line therapy showed a reduction in symptoms in a quite long-follow-up, avoiding SRT in up to 70% of them. Patients who might benefit the most from the therapy are those who reached LVOTG &amp;lt;50 mmHg during treatment.

Frailty and MECKI score in predicting mortality in advanced heart failure

Abstract Background Physical frailty (PF), characterized by decreased physiological reserves and increased vulnerability to stressors, has emerged as a significant predictor of adverse outcomes in heart failure (HF). The MECKI (Metabolic Exercise Cardiac Kidney Index) score, a composite index derived from cardiopulmonary exercise testing parameters and renal function, has shown promise in predicting outcomes in HF patients. Purpose The aim of the study is to evaluate the independent prognostic role of the MECKI score and PF on mortality in patients with advanced heart failure (AHF). Methods Ninety-one patients with AHF were enrolled, including 42 on the heart transplant waiting list, 10 undergoing evaluation for listing, and 49 removed from the list due to transplant contraindications or reaching age limits. All patients included in the study were on medically optimized therapy. MECKI score assessment was conducted upon enrollment, along with hematological and biochemical evaluations including creatinine, hemoglobin, Na+, and NT-pro-BNP levels. Patients also underwent echocardiography assessing ejection fraction (EF), TAPSE, Inferior Vena Cava (IVC) diameters, and PASP. Additionally, all patients performed the 6-minute walk test (6MWT) and cardiopulmonary exercise testing with assessment of VO2max and VE/VCO2. Frailty was assessed based on modified Fried criteria. Results The mean age was 56.5±11.2 years. Mean NYHA class was 2.7±0.6, EF was 28.8±7.6, TAPSE was 17.5±4.2, and NT-proBNP was 2912.2±3249.0. Mean VO2max was 12.0±3.5 ml/kg/min, and mean distance walked during 6MWT was 278.8±137.2 meters. Mean frailty score was 3.2±1.2 while mean MECKI score was 15.2±14,2. During a mean follow-up of 32.0±15.3 months, there were 21 deaths, 15 patients received heart transplants, and 1 patient underwent LVAD implantation. Stratified data for mortality showed statistically significant differences in deceased patients compared to survivors regarding NYHA class (3.2±0.3 vs 2.6±0.7; p&amp;lt;0.001), Frailty Index (4.4±0.7 vs 2.7±1.13; p&amp;lt;0.001), NT-proBNP (5425.0±2863.0 vs 1731.0±1618.0; p&amp;lt;0.001), VE/VCO2 (42.7±12.3 vs 35.0±9.1; p&amp;lt;0.003), and MECKI score (29.4±15.2 vs 11.2±11.2; p&amp;lt;0.001). Linear regression analysis demonstrated a significant positive correlation for Frailty Index and MECKI score with age, NYHA class, VE/VCO2, IVC, and NT-proBNP, while showing a negative correlation with TAPSE, VO2max, and 6-minute walk test. Cox regression analysis on mortality demonstrated the predictive role of Frailty (HR=2.121; 95%CI=1.017-4.421; p=0.045) and MECKI score (HR=1.043; 95%CI=1.009-1.079; p=0.014) independently from age, sex, NYHA class, NT-proBNP, EF, TAPSE, VO2max, 6MWT, and IVC diameter. ROC curves demonstrated an AUC of 0.896 for MECKI score and mortality, and 0.884 for Frailty Index and mortality. Conclusions The MECKI score and physical frailty are both valid independent predictors of mortality in patients with advanced heart failure.

Effect of Fontan circulation on body composition and correlation with exercise capacity

Abstract Background There is growing attention to body composition in Fontan patients (FP) and preliminary results suggest that FP have an increased fat mass and a decreased muscle mass compared to the healthy population. FP also have reduced exercise capacity compared to health peers. However, data are still limited. Purpose The aim of our study was to compare the body composition (BC) of adult patients with Fontan circulation and healthy controls and to assess the correlation with functional capacity. Material and Methods Case control study on adult patients with Fontan circulation (FP) and age and gender matched healthy controls (HC). The following characteristics were recorded: age, gender, morphology of the systemic ventricle, type of Fontan circulation, level of physical activity (PAR, 0-7 points, 0 = sedentary - 7 = running &amp;gt; 3hours/week). S-score muscle, T-score bone, fat mass (FM%), intra-muscular adipose tissue (IMAT), abdominal adipose tissue (AAT), intracellular water (ICW), extracellular water (ECW), total body water (TBW), were measured through multi-frequency bioelectrical impedance technology in the FP and in the HC. Correlation was made for FP between body composition and oxygen consumption at peak exercise (VO2 peak), VE/VCO2 slope and NYHA class as indicator of functional capacity. Results Thirty Fontan patients (FP, mean age 24±6 yrs, 70% males) and 30 matched healthy controls (HC) were recruited in the study. The systemic ventricle was dominant left in 14 patients (47%), dominant right in 16 (53%). Four patients had atrio-pulmonary connection, the remaining were palliated with a lateral tunnel (n=26, 87%). The analysed parameters are summarised in Table 1. PAR was reduced in FP compared to HC (median 4.0 points in FP versus 7.0 points in HC, p 0.024). There was no difference in the BMI, fat mass, and body water between FP and HC. However, FP showed significantly lower S and T scores compared to the HC (S-score: -0,75 in FP versus 0,0 in HC, p 0.003. T-score: -0.9 in FP versus -0.25 in HC, p 0.001). In FP, NYHA Class was I in 28 patients (93%), II in 1 (3,5%) and III in 1 (3.5%) patient. The mean VO2 was 23.4±5.4mlO2/kg/min and mean VE/VCO2 slope 32±7. There was a direct correlation between VO2 peak and the PAR (rho 0.443, p 0.044). No correlation was found between the VO2 peak, the VE/VCO2 slope or the NYHA Class and the analysed parameters of body composition. Conclusion FP have reduced muscle mass, and bone density compared to HC. In addition to the known reduced efficiency of the cardiac pump during exercise in FP, the lower level of conditioning contributes to the reduced exercise capacity in this population. Programs aiming to encourage physical activity are key and should be part of patients’ care.

Evaluation of clinical need, effectiveness, and safety of the first myosin inhibitor mavacamten in real world patients

Abstract Background Hypertrophic cardiomyopathy has a high burden of heart failure if obstruction in the left-ventricular outflow tract (HOCM) is present. Mavacamten is the first available myosin inhibitor specifically addressing the underlying pathophysiology of hypercontractility in HOCM. Based on positive results of respective approval studies, mavacamten is recommended for therapy of HOCM in recent ESC guidelines but clinical real world data are lacking. Methods In this ongoing study, consecutive patients with current or previous diagnosis of HOCM presenting at a single referral centre for hypertrophic cardiomyopathy from March 2023 to February 2024 were included and analysed by treatment modality. In patients initiating mavacamten therapy, clinical data of 4 weekly follow ups were assessed, including efficacy (NYHA class, LVOT gradient and biomarkers) and adverse events. Results A total of 72 patients were included (59.1 ± 13.0 years, 68% male). Mavacamten treatment was started in 30/72 (41.7%) patients, 3/72 (4.1%) were referred for transcatheter septal reduction therapy (SRT), and 39/72 (54.2%) did not require any escalation in their treatment due to low gradient with current treatment or few symptoms. Of the latter, 3/39 (7.7%) had previous SRT, 36/39 (92.3%) were stable on negative inotropic medication. The average age of patients selected for mavacamten therapy was 58.0 ± 13.0 years, 80% were male, and the average thickness of the interventricular septum was 18.8 ± 2.8 mm. 22/30 (73.3%) were on baseline therapy with beta-blockers, 3/30 (10.0%) with calcium channel blockers, 2/30 (6.7%) with both, and 4/30 (13.3%) had additional treatment with disopyramide. As shown in table 1, there was a significant decrease in the LVOT gradient under Valsalva maneuver observed 4 weeks after initiation of mavacamten treatment (p=0.006), while the reduction in peak exercise LVOT gradient was statistically significant after 8 weeks (p=0.02). Simultaneously, a significant decrease in Nt-pro-BNP levels was observed after 4 weeks of therapy (p&amp;lt;0.001). A significant drop in troponin T levels compared to baseline was only noted after 20 weeks of treatment (p=0.02). At baseline, 66.7% patients were classified as NYHA class III/IV, and 12.5% after 20 weeks of treatment. 75% of the patients improved ≥1 NYHA class after 20 weeks. Regarding adverse events, there was one case of ejection fraction dropping to &amp;lt;50%, which resolved after halting treatment. Additionally, there were two reports of dizziness and one reported syncope, all of which resolved without taking any actions. Conclusion In this real-world single centre HOCM cohort, 41.7% of patients had an indication for myosin inhibitor therapy. The safety and effectiveness in lowering the LVOT gradient and improving NYHA class were consistent with findings from the approval studies.

Effect of alcohol septal ablation on exercise hemodynamics, symptoms and quality of life in patients with obstructive hypertrophic cardiomyopathy

Abstract Background The comprehensive impact of septal reduction therapy in patients with obstructive hypertrophic cardiomyopathy (HCM), including exercise hemodynamics and quality of life (QoL), remains poorly understood. Purpose The aim of this study was to evaluate the effect of alcohol septal ablation (ASA) in mitigating left ventricular outflow tract (LVOT) obstruction, reducing filling pressures during exercise, and the effect on QoL in patients with obstructive HCM. Methods This prospective study enrolled 24 patients with obstructive HCM referred for ASA. Evaluations included right-heart catheterization and echocardiography performed at rest and during exercise, before and approximately six months post-ASA. The primary outcome was the difference in filling pressures assessed by pulmonary capillary wedge pressure (PCWP) at 75 Watt (W). Secondary outcome assessed LVOT gradients, New York Heart Association (NYHA) functional class, Kansas City Cardiomyopathy Questionnaire Overall Summary Score (KCCQ-OSS), and exercise capacity. Results ASA significantly reduced PCWP at 75 W (24 ± 10 mmHg vs 33 ± 9 mmHg; P = 0.0001) (Figure 1), decreased LVOT gradients at rest (49 mmHg (24-90) vs 17 mmHg (14-27); P=0.002), at peak exercise (45 mmHg (29-90) vs 32 mmHg (21-61); P = 0.02), and post-exercise (96 mmHg (34-161) vs 28 mmHg (21-62); P=0.004). The KCCQ-OSS score was also significantly reduced (75.6 ± 18.8 vs 64.7 ± 16; P = 0.006), without affecting peak oxygen consumption (19.9 ± 4.8 ml/kg/min vs 18.7 ± 3.6 ml/kg/min; P = 0.26). The improvement in the KCCQ score was confirmed by changes in NYHA functional class. Before ASA treatment, 54% of patients were in NYHA functional class III compared to 13% after ASA (P &amp;lt; 0.0005). Conclusion ASA substantially reduces exercise-induced filling pressures, reduces LVOT obstruction, and improves symptoms and QoL in obstructive HCM, without impacting maximal exercise capacity.

HFpEF is associated with a higher risk of hospitalization or death in men undergoing transcatheter edge-to-edge tricuspid valve repair

Abstract Aims There are fundamental differences between women and men regarding the pathophysiology and etiology of tricuspid regurgitation (TR) as well as underlying comorbidities. The aim of this study was to analyze differences between female and male patients undergoing transcatheter edge-to-edge tricuspid valve repair for TR (T-TEER) and to identify gender-specific predictors of adverse outcome. Methods and Results In this monocentric study 197 consecutive patients (96 women and 101 men) undergoing T-TEER at our university heart center were included. Prevalence of heart failure with preserved ejection (HFpEF) was higher in women than in men (63% vs. 37.3%; p=0.001) whereas men more often had heart failure with reduced ejection fraction (HFrEF; 62.7% vs. 37%; p=0.001). Women had more severe symptoms (86.2% in New York Heart Association (NYHA) class III-IV vs. 72.3% in men; p=0.022). Device success rates (p=0.64), procedural characteristics (e.g. number of clips, p=0.17) and postprocedural results (TR grade post clip, p=0.1) were comparable. One-year mortality did not differ between genders (5.9% in women, 8.6% in men; p=0.53). Likewise, no significant difference could be found with regard to heart failure hospitalizations within one year after T-TEER (20.3% in women, 18.8% in men, p=0.82). Strikingly, prevalence of HFpEF was substantially higher than of HFrEF in men that reached the combined endpoint of one-year mortality or rehospitalization for heart failure (61.5% vs. 38.5%; p = 0.08). In women, no relevant difference could be found (27.0% vs. 32.4%; p=0,623). To identify gender-specific predictors of the combined endpoint, a cox regression analysis was performed. In men, HFpEF emerged as an independent predictor (HR 2.703; 95% CI 1.069 – 6.837; p = 0.036). In women, NYHA functional class proved to be an independent risk factor (Hazard Ratio (HR) 3.362; 95% confidence interval (CI) 1.085 – 10.415; p=0.036) for the combined endpoint whereas HFpEF was not associated with adverse outcome (HR 0.865, 95% CI 0.259 – 2.885, p=0.81). Conclusions Women undergoing T-TEER were more symptomatic and had higher prevalence of HFpEF compared to men. Nevertheless, HFpEF emerged as an independent predictor of the combined endpoint of one-year mortality or rehospitalization for heart failure only in men. NYHA functional class independently predicted adverse outcome in women.

Tafamidis for the treatment of transthyretin cardiac amyloidosis (ATTR-CM) - Real-world data from a tertiary center

Abstract Introduction Tafamidis 61mg was shown to be the first treatment to reduce cardiovascular hospitalizations and to improve survival in patients with Transthyretin Amyloid Cardiomyopathy (ATTR-CM). Real-world data on the efficacy of transthyretin (TTR) stabilizers in patients with Heart Failure (HF) due to ATTR-CM is scarce. Purpose We aimed to assess the use of tafamidis in our center, that features a dedicated Cardiomyopathy Clinic. Methods We conducted an all-comers single-centre prospective study of consecutive patients with symptomatic HF due to ATTR-CM followed up to November 2023. As per site protocol, the diagnosis was established according to the ESC algorithm and all patients were assessed at least twice yearly. Disease management plans include HF treatment tailored to ATTR-CM (CHAD-STOP) and treatment with disease-modifying tafamidis 61mg. The latter is considered in patients who meet the local protocol criteria, including key inclusion criteria - NYHA class I-II - and none of the exclusion criteria - e.g. estimated glomerular filtration rate (GFR) &amp;lt;25mL/min. The primary endpoint was time to cardiovascular hospitalization or all-cause death. Results A total 226 ATTR-CM patients were identified, of whom 181 had a follow-up of at least 6 months and were included in the analysis (mean age 84 ± 7 years; 81% males, 16% hATTR-CM). Overall, there were 84 patients treated with tafamidis. Compared to non-treated patients, those receiving tafamidis were younger (mean age 83 vs. 86 years, p=0.007), more often with a lower NYHA functional class (e.g., NYHA I-II: 92 vs. 62%; p&amp;lt;0.001), scored higher on performance scales (e.g., Karnovsky &amp;gt;70: 67 vs 33%; p&amp;lt;0.001), and had less comorbidities (e.g., GFR: 40 vs. 30mL/min; p&amp;lt;0.001). Over a median follow-up of 16 months, 68 (37.6%) patients met the primary endpoint (49.5% vs. 22.6%, respectively; p&amp;lt;0.001). In multivariate analysis, treatment with tafamidis was an independent predictor of the primary endpoint (HR 0.24; 95%CI 0.13-0.43; p&amp;lt;0.001), adjusted for NYHA functional class (HR for NYHA I-II vs. III-IV: 0.54; 95%CI 0.32-0.92; p=0.023), NT-proBNP (HR 1.83; 95%CI 1.09-3.08; p=0.023) and high-sensitivity Troponin T (HR per 10 units: 1.01; 95%CI 1.01-1.02; p=0.024). These findings were consistent in multivariate analysis with any combination of the prior variables with either creatinine, GFR, albumin, or TTR type (wild-type vs. mutated). These results were primarily driven due to association of tafamidis treatment with increased survival. Conclusion The use of tafamidis in a cohort of patients with symptomatic HF and ATTR-CM was shown to be independently associated with reduced cardiovascular hospitalization or all-cause death. The effect had a similar magnitude of effect to that of the ATTR-ACT trial, suggesting that our local protocol based on the key criteria from this trial may allow for appropriate patient selection.

Clinical presentation, risk factors and six-month outcomes in Ugandan women with peripartum cardiomyopathy: prospective case-control study

Abstract Background Peripartum Cardiomyopathy (PPCM) frequently affects women of African descent. There is scarcity of data pertaining to the clinical presentation and outcomes of PPCM patients in Uganda, particularly when employing modern diagnostic tools and adhering to guideline-directed medical therapy (GDMT). Purpose We aimed to delineate the clinical characteristics, echocardiographic findings, and 6-month outcomes among women in Uganda diagnosed with PPCM. Methods Prospective case-control design conducted from June 2022 to August 2023. We enrolled 80 PPCM cases and 80 matched controls. Inclusion criteria comprised: (i) peripartum state, (ii) signs and symptoms of HF, (iii) left ventricular (LV) ejection fraction ≤ 45%, and (iv) exclusion of alternative causes of HF. Controls were healthy women matched for age and parity. Over a 6-month period, enrolled PPCM cases were diligently monitored while on GDMT. All participants underwent a physical examination, 12-lead electrocardiography, and a detailed echocardiography incorporating global longitudinal strain measurements at both baseline and the 6-month follow-up. Results At baseline, amongst the cases, the mean age was 33.6 ± 6.6 years, mean parity was 3±2.1 pregnancies. HF onset occurred within 9.5±6.5 weeks post-delivery. A total of 20 (25.0%) cases presented in NYHA Class 4 with dyspnea as the predominant symptom in 79.95% of cases. Atrial fibrillation (AF) was observed in 2(2.5%) patients. In comparison with healthy controls, high gravidity (0.2±1.0; p&amp;lt;0.001) was identified as the only predictor of PPCM in this study. (Table 1) At 6-months follow up, the mean LV ejection fraction (LVEF) showed improvement from 35.7±-11.0% to 45.9 ± 15.7% (p&amp;lt;0.001) accompanied by a reduction in left ventricular diameter (LVD) from 6.1±0.7 cm to 5.5±1cm(p&amp;lt;0.001). At the 6-month follow-up, recovery (LVEF ≥ 50%) occurred in 37 cases (55%), and 52 (65%)patients were asymptomatic (NYHA Class 1) at the 6-month follow-up. Six-month mortality data were available for 5(6.3%) women, and one patient successfully underwent the insertion of an Implantable Cardioverter-defibrillator for recurrent Ventricular Tachycardia. Regarding GDMT, 55 participants received Bromocriptine as part of their medication. Twenty(25%) patients received anticoagulation for AF(n=2) or intracardiac thrombus (n=6, 1.67%) (Figure 1) resulting in 2(2.5%) strokes within the 6-month follow-up. Conclusion The current study demonstrated a comparable mortality rate on GDMT, aligning with contemporary global studies at 7.5%. The observed high thrombus burden in patients may indicate suboptimal adherence to anticoagulation therapy. While the incidence of PPCM remains undefined in Uganda, the primary predisposing factor identified was high gravidity. A noteworthy proportion of patients exhibited incomplete LV recovery, suggesting the need for larger studies to comprehensively define the maternal and fetal outcomes in Uganda.Figure 1Table 1

Real-world experience with mavacamten in obstructive hypertrophic cardiomyopathy: observations from a tertiary care center

Abstract Background In symptomatic obstructive hypertrophic cardiomyopathy (oHCM) patients, mavacamten is commercially approved to help improve left ventricular (LV) outflow tract (LVOT) gradients, symptoms, and reduce eligibility for septal reduction therapy (SRT) under the risk evaluation and mitigation strategy (REMS) program. We sought to prospectively report the initial real-world clinical experience with the use of commercially available mavacamten in a multi-hospital tertiary healthcare system. Methods We studied the first 150 consecutive oHCM patients (mean age 65 years, 53% women, 83% on betablockers and 61% in New York Heart Association [NYHA] class III) who were initiated on 5 mg of mavacamten with dose titrations using symptom assessment and echocardiographic measurements of LVOT gradient and LV ejection fraction (LVEF) measurements. We measured changes in NYHA class, LVEF, LVOT gradients (resting and Valsalva) at baseline, 4, 8 and 12 weeks. Results At 261±143 days (range of 31-571 days), 69 (46%) patients had ≥1 NYHA class, 27 (18%) additional patients had ≥2 NYHA class improvement and 40 (27%) reported a no change in symptoms. The mean LVOT gradient at rest decreased from 41±33 mmHg at baseline to 16±17 mmHg at 4 weeks, 16±16 mmHg at 8 weeks and 15±16 mmHg at 12 weeks. The mean drop in resting LVOT gradient from baseline to last follow-up was 26±3 mmHg (p&amp;lt;0.0001). The mean Valsalva LVOT gradient decreased from 72±43 mmHg at baseline to 29±31 mmHg at 4 weeks, 29±28 mmHg at 8 weeks and 30±29 mmHg at 12 weeks (p&amp;lt;0.001). The mean reduction in post Valsalva LVOT gradient from baseline to last follow-up was 43±4 mmHg (p&amp;lt;0.0001). At baseline, 100% patients had Valsalva LVOT gradients ≥ 30 mm Hg and 142 (95%) had ≥ 50 mm Hg. After initiation of mavacamten therapy, Valsalva LVOT gradient ≥ 30 mm Hg was observed in 44/150 (29%) at 4 weeks, 40/141 (28%) at 8 weeks and 41/136 (30%) at 12 weeks. In 40 patients who reported no symptomatic improvement, the mean Valsalva LVOT gradient decreased from 73±39 mmHg at baseline to 34± 27 mmHg at 4 weeks, 35±28 mmHg at 8 weeks and 30±24 mmHg at 12 weeks (P&amp;lt;0.001). The mean LVEF at baseline was 66±6% and changed to 64±5% at 4 weeks, 63±5% at 8 weeks and 62±7% at 12 weeks (p&amp;lt;0.0001). No patient underwent SRT, developed LVEF ≤30% or developed heart failure requiring admission. Three (2%) patients needed temporary interruption of mavacamten due to LVEF&amp;lt;50. During follow up, dose reduction or discontinuation of background HCM therapy (betablockers, calcium channel blockers and disopyramide) occurred in 33 (22%) patients. The breakdown of various drug doses of mavacamten, at last follow-up, is as follows: 2.5 mg (n=29, 19%), 5 mg (n=80, 53%), 10 mg (n=37, 25%) and 15 mg (n=3, 2%). Conclusions In a real-world study in symptomatic oHCM patients at a multi-hospital tertiary care referral center, we demonstrate the efficacy and safety of prescribing mavacamten under the REMS program.