The value of blood flow velocity and pressure gradient in differentiating patients with different types of heart failure.

Background: Patients with different types of heart failure (HF) exhibit varying rates of blood flow through cardiac chambers and pressure gradients across the aortic valve, attributed to differing degrees of myocardial contractility. Assessment of these dynamics offers insights into early HF diagnosis. This study aimed to analyze left ventricular outflow tract (LVOT) blood flow parameters, specifically peak blood flow velocity and pressure gradient derived from four-dimensional flow cardiovascular magnetic resonance (4D flow CMR), and to evaluate 4D flow CMR's utility in distinguishing HF types. Methods: This prospective cross-sectional study recruited 115 HF patients from January 2019 to May 2022 at the General Hospital of Ningxia Medical University, classified by the New York Heart Association Cardiac Function Classification of Heart Failure as class II-IV, alongside a control group (n=30). Participants underwent cardiovascular magnetic resonance (CMR), including 4D flow. HF patients were categorized into heart failure with reduced ejection fraction (HFrEF, n=55), heart failure with mildly reduced ejection fraction (HFmrEF, n=30), and heart failure with preserved ejection fraction (HFpEF, n=30), based on ejection fraction. The cardiac functional parameters and aortic valve flow indices were measured using Circle Cardiovascular Imaging. LVOT 4D flow data were obtained 3 mm below the junction of the aortic valve leaflets, assessing peak velocities above and below the valve. Differences in cardiac function and blood flow parameters between groups were analyzed using one-way analysis of variance (ANOVA). The accuracy of these parameters in identifying subgroups was assessed using the receiver operating characteristic (ROC) curve. Results: Analysis of conventional cardiac function parameters revealed that left ventricular ejection fraction (LVEF) was significantly lower in the HFrEF and HFmrEF groups compared to the HFpEF and control groups (P<0.01). Additionally, end-diastolic volume and end-systolic volume were significantly higher in the HFrEF and HFmrEF groups than in the HFpEF and control groups (P<0.01). However, there were no significant differences in cardiac function parameters between the HFpEF and control groups (P>0.05). Significant differences were observed in aortic valve peak pressure gradients (Supra-APGmax) among the four study groups (5.01±1.09 vs. 6.23±2.94 vs. 7.63±1.81 vs. 8.89±2.97 mmHg, P<0.05). Aortic valve peak velocities in the HFrEF group differed significantly from the HFpEF and control groups (111.31±12.05 cm/s vs. 137.2±16 vs. 147.15±24.55 cm/s, P<0.001). The ROC curve for the pressure gradient below the aortic valve had an area under the curve (AUC) of 0.728 [95% confidence interval (CI): 0.591-0.864, P=0.002], with an optimal threshold of 4.72 mmHg (sensitivity: 0.8, specificity: 0.7, Youden index: 0.5). Conclusions: HF patients exhibit reduced pressure gradients across the aortic valve during systole, indicative of altered intracardiac blood flow dynamics. Combining aortic valve velocities and pressure gradients can aid in distinguishing different types of HF, including HFpEF patients.

Impact of Aficamten on Echocardiographic Cardiac Structure and Function in Symptomatic Obstructive Hypertrophic Cardiomyopathy.

BACKGROUND: Aficamten, a next-in-class cardiac myosin inhibitor, improved peak oxygen uptake (pVO2) and lowered resting and Valsalva left ventricular outflow (LVOT) gradients in adults with symptomatic obstructive hypertrophic cardiomyopathy (oHCM) in SEQUOIA-HCM (Phase 3 Trial to Evaluate the Efficacy and Safety of Aficamten Compared to Placebo in Adults With Symptomatic oHCM), a phase 3, multicenter, randomized, double-blinded, placebo-controlled study. OBJECTIVES: The authors sought to evaluate the effect of aficamten on echocardiographic measures of cardiac structure and function in SEQUOIA-HCM. METHODS: Serial echocardiograms were performed over 28 weeks in patients randomized to receive placebo or aficamten in up to 4 individually titrated escalating doses (5-20 mg daily) over 24 weeks based on Valsalva LVOT gradients and left ventricular ejection fraction (LVEF). RESULTS: Among 282 patients (mean age 59 ± 13 years; 41% female, 79% White, 19% Asian), mean LVEF was 75% ± 6% with resting and Valsalva LVOT gradients of 55 ± 30 mm Hg and 83 ± 32 mm Hg, respectively. Over 24 weeks, aficamten significantly lowered resting and Valsalva LVOT gradients, and improved left atrial volume index, lateral and septal e' velocities, and lateral and septal E/e' (all P ≤ 0.001). LV end-systolic volume increased and wall thickness decreased (all P ≤ 0.003). Aficamten resulted in a mild reversible decrease in LVEF (-4.8% [95% CI: -6.4 to -3.3]; P < 0.001) and absolute LV global circumferential strain (-3.7% [95% CI: 1.8-5.6]; P < 0.0010), whereas LV global longitudinal strain was unchanged. Several measures, including LVEF, LVOT gradients, and E/e' returned to baseline following washout. Among those treated with aficamten, improved pVO2 and reduction in N-terminal pro-B-type natriuretic peptide (NT-proBNP) were associated with improvement in lateral e' velocity and septal and lateral E/e' (all P < 0.03), whereas improvement in Kansas City Cardiomyopathy Questionnaire Clinical Summary Scores (KCCQ-CSS) was associated with a decrease in both LVOT gradients (all P < 0.001). CONCLUSIONS: Compared with placebo, patients receiving aficamten demonstrated significant improvement in LVOT gradients and measures of LV diastolic function, and several of these measures were associated with improvements in pVO2, KCCQ-CSS, and NT-proBNP. A modest decrease in LVEF occurred yet remained within normal range. These findings suggest aficamten improved multiple structural and physiological parameters in oHCM without significant adverse changes in LV systolic function. (Phase 3 Trial to Evaluate the Efficacy and Safety of Aficamten Compared to Placebo in Adults With Symptomatic oHCM [SEQUOIA-HCM]; NCT05186818).

Assessing aortic flow with doppler echocardiography in cardiogenic shock: A crucial diagnostic tool.

PURPOSE: Cardiogenic shock still has a high mortality. In order to correctly manage these patients, it is useful to have available haemodynamic parameters, invasive and non-invasive. The aim of this review is to show the current evidence on the use of echocardiographic aortic flow assessment by left ventricular outflow tract - velocity time integral. METHODS: Publications relevant to the discussion of echocardiographic aortic flow assessment by left ventricular outflow tract - velocity time integral and cardiogenic shock, were retrieved from PubMed®. RESULTS: Left ventricular outflow tract - velocity time integral is an easily sampled and reproducible parameter that has already been shown to have prognostic value in various cardiovascular pathologies, including myocardial infarction and heart failure. Although there are still few data available in the literature, the LVOT-VTI also seems to have an important role in CS from prognosis to guidance in the escalation/de-escalation of vasoactive therapy and to support devices by allowing an estimate of patient's probability of response to fluid administration. CONCLUSION: Aortic flow assessment can become a very useful invasive parameter in the management of cardiogenic shock.

TAVR for Severe Aortic Valve Stenosis with LVOT Stenosis: A Case Report.

Transcatheter aortic valve replacement (TAVR) has been recently indicated for the treatment of patients with severe aortic stenosis in all risk profiles. At present, TAVR has become mature at home and abroad, but the relevant experience is deficient in the treatment of aortic valve stenosis with outflow tract stenosis. One case of a high-risk surgical patient was included in this paper who suffered from severe aortic valve stenosis with left ventricular outflow tract (LVOT) stenosis. In this case, TAVR was performed with deep implantation of a new valve and both aortic valve stenosis and LVOT stenosis were treated through a single TAVR procedure. This case highlights the vital role of such treatment in dealing with both aortic valve stenosis and LVOT stenosis through a single TAVR procedure, thus providing valuable information for similar cases.

An Interesting Case of Wolf-Parkinson-White Syndrome in a Young Patient With Sensorineural Deafness.

Wolff-Parkinson-White (WPW) syndrome is a condition associated with tachycardia due to accessory pathways in the heart, and it is one of the most common causes of tachycardia in infants and children. WPW may also be associated with mitochondrial encephalomyopathy, lactic acidosis, stroke-like episodes (MELAS syndrome) or LEOPARD syndrome (LS). We report a case of pre-excitation WPW syndrome in a 17-year-old man who was brought to the hospital by ambulance following the collapse. WPW syndrome type A was diagnosed from precordial leads. Electrocardiography (ECG) revealed a short PR interval, delta waves, and positive waves with dominant R in all pericardial leads. Blood test results showed an isolated elevated ALT level. Subsequent echocardiography was unremarkable, with an ejection fraction of 55%, apart from septal and inferior wall dyssynchrony. With regard to the past medical history, he had sensorineural deafness (SND) since childhood and had a family history of SND. Consequently, the patient was transferred to the cardiac electrophysiology department at another hospital after consultation and underwent ablation. A successful post-ablation electrocardiogram revealed the resolution of the WPW syndrome signs and post-ablation features, such as peak T waves.

Discontinuation of afterload-reducing drugs decreases left ventricular outflow tract obstruction in hypertrophic obstructive cardiomyopathy.

Background: Hypertrophic cardiomyopathy (HCM), the most common genetic heart disease, is classified into hypertrophic non-obstructive and hypertrophic obstructive cardiomyopathy (HOCM). Patients with HOCM and coexisting heart failure or arterial hypertension are often prescribed afterload-reducing drugs. Although recommended in current guidelines, data on the direct effect of discontinuing afterload-reducing medication are scarce. This study aims to demonstrate the benefit of discontinuing afterload-reducing medication in HOCM patients. Methods: This monocentric retrospective analysis included 24 patients with HOCM with afterload-reducing medication, including angiotensin-converting enzyme inhibitors, angiotensin-1 receptor blocker and dihydropyridine-calcium channel blocker, at their first outpatient visit. Effects of discontinuing this medication on LVOTO were examined compared to patients with persistent use despite medical advice. Results: 16 patients discontinued their afterload-reducing drugs, resulting in a significant decrease in median LVOT gradient from 86.5 [60.5-109.3] mmHg to 61.5 [28.3-97.50] mmHg (p = 0.0004). In 6 patients, beta-blocker therapy was initiated simultaneously, or the dose was increased. Regardless, LVOT gradient reduction was also significant in the remaining 10 patients (p = 0.001). The gradient was not changed significantly in the 8 patients continuing their afterload-reducing medication. Conclusions: Discontinuation of afterload-reducing drugs significantly decreases LVOTO. Our study underscores the significance of abstaining from afterload-reducing drugs in HOCM patients, particularly in patients with concomitant hypertension or heart failure. According to recently published European guidelines, HOCM patients should preferably be treated with beta-blockers or non-dihydropyridine-calcium channel blockers.

Eighteen-Month Real-World Experience Using Mavacamten for Treatment of Obstructive Hypertrophic Cardiomyopathy in a Racially Diverse Population.

BACKGROUND: Patients with obstructive hypertrophic cardiomyopathy have increased symptomatic burden. Mavacamten was recently approved for treatment of obstructive hypertrophic cardiomyopathy based on 2 randomized controlled trials. However, its use under real-world conditions and in diverse populations is under-studied. METHODS AND RESULTS: This was a prospective observational cohort study of patients seen at the Johns Hopkins HCM center and prescribed mavacamten for obstructive hypertrophic cardiomyopathy between July 7, 2022 and January 6, 2024. Patients were followed longitudinally, with serial echocardiography and clinical evaluation as mandated by the risk evaluation and mitigation strategy program. Sixty-six patients received mavacamten (mean age 59 years, 47% male, 29% non-White [Black, Hispanic/Latino, Asian, Native Hawaiian or Pacific Islander], 47% obese). Before treatment, all patients had New York Heart Association class II (51.5%) or III (48.5%) heart failure symptoms. Initial maximum peak left ventricular outflow tract gradient was 107±46 mm Hg. Median treatment duration was 9 months. For patients on mavacamten after ≥6 months (n=43), symptoms improved by ≥1 New York Heart Association class in 72% of patients, and peak left ventricular outflow tract gradient decreased by 80±46 mm Hg, eliminating hemodynamically significant left ventricular outflow tract obstruction in 79.1% of patients. Mavacamten was temporarily discontinued in 3 patients due to left ventricular ejection fraction decrease <50%. There were no medication-related adverse events. Effectiveness and safety were similar between White and non-White patients, but symptomatic relief was attenuated in patients with body-mass index ≥35 kg/m2. CONCLUSIONS: Mavacamten was effective and safe when used under real-world conditions in a racially diverse population of symptomatic patients with obstructive hypertrophic cardiomyopathy. Patients with comorbid obesity were less likely to experience symptomatic improvement while on mavacamten.

Changes in left ventricular-aortic angulation are associated with the development of obstruction in hypertrophic cardiomyopathy.

OBJECTIVE: To characterize changes in ventricular morphology in patients with hypertrophic cardiomyopathy who develop left ventricular (LV) outflow tract obstruction. METHODS: We reviewed patients with hypertrophic cardiomyopathy with LV outflow tract obstruction who underwent septal myectomy from May 2012 to June 2023. Among 68 patients initially without obstruction documented up to 7.6 years (interquartile range, 6.3-9.4 years) before the operation, a comparison was made with 78 patients with nonobstructive hypertrophic cardiomyopathy over a similar period. Patients who did not develop obstruction were matched with those who did on sex, age, and maximum septal wall thickness during the initial echocardiography, identifying 41 matched pairs. Echocardiographic data, including 5 measures of angulation, were compared between the groups. RESULTS: The median interval between echocardiographic assessments was 7.5 years (interquartile range, 6.3-8.1 years) among patients with obstruction versus 7.3 years (interquartile range, 6.2-9.0 years) in patients without nonobstruction. Patients with obstruction were more likely to have hypertension at both times. The maximum septal wall thickness increased within both groups (both P values < .001), but the magnitude of increase was not different between groups (P = .130). Patients with obstruction exhibited a greater increase in LV mass (P < .001) compared with patients without obstruction (P = .004). Aortic angulation significantly increased in 4 of the 5 measurements (all P values < .001) in patients with obstruction, whereas patients with no obstruction showed no change. Anterior and posterior mitral valve leaflet lengths and coaptation lengths remained similar in both groups over time. CONCLUSIONS: The development of LV outflow tract obstruction in patients with hypertrophic cardiomyopathy was associated with progressive LV outflow tract angulation and increased LV hypertrophy, as reflected by LV mass. Progression to obstruction was not related to changes in the mitral valve leaflet morphology.

Utilizing Alcohol Septal Ablation for Mitigating Left Ventricular Outflow Tract Obstruction in Cardiac Amyloidosis: A Case Report.

Alcohol septal ablation (ASA) has been widely used in relieving the left ventricular outflow tract (LVOT) obstruction caused by hypertrophic obstructive cardiomyopathy (HOCM). There is limited data about the utility of ASA in cases of cardiac amyloidosis with LVOT obstruction. Our patient is 71-year-old male with a history of multiple myeloma complicated by cardiac amyloidosis and end-stage renal disease on hemodialysis who presented from the dialysis center due to hypotension. The patient was admitted to our hospital for further workup. He underwent echocardiography that showed severely elevated LVOT gradient pressures and the decision was made to proceed with ASA, which led to significant improvement in the LVOT gradient pressures and the patient being able to tolerate his dialysis sessions.

Echocardiography-Derived Forward Left Ventricular Output Improves Risk Prediction in Systolic Heart Failure.

BACKGROUND: Although it is widely used to classify patients with heart failure (HF), the prognostic role of left ventricular ejection fraction (LVEF) is debated. The aim of this study was to test the hypothesis that echocardiographic measures of forward left ventricular (LV) output, being more representative of cardiac hemodynamics, might improve risk prediction in a large cohort of patients with HF with systolic dysfunction. METHODS: Consecutive stable patients with HF with LVEF <50% on guideline-recommended therapies undergoing echocardiography including the evaluation of forward LV output (i.e., LV outflow tract [LVOT] velocity-time integral [VTI], stroke volume index [SVi], and cardiac index) over a 6-year period were selected and followed for the end point of cardiac and all-cause death. RESULTS: Among the 1,509 patients analyzed (mean age, 71 ± 12 years; 75% men; mean LVEF, 35 ± 9%), 328 (22%) died during a median follow-up period of 28 months (interquartile range, 14-40 months), 165 (11%) of cardiac causes. On multivariable regression analysis, LVOT VTI (P < .001), SVi (P < .001), and cardiac index (P < .001), but not LVEF (P > .05), predicted cardiac and all-cause death. The optimal prognostic cutoffs for LVOT VTI, SVi, and cardiac index were 15 cm, 38 mL/m2, and 2 L/min/m2, respectively. Adding each of these measures to a multivariable risk model (including clinical, biohumoral, and echocardiographic markers) improved risk prediction (P < .001). Among the different measures of forward LV output, cardiac index was less accurate than LVOT VTI and SVi. CONCLUSIONS: The echocardiographic evaluation of forward LV output improves risk prediction in patients with HF across a wide LVEF spectrum over other well-established clinical, biohumoral, and echocardiographic prognostic markers.

New echocardiographic screening tool for left ventricular tract obstruction risk assessment in TMVR.

BACKGROUND: Transcatheter mitral valve replacement (TMVR) is an alternative to conventional surgery to treat severe mitral disease but its use is limited by the risk of left ventricular outflow tract obstruction (LVOTO). Screening depends on ECG-gated computed tomography (CT) that is not widely available and requires contrast. We developed and validated a transthoracic echocardiographic (TTE) method to assess the risk of LVOTO after TMVR with the Tendyne System. METHODS: We measured the LVOT longitudinal area on preoperative TTE dataset of patients screened for TMVR. The LVOT was measured as the box-area included by the aortic valve annulus, the anterior mitral leaflet (AML), the c-septum distance line, and the respective length of the AML on the interventricular septum. We analyzed the correlation between the TTE LVOT-box and the CT-measured neoLVOT area. Prediction performance for eligible patients was tested with ROC curves. RESULTS: Thirty-nine patients were screened, out of 14 patients (36%) not eligible for TMVR, 8 had risk of LVOTO. We found a linear correlation between the TTE LVOT-box and the CT-measured Neo-LVOT (r = 0.6, p = 0.002). ROC curve showed that the method is specific and sensitive and the cut-off value of the measure LVOT-box is 350 mm2. CONCLUSIONS: The proposed method is reliable to evaluate the risk of LVOTO after TMR with the Tendyne System. It is quick and easy and can be used as a first-line assessment in the outpatient clinic. Patients with LVOT-box <350 mm2 should not be further screened with ECG-gated cardiac CT.

Original Article--Mid-term Clinical and Hemodynamic Outcomes in Middle Age Patients Post Trifecta™ (Abbott) Aortic Valve Replacement: A Single-center Study.

Background: Trifecta™ is an externally wrapped bovine pericardial aortic valve with a favorable hemodynamic performance and acceptable rate of freedom from structural valve degeneration. However, recent reports of early Trifecta valve failure question its durability. Rheumatic valve disease in young population is predominant, there is no data on the Trifecta performance, durability or safety available worldwide over the middle age population, as well as there are no previous similar reports from Middle East region. We report on mid-term clinical and hemodynamic performance of Trifecta valve in middle age population with small aortic annuls, a single center report. Methods: A retrospective study of all patients who underwent Trifecta™ SAVR between June 2014 and December 2019 at a cardiac center in Riyadh, Saudi Arabia. Hemodynamic performance was analyzed by longitudinal Doppler echocardiography. Results: During a 5.5-year period, a total of 24 patients underwent SAVR with Trifecta™ valves at our heart center. Patients had a median age of 47.1 years (IQR = 37) and male:female ratio of 1:1. Patients had a median EuroScoreII of 2 (IQR = 1). The 19 mm valve was the most frequently used (29.2%) followed by the 21 mm and 23 mm (both 25.0%). Concomitant procedure was (54.2%). All patients survived their operations and most (87.0%) had their symptoms relieved. A 30-day mortality rate of 4.2%. Patients were followed up for a median of 58.5 months (IQR = 38), during which 8.3% required re interventions, giving an overall freedom from re-intervention of 91.7%. The re-intervention after valve implantation was recorded in two (8.3%). These re-interventions were performed 3 years post initial surgery and were due to structural valve deterioration (SVD). The mean gradient reduced significantly from 43.6 ± 23.9 mmHg (median = 41, IQR = 21) at baseline to 13.0 ± 7.4 mmHg (median = 11, IQR = 5) one-week post intervention (p < 0.001). The average mean gradients were stable from post-operation to follow-up (13.0 ± 7.4 vs 14.1 ± 6.4 mmHg). Conclusion: Our study confirms excellent mid-term durability, clinical and hemodynamic performance of the Trifecta™ valve in middle age population, despite the fact of non-statically significant trend-up of transvalvular gradient over the follow up period. Further long-term studies with larger sample-size are warranted to confirm these results.

Standardization and validation of neoLVOT assessment using three-dimensional trans-esophageal echocardiography before trans-catheter mitral valve replacement.

BACKGROUND: Trans-catheter mitral valve replacement (TMVR) procedures had emerged as an alternative solution for patients who are at high risk for mitral valve surgery. Although cardiac computed tomography (CT) remains the standard method for procedural planning, there is no full agreement on the best systolic phase for quantitation of the neoLVOT. Furthermore, a new three-dimensional trans-esophageal echocardiography (3DTEE) based software was developed to serve as filter and or an alternative for patients who cannot have CT due to any contraindication. AIM: To determine the systolic phase of the cardiac cycle that shows the narrowest NeoLVOT area in order to standardize the way of using these software and then to validate the 3DTEE-based software against the CT-based one as a gold standard, in mitral valve annulus (MA) and NeoLVOT assessment. METHODS: A single center, observational, retrospective study. Initially, a sample of 20 patients (age 62 ± 4 years, 70% men) had CT-based analysis at mid-diastole (80%), early-systole (10%), mid-systole (20%), late-systole (30%-40%), in order to detect the best systolic phase at which the neoLVOT area is the narrowest after TMVR. Then, the end systolic phase was standardized for the analysis of 49 patients (age 57 ± 6 years, 60% men), using both the commercially available CT-based software and the newly available 3DTEE-based software (3mensio Structural Heart, Pie Medical Imaging, The Netherlands). The 3DTEE derived parameters were compared with the gold standard CT-based measurements. RESULTS: The neoLVOT area was significantly narrower at end-systole (224 ± 62 mm2), compared to early-systole (299 ± 70 mm2) and mid-systole (261 ± 75 mm2), (p = .005). Excellent correlation was found between 3DTEE and CT measurements for MA AP diameter (r = .96), IC diameter (r = .92), MA area (r = .96), MA perimeter (r = .94) and NeoLVOT area (r = .96), (all p-values < .0001). Virtual valve sizing was based on annulus measurement and was identical between CT and 3DTEE. Interobserver and intraobserver agreements were excellent for all the measurements with ICCs > .80. CONCLUSIONS: End-systole is the phase that shows the narrowest neoLVOT and hence should be the standard phase used during the analysis. The 3DTEE based analysis using this new software is reliable compared to the CT-based analysis and can be serve as an alternative analysis tool in patients who cannot have CT for any clinical contraindication or as a screening test and/or filter for all patients before proceeding to a detailed CT scan.

Transseptal BATMAN for High-Risk Valve-in-Ring Procedures: A Case Series.

Valve-in-ring procedures represent a feasible solution for high-risk patients with surgical repair failure. The risk of left ventricular outflow tract obstruction increases the challenge, and transcatheter approaches to prevent it are technically demanding and often do not resolve it. We demonstrate the feasibility and safety of a transseptal balloon-assisted translocation of the anterior mitral leaflet for valve-in-ring implantation.

The left ventricular outflow tract and carotid artery velocity time integrals.

The left ventricular outflow tract velocity time integral (LVOT VTI) is commonly used in the intensive care unit as a measure of stroke volume (SV) and how the SV changes in response to an intervention; therefore, the LVOT VTI is used to guide intravenous fluid management. Various peripheral Doppler surrogates are proposed to infer the LVOT VTI (e.g., measures from the common carotid artery). A recently-described, novel method of insonation has an excellent ability to detect change in the LVOT VTI. This approach raises important facets of Doppler flow and insonation error, as well as the general principles at play when using a peripheral artery to infer changes from the left ventricle. Relating the VTI of a peripheral artery to the LVOT VTI was recently described mathematically and may help clinicians think about the Doppler relationship between central and peripheral flow.