A rare pediatric case of left ventricular outflow tract infective endocarditis in hypertrophic cardiomyopathy.
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ABSTRACT:
SUBMITTER: Lee ME
PROVIDER: S-EPMC8307343 | biostudies-literature | 2020 Dec
REPOSITORIES: biostudies-literature
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A rare pediatric case of left ventricular outflow tract infective endocarditis in hypertrophic cardiomyopathy.
Lee Madonna E ME Kemna Mariska M Schultz Amy H AH McMullan David Michael DM
JTCVS techniques 20200922
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Project description:Hypertrophic cardiomyopathy (HCM) is a common genetic disorder characterised by unexplained left ventricular hypertrophy. Left ventricular outflow tract obstruction is an integral component of the disease, often resulting in significant symptoms, but also carrying a risk of progression to heart failure and death. Advancements in our understanding of the pathophysiology of HCM have led to the development of new therapies targeting the molecular basis of the disease at the level of the cardiac sarcomere, the basic contractile apparatus of the myocardium. Myosin modulators are a novel class of small molecules which target cardiac myosins directly to modulate cardiac contractility. The myosin inhibitors present the first advancement in pharmacological management of obstructive HCM in almost 35 years, with a growing body of evidence for the safety, tolerability and efficacy of mavacamten, and to a lesser extent aficamten. The aim of this review is to summarise the current management of patients with obstructive HCM and review the most recent available data from clinical trials pertaining to myosin inhibition.
| S-EPMC9666864 | biostudies-literature
Project description: Not available
| S-EPMC7581563 | biostudies-literature
Project description:Hypertrophic cardiomyopathy (HCM) is one of the most common genetic cardiac disorders and is characterized by different phenotypes of left ventricular hypertrophy with and without obstruction. The effects of left ventricular outflow tract (LVOT) obstruction based on different anatomies may be hemodynamically relevant and influence therapeutic decision making. Cardiovascular magnetic resonance (CMR) provides anatomical information. We aimed to identify different shapes of LVOT-obstruction using Cardiovascular Magnetic Resonance (CMR). The study consisted of two parts: An in-vivo experiment for shape analysis and in-vitro part for the assessment of its hemodynamic consequences. In-vivo a 3D depiction of the LVOT was created using a 3D multi-slice reconstruction from 2D-slices (full coverage cine stack with 7 slices and a thickness of 5-6 mm with no gap) in 125 consecutive HOCM patients (age = 64.17 +/- 12.655; female n = 42). In-vitro an analysis of the LVOT regarding shape and flow behavior was conducted. For this purpose, 2D and 4D measurements were performed on 3D printed phantoms which were based on the anatomical characteristics of the in-vivo study, retrospectively. The in-vivo study identified three main shapes named K- (28.8%), X- (51.2%) and V-shape (10.4%) and a mixed one (9.6%). By analyzing the in-vitro flow measurements every shape showed an individual flow profile in relation to the maximum velocity in cm/s. Here, the V-shape showed the highest value of velocity (max. 138.87 cm/s). The X-shape was characterized by a similar profile but with lower velocity values (max. 125.39 cm/s), whereas the K-shape had an increase of the velocity without decrease (max. 137.11 cm/s). For the first time three different shapes of LVOT-obstruction could be identified. These variants seem to affect the hemodynamics in HOCM.
| S-EPMC11561129 | biostudies-literature
Project description:AimsDynamic left ventricular (LV) outflow tract obstruction (LVOTO) is associated with symptoms and increased risk of developing heart failure in hypertrophic cardiomyopathy (HCM). The association of LVOTO and LV twist mechanics has not been well studied in HCM. The aim of the study was to compare the pattern of LV twist in patients with HCM associated with asymmetrical septal hypertrophy with and without LVOTO.Methods and resultsEchocardiography (including speckle tracking) was performed in 212 patients with HCM, divided according to the absence (n = 130) or presence (n = 82) of LVOTO (defined as peak pressure gradient ≥30 mmHg either at rest and/or with Valsalva manoeuvre). Patients with LVOTO were older, had smaller LV dimensions, a higher LV ejection fraction (LVEF), a longer anterior mitral valve leaflet length, and a higher early transmitral pulsed wave to septal tissue Doppler velocity ratio (E/E'). A univariate analysis showed that peak twist was significantly higher in patients with LVOTO compared with patients without LVOTO (19.7 ± 7.3 vs. 15.7 ± 6.0, P = 0.00015). Peak twist was similarly enhanced in patients with LVOTO, manifesting only during Valsalva (19.2 ± 5.6, P = 0.007) and patients with resting LVOTO (19.9 ± 8.0, P = 0.00004) compared with patients without LVOTO (15.7 ± 6.0). A stepwise forward logistic regression analysis showed that LVEF, LV end-systolic dimension indexed to body surface area, anterior mitral valve leaflet length, E/E', and peak twist were all independently associated with LVOTO.ConclusionThis study demonstrates that increased peak LV twist is independently associated with LVOTO in patients with HCM. Peak twist was similarly exaggerated in patients with only latent LVOTO, suggesting that it may play a contributory role to LVOTO in HCM.
| S-EPMC10442061 | biostudies-literature
Project description:BackgroundHypertrophic 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.MethodsThis 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.Results16 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.ConclusionsDiscontinuation 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.
| S-EPMC11286422 | biostudies-literature
Project description:AimsNot all obstructive hypertrophic cardiomyopathy (HCM) patients are symptomatic. The relation between obstructive HCM and symptoms is not well understood. The hypothesis of this study is that left-ventricular outflow tract (LVOT) acceleration time (AT) is associated with symptoms.MethodsWe included 187 patients (61% men, mean age 55 ± 14 years) with obstructive HCM, defined as a maximal wall thickness ≥ 15 mm and a resting or provoked LVOT peak gradient ≥ 30 mmHg. Peak velocity (PV), left-ventricular (LV) ejection time (ET), and AT (the time between LVOT flow onset and the moment of PV) were measured on continuous-wave (CW) Doppler tracings. Logistic and Cox proportional hazard regression analyses were used to evaluate the relation between symptoms [New York Heart Association (NYHA) class ≥ II] and echocardiographic measurements, including AT. Reproducibility was assessed using the intraclass correlation coefficient (ICC).ResultsSymptomatic patients were more often female and had higher mean AT values. Logistic regression demonstrated a significant association between AT and symptomatic status (odds ratio 1.31 per 10 ms, p < 0.01) after adjustment for sex, negative inotropes, PV, LVOT diameter, and diastolic dysfunction. AT was independently associated with symptoms and septal reduction during follow-up (hazard ratio 1.09 per 10 ms, p < 0.05). The ICC was 0.98 with a mean difference of 0.28 ± 8.4 ms.ConclusionIn obstructive HCM patients, increased AT is significantly related to symptoms after adjustment for sex, negative inotropes, PV, LVOT diameter, and diastolic dysfunction, and is associated with the symptomatic status during follow-up. AT represents an easily measured echocardiographic variable with excellent inter-reader reproducibility.
| S-EPMC8363723 | biostudies-literature
Project description: Not available
| S-EPMC5805115 | biostudies-literature
Project description:BackgroundLeft ventricular outflow tract obstruction (LVOTO) is common in hypertrophic cardiomyopathy (HCM) but has not been reported in hereditary transthyretin amyloidosis (ATTRv).Case summaryHere, we describe a 67-year-old male patient with a hypertrophic phenotype who was initially diagnosed with LVOTO attributed to HCM. Echocardiographic features included a hyperdynamic left ventricular ejection fraction, severe septal hypertrophy, and elongated residual mitral leaflets permitting their systolic anterior motion. The patient was childless, and family history was negative for any cardiovascular disease. Significant coronary artery disease was diagnosed. In cardiac magnetic resonance imaging, the concurrent presence of different late enhancement patterns initially prevented an unequivocal diagnosis. Due to progressive neuropathy, genetic testing for ATTRv was performed and identified a pathogenic variant in the TTR gene. Cardiac involvement was confirmed by amyloid scintigraphy with a Perugini score of 3, and treatment with ribonucleic acid silencing therapy was commenced.DiscussionThe present case illustrates that LVOTO can occur in patients with ATTRv. While cardiac imaging pointed towards sarcomeric HCM, the coexisting red flags initiated re-evaluation of the diagnosis suggesting cardiac amyloidosis as a potential cause. The presence of non-cardiac red flags of transthyretin amyloidosis (ATTR) should initiate specific diagnostic work-up even if ATTR is unlikely based on cardiac imaging.
| S-EPMC11799945 | biostudies-literature
Project description:BackgroundLeft ventricular outflow tract (LVOT) obstruction is an important determinant of the management of hypertrophic cardiomyopathy (HCM). With a nationwide shortage of amyl nitrite in 2019, we implemented a 'repetitive squat-to-stand' manoeuvre to provoke LVOT obstruction during echocardiography.Case summaryA 64-year-old female was referred with symptomatic HCM refractory to pharmacologic therapy. Transthoracic echocardiography showed minor LVOT obstruction with conventional imaging at rest and during Valsalva manoeuvre, but severe obstruction was confirmed with the repetitive squat-to-stand manoeuvre. Alcohol septal ablation via the first septal perforator was performed with subsequent resolution of symptoms.DiscussionDue to the dynamic nature of LVOT obstruction, a series of provocative manoeuvres including Valsalva manoeuvre, inhalation of amyl nitrite, and exercise are often necessary to maximally augment ventricular obstruction. The recent unavailability of amyl nitrite during a nationwide shortage prompted the implementation of a protocol of repetitive squat-to-stand manoeuvre in our echocardiography laboratory. Rising from the squatting position decreases preload and afterload, both of which augment dynamic LVOT obstruction. Repetition of squatting and standing appears to enhance the sensitivity of the manoeuvre, particularly when exertional symptoms are reproduced. In this case, repetitive squat-to-stand manoeuvre led to the identification of severe LVOT obstruction which may not have been diagnosed otherwise, alteration of treatment to septal reduction therapy, and subsequent resolution of symptoms.
| S-EPMC7898584 | biostudies-literature
Project description: Not available
| S-EPMC7791289 | biostudies-literature