Left Ventricular Sarcoma Causing Dynamic Outflow Tract Obstruction and Cardiogenic Shock: A Case Report

Background: Takotsubo cardiomyopathy (TC) can cause dynamic left ventricular outflow tract (LVOT) obstruction leading to cardiogenic shock. Due to differential wall-motion abnormalities, flow acceleration in the LVOT can lead to systolic anterior motion (SAM) of the mitral valve which worsens LVOT obstruction and leads to hemodynamic compromise. We present a case of overlap between TC and hypertrophic obstructive cardiomyopathy (HOCM) with worsening LVOT obstruction leading to cardiogenic shock. Case: A 76-year-old female presented with typical chest pain, nausea, and worsening shortness of breath. On examination, she was hypotensive and cold to touch. EKG showed T-wave inversions in lateral leads with elevated cardiac troponins. Coronary angiogram showed non-obstructive coronary artery disease, and right heart catheterization revealed elevated filling pressures. Transthoracic echocardiogram (TTE) showed septal hypertrophy and SAM of the mitral valve causing dynamic LVOT obstruction (Figure 1) and ejection fraction (EF) of 20%. TTE with contrast showed apical ballooning consistent with TC (Figure 2). Decision Making: Initially with diuresis, she became hypotensive so it was stopped and she received controlled intravenous fluids along with phenylephrine to reduce LVOT obstruction. Her blood pressure improved and she was started on low-dose metoprolol succinate along with ivabradine to reduce heart rate. She tolerated this therapy and hemodynamics stabilized. Conclusion: Cardiogenic shock can be a severe complication of TC. Early detection of LVOT obstruction in cardiogenic shock is important as traditional management strategies of increased inotropy and reduction of afterload can be fatal in these cases. The treatment strategy focuses on reducing the LVOT gradient and includes fluid administration to increase preload, beta-blockers to increase diastolic filling time, and vasopressors to raise afterload.

Left Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy: Do We Need to Stand to Be Counted

Dynamic left ventricular outflow tract obstruction complicated with takotsubo cardiomyopathy: The acute phase of takotsubo cardiomyopathy manifests latent left ventricular outflow tract obstruction

Bail-Out Alcohol Septal Ablation for Hypertrophic Obstructive Cardiomyopathy in a Patient With Takotsubo Cardiomyopathy-Induced Cardiogenic Shock.

2011;58;2703-2738; originally published online Nov 8, 2011; J. Am. Coll. Cardiol. W. Yancy Rakowski, Christine E. Seidman, Jeffrey A. Towbin, James E. Udelson, and Clyde Fifer, Mark S. Link, Srihari S. Naidu, Rick A. Nishimura, Steve R. Ommen, Harry Bernard J. Gersh, Barry J. Maron, Robert O. Bonow, Joseph A. Dearani, Michael A. Thoracic Surgeons Society for Cardiovascular Angiography and Interventions, and Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, of Thoracic Surgery, American Society of Echocardiography, American Society Developed in Collaboration With the American Association for Guidelines Cardiology Foundation/American Heart Association Task Force on Practice Cardiomyopathy: Executive Summary: A Report of the American College of 2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic This information is current as of May 14, 2012 http://content.onlinejacc.org/cgi/content/full/58/25/2703 located on the World Wide Web at: The online version of this article, along with updated information and services, is

Although dobutamine stress echocardiography (DSE) is frequently associated with dynamic left ventricular outflow tract obstruction (DLVOTO), little is known about its clinical significance in women with suspected coronary artery disease (CAD). One hundred and two female patients (57±10 years) who underwent DSE as part of the Korean women's chest pain registry study were included. Doppler echocardiography was performed during DSE to assess the presence of DLVOTO. Patients with DLVOTO (n=52) were older than those without DLVOTO (n=50; P=0.001). Hypertension was more prevalent in patients with DLVOTO (P=0.02). Patients with DLVOTO had smaller LV diameter, but higher LV mass index and relative wall thickness (P<0.05 for all). LV diastolic function (as reflected by late diastolic velocity, deceleration time of early diastolic velocity [E], and ratio of E velocity to early diastolic mitral annular velocity), was worse in patients with DLVOTO (P<0.05 for all). Patients with DLVOTO had shorter exercise time (P=0.02) and lower amount of work (P=0.04) than patients without DLVOTO. DSE-provoked DLVOTO was not related to the presence of CAD in these patients. In Korean women with suspected CAD, DSE-provoked DLVOTO is correlated with LV concentric remodeling and LV diastolic dysfunction, and may be associated with limited exercise tolerance and symptoms of chest pain.

In patients without hypertrophic obstructive cardiomyopathy (HOCM), dynamic left ventricular outflow tract obstruction (DLVOTO) can cause ischaemia. Little is known about incidence and predictors of DLVOTO in patients without HOCM. In 300 patients referred for exercise echocardiography, assessment of DLVOTO at rest and with Valsalva and of the presence of systolic anterior motion of the mitral valve leaflets (SAM) was performed. Within 90 s post-exercise, wall motion, SAM, and DLVOTO were assessed again. A significant DLVOTO was defined as late-peaking Doppler velocity of >or=2.5 m/s (25 mmHg). Excluded were 7 patients with HOCM and 13 with inadequate image quality. There were 280 patients, aged 64(11) years. Coronary artery disease was found in 38% of patients; 44% were receiving beta-blocker therapy and 35% had hypertension. At rest, ejection fraction was 59 +/- 9%; left ventricular hypertrophy (LVH) was present in 21%, SAM in 16%, DLVOTO >or=25 mmHg at rest in 0.7%, and with Valsalva in 3%. At peak, echocardiographic signs of ischaemia occurred in 44%, and significant DLVOTO in 5% (13 patients). By multivariate analysis, it was found that independent predictors of significant DLVOTO at peak were chordal SAM at peak, smaller left ventricle at end-systole, higher systolic blood pressure at peak, younger age and increased septal wall thickness. Significant DLVOTO was a possible cause of symptoms and/or ischaemia in at least 6 of the 13 patients. Haemodynamically significant exercise-induced DLVOTO can occur without HOCM. Chordal SAM at peak, small, hyperdynamic left ventricles, increased septal wall thickness, and younger age are the best predictors.

Concurrent lesions of dynamic left ventricular outflow tract obstruction (DLVOTO) with aortic stenosis pose a challenge in the measurement of the pressure gradient and severity of each lesion. Determining the true culprit lesion is difficult and challenging. The establishment of true culprit lesion is crucial in deciding the future course of action. We present two cases of concurrent DLVOTO and aortic stenosis. Although the composition of lesions is similar, the severity of each lesion was different and described a variety of technical problems. Finding the culprit through the shape of the stenotic jet from the continuous wave Doppler as well as other different technical approaches is the critical point of this case report. The first patient showed nonsignificant DLVOTO with severe aortic stenosis in which transthoracic echocardiography (TTE) alone was sufficient to find the culprit. Meanwhile, the second patient concluded to have significant DLVOTO with moderate aortic stenosis based on TTE and transesophageal echocardiography examination data. Jet morphology from Doppler examination is a crucial finding to differentiate DLVOTO with aortic stenosis, along with other parameters that might help find the dominant lesion. Multiple modalities with several tailor-made technical considerations might be needed to establish a culprit lesion.

Background.Hypertrophic cardiomyopathy (HCM) in pediatric solid organ transplant recipients has been reported in association with use of calcineurin inhibitors. However, data on the incidence and prevalence of HCM in adult posttransplant patients are limited. We sought to describe the clinical characteristics of solid organ transplant recipients who were diagnosed with HCM from 2011 to 2021 at a single center.Methods.Patients who had undergone solid organ transplant and exhibited left ventricular hypertrophy with left ventricular wall thickness ≥13 mm on transthoracic echocardiography were included. Clinical history, pedigree analysis, clinical genetic testing, transthoracic echocardiography, cardiac magnetic resonance imaging, treatment, and follow-up testing results were collected. Categorical variables were described as n (%). Continuous variables were described with medians and interquartile ranges and compared using the Wilcoxon rank-sum and Kruskal-Wallis tests. A 2-sided P < 0.05 was considered statistically significant.Results.Three lung, 5 kidney, and 4 liver transplant recipients from 12 different families were included. Seven patients (58%) did not carry a preexisting diagnosis of hypertension, and none had a history of aortic or subaortic stenosis. A majority of patients exhibited asymmetric septal hypertrophy (67%; medial septal thickness versus left ventricular posterior wall thickness 17 versus 13 mm; P < 0.001) and dynamic left ventricular outflow tract (LVOT) obstruction (58%). All patients were managed long term with calcineurin inhibitors. Clinical genetic testing in 6 patients identified 2 with disease-causing variants in 2 sarcomere genes, myosin binding protein-C and myosin heavy chain 7. Four patients (33%) underwent successful septal reduction therapy for treatment of symptomatic LVOT obstruction.Conclusions.Symptomatic HCM with dynamic LVOT obstruction can develop in solid organ transplant recipients, and genetic testing can identify individuals with sarcomeric HCM. Medical management and septal reduction therapies are treatment options for severe symptomatic disease.

Hypertrophic cardiomyopathy (HCM) is a common monogenetic cardiac disease with a prevalence of 0.2%1 that usually is inherited as an autosomal dominant trait with variable penetrance and expression. Clinically, HCM is defined by the presence of left ventricular (LV) hypertrophy (LVH) unexplained by abnormal loading conditions.1 The natural history varies from an asymptomatic course to drug-refractory angina/dyspnea, sudden cardiac death (SCD), and end-stage heart failure. The incidence of HCM-related SCD is approximately 1% to 2% in children and adolescents and 0.5% to 1% in adults.2,3 In this review, we examine the impact of advanced diagnostic imaging technologies on HCM, with particular emphasis on the detection of particular genetic subtypes. In ≈60% of cases, HCM is inherited as an autosomal dominant trait caused by mutations in genes coding for cardiac sarcomere proteins (Figure 1).4–7 Mutations in genes encoding Z-disc proteins and proteins involved in calcium regulation account for <5% of cases. The absence of sarcomeric gene mutations can be explained by limitations of current mutation detection techniques or mutations in as yet unidentified genes, but in some cases, hypertrophy is caused by other diseases that mimic the phenotype of sarcomeric protein disease. Table 1 lists genetic disorders associated with HCM. Figure 1. A schematic illustration of a cardiac sarcomere. View this table: Table 1. Currently Known Genes Implicated in HCM More than 600 different sarcomeric gene mutations are reported in patients with HCM.8–16 The majority are missense mutations, but nonsense, frameshift, and in-frame insertion/deletion mutations also occur. Most mutations are characterized by incomplete penetrance and variable clinical expression likely due to locus heterogeneity and the effect of mutations at different locations within the same gene. Most mutations probably have a dominant negative effect on sarcomere function, but in some cases, haploinsufficiency (ie, only a single functional …

Left ventricular outflow tract obstruction caused by massive mitral annular calcification in a patient with hypertensive heart disease

Prevalence of dynamic left ventricular outflow tract obstruction (DLVO) during dobutamine stress-echo (DSE) seems disproportionally high among diabetic patients. We retrospectively identified 212 diabetic (D+) and 212 non diabetic (D-) subjects, who underwent DSE for suspected coronary artery disease (CAD); we evaluated DSE-induced DLVO prevalence and correlates. During DSE, 105 patients in D+ (50%) and 83 in D- group (39%, P = 0.032) developed a DLVO, with similar maximum gradient (94 ± 49 mmHg in D+ vs. 86 ± 49 mmHg in D-, P = NS). D+ and D- patients with DLVO showed reduced LV end-diastolic and end-systolic dimension. Compared with diabetic subjects without DLVO, diabetic patients with DLVO had higher left ventricular (LV) ejection fraction (EF), lower LV mass index; diastolic function was normal in a higher proportion of cases. Non diabetic patients with moderate or severe DLVO had higher LV EF compared with patients without DLVO. At multivariate analysis, in D+ patients, the only independent predictor was a smaller LV end-diastolic diameter (HR 0.779, CI 0.655-0.926, P = 0.005); in D- patients lower age (HR 0.878, CI 0.806-0.957, P = 0.003), higher LV EF (HR 1.087, CI 1.003-1.177, P = 0.042) and lower peak WMSI (HR 0.017, CI 0.001-0.325, P = 0.007) were associated to presence of DLVO. In D+ patients, during a median follow-up of 924 ± 134 days, we observed 11 new cardiac events, only 1 in patients with DLVO (P = 0.0041). DSE-provoked DLVO had a very high prevalence in patients evaluated for suspected CAD, especially among diabetic patients; echocardiographic predictors were a reduced LV dimension in D+ and a preserved systolic function, both at rest and at peak stress, in D- patients.

Dynamic left ventricular outflow tract (LVOT) obstruction remains a clinically significant perioperative risk factor in candidates for liver transplantation (LT). Among patients with end-stage liver disease (ESLD), conventional resting echocardiography may inadequately characterize the hemodynamic burden of provoked LVOT obstruction, resulting in incomplete cardiovascular risk stratification before transplantation. This report details the use of an invasive preload-provocation fluid challenge to distinguish dynamic from fixed LVOT obstruction, thereby directly impacting anesthetic planning and transplant candidacy.A 69-year-old female patient with drug-induced ESLD displayed a resting transthoracic LVOT gradient of 84 mmHg while on beta-blocker therapy. Cardiac catheterization revealed a baseline intracavitary mid-cavity-to-LVOT gradient of 60 mmHg. Administration of a 500 mL 5% albumin preload over 10 minutes resulted in a reduction of the gradient to 16 mmHg, confirming a load-dependent dynamic obstruction consistent with ESLD physiology rather than fixed LVOT obstruction. Following reassessment, the patient successfully underwent LT utilizing advanced invasive hemodynamic monitoring. Anesthetic management prioritized afterload support and meticulously controlled volume administration, yielding intraoperative cardiovascular stability. Postoperatively, echocardiography demonstrated resolution of LVOT gradients and preserved graft function at six months.This proof-of-concept case underscores the utility of a standardized invasive preload challenge in distinguishing dynamic from fixed LVOT obstruction and guiding perioperative strategy. In the presence of preload-responsive gradients and careful afterload maintenance, LVOT obstruction should not be considered an absolute contraindication to LT. Integration of this diagnostic approach into pre-transplant assessment protocols may refine cardiovascular risk stratification and expand LT eligibility among patients presenting with this unique hemodynamic profile.

Late-onset dynamic outflow tract gradient in the setting of tako-tsubo cardiomyopathy: An interesting phenomenon with potential implications?

Sigmoid Ventricular Septum (SVS) is a type of hypertrophic cardiomyopathy characterized by a reduced angle between the basal interventricular septum and the ascending aorta, and SVS can lead to dynamic Left Ventricular Outflow Tract obstruction (LVOTO) during hypercontractile states. Patients experiencing LVOTO may manifest symptoms such as angina, syncope, etc. Radiofrequency ablation (RFA) has been utilized to treat patients with hypertrophic obstructive cardiomyopathy, but there is no reports on its use in treating LVOTO resulting from SVS. Our report describes two cases of SVS treated with endocardial ablation to improve LVOTO. Case 1: A 74-year-old female patient with angina and syncope was admitted to the hospital and diagnosed with SVS by transthoracic echocardiogram. The patient exhibited LVOTO and Systolic Anterior Motion (SAM) phenomena during the administration of the dobutamine stress test. After RFA was performed, the patient's symptoms significantly improved. Additionally, septum decreased from 16 to 13 mm after ten months, and the morphological changes associated with SVS also disappeared. Case 2: A 57-year-old female was admitted to the hospital due to recurrent chest pain after physical activity for more than four years. The transthoracic echocardiogram indicated that the patient met the diagnostic criteria for SVS, and LVOTO and SAM phenomenaoccurred following dobutamine stress test. The patient had significant improvement in symptoms after undergoing RFA treatment. These two cases represent the first documented instances where dynamic LVOTO caused by SVS could be effectively managed through endocardial RFA.