Ventricular Outflow Tract Obstruction Research Articles

Left Ventricular Outflow Tract Obstruction (LVOTO) Causing Syncope in Hypertrophic Cardiomyopathy (HCM): A Case Report

Background: Hypertrophic cardiomyopathy (HCM) is one of the most common inherited cardiovascular diseases. HCM can sometimes result in dynamic obstruction of the left ventricular outflow (LVOT) tract that can subsequently compromise adequate cardiac output. The symptoms of left ventricular outflow tract obstruction (LVOTO) can range from mild symptoms of impaired cardiac output to sudden cardiac death (SCD) in extreme cases. Case Description: A 68-year-old female with comorbidities including obesity, obstructive sleep apnea (OSA), and hyperlipidemia but no prior diagnosis of HCM or known family history of HCM, presented after a witnessed syncope and subsequent head injury, who had an extensive evaluation of the etiology of her syncope. Transthoracic echocardiogram (TTE) revealed presence of HCM with evidence of dynamic LVOTO. The patient underwent a transesophageal echocardiogram (TEE) which showed hypertrophy of LVOT in the septal area causing moderate increase in LVOT mean gradient. The patient was started on medical management with diltiazem that was not tolerated because of bradycardia. The patient had a stable clinical course without any hemodynamic events and was discharged home with outpatient Cardiology follow-up. The patient was also advised to get a referral for a cardiac magnetic resonance (CMR) for further evaluation of HCM and the potential for dynamic LVOTO. Conclusions: HCM can cause dynamic LVOTO that can lead to symptoms including fatigue, chest pain, presyncope, and syncope. Extreme cases of LVOTO can lead to SCD. Medical management is imperative in HCM with symptoms of LVOTO. In extreme cases risk stratification for implantable cardioverter defibrillator (ICD) implantation is indicated to prevent SCD

Variant cardiac transthyretin amyloidosis presenting as hypertrophic cardiomyopathy with left ventricular outflow tract obstruction, a case report

Abstract Background Left ventricular outflow tract obstruction (LVOTO) is common in hypertrophic cardiomyopathy (HCM), but has not been reported in hereditary transthyretin amyloidosis (ATTRv). Case summary Here, 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 (SAM). The patient was childless and family history was negative for any cardiovascular disease. Significant coronary artery disease was diagnosed. In cardiac magnet resonance imaging (CMR), 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 ribonucleinacid (RNA) silencing therapy was commenced. Discussion The 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 ATTR amyloidosis should initiate specific diagnostic work-up even if ATTR is unlikely based on cardiac imaging.

Factors associated with high-intensity physical activity and sudden cardiac death in hypertrophic cardiomyopathy

BackgroundHigh-intensity physical activity has traditionally been discouraged in patients with hypertrophic cardiomyopathy due to concerns about triggering sudden cardiac death. However, current guidelines adopt a more liberal stance, and evidence...

Kissing balloon technique as rescue strategy to treat left ventricular outflow tract obstruction after transcatheter mitral valve replacement: a case report

Abstract Background Transcatheter mitral valve replacement (TMVR) has become a viable, minimally invasive treatment for inoperable patients with severe mitral valve disease, particularly among elderly individuals with significant comorbidities. A key complication of TMVR is left ventricular outflow tract (LVOT) obstruction, necessitating various preventive and therapeutic strategies. This report presents a case of severe LVOT obstruction following TMVR and highlights the effective application of the kissing balloon technique as a therapeutic intervention. Case Presentation A 79-year-old female with New York Heart Association class IV dyspnea due to severe mitral stenosis and a high operative risk (STS-PROM score 6.2%) underwent TMVR. Pre-procedural evaluations indicated significant mitral valve calcification and a mitral valve area of 0.9 cm². After successful TMVR deployment, post-implantation echocardiography revealed an LVOT pressure gradient of 53/85 mmHg, prompting the use of the kissing balloon technique, which reduced the gradient to 28 mmHg. Follow-up assessments showed normal mitral valve function and stable LVOT gradients during short-term follow-up throughout the patient’s hospital stay. Discussion LVOT obstruction is a potentially life-threatening complication of TMVR, often associated with high mortality rates due to hemodynamic impairment. This complication can arise from various anatomical factors and valve positioning issues. Several strategies have been developed to address LVOT obstruction, including the laceration of the anterior mitral leaflet and alcohol septal ablation. The successful implementation of the kissing balloon technique in this case underscores its potential to improve outcomes in LVOT obstruction.

A Case Report of Left Ventricular Outflow Tract Obstruction Due to Early Growth of a Calcified Amorphous Tumour Despite Normal Renal Function

A Case Report of Left Ventricular Outflow Tract Obstruction Due to Early Growth of a Calcified Amorphous Tumour Despite Normal Renal Function

Efficacy and safety of cardiac myosin inhibitors for symptomatic hypertrophic cardiomyopathy: a meta-analysis of randomized controlled trials

IntroductionHypertrophic cardiomyopathy (HCM) is a common genetic heart disorder. It is characterized by left ventricular hypertrophy and impaired cardiac function, with forms categorized into obstructive (oHCM) and nonobstructive (nHCM). Traditional treatments address symptoms but not the underlying disease mechanism, highlighting the need for novel therapies. Cardiac myosin inhibitors such as mavacamten and aficamten present potential new treatment options.MethodsA meta-analysis of randomized controlled trials (RCTs) was conducted following PRISMA guidelines. Studies comparing cardiac myosin inhibitors with placebo were reviewed, and outcomes related to NYHA functional class, Kansas City Cardiomyopathy Questionnaire Clinical Summary Score (KCCQ-CSS), LVOT gradients, and left ventricular ejection fraction (LVEF) were analyzed.ResultsSix RCTs involving 826 participants demonstrated that mavacamten and aficamten significantly improved NYHA functional class and KCCQ-CSS scores. The incidence of treatment-emergent adverse events (TEAEs) and serious adverse events (SAEs) was similar between the treatment and control groups, indicating a comparable safety profile.ConclusionCardiac myosin inhibitors are effective in improving cardiac function and reducing LVOT obstruction in HCM patients. They offer a promising alternative to current treatments, with a safety profile comparable to placebo. Further research is needed to confirm long-term benefits.

Genetic variations in PTPN11 lead to a recurrent Left Ventricular Outflow Tract Obstruction phenotype in childhood hypertrophic cardiomyopathy

ObjectiveLeft ventricular septal myotomy provides a favorable prognosis for children with hypertrophic obstructive cardiomyopathy (HOCM). However, some children still suffer from recurrent left ventricular outflow tract obstruction (LVOTO) after surgery. Poor prognosis exists for HOCM caused by PTPN11 mutation. Therefore, the aim of this study was to determine the clinical features of recurrent obstruction in children with HOCM caused by pathogenic mutations in the PTPN11 gene. MethodsA total of 56 children were diagnosed with HOCM underwent septal myectomies. Whole exome sequencing of 49 pediatric cardiomyopathies associated genes (including PTPN11) were performed. We performed hematoxylin-eosin(H&E), Masson, and wheat germ agglutinin (WGA)staining of tissues positive for PTPN11 and those negative for PTPN11 were conducted. ResultsWhole exome sequencing results showed 11 PTPN11 mutation (19.6%) children. In long-term follow-up (median 37 months, maximum 9 years), children with PTPN11 mutation had 6(54.5%) recurrent LVOTO compared with other groups (P=.015), but similar survival rates(P=.514). The mean postoperative time to recurrent obstruction was 22±27 months. Children with PTPN11 mutation were 9-fold more likely to experience the risk associated with recurrent obstruction (95% CI = 1.77-45.81, P<.001). H&E, Masson and WGA staining also revealed more cardiomyocyte hypertrophy in PTPN11 mutation tissues. See Figure 4 for a graphical abstract of the study. ConclusionChildren with PTPN11 mutation-associated hypertrophic cardiomyopathy have a higher risk of recurrent LVOTO.

Modulating energy metabolism to treat non-obstructive hypertrophic cardiomyopathy? Insights from IMPROVE-HCM.

Hypertrophic cardiomyopathy (HCM) is the most common genetic heart disease worldwide and may present with or without dynamic left ventricular outflow tract obstruction (LVOTO). Significant advances have been made in the management of obstructive HCM. On the other hand, despite their significant symptomatic burden, patients with non-obstructive HCM (nHCM) (i.e., without LVOTO) still do not have evidence-based therapeutical options. The recent IMPROVE-HCM study, a phase 2 randomized, double-blinded trial, aims to place a first step in filling this gap in knowledge. The study assessed the safety (primary endpoint) and efficacy (secondary endpoint) of ninerafaxstat, a novel cardiac mitotrope drug that increases adenosine triphosphate production. We highlighted the main findings of the trial, contextualizing these results within the larger landscape of completed and ongoing trials in nHCM.

Pharmacist-directed Mavacamten Treatment For Obstructive Hypertrophic Cardiomyopathy Is Associated With Symptom Improvement And Reduction In Left Ventricular Outflow Tract Obstruction

Pharmacist-directed Mavacamten Treatment For Obstructive Hypertrophic Cardiomyopathy Is Associated With Symptom Improvement And Reduction In Left Ventricular Outflow Tract Obstruction

Right Ventricular Outflow Tract Obstruction: Transcatheter Correction In Tetralogy Of Fallot: A Pilot Study.

This study evaluated transcatheter approach for relieving right ventricular outflow tract (RVOT) obstruction using combined non-compliant balloon dilatation of the RVOT and conal artery occlusion in patients with Tetralogy of Fallot (TOF), both uncorrected and post-intra-cardiac repair (ICR) restenosis. A prospective study was conducted from January 2022 to June 2023, including 40 symptomatic patients aged over 12 years with RVOT obstruction in TOF. Exclusion criteria included moderate to severe pulmonary regurgitation. The procedure involved two steps: non-compliant balloon dilatation of the RVOT and conal artery embolization using alcohol or coils or both. Clinical and echocardiographic assessments were performed pre-procedure, immediately post-procedure, and at 1-year follow-up. Thirty-five patients completed the study. Mean age was 19.8 (±4.2) years, with a male-to-female ratio of 4:3. Twenty-five patients had uncorrected TOF, and ten were post-ICR. Post-procedure, uncorrected patients showed a significant increase in oxygen saturation from 84.7 (±1.4) % to 94.6 (±1.2) %. Procedural success was 91.4%, with three patients experiencing significant restenosis. No procedural complications were observed. There were no arrhythmic events till the 1st year of follow up. At 1-year follow-up, the mean RVOT pressure gradient was significantly reduced, and all patients remained symptom-free. In conclusion, combined balloon pulmonic valvuloplasty and conal artery occlusion is a safe and effective method for alleviating RVOT obstruction in TOF patients, showing promising intermediate-term outcomes with minimal complications.

Beyond hypertrophic cardiomyopathy: unmasking alternative causes of LVOT obstruction on CMR.

Stress/Takotsubo cardiomyopathy (TCM) is a transient regional left ventricular (LV) systolic dysfunction, often mimicking acute myocardial infarction with normal coronary arteries. Rarely TCM can mimic hypertrophic cardiomyopathy (HCM). We describe a case where TCM presented with LV hypertrophy (LVH) and left ventricular outflow tract obstruction (LVOTO) which resolved on follow-up.

Tip-to-Base LAMPOON Procedure for a Valve-in-Valve Transcatheter Mitral Valve Replacement

Left ventricular outflow tract obstruction is a life-threatening complication of transcatheter mitral valve replacement caused by septal displacement of the anterior mitral leaflet. This complication can be avoided by using of a new technique, called LAMPOON or Laceration of Anterior Mitral leaflet to Prevent left ventricular Outflow ObstructioN. LAMPOON procedure involves splitting the anterior mitral leaflet just before transcatheter mitral valve replacement, which would otherwise be displaced anteriorly by the mitral valve implant and cause left ventricular outflow obstruction. In this case report, we discuss our initial experience with the tip-to-base LAMPOON technique to prevent left ventricular outflow obstruction.

Interventional Treatment of Acute Right Ventricular Outflow Tract Infectious Endocarditis: A Bridge to Surgical or Percutaneous Pulmonary Valve Replacement.

Infective endocarditis (IE) poses significant risks following percutaneous pulmonary valve implantation (PPVI) or surgical replacement (PVR). This study evaluates the effectiveness of emergency percutaneous treatment in stabilizing patients with severe right ventricular dysfunction or obstructive cardiac shock, allowing for delayed surgical or percutaneous valve replacement. This retrospective study examines 16 patients (age 19.9 [15.1-43.3] years) with right-sided IE treated with primary percutaneous intervention. Data collected included cardiac diagnosis, valve types, pathogens identified, procedural details, and outcomes. Underlying diagnoses included aortic valve stenosis post-Ross surgery (43%), repaired tetralogy of Fallot (25%), common arterial trunk (18%), and repaired double outlet right ventricle (14%). Valve types varied, with Contegra Ven-Pro (56%) and Melody-valve (25%) being the most common. Diagnosis of IE occurred 66.5 (28.3-87.4) months postlast intervention/surgery, with emergency percutaneous treatment performed 2 (1-17) days postdiagnosis. Clinical presentations included obstructive cardiac shock (50%) and septic shock (25%). Preintervention RVOT velocity was 4.4 (4.2-5) m/s, with severely reduced RV function in 68%. Interventions included balloon dilatation, uncovered or covered stent implantation, and Melody valve implantation. Immediate resolution of RVOT obstruction was achieved in all patients, with significant reductions in RV systolic pressures (mean reduction 42.0 (30.2-50.0) mmHg) and improved RV function. No periprocedural deaths occurred, but one complication was noted. Follow-up included surgical repair (68%) and PPVI (18%). Mortality included one IE relapse and one surgical death. Emergency percutaneous interventions can stabilize patients with RVOT obstruction due to IE, enabling delayed surgical or percutaneous interventions.

Tri-leaflet mitral valve in hypertrophic obstructive cardiomyopathy

AbstractA tri-leaflet mitral valve (MV) is an extremely rare phenomenon, often associated with significant mitral regurgitation (MR). It may represent a phenotypic expression of hypertrophic obstructive cardiomyopathy (HOCM). We present the case of a 69-year-old woman with HOCM, a tri-leaflet MV, and systolic anterior motion (SAM)–induced severe MR. Despite receiving optimal medical therapy, her refractory symptoms and significant MR prompted surgical consideration. The patient underwent surgical myectomy, resulting in improvement of left ventricular outflow tract obstruction, SAM-induced MR, and symptomatic relief. This case illustrates a unique presentation of HOCM with a tri-leaflet MV and significant MR, successfully managed with a tailored surgical myectomy.

A Triple Threat: A Case Report Detailing Surgical Management for Hypertrophic Cardiomyopathy, Flail Mitral Valve and Severe Pulmonary Hypertension†.

Pulmonary hypertension is an independent predictor of all-cause mortality in patients with hypertrophic cardiomyopathy managed medically and often precludes patients from undergoing cardiopulmonary bypass due to increased surgical morbidity and mortality. In studies specifically evaluating surgical myectomy, however, survival is favorable in patients with moderate-to-severe pulmonary hypertension. We present a case of a 74-year-old male with six months of dyspnea with minimal exertion. A diagnostic work-up with transthoracic echocardiogram showed asymmetric left ventricular hypertrophy, left ventricular outflow tract obstruction with a peak gradient of 200 mmHg, right ventricular systolic pressure of 99 mmHg, systolic anterior motion of the mitral valve and flail anterior mitral leaflet. The patient was evaluated by a multi-disciplinary team and underwent extended septal myectomy and mitral valve repair with significant improvement in functional capacity post-operatively. While pulmonary hypertension increases the risk of morbidity and mortality during cardiopulmonary bypass, moderate-to-severe pulmonary hypertension in hypertrophic cardiomyopathy with outflow tract obstruction is a unique indication for septal reduction therapy that may not be associated with higher surgical mortality.

A case of a young man with secondary hypertension

Background: Secondary hypertension is characterized by an elevated blood pressure greater than 140/90 mmHg, which occurs as a consequence of other diseases. The common etiologies of secondary hypertension include renal parenchymal causes, endocrine disorders, and vascular pathologies like coarctation of the aorta (CoA). Case presentation: A 20-year-old patient was admitted to our hospital as he complained of headache and palpitations since one week. On examination, the blood pressure in his right upper limb was 180/100 mmHg. The volume of the femoral and the dorsalis pedis pulses was found to be reduced bilaterally. The patient was started on antihypertensive medication labetalol 10 mg injection intravenously immediately. After clinical suspicion and a series of investigations, the patient was diagnosed with severe CoA, distal to the origin of the left subclavian artery via computed tomography (CT) aortogram. The patient was managed by coarctoplasty with stenting. Discussion: The most striking examination findings indicative of CoA include decreased lower limb pulses and a blood pressure difference of &gt;20 mmHg across both the lower and upper extremities. It is important to evaluate the blood pressure in both upper and lower limbs to diagnose obstructive vascular diseases. Conclusion: The presence of multiple well-developed collaterals can often mask symptoms and delay the detection of hypertension in patients with CoA. Patients with CoA require regular follow-up to monitor left ventricular outflow tract obstruction, and patients with severe CoA should be treated interventionally to prevent complications including aortic aneurysm and dissection. The patient was managed by coarctoplasty with stenting and recovered well post-surgery.

Tailored Therapies for Cardiogenic Shock in Hypertrophic Cardiomyopathy: Navigating Emerging Strategies.

Hypertrophic cardiomyopathy (HCM) is a complex and heterogeneous cardiac disorder, often complicated by cardiogenic shock, a life-threatening condition marked by severe cardiac output failure. Managing cardiogenic shock in HCM patients presents unique challenges due to the distinct pathophysiology of the disease, which includes dynamic left ventricular outflow tract obstruction, diastolic dysfunction, and myocardial ischemia. This review discusses current and emerging therapeutic strategies tailored to address the complexities of HCM-associated cardiogenic shock and other diseases with similar pathophysiology that provoke left ventricular outflow tract obstruction. We explore the role of pharmacological interventions, including the use of vasopressors and inotropes, which are crucial in stabilizing hemodynamics but require careful selection to avoid exacerbating the outflow obstruction. Additionally, the review highlights advancements in mechanical circulatory support devices such as extracorporeal membrane oxygenation (ECMO) and left ventricular assist devices (LVADs), which have become vital in the acute management of cardiogenic shock. These devices provide temporary support and bridge patients to recovery, definitive therapy, or heart transplantation, which remains a critical option for those with end-stage disease. Furthermore, the review delves into the latest research and clinical trials that are refining these therapeutic approaches, ensuring they are optimized for HCM patients. The impact of these treatments on patient outcomes, including survival rates and quality of life, is also critically assessed. In conclusion, this review underscores the importance of a tailored therapeutic approach in managing cardiogenic shock in HCM patients, integrating pharmacological and mechanical support strategies to improve outcomes in this high-risk population.

ИНФАРКТ МИОКАРДА БЕЗ ОБСТРУКТИВНЫХ ИЗМЕНЕНИЙ КОРОНАРНЫХ АРТЕРИЙ У БОЛЬНОГО С ГИПЕРТРОФИЧЕСКОЙ КАРДИОМИОПАТИЕЙ

Background. Myocardial infarction with non-obstructive coronary arteries is defined by the presence of clinical symptoms and signs of myocardial infarction despite angiographic findings of normal or near-normal coronary arteries. Myocardial infarction with non-obstructive coronary arteries can be classified into myocardial infarction type 1 or type 2. Hypertrophic cardiomyopathy is a possible underlying etiology of myocardial infarction with non-obstructive coronary arteries. The aim. To present the diagnosis of myocardial infarction in a patient with hypertrophic cardiomyopathy. Case descriptions. A clinical case of myocardial infarction with non-obstructive coronary arteries in a man with hypertrophic cardiomyopathy is presented. 57-year-old man presented with retrosternal chest pain that appeared at rest but during an attack of tachyarrhythmia. Electrocardiogram showed right bundle branch block and T inversion in the I, avL, V1-V6. Cardiac troponin T was with a rise and fall in the level. Echocardiography demonstrated asymmetrical septal hypertrophy with thickness of the basal and medium segments of the septum 18 mm, systolic anterior motion of the mitral valve, and left ventricular outflow tract obstruction with a peak systolic pressure gradient of 50 mmHg, diastolic dysfunction of the left ventricle. Coronary angiography revealed stenosis of the left anterior descending artery &lt; 50 %. All these data allow us to diagnose the myocardial infarction with non-obstructive coronary arteries. Type 2 infarction is most likely, however, the absence of an intracoronary imaging does not completely exclude type 1 myocardial infarction. Conclusion. The clinical case demonstrates myocardial infarction in the absence of obstructive coronary artery disease in patient with hypertrophic cardiomyopathy. To exclude plaque disruption intracoronary imaging should be performed.

Modified-Bentall Single-Patch Konno Enlargement Technique for Aortic Stenosis and Prosthesis-Patient Mismatch.

Aortic stenosis and prosthesis-patient mismatch complicate surgery for patients with small left ventricular outflow tracts. We present outcomes of a modified-Bentall single-patch Konno enlargement (BeSPoKE) technique for complex left ventricular outflow tract obstruction in adults. The BeSPoKE technique facilitates a true outflow tract enlargement via an anterior septo-ventriculoplasty, using a single pericardial patch, followed by composite aortic valve-graft root replacement. Postoperative outflow tract geometry and valvular physiology were compared to preoperative measurements using echocardiography and computed tomographic angiography; two-year clinical outcomes were assessed. From October 2017 to March 2022, 25 adults (median age 60 years; 84% female) underwent a BeSPoKE repair. Mean preoperative aortic valve gradient was 44 ± 19mmHg. Twenty-one (84%) patients had previous aortic valve replacements with prosthesis-patient mismatch; median implant size preoperatively was 19mm. Postoperatively, all patients received a prosthesis of at least 21mm, with a median upsizing of 2 (15th-85th Percentile, 2-3 sizes). Mean postoperative aortic valve gradient was 8.5 ± 4.1mmHg (P<.001); mean 2-year gradient was 8.3 ± 1.3mmHg. All patients with bioprosthetic replacements qualified for future transcatheter valve replacements. Postoperative complications included atrial fibrillation (9; 36%) and complete heart block requiring pacemaker placement (8; 32%). There were zero operative mortalities and no reoperations reported. There were 2 late non-cardiac-related deaths; 2-year survival was 92%. The modified-Bentall single-patch Konno enlargement technique facilitates larger prosthesis placement, improves hemodynamics, and enables future transcatheter reinterventions. This approach is a safe treatment for complex left ventricular outflow tract obstruction and prosthesis-patient mismatch in adults.

The Association of Takotsubo Cardiomyopathy, Left Ventricular Outflow Tract Obstruction, and Hypotension

The Association of Takotsubo Cardiomyopathy, Left Ventricular Outflow Tract Obstruction, and Hypotension