Transaortic extended left ventricular septal myectomy in an adult with hypertrophic obstructive cardiomyopathy

2020 ◽  

In symptomatic patients with an obstructive variant of hypertrophic cardiomyopathy and no response to maximal medical therapy, we recommend a septal myectomy. It is considered the gold standard for treatment of the basal variant of hypertrophic cardiomyopathy. It has several advantages over alcohol septal ablation, such as the immediate relief of the obstruction and the ability to reduce the septal thickness significantly, to eliminate the potential for midventricular obstruction, and to rule out any other etiologies of left ventricular outflow tract obstruction, which include the presence of abnormalities in the mitral valve subvalvular apparatus such as the presence of anomalous chords, which occured in the current case, and anomalous papillary muscles. In experienced hands, the technique is safe and is associated with excellent outcomes with improved quality of life and potential for survival benefit.

2011 ◽  
Vol 9 (2) ◽  
pp. 108 ◽  
Author(s):  
Constantinos O’Mahony ◽  
Saidi A Mohiddin ◽  
Charles Knight ◽  
◽  
◽  
...  

Hypertrophic cardiomyopathy (HCM) is an inherited myocardial disorder characterised by left ventricular hypertrophy. A subgroup of patients develops limiting symptoms in association with left ventricular outflow tract obstruction (LVOTO). Current international guidelines recommend that symptomatic patients are initially treated by alleviating exacerbating factors and negatively inotropic medication. Drug-refractory symptoms require a comprehensive evaluation of the mechanism of LVOTO and review by a multidisciplinary team to consider the relative merits of myectomy, alcohol septal ablation (ASA) and pacing. This article provides a brief overview of HCM and the pathophysiology of LVOTO, and reviews the use of ASA in patients with drug-refractory symptoms secondary to LVOTO.


Author(s):  
B.M. Todurov ◽  
◽  
G.I. Kovtun ◽  
A.V. Khokhlov ◽  
O.V. Pantazi ◽  
...  

Hypertrophic obstructive cardiomyopathy іs a relatively common condition and one of the most common causes of sudden cardiac death in young age. One of the options for the surgical treatment of this pathology is septal myoectomy, which has been the gold standard for decades. However, despite this, surgical treatment is intended for young patients with a low risk of postoperative complications, while patients with concomitant diseases and a higher surgical risk require alternative treatment. Today, alcohol septal ablation is considered an effective, minimally invasive method for treating hypertrophic obstructive cardiomyopathy in patients with a left ventricular outflow tract gradient ≥ 50 mm Hg. The article presents the experience of using alcohol septal ablation in 57 patients with obstruction of the left ventricular outflow tract. Key words: alcoholic septal ablation, hypertrophic cardiomyopathy, left ventricular outflow tract obstruction.


Cardiology ◽  
2017 ◽  
Vol 137 (1) ◽  
pp. 58-61 ◽  
Author(s):  
Felix Thomas ◽  
Florian Rader ◽  
Robert J. Siegel

Objectives: Current nonpharmacological therapies for symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM), including septal myectomy and alcohol septal ablation (ASA), carry significant risks for serious cardiac conduction abnormalities. We present a review of the currently available published data regarding the novel use of the relatively low-risk MitraClip® system in the treatment of symptomatic patients. Methods: Data were collected from 4 separate studies on the use of the MitraClip on 15 symptomatic HOCM patients with systolic anterior motion (SAM) of the mitral valve apparatus. Information regarding the degree of mitral regurgitation (MR), left-ventricular outflow tract (LVOT) gradient, and NYHA class was consolidated. Results: After MitraClip treatment, all patients had a resolution of SAM, a reduction in MR, and a reduction in the LVOT gradient from a mean of 75.8 ± 39.7 to 11.0 ± 5.6 mm Hg. Nearly all patients demonstrated improvements in symptoms by either new NYHA class designations or improved exercise tolerance. The procedure was not associated with conduction abnormalities or arrhythmias. Conclusion: MitraClip therapy may be a safe and effective treatment for symptomatic HOCM patients; it can help to avoid the potential risks associated with alternative therapies in high-risk surgical patients.


ESC CardioMed ◽  
2018 ◽  
pp. 1459-1462
Author(s):  
Steve R. Ommen

Left ventricular outflow tract obstruction in hypertrophic cardiomyopathy can result in considerable symptoms. While pharmacological therapies are the first-line treatment for most patients, there are invasive therapies that have shown excellent success in relieving these drug-refractory symptoms. Surgical septal myectomy and percutaneous alcohol septal ablation, each with relative merits and risks, offer high success rates when performed in the context of specialized hypertrophic cardiomyopathy programmes.


2020 ◽  
Vol 31 (2) ◽  
pp. 158-165
Author(s):  
Alexander V Afanasyev ◽  
Alexander V Bogachev-Prokophiev ◽  
Maxim G Kashtanov ◽  
Dmitriy A Astapov ◽  
Anton S Zalesov ◽  
...  

Abstract OBJECTIVES There is very little evidence comparing the safety and efficacy of alcohol septal ablation versus septal myectomy for a septal reduction in patients with hypertrophic obstructive cardiomyopathy. This study aimed to compare the immediate and long-term outcomes of these procedures. METHODS Following propensity score matching, we retrospectively analysed outcomes in 105 patients who underwent myectomy and 105 who underwent septal ablation between 2011 and 2017 at 2 reference centres. RESULTS The mean age was 51.9 ± 14.3 and 52.2 ± 14.3 years in the myectomy and ablation groups, respectively (P = 0.855), and postoperative left ventricular outflow tract gradients were 13 (10–19) mmHg vs 16 (12–26) mmHg; P = 0.025. The 1-year prevalence of the New York Heart Association class III–IV was higher in the ablation group (none vs 6.4%; P = 0.041). The 5-year overall survival rate [96.8% (86.3–99.3) after myectomy and 93.5% (85.9–97.1) after ablation; P = 0.103] and cumulative incidence of sudden cardiac death [0% and 1.9% (0.5–7.5), respectively P = 0.797] did not differ between the groups. The cumulative reoperation rate within 5 years was lower after myectomy than after ablation [2.0% (0.5–7.6) vs 14.6% (8.6–24.1); P = 0.003]. Ablation was associated with a higher reoperation risk (subdistributional hazard ratio = 5.9; 95% confidence interval 1.3–26.3, P = 0.020). At follow-up, left ventricular outflow tract gradient [16 (11–20) vs 23 (15–59) mmHg; P < 0.001] and prevalence of 2+ mitral regurgitation (1.1% vs 10.6%; P = 0.016) were lower after myectomy than after ablation. CONCLUSIONS Both procedures improved functional capacity; however, myectomy better-resolved classes III–IV of heart failure. Septal ablation was associated with higher reoperation rates. Myectomy demonstrated benefits in gradient relief and mitral regurgitation elimination. The results suggest that decreasing rates of myectomy procedures need to be investigated and reconsidered.


2020 ◽  
Vol 7 (3) ◽  
pp. 655
Author(s):  
Vedanth Gopalan ◽  
Pavaneel Bhandari ◽  
Anant A. Takalkar

Background: Hypertrophic cardiomyopathy is highly heterogeneous with a diverse anatomy, pathophysiology, and clinical course. It is obstruction to left ventricular outflow that has become the major hallmark of the disease. Septal myectomy has been the gold standard treatment for the relief of left ventricular outflow tract obstruction and cardiac symptoms in both adults and children with obstructive hypertrophic cardiomyopathy. Objective of the study was to evaluate effect of Myomectomy and its impact on survival for a period of one year.Methods: The study design is a retrospective record based observational study. Data was retrieved from previous records both electronic as well as manual records of all the patients who underwent myectomy with or without concomitant procedures such as mitral valve replacement or aortic valve replacement or coronary artery bypass surgery during 2014 to 2018.Results: Majority of the patients 11 (52.4%) in fourth decade i.e. 40-59 years age group. majority were males i.e. 16 (76.2%) and remaining 5 i.e. 23.8% were females. Male to female ratio was 3.2:1. Dyspnoea was present 81% and chest pain in 76.2%. Preoperative LVOT gradient was 86.86±20.33 and post-operative gradient was 23.47±20.49.Conclusions: Operative techniques have evolved from simple myotomy to the present method of extended septal myectomy which can be done in all adult cases of hypertrophic obstructive cardiomyopathy. 


Author(s):  
Hao Cui ◽  
Xi Wu ◽  
Shuiyun Wang ◽  
Bing Tang ◽  
Changsheng Zhu ◽  
...  

AbstractBackground:NT-proBNP level is a predictive factor in hypertrophic cardiomyopathy patients, in which left ventricular outflow tract obstruction contributes to an increased NT-proBNP level. However, studies regarding the influence of septal myectomy on NT-proBNP level in hypertrophic obstructive cardiomyopathy are lacking.Methods:One hundred and eighty-two patients who underwent septal myectomy in Fuwai Hospital from May 2011 to March 2016 and were included in the present study. Preoperative and follow-up NT-proBNP was retrospectively collected. Correlation analysis was performed to determine the factors affecting preoperative NT-proBNP and postoperative decrease.Results:The cohort had a median age of 46.2 [IQR: 36.5–53.1] years, and 106 (58.2%) patients were male. NT-proBNP decreased to 816.5 [IQR: 400.3–1661.8] pg/mL from preoperative 1732.4 [IQR: 819–3296.5] pg/mL (p<0.001). Baseline NT-proBNP was correlated with gender (p<0.001), maximum septal thickness (p<0.001), and resting pressure gradient (p=0.006). The extent of NT-proBNP decrease was positively correlated with age (p<0.001), baseline NT-proBNP (p<0.001), follow-up time (p=0.020), and enlargement of the ascending aorta (p=0.019). NT-proBNP exhibited a persistent decrease after myectomy.Conclusions:Myectomy significantly reduced NT-proBNP level in hypertrophic obstructive cardiomyopathy patients, in which a time-dependent manner of decrease indicated myocardial remodeling of the heart after myectomy.


2021 ◽  

Hypertrophic obstructive cardiomyopathy is the most common inherited cardiomyopathy. Septal myectomy is a low-risk operation and remains the first septal reduction therapeutic option. We present a patient with hypertrophic obstructive cardiomyopathy requiring extended septal myectomy and concomitant left ventricular outflow tract intervention. In addition to septal reduction therapy, this patient also underwent anterior mitral valve plication, trigonal release, and secondary chordal division to relieve the obstruction. A tailored approach to hypertrophic obstructive cardiomyopathy with a comprehensive left ventricular outflow tract intervention is necessary to ensure the best hemodynamic outcome. Preoperative heart failure and recurrent syncope fully resolved after this intervention.


2013 ◽  
Vol 59 (6) ◽  
pp. 285-288
Author(s):  
Blesneac Cristina ◽  
Benedek Theodora ◽  
Togănel Rodica ◽  
Benedek I

Abstract Background: Hypertrophic cardiomyopathy, one of the most common inherited cardiomyopathies, is a heterogeneous disease resulting from sarcomeric protein mutations, with an incidence in the adult population of 1:500. Current information on the epidemiology and outcomes of this disease in children is limited. Methods: Thirty-four children diagnosed with hypertrophic cardiomyopathy in the Pediatric Cardiology Department from Tîrgu Mureș were evaluated concerning familial and personal history, clinical, paraclinical and therapeutic aspects. Hypertrophic cardiomyopathy was defined by the presence of a hypertrophied, non-dilated ventricle, in the absence of a cardiac or systemic disease that could produce ventricular hypertrophy. Results: The youngest diagnosed child was a neonate, a total of 10 patients being diagnosed until 1 year of age. In 6 cases a positive familial history was found. Noonan syndrome was found in 2 cases. Only 21 patients were symptomatic, the predominant symptoms being shortness of breath on exertion with exercise limitations. Left ventricular outflow tract obstruction was present in 21 cases (61.7%). Twenty-four patients were on β-blocking therapy, while 4 patients underwent septal myectomy. Conclusions: Hypertrophic cardiomyopathy is a heterogeneous disorder in terms of evolution, age of onset, type and extent of hypertrophy, and the risk of sudden death. It can affect children of any age. There is a need for a complex evaluation, including familial and personal anamnesis, clinical examination, electrocardiogram and echocardiography of all patients. It is highly important to develop screening strategies, including genetic testing, for an early diagnosis, especially in asymptomatic patients with a positive familial background


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