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https://www.readbyqxmd.com/read/28793143/intraoperative-diagnosis-of-anderson-fabry-disease-in-patients-with-obstructive-hypertrophic-cardiomyopathy-undergoing-surgical-myectomy
#1
Franco Cecchi, Maria Iascone, Niccolò Maurizi, Laura Pezzoli, Irene Binaco, Elena Biagini, Maria Laura Fibbi, Iacopo Olivotto, Federico Pieruzzi, Ana Fruntelata, Lucian Dorobantu, Claudio Rapezzi, Paolo Ferrazzi
Importance: Diagnostic screening for Anderson-Fabry cardiomyopathy (AFC) is performed in the presence of specific clinical red flags in patients with hypertrophic cardiomyopathy (HCM) older than 25 years. However, left ventricular outflow tract obstruction (LVOTO) has been traditionally considered an exclusion criteria for AFC. Objective: To examine a series of patients diagnosed with HCM and severe basal LVOTO undergoing myectomy in whom the diagnosis of AFC was suspected by the cardiac surgeon intraoperatively and confirmed by histological and genetic examinations...
August 9, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28762919/-effectiveness-of-extended-myectomy-in-patients-with-hypertrophic-cardiomyopathy-with-midventricular-obstruction
#2
A V Bogachev-Prokophiev, S I Zheleznev, M S Fomenko, R M Sharifulin, A V Afanasyev, O Yu Malakhova, A M Karaskov
PURPOSE: to assess effectiveness and safety of extended myectomy performed in patients with hypertrophic obstructive cardiomyopathy (HOCM) with midventricular obstruction. MATERIAL AND METHODS: Between 2010 and 2013 185 HOCM patients were operated for left ventricular outflow tract (LVOT) obstruction. Among these patients 32 had midventricular obstruction. Their age was 22-74 (mean 51.9+/-14.2) years. Mean peak gradient across LVOT was 89.1+/-20.4 mm Hg, thickness of interventricular septum was 26...
May 2017: Kardiologiia
https://www.readbyqxmd.com/read/28728695/alcohol-septal-ablation-for-obstructive-hypertrophic-cardiomyopathy-a-word-of-balance
#3
REVIEW
Paul Sorajja
The management of drug-refractory symptoms of obstructive hypertrophic cardiomyopathy has long been debated and is primarily centered on the choice between surgical myectomy and alcohol septal ablation. Decision making in these patients requires consideration of procedural risk, expertise and efficacy, and the long-term impact on patients' survival. Although there have been numerous reports on these procedures that may help guide decision making, these data continue to be self-reported and voluntary. Greater insight into the real-world experience for these therapies and how they should be applied in practice would be gained from mandatory reporting, akin to other multidisciplinary, transcatheter-based therapies...
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28728694/alcohol-septal-ablation-for-obstructive-hypertrophic-cardiomyopathy-a-word-of%C3%A2-endorsement
#4
REVIEW
Max Liebregts, Pieter A Vriesendorp, Jurrien M Ten Berg
Twenty years after the introduction of alcohol septal ablation (ASA) for the treatment of obstructive hypertrophic cardiomyopathy, the arrhythmogenicity of the ablation scar appears to be overemphasized. When systematically reviewing all studies comparing ASA with myectomy with long-term follow-up, (aborted) sudden cardiac death and mortality rates were found to be similarly low. The focus should instead shift toward lowering the rate of reinterventions and pacemaker implantations following ASA because, in this area, ASA still seems inferior to myectomy...
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28681495/underappreciated-occurrence-of-discrete-subaortic-membranes-producing-left-ventricular-outflow-obstruction-in-hypertrophic-cardiomyopathy
#5
Muhil Kannappan, Barry J Maron, Hassan Rastegar, Natesa G Pandian, Martin S Maron, Ethan J Rowin
Subaortic obstruction due to systolic anterior motion (SAM) of the mitral valve with ventricular septal contact is a major cause of progressive heart failure symptoms in patients with hypertrophic cardiomyopathy (HCM). However, we have recently observed a unique, but not uncommon subgroup of HCM patients with outflow tract obstruction due only to discrete subaortic membrane or who have a membrane in addition to SAM-septal contact. HCM patients with subaortic membranes may be at increased risk for developing progressive heart failure symptoms...
July 6, 2017: Echocardiography
https://www.readbyqxmd.com/read/28658286/mybpc3-mutations-are-associated-with-a-reduced-super-relaxed-state-in-patients-with-hypertrophic-cardiomyopathy
#6
James W McNamara, Amy Li, Sean Lal, J Martijn Bos, Samantha P Harris, Jolanda van der Velden, Michael J Ackerman, Roger Cooke, Cristobal G Dos Remedios
The "super-relaxed state" (SRX) of myosin represents a 'reserve' of motors in the heart. Myosin heads in the SRX are bound to the thick filament and have a very low ATPase rate. Changes in the SRX are likely to modulate cardiac contractility. We previously demonstrated that the SRX is significantly reduced in mouse cardiomyocytes lacking cardiac myosin binding protein-C (cMyBP-C). Here, we report the effect of mutations in the cMyBP-C gene (MYBPC3) using samples from human patients with hypertrophic cardiomyopathy (HCM)...
2017: PloS One
https://www.readbyqxmd.com/read/28642712/investigations-into-the-sarcomeric-protein-and-ca-2-regulation-abnormalities-underlying-hypertrophic-cardiomyopathy-in-cats-felix-catus
#7
Andrew E Messer, Jasmine Chan, Alex Daley, O'Neal Copeland, Steven B Marston, David J Connolly
Hypertrophic cardiomyopathy (HCM) is the most common single gene inherited cardiomyopathy. In cats (Felix catus) HCM is even more prevalent and affects 16% of the outbred population and up to 26% in pedigree breeds such as Maine Coon and Ragdoll. Homozygous MYBPC3 mutations have been identified in these breeds but the mutations in other cats are unknown. At the clinical and physiological level feline HCM is closely analogous to human HCM but little is known about the primary causative mechanism. Most identified HCM causing mutations are in the genes coding for proteins of the sarcomere...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28609808/resection-of-subaortic-membrane-for-discrete-subaortic-stenosis
#8
Sachin Talwar, Abhishek Anand, Saurabh Kumar Gupta, Sivasubramanian Ramakrishnan, Shyam Sunder Kothari, Anita Saxena, Rajnish Juneja, Shiv Kumar Choudhary, Balram Airan
BACKGROUND: We reviewed the long-term results of surgery for discrete subaortic membrane (SubAM) from a single institute. METHODS: A retrospective review of medical records of all patients (n = 146) who underwent resection of a SubAM for discrete subaortic stenosis between 1990 and 2015 at the All India Institute of Medical Sciences, New Delhi, India was undertaken. RESULTS: Median age at surgery was 9.0 years (9 months-47 years). There was one early death...
July 2017: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/28575282/mitral-valve-repair-or-replacement-in-hypertrophic-obstructive-cardiomyopathy-a-prospective-randomized-study%C3%A2
#9
Alexander Bogachev-Prokophiev, Alexander Afanasyev, Sergey Zheleznev, Michael Fomenko, Ravil Sharifulin, Eugenie Kretov, Alexander Karaskov
OBJECTIVES: The optimal surgical strategy for concomitant mitral valve intervention during myectomy remains controversial. The purpose of this study was to compare the results of mitral valve replacement versus repair in patients with hypertrophic obstructive cardiomyopathy and severe mitral regurgitation. METHODS: Between 2010 and 2013, a total of 88 patients with hypertrophic obstructive cardiomyopathy and severe mitral regurgitation were randomly assigned to undergo either mitral valve replacement or repair in addition to extended myectomy...
May 29, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28510774/combined-surgery-for-simultaneous-treatment-of-congenital-ptosis-and-coexisting-strabismus
#10
Feng Zhou, Ming Ouyang, Dahui Ma, Guiqin Liu, Hongbo Cheng
PURPOSE: To evaluate the clinical outcomes of single-stage surgery for treatment of patients with congenital ptosis and coexisting strabismus. METHODS: A retrospective analysis was performed on 10 patients (17 eyes) with congenital ptosis and coexisting strabismus. Patients were treated with levator resection or frontalis suspension for ptosis and ocular muscle surgery for strabismus, performed as a single-stage procedure. Levator resection was performed in 9 patients (15 eyes) and frontalis muscle flap suspension was performed in 1 patient (2 eyes) with blepharophimosis...
May 15, 2017: Journal of Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/28510770/inferior-oblique-overaction-anterior-transposition-versus-myectomy
#11
Zhale Rajavi, Mohadeseh Feizi, Narges Behradfar, Mehdi Yaseri, Shima Sayanjali, Tahmine Motevaseli, Hamideh Sabbaghi, Mohammad Faghihi
PURPOSE: To compare the efficacy of inferior oblique myectomy and anterior transposition for correcting inferior oblique overaction (IOOA). METHODS: This retrospective study was conducted on 56 patients with IOOA who had either myectomy or anterior transposition of the inferior oblique muscle from 2010 to 2015. The authors compared preoperative and postoperative inferior oblique muscle function grading (-4 to +4) as the main outcome measure and vertical and horizontal deviation, dissociated vertical deviation (DVD), and A- and V-pattern between the two surgical groups as secondary outcomes...
July 1, 2017: Journal of Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/28483207/usefulness-of-preoperative-transforming-growth-factor-beta-to-predict-new-onset-atrial-fibrillation-after-surgical-ventricular-septal-myectomy-in-patients-with-obstructive-hypertrophic-cardiomyopathy
#12
Ying Guo, Xi Wu, Xinxin Zheng, Jie Lu, Shuiyun Wang, Xiaohong Huang
Postoperative atrial fibrillation (AF) occurs frequently after cardiac surgery and contributes significantly to mortality. Transforming growth factor-beta (TGF-β) is associated with postoperative AF after coronary artery bypass grafting and valve surgery. We performed a prospective study to evaluate the role of TGF-β as a predictor of AF after myectomy. A total of 109 consecutive obstructive hypertrophic cardiomyopathy patients without previous AF who underwent myectomy were identified. We measured plasma TGF-β levels before surgery, monitored heart rhythm until discharge, and followed patients for a mean of 36 ± 10 months...
July 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28476777/disease-stage-dependent-changes-in-cardiac-contractile-performance-and-oxygen-utilization-underlie-reduced-myocardial-efficiency-in-human-inherited-hypertrophic-cardiomyopathy
#13
Ahmet Güçlü, Paul Knaapen, Hendrik J Harms, Rahana Y Parbhudayal, Michelle Michels, Adriaan A Lammertsma, Albert C van Rossum, Tjeerd Germans, Jolanda van der Velden
BACKGROUND: Reduced myocardial efficiency represents a target for therapy in hypertrophic cardiomyopathy although therapeutic benefit may depend on disease stage. Here, we determined disease stage-dependent changes in myocardial efficiency and effects of myectomy surgery. METHODS AND RESULTS: Myocardial external efficiency (MEE) was determined in 27 asymptomatic mutation carriers (genotype positive/phenotype negative), 10 patients with hypertrophic obstructive cardiomyopathy (HOCM), 10 patients with aortic valve stenosis, and 14 healthy individuals using [(11)C]-acetate positron emission tomography and cardiovascular magnetic resonance imaging...
May 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28468134/use-of-acellular-dermal-matrix-in-treatment-of-congenital-muscular-torticollis-in-patients-over-eight-years-of-age
#14
Hyung Min Hahn, Kyung Hoon Cook, Il Jae Lee, Dong Ha Park, Myong Chul Park
BACKGROUND: Treatment for neglected or recurred congenital muscular torticollis should be differentiated from primary patients due to the long-standing adjacent tissue contracture. The aim of this study was to evaluate the effect of acellular dermal matrix (ADM) on surgery of recurred and neglected patients of congenital muscular torticollis. METHODS: Forty-nine patients were included in the study. All patients underwent resection at the distal end of the sternocleidomastoid muscle...
May 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28396501/mechanism-of-progressive-heart-failure-and-significance-of-pulmonary-hypertension-in-obstructive-hypertrophic-cardiomyopathy
#15
Michele Covella, Ethan J Rowin, Nicholas S Hill, Ioana R Preston, Alberto Milan, Alexander R Opotowsky, Barry J Maron, Martin S Maron, Bradley A Maron
BACKGROUND: There are limited data on the prevalence, pathophysiology, and management implications of pulmonary hypertension in patients with obstructive hypertrophic cardiomyopathy and advanced heart failure. METHODS AND RESULTS: To assess the clinical significance of measured cardiopulmonary hemodynamics in hypertrophic cardiomyopathy patients with heart failure, we retrospectively assessed right heart catheterization data in 162 consecutive patients with outflow tract gradients (median [interquartile range], 90 mm Hg [70-110 mm Hg]), 59±11 years old, and 49% men, predominately New York Heart Association class III/IV status...
April 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/28379420/improvements-in-left-ventricular-twist-mechanics-following-myectomy-for-hypertrophic-cardiomyopathy-with-mid-ventricular-obstruction
#16
Srilakshmi M Adhyapak, Prahlad G Menon, V Rao Parachuri
We aimed to evaluate left ventricle twist mechanics in mid-ventricular obstructive and apical type of hypertrophic cardiomyopathy and changes induced by myectomy. We studied 3 consecutive patients by cardiac magnetic resonance preoperatively and 6 weeks after myectomy. We calculated the apical and basal rotations at the base and apex respectively. All 3 patients underwent myectomy by the standard described technique. The basal rotations remained the same, while there was an improvement in the maximal apical rotation from 0...
March 31, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28363351/comparison-of-ventricular-septal-measurements-in-hypertrophic-cardiomyopathy-patients-who-underwent-surgical-myectomy-using-multimodality-imaging-and-implications-for-diagnosis-and-management
#17
COMPARATIVE STUDY
Dermot Phelan, Brett W Sperry, Paaladinesh Thavendiranathan, Patrick Collier, Zoran B Popović, Harry M Lever, Nicholas G Smedira, Milind Y Desai
Accurate and reproducible quantification of ventricular septal (VS) thickness in hypertrophic cardiomyopathy (HC) is essential for diagnosis, surgical planning, and risk stratification. We sought to compare VS thickness measurements using transthoracic echocardiography (TTE), transesophageal echocardiography (TEE), and cardiac magnetic resonance (CMR) in patients with HC. Prospectively reported measurements of VS thickness were compared using analysis of variance and Bland-Altman plots in 90 consecutive patients with HC who underwent a TTE, TEE, and CMR within 3 months...
May 15, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28343653/swallow-induced-syncope-and-carotid-sinus-hypersensitivity-coincident-or-associated-conditions
#18
Eed Abu Zaid, Moti Haim, Hilmi Alnsasra, Yuval Konstantino
Swallow induced syncope is a rare clinical condition which is thought to result from an abnormal vagal reflex leading to bradycardia and cerebral hypoperfusion. It mostly occurs in patients with organic or functional disorders of the esophagus, and often requires permanent pacemaker implantation, along with treatment of the underlying esophageal pathology. In the following case, we report of a 71-year-old male with achalasia post per oral endoscopic myectomy, who presented with syncope and documented AV-block while eating solid food...
July 2017: Journal of Electrocardiology
https://www.readbyqxmd.com/read/28337484/first-reported-case-of-per-anal-endoscopic-myectomy-paem-a-novel-endoscopic-technique-for-resection-of-lesions-with-severe-fibrosis-in-the-rectum
#19
David Ozzie Rahni, Takashi Toyonaga, Yoshiko Ohara, Francesco Lombardo, Shinichi Baba, Hiroshi Takihara, Shinwa Tanaka, Fumiaki Kawara, Takeshi Azuma
Background and study aims A 54-year-old man was diagnosed with a rectal tumor extending through the submucosal layer. The patient refused surgery and therefore endoscopic submucosal dissection (ESD) was pursued. The lesion exhibited the muscle retraction sign. After dissecting circumferentially around the fibrotic area by double tunneling method, a myotomy was performed through the internal circular muscle layer, creating a plane of dissection between the internal circular muscle layer and the external longitudinal muscle layer, and a myectomy was completed...
March 2017: Endoscopy International Open
https://www.readbyqxmd.com/read/28329292/surgical-septal-myectomy-or-alcohol-septal-ablation-which-approach-offers-better-outcomes-for-patients-with-hypertrophic-obstructive-cardiomyopathy
#20
Shi Sum Poon, Mark Field, Dhiraj Gupta, Duke Cameron
A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether surgical septal myectomy (SM) is more beneficial than alcohol septal ablation (ASA) in patients with hypertrophic obstructive cardiomyopathy. Altogether 218 articles were found using the reported search, of which 15 studies represented the best evidence to answer the clinical question. There were 14 observational studies and 1 meta-analysis study. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these articles are tabulated...
June 1, 2017: Interactive Cardiovascular and Thoracic Surgery
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