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Hypertrophic cardiomyopathy anesthesia

Roman N Fedosiuk, Liliia O Shchupachynska
The article is based on the case analysis of a sudden and unexpected intraoperative death of a 51-year-old female patient with hypertrophic cardiomyopathy, who was undergoing a non-cardiac operation in a non-cardiosurgery clinic, from acute precipitation of left ventricular outflow tract obstruction provoked by surgery and anesthesia. It emphasizes the importance of raising non-cardiac anesthesiologists' awareness of the issue and having clear standards of pre-operative evaluation and perioperative management of patients with hypertrophic cardiomyopathy in order to avoid fatal medical errors...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
Rajnish Kumar, Bibha Kumari
Hypertrophic cardiomyopathy (HCM) is a genetic myocardial disease usually characterized by asymmetric ventricular septal hypertrophy. HCM is an important cause of sudden cardiac death in adolescents and young adults. We are presenting a case report, ten years boy came in emergency with sudden loss of consciousness (witness cardiac arrest). Child was revived after cardiopulmonary resuscitation and send to coronary care unit. Echocardiography findings were suggestive of HCM. There was history of sudden death of her mother and maternal uncle...
January 2018: Anesthesia, Essays and Researches
Tomoyuki Suzuki, Masaki Hata, Kazuhiro Yamaya, Takeshi Saitou, Fumiya Haba, Masahiro Matsuno
Emergent ascending aortic replacement and extended myectomy were performed in a woman with acute aortic dissection who was aged 63 years. Preoperative transthoracic echocardiography performed in the intensive care unit showed only slight left ventricular outflow tract (LVOT) obstruction, but intraoperative transesophageal echocardiography after induction of anesthesia revealed pericardial effusion, systolic anterior motion(SAM), and associated mitral regurgitation(MR). Perioperative SAM and MR are sometimes facilitated under various hemodynamic conditions, but in this case, the left ventricular wall was thick and LVOT appeared to be obstructive by a hypertrophied septum...
December 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
Alana Kirkwood, Grant Stuart, Louise Harding
BACKGROUND: Donohue syndrome is a rare autosomal recessive disorder of insulin resistance, causing a functional defect in insulin receptor function, and affecting the ability of the insulin to bind the receptor. Features include severe hyperinsulinism and fasting hypoglycemia, along with severe failure to thrive despite feeding. An accelerated fasting state results in muscle wasting, decreased subcutaneous fat, and an excess of thick skin. A reduced thoracic diameter is accentuated by increased abdominal distension, which impacts on respiratory reserve...
January 2018: Paediatric Anaesthesia
Duc H Do, Jason Bradfield, Olujimi A Ajijola, Marmar Vaseghi, John Le, Siamak Rahman, Aman Mahajan, Akihiko Nogami, Noel G Boyle, Kalyanam Shivkumar
BACKGROUND: Novel therapies aimed at modulating the autonomic nervous system, including thoracic epidural anesthesia (TEA), have been shown in small case series to be beneficial in treating medically refractory ventricular tachycardia (VT) storm. However, it is not clear when these options should be considered. We reviewed a multicenter experience with TEA in the management of VT storm to determine its optimal therapeutic use. METHODS AND RESULTS: Data for 11 patients in whom TEA was instituted for VT storm between July 2005 and March 2016 were reviewed to determine the clinical characteristics, outcomes, and role in management...
October 27, 2017: Journal of the American Heart Association
Maria Enrica Antoniucci, Christian Colizzi, Gabriella Arlotta, Maria Calabrese, Michele Corrado, Sergio Guarneri, Lorenzo Martinelli, Andrea Scapigliati, Roberto Zamparelli, Franco Cavaliere
INTRODUCTION: Dynamic obstruction of right ventricle outflow tract (RVOTO) is a rare condition that may acutely cause severe heart failure. It has been reported in some hypertrophic cardiomyopathies, after lung transplantation, and in some cases of hemodynamic instability after cardiopulmonary bypass. PRESENTATION OF CASE: We report the case of a 71-year-old man who developed severe hypotension during the induction of general anesthesia for surgical coronary revascularization...
2017: International Journal of Surgery Case Reports
Joshua A Stern, Svetlana Markova, Yu Ueda, Jae B Kim, Peter J Pascoe, Marc J Evanchik, Eric M Green, Samantha P Harris
Hypertrophic cardiomyopathy (HCM) is an inherited disease of the heart muscle characterized by otherwise unexplained thickening of the left ventricle. Left ventricular outflow tract (LVOT) obstruction is present in approximately two-thirds of patients and substantially increases the risk of disease complications. Invasive treatment with septal myectomy or alcohol septal ablation can improve symptoms and functional status, but currently available drugs for reducing obstruction have pleiotropic effects and variable therapeutic responses...
2016: PloS One
Wei-Wen Lim, Mathias Baumert, Melissa Neo, Pawel Kuklik, Anand N Ganesan, Dennis H Lau, Tatiana Tsoutsman, Christopher Semsarian, Prashanthan Sanders, David A Saint
Hypertrophic cardiomyopathy (HCM) is a common heritable cardiac disorder with diverse clinical outcomes including sudden death, heart failure, and stroke. Depressed heart rate variability (HRV), a measure of cardiac autonomic regulation, has been shown to predict mortality in patients with cardiovascular disease. Cardiac autonomic remodelling in animal models of HCM are not well characterised. This study analysed Gly203Ser cardiac troponin-I transgenic (TG) male mice previously demonstrated to develop hallmarks of HCM by age 21 weeks...
January 2016: Clinical and Experimental Pharmacology & Physiology
Yuri Tsukano, Michiko Sugita, Yoshihiro Ikuta, Tatsuo Yamamoto
Combined spinal-epidural anesthesia (CSEA) was given to a 27-year-old woman with hypertrophic obstructive cardiomyopathy (HOCM) for a selective cesarean section. After the injection of uterotonic drug via uterine muscle and a vein after delivery, the patient developed dyspnea, tachycardia, ST-change on elecrocardiogram and hypotension. It is important in HOCM patients to control heart rate and left ventricular contractile force. We started to infuse beta-blocker (landiolol, 10 μg x kg(-1) x min(-1)) and improved these symptoms of the patient...
June 2015: Masui. the Japanese Journal of Anesthesiology
E Ashikhmina, M K Farber, K A Mizuguchi
BACKGROUND: Advances in understanding the pathogenesis, diagnosis and management of hypertrophic cardiomyopathy have resulted in increased longevity and a better quality of life of affected patients considering pregnancy. Several case series which focused predominantly on obstetric details have reported generally good outcomes. However, there remains a paucity of data on the specifics of obstetric anesthesia in women with hypertrophic cardiomyopathy. METHODS: After Institutional Review Board approval, we reviewed antepartum transthoracic echocardiograms, cardiology, obstetric, anesthetic, and nursing labor records with a focus on anesthesia for labor and delivery and early postpartum complications in patients with hypertrophic cardiomyopathy who delivered between January 1993 and December 2013...
November 2015: International Journal of Obstetric Anesthesia
Kotaro Hori, Tadashi Matsuura, Takashi Mori, Kiyonobu Nishikawa
BACKGROUND: Physician-performed transthoracic echocardiography (TTE) is still seldom used during anesthesia. Despite its various advantages, there are only a few reports of intraoperative TTE. We report 3 cases in which intraoperative TTE was successfully used. CASE PRESENTATION: A 75-year-old woman (Case 1) was scheduled for a posterior spinal fusion. When the wound was being closed, systolic blood pressure suddenly dropped to 30 mmHg. TTE revealed hypokinesis in the antero-septal region...
2015: BMC Anesthesiology
Yoshihisa Fujita, Nobuyuki Kagiyama, Yuka Sakuta, Masatsugu Tsuge
BACKGROUND: Perioperative dynamic left ventricular outflow obstruction associated with systolic anterior motion of the mitral valve is well recognized as a cause for unexplained sudden hypotension in perioperative settings, even without underlying heart diseases such as hypertrophic obstructive cardiomyopathy. We treated a patient who experienced sudden hypoxemia without severe hypotension during emergence from anesthesia after an uneventful laparoscopic cholecystectomy. CASE PRESENTATION: A 65-year-old female patient with a history of hypertension presented a sudden decrease in oxygen saturation to 80% after an uneventful cholecystectomy...
April 16, 2015: BMC Anesthesiology
Humphrey Lam, Shannon Dare, Thanh Nguyen, Thomas Austin
Cardiofaciocutaneous syndrome is a rare syndrome that is characterized by distinct craniofacial features, cardiac abnormalities, and multiple organ involvement. Patients may present with pulmonary stenosis, hypertrophic cardiomyopathy, micrognathia, a short neck, laryngomalacia, and tracheomalacia; all of which may significantly impact the perioperative course of these patients. We describe a 6-year-old child with cardiofaciocutaneous syndrome presenting for an orthopedic procedure. He had an uneventful perioperative and postoperative course...
April 1, 2015: A & A Case Reports
Kristin Moreth, Luciana Caminha Afonso, Helmut Fuchs, Valérie Gailus-Durner, Hugo A Katus, Raffi Bekeredjian, Lorenz Lehman, Martin Hrabě de Angelis
Consistent protocols for the assessment of diastolic and systolic cardiac function to assure the comparability of existing data on preclinical models are missing. Calcineurin transgene (CN) mice are a preclinical model for hypertrophic and failing hearts. We aimed at evaluating left and right ventricular structural and functional remodeling in CN hearts with an optimized phenotyping protocol. We developed a protocol using techniques and indices comparable to those from human diagnostics for comprehensive in vivo cardiac screening using high-frequency echocardiography, Doppler, electrocardiography and cardiac magnetic resonance (CMR) techniques...
April 2015: International Journal of Cardiovascular Imaging
Maythem Saeed, Steven W Hetts, Robert Jablonowski, Mark W Wilson
Myocardial pathologies are major causes of morbidity and mortality worldwide. Early detection of loss of cellular integrity and expansion in extracellular volume (ECV) in myocardium is critical to initiate effective treatment. The three compartments in healthy myocardium are: intravascular (approximately 10% of tissue volume), interstitium (approximately 15%) and intracellular (approximately 75%). Myocardial cells, fibroblasts and vascular endothelial/smooth muscle cells represent intracellular compartment and the main proteins in the interstitium are types I/III collagens...
November 26, 2014: World Journal of Cardiology
Praveen Kerala Varma, Suneel Puthuvassery Raman, Praveen Kumar Neema
Hypertrophic cardiomyopathy (HCM) poses many unique challenges regarding the conduct of anesthesia and surgery. Adequate preload, control of sympathetic stimulation, heart rate, and increased afterload are required to decrease the left ventricular outflow tract obstruction. Comprehensive intraoperative transesophageal echocardiography (TEE) examination confirms the diagnosis, elucidates the pathophysiology, and identifies the various anomalies of mitral valve apparatus and allows assessment of the adequacy of surgery...
July 2014: Annals of Cardiac Anaesthesia
Takashi Kondo, Shinji Kusunoki, Masahiko Kuroda, Masashi Kawamoto
Liver transplantation with hypertrophic obstructive cardiomyopathy is associated with acute hemodynamic changes, which can exacerbate left ventricular outflow tract obstruction during surgery. Therefore, selection of general anesthetic agents is important, as most can result in hemodynamic instability by reducing systemic vascular resistance and blood pressure. We report successful anesthetic management in a case of living donor liver transplantation with hypertrophic obstructive cardiomyopathy using ketamine, propofol, and fentanyl to avoid vasodilation by anesthetic agents...
December 2013: Hiroshima Journal of Medical Sciences
Teruya Nakamura, Naosumi Sekiya, Taro Nakazato, Yoshiki Sawa
Systolic anterior motion of the mitral valve is usually associated with hypertrophic obstructive cardiomyopathy that results in left ventricular outflow tract obstruction. This is sometimes treated by volume loading or negative inotropics such as beta blockers. We report a rare case of dynamic systolic anterior motion of the mitral valve, in which a beta blocker did not diminish but anesthetic agents masked intraoperatively. The patient underwent mitral valve replacement, and left ventricular outflow tract obstruction was successfully treated...
February 2014: Asian Cardiovascular & Thoracic Annals
Yousuke Imai, Yoshitsugu Yamada
Mitochondrial diseases are caused by a decrease in ATP production due to mutations of mitochondrial or mitochondria-related nuclear DNA. Their effects are likely to appear in tissues with a high energy demand, including skeletal muscle, nervous, and cardiovascular systems. Cardiac manifestations of mitochondrial diseases can be divided into cardiomyopathies, which are primarily hypertrophic and dilated cardiomyopathies, and electropathies, which are primarily conduction system disease and ventricular pre-excitation...
January 2014: Masui. the Japanese Journal of Anesthesiology
Takeshi Omae
Hypertrophic cardiomyopathy is a common inherited cardiovascular disease present in one in 500 of the general population. It is caused by more than 1,400 mutations in 11 or more genes encoding proteins of the cardiac sarcomere. In the absence of evidence of any other cardiac or systemic disease that could have resulted in the hypertrophic event, diagnosis of hypertrophic cardiomyopathy requires a hypertrophied non-dilated left ventricle. It is associated with a significant risk for anesthesia. During anesthesia in patients diagnosed with hypertrophic cardiomyopathy, it is essential to maintain relatively slow heart rate, prevent hypovolemia, maintain or increase systemic vascular resistance, and avoid propofol as the sole anesthetic agent...
January 2014: Masui. the Japanese Journal of Anesthesiology
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