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Cardiomyopathy
Published in Charles Theisler, Adjuvant Medical Care, 2023
Heart conditions such as tachycardia or high blood pressure, viral infections, nutritional (e.g., vitamin B1) deficiencies, and certain diseases are typical causes of cardiomyopathy. Some individuals with cardiomyopathy have no signs or symptoms and need no treatment.1 For others, symptoms of heart failure develop quickly, are severe, and significant complications can occur. Symptomatic cardiomyopathy is a serious disease associated with high mortality and morbidity. Treatment in asymptomatic individuals with hypertrophic cardiomyopathy is controversial, and no conclusive evidence has been found that medical therapy is beneficial.2
Hypertrophic Cardiomyopathy
Published in Takahiro Shiota, 3D Echocardiography, 2020
Marta Sitges, Filip Loncaric, Takahiro Shiota
Diagnosis of hypertrophic cardiomyopathy is now mainly based on cardiac imaging methods, including 2D echocardiography, magnetic resonance (MR), and multislice computed tomography (CT) scanning. LV thickening is associated with a nondilated cavity with hyperdynamic motion and, usually, systolic chamber obliteration. Typically, the diagnosis is made on the presence of a maximal LV wall thickness of at least 15 mm in the absence of any other disease capable of inducing such a degree of LV hypertrophy noted by 2D echocardiography.1
Anesthetic Management for Surgical Myectomy in Hypertrophic Cardiomyopathy
Published in Srilakshmi M. Adhyapak, V. Rao Parachuri, Hypertrophic Cardiomyopathy, 2020
Heather K. Hayanga, Jeremiah W. Hayanga, Joseph McGuire, Vinay Badhwar
Hypertrophic cardiomyopathy is a common genetic cardiovascular disorder. Surgical repair poses unique challenges for the cardiac anesthesiologist but with careful and thoughtful consideration of the prevailing physiologic milieu, a safe and reproducible algorithm of care may be achieved utilizing TEE and select pharmacologic agents to ensure safe outcomes.
A special case of hypertrophic cardiomyopathy with a differential diagnosis of isolated cardiac amyloidosis or junctophilin type 2 associated cardiomyopathy
Published in Acta Clinica Belgica, 2021
Sévérine De Bruijn, Xavier Galloo, Gilles De Keulenaer, Edgard A. Prihadi, Christiane Brands, Mark Helbert
As both ECG and echocardiography were suggestive for infiltrative pathology, further cardiac investigations were performed. A 24h-holter revealed no arrhythmias. Gadolinium-enhanced conventional inversion-recovery cardiac MRI confirmed concentric hypertrophy of the LV as well as of the right ventricle. Moreover, contrast capture appeared to be most prominent in the septal and basal regions of the LV myocardium, suggestive of an infiltrative cardiomyopathy, more specific with cardiac amyloidosis (Figure 2). Further work-up of cardiac amyloidosis was carried out through haemato-flowcytometry, serum protein electrophoresis and immunofixation, urine biochemistry, measurement of free light chains in serum and urine, and abdominal ultrasound, which all were normal. Congo red staining was performed on prostate tissue, which had been obtained during prostate surgery 10 years before, but this did not show amyloid deposits. Finally, nuclear imaging with technetium 99mTc-methylene diphosphonate was carried out which demonstrated heavy tracer fixation in the myocardium strongly suggestive of cardiac ATTR (Figure 3). To confirm the diagnosis with histological evidence right heart catheterization with endomyocardial biopsy was proposed, but unfortunately refused by the patient. Since the advanced age of the patient and low probability of therapeutical implications of making a distinction between CA and another form of hypertrophic cardiomyopathy, we refrained from invasive diagnostic tests.
Importance of cardiovascular examination in patients with multiple lentigines: two cases of LEOPARD syndrome with hypertrophic cardiomyopathy
Published in Acta Clinica Belgica, 2019
Tomas Jurko, Alexander Jurko, Jana Krsiakova, Alexander Jurko, Milan Minarik, Michal Mestanik
Both our patients had hypertrophic cardiomyopathy. Early diagnosis of hypertrophic cardiomyopathy in children and adolescents is of great importance due to risk of sudden death [11]. The second patient underwent surgery for moderate obstruction of the left ventricular outflow tract and aortic valve repair. Postoperative follow-up was uneventful and the results of surgical repair were excellent. All patients with LEOPARD syndrome should undergo periodic cardiac assessment with electrocardiographic examination, echocardiography, computed tomography scan or magnetic resonance imaging since heart conduction defects and cardiomyopathy tend to be progressive [5]. New progressive diagnostic methods such as high-resolution multi-slice CT could help to choose the most appropriate management in these patients [12,13].
Pharmacological and non-pharmacological treatment of obstructive hypertrophic cardiomyopathy
Published in Expert Review of Cardiovascular Therapy, 2018
Luis F. Hidalgo, Srihari S. Naidu, Wilbert S. Aronow
Patients with hypertrophic cardiomyopathy can present with chest pain, dyspnea, syncope, palpitations, sudden cardiac death, or be asymptomatic [1,4]. Left ventricular outflow obstruction is not the sole etiology of these symptoms. Indeed, other related anatomic variables include microvascular coronary disease, decreased stroke volume due to smaller cavity and decreased compliance of the myocardium (diastolic dysfunction), mitral regurgitation, autonomic dysfunction with inability to increase systolic blood pressure during exercise, atrial and ventricular arrhythmias, and secondary pulmonary hypertension [5]. Many of these features can be present in the patient at the same time, leading to reduced cardiac output and hypervolemia over time. However it is important to identify and prioritize the main pathophysiologic processes causing the symptoms, in order to tailor therapy. In most cases, diastolic dysfunction predates the other anatomic variables by many years [6], if not decades, but the development of obstruction often triggers more rapid deterioration through a more significant drop in cardiac output, worsening diastolic dysfunction, mitral regurgitation, arrhythmias, and pulmonary hypertension [7]. It is for this reason that relief of obstruction has been targeted as a pivotal step in the modification of this natural history.