The patient was discharged home and subsequently died of a VF arrest while running to catch a bus. Autopsy showed septal hypertrophy consistent with HCM. HCM showing features of asymmetrical septal hypertrophy:. Special interests in diagnostic and procedural ultrasound, medical education, and ECG interpretation. This site uses Akismet to reduce spam.
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Mitral regurgitation may also be noted. Cardiac MRI plays a key role in risk stratification Negative prognostic indicators in high-risk patients include:. Treatment depends on the severity of the disease. The goal is to relieve symptoms and prevent sudden cardiac death in high-risk patients. The base of treatment is to control heart rate by avoiding extreme physical efforts and by giving medications e.
Other treatment options include septal myomectomy, septal ablation alcohol arterial embolization , and placement of an implantable cardioverter-defibrillator device. Hypertrophic cardiomyopathy was first described and originally termed idiopathic hypertrophic subaortic stenosis IHSS by Brent et al.
The term hypertrophic cardiomyopathy HCM came into common use in the latter half of the s replacing IHSS, which was considered an inappropriate characterization.
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This murmur can be distinguished from aortic stenosis, however, in that it becomes less intense with maneuvers that increase venous return or vascular resistance as with squatting or hand grip, and becomes more prominent and harsh with maneuvers that decrease ventricular filling as with the Valsalva maneuver or standing up from a squatting position.
In these instances, the practitioner should listen for a murmur of aortic insufficiency since this can be present as well. When severe outflow obstruction is present, S2 may be paradoxically split due to the prolonged outflow time from the obstruction to flow. Other physical exam findings that are consistent with hypertrophic cardiomyopathy include a prominent jugular a wave due to hypertrophy and poor right ventricle compliance. Due to hypertrophy, a heave may be present and the point of maximal impulse is often diffuse and displaced.
Carotid pulsation has a classic bifid pattern which is a rapid upstroke followed by a second peak. It should be noted that there are patients with genotype positive hypertrophic cardiomyopathy that have no or minimal physiologic expression of their disease.
These patients may have no abnormal physical exam findings although they do have the diagnosis of hypertrophic cardiomyopathy. Since there are patients with this disease process that have no physical manifestations of the disease, there is not a physical exam finding that confirms the diagnosis. Instead, exam findings of ventricular hypertrophy in the proper setting should lead a clinician to get imaging studies to aid in diagnosis.
Please see the section on imaging studies for further information about tests that are useful to order. Genetic testing may be useful to help identify patients that have mutations that portend a higher likelihood of sudden cardiac death. For example, beta myosin heavy chain mutations and troponin T mutations may be associated with higher rates of sudden death.
However, elderly patients identified with this disease often have minimal symptoms, even when outflow gradients are present. These patients commonly have mutations in cardiac myosin binding protein C and troponin I genes. Once a patient with hypertrophic cardiomyopathy has been identified it is important to test their first degree relatives for this disease. Two dimensional echocardiography is the diagnostic modality of choice for this disease.
It is widely available and efficient at diagnosing ventricular hypertrophy. Hypertrophic cardiomyopathy can present with just a genotype diagnosis and no initial evidence of hypertrophy on echocardiogram. In fact, hypertrophy is often first apparent during late adolescence. Hypertrophy can be diffuse or only be found in specific segments of the myocardium. If hypertrophy is present in only one area, it is found in decreasing order in the septum, apex, and the mid-ventricle respectively.
When echocardiography does not identify hypertrophy, magnetic resonance imaging MRI can be useful in identifying specific segments of hypertrophy that may be missed with the aforementioned modality. While electrocardiograms are often abnormal in patients with hypertrophic cardiomyopathy, there is no clear pattern that will aid in the diagnosis of this disease.
In fact, there is no association between voltage on electrocardiogram and degree of hypertrophy on echocardiograph. Management of hypertrophic cardiomyopathy is determined by symptom manifestation. In patients that are asymptomatic without concerning risk factors for sudden cardiac death there is no evidence that prophylactic therapy makes any difference in disease progression. However, there is minimal data to support this assertion.
The vast majority of patients do not have massive hypertrophy and management is targeted at control of symptoms of heart failure, ischemia, atrial fibrillation and prevention of sudden cardiac death.
Largely these are managed through medical therapy; however, in certain instances surgical intervention may be useful. Patients that present to the hospital with hypertrophic cardiomyopathy will likely be presenting due to a heart failure exacerbation. Since the majority of patients have diastolic heart failure, management is based on relieving outflow track obstruction when present , controlling hypertension and relieving congestion.
Symptom management has been best described by use of beta-blockade as well as non-dihydropyridines specifically Verapamil.
These medications slow heart rate leading to a prolongation in diastole and increased ventricular filling time. This in turn leads to greater cardiac output. Diuresis with loop diuretics is advisable in patients that have evidence of volume overload, but they should be used cautiously as to not decrease preload too dramatically.
These patients are also very sensitive to loss of their atrial kick, and may present with an exacerbation during an episode of atrial fibrillation. Patients with hypertrophic cardiomyopathy are prone to episodes of atrial fibrillation. Due to wall hypertrophy and their dependence on ventricular filling to get adequate cardiac output, a rapid ventricular rate can make patients quite symptomatic.
As in the general population, medical therapy to control the ventricular rate with beta-blockade or a non-dihydropyridine is the first line of therapy. If patients fail medical management and remain symptomatic atrioventricular AV node ablation and pacemaker implantation may be necessary to prevent episodes of tachycardia. Therefore, practitioners should have a very low threshold for initiating anticoagulation therapy. Practitioners should pay close attention to signs of volume overload.
They should monitor lung sounds for evidence of crackles indicating pulmonary edema. Furthermore, a thorough cardiac exam daily will help with management. Assessing degree of jugular venous distention and edema will help determine the extent of congestion that a patient is experiencing. Furthermore, cardiac auscultation should be used to ensure a patient is in sinus rhythm, or rate controlled if they are experiencing atrial fibrillation.
As with congestive heart failure exacerbations in patients without hypertrophic cardiomyopathy, response to treatment can be monitored by symptom resolution and improvement in physical exam findings of venous congestion.
There is no laboratory test or diagnostic study that needs to be followed on a regular basis to determine resolution of an exacerbation. However, in patients being treated with diuresis it may be wise to follow electrolyte levels and monitor kidney function to ensure patients are not pushed into a pre-renal state due to diuresis.
Medical management of heart failure symptoms should be initiated as outlined in the immediate management section. There is no evidence that beta-blockers or non dihydropyridines are more efficacious at treating symptoms.
It is often based on physician preference and monitoring symptom control in patients. If these medications do not relieve symptoms of heart failure or chest pain then disopyramide, an antiarrhythmic agent, may be considered.
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