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Myocardial Disease: Difference between revisions
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|bgcolor="F0F8FF"|'''''Myocardial Disease''''' | |||
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Overall, myocardial diseases can be subdivided into two types: '''''primary and secondary myocardial diseases'''''. Whereas the primary type most commonly has a genetic cause, secondary myocardial diseases are mostly acquired but may be precipitated by a genetic background. Overall, the dilated type is the most prevalent manifestation of cardiomyopathy, and may be induced by a multitude of precipitating factors, such as chronic ischemia or alcohol abuse. The most common primary myocardial disease is hypertrophic cardiomyopathy, which is associated with a wide variety of genetic abnormalities. In the light of their substantially larger prevalence, we will first describe the secondary myocardial diseases, after which the less frequently occurring primary myocardial diseases will be discussed. | |||
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==Myocardial Disease - Secondary== | ==Myocardial Disease - Secondary== | ||
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====Treatment==== | |||
Treatment focuses on avoiding complications. | Treatment focuses on avoiding complications. | ||
*Medication: | *Medication: | ||
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A two-layered structure of the myocardium, with a thin compacted epicardial band and a thick non-compacted endomyocardial part, overall resulting in a thickened myocardium. LVNC is diagnosed by the ratio between the maximal non-compacted and compacted myocardial layer thickness measured during end-systole. In adults, a non-compacted/compacted thickness ratio of =2 confirms the diagnosis, whereas a ratio of =1.4 is considered diagnostic in children. The non-compacted layer is further characterized by prominent trabeculations, with intertrabecular spaces in direct connection with the left ventricular cavity. The non-compacted regions are predominantly found in the lateral, apical or inferior wall of the left ventricle. Where normal myocardium, originally consisting of a sponge-like structure of myocardial fibres, shows a pattern of basal to apical, and epicardial to endocardial compaction of the myocardial fibres during the 5th to 8th week of embryonal development, non-compaction supposedly originates from halting of this process due to a genetic defect. | A two-layered structure of the myocardium, with a thin compacted epicardial band and a thick non-compacted endomyocardial part, overall resulting in a thickened myocardium. LVNC is diagnosed by the ratio between the maximal non-compacted and compacted myocardial layer thickness measured during end-systole. In adults, a non-compacted/compacted thickness ratio of =2 confirms the diagnosis, whereas a ratio of =1.4 is considered diagnostic in children. The non-compacted layer is further characterized by prominent trabeculations, with intertrabecular spaces in direct connection with the left ventricular cavity. The non-compacted regions are predominantly found in the lateral, apical or inferior wall of the left ventricle. Where normal myocardium, originally consisting of a sponge-like structure of myocardial fibres, shows a pattern of basal to apical, and epicardial to endocardial compaction of the myocardial fibres during the 5th to 8th week of embryonal development, non-compaction supposedly originates from halting of this process due to a genetic defect. | ||
====Clinical Presentation and Diagnosis==== | |||
Age of onset may be highly variable, with cyanosis, failure to thrive or dysmorphic features described in the neonatal period, to adult patients presenting with LV failure or ventricular arrhythmia. Possibly, sudden cardiac death entails one of the manifestations of LVNC, although evidence is only limited at this moment. Owing to technical advances in the field of echocardiography, predominantly an increase in image resolution, LVNC has only recently been recognized as an independent entity in the spectrum of cardiomyopathies, which supposedly has frequently been misdiagnosed as HCM in the past. | Age of onset may be highly variable, with cyanosis, failure to thrive or dysmorphic features described in the neonatal period, to adult patients presenting with LV failure or ventricular arrhythmia. Possibly, sudden cardiac death entails one of the manifestations of LVNC, although evidence is only limited at this moment. Owing to technical advances in the field of echocardiography, predominantly an increase in image resolution, LVNC has only recently been recognized as an independent entity in the spectrum of cardiomyopathies, which supposedly has frequently been misdiagnosed as HCM in the past. | ||
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Considering the genetic background of LVNC, inheriting most commonly autosomal dominant, echocardiographic evaluation of family members of LVNC patients is pertinent. | Considering the genetic background of LVNC, inheriting most commonly autosomal dominant, echocardiographic evaluation of family members of LVNC patients is pertinent. | ||
====Treatment and Prognosis==== | |||
Treatment strategies are mainly extrapolated from other cardiomyopathies: standard heart failure therapy in case of LV dysfunction, beta-blockade and/or amiodarone in non-sustained VT in the absence of LV dysfunction, and ICD placement indicated in patients with LVEF <35%, sustained VT or recurrent unexplained syncope. Routine anticoagulation in not indicated, but anticoagulation may be instituted in case of ventricular dilatation or systolic LV function impairment. | Treatment strategies are mainly extrapolated from other cardiomyopathies: standard heart failure therapy in case of LV dysfunction, beta-blockade and/or amiodarone in non-sustained VT in the absence of LV dysfunction, and ICD placement indicated in patients with LVEF <35%, sustained VT or recurrent unexplained syncope. Routine anticoagulation in not indicated, but anticoagulation may be instituted in case of ventricular dilatation or systolic LV function impairment. | ||
The prognosis of LVNC has currently not yet been defined. One small report indicates a high mortality risk, owing to a high prevalence of sudden death, but community-based populations may well show a different prognosis as many patients with LVNC are non-symptomatic at the initial diagnosis. | The prognosis of LVNC has currently not yet been defined. One small report indicates a high mortality risk, owing to a high prevalence of sudden death, but community-based populations may well show a different prognosis as many patients with LVNC are non-symptomatic at the initial diagnosis. | ||