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It is characterized by hypokinetic areas involving the free wall of the ventricle, with fibrofatty replacement of the myocardium, with associated arrhythmias often originating in the right ventricle. The nomenclature ARVD is currently thought to be inappropriate and misleading as ACM does not involve dysplasia of the ventricular wall.
Bernheim syndrome is believed to be the rightward shift of the ventricular septum compressing the right ventricle without causing pulmonary congestion. [3] This was first described by Hippolyte Bernheim in which he presented 10 patients with signs and symptoms of right sided heart failure whose postmortem autospy reveals a ventricular septum that invaded the right ventricle space. [1]
An important potential finding with echo is McConnell's sign, where only the RV apex wall contracts; [7] it is specific for right heart strain and typically indicates a large PE. [8] On an electrocardiogram (ECG), there are multiple ways RV strain can be demonstrated. A finding of S1Q3T3 [b] is an insensitive [10] sign of right heart strain. [11]
The pathophysiology of pulmonary heart disease (cor pulmonale) has always indicated that an increase in right ventricular afterload causes RV failure (pulmonary vasoconstriction, anatomic disruption/pulmonary vascular bed and increased blood viscosity are usually involved [1]), however most of the time, the right ventricle adjusts to an overload in chronic pressure.
This results in right atrial and right ventricular strain and dilation, right ventricular hypokinesis and marked diastolic dysfunction. Subacute effects of exercise include increased expression of cardiac fibrotic markers including TGF, fibronectin-1, collagens, MMP-2 and TIMP1.
Echocardiography can be used to directly visualise right ventricular wall thickness. The preferred technique is the trans-oesophageal approach giving a view of 4 chambers. The normal thickness of a right ventricular free wall ranges from 2-5 millimetres, with a value above 5 mm considered to be hypertrophic. [10]
When the right side of the heart is more underdeveloped than the left side, this is known as hypoplastic right heart syndrome. HRHS is known for the pulmonary valve, the tricuspid valve, right ventricle, and the pulmonary artery all failing to form properly. HRHS also causes the right ventricle to be a fair amount smaller than the left side. [3]
A ventricular outflow tract obstruction means there is a limitation in the blood flow out of either the right or left ventricles of the heart, depending on where the obstruction is. This can lead to cardiac hypertrophy , dilatation of the heart, and ultimately heart failure in some cases. [ 1 ]
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