From sudden stroke to heart transplant: unmasking restrictive cardiomyopathy in an adolescent: a case report

Figures

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  Figure 1. (A) Digital subtraction angiography, lateral projection, demonstrates abrupt occlusion of the M1 segment of the right middle cerebral artery (marked with *). (B) Non-contrast axial head computed tomography shows a region of hypoattenuation involving the right basal ganglia, as well as the anterior and posterior limbs of the internal capsule. Subtle loss of grey–white matter differentiation is seen in the affected regions, and there is early effacement of the ipsilateral anterior horn of the lateral ventricle. (C) Axial diffusion-weighted magnetic resonance imaging demonstrates restricted diffusion in the right basal ganglia and internal capsule, correlating with acute ischaemia. There is associated swelling and partial compression of the adjacent frontal horn of the right lateral ventricle, consistent with early mass effect.

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  Figure 2. (A) Chest X-ray with moderate cardiomegaly, bilateral pleural effusions, and pulmonary oedema. (B). The 15-lead electrocardiogram demonstrates biatrial enlargement, with tall, peaked P waves in the inferior leads consistent with right atrial enlargement, and broad, bifid P waves in lead II with a negative terminal P-wave in V1 consistent with left atrial enlargement. There are also diffuse ST-T wave abnormalities (flattened to mildly inverted T waves across precordial and limb leads).

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  Figure 3. (A) Apical four-chamber echocardiographic view showing severe biatrial enlargement, with a markedly increased left atrial volume index of 68.7 mL/m2. The left ventricular chamber size remains within normal limits, highlighting the disproportionate degree of atrial dilatation relative to ventricular dimensions. (B) Pulsed-wave Doppler of mitral inflow showing an elevated E/A ratio (2.72) with a shortened deceleration time (116 ms) according to age values, findings consistent with chronically elevated left ventricular filling pressures and restrictive physiology.

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  Figure 4. (A) Axial cardiac magnetic resonance imaging using steady-state free precession sequence demonstrating qualitatively severe biatrial dilation with reduced ventricular volumes (left ventricular end-diastolic volume index 65 mL/m2 and right ventricular end-diastolic volume index 71 mL/m2) and preserved biventricular function (left ventricular ejection fraction 63% and right ventricular ejection fraction 66%) in keeping with restrictive cardiomyopathy. (B) Associated findings include thickened interlobar fissures, interlobular septal thickening accompanied by areas of pulmonary lymphangiectasia and bilateral pleural effusions (right > left). These features reflect systemic venous and lymphatic congestion secondary to impaired diastolic filling.