Large left ventricular apical aneurysm in a child with Noonan syndrome and hypertrophic cardiomyopathy

Figures

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  Figure 1. Transesophageal echocardiography (a) shows left ventricular (LV) outflow obstruction due to systolic anterior motion of the anterior mitral leaflet obstruction leading to mitral regurgitation into the left atrium. Modified transgastric view (b) shows apical LV aneurysm. Red arrow marks the junction between the left ventricle and left ventricular aneurysm. The outflow gradient (c) is close to 190 mmHg, and the spectral Doppler at the mouth of the aneurysm (d) shows to and fro flows. LV: Left ventricular, LA: Left atrium, LVA: Left ventricular aneurysm

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  Figure 2. Magnetic resonance long axial images (a and b) show hypertrophied left ventricle (LV) and left ventricular apical aneurysm (LVA), that penetrates through the left dome of the diaphragm. LA: Left atrium, Ao: Aortic root

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  Figure 3. High left atrial pressure (a) is noted after transseptal puncture, while the ascending aorta showed a spike and dome pattern (b). Simultaneous pressures (c) from the left ventricle and ascending aorta showed high peak to peak (P-p) and mean gradients, as well as high filling pressures (d)

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  Figure 4. Transseptal catheter advanced into the left ventricular aneurysm delineates it in right anterior oblique view in diastolic (a) and systolic frames (b). The mid-cavity obstruction is also shown in the left anterior oblique view in diastole (c) and systole (d). LVA: Left ventricular aneurysm

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  Figure 5. Electrocardiogram before surgery (a) showed left ventricular hypertrophy; following surgery, there was left bundle branch block (b)

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  Figure 6. Postsurgical transesophageal echocardiogram (a) demonstrates relief of outflow obstruction. Magnetic resonance imaging shows a reduction in septal thickness after myectomy (b) and elimination of the apical aneurysm (c). LA: Left atrium, RA: Right atrium, LV: Left ventricle, RV: Right ventricle, IVS: Interventricular septum

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  Figure 7. Contrast-enhanced computed tomography shows blowout of the apical closure patch resulting in a large pseudoaneurysm (a) from the left ventricle with calcified pledgeted sutures seen within the aneurysm. A large 5-cm superior mesenteric artery aneurysm with thrombus layers is seen in the coronal slice (b) of the abdomen. A large frontoparietal hemorrhage with surrounding edema (c) causes midline shift and obliteration of ventricles and sulci. Positron emission tomography (d) shows uptake of tracer in the mycotic aneurysm in the abdomen. LA: Left atrium, LV: Left ventricle, Ao: Aorta