Analyze This Image: What Is Being Shown Here?

Ronald H. Wharton, MD

Disclosures

May 31, 2019

Explanation

The correct answer is cyanosis.

Reduced excursion of the mitral leaflets during diastole is shown in the apical four-chamber view (Figure 1). The color Doppler image (Figure 2) shows turbulent flow entering the left ventricle during diastole. The continuous-wave Doppler image through the mitral valve (Figure 3) demonstrates a diastolic gradient of almost 4 mm Hg, with a heart rate of 55 beats/min, which is consistent with mild mitral stenosis.

These images, however, do not represent a rheumatic mitral valve. The leaflets do not have the characteristic thickening of rheumatic disease, and the posterior leaflet is not "fixed" as would be expected in rheumatic mitral stenosis. A subtle finding in the apical 4-chamber view is the very small aorta behind the left atrium. Moreover, if one looks closely at the short-axis image (Figure 4), one sees that there is only one papillary muscle.

The findings from these images demonstrate part of the Shone complex (also referred to as Shone syndrome or Shone anomaly) of lesions that cause obstruction to flow on the left side of the heart.

The following are additional images obtained from the same study.

 

Figure 5

Figure 6

Figure 7

Figure 8

Figure 9

Looking closely at Figure 5, one sees not only what appears to be a subaortic membrane, but also a very small descending thoracic aorta behind the left atrium.

Figures 6 and 7 demonstrate a bicuspid aortic valve with turbulence, which is apparent in the color image.

Figures 8 and 9 demonstrate aortic coarctation with the corresponding increase in the velocity of blood flow.

Patients with subaortic membranes typically have systolic murmurs. Bicuspid valves often cause some degree of aortic stenosis, which would also lead to a systolic murmur. Coarctation leads to hypertension in the upper extremities and claudication in the lower extremities. Dyspnea is the usual symptom when mitral stenosis is present, especially when the patient engages in activities that raise his or her heart rate, which decreases diastolic filling time. Cyanosis would not be expected in this syndrome.

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