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Left ventricular non-compaction in a patient with ankylosing

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ABSTRACT

A 58 years old male with a long-standing history of HLA-B27 positive ankylosing spondylitis presented with increasing fatigue and dyspnea on exertion. He had left ventricular dysfunction and enlargement, flail right coronary leaflet of aortic valve with severe eccentric aortic insufficiency along with left ventricular non-compaction in echocardiography. The most common cardiac manifestations of ankylosing spondylitis are aortic insufficiency and conduction disturbances. Involvement of myocardium, in the form of dilated cardiomyopathy and restrictive cardiomyopathy, has also been reported. This case presents a very rare association of ankylosing spondylitis with non-compaction cardiomyopathy.

No MeSH data available.


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Mentions: A 58 years old male with a 16-year history of HLA-B27+ ankylosing spondylitis presented with increasing dyspnea on moderate exertion and easy sense of fatigue. His joint pain was controlled effectively with non-steroidal anti-inflammatory drugs. He never smoked and the remaining of his medical history was unremarkable. Family history for cardiac or rheumatologic disorders was negative. On physical exam, he appeared tachycardic with a blood pressure of 135/60 mm Hg. Apical impulse was displaced to left and there was a diastolic murmur and S3 gallop on cardiac auscultation. In transthoracic echocardiography there was left ventricular enlargement with left ventricular end diastolic diameter of 7.2 cm and left ventricular end systolic diameter of 6.1 cm. Left ventricular ejection fraction was 32% (Supplementary Video 1). Right coronary cusp (RCC) of aortic valve (AV) was thick and fibrotic. RCC was flail causing significant defect in coaptation that yielded severe eccentric turbulent jet towards anterior leaflet of mitral valve (Supplementary Video 2). There was also biventricular non-compaction (Figure 1). Descending aorta holodiastolic flow reversal was also noted. In transesophageal echocardiography dilation of sinus of valsalva was noted (Supplementary Video 3). The patient refused surgery and opted to be managed medically.


Left ventricular non-compaction in a patient with ankylosing
© Copyright Policy
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC5304103&req=5

Mentions: A 58 years old male with a 16-year history of HLA-B27+ ankylosing spondylitis presented with increasing dyspnea on moderate exertion and easy sense of fatigue. His joint pain was controlled effectively with non-steroidal anti-inflammatory drugs. He never smoked and the remaining of his medical history was unremarkable. Family history for cardiac or rheumatologic disorders was negative. On physical exam, he appeared tachycardic with a blood pressure of 135/60 mm Hg. Apical impulse was displaced to left and there was a diastolic murmur and S3 gallop on cardiac auscultation. In transthoracic echocardiography there was left ventricular enlargement with left ventricular end diastolic diameter of 7.2 cm and left ventricular end systolic diameter of 6.1 cm. Left ventricular ejection fraction was 32% (Supplementary Video 1). Right coronary cusp (RCC) of aortic valve (AV) was thick and fibrotic. RCC was flail causing significant defect in coaptation that yielded severe eccentric turbulent jet towards anterior leaflet of mitral valve (Supplementary Video 2). There was also biventricular non-compaction (Figure 1). Descending aorta holodiastolic flow reversal was also noted. In transesophageal echocardiography dilation of sinus of valsalva was noted (Supplementary Video 3). The patient refused surgery and opted to be managed medically.

View Article: PubMed Central - HTML - PubMed

ABSTRACT

A 58 years old male with a long-standing history of HLA-B27 positive ankylosing spondylitis presented with increasing fatigue and dyspnea on exertion. He had left ventricular dysfunction and enlargement, flail right coronary leaflet of aortic valve with severe eccentric aortic insufficiency along with left ventricular non-compaction in echocardiography. The most common cardiac manifestations of ankylosing spondylitis are aortic insufficiency and conduction disturbances. Involvement of myocardium, in the form of dilated cardiomyopathy and restrictive cardiomyopathy, has also been reported. This case presents a very rare association of ankylosing spondylitis with non-compaction cardiomyopathy.

No MeSH data available.