Page 36 - Clinical Pearls in Cardiology

24 Clinical Pearls in Cardiology

primum atrial septal defects, ventricular septal defects
and atrioventricular regurgitation due to various types
of endocardial cushion defects.
36. What is the importance of identifying Marfanoid
habitus in a patient?
Skeletal manifestations producing the so called
Marfanoid habitus are the cardinal signs of Marfan’s
syndrome. This autosomal dominant disorder results
from mutations of the fibrillin gene. The most serious
complications associated with this syndrome are the
cardiovascular complications such as mitral valve
prolapse, dilatation of the aortic root with aortic valve
incompetence and aortic wall dissection. Marfanoid
habitus is characterized by the following features:
• Tall stature and skeletal disproportion with arm span
greater than height
• Long and thin “spider” fingers (arachnodactyly) with
positive “thumb” sign (Steinberg sign) and “wrist”
sign
• Pectus excavatum with sternal depression
• Generalized hypermobility of joints
• Bilateral symmetrical upward dislocation of lens and
iridodonesis
• High arched palate with crowded teeth and dental
malocclusion.
37. What is the importance of waist-to-hip ratio?
Regional distribution of fat is of greater prognostic
significance than the absolute degree of obesity. The
waist-hip ratio provides a simple assessment of visceral
adipose fat. Subjects with a ‘pear-shaped’ configuration
and a waist-hip ratio of less than 0.8 in females or less
than 0.9 in males have a good prognosis. Those with