Differential Clubbing and Cyanosis: Symptoms of Eisenmenger’s Syndrome

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The New England Journal of Medicine

A 30-year-old woman presented into the outpatient clinic for adults with known congenital heart disease and a 1-year history of progressive exertional dyspnea, hemoptysis and fatigue. On clinical examination, the patient showed signs of subtle clubbing in the fingers, whereas, marked marked clubbing and mild cyanosis on the toes. It was further noted that the patient has a left parasternal heave. In addition to this, the patient also presented with a audible systolic ejection murmur in the pulmonic area and a palpable second heart sound.

A contrast echocardiography was performed on the patient, which showed a bidirectional flow through a patent ductus arteriosus, elevated mean pulmonary-artery pressure of 68 mm Hg and right arterial and ventricular dilation.

Differential cyanosis and clubbing is a typical presentation of Eisenmenger’s syndrome. A reversal in the shunt causes the deoxygenated blood from the right ventricle to be shunted distal to the left subclavian artery (LSCA), to the aorta. Sparing both upper extremities, this leads to selectively affecting the lower extremities. However, the large sized PDA in this case caused a jet effect and a selective streaming of deoxygenated blood to the descending aorta and LSCA, affecting the left upper extremity.

Einsenmenger’s syndrome

Eisenmenger’s syndrome presents with an eventual bidirectional or pulmonary-to-systemic shunting of blood and pulmonary hypertension caused by a long-standing systemic-to-pulmonary circulatory shunt. Deoxygenated blood is delivered to the aorta distal to the left subclavian artery from the right ventricle. Thus, the upper extremities are spared from the effects of the shunt. Whereas, resulting in differential clubbing and cyanosis since the lower extremities are not spared.

References

Anoop, T. M., & George, K. C. (2011). Differential clubbing and cyanosis. New England Journal of Medicine364(7), 666-666.

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Dr. Aiman Shahab is a dentist with a bachelor’s degree from Dow University of Health Sciences. She is an experienced freelance writer with a demonstrated history of working in the health industry. Skilled in general dentistry, she is currently working as an associate dentist at a private dental clinic in Karachi, freelance content writer and as a part time science instructor with Little Medical School. She has also been an ambassador for PDC in the past from the year 2016 – 2018, and her responsibilities included acting as a representative and volunteer for PDC with an intention to make the dental community of Pakistan more connected and to work for benefiting the underprivileged. When she’s not working, you’ll either find her reading or aimlessly walking around for the sake of exploring. Her future plans include getting a master’s degree in maxillofacial and oral surgery, settled in a metropolitan city of North America.

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