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 Medicine, 364(7), 666-666.
