Differential Clubbing and Cyanosis: Classic Signsof Patent Ductus Arteriosus With EisenmengerSyndromeSunil Kumar Srinivas, MD, and Cholenahally Nanjappa Manjunath, MD, DM

A 28-year-old woman with congenitalheart disease presented with progres-sive dyspnea and fatigue during the

past year. On examination, she had clubbingand cyanosis of the toes but not the fingers(Figure, A). Oxygen saturation was 84% inthe toes and 96% in the fingers. The patientalso had a left parasternal heave and a loud sec-ond sound but no murmur. The electrocardio-gram showed sinus rhythm with a rightwardaxis and right ventricular (RV) hypertrophy

(Figure, B). Chest radiography revealed adilated main pulmonary artery and rightdescending pulmonary artery with RV apex(Figure, C). Two-dimensional echocardio-graphy revealed a large patent ductus arteriosus(PDA) (Figure, D [arrow]) with predominantright-to-left shunting (Figure, D and E andSupplemental Video 1, available online athttp://www.mayoclinicproceedings.org). Pul-monary artery systolic pressure was 92 mmHg. Contrast echocardiography confirmed the

FIGURE. A, Clubbing and cyanosis of the toes but not the fingers in a patient with congenital heart disease.B, Electrocardiogram showed sinus rhythm with a rightward axis and right ventricular hypertrophy. C, Chestradiograph revealed a dilated main pulmonary artery (MPA) and right descending pulmonary artery (RDPA),with right ventricular (RV) apex. D, Two-dimensional echocardiogram revealed a large patent ductusarteriosus (arrow). E, Echocardiogram showed predominant right-to-left shunting. F, Contrast echocar-diogram confirmed the right to left shunting across the patent ductus arteriosus (arrow).

From the Department ofCardiology, Sri JayadevaInstitute of CardiovascularSciences and Research,Bangalore, Karnataka, India.

Mayo Clin Proc. n September 2013;88(9):e105-e106 n http://dx.doi.org/10.1016/j.mayocp.2013.02.016www.mayoclinicproceedings.org n ª 2013 Mayo Foundation for Medical Education and Research

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right-to-left shunting across the PDA (Figure, F[arrow] and Supplemental Video2, available on-line at http://www.mayoclinicproceedings.org). Computed tomography also confirmedthe large PDA. The patient wasmanagedmedi-cally and advised against pregnancy.

Longstanding PDA results in pulmonary hy-pertensionwith eventual pulmonary-to-systemicreversal of the shunting and development ofEisenmenger syndrome.1 Deoxygenated bloodfrom the RV is directed from the PDA to theaorta distal to the left subclavian artery, thuscausing clubbing and cyanosis in the lower, butsparing the upper extremities.2 Also, develop-ment of Eisenmenger syndrome abolishes theclassic “machinery” murmur of PDA, resultingin difficulty in arriving at a diagnosis. The devel-opment of severe pulmonary hypertension and

Eisenmenger syndrome precludes surgical or de-vice closure of PDA. In the longer term, progres-sive decline in exercise capacity or symptomaticright heart failure can be treated with heart andlung transplant.3

SUPPLEMENTAL ONLINE MATERIALSupplemental material can be found online athttp://www.mayoclinicproceedings.org.

1. Wald R, Crean A. Differential clubbing and cyanosis in a patientwith pulmonary hypertension. CMAJ. 2010;182(9):E380.

2. Anoop TM, George KC. Differential clubbing and cyanosis.N Engl J Med. 2011;364(7):666.

3. Diller GP, Dimopoulos K, Broberg CS, et al. Presentation, survivalprospects, and predictors of death in Eisenmenger syndrome: acombined retrospective and case control study. Eur Heart J.2006;27(14):1737-1742.

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