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A young female with cyanosis and clubbing

  • Praveen K. Gupta
    Affiliations
    Department of Cardiology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India
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  • Raja J. Selvaraj
    Correspondence
    Corresponding author at: Jawaharlal Institute of Post graduate Medical Education and Research, Pondicherry 605006, India.
    Affiliations
    Department of Cardiology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India
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Published:February 27, 2018DOI:https://doi.org/10.1016/j.ejim.2018.02.023

      Highlights

      • Cyanosis is typically asymmetric in patent ductus arteriosis with shunt reversal.
      • Because of the location of the shunt beyond the origin of the innominate artery, the right upper limb is typically spared.
      • Clubbing also exhibits the same pattern and may be easier to recognise in the presence of mild desaturation.
      A 21 year old female, deaf-mute since birth, presented with a history of exertional dyspnea since 8 years. On auscultation, she had a single, loud second heart sound and no murmurs. Fig. 1 is a photograph showing the hands and feet. Pulse oximeter readings from both upper limbs are also shown. What is the diagnosis?
      Fig. 1
      Fig. 1Photograph showing hands and feet with pulse oximetry.

      1. Discussion

      The photograph shows clubbing that is prominent in the toes and in the fingers of the left hand, but absent in the fingers of the right hand. The pulse oximetry readings confirm desaturation in the left upper limb, but not in the right. Differential cyanosis and clubbing are diagnostic of patent ductus arteriosus (PDA) with reversal of shunt due to severe pulmonary hypertension. The innominate artery arises proximal to the PDA and is therefore spared from the right to left shunt. This diagnosis was confirmed on echocardiography. Association with sensorineural deafness makes this likely to be part of Congenital Rubella syndrome. She is on treatment with oral sildenafil for the pulmonary hypertension.

      Financial support

      None.

      Conflict of interest

      No conflicting relationship exists for authors.