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Unusual combination of holt-oram syndrome and persistent left superior vena cava

Sergio Thal1, Ravichandra Boyella, Reza Arsanjani

  • 1Southern Arizona VA Health Care System and Sarver Heart Center, University of Arizona, Tucson, AZ 85723, USA. Sergio.thal@va.gov

Congenital Heart Disease
|December 2, 2011
PubMed

Insights

Holt-Oram syndrome, a congenital condition, rarely co-occurs with persistent left superior vena cava. This rare combination complicated a cardiac resynchronization therapy device implant.

Area of Science:

  • Cardiology
  • Genetics
  • Medical Imaging

Background:

  • Holt-Oram syndrome is characterized by congenital heart defects and upper limb abnormalities.
  • Persistent left superior vena cava (PLSVC) is a rare congenital anomaly, infrequently associated with Holt-Oram syndrome.

Observation:

  • A 63-year-old male with Holt-Oram syndrome and a history of atrial septal defect repair underwent evaluation for cardiac resynchronization therapy (CRT) device implantation.
  • During the procedure, an unusually large coronary sinus and a persistent left superior vena cava draining into it were identified.
  • These findings complicated the intended transvenous lead placement for CRT.

Findings:

  • The case highlights an uncommon association between Holt-Oram syndrome and persistent left superior vena cava.
  • This combination was incidentally discovered during an attempted cardiac resynchronization therapy device implant.
  • The anatomical variations presented significant challenges for device lead implantation.

Implications:

  • Early recognition of this rare combination is crucial for planning device implantation procedures.
  • Awareness of this association can help avoid potentially unsuccessful interventions.
  • This case underscores the importance of advanced imaging in managing complex congenital cardiac anomalies.
Abstract

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