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A Constellation of Cardiac Anomalies: Beyond Shone's Complex

Neeraj K Ganju1, Arvind Kandoria1, Suresh Thakur2

  • 1Department of Cardiology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India.

Heart Views : the Official Journal of the Gulf Heart Association
|June 14, 2016
PubMed

Insights

Shone's anomaly, a rare congenital heart defect, involves four left-sided obstructive lesions. This case details a unique presentation in an adolescent with additional complex cardiac anomalies, emphasizing comprehensive echocardiography.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • Shone's anomaly is a rare congenital cardiac malformation defined by four specific left-sided obstructive lesions.
  • These lesions include supravalvular mitral membrane, parachute mitral valve, subaortic stenosis, and coarctation of the aorta.

Observation:

  • A unique case of Shone's complex is presented in a 14-year-old male.
  • The patient exhibited the four characteristic lesions plus bicuspid aortic valve, sinus of Valsalva aneurysm, patent ductus arteriosus, ventricular septal defect, persistent left superior vena cava, and severe pulmonary hypertension.

Findings:

  • This case highlights an exceptionally complex presentation of Shone's anomaly.
  • The coexistence of multiple congenital cardiac anomalies was noted, extending beyond the typical four lesions.

Implications:

  • A high index of clinical suspicion is crucial for diagnosing complex congenital cardiac malformations like Shone's anomaly.
  • Comprehensive echocardiography is vital for identifying all associated anomalies and guiding patient management.

Keywords:
Coarctation of aortaShone's complexpatent ductus arteriosusventricular septal defect

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