What are the associations and indications for surgery in pectus excavatum?

Pectus Excavatum: Associations and Indications for Surgery

Pectus excavatum is often associated with cardiac dysfunction, and while cosmesis is the most common indication for surgery, repair should be considered when there are significant cardiopulmonary symptoms affecting quality of life. 1

Associated Conditions and Physiological Impact

Cardiac Associations

• Pectus excavatum is often associated with cardiac dysfunction (15-20% of cases) including:
  • Mitral valve prolapse (in approximately 15% of patients) 2
  • Cardiac compression/displacement 1, 2
  • Diminished right ventricular volume 2
  • Bundle branch block or other cardiac pathology secondary to compression 2

Respiratory Associations

• Respiratory dysfunction is not usually present in mild cases but may occur in severe deformities:
  • Restrictive pulmonary disease in severe cases 2
  • Paradoxical respiratory chest wall motion 2
  • Shortness of breath with exercise 2

Other Associations

• Often seen with connective tissue disorders:
  • Marfan syndrome 1, 3
  • Other connective tissue disorders (73% of patients requiring cardiac surgery had confirmed connective tissue disorders) 3
• May be associated with:
  • Straight thoracic spine 1
  • Other skeletal abnormalities 1
  • Noonan syndrome 1

Indications for Surgical Repair

Primary Indications

• Cosmetic concerns are the most common indication for surgery 1, 4
• Surgical treatment is indicated when two or more of the following are present:
  • Severe, symptomatic deformity 2
  • Progressive deformity 2
  • Cardiopulmonary compromise affecting quality of life 1
  • Significant psychological impact affecting quality of life 1

Objective Criteria Supporting Surgical Intervention

• CT scan with a pectus index (Haller index) greater than 3.25 2
• Cardiac compression/displacement and/or pulmonary compression 2
• Pulmonary function studies showing restrictive disease 2
• Cardiac pathology secondary to compression 2
• Failed previous repair(s) 2

Timing and Approach to Surgery

Timing Considerations

• Delay pectus excavatum repair if aortic root dilation is present and approaching surgical thresholds (≥4.5 cm), as aortic root surgery takes precedence 1
• Cardiovascular management always takes priority over pectus repair 1

Surgical Approaches

• Minimally invasive "Nuss" approach can be successful in adults with resolution of symptoms and improved quality of life 4
• Modified Ravitch procedure may be used, especially when combined with cardiac surgery 3

Special Considerations

Combined Cardiac and Pectus Surgery

• Simultaneous repair of pectus excavatum during cardiac surgery can be safely performed by a multidisciplinary team in selected cases 3
• However, some evidence suggests avoiding simultaneous cardiac and pectus excavatum repair due to potential major complications 5
• For patients requiring an extracardiac conduit, initial repair of pectus excavatum followed by cardiac repair (after 6 weeks or later) is recommended to prevent compression of the conduit 5

Post-Repair Considerations

• Pectus repair remains intact even after subsequent median sternotomy for cardiac procedures 6
• Continued medical therapy for cardiovascular protection is essential even after surgical intervention in patients with associated connective tissue disorders 1

In summary, while cosmesis is the most common indication for pectus excavatum repair, the decision for surgical intervention should be based on a combination of factors including severity of deformity, cardiopulmonary symptoms, psychological impact, and objective measurements such as the Haller index.