From the Guidelines
Pectus excavatum can contribute to pulmonary hypertension in severe cases, and surgical correction may be recommended to relieve cardiac compression and prevent long-term pulmonary vascular changes, as supported by the most recent guidelines 1.
Diagnosis and Evaluation
Patients with significant pectus excavatum (typically with a Haller index >3.25) may experience cardiac compression and decreased right ventricular output, leading to increased pulmonary arterial pressures over time. Evaluation should include:
- Echocardiography to assess cardiac function and pulmonary arterial pressures
- Pulmonary function tests to evaluate lung function
- Possibly cardiac MRI to assess the degree of cardiac compression and pulmonary vascular resistance
Treatment and Management
For patients with documented pulmonary hypertension related to severe pectus excavatum, surgical correction through procedures like the Nuss or Ravitch technique may be recommended to relieve cardiac compression. The timing of intervention is crucial, with better outcomes typically seen when correction occurs before permanent pulmonary vascular changes develop. Medical management with pulmonary vasodilators may be necessary in established pulmonary hypertension cases, though addressing the anatomical cause through surgery remains the definitive treatment when the connection between the chest wall deformity and pulmonary hypertension is clear, as outlined in the 2022 update of the ACR Appropriateness Criteria for suspected pulmonary hypertension 1.
Monitoring and Follow-up
Regular cardiopulmonary monitoring is essential for patients with moderate to severe pectus excavatum to detect early signs of pulmonary hypertension before irreversible changes occur. This monitoring should include regular echocardiography, pulmonary function tests, and possibly cardiac MRI to assess the degree of cardiac compression and pulmonary vascular resistance, as recommended by the European Respiratory Journal 1.
From the Research
Pectus Excavatum and Pulmonary Hypertension
- Pectus excavatum is a congenital deformity of the chest wall that can affect cardiopulmonary function, with studies suggesting a correlation between the severity of the deformity and reduced pulmonary function 2, 3.
- The Haller index is a commonly used measure to assess the severity of pectus excavatum, with higher scores indicating more severe deformities 2, 3.
- Research has shown that patients with more severe pectus excavatum (Haller index > 3.25) are more likely to experience cardiopulmonary impairment, including reduced oxygen pulse and maximum oxygen uptake 3.
- Pulmonary hypertension is a potential complication of pectus excavatum, although the exact relationship between the two conditions is not fully understood 4, 5.
- Studies have investigated the use of bosentan and sildenafil in the treatment of pulmonary arterial hypertension, with mixed results 4, 5.
- Bosentan has been shown to decrease the plasma concentration of sildenafil when co-administered, which may impact the efficacy of treatment for pulmonary hypertension 4.
- The impact of pectus excavatum on cardiac morphology and function has been evaluated using multimodality imaging, with findings suggesting that patients with more severe deformities are more likely to experience cardiac abnormalities, including septal motion abnormalities and reduced exercise capacity 6.
Key Findings
- Increasing severity of pectus excavatum is associated with reduced pulmonary function and cardiopulmonary impairment 2, 3.
- The Haller index is a useful measure for assessing the severity of pectus excavatum and predicting cardiopulmonary function 2, 3.
- Bosentan and sildenafil may be used in the treatment of pulmonary arterial hypertension, although the optimal treatment strategy is not yet clear 4, 5.
- Pectus excavatum can have a significant impact on cardiac morphology and function, particularly in patients with more severe deformities 6.