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Hemodynamics in Adults With the Shone Complex.

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Adults with Shone complex (SC) exhibit significant heart and pulmonary remodeling, with high rates of elevated pressures. This patient group faces a substantial risk of mortality and cardiac transplantation.

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Area of Science:

  • Cardiology
  • Cardiovascular Surgery
  • Pediatric Cardiology

Background:

  • Shone complex (SC) involves multiple left-sided obstructive lesions, increasing risk for left ventricular (LV) remodeling, diastolic dysfunction, and pulmonary hypertension.
  • Hemodynamic profiles in adult SC patients remain largely undescribed.
  • Understanding these hemodynamics is crucial for managing SC progression and outcomes.

Purpose of the Study:

  • To describe the hemodynamic characteristics of adult patients diagnosed with Shone complex.
  • To identify remodeling patterns and associated pressures in this cohort.
  • To report on clinical outcomes including mortality and transplantation.

Main Methods:

  • Retrospective chart review of 25 adult SC patients undergoing cardiac catheterization (2002-2019).
  • SC defined by multiple left-sided obstructive lesions and abnormal mitral valve.
  • Analysis of hemodynamic data (LV end-diastolic pressure, pulmonary artery pressures) and clinical outcomes.

Main Results:

  • The cohort (median age 32) frequently presented with coarctation of the aorta (84%) and prior valve replacements.
  • Elevated LV end-diastolic pressure (>15 mm Hg) was seen in 71% of patients.
  • Pulmonary artery pressures were significantly elevated, with 96% exceeding 20 mm Hg.

Conclusions:

  • Adult SC patients demonstrate significant left-sided heart and pulmonary vascular remodeling.
  • High prevalence of elevated LV and pulmonary artery pressures indicates severe hemodynamic compromise.
  • The study reveals high mortality (28%) and cardiac transplant rates (12%) in this cohort.