Physiological adaptations to chronic hypoxemia in eisenmenger syndrome

Bowater, Sarah Elizabeth (2014). Physiological adaptations to chronic hypoxemia in eisenmenger syndrome. University of Birmingham. M.D.

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Abstract

Eisenmenger syndrome is characterised by severe, lifelong hypoxaemia and pulmonary hypertension. Despite this, patients do surprisingly well and report a reasonable quality of life. This thesis describes a series of experiments investigating the adaptations that occur in these patients in response to the chronic hypoxaemia.

Patients with Eisenmenger syndrome have severely limited exercise tolerance when assessed using cardiopulmonary exercise testing. However, they appear to maintain aerobic metabolism until late on in exercise. Studies using skeletal muscle 31P MRS during and throughout recovery from exercise showed that these patients have similar mitochondrial oxidative capacity compared to healthy controls.

Echocardiography showed that patients with Eisenmenger syndrome have preserved right and left ventricular systolic function. However they have evidence of right ventricular diastolic dysfunction as evidenced by impaired early diastolic relaxation. The cardiac 31P MRS study demonstrated that despite the normal systolic function shown on echocardiography, there is impairment of septal energetics as revealed by a reduction in PCr/ATP ratio.

The results presented in this thesis indicate that adult patients with Eisenmenger syndrome have undergone beneficial adaptations to the severe hypoxaemia that they are exposed to from infancy.

Type of Work:

Thesis (Higher Doctorates > M.D.)

Award Type:

Higher Doctorates > M.D.

Supervisor(s):