Abstract

A 15 year old girl was referred to the paediatric outpatient clinic by her GP with a 2 year history of chest pain at rest and during exercise. Pectus excavatum was noted and on examination there was tenderness of the costochondral joints.

A chest x-ray revealed apparent cardiomegaly, possibly secondary to the pectus excavatum. While the results of the initial echocardiogram were unremarkable, ECG changes were noted in the ST-T segments for the inferior leads. Subsequently, exercise tolerance tests were carried out, consisting of 12 min on a treadmill. This elicited ST-T changes in the anterior leads. An heart MRI scan showed a dialted right ventriclu with impaired right ventricular systolic function. A repeat MRI scan with dobutamine confirmed this with the EF being 37%. Left ventricular systolic function was found to be at the lower end of normal, potentially consequent to the right ventricular dilatation.

The patient continued to experience chest pain and breathlessness at rest, and repeat echocardiogram confirmed dilatation of the right ventricle with right ventricular dysfunction (left ventricle had normal contactility and no dilatation). There was also mild trucusipd regurguation at 2.6m/sec. Repair of the pectus excavatum took place 6 months later. During the surgery, it was noted that removal of the xiphisternum resulted in immediate decompression of the right ventricle. Thoracic surgeons were able to release tissues and increase the AP diameter of the chest with a modified Ravitch procedure using prolene mesh. The post-operative echocardiogram showed improved right ventricular contactility, no tricuspid or mitral regurgiation and no outflow obstruction. The patient has had a repeat stress test which was completely normal 6 months post surgery.