1357813 - A diaphragm next to the heart

This is a 41 years-old-male with previous history of hypertension, dyslipidemia, and a family history of premature coronary artery disease, who presented to the cardiology clinic with mild exertional dyspnea and intermittent chest pain. Physical exam was unremarkable. On evaluation, an echocardiogram revealed hypokinesis of the basal and mid segments of the lateral wall, preserved left ventricle (LV) ejection fraction (60%), and decreased LV global longitudinal strain (-15%).

Diagnostic Techniques and Their Most Important Findings:

Given his pretest likelihood for CAD, coronary angiography was performed, revealing normal coronary arteries. The persistence of symptoms prompted the evaluation with cardiac magnetic resonance (CMR), where a large diaphragmatic hernia was evident (Figure 1). The short axis SSFP demonstrates flattening in diastole of the LV lateral wall, indicating mechanical compression of the heart caused by the diaphragm eventration into the thorax. Most of the upper abdominal organs were displaced upwards, occupying the left hemithorax and shifting the heart rightwards and posteriorly. There was no evidence of late gadolinium enhancement. The patient was referred to the general surgical, where a conservative approach was preferred.

 

Learning Points from this Case:

Diaphragmatic eventration (DE) is the abnormal elevation of a portion or entire hemidiaphragm due to a lack of muscle or nerve function while maintaining its anatomical attachments. A limited number of case reports have suggested the incidence is <0.05%, with male predominance and more commonly affecting the left hemidiaphragm. DE is the result of congenital insufficiency of the diaphragmatic musculature.

Most cases go unrecognized until adult life, diagnosed as incidental findings on routine chest evaluation. Generally, patients are asymptomatic; however, some may present dyspepsia, dysphagia, gastroesophageal reflux, and/or epigastric pain. Adults may also complain of chest pain or dyspnea. Therefore, DE should be considered a rare cause of chest pain and be included in the differential of atypical cases or bizarre presentations. The compression of the heart has been reported. The evaluation with CMR provides an assessment of the cardiac function that may render necessary surgical intervention.