Pectus Excavatum With Compression of the Inferior Vena Cava

Abstract

Pectus excavatum (PE) is a common disorder of the chest wall that affects 1 in 500 of the general population. Affected individuals have cosmetic and, less commonly, cardiopulmonary consequences that typically present during the adolescent years. The latter is appreciated only in those with severe phenotypes, especially if associated with a “straight back” that further limits the anterior-posterior thoracic dimensions. Although the cardiopulmonary complications of PE have been well described, we describe a unique presentation of a patient who had experienced years of intractable syncope before receiving corrective surgery for severe PE. The role of cardiovascular magnetic resonance imaging (CMR) in her diagnosis and subsequent treatment is also highlighted. A 22-year-old woman presented with a long-standing history of recurrent syncope, beginning in early adolescence. These episodes typically occurred after prolonged periods of standing or walking, beginning with a prodrome of diaphoresis and blurred vision and culminating in loss of consciousness. She described self-taught maneuvers that reduced these symptoms, including twisting her torso to the side and performing rapid, shallow breathing. In spite of these maneuvers, she continued to be limited by frequent syncope and ultimately stopped attending school at 16 years of age. On examination, she had a marked pectus deformity with a straight back appearance. Her heart sounds were normal, with a Grade II out of VI systolic ejection murmur at the left sternal border. There was no jugular venous distention and no peripheral edema. Following indentification of the chest wall deformity further questioning revealed that both her brother and paternal uncle had severe PE, suggesting an autosomal dominant inheritance …