Paragangliomas (PGLs) are rare neuroendocrine tumors that arise from the
autonomic ganglia. A 34-year-old woman was admitted to our department
with palpitation, fatigue and chest pain under stress. Past medical
history included a para-carotid PGL surgically removed 5 years earlier.
Her family history was notable for familiar PGL with SDH mutation. At
stress-echocardiography, was recorded a marked ST-elevation on
precordial-leads, concomitant to new onset anterior septum hypo-kinesis,
and therefore the patient was proposed for coronary angiography.
Although angiographic evidence of atherosclerosis were not recorded, was
found that Left Anterior Descending Coronary Artery gave off some
branches towards a vascular structure (3×2 cm) in contiguity with the
left coronary sinus of the aortic root. Subsequent Compute-Tomography
and Magnetic-Resonance clarified the anatomical relationships of the
mass with adjacent cardiac structures, in particular the mass was
located behind the pulmonary artery trunk and lateral to aortic root,
lying above the first portion of the anterior descending coronary
artery. Subsequently a careful discussion about the risks and benefits
of all therapeutic opportunity, the open surgical resection was deemed
the best option for the patient despite the complex clinical anatomy.
After standard cannulation for CPB and cardioplegic arrest, transection
of the pulmonary artery and the aorta was performed granting adequate
exposure of the mass and identification of afferent and efferent
vascularization for complete resection though a cleavage plane. To
ensure adequate hemostasis on a such critical area, the continuity of
the pulmonary trunk was ensured only after removing aortic cross-clamp.
The post-operative course was uneventful