Case report: retroperitoneoscopic pheochromocytoma removal in an adult with Eisenmenger's syndrome

Patients with uncorrected or palliated, complex congenital heart lesions requiring surgery can benefit from laparoscopic techniques, but retroperitoneal insufflation may render them hemodynamically unstable. Alterations in cardiopulmonary physiology during retroperitoneal insufflation have been studied, yet there are no cases detailing this approach in patients with congenital heart lesions. We present a case of a pheochromocytoma removal via retroperitoneoscopy in a patient with a palliated, complex heart lesion.

A 28-yr-old woman was admitted for removal of a pheochromocytoma through retroperitoneoscopy. The main feature of her heart disease was a complete atrioventricular canal defect. She eventually developed Eisenmenger's syndrome and became chronically cyanotic. Retroperitoneal insufflation with CO2 gas did not change hemodynamic variables. Significant increases in peak airway pressures were encountered, possibly due to the distending effects of insufflation, or due to increasing the minute ventilation to reduce exogenous CO2. Arterial CO2 remained stable, but a significant increase between end-tidal and arterial levels became apparent with insufflation. Tumour manipulation led to systemic (and possibly pulmonary) hypertension, which exacerbated ventricular dysfunction. This condition resulted in atrioventricular valve regurgitation, as seen on transesophageal echocardiography, and diminished pulmonary blood flow with subsequent desaturation. These changes resolved with antihypertensive medications. The patient's trachea was extubated four hours postoperatively, and she recovered uneventfully.

Patients with altered cardiopulmonary physiology may tolerate retroperitoneoscopic insufflation with relative hemodynamic stability. Appropriate use of short-acting, vasoactive drugs and aggressive monitoring of PaCO2 and hemodynamic variables is required.