In routine practice, the imaging work-up for screening SDHx mutation carriers should include thoraco-abdomino-pelvic computed tomography, head and neck magnetic angiography, and somatostatin receptor scintigraphy. Expert centralized image assessment is recommended.

See also Huisman MV, Klok FA. Magnetic resonance imaging for diagnosis of acute pulmonary embolism: not yet a suitable alternative to CT-PA.This issue, pp 741-2; and Sodhi KS, Saxena AK. Diagnostic accuracy of computed tomography pulmonary angiography and magnetic resonance imaging for an acute pulmonary embolism. This issue, pp 961.Summary. Background: Magnetic resonance imaging (MRI) has not been validated as an alternative diagnostic test to computed tomography angiography (CTA) in patients with suspicion of a pulmonary embolism (PE). Objectives: To evaluate performance of current MRI technology in diagnosing PE, in reference to a 64-detector CTA. Patients/methods: Prospective investigation including 300 patients with a suspected PE, referred for CTA after assessment of clinical probability and D-dimer testing. MRI protocol included unenhanced, perfusion and angiographic sequences. MRI results were interpreted by two independent readers, to evaluate inter-reader agreement. Sensitivity and specificity were evaluated globally and according to PE location and to clinical probability category. Results: Of 300 enrolled patients, 274 were analyzed and 103 (37.5%) had a PE diagnosed by CTA. For patients with conclusive MRI results (72% for reader 1, 70% for reader 2), sensitivity and specificity were 84.5% (95% confidence interval [CI], 74.9-91.4%) and 99.1% (95% CI, 95.1-100.0%), respectively, for reader 1, and 78.7% (95% CI, 68.2-87.1%) and 100% (95% CI, 96.7-100.0%) for reader 2. After exclusion of inconclusive MRI results for both readers, inter-reader agreement was excellent (kappa value: 0.93, 95% CI: 0.88-0.99). Sensitivity was better for proximal (97.7-100%) than for segmental (68.0-91.7%) and sub-segmental (21.4-33.3%) PE (P < 0.0001). Sensitivity was similar for both readers within each clinical probability category. Conclusions: Current MRI technology demonstrates high specificity and high sensitivity for proximal PE, but still limited sensitivity for distal PE and 30% of inconclusive results. Although a positive result can aid in clinical decision making, MRI cannot be used as a stand-alone test to exclude PE.

Conservative surgery is indicated for all symptomatic patients. The incidence of valve repair is high when leaflet mobilization is performed. Valve replacement can be avoided in most cases. Functional and hemodynamic results are excellent.