Crushed from the Outside, Leaking from the Inside: A Dual Cardiac Challenge in Marfan Syndrome.

Description of Clinical Presentation: This clinical case addresses the complex management of a 14-year-old patient seen at the outpatient clinic with complaints of occasional precordial pain. Marfan syndrome was diagnosed.

His medical history noted a cardiac murmur since early childhood; an echocardiogram was performed diagnosing severe mitral insufficiency with a bad ecographic window. Preoperative examens were performed noting on simple Chest X-Ray, pectum excavatum and scoliosis.

Diagnostic Techniques and Their Most Important Findings: Ct chest demonstrated Pectus excavatum (PE) with Haller index of 4.8 that was compressing the right ventricle (RV). An extrinsic right-sided obstruction confirmed on magnetic resonance, caused by presented with a dual hemodynamic problem: left-sided volume overload due to severe mitral regurgitation inherent to MFS, and an extrinsic right-sided obstruction caused by severe PE.

Given the interaction of both conditions, a staged surgical approach was chosen, first correcting the chest deformity to relieve the RV compression and then performing a mitral valve repair.

Post surgical functional evaluation needed to be performed using Cardiovascular Magnetic Resonance (CMR), considered the gold standard for MFS due to its accuracy and lack of radiation. However, the retrosternal metal bar was believed to cause massive artifacts on standard CMR sequences (bSSFP), obscuring the cardiac chambers and making evaluation impossible.

In multidisciplinary consensus it was clear that echocardiogram window wasn’t good enough to assess the new heart function and MRI was performed using advanced Metal Artifact Reduction (MAR) protocol was implemented. Fast spin-echo (FSE) sequences combined with the View Angle Tilting (VAT) technique were used, which are inherently less sensitive to the magnetic distortions caused by the metal. This strategy allowed for clear, diagnostic images, enabling a reliable delineation of the ventricles.

The postoperative CMR with MAR demonstrated the success of the intervention: the RV recovered its normal shape and its systolic function improved dramatically, with a mild increase in ejection fraction (from 40% to 46%). The left ventricle parameters remained stable considering the mitral valve repair.

Learning Points from this Case: The case underscores that PE correction in these patients is a crucial therapeutic step and not merely cosmetic. Furthermore, it conclusively demonstrates that CMR, through the use of personalized sequences, is an indispensable and viable tool for postoperative evaluation in patients with metallic implants, allowing for the objective quantification of surgical success and guiding the future management of the underlying valve disease.

Fig. 1: A.- Presurgical XRay B.- Presurgical CT with severe haller index C.- Post surgical Xray with thoracic bars. D.- Presurgical CMR with cardiac compression.
Fig. 2: A-B-C.- Presurgical CMR. D-E-F.- Postsurgical CMR