•Serial echocardiograms which showed marked uniform hypertrophy with ground glass appearance of the left ventricle and severely reduced systolic function;

• PET-CT with no hyperaccumulation of the radiopharmaceutical;

• Cardiac MRI with evidence of severely hypocontractile left ventricle and wall changes which could be associated with amyloid infiltration;

• myocardial scintigraphy that does not give evidence for the deposit of the amyloid substance of the ATTR type in the heart;

• biopsy of umbilical fat and cardiac biopsy, both negative for deposition of substance AL with Congo Red staining;

• Right cardiac catheterization with evidence of post-capillary pulmonary hypertension.

 

The patient was subjected to several exacerbations of heart failure which required continuous infusion therapy with inotropes and, ultimately, heart transplantation. At the time of surgery, the heart appeared enlarged, extremely hypocontractile and with rigid walls, with poor compliance of the ventricular muscle wall. The patient responded well to the surgical procedure, in the absence of signs and symptoms of rejection, with improvement of general clinical condition.

Conclusion

At the end of the surgical procedure and the rehabilitation period, the patient was discharged home, without showing signs and symptoms of graft rejection or multiple myeloma exacerbations, in stable and satisfactory general clinical conditions