PMC9975344_01
Case 1. (A) Immediate postnatal transthoracic echocardiogram in apical 4-chamber view showing a large hyperechogenic and homogeneous mass of 37 mm x 20.7 mm in size (area of 6.78 cm2), which occupies a large part of the right ventricular cavity, compatible with a giant rhabdomyoma. (B-D) Echocardiographic control at 2, 3, and 13 weeks after treatment with everolimus showed a significant reduction in the size of the rhabdomyoma.
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F1
This mass was located in a significant part of the right ventricular cavity, limiting the opening of the tricuspid valve and slightly obstructing the right ventricular outflow tract (Figure 1A). Echocardiographic follow-up demonstrated a reduction in the mass area of 11.3%, 38.6%, and 47.4% in the first, second, and third weeks, respectively Figures 1B,C, 2).
Comparative regression in the size of CRHMs after initiation of treatment with low dose everolimus. An accelerated decrease of close to 50% of the initial area of the CRHM is evident in both cases at three weeks of treatment. The maximum reduction in the mass area was reached at 17 weeks of treatment for case 1 and 12 weeks of treatment for case 2, the two of them close to 79%. In both patients, the serum everolimus level was maintained in therapeutic ranges (target through concentrations between 5 and 15 ng/ml). Once treatment was discontinued CRHM experienced rebound growth, being particularly accelerated in case 1. The restart of everolimus in case 1 demonstrated a rapid reduction in mass size. Case 2 CRHM maintained a stable size without signs of hemodynamic repercussions after undergoing rebound growth.
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F2
Echocardiographic follow-up showed a reduction in the mass area of 2.8%, 42.1%, and 48.9% in the first, second, and third weeks, respectively (Figures 2, 3B,C). Ten months after the end of treatment, the CRHM reached 92.7% of the initial area, with no changes in size or signs of hemodynamic compromise since then (Figure 2).
PMC9975344_02
Comparative regression in the size of CRHMs after initiation of treatment with low dose everolimus. An accelerated decrease of close to 50% of the initial area of the CRHM is evident in both cases at three weeks of treatment. The maximum reduction in the mass area was reached at 17 weeks of treatment for case 1 and 12 weeks of treatment for case 2, the two of them close to 79%. In both patients, the serum everolimus level was maintained in therapeutic ranges (target through concentrations between 5 and 15 ng/ml). Once treatment was discontinued CRHM experienced rebound growth, being particularly accelerated in case 1. The restart of everolimus in case 1 demonstrated a rapid reduction in mass size. Case 2 CRHM maintained a stable size without signs of hemodynamic repercussions after undergoing rebound growth.
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F2
Echocardiographic follow-up showed a reduction in the mass area of 2.8%, 42.1%, and 48.9% in the first, second, and third weeks, respectively (Figures 2, 3B,C). Ten months after the end of treatment, the CRHM reached 92.7% of the initial area, with no changes in size or signs of hemodynamic compromise since then (Figure 2).
Case 2. (A) Immediate postnatal transthoracic echocardiogram in apical 4-chamber projection showing a giant rhabdomyoma measuring 30 mm x 17.3 mm (area of 4.4 cm2), adhered to the interventricular septum, and occupying a large part of the right ventricular cavity. (B-D) Echocardiographic control at 2, 4, and 11 weeks of treatment with everolimus, showed a significant reduction in the size of the rhabdomyoma.
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F3
It was adhered to the interventricular septum, occupying a large part of the right ventricular cavity, limiting the opening of the tricuspid valve and displacing the interventricular septum towards the left ventricle, without outflow tract obstruction (Figure 3A). Echocardiographic follow-up showed a reduction in the mass area of 2.8%, 42.1%, and 48.9% in the first, second, and third weeks, respectively (Figures 2, 3B,C).