**2. Case report**

We present the case of a 69-year-old woman with a history of endometrial carcinoma in 1996, who underwent a total hysterectomy and bilateral adnexectomy. The patient also received chemotherapy (doxorubicin and cisplatinum) and local radiotherapy (50 Gy) because of a single lung metastasis, with total remission during later follow-up. About 10 years later following radiotherapy, she had increasing breathlessness and a new pansystolic murmur at the left sternal edge and progressed insidiously to retrograde left heart failure [1].

An ECG showed a sinus rhythm and complete right bundle branch block. A chest X-ray revealed a normal cardiac contour with upper right pleural thickening with a sequelar appearance, and blood flow redistribution consistent with pulmonary edema. A transthoracic and transesophageal echocardiogram showed thickening and retraction of the mitral valve, severe mitral regurgitation, and severe pulmonary hypertension. The left ventricular function was normal. An assessment of myocardial perfusion with SPECT and coronary angiography were normal. During follow-up, a transthoracic echocardiogram (TTE) revealed an image consistent with a primary or metastatic cardiac tumor on the posteromedial papillary muscle (**Figure 1A**, https://www.dropbox.com/s/q9mtt67rcvps82u/Movies%201%20 and%202.rar?dl=1). Cardiac magnetic resonance imaging (MRI) revealed a solid mass on the posteromedial papillary muscle with late enhancement, consistent with a primary cardiac tumor (**Figure 1B**).

During surgery, the mitral valve was thickened, and the patient underwent mitral valve replacement with an SJM #29 biological prosthesis with resection of the tumor located in the posteromedial papillary muscle. During the postoperative period, the retrograde heart failure regressed, pulmonary pressures were normalized, and the patient remained symptom-free. A pathological examination revealed the presence of a tumor mass with a core of dense connective tissue surrounded by a layer of hyperplastic endocardial cells characteristic of a papillary fibroelastoma (**Figure 2**); the mitral valve had signs of radiotherapy-induced damage with increased collagen and dysmorphic nuclei. After 8 years of follow-up, the patient remains asymptomatic.

**Figure 2.** Pathology examination. A: Panoramic image of the papillary fibroelastoma and the villi. B: With fluorescence

Actinic Papillary Fibroelastoma of the Left Ventricle http://dx.doi.org/10.5772/intechopen.81024 127

Radiosensitivity of the myocardium, pericardium, and great vessels is an issue of great concern. Previously, the heart was thought to be a radio-resistant organ; however, this theory was subsequently abandoned when late cardiac involvement was found in young patients, who

The prevalence of cardiac disease associated with radiation may clinically manifest after a very prolonged time period, and its incidence is on the rise because of the increasing survival of cancer patients. Modern treatment techniques seem to have decreased the toxicity, but long-term results that allow an assessment of its effects are still lacking. It is worth noting that survivors who do not experience recurrence of the original tumor or develop a second malignant tumor have a greater risk of cardiovascular death than the general population, and

Radiation causes progressive and irreversible damage; acute and chronic pericardial manifestations are the most frequent manifestations seen in daily practice. However, valvular lesions,

had received radiotherapy treatment due to Hodgkin's disease [2].

**3. Discussion**

in some series that risk is fivefold [3].

technique, the same papilla shows an axis of elastic fibers.

Parasternal short-axis view from a transthoracic echocardiogram showing a solid mass on the posteromedial papillary muscle consistent with a primary cardiac tumor (papillary fibroelastoma). A mild pericardial effusion and large left pleural effusion are also visible.

**Figure 1.** Transesophageal echocardiography. Longitudinal view of the left ventricle at 119° (A) and cardiac MRI (B) shows a solid mass on the posteromedial papillary muscle (arrows), measuring 21.9 × 14.6 mm with gadolinium enhancement consistent with a primary cardiac tumor. LA = left atrium; LV = left ventricle; RV = right atrium; Ao = ascending aorta.

**Figure 2.** Pathology examination. A: Panoramic image of the papillary fibroelastoma and the villi. B: With fluorescence technique, the same papilla shows an axis of elastic fibers.

During surgery, the mitral valve was thickened, and the patient underwent mitral valve replacement with an SJM #29 biological prosthesis with resection of the tumor located in the posteromedial papillary muscle. During the postoperative period, the retrograde heart failure regressed, pulmonary pressures were normalized, and the patient remained symptom-free. A pathological examination revealed the presence of a tumor mass with a core of dense connective tissue surrounded by a layer of hyperplastic endocardial cells characteristic of a papillary fibroelastoma (**Figure 2**); the mitral valve had signs of radiotherapy-induced damage with increased collagen and dysmorphic nuclei. After 8 years of follow-up, the patient remains asymptomatic.
