Mitral Prolapse Caused by Lymphangioma

Abstract

A 62-year-old female patient with known mitral-valve prolapse for the previous five years presented with progressive dyspnea and intermittent palpitations. This clinical presentation was investigated by two-dimensional echocardiography which revealed moderate mitral regurgitation due to a pedunculated mass oscillating between the left atrium and the left ventricle. Successful operative treatment consisted of en-bloc resection of the tumor from the anterior mitral valve leaflet and its primary cords and subsequent reconstruction of the mitral valve. Pathohistological examination revealed a cystic lymphangioma originating from mitral-valve tissue. To our knowledge this is the first reported case in the literature.

Cardiac lymphangioma

“…Of the remaining cases, seven lymphangiomas grew within the pericardium, in another six cases the growth was mainly localized to the atrium or ventricle, and in the remaining four cases the cyst-like tumors grew into the heart cavity. In one of these cases the tumor grew into the mitral valve [20]. Current literature cases have also described the symptoms of lymphangiomas.…”

“…In addition, the literature has discussed 17 cases of lymphangiomas localized in the heart [11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27]. From these cases, 2 cases were diagnosed as a mixed hemolymphangioma.…”

Lymphangiomatosis Involving the Inferior Vena Cava, Heart, Pulmonary Artery and Pelvic Cavity

“…Although lymphangioma can occur in any region subserved by the lymphatic system (e.g., the head, neck, axilla, retroperitoneum, spleen, colon, intra-abdominal mesentery, esophagus, mediastinum and chest wall), cardiac manifestations of this disease entity are rare (1-3). In addition, cardiac and pelvic involvement, which we refer to as lymphangiomatosis, has not been previously described.…”

“…In addition, cardiac and pelvic involvement, which we refer to as lymphangiomatosis, has not been previously described. The majority of reported cases of cardiac lymphangioma have occurred in children younger than 10 years (1-5), and symptoms appear to depend on sites and the extent of involvement, ranging from congestive heart failure, syncope, arrhythmia and palpitations to cardiac tamponade (5). …”

“…However, cardiac lymphangioma is rarely encountered (1, 2) and lymphangiomatosis involving the inferior vena cava (IVC), heart, pulmonary artery and pelvis has not been previously described. We report here a case of lymphangiomatosis that presented as a mixed cystic/solid component tumor in the IVC that traversed the right heart to the right pulmonary artery with a previous immediate history of another huge cystic mass in the pelvic cavity.…”