FIG 52.3 Heart shown from the left side A huge rhabdomyoma has grown in the anterosuperior wall of the left ventricle, compromising the ventricular cavity and producing cardiac failure Obstruction Primary cardiac tumors with intracavitary extension may cause obstruction to inflow and outflow tracts, systemic and pulmonary veins and arteries, coronary arteries, and interfere with valvar function (Fig 52.4A) The signs and symptoms are thus myriad and dependent on the chamber involved and the size and nature of the tumor They can mimic valvar disease, left- and right-sided heart failure, coronary ischemia, and cardiac tamponade All these obstructive manifestations may have a sudden onset, such as in the case of a pedunculated highly mobile left atrial tumor, which can cause syncope, sudden unexpected death, or acute pulmonary edema.1,3 Such findings are often intermittent and may relate to the posture of the patient As stated before, the picture may be further complicated by pulmonary embolism and secondary pulmonary hypertension In fetuses, obstruction is noted to happen at any stage of gestation, and obstructions to systemic venous return and/or both outflow tracts simultaneously are noted to be particularly worrisome.28 FIG 52.4 Rhabdomyoma (asterisk) of the left ventricular outflow tract with intracavitary extension in (A) diastolic and (B) systolic frames Note color flow turbulence during systole This patient was managed conservatively, with resolution of outflow tract obstruction as the tumor regressed Physical Examination In general, physical examination is nonspecific Murmurs, when present, are in themselves nonspecific, but certain atypical findings may suggest a cardiac tumor rather than primary valvar or myocardial disease Such findings include postural variation in the intensity of the murmur, and the presence of a so-called tumor “plop.” The plop has been described particularly in patients with left atrial myxoma, being characteristic of a pedunculated and highly mobile tumor This plop was heard in only approximately 16% of the patients in one large series.15 It most likely results from sudden tension on the stalk of the tumor as it prolapses during diastole into the left ventricular cavity, along with the possible impact of the mass on the ventricular wall.29 Dermatologic findings can also alert the clinician to certain types of tumors Tuberous sclerosis complex (TSC) is associated with rhabdomyomas and characteristic skin lesions such as facial angiofibromas, hypomelanotic macules, shagreen patches, and periungual fibromas.30 Carney complex is associated with atrial myxomas These patients have a variety of skin pigmentation abnormalities, including intense freckling and blue nevuses.31 Gorlin syndrome is associated with fibromas and is characterized by multiple basal cell carcinomas.1,31