Published online by Cambridge University Press: 18 April 2005
A premature infant, who was born following 35 weeks of gestation, developed respiratory distress and required ventilation with oxygen soon after birth. Cardiac examination revealed an intermittently irregular heart beat and cardiomegaly, with a grade 2/6 nonspecific ejection systolic murmur that was best heard over both sides of the lower sternal areas. Peripheral pulses were normal, and she had no signs of congestive heart failure. Intermittent premature ventricular contractions were confirmed on the electrocardiographic monitor. The cross-sectional echocardiogram, a sub-xyphoid long-axis projection of which is seen in Figure 1, showed a huge cardiac tumor all around the left ventricle, along with two small intra-cavitary tumors. The diagnosis of tuberous sclerosis was made subsequently. By seven months of age, the tumors had disappeared completely (Fig. 2).
Figure 1.
Figure 2.
Pre-natal diagnoses of rhabdomyoma are made by fetal echocardiography during routine evaluation, or when the fetus has an arrhythmia, although such an examination was not performed in this patient. A thorough knowledge of intra-cardiac tumors and their natural history1 is essential for authoritative family counseling, and to avoid inappropriate termination of pregnancy.