Giant heart tumors in infants leading to sudden, unexpected death: Description of two cases

Abstract

Primary cardiac tumors (PCT) are extremely rare in the pediatric population, with an incidence of 0.0017–0.027%. 1 More than half are diagnosed within the first year of life. 1,2 The vast majority of these tumors are benign, however some of them manifest the potential for malignant arrhythmias, which can lead to sudden cardiac death. 1 The types of heart tumors encountered in infants and children are different from those seen in adults. The most common intracardiac tumor (PCT) in pediatrics is rhabdomyoma (45%) and in adults it is myxoma. 1,3 The clinical signs and symptoms depend especially on the size and location, rather than the tumor type. 2,3 The clinical manifestations of PCT vary from asymptomatic presentations (despite enormous size) to lifethreatening cardiac events. 3 Unfortunately, they are often missed until autopsy. We report two cases of sudden infant death, both caused by a primary cardiac tumor and both diagnosed postmortem. Case report 1 A 12-month-old, previously healthy male infant died suddenly and unexpectedly, collapsing on the floor during play. His mother noticed convulsions, evidence of cyanosis, and breathing difficulties. The child quickly lost consciousness and suffered a cardiac arrest. The attempted resuscitation carried out by the mother, and then by an ambulance team, failed. The doctor observed significant anisocoria but could not determine the cause of death. Apart from sporadic infections of the upper respiratory tract, the child had not been ill. The parents had not observed anything worrying in his development or behavior. The complete medical documentation, including prenatal history, and somatic and psychomotor development, shows that the proband was the first child of the 22-year-old mother, from her first pregnancy, which developed normally and ended in the 39th week in spontaneous delivery. At birth the child weighed 4100 g, measured 57 cm, and was in good general condition, with an Apgar score of 10 in the first and subsequent minutes. After birth, apart from jaundice between the 3rd and 5th days with the maximum bilirubin concentration of 248 mmol/L, no abnormalities were observed. At that time, the infant underwent abdominal ultrasonography, which did not detect any abnormalities. Auscultation demonstrated normal heart sounds without any pathological murmurs. In subsequent months of the infant’s life only typical preventive physical examinations were conducted, which did not demonstrate any irregularities.