Fetal Primary Cardiac Tumors During Perinatal Period

Abstract

Fetal primary cardiac tumors are rare, but they may cause complications, which are sometimes life threatening, including arrhythmias, hydrops fetalis, ventricular outflow/inflow obstruction, cardiac failure, and even sudden death. Among fetal primary cardiac tumors, rhabdomyomas are most common, followed by teratomas, fibromas, hemangiomas, and myxomas. Everolimus, a mammalian target of rapamycin inhibitor, has been reported to be an effective drug to cause tumor remission in three neonates with multiple cardiac rhabdomyomas. Neonatal cardiac surgery for the resection of primary cardiac tumors found by fetal echocardiography has been reported sporadically. However, open fetal surgery for pericardial teratoma resection, which was performed successfully via a fetal median sternotomy in one case report, could be a promising intervention to rescue these patients with large pericardial effusions. These recent achievements undoubtedly encourage further development in early management of fetal cardiac tumors. Owing to the rarity of fetal primary cardiac tumors, relevant information in terms of prenatal diagnosis, treatment, and prognosis remains to be clarified.

Fetal Cardiac Myxomas

“…Similar to other fetal cardiac tumors that could be visible at 17 weeks' gestation [32], fetal cardiac myxomas could be detected at 18 weeks' gestation [6]. However, fetal cardiac tumors can be misdiagnosed as other types of benign tumors, such as rhabdomyomas [24] and hemangiopericytoma [32]. The distinctive features of fetal cardiac rhabdomyomas may offer differential diagnostic evidence by their predominant multiplicity, spontaneous regression, tuberous sclerosis relativity, predilections of adverse cardiac events (arrhythmias, hydrops fetalis, cardiomegaly, heart valve dysfunction, heart failure and even sudden death), whereas hemangiopericytoma was reported to arise from the right atrium and was sensitive to chemotherapy [33].…”

“…determine the presence, attachment location, involvement of heart valves and singularity or multiplicity of the tumors, which warrant careful observations by transabdominal and postnatal transthoracic multi-sectional views [31]. Similar to other fetal cardiac tumors that could be visible at 17 weeks' gestation [32], fetal cardiac myxomas could be detected at 18 weeks' gestation [6]. However, fetal cardiac tumors can be misdiagnosed as other types of benign tumors, such as rhabdomyomas [24] and hemangiopericytoma [32].…”

Fetal cardiac rhabdomyomas treated with maternal sirolimus

“…Primary fetal cardiac tumors are rare with an incidence of approximately 0.14%. The majority of these tumors are classified as benign and include rhabdomyomas, teratomas, fibromas, hemangiomas, and myxomas. The most common among these tumors are rhabdomyomas .…”

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“…Aun así, todos estos tumores requieren vigilancia estrecha, sobre todo los de localización o dimensiones que pueden alterar la hemodinamia, producir disritmias o insuficiencia cardiaca durante el periodo fetal y posnatal temprano (estos son los únicos casos susceptibles de intervención quirúrgica). [9][10][11][12][13] El objetivo de este trabajo es reportar la primera serie de casos de detección prenatal de rabdomiomas. 14,15,16…”