Fetal cardiac tumors: prenatal diagnosis and outcomes

Carrilho, Milene Carvalho; Tonni, Gabriele; Júnior, Edward Araujo

doi:10.5935/1678-9741.20150003

Cited by 11 publications

(19 citation statements)

References 8 publications

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“…Although FPCTs are extremely rare and most are benign, they may cause intracardiac flow obstruction, heart valve insufficiency, arrhythmia, heart failure, and hydrops fetalis, or even fetal death. 5 These tumors tend to appear between 20-and 30-week gestation, and the earliest diagnosis was at 17 weeks. Echocardiography plays an important role in the prenatal diagnosis of neoplasmas and in the guidance of in utero pericardiocentesis.…”

Section: Diagnostic Evaluation and Differential Diagnosismentioning

confidence: 99%

“…4 Although the incidences of various types of tumors varied from fetuses to neonates and children, cardiac rhabdomyomas, teratomas, and fibromas remained the three most common types. 5 Nevertheless, fetal information was always limited (Figure 1). Murmurs, cyanosis, arrhythmias, and cardiac failure are the major presenting signs of FPCTs during the perinatal period.…”

Section: Introductionmentioning

confidence: 99%

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Fetal Primary Cardiac Tumors During Perinatal Period

Yuan

2017

Pediatrics & Neonatology

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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.

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Section: Diagnostic Evaluation and Differential Diagnosismentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Fetal Primary Cardiac Tumors During Perinatal Period

Yuan

2017

Pediatrics & Neonatology

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“…In echocardiographic examination cardiac tumors were morphologically homogenous, hyperechogenic with benign and regular contours and oval shape, likewise in Carvalho et al publication 9 . Unregular shape and echogenicity occured in our series, in teratoma tumor case (patient nr 5, Table 1).…”

Section: Discussionmentioning

confidence: 86%

“…In comparison to others, teratoma occurs not in the heart, but with close contact with aorta or pulmonary artery 11,12 . Fetuses with teratoma are presenting various clinical symptoms, from asymptomatic, to mild pericadial effusion up to hydrops fetalis.…”

Section: Hydrops Testismentioning

confidence: 99%

Single Fetal Cardiac Tumors and Follow-Up Based on 13 Cases from the Fetal Cardiac Referral Center in 1993-2017

Żalińska

Korabiewska

Krekora

et al. 2017

Prenatal Cardiology

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Single fetal cardiac tumors and follow-up based on 13 cases from the Fetal Cardiac Referral Center in Prenat Cardio 2017 Jan; 7(1):43-49 DOI: 10.1515/pcard-2017-0007 INTRODUCTIONFetal cardiac tumors are rare anomalies: 0,08-0,2% 1,2, while data from Polish Nationwide Register of Fetal Cardiological Problems (in polish-ORPKP), suggested higher prevalence (1,05% Single fetal cardiac tumors are some sort of enigma and for now, there is no unitary algorythm of proceeding, that is why it become the aim of our work. MATERIALThe analysis included 13 cases from our unit Fetal Cardiology Department -Referral Center in the middle of Poland, during the years 1993-2017. Study group were women at the age of 18-37 years, average gestational age during targeted echocardiography examination was 34 weeks of pregnancy. The earliest registration of cardiac tumor was in 20th week of pregnancy, and the latest was during perinatal period (40th week of pregnancy).Corresponding author : a.b.zalinska@gmail.com Submitted: 2017-11-16; accepted: 2017-11-25 Results: Ten out of 13 fetuses with single cardiac tumors (SFCT) had cardiomegaly: on average HA/CA was 0,49. The size of the tumor was different: the smallest one -6 x 6 mm, the biggest 47 x 47 mm. The way of the delivery: in 10 cases there was CS and in 3 cases natural delivery. Birth weight was from 2000-3950 g (average 2989,2 g). Cardiosurgical resection of the tumor was performed on 4 newborns: at 2nd, 4th, 8th and 16th day of life (average 7,5 day). Four neonatal deaths were registered (31%): in 1st 2nd and 11th day (before surgery) and in the 28th day after the operation.Conclusions: Single fetal cardiac tumors (SFCT) can be diagnosed at 20 weeks of pregnancy, which allows to start echocardiographic monitoring, taking into consideration the potential risk of hemodynamic progression. SFCT can be the first sign of tuberous sclerosis complex in later prenatal or postnatal life. SFCT other than rhabdomyoma can be asymptomatic in newborn, but may require an early cardiosurgical resection.

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“…The rhabdomyomas are the most common type of cardiac tumours in foetuses, newborns and children 1 . Presence of multiple cardiac rhabdomyomas is one of the major features of tuberous sclerosis (TSC) and they can often be detected in the prenatal period.…”

Section: Introductionmentioning

confidence: 99%

Single giant mediastinal rhabdomyoma as a sole manifestation of TSC in foetus

Godava¹,

Filipová²,

Dubrava³

et al. 2017

BIOMED PAP

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Background. Presence of multiple cardiac rhabdomyomas is one of the major features of Tuberous sclerosis (TSC), but isolated progressing single giant rhabdomyoma is very rare and not typical of TSC. Case report. This report presents family without obvious history of TSC with occurrence of giant mediastinal rhabdomyoma affecting the haemodynamics in male foetus, without other TSC symptoms. Girl from the next gravidity had prenatally detected multiple rhabdomyomas and small subcortical tuber of brain detected after birth. DNA analysis found novel c.4861A>T TSC2 variant and large deletion in TSC2 in tumour tissue from male foetus. The novel TSC2 variant was also present in the girl and her healthy father, in silico analysis suggested its functional effect on TSC2. Brain MRI of the father detected mild TSC specific abnormality. Conclusion. We suggest the novel TSC2 mutation is a cause of mild TSC in this family and has reduced expression. The clinical and molecular findings in this family also emphasize that TSC diagnosis should be also evaluated in case of single giant foetal cardiac rhabdomyoma.

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Fetal cardiac tumors: prenatal diagnosis and outcomes

Cited by 11 publications

References 8 publications

Fetal Primary Cardiac Tumors During Perinatal Period

Fetal Primary Cardiac Tumors During Perinatal Period

Single Fetal Cardiac Tumors and Follow-Up Based on 13 Cases from the Fetal Cardiac Referral Center in 1993-2017

Single giant mediastinal rhabdomyoma as a sole manifestation of TSC in foetus

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