Ventricular fibromas in children, arrhythmia risk, and outcomes: A multicenter study

Jones, John Paul; Ramcharan, Tristan; Chaudhari, Milind; Bhole, Vinay; McLeod, Karen; Sadagopan, Shankar; Uzun, Orhan; Parry, Andrew J.; Till, Jan; McMahon, Colin J.; Stuart, Graham; Walsh, Mark A.

doi:10.1016/j.hrthm.2018.06.018

Cited by 34 publications

(17 citation statements)

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“…It remains a rare disease entity, with incomplete understanding of its natural course. Due to concern for its known association with malignant arrhythmia, surgical resection is often considered as first line treatment 3,4 . A literature review of 178 patients with cardiac fibroma report young age and septal involvement as factors indicative of poor prognosis, leading to suggested surgical resection 1 in a patient such as the present case.…”

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confidence: 99%

Fourteen-Year Follow-Up Cardiac Magnetic Resonance Imaging of a Large Septal Cardiac Fibroma

Lee

Kramer

2019

Circ: Cardiovascular Imaging

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Cardiac fibroma is the second most common primary pediatric cardiac tumor after rhabdomyoma. Although benign, it does not usually regress and may cause cardiac chamber flow obstruction, arrhythmia, coronary artery compromise, thromboembolic events, and sudden death 1, 2 . It remains a rare disease entity, with incomplete understanding of its natural course. Due to concern for its known association with malignant arrhythmia, surgical resection is often considered as first line treatment 3,4 . A literature review of 178 patients with cardiac fibroma report young age and septal involvement as factors indicative of poor prognosis, leading to suggested surgical resection 1 in a patient such as the present case. Here we present follow-up of a presumed cardiac fibroma with cardiac magnetic resonance imaging (CMR) showing unchanged absolute size, but reduced relative size in over a span of 14 years.

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confidence: 99%

Fourteen-Year Follow-Up Cardiac Magnetic Resonance Imaging of a Large Septal Cardiac Fibroma

Lee

Kramer

2019

Circ: Cardiovascular Imaging

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“…The management of asymptomatic patients with cardiac fibromas remains controversial. Surgical tumor resection is generally recommended to eliminate symptoms ( 12 , 13 ), but is also performed in many asymptomatic patients ( 7 , 21 ). Herein, 1/3 of the surgically managed patients were asymptomatic because of the concern about potentially life-threatening ventricular arrhythmias.…”

Section: Discussionmentioning

confidence: 99%

“…Rhabdomyomas have a peculiar characteristic of spontaneous regression, making the primary strategy of watchful waiting for asymptomatic patients. Patients with cardiac fibromas have a greater tendency to develop life-threatening arrhythmias, e.g., ventricular tachycardia (VT), in comparison to patients with other histotypes ( 11 , 12 ). The presence of tumor-related symptoms has been well-recognized as the indication of surgical resection of intramyocardial tumors ( 10 , 13 ).…”

Section: Introductionmentioning

confidence: 99%

Surgery for Primary Cardiac Tumors in Children: Successful Management of Large Fibromas

Qian

Yang

et al. 2022

Front. Cardiovasc. Med.

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BackgroundPediatric primary cardiac tumors (PCTs) are rare. Its clinical features and prognoses are not well defined. The management of asymptomatic patients with cardiac fibromas remains controversial.ObjectiveWe aimed to examine our experience in surgical resection of pediatric PCT, with specific focuses on the management of large fibromas.MethodsThis study included all the children who underwent surgical resection of PCT in our institution between December 2008 and June 2021. The last follow-up was performed between June 1st and August 26th, 2021. Kaplan–Meier method was used to estimate the postoperative survival, freedom from reoperation, event-free survival, and also related risk factors. The tumor volume and volume index (volume divided by body surface area) were measured for cardiac fibromas.ResultsOf the 39 patients with median operative age of 9.5 [interquartile range (IQR): 1.2–16.5] years, 35 (89.7%) had benign tumors (fibromas for 15, myxomas for 13, and others for 7). The length and volume of fibromas were independent of age and symptoms (Ps > 0.05). The fibroma volume index was negatively correlated with age (P = 0.039), with a mean value of 105 ± 70 ml/m2. Of the 15 patients with fibromas, 5 were asymptomatic, 4 received partial resection, 4 required transmural resection, and 4 presented postoperative left ventricular (LV) dysfunction (ejection fraction <50%). During the median follow-up period of 3.1 years and maximum of 12.5 years, adverse events included 2 early and 1 late death, 4 reoperations, 4 tumor recurrences, and 1 LV dysfunction lasting over one year. The 8-year survival, freedom from reoperation, and event-free survival rates were 90.4, 81.8, and 64.2%, respectively. Malignant tumor (P < 0.001) was associated with more adverse events. Transmural resection (P = 0.022) and larger tumor volume index than LV end-diastolic volume (P = 0.046) were risk factors for LV dysfunction following fibromas resection.ConclusionPediatric surgery for PCT can be performed with low mortalities and few adverse events. The size of cardiac fibroma in children relatively decreases with the increase of age. Larger tumor volume index than LV end-diastolic volume index and transmural tumor resection predicts postoperative LV dysfunction.

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“…The majority of patients were weaned off of their anti-arrhythmic therapies following resection. 9 In summary, symptomatic cardiac fibromas should be considered for complete resection due to arrhythmia burden and impedance of blood flow due to tumour mass effect. This allows for resolution of symptomatology without the need for other surgical palliative strategies or cardiac transplantation.…”

Section: Discussionmentioning

confidence: 99%

A large ventricular fibroma requiring surgical resection in a symptomatic 3-month-old infant

2019

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Ventricular fibromas in children, arrhythmia risk, and outcomes: A multicenter study

Cited by 34 publications

References 20 publications

Fourteen-Year Follow-Up Cardiac Magnetic Resonance Imaging of a Large Septal Cardiac Fibroma

Fourteen-Year Follow-Up Cardiac Magnetic Resonance Imaging of a Large Septal Cardiac Fibroma

Surgery for Primary Cardiac Tumors in Children: Successful Management of Large Fibromas

A large ventricular fibroma requiring surgical resection in a symptomatic 3-month-old infant

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