Prophylactic resection of an incidental right ventricular fibroma in a child

Cardiac fibromas are rare lesions which occur more often in infants and children than in adults. These tumors are benign proliferations of connective tissue most often found in the left ventricular myocardium or septum. In an 8-month-old infant with cyanosis and progressive exertional dyspnea, a huge cardiac tumor obstructing the right ventricular outflow tract (RVOT) was diagnosed by means of 2-dimensional echocardiography and cardiac catheterization. At surgery, a whitish gray solitary tumor measuring 5.0 x 4.5 cm could be well visualized. It was nearly totally resected, and the RVOT was reconstructed with an Equine pericardial patch. Histologic examination classified the tumor as a fibroma. Although surgical mortality in cardiac fibroma with RVOT obstruction is extremely high, early diagnosis and prompt excision of the tumor is mandatory in relieving its dangerous symptoms.

Cardiac fibromas are primarily detected in infants and children but are extremely rare in adults. We report a case of a cardiac fibroma in a 37 year old male and review the literature on this subject. The patient was referred for evaluation of a cardiac mass. Echocardiography and computed tomography examinations revealed it as an inhomogeneous mass with calcifications. Subsequently, the lesion was completely excised and confirmed to be a cardiac fibroma. Cardiac fibromas account for only a very small percentage of all cardiac tumors. The occurrence and severity of symptoms caused by cardiac fibromas are primarily determined by the sites and size of lesions. Imaging techniques are very sensitive in diagnosing cardiac fibromas, defining the extent and planning surgical approach. Surgical treatment of cardiac fibromas gives excellent early and late survival. Although cardiac fibromas are benign, their behavior is unpredictable. Surgery appears to be the optimal treatment for patients with resectable tumors.

Primary cardiac fibroma is exceedingly rare. This condition involves a significant risk of life-threatening arrhythmias during follow-up and its prognosis is not as favourable as other benign tumours. We report a case of cardiac fibroma that was preoperatively diagnosed with echocardiography and magnetic resonance imaging. This fibroma was excised early as a preventative measure to avoid sudden death. A 46-year-old woman presented to our hospital with a 1-year history of chest tightness at rest. Echocardiography showed a large, isoechoic, well-circumscribed mass within the left ventricular myocardium with calcified tissue. Magnetic resonance imaging showed an intramural ventricular mass with iso signal intensity on T1-weighted imaging and low-signal intensity on T2-weighted imaging. There was no enhancement on first-pass perfusion imaging and homogeneous hyperenhancement on late gadolinium enhancement imaging. These features suggested a diagnosis of cardiac fibroma. Complete resection was performed to avoid sudden death and pathological analysis confirmed the tumour as cardiac fibroma. The patient was discharged 9 days after surgery and remains disease-free 5 months after surgery. Cardiac fibroma is a pathologically benign tumour with an unfavourable prognosis because of lethal arrhythmias, which can be controlled by its resection. Thus, it is important to preoperatively distinguish cardiac fibroma from other benign tumours, in order to prioritize surgical intervention for those with cardiac fibromas. Preoperative diagnosis with echocardiography and magnetic resonance imaging and early preventative surgery are the keys to improve prognosis of patients with cardiac fibromas.

Surgical Considerations for Large Asymptomatic Cardiac Fibromas in the context of Fatal Ventricular Arrhythmias

Cardiac fibromas (CF) are the second most common cardiac tumors in children. They can be aggressive tumors despite their benign histopathologic nature, accounting for the highest mortality rate among primary cardiac tumors. CF usually show a progressive growth and spontaneous regression is rare. Therefore, a complete surgical excision is the preferred therapeutic approach when patients become symptomatic or if mass-related life-threatening complications are anticipated, even in asymptomatic patients. However, some cases are not good candidates for surgical excision due to the impossibility of preserving a normal cardiac anatomy or function after the tumor resection. Orthotopic heart transplantation (OHT) can be an exceptional but adequate alternative for some giant unresectable CF in children. In this article, we report our experience with the case of a 7-month-old infant with a giant unresectable cardiac fibroma who was successfully managed through OHT.

Clinical Impact of Cardiac Fibromas

Cardiac fibromas are rare benign tumors usually seen in the pediatric population. Generally, long-term survival after surgical resection is favorable, and recurrence of fibroma has been hardly reported. Herein, we report a case of a 34-year-old woman who presented with ventricular tachycardia 21 years after resection of a cardiac fibroma and was found to have a recurrent giant cardiac fibroma. We performed a complete resection of the recurrent fibroma. At the 2-year follow-up, she remains asymptomatic with no evidence of ventricular tachycardia or recurrence of fibroma.

Conservative management of left ventricle cardiac fibroma in an adult asymptomatic patient

Cardiac fibromas are rare primary cardiac tumors. Although they constitute a small portion of cardiac neoplasms, they are the most common primary cardiac neoplasm group in childhood after rhabdomyomas. Cardiac fibromas, composed of connective tissue and fibroblasts, are benign. They may be asymptomatic, cause symptoms such as intracavitary obstruction, coronary artery compression, thromboembolic events, and conduction defects, or result in sudden death. Approximately 180 to 200 cases of cardiac fibromas have been reported in the literature, and most are located in the ventricles. Since fibromas are rare, optimal treatment strategies are unclear. Surgical excision is recommended for tumors that cause arrhythmias, heart failure, or intracavitary obstruction. This video article presented the surgical excision of a 6.2×5×4.3 cm cardiac fibroma originating from the anterior wall of the right ventricle in an 11-month-old infant. Postoperative transesophageal echocardiography showed that the tumor was completely removed and that the right ventricular and tricuspid valve functions were good.

Primary cardiac tumors are exceedingly rare in the pediatric population, with cardiac fibromas (CFs) representing the second most common benign neoplasm. Although histologically benign, these intramural tumors frequently precipitate life-threatening, intracavitary obstruction, and sudden cardiac death. We present the case of a 9-year-old male who presented with exercise-induced syncope and was found to have a large, arrhythmogenic interventricular septal fibroma. The patient underwent successful complete surgical excision with patch reconstruction, resulting in immediate resolution of his arrhythmia burden. However, 5 years postoperatively, he developed a late, infiltrative recurrence at the patch margin, precluding complete re-resection. Despite partial debulking and implantable cardioverter-defibrillator placement, the patient experienced progressive left ventricular dysfunction and refractory heart failure, ultimately succumbing to the disease at age 19 while awaiting heart transplantation. This case underscores the unpredictable long-term biological behavior of cardiac fibroma. It highlights the critical diagnostic role of multimodal imaging, the surgical challenges of septal reconstruction, and the rare but devastating potential for late recurrence. Furthermore, it emphasizes the necessity of lifelong surveillance and the complex interplay between repeated surgical interventions, chronic arrhythmia, and progressive myocardial failure in pediatric patients with recurrent cardiac fibromas.

Primary cardiac tumors in children are very rare and may be associated with severe arrhythmias and sudden infant death syndrome. These cardiac arrhythmias vary depending on the location and size of the tumor. Sixty-four percent of children with cardiac fibroma, the second most common benign cardiac tumor in children, have ventricular arrhythmias, affecting therapeutic management and risk profile of these children. We report on two siblings with cardiac fibromas whose clinical presentations differed depending on their locations and size of the tumors. The first child, a three-year-old girl, was diagnosed with a cardiac fibroma in the left ventricle at the age of 8 months after surviving resuscitation due to ventricular fibrillation. Secondary prophylactic implantation of an ICD was performed. On propranolol, no further malignant arrhythmias have occurred to date. The seven-month-old brother was diagnosed postnatally with a cardiac tumor adjacent to the right ventricle. A few weeks after birth, the boy had refractory supraventricular tachycardia and ventricular arrhythmia that only resolved with amiodarone. In genetic testing, Gorlin–Goltz syndrome was diagnosed in both children. Conservative pharmacological therapy is a therapeutic strategy for asymptomatic patients with cardiac fibromas. The anti-arrhythmic medication depends on the location of the tumor. Implantation of an ICD should be performed in cases of malignant arrhythmias. In rare cases, there is an association between cardiac tumors and genetic syndromes, such as Gorlin–Goltz syndrome. These should always be considered when such a tumor is diagnosed.

Nevoid basal cell carcinoma syndrome (NBCCS), also referred to as Gorlin's syndrome, is an autosomal dominant inherited condition that predisposes affected individuals to various tumors such as cardiac fibromas. Though technically benign, cardiac fibromas may result in malignant arrhythmias and sudden death. The pertinent literature pertaining to pediatric cases of cardiac fibromas and their clinical features were reviewed. We present the case of an asymptomatic teenage with de novo NBCCS who was diagnosed with both NBCCS and cardiac fibroma later in life. The patient was noted to have clinically significant ventricular arrhythmias that were eliminated with tumor resection. There are no established best practice guidelines for the management of cardiac fibromas in patients with NBCCS. Given the risk of sudden arrhythmic death, the presence of ventricular arrhythmias should prompt strong consideration of tumor resection.

ABSTRACTObjective:Pediatric cardiac tumors are rare and, among them, 90% are benign. Cardiac fibroma is the second most frequent tumor, after rhabdomyoma. The objective of this study is to report a case of cardiac fibroma diagnosed incidentally in a patient admitted with acute viral bronchiolitis.Case description:A 5-month-old male infant was admitted to the pediatric emergency department with acute viral bronchiolitis requiring hospitalization. He presented a detectable respiratory syncytial virus in oropharyngeal swab, blood test with lymphocytosis and a chest radiography revealed cardiomegaly. Further cardiologic testing was performed detecting elevation of cardiac biomarkers, an electrocardiogram with alteration of left ventricular repolarization and echocardiogram with a heterogeneous mass in the left ventricular, with areas of calcification. A chest angiotomography suggested rhabdomyosarcoma or cardiac fibroma and a magnetic resonance showed a mass, with characteristics suggesting fibroma. The final diagnosis was made after two cardiac catheterizations for biopsy of the lesion, confirming cardiac fibroma by anatomopathological examination. Because the patient had moderate to severe systolic dysfunction, he was submitted to heart transplant.Comments:One third of cardiac fibromas are asymptomatic, generally diagnosed late through tests ordered for other reasons. The gold-standard test for definitive diagnosis is biopsy. Cardiac fibroma usually does not present spontaneous regression and, in most cases, partial or total surgical resection is necessary. When tumors are unresectable, heart transplantation should be indicated. It is essential to have detailed characterization of the cardiac mass to establish the most appropriate therapeutic approach for each patient.

EDITOR: In the present day practice of anaesthesia, unnecessary laboratory tests in healthy individuals during preoperative preparation are usually avoided. Especially in children, the prevalent view is not to perform preoperative laboratory tests before minor procedures provided that both personal and family history, and clinical examination are normal. The case of an apparently healthy five-and-a-half year old male, body weight 19 kg, who exhibited intraoperative electrocardiographic (ECG) disorders that finally led to the diagnosis of a heart tumour is presented. The patient had no abnormal perinatal history and was the third child of apparently healthy parents. He was scheduled for tonsillectomy and ade-noidectomy. The child's medical history included an episode of bronchiolitis at 18 months of age and an episode of laryngospasm at 30 months, both of which responded to treatment. Preoperative clinical examination showed nothing abnormal apart from a mild spinal scoliosis and mild nasal congestion. The child had no symptoms or signs to indicate a heart disorder and therefore a preoperative ECG was not performed. A chest radiograph and a complete blood count were performed with normal findings. Thirty minutes before induction of anaesthesia, midazolam 8 mg was given orally for premedication. Anaesthesia was provided with propofol 50 mg, fentanyl 0.05 mg, and maintained with N2O/O2 (60/40) and sevoflurane 1.0-1.5%. Atracurium 10 mg was given for muscular relaxation and a plain 5.5 mm endotracheal tube inserted. Acetaminophen (paracetamol) 400 mg was given rectally for postoperative analgesia. Intraoperative monitoring included continuous ECG (leads II and V5), pulse oximetry (SPO2), non-invasive blood pressure (NIBP) and capnography (ETCO2). The ECG showed a normal sinus rhythm of 120 beats min−1. During surgery, three episodes of wide complex tachycardia of 140-170 beats min−1 occurred, each lasting less 1 min. Heart rate decreased to 120 beats min−1 automatically followed by negative T-waves for several seconds and then reverted to sinus rhythm. These episodes were unrelated to surgical or anaesthetic manipulations such as intubation or mouth gag placement. Neither blood pressure nor SPO2 were affected during these episodes. The operation was successfully completed and the child was fully awake and extubated after atropine 0.2 mg and neostigmine 1 mg had been given for antagonism of the residual effects of neuromuscular blockade. On departure from the operating room, the patient's vital signs were: heart rate, 110 beats min−1; blood pressure, 90/60 mmHg; and SPO2, 100%. The child was transported to the postanaesthesia care unit (PACU) with a heart rate of 110 beats min−1. Twenty minutes later, he suddenly developed supraventricular tachycardia of 180 beats min−1 with wide QRS, which was primarily treated with lidocaine 20 mg, and fentanyl 0.05 mg was given intravenously (i.v.). Because the dysrhythmia persisted, a bolus of amiodarone 50 mg was given i.v. followed by an infusion at 10 mL h−1 (50 mg in NaCl 0.9% 250 mL). The heart rate finally reduced to 110-120 beats min−1. Although the postanaesthetic condition of the child was extremely good and despite the fact that the amiodarone infusion was continued, there were many episodes of non-persistent supraventricular tachycardia (140 beats min−1) followed by a few ventricular extrasystoles during the child's stay in the PACU (Figs 1-3).Figure 1: Patient's ECG during an episode of tachycardia (144 beats min−1).Figure 2: ECG 7 min later.Figure 3: ECG 12 min later.The next day, the child was transported to the Cardiology Department at Hippokratio Children's General Hospital. The amiodarone infusion was discontinued 18 h later because of bradycardia. Amiodarone was replaced by propranolol 25 mg four times daily. At that time, the ECG showed negative T-waves in lead II, aVF, V4, V5, biphasic T-waves in V6 and positive T-waves in aVR. The two-dimensional echocardiogram revealed a large echogenic mass involving the apex of the left ventricle and the apical portion of the intraventricular septum. No left ventricular outflow tract obstruction or pericardial effusion was observed and the left ventricular systolic function was satisfactory. Magnetic resonance imaging (MRI) also showed a large mass (5.6 × 3.7 × 3.4 cm) involving the inferoseptal and apical wall of the left ventricle, probably a fibroma (Fig. 4). Seven months later, the child was successfully operated on in the Children's Hospital, Boston, MA, USA, and the tumour was completely removed. The operating findings confirmed a huge fibroma (5.4 × 4.5 × 2.7 cm), which was extended to the endocardium on both right and left ventricles and the posterior apical septum. The child is now in a very good physical condition. Although he is under oral medication consisting of amiodarone 100 mg daily and atenolol 12.5 mg daily, his ECG presents permanent disorders such as negative T-waves in lead II, aVF and in V3-V6.Figure 4: Transoesophageal echocardiography image showing a large tumour involving the inferoseptal and apical wall of the left ventricle. Reproduced with the kind permission of the Children's Hospital, Boston, MA, USA.Primary heart tumours are uncommon in all age groups, with an incidence rate of 0.0017% in a large autopsy series reviewed by Straus and Merliss [1]. Primary heart tumours are extremely rare in infants and children under 16 yr of age [2]. Nadas and Ellison [3] reviewed the large experience of the Boston Children's Hospital and cited a frequency of 0.027% among 11 000 paediatric autopsies. These studies were all carried out before the routine application of cross-sectional electrocardiography. According to data from 1980 to 1995 from the Hospital for Sick Children at Toronto, Canada [4], 27 640 subjects (from fetuses to patients of 18 yr of age) were referred for cardiac disease, and 56 of these had a primary cardiac tumour. The most common primary heart tumours that occur in children are rhabdomyomas and cardiac fibromas [2,5]. Cardiac fibromas are histologically benign but potentially lethal. Clinical signs and symptoms depend largely on the location and size of the tumour. Symptoms might include heart failure, arrhythmias (supraventricular or ventricular tachycardia is a known complication of these tumours and may cause sudden death) [6], murmurs, sudden death, cyanosis and chest pain. Fibromas can present as intramural or intracavitary masses. The left ventricular free wall and the septum are most often involved [7]. Right ventricular fibromas are rare. Occasionally, a cardiac fibroma might grow to an enormous size while the patient is asymptomatic. In a retrospective review of the files of the Armed Forces Institute of Pathology between 1967 and 1993, 23 cases of cardiac fibromas were identified. Two were incidental findings [8]. The present case made us reconsider the need for further preanaesthetic investigation in children in order to detect similar cases before a scheduled operation. The current data suggest that there is no need for preoperative ECG if there are no abnormal findings from clinical examination. Additionally, the possibility of a preanaesthetic diagnosis of such a disorder by a single ECG is very small. Further, establishing echocardiography as a routine preanaesthetic evaluation is extremely expensive. This fact, in addition to the rarity of such disorders, led us to the decision of maintaining the same practice of avoiding unnecessary examinations. Although there is no doubt that such rare cases may cause major problems during anaesthesia, it was considered that it would be rather unusual for the anaesthesiologist to face medico-legal consequences because the vast majority would not perform such investigations in children for routine surgery merely on the off-chance of detecting an extremely rare condition. A. Leontidou K. Papadopoulou K. Vlachtsis A. Douvantzi A. Zambouri P. Petropoulou Departments of Anaesthesiology, Paediatrics and Laryngology; Papageorgiou General Hospital; Thessaloniki, Greece

Hybrid Procedure for Obstructive Neonatal Left Ventricular Tumors