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      A Mass Causing Right Ventricular Outflow Obstruction - A Dreadful Complication

      case-report

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          Abstract

          The most common cardiac tumour in the pediatric age group is rhabdomyoma. These are usually located in the ventricles, either in the ventricular septum or free wall. Cardiac tumours in early infancy may lead to severely compromised blood flow due to inflow or outflow tract obstruction. The diagnosis of cardiac rhabdomyoma can be established by transthoracic echocardiography (TTE). Rhabdomyomas have a natural history of spontaneous regression; surgical intervention is reserved for patients with symptoms of severe obstruction or hemodynamic instability. In this study, a case of two-year old child who presented with failure to thrive and underwent excision of pedunculated mass from the right ventricular outflow tract was reported.

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          Most cited references9

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          Cardiac tumours in children

          Cardiac tumours are benign or malignant neoplasms arising primarily in the inner lining, muscle layer, or the surrounding pericardium of the heart. They can be primary or metastatic. Primary cardiac tumours are rare in paediatric practice with a prevalence of 0.0017 to 0.28 in autopsy series. In contrast, the incidence of cardiac tumours during foetal life has been reported to be approximately 0.14%. The vast majority of primary cardiac tumours in children are benign, whilst approximately 10% are malignant. Secondary malignant tumours are 10–20 times more prevalent than primary malignant tumours. Rhabdomyoma is the most common cardiac tumour during foetal life and childhood. It accounts for more than 60% of all primary cardiac tumours. The frequency and type of cardiac tumours in adults differ from those in children with 75% being benign and 25% being malignant. Myxomas are the most common primary tumours in adults constituting 40% of benign tumours. Sarcomas make up 75% of malignant cardiac masses. Echocardiography, Computing Tomography (CT) and Magnetic Resonance Imaging (MRI) of the heart are the main non-invasive diagnostic tools. Cardiac catheterisation is seldom necessary. Tumour biopsy with histological assessment remains the gold standard for confirmation of the diagnosis. Surgical resection of primary cardiac tumours should be considered to relieve symptoms and mechanical obstruction to blood flow. The outcome of surgical resection in symptomatic, non-myxomatous benign cardiac tumours is favourable. Patients with primary cardiac malignancies may benefit from palliative surgery but this approach should not be recommended for patients with metastatic cardiac tumours. Surgery, chemotherapy and radiotherapy may prolong survival. The prognosis for malignant primary cardiac tumours is generally extremely poor.
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            Pediatric primary benign cardiac tumors: a 15-year review.

            An increase in the incidence of primary cardiac tumors has been reported since the development or enhancement of noninvasive imaging modalities. We identified 56 children with primary cardiac tumors. Forty-four (78%) children had rhabdomyomas, 6 (11%) fibromas, 1 (2%) pericardial teratoma, 1 (2%) epicardial lipoma, 1 (2%) multicystic hamartoma, and 3 (5%) unspecified tumors. The mean age at diagnosis was 19 +/- 35 months (median 4.7 months, range 0.03 to 204 months), excluding 12 patients who were given the diagnosis before birth. Among 27,640 patients assessed for cardiac disease, the incidence of tumors was 0.06% (1980 to 1984), 0.22% (1985 to 1989), and 0.32% (1990 to 1995). Diagnosis was made in 55 of 56 patients by echocardiography. Catheterization was performed in five patients and magnetic resonance imaging in nine. No tumor-related deaths occurred. Nine patients had surgery because of hemodynamically significant obstruction or arrhythmias. Partial or complete regression occurred in 24 (54%) of 44 patients with rhabdomyomas. Overall, the prognosis was excellent. Individualized surgery allowed early safe treatment of symptomatic tumors.
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              Cardiac rhabdomyomas and obstructive left heart disease: histologically but not functionally benign.

              Cardiac rhabdomyoma is the most common primary heart tumor in infants. Spontaneous regression of such tumors is common, particularly with smaller lesions, followed by resolution of symptoms. Based on our data on spontaneous involution, our institutional philosophy has been one of expectant management in the absence of life-threatening symptoms. However, surgical intervention sometimes is required for the extirpation of a rhabdomyoma from the left ventricular outflow tract. A retrospective review was conducted of 30 children in whom a rhabdomyoma was diagnosed over a 27-year period. Twenty-three percent (7/30) of the children required surgical extirpation of the tumor from within their left ventricular outflow tract, although a total of 94% had left ventricular involvement. There were no deaths. To date, no child has required reexcision of tumor. The natural history of rhabdomyoma is one of spontaneous regression (the 23 children who did not undergo surgical intervention are alive and continue to be followed up medically). We recommend surgical excision to alleviate acute outflow tract obstruction with reliance on the tumor's natural history of regression to achieve long-term freedom from reoperation. Although operation has been recognized as lifesaving, we were somewhat surprised to find that greater than 20% of our pediatric population required operative intervention.
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                Author and article information

                Contributors
                Role: MSRole: MCh (CTVS)Role: DNB (CTS)Role: MNAMS
                Role: MDRole: MS
                Journal
                Braz J Cardiovasc Surg
                Braz J Cardiovasc Surg
                rbccv
                Brazilian Journal of Cardiovascular Surgery
                Sociedade Brasileira de Cirurgia Cardiovascular
                0102-7638
                1678-9741
                Nov-Dec 2019
                Nov-Dec 2019
                : 34
                : 6
                : 772-774
                Affiliations
                [1 ]Department of Cardiothoracic and Vascular Surgery, Advanced Cardiac Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
                [2 ]Department of Cardiothoracic and Vascular Surgery, Advanced Cardiac Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
                Author notes
                Correspondence Address: Rupesh Kumar, https://orcid.org/0000-0002-6864-0237, Department of Cardiothoracic and Vascular Surgery, Institute of Medical Education and Research, Chandigarh, India. Room 4018, 4th Floor - Zip Code: 160012. E-mail: rkctvs@ 123456gmail.com
                Author information
                http://orcid.org/0000-0002-6864-0237
                Article
                10.21470/1678-9741-2018-0314
                6894037
                31793255
                454b9807-b0ad-4cd0-8d01-555e0cd7dd21

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 21 August 2018
                : 14 October 2018
                Categories
                Case Report

                heart neoplasms - surgery,rhabdomyoma,ventricular septum,echocardiography,hemodynamics

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