To the editor:
A 3-year-old male child presented to our hospital with a known diagnosis of intra-myocardial mass. The child was diagnosed with an intramyocardial mass during the 7th month of gestation and was delivered with normal vaginal delivery at full term. He had normal developmental history. The patient was prescribed tablet propranolol, in previous consultations at another hospital. The child had gradually worsening dyspnoea on exertion since a year before presentation. At presentation, transthoracic echocardiography showed significantly hypertrophied inter-ventricular septum (IVS), severe right ventricular outflow tract (RVOT) obstruction with a mean pressure gradient of 80 mmHg at rest, and left ventricular outflow tract (LVOT) narrowing. Magnetic resonance imaging (MRI) showed a well-defined enhancing lesion involving the basal, mid, and anteroseptal regions of the IVS, and the anterior wall of the right ventricle (RV) which was isointense on T1W and mildly hyperintense on T2W/STIR sequences. The lesion measured 4.16 cm × 2.59 cm × 4.36 cm. In comparison with the previous MRI, an increase in the size of the lesion and grade of RVOT obstruction were noted. In the operation theatre, after induction and intubation, trans-oesophageal echocardiography (TEE) was done. The mass was located in the basal, mid, and anteroseptal regions of the IVS and anterior RV wall, was echogenic in appearance, and measured 4.57 cm × 2.36 cm as evident in [Figures 1a-c]. The mass over the anterior wall of RV and IVS was excised, followed by reconstruction of the RVOT by a pericardial patch. Through a transaortic incision, some more of the IVS mass was excised, to relieve LVOT narrowing. The IVS mass was not amenable for complete excision. The tissue was sent for histopathological evaluation. After excision of the mass, the pressure gradient (peak/mean) across the LVOT was 10.7/5.48 mmHg and that across the RVOT was 8.67/5.12 mmHg on TEE. TEE provided exact information about the size, site, and extent of the mass. The mass was diagnosed as arteriovenous hemangioma [Figure 1d]. The patient was extubated on the second postoperative day and his course was uneventful till discharge. The patient regularly reported for follow-up, and the recent assessment after 1 year shows that there is no change in the size of the mass and the pressure gradients across LVOT and RVOT. At present, the patient has normal physical activity and does not require any medication.
Figure 1.
(a) Mid-oesophageal four chamber view; (b) Mid-oesophageal AV long-axis view; (c) Trans-gastric mid short-axis view (white arrows pointing at the mass involving the interventricular septum and the anterior wall of the right ventricle); (d) Histopathological image of the cardiac arteriovenous hemangioma
Cardiac hemangiomas (classified as cavernous, arteriovenous, and capillary types) account for <2% of all cardiac tumors and are diagnosed incidentally.[1,2] Hemangiomas were found in the IVS in 6 out of 56 cases in one report.[3] Hemangiomas may present in the 5th decade of life.[4] In this case, the hemangioma was not diagnosed incidentally as the patient presented with dyspnoea on exertion. Cardiac hemangiomas have been known to cause arrhythmia, pericardial effusion, congestive heart failure, coronary insufficiency, and sudden death but those were absent in our patient.[5] Due to clinical symptoms and growth appreciated on serial MRI, surgical excision was planned. Surgical resection is the treatment of choice for symptomatic lesions.[6] The patient’s post-operative period was uneventful and there is no significant growth in the size of the mass on serial follow-up since surgery. MRI is highly accurate and provides dimensions and extent of the tumor. TEE is an extremely useful modality to determine the size and extent of an intracardiac lesion, which may also guide the surgical excision. In this case, early age of presentation, gradually worsening symptoms, and the rare occurrence of the hemangioma (in the IVS) were the distinguishing features of the hemangioma.
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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
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