European Heart Journal Advance Access originally published online on September 11, 2006
European Heart Journal 2007 28(5):521; doi:10.1093/eurheartj/ehl238
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A rare case of single giant cardiac hamartomas in an adult patient
1 Department of Cardiology, Evangelic Foundation, Naples, Italy
2 Department of Cardiology, Second University of Naples, Via G. Martucci 35, 80121 Naples, Italy
3 Department of Cardiology, Monaldi Hospital, Naples, Italy
* Corresponding author. Tel: +39 081 7618525; fax: +39 081 7145205. E-mail address: adandrea{at}napoli.com
We describe a 48-year-old man who was referred to our hospital because of increasing dyspnoea and fatigue. He had no comorbidities or history of cardiovascular disease. Physical findings at admission were within normal limits, except for a pulse rate of 105 b.p.m. The 12-lead ECG showed diffused ST depression, with T-wave inversion in V1–V6 leads. Chest radiography revealed cardiomegaly and pleural and pericardial effusions. At echocardiography, a solid hyperechoic mass located in the apical and lateral left ventricular myocardium, protruding into and deforming the ventricular chamber, was evidenced (Panels A and B). In addition, moderate circumferential pericardial effusion was evident. Cardiac-gated magnetic resonance confirmed the presence of a mass located at lateral wall and at the apex of the left ventricle (Panels C–E). The size of the mass was estimated to be 4.5 x 5 cm2. There was no evidence of an extra-cardiac primary site or distant metastasis. A biopsy of the cardiac tumour, guided by echocardiography, showed a tumour cytologically similar to a hamartoma. In fact, it was composed of enlarged, vacuolated cells with sparse cytoplasm that resembled altered myocytes. Cells stained strongly with periodic-acid Schiff stains because of their high glycogen content. ‘Spider cells’, characterized by a centrally located nucleus with radial extensions to the cell periphery, were evidenced (Panel F). The patient remains alive and asymptomatic after 18 months of follow-up.
Cardiac hamartomas are a rare type of benign tumour affecting the heart. They represent up to 90% of cardiac tumours in infants and children and are usually discovered in patients <1 year of age. Although rare, such benign tumours of skeletal and cardiac muscles can occur in the adult. In the heart, they may be associated with the syndrome of tuberous sclerosis in 50–86% of cases. Because the majority of cardiac rhabdomyomas regress spontaneously, surgery is not routinely required. However, patients with life-threatening symptoms, usually those secondary to left ventricular outflow tract obstruction or refractory arrhythmias, respond well to surgical excision.
Panel A. Transthoracic echocardiography. Parasternal short-axis view. A solid hyperechoic mass located in the apical and lateral left ventricular myocardium, protruding into and deforming the ventricular chamber, is evidenced (arrows). In addition, moderate circumferential pericardial effusion is evident.
Panel B. Transthoracic echocardiography. Apical two-chamber view. A solid hyperechoic mass located in the apical and lateral left ventricular myocardium, protruding into and deforming the ventricular chamber, is evidenced (arrows). In addition, moderate circumferential pericardial effusion is evident.
Panel C. T1-weighted short-axis gradient-echo MRI images showing a large mass with low-intensity tissue signal in the left ventricular myocardium at the level of lateral and apical walls (arrows).
Panel D. T1-weighted long-axis gradient-echo MRI images showing a large mass with low-intensity tissue signal in the left ventricular myocardium at the level of lateral and apical walls (arrows).
Panel E. T2-weighted short-axis gradient-echo MRI image. The recordings were performed 20 s after gadolinium contrast infusion, demonstrating poor perfusion of the left ventricular mass, without significant hyperenhancement of tumour during first-pass contrast infusion (arrows).
Panel F. Hamartomas, microscopic features. High-power photomicrograph (original magnification, x 300; H&E stain) demonstrates typical vacuolated cells of rhabdomyoma. Cytoplasmic streaming is a typical artifact that results in ‘spider cells’ (arrows).
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