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Ann Thorac Surg 2001;71:1673-1675
© 2001 The Society of Thoracic Surgeons

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Case report

Left ventricular hamartoma associated with ventricular tachycardia

^a Division of Cardiac Surgery, Massachusetts General Hospital, Boston, Massachusetts, USA
^b Division of Electrophysiology, Massachusetts General Hospital, Boston, Massachusetts, USA
^c Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, USA

Accepted for publication July 10, 2000.

^* Address reprint requests to Dr Torchiana, Department of Cardiac Surgery, Massachusetts General Hospital, 55 Fruit St, Boston, MA 02114 (Email: dtorchiana{at}partners.org).

	Abstract

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Cardiac hamartomas are a rare type of benign tumor affecting the heart. We describe a 33-year-old patient who presented with a wide complex tachycardia. Diagnostic imaging revealed a mass in the patient’s left ventricular wall, near the apex of the heart. The mass was surgically resected and appeared benign. Its pathology was that of a hamartoma of mature cardiac myocytes. Postoperative electrophysiology evaluation showed no inducible focus and the patient remains alive and asymptomatic after 2 years of follow-up.

	Introduction

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A hamartoma is a focal benign overgrowth of differentiated mature cells that are typically native to the affected organ [1]. Hamartomas may be single or multiple and are treated by surgical excision when appropriate.

A 33-year-old man was admitted to the Emergency Department of the Massachusetts General Hospital for wide complex tachycardia. The patient, a physician, had driven himself to the hospital after experiencing an episode of shortness of breath, palpitations, and light headedness that lasted approximately 15 minutes. The symptoms had spontaneously appeared while the patient was seated, though he recalled similar symptoms occurring during past basketball games and a recent golf game. He was given lidocaine, which succeeded in terminating his tachycardia. His electrolytes were normal. The patient did not have any chest pain, and did not consume alcohol, tobacco, or caffeine, or use any illicit drugs. His only medication was Axi, taken as needed for symptoms of dyspepsia. He also had a history of hypertension, hypertriglyceremia, and hypercholesterolemia. His family history was remarkable for a grandfather who had had a stroke, and a grandmother who had had cervical cancer.

An echocardiogram revealed a transmural homogeneous and echodense mass located near the apex of the patient’s left ventricle (Fig 1). In retrospect, the mass could also be delineated in a previous echocardiogram (done while investigating the patient’s hypertension four years earlier). The ejection fraction was 66%. Magnetic resonance imaging (MRI) confirmed the presence of a hypointense mass in the lateral free wall of the heart, from the midventricular level to near the apex. It was measured to be 4.5 x 3.1 x 4.4 cm. T2-weighted images of the mass suggested that it was encapsulated, and mostly contained in the myocardium (Fig 2). In one area, however, it appeared to reach the endocardium. After injection of gadolinium, the mass became isointense with the myocardium, indicating that it was well vascularized. A coronary angiogram found a small vascular arcade around the mass, stemming from a diagonal branch of the left anterior descending artery. The existence of the mass four years prior, its smooth contour and possible encapsulation all led to an initial diagnosis of a benign tumor.

View larger version (6K): [in this window] [in a new window]   Fig 1. ECHO showing a homogeneous mass (arrow) near apex of LV.

View larger version (7K): [in this window] [in a new window]   Fig 2. MRI showing a hypointense mass (arrow) near the LV apex extending to the endocardium.

Pathologic examination of the tumor revealed a whorled, white mass, with small islands of browner tissue. Microscopically, these were found to be areas of mature myocytes, variably interspersed among fibroblasts and collagen. At the border of the tumor, the fibroblasts interdigitated with the surrounding myocardium, which was thought to be the reason why the tumor appeared to be encapsulated with MRI imaging. The final diagnosis was "hamartoma of mature cardiac myocytes."

The patient had an uneventful postoperative course. Intravenous lidocaine was used immediately postprocedure, and was discontinued the following morning at which point he was commenced on atenolol 50 mg daily. No ventricular tachycardia or ventricular ectopy was observed on telemetry and after 5 days observation, the patient was discharged on atenolol 100 mg bid. He was given a 30-day event recorder which he was told to activate if he had any palpitations.

Three months later, he was readmitted off atenolol for 24 hours for an electrophysiology study. Programmed ventricular stimulation was performed with 1 to 3 ventricular extrastimuli, both off and on intravenous isoprenaline at 2 right ventricular sites. No ventricular arrhythmia was inducible. This study was performed in part to exclude the possibility of a ventriculotomy related ventricular tachycardia. A follow-up MRI confirmed that the tumor had been fully excised, and that the left ventricular cavity was not significantly altered. An echocardiogram also showed no evidence of ventricular abnormalities or valve malfunction. Two years postoperatively, the patient is doing well on atenolol and Norvasc.

	Comment

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Cardiac tumors are rare and usually benign [1]. Hamartomas are one of the rarest types of benign cardiac tumors, and only small series have been reported in the literature [2–8]. Other benign cardiac tumors, in order of frequency, include myxomas, fibromas, rhabdomyomas, and lipomas.

Recently, Burke and associates reviewed three cases involving hamartomas of mature cardiac myocytes in a 22-year-old patient , 28-year-old patient , and 9-year-old patient [2]. The tumors were characterized by a lack of encapsulation and hypertrophy of the disarrayed myocytes. The lesions found in the first two patients were localized to the right ventricle and right atrium, respectively. The youngest patient’s heart, examined postmortem, had multiple nodules scattered throughout the heart, including papillary muscles.

Tanimura and coworkers reported a cardiac hamartoma at the apex of the heart in a 68-year-old male with retroperitoneal leiomyosarcoma [3]. This patient’s tumor contained additional mature adipose tissue. Park, Franciosi and others have also described hamartomas located in the left ventricle, but these have been classified as fibrous or vascular hamartomas [4–7]. Sturtz and colleagues [8] reported a 24-year-old human with palpitations and a hamartoma of mature cardiac myocytes in the interventricular septum which could only be partially resected.

Our patient’s hamartoma was associated with a wide complex tachycardia that originated from the left ventricle. Incessant ventricular tachycardia has been seen in a number of children with myocardial hamartomas [9]. There are a number of case reports of patients with ventricular tumors who had telemtry guided suppression of VT with antiarrhythmia drugs without surgery [10–13]. In our patient, the tachycardia appears to have been ablated by surgical excision which was undertaken for diagnosis and tumor removal as well.

In summary, we have presented a case where the diagnosis of a benign cardiac tumor was made with diagnostic imaging techniques, and surgical intervention was effective in removing a hamartoma of mature cardiac myocytes. The QRS morphology during VT, the lack of symptoms in follow-up, and the negative postoperative EP study off β-blockade and with intravenous isoprenaline are strong evidence that the VT was tumor-related and cured by its excision.

	References

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1. Burke A, Virmani R. Benign tumors of cardiac myocytesIn: Rosai J, Sobin LH, editors. Atlas of tumor pathology. Series 3, Fascicle 16. Washington, DC: Armed Forces Institute of Pathology; 1996.
2. Burke AP, Ribe JK, Bajaj AK, et al. Hamartoma of mature cardiac myocytes Hum Pathol 1998;29:904-909.[Medline]
3. Tanimura A, Kato M, Morimatsu M. Cardiac hamartoma. a case report. Acta Pathologica Japan 1988;38:1481-1484.
4. Franciosi RA, Gay RM, Ah-Tye P. Vascular hamartoma of the heart in a child Am Heart J 1970;79:676-682.[Medline]
5. Feldman PS, Meyer MW. Fibroelastic hamartoma (fibroma) of the heart Cancer 1976;1:314-323.
6. Parks FR, Adams F, Longmire WP. Successful excision of a left ventricular hamartoma Circulation 1962;26:1316-1320.[Abstract/Free Full Text]
7. Franciosi RA, Gay RM, Ah-Tye P. Vascular hamartoma of the heart in a child Am Heart J 1970;79:676-682.[Medline]
8. Sturtz CL, Abt AB, Leuenberger UA, et al. Hamartoma of mature cardiac myocytes. a case report. Mod Pathol 1998;11:496-499.[Medline]
9. Garson Jr A, Smith RT, Moak JP, et al. Incessant ventricular tachycardia in infants. myocardial hamartomas and surgical cure. J Am Coll Cardiol 1987;10:619-626.[Abstract]
10. Fukushima KK, Mitani T, Hashimoto K, et al. Ventricular tachycardia in a patient with cardiac lipoma J Cardiovasc Electrophysiol 1999;10:1161.[Medline]
11. Enbergs A, Borrggrefe M, Kurlemann G, et al. Ventricular tachycardia caused by rhabdomyoma in a young adult with tuberous sclerosis Am Heart J 1996;132:1263-1265.[Medline]
12. Miyashita T, Miyazawa I, Kawaguchi T, et al. A case of primary cardiac B cell lymphoma associated with ventricular tachycardia, successfully treated with systemic chemotherapy and radiotherapy. a long term survival. Jpn Circ J 2000;62:135-138.
13. Leak D. Amiodarone for control of recurrent ventricular tachycardia secondary to cardiac metastasis Tex Heart Inst J 1998;25:198-200.[Medline]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): David F Torchiana Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Dinh, M. H. T. Articles by Torchiana, D. F. Search for Related Content PubMed PubMed Citation Articles by Dinh, M. H. T. Articles by Torchiana, D. F.