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A 51 year old patient with dyspnea on exertion

A 51-year-old patient presented with dyspnea on exertion NYHA II – III. Recently, there were tachycardic atrial fibrillations as well as a severely reduced LV function described. A chest x-ray was diagnosed without any pathological findings.
Therefore, he was transferred to our hospital for a coronary angiography and cardiac work-up.  The patient denied any angina pectoris at rest or on exertion, syncopes or peripheral edema. There were a fatigue and a non-productive dry cough for weeks. He had no elevated temperature, the blood pressure was in a normal range (110 / 70 mmHg). Otherwise, the patient did not have any previous diseases.
His ECG at our hospital showed a sinus rhythm (85 bpm) with atrial extra systoles but without any ST segment changes. Blood analysis revealed a slightly elevated c-reactive protein (1,0 mg/dl; normal: < 0,5 mg/dl) and leukocyte count (11/nl; normal: < 10/nl). All other values including the troponin T were in the normal range.  
In the transthoracic echocardiography (TTE) the left atrium (25 cm2) and the left ventricle (LVEDD 59 mm, LVESD 49 mm) were slightly enlarged. The right sided chambers were normal in size without evidence of hypertrophy. The systolic LV function was diffuse mildly to moderately reduced (EF triplan 45 %). There was no vitium detected.  We found a small pericardial effusion basal posterior of the left ventricle. Furthermore, a mainly hypodense structure with a diameter of around 7 x 4 cm could be seen, visually smoothly bounded,  adjacent to the left atrium and  leading to a mild compression.
Myocardial Disease




Figure 1: TTE, 4- and 2- chamber view


Figure 2: TTE, 3- chamber view

In the transoesophageal echocardiography the structure reached up to the ascending aorta, the superior cava vein, the left sided pulmonary veins and to the pulmonary artery, with a quite smooth and hyperdense borders.


Figure 3: TOE, structure adjacent to the ascending aorta, the superior cava vein and the right pulmonary artery

During the coronary angiography, a coronary artery disease could be excluded. But there was a big vessel from the RCX reaching to this well vascularized structure.


Figure 4: coronary angiography, left coronary artery, AP projection 

In addition, a cardiovascular magnetic resonance study (CMR) was performed. The same extension was seen. There was no definite infiltration of cardial structures. After intravenous application of gadolinium contrast agent (30 ml Prohance) a fast and inhomogeneous enhancement within in the structure was demonstrated.


Figure 5: CMR, 3- chamber view, 2 min after application oft he contrast agent

Questions:

1.  What additional work-up would you perform order to confirm?
2.  What is your differential diagnosis?

Answers from the previous case of the month

The patient got a screening with a CT scan of the thorax and a sonography of the abdomen without providing hints for a neoplasm or an adenopathy. The urologic work-up showed normal values for ß- HCG and α-fetoprotein and ruled out a germ cell tumor.  A gastroscopy showed an impression of the esophagus at 30 cm.
Because of the vascularisation and the size of the tumor we did not perform a biopsy. Therefore, the patient was transferred to surgery. Intraoperatively, the tumor showed an extension covering half the left atrium with contact to the superior cava vein, the pulmonary artery and the pulmonary veins as well as an adhesion to the lateral atrial wall. The tumor was totally resected and the walls of the left atrium and great vessels partially replaced by Dacron.
The tumor had a weight of about 200 g and a size of 7x4x5 cm. Histologically, the pathologists observed a angiolipomatous proliferation with nerve fascicles and proliferating lymphatic tissue and fibrotic septa, multiple vessels and nerves. The final diagnosis was a cardiac hamartoma with an adjacent mediastinitis.


Figure  6: cardiac hamartoma

Secondary cardiac tumors (i.e. metastatic malignant tumors) are much more common than primary cardiac tumors. Tumors of cardiac origin are very seldom (0.3 % of autopsies). About 75 % of them are benign. The most common benign tumors in adults are myxomas.
Primary cardiac tumors can lead to a wide variety of symptoms and systemic reactions like fever, malaise or arthralgias. The specific symptoms are mainly related to their anatomical location. Because of their size or their location they can lead to an intracavitary or valvular obstruction. Furthermore, they can produce rhythm disorders like atrial fibrillation or flutter, AV blocks or ventricular arrhythmias. Also, fragments of the tumor or thrombi can embolize, concerning to their location to the systemic or pulmonary circulation.
The differentiation between benign or malignant tumors is often difficult. Usually, benign tumors are more often on left side, show a slow growth, no invasion of cardiac or mediastinal structures and no pericardial effusion. Therefore, a total resection is the therapy of choice.
Hamartomas are very rare (< 1 % of the benign cardiac tumors) and can be diagnosed at any age. They usually remain asymptomatic for a long time. Mostly, they are located in the left ventricle. There are only few case reports in literature.

 

Notes to editor


Helena Grawe, Armin Grawe, Franz-Josef Beck, Christian Vahl, Torsten Hansen. Dr. Horst Schmidt Klinik, Zentrum Innere Medizin I, Kardiologie, Wiesbaden; Herz-Thorax-Gefäßchirurgie und Institut für Pathologie, Universitätsmedizin, Mainz, Germany
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.