A young woman with chest pain and dyspnoea

A woman in her early thirties, with a history of mostly good health, was admitted acutely to the cardiology department with a diagnosis of chest pain. She had recently experienced gradually increasing functional dyspnoea.

In the week prior to hospitalisation, she had had respiratory chest pain with radiation to the throat and jaw. She had also had intermittent fever on a number of occasions in recent weeks. She had had a spontaneous abortion about six weeks earlier, after 8 – 9 weeks of pregnancy.

Upon admission, vital signs were as follows: pulse 106/min, regular, blood pressure 110/77 mm Hg, temperature 37.0 °C, respiratory rate 16/min. Auscultation of the heart revealed a murmur over the entire precordium. Palpation of the abdomen revealed a firm mass in the lower right quadrant that was sore upon direct palpation.

An ECG revealed sinus tachycardia, but no definitive pathology. Blood tests showed a moderately elevated CRP level of 59 mg/l (<5 mg/l) and elevated D-dimer of 1.80 mg/l (<0.50 mg/l), troponin T 66 pmol/l (< 15 pmol/l) and proBNP 244 pmol/l (< 33 pmol/l).

Upon discovery of the murmur, a preliminary echocardiographic examination was performed in Accident and Emergency. This revealed two large masses on the right side of the heart. The largest mass measured 3.5 x 3 cm, was located within the atrium and adherent to the tricuspid valve apparatus. In addition, there was a smaller mass that also penetrated the right ventricle.

There was clear obstruction of normal blood flow in the tricuspid ostium. Pericardial effusion was also seen, particularly along the right ventricle, with a maximum size of 1.5 cm but no evidence of tamponade. The mitral and aortic valves on the left side of the heart appeared normal. The findings were confirmed with a cardiac CT scan, which also raised suspicion of infiltration of tumour masses into the right ventricular wall and pericardium (Fig. 1).

The patient’s symptoms of chest pain and dyspnoea and the clinical finding of murmur were judged to be related to a cardiac tumour, with a primary tricuspid valve obstruction. The pericardial effusion was considered to be of little haemodynamic significance and we therefore decided against drainage.

It can be difficult to distinguish tumours from other masses such as vegetations or thrombotic masses. For this reason, we chose to treat the patient both for primary cardiogenic thrombus, with low molecular weight heparin, and as for endocarditis, with broad spectrum antibiotics in the form of penicillin and gentamycin.

Further diagnostic imaging was performed, including CT thorax, abdomen and pelvis. This verified the echocardiographic finding of an expansive process in the right side of the heart with pericardial effusion. An additional large expansive process measuring 11 x 8 cm was found in the right side of the abdomen, with varying density and contrast enhancement, calcifications and abundant vascularisation. The pouch of Douglas was also shown to contain free fluid and masses with minor contrast enhancement (Fig. 2).

An interdisciplinary discussion took place between cardiologists, thoracic surgeons, oncologists, gynaecologists and gastrointestinal surgeons. Intervention with respect to the cardiac abnormalities was considered to be too risky. Acute cardiac surgery was seen as a last resort, but we continuously monitored vital indication for surgery.

The pathology in the abdomen and pelvis close to the right ovary was perceived as relatively accessible for biopsy. The biopsy was performed under ultrasound guidance, and the sample was prepared as frozen sections with rapid results a priority. After collection of the sample, the patient was placed on high dose steroids in the form of dexamethasone 3 mg x 3.

The day after the biopsy, preliminary results from the pathology department were available: high grade malignant lymphoma. As the lymphoma was considered potentially life-threatening, direct anti-tumour therapy was started that same day with cyclophosphamide and dexamethasone at an increased dose of 4 mg x 4. Allopurinol and intravenous fluids were given as tumour lysis prophylaxis.

Only hours after treatment, the patient noticed decreasing chest pain and reduced dyspnoea. Two days later, it was possible to perform cardiac MRI. This confirmed persistent tumours in the right atrium and right ventricle with growth through the ventricular wall and fusion of the tricuspid leaflet (Fig. 3).

A few days later, the final results became available from the pathology department. The conclusion was that the patient had a high grade malignant lymphoma, morphologically and immunophenotypically consistent with diffuse large B-cell lymphoma of the germinal centre subtype (positive for B-cell markers and BCL6, negative for BCL2, MYC, CD10 and MUM1). There was evidence of very high cell turnover: the Ki-67 proliferative index was almost 100 % (Fig. 4).

The disease was detected in the right side of the heart, bilaterally in the kidneys and as a tumour in the abdomen and pelvis. All manifestations were extranodal, and the lymphoma was thus classified as stage IV. After initial treatment, we continued with the first main treatment, namely the standard chemotherapy regimen for non-Hodgkin's lymphoma, CHOP: cyclophosphamide, vincristine, doxorubicin and prednisolone.

During this period, the patient was monitored in the cardiac observation unit. No arrhythmias or other complications of treatment were observed. She received low dose fractionated heparin as thrombosis prophylaxis. Repeated MRI scans, performed three days after initiation of the CHOP regimen, showed significant regression of the mass in the right side of the heart and almost total regression of the intracavitary components. The right atrioventricular opening was significantly larger than in the initial MRI scan (Fig. 3).

The patient responded well to the initial treatment. However, the condition was considered to be high-risk disease owing to the extranodal involvement, therefore we decided to provide further cycles of treatment. The first cycle was performed 25 days after the initial hospitalisation. There were no notable complications, and she was discharged to her home following post-treatment observation. Forty days had passed since she was first hospitalised.

The patient underwent a further treatment cycle. Radiological assessment after the second treatment showed almost total regression of the abdominal tumour (Fig. 3). We have continued with the R-CHOP regimen, which has kept the disease in remission ever since.