Interesting case of regional left ventricular hypertrophy

Abstract

This is an account of an interesting case with an unusual cardiac presentation. He is a man in his 60s who presented with chest tightness to the accident and emergency unit. The initial thoughts were of acute coronary syndrome or acute aortic syndrome. The initial set of investigations was non-conclusive. His echocardiogram which was done during hospital admission showed asymmetric hypertrophy of the heart muscle. It was prudent to assess that new finding with an MRI scan. The patient presented to the hospital twice during the investigation and was treated for a lower respiratory tract infection. The MRI report showed an interventricular mass lesion extending to the right ventricular free wall with angiosarcoma being high up in the differential diagnosis. Going through the heart team discussion, the decision was to go for a transcatheter biopsy. The biopsy showed B-cell lymphoma. The treatment started and interestingly with satisfactory results.

Background

Primary cardiac tumours (PCTs) are considered exceedingly rare. Their incidence is depicted to be a figure between 1 and 1.5 in 1000 patients.¹ Among those cases, only 9.7% are found to be a PCT with higher prevalence in Europe and North America.² Primary cardiac lymphomas (PCLs) were accounting for 1% of PCT.³ There are two main definitions for PCLs. According to the first definition, lymphomas solely affect the heart or the pericardium. Based on the second definition, the chief burden of the tumour is placed on the heart or the pericardium, and limited extracardiac involvement is identified.⁴

Case presentation

A man in his 60s was admitted to the local hospital with a persistent cough and chest tightness. His medical history included essential hypertension and basal cell carcinoma that was excised in 2019. His ECG showed sinus rhythm with deep T wave inversion in the early septal leads. His cardiac biomarkers were normal, but he had a raised C-reactive protein (CRP) of 60 and a leucocytosis which was predominantly neutrophils. Because he presented with chest pain radiating to the back, a CT aortogram was performed in accident and emergency, which excluded an acute aortic syndrome, but it demonstrated a small circumferential pericardial fluid of about 2.5 cm and some mediastinal lymph nodes measuring up to 11 mm (figure 1).

Figure 1.

Axial image from the CT pulmonary angiogram that shows moderate circumferential pericardial effusion.

A subsequent transthoracic echocardiogram confirmed the effusion but slightly disagreed on dimensions with effusion dimensions between 1.3 and 1.6 cm. Interestingly, it showed marked thickening of both ventricles. A septal predominance thickening of 2.2 cm versus 1.2 cm at the posterior wall of the left ventricle (LV) (figure 2). Looking at the right ventricle (RV) proved it to be hypertrophied more notably at its apex. The RV was not dilated and showed selective impaired function at the apex. At this point, he was managed as Dressler syndrome secondary to a presumed recent infarction until more information is available. Although amyloid was the more likely diagnosis, an outpatient cardiac MRI scan was requested to further characterise the abnormal myocardium.

Figure 2.

Initial echocardiogram. Image left: apical four chambers view that shows apical and interventricular (green arrow) hypertrophy. Image right: parasternal short axis image that shows the left ventricular dimensions. Both pictures demonstrate a small rim of pericardial effusion.

Three weeks later, he was readmitted with chest discomfort at the lower part of the chest, lethargy, fever and shortness of breath. The MRI was achieved before this admission, and the result was available to the acute medical team on admission. A cardiac MRI scan was performed, which identified a 6.2×6.5 cm space-occupying lesion within the heart muscle (figure 3). The lesion was attached to the anterior surface of the heart and infiltrated the intraventricular septum and the right ventricular outflow tract (RVOT). A provisional diagnosis of cardiac angiosarcoma was proposed. Given his chest discomfort, shortness of breath and a new diagnosis of possible malignancy, a CT pulmonary angiogram was arranged. This showed a persisting similar-size pericardial effusion, basal lung consolidation on both sides and widespread mediastinal lymphadenopathy. He was admitted to the hospital to be treated for pneumonia.

Figure 3.

An initial cardiac MRI picture shows a bulky mass that lies in the interventricular septum.

Outcome and follow-up

The structural heart multidisciplinary specialist team discussed his case at the tertiary hospital and was not keen to have a sternotomy as an option for biopsy. Considering this to be a sarcoma with a likely poor prognosis, this was a very aggressive option to the team. They opted for a percutaneous option which was successfully achieved. The specimen was sent to the local Haematolymphoid Diagnostic Service. The cells expressed CD20, CD10, BCL6 and BCL2. They were negative for CD5 CD23, cyclinD1 and SOX11. The proliferation was low at approximately 5%. The features are those of a low proliferative B cell lymphoma with an immunophenotype consistent with follicular lymphoma (figure 4). A subsequent positron emission tomography scan (PET CT) showed high metabolic uptake in the myocardial mass in question with additional PET-avid areas in the scapula and lymph node infiltration around the neck and the mediastinum. The treatment protocol of cyclophosphamide, doxorubicin, obinutuzumab, prednisolone, and vincristine (O-CHOP) was commenced in accordance with National Institute of Clinical Excellence (NICE) guidelines given it was follicular lymphoma with Follicular Lymphoma International Prognostic Index (FLIPI) score of 2 or more.⁵

Figure 4.

Image A: myocardial biopsy with a dense cellular infiltrate with crush artefact, ×20; image C: an interstitial infiltrate of lymphoid cells, ×400; image B: the infiltrating cells are strongly positive for CD20, indicating that they are B-lymphocytes. The slides courtesy of Dr Paul Bishop, Pathology Department at Wythenshawe Hospital, Manchester.

After 6 cycles of O-CHOP treatment had been completed, a follow-up PET CT and cardiac MR (CMR) were repeated. The PET CT showed complete metabolic response allowing for the active metabolism of the cardiac muscle tissue. No new FDG uptake was detected (figure 5). The repeated CMR showed substantial reduction in the mass size. There remains, however, thickening of the basal LV anteroseptum and RV septum/RVOT up to a maximal wall thickness of 17 mm (figure 6, video 1). He has been declared to be in remission.

Figure 5.

(A) PET CT axial view of the chest that shows FDG avid lesion at the apex and anterior wall extending into the intraventricular septum. (B) PET CT after completion of the treatment that shows complete metabolic response.

Figure 6.

Cardiac MR (CMR) images showing left: CMR apical four chambers and right: CMR two chambers view demonstrate a substantial reduction in the mass size. There remains, however, thickening of the basal left ventricle anteroseptum and right ventricle septum/right ventricular outflow tract up to a maximal wall thickness of 17 mm.

Video 1. Cardiac MR images showing pretreatment and post-treatment images. There is 6-month interval between the images.

bcr-2023-257882supp001.mp4 ^{(668.9KB, mp4)}

DOI: 10.1136/bcr-2023-257882.video01

Discussion

Out of different histological types identified, diffuse large B cell lymphoma is the most prevalent, with Burkitt lymphoma, T cell lymphoma, small lymphocyte lymphoma and plasmablastic lymphoma following in terms of frequency.^6–8

Diagnosing PCL usually requires multimodality assessment. Cardiac MRI remains the ideal modality for diagnosing PCL. Distinguishing cardiac lymphoma from angiosarcoma still lacks defining criteria. Central liquefaction necrosis (or necrotic areas) was absent in all lymphomas. In contrast, they showed homogeneous tumour enhancement. The sample size only included 12 patients.⁹

Achieving a tissue diagnosis in intracardiac masses is a difficult clinical challenge. If a significant pericardial effusion is present, it could yield a reasonable cellular sample to diagnose. Otherwise, the choice will be minithoracotomy or right heart catheterisation.¹⁰ A percutaneous option has been considered in an another report as well.¹¹

The importance of diagnosing cardiac lymphoma stems from the success rates of treatment. 73% of patients received chemotherapy treatment, 15% received some form of surgery including diagnostic sampling and 13% received radiotherapy. An overall median survival rate of 2.2 years was reported with 1-year, 5-year and 10-year survival rates which were 61%, 35% and 28%, respectively. Chemotherapy is the predominant treatment modality and is associated with improved survival, while surgical therapy and radiotherapy are not associated with extended survival. Increasing age is independently associated with worse outcomes.¹²

Learning points.

• This case shows how lymphoma can mimic various clinical syndromes and has proven difficult to diagnose without achieving multimodality radiological and pathological assessment.

• It humbled us to find the diagnosis changing as more investigations are requested. This would invite us in the future to keep a wider differential diagnosis for patients with similar presentations.

• Cardiology multidisciplinary team for cardiac oncology is a service that is rarely needed. It has proved challenging to navigate access to different services in this situation. It would help in the future if a clear pathway were available to patients with similar presentations.

Ethics statements

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