Occult malignancy presenting as constrictive pericarditis
Darren Porter
0
Mehmood Jadoon
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Damian McGrogan
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Onyekwelu Nzewi
0
0
Department of Cardiothoracic Surgery, Royal Victoria Hospital
, Grosvenor Road, Belfast BT12 6BA,
Northern Ireland, UK
Metastatic tumour progression to the pericardium is generally characterised by an effusional pericarditis. It is extremely rare for tumour to metastasise to the pericardium and cause constrictive pericarditis in the absence of a pericardial effusion. We report the recent case of a patient who was referred to our centre with constrictive pericarditis. Following pericardectomy and histopathological analysis this was found to be secondary to an occult metastatic adenocarcinoma. 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.
1. Introduction
Constrictive pericarditis is most often idiopathic or it
occurs following cardiothoracic surgery or radiation therapy
to the mediastinum, it is rarely caused by malignancy.
When metastatic cancer involves the pericardium, it
typically causes pericardial effusion (with or without
tamponade). Few case reports of metastatic lung cancer causing
constrictive pericarditis have been reported and fewer still
describe constrictive pericarditis as the first manifestation
of the neoplastic process. We report the case of a
33-yearold patient with an occult malignancy presenting as
constrictive pericarditis.
2. Case report
A 33-year-old patient presented with a six months history
of dyspnoea, general malaise, weight loss, ascites and ankle
oedema. Chest X-ray demonstrated bilateral pleural
effusions. Autoimmune and vasculitic screens were normal and
tumour markers were negative. Computed tomography
pulmonary angiogram (CTPA) revealed bilateral pleural
effusions, pericardial thickening and a small sub-segmental
pulmonary embolism with no obvious lung pathology. A
pleural tap was performed and this was negative for TB,
infection or malignancy. Transthoracic echocardiography
demonstrated a thickened pericardium, a dilated superior
vena cava (SVC) and inferior vena cava (IVC) which raised
the possibility of constrictive pericarditis. A cardiac
magnetic resonance imaging (MRI) confirmed this diagnosis with
classical septal bouncing with respiratory motion and
markedly thickened pericardium (Fig. 1).
Radical pericardectomy was carried out through a
conventional median sternotomy incision. An unusual finding was
uniformly marked thickening that completely encased the
SVC, the IVC and the pulmonary trunk, and even beyond
the phrenic nerves. The phrenic nerves were dissected out
carefully and the entire posterior thickened pericardium
was removed to free the heart completely (Fig. 2).
Following removal of the pericardium there was an
immediate improvement in blood pressure and a drop in central
venous pressure (CVP) from 29 mmHg to 19 mmHg.
Histopathology of pericardium indicated a differential diagnosis
of metastatic adenocarcinoma or mesothelioma; however,
malignant mesothelioma was excluded using
immunohistochemistry and metastatic lung adenocarcinoma was
confirmed. Anterior hilar nodes and thymic tissue were also
extensively replaced with metastatic adenocarcinoma.
With a working diagnosis of occult metastatic lung
adenocarcinoma, the patient underwent a CT chest, abdomen
and pelvis on day 6 postoperatively which revealed no gross
lesion. Despite the marked improvement and resolution of
the symptoms of constrictive pericarditis the patient
unfortunately died 29 days postoperatively from complications
secondary to repeated pulmonary emboli.
3. Discussion
Constrictive pericarditis is most commonly idiopathic or
it occurs secondary to mediastinal radiotherapy or following
cardiac surgery. Less common aetiologies include infection,
connective tissue disorders, malignancy, uraemia, and
sarcoidosis w1x, tuberculosis is a rare cause of constrictive
pericarditis in the Western world w2x.
Constrictive pericarditis can rarely be caused by
malignancy, however, malignancy may also manifest as
pericardial effusion (with or without tamponade) or an encased
heart with thickening of both visceral and parietal layers,
resulting in a constrictive physiology.
D. Porter et al. / Interactive CardioVascular and Thoracic Surgery 12 (2011) 10461047
Fig. 1. Cardiac magnetic resonance imaging: Pericardial thickening (arrow).
Fig. 2. Thickened pericardium following pericardectomy.
Metastatic involvement of the heart is much more
common than primary tumours. The prevalence of such
metastatic involvement has been reported as ranging from 15%
to 30% in autopsies performed for cases of neoplastic
disease and 4% of general autopsies w3x. Lung and breast
cancers are the most frequent causes of malignant
pericardial disease; however, lymphoma and mesothelioma can
also involve the pericardium w4x. The most common cell
type to metastasise to the heart is adenocarcinoma w5x.
Cardiac involvement can develop by retrograde lymphatic,
haematogenous, direct or transverse extension. The cardiac (...truncated)