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Logo of thijTexas Heart Institute JournalSee also Cardiovascular Diseases Journal in PMCSubscribeSubmissionsTHI Journal Website
Tex Heart Inst J. 2010; 37(4): 457–460.
PMCID: PMC2929872

Left Ventricular Rupture Due to HIV-Associated T-Cell Lymphoma


Patients with lymphoma can develop cardiac involvement that includes malignant pericardial effusions and myocardial infiltration, but extensive myocardial invasion by tumor with resultant rupture has been reported only rarely. We report a case of a patient with human immunodeficiency virus and T-cell lymphoma who presented with signs and symptoms that were suggestive of a non-ST-elevation myocardial infarction. Plans were made for cardiac catheterization, but the patient developed thrombocytopenia after the initiation of heparin and eptifibatide. Cardiac catheterization was deferred, and shortly afterwards he had a witnessed cardiac arrest in the hospital and could not be resuscitated. Autopsy revealed transmural infiltration of the myocardium with lymphoma and resultant rupture of the left ventricular free wall. To our knowledge, this is the 1st reported case of left ventricular free-wall rupture due to transmural infiltration by human-immunodeficiency-virus–associated peripheral T-cell lymphoma.

We conclude that noncoronary causes of chest pain, including direct myocardial infiltration, should be considered in immunocompromised patients with lymphoma.

Key words: Acute coronary syndromes, heart neoplasms/secondary, heart rupture, HIV infections/complications, lymphatic metastasis, lymphoma, AIDS-related, lymphoma, T-cell/pathology, myocardium/pathology, neoplasm invasiveness, pericardium/pathology

Cardiac involvement with non-Hodgkin's lymphoma (NHL) is rare clinically but has been reported to occur in 10% to 30% of lymphoma autopsy cases.1 Although malignant pericardial effusions are the most frequently reported manifestation of cardiac involvement with lymphoma, direct infiltration of the myocardium and endocardium has also been seen at autopsy. In a recent series of 153 lymphoma autopsies, cardiac involvement, including malignant pericardial effusion, was found in 16% of cases.2 Among these patients, only a minority had symptoms possibly attributable to cardiac tumor burden, including heart failure, tamponade, and sudden death; in the other cases, cardiac involvement was not suspected until autopsy.

The risk of NHL is 60 times higher in patients with human immunodeficiency virus (HIV) infection than in the general population.3 When compared with NHL in the general population, most HIV-associated NHLs are high-or intermediate-grade B-cell malignancies, but T-cell lymphomas have also been reported with increased frequency. Approximately 85% of HIV-associated NHLs have extranodal involvement, often in unusual sites, such as the orbit, heart, muscles, adrenal glands, skin, and salivary glands.3 When compared with other types of lymphoma, T-cell lymphoma may involve the heart and great vessels with increased frequency. The mechanism of this positive cardiac tropism is uncertain, but it might be related to an increased propensity of T-cell lymphoma to metastasize in a hematogenous pattern.

Cardiac lymphomas have been reported in HIV-infected patients, and most of these have been intermediate-or high-grade B-cell malignancies.4 Although the outcome of HIV-associated cardiac lymphoma is generally poor, successful treatment with chemotherapy has been reported,5 which emphasizes the importance of early diagnosis.

Case Report

In October 2008, a 62-year-old man was admitted after 4 days of dyspnea, fatigue, and intermittent right-sided chest pain. He reported associated diaphoresis and orthopnea but denied weight gain or new-onset edema.

His recent medical history was notable for stage IVB peripheral T-cell lymphoma diagnosed 4 months earlier. At diagnosis, staging had shown widespread supra- and infradiaphragmatic nodal involvement as well as multiple extranodal involvement including muscles, bilateral adrenal glands, and subcutaneous nodules, but not bone marrow. The patient had achieved a complete response to induction chemotherapy consisting of GND (Gemcitabine, Navelbine, and Doxil) and of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) augmented by high-dose methotrexate. He remained in remission for 2 weeks, at which time he developed isolated central-nervous-system relapse presenting as right-arm weakness. He was found to have a 2.1-cm homogeneously enhancing mass in the left motor cortex consistent with recurrent lymphoma, for which he underwent whole-brain irradiation. Immediately after the completion of brain irradiation, he developed recurrent lymphoma systemically, including bilateral adrenal masses and possibly invasion of the kidneys.

His earlier medical history was notable for HIV diagnosed more than 25 years earlier and for a non-ST-elevation myocardial infarction in association with coronary artery disease, which had been treated 3 years earlier via placement of a bare-metal stent in the left anterior descending coronary artery.

On examination, the patient was dyspneic at rest, needing to stop mid-sentence when talking, and he was using accessory muscles of respiration. His blood pressure was 106/51 mmHg, his heart rate was 81 beats/min, and there was no pulsus paradoxus. The jugular venous pulsation was elevated at 10 cm but without Kussmaul's sign. Cardiac auscultation was notable for an old 2/6 systolic ejection murmur at the base and an S4 heart sound. Crackles were present bilaterally at the lung bases. There were no subcutaneous nodules, hepatosplenomegaly, or lymphadenopathy. A chest radiograph was consistent with pulmonary edema, and an electrocardiogram (ECG) showed evidence of a prior anteroseptal myocardial infarct, with more prominent ST elevation in leads V1 through V3 than had been seen on an ECG 3 weeks earlier. The troponin I level was elevated at 0.16 ng/mL, the total creatine kinase level was 157 U/L, and the creatine kinase-MB level was 30 ng/mL.

On the basis of these findings, an acute coronary syndrome was strongly suspected. The patient was started on aspirin and on intravenous heparin and eptifibatide, with plans for cardiac catheterization. An echocardiogram showed a left ventricular (LV) ejection fraction of 0.65 to 0.70, with apical hypokinesis and a hyperdynamic mid ventricle. There was a small, free-flowing pericardial effusion without echocardiographic evidence of tamponade. On repeat testing immediately before cardiac catheterization and approximately 12 hours after the initiation of heparin and eptifibatide infusions, the patient's platelet count was noted to have decreased from 106 to 38 × 109/L, without any change in white blood cell count or hematocrit level. The eptifibatide and heparin were discontinued and plans for catheterization were deferred, given (especially) the resolution of all symptoms with medical therapy. An evaluation—including measurement of the patient's anti-platelet factor 4 antibodies, lactate dehydrogenase, and haptoglobin—was begun to look for the cause of the patient's new thrombocytopenia.

The patient remained stable without any change in his vital signs over the next 6 hours but then experienced abrupt onset of chest pain and worsened shortness of breath that evening. As the patient was being examined, he suddenly became pulseless. Telemetry monitoring revealed a new right bundle branch block with a prolonged PR interval followed by abrupt complete heart block. The patient regained a pulse after immediate cardiopulmonary resuscitation (CPR) followed by the administration of 1 mg of epinephrine and atropine, but within 10 minutes he again went into asystole, requiring re-initiation of CPR. He was started on an epinephrine drip and transcutaneous pacing. A secondary survey revealed absent breath sounds over the left hemithorax. Needle thoracostomy released more than 4 L of blood from the patient's chest. The patient again lost a pulse. Bilateral femoral central venous lines were placed, with infusion of more than 12 L of crystalloid. Despite continued CPR, fluid resuscitation, and multiple rounds of vasopressor agents, the patient did not regain a pulse and was pronounced dead.

Consent was obtained for an autopsy. Gross findings revealed widespread extranodal lymphoma involving the kidneys, adrenal glands, thyroid, psoas muscle, lungs, bowel, brain, and heart. There was no involvement of the bone marrow or the meninges. Examination of the heart showed the pericardial sac to be markedly thickened by extensive tumor (Fig. 1A). There was a transmural free-wall rupture of the anterolateral left ventricle, remote from an anteroseptal scar from prior myocardial infarction (Fig. 1B), with a pericardial effusion of approximately 30 cc of blood and clots. There was no obstructive epicardial coronary artery thrombus, and the patient's stent was patent. Microscopic examination of the heart disclosed extensive and diffuse tumor infiltrates and, in the lateral LV free wall, sheets of malignant lymphocytes along the site of rupture (Fig. 2). No acute myocardial infarct was present.

figure 13FF1
Fig. 1 A) Gross autopsy revealed thickened pericardium that was diffusely infiltrated with tumor. There were focal areas of nodular involvement. The pericardial sac appeared intact. B) The left ventricular free wall had a focal area of transmural rupture, ...
figure 13FF2
Fig. 2 A) Microscopic examination with hematoxylin and eosin stain (orig. ×25) revealed transmural tumor infiltration tracking from epicardium to endocardium. B) On high-power examination (orig. ×400), these cells were multinucleated and ...


Although uncommon, direct cardiac infiltration of tumor can have disastrous consequences. This patient's initial symptoms, in conjunction with his known history of coronary artery disease, were most consistent with an acute coronary syndrome. In this instance, however, the pseudoinfarct pattern seen on the ECG probably resulted from replacement of cardiac muscle by tumor; the elevated troponin level may have been caused by cardiomyocyte damage from infiltrating tumor. The patient tolerated heparin and eptifibatide infusions without immediate complication, which suggests that his later hemodynamic collapse was an immediate consequence of sudden rupture. It is unclear whether the thrombocytopenia resulted from the infusion of eptifibatide or heparin, or was a consequence of underlying disseminated intravascular coagulation and progression of the patient's lymphoma.

On microscopic examination, the lymphoma cells appeared to lack cohesion, which quite possibly predisposed the heart to free-wall rupture in the setting of thrombocytopenia. Once the LV ruptured, cardiac tamponade ensued abruptly, despite the presence of only 30 cc of blood in the pericardial space, due to a poorly compliant pericardium that was also heavily infiltrated with tumor. No direct connection could be found between the pericardial sac and the pleural space; it is unclear whether the free-wall rupture tracked into the pleural space, or whether the massive hemothorax resulted from resuscitation-related bleeding in the setting of thrombocytopenia.

To our knowledge, this is the 1st reported case of LV free-wall rupture due to transmural infiltration of HIV-associated peripheral T-cell lymphoma. Other authors have reported right ventricular rupture from a likely T-cell lymphoma,6 T-cell lymphoma metastatic to the right ventricle that was responsive to chemotherapy,7 and natural killer/T-cell lymphoma directly invading the left atrium and interventricular septum.8 Although many diagnoses are made only at autopsy, diagnosis of cardiac infiltration by lymphoma has in some cases been made by means of cardiac magnetic resonance imaging7 or endomyocardial biopsy.9

Patients with lymphoma can have many causes of chest pain. The differential diagnosis includes not only acute coronary syndromes and pulmonary embolism, but also cardiac dysfunction due to direct involvement of the tumor with the pericardium or myocardium. In conclusion, direct myocardial infiltration, although rare, should be considered in immunocompromised patients with lymphoma and symptoms of chest pain. Pre-mortem diagnosis requires a high index of suspicion in these critically ill patients, for whom there may be effective therapy.


Address for reprints: Ehrin J. Armstrong, MD, 505 Parnassus Ave., Box 0124, San Francisco, CA 94143–0124

E-mail: moc.liamg@gnortsmra.nirhe


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