Int J Cardiovasc Sci. 2016;29(6):517-519 517

CASE REPORT

Polycythemia Vera: A Rare Ethiology of Heart Failure Monyck Barros Cardoso1, Marcelo Foradini Albuquerque2, Rosa Regina Sannuti Pais3, Renata Rodrigues Teixeira de Castro1,2,3 Hospital Geral de Nova Iguaçu1, Nova Iguaçu, RJ; Universidade Iguaçu2, Nova Iguaçu, RJ; Hospital Naval Marcílio Dias3, Rio de Janeiro, RJ − Brazil

Introduction Case report

The prevalence of heart failure (HF) with systolic The patient is a 47-year-old dark-skinned man, born dysfunction exceeds 20 million patients worldwide, in Belo Horizonte (MG) and currently working as a which corresponds to 2% of the Western population.1 gardener in the state of Rio de Janeiro. He sought the Over the past two decades, there have been major emergency service reporting dyspnea for 2 months, advances in the outpatient treatment of individuals with initially to moderate effort, which had progressed to HF, including the use of not only pharmacotherapy (use dyspnea at rest in the previous week. Associated with of beta-blockers and aldosterone receptor blockers),2 but the presentation, he reported orthopnea and paroxysmal also implantable devices (resynchronization devices and nocturnal dyspnea. He denied chest pain, fever, weight implantable defibrillators).3 Despite all these advances, loss, or recent infections. In his past medical history, he the mortality of patients with HF remains high, exceeding, had no reports of comorbidities, hospitalizations, previous for example, many oncological diseases. Another major diseases, or surgeries. He denied allergies, smoking, and treatment challenge in this area refers to the outcomes alcoholism. Despite working as a gardener, the patient considered as secondary but also important, such as the was a retired military and had worked with ship boilers improvement in quality of life and the reduction in the for several years. He was unaware of the cause of death number of hospitalizations in these patients.1 of his parents and had two sisters who were alive and With regard to the etiology, there are different causes apparently healthy. There were no other relevant data in of HF, and their recognition can be crucial for treatment his medical history. optimization. Ventricular dysfunction secondary to On physical examination, the patient was eupneic at myocardial ischemia, arrhythmias, and hypothyroidism, rest. Had no pallor of conjunctiva or mucous membranes, among others, should be treated with a focus on the was anicteric, acyanotic, hydrated, and presented lower underlying disease.2 Thus, the identification of the HF extremities edema. He presented jugular venous distension cause must be pursued until possibilities are exhausted, at 45°, a two-paced regular cardiac rhythm, normal cardiac so that its treatment can be individualized. In fact, the sounds, a heart rate of 92 bpm, and blood pressure of 110 x so-called cardiomyopathies of idiopathic origin have 60 mmHg. There were no murmurs or pericardial friction. a worse prognosis when compared with those whose On pulmonary auscultation, he presented decreased etiology is known.4 breath sounds at both lung bases, with crackles in the The objective of this study was to report a case of HF lower two-thirds of both lungs. His abdomen was soft, secondary to polycythemia vera, a rare hematologic disease. tympanic, with peristaltic movements, and painful on deep palpation in the right hypochondrium. His liver had normal measurements and a blunt edge, and was painful Keywords to palpation. His Traube's space was occupied, but his Heart Failure / etiology; Polycythemia Vera / spleen was not palpable. There was no peripheral edema. etiology; Microcirculation; Hematologic Diseases / Considering a diagnosis of HF, treatment with physiopathology. vasodilators (angiotensin converting enzyme inhibitor)

Mailing Address: Renata Castro • Avenida Abílio Augusto Távora, 2.134, Jardim Nova Era. Postal Code: 26275-580. Nova Iguaçu, RJ - Brazil E-mail: [email protected]

DOI: 10.5935/2359-4802.20170010 Manuscript received July 25, 2016; revised manuscript November 21, 2016; accepted December 4, 2016. Int J Cardiovasc Sci. 2016;29(6):517-519 Cardoso et al. Case Report Heart failure and polycythemia vera 518

and a diuretic (furosemide) was initiated and tests were hypervolemia and the increase in hematocrit may justify requested. Since the patient lived far from the hospital, it the higher cardiac output and aerobic capacity. However, was decided to admit him to better evaluate and stabilize this statement is true up to certain limits. his clinical condition. In fact, extreme increases in hematocrit may increase the His tests revealed polycythemia (with a hematocrit blood viscosity to levels that lead to higher blood viscosity of 50.7% and a hemoglobin level of 17.1 g%) with levels to hypercoagulability. However, the thrombophilic normocitosis. Leukogram, biochemistry, and liver mechanism in patients with polycythemia vera is, in fact, and thyroid function tests were normal. Transthoracic even more complex. It includes not only the increased echocardiography revealed dilated cardiomyopathy with hematocrit and platelet count rates, but also the interactions a left ventricular ejection fraction of 24%. among platelets, leukocytes, and cellular products and a 7 Despite the patient's clinical improvement, his medical reduction in endogenous anticoagulants. Most of the few history precluded an identification with a certainty of the cases found in the literature in which polycythemia vera has cause of his severe ventricular dysfunction, considering led to HF occurred due to hypercoagulability. These cases that he was young and had no classic risk factors for HF. To have reported massive intraventricular thrombi causing search for his condition's etiology, myocardial scintigraphy obstructive HF, with no direct damage to the mechanisms 8,9 with dipyridamole was performed, blood cultures were of ventricular contraction. collected, and tuberculosis, HIV, Chagas disease, and In the international literature, we found only one case of collagen diseases were investigated. All these tests were polycythemia vera that evolved to ventricular dysfunction negative. Considering the increased hematocrit, we chose due to cardiomyocyte necrosis and slow blood flow in the to perform a pulmonary evaluation with spirometry at coronary microcirculation, probably due to hyperviscosity.10 rest, which was also normal. In spite of our patient having refused to undergo Finally, his increased hematocrit in the absence endomyocardial biopsy, the absence of intracardiac thrombi of smoking or lung disease led to an investigation of on echocardiography makes the hypothesis of microinfarcts polycythemia vera, which was confirmed. The patient was the only possible pathophysiological mechanism for offered the option to undergo endomyocardial biopsy but his ventricular dysfunction. It is worth noting that the refused it. Cardiac magnetic resonance was not available; negative outcome for myocardial ischemia on myocardial therefore it was not performed. scintigraphy does not exclude our pathophysiological hypothesis since this method does not have adequate With the diagnosis of polycythemia vera, aspirin was sensitivity to identify microinfarcts. added to the patient's treatment. After hospital discharge, the patient was referred to a cardiology and hematology As illustrated in this case, the certainty of the etiological service at his city for follow-up. association in cases of HF is not always simple and straightforward. In fact, the reasonable pathophysiological Discussion implication and the absence of other possible etiologies are the main factors that lead to the etiological definition Polycythemia vera, a myeloproliferative disease, was (or strong suspicion) in most cases of nonischemic HF in first described by Vasquez in 1892 and, subsequently, by clinical practice. Osler in 1903, and is also known as Osler-Vaquez disease.5 In the present case, once the main HF causes among It is a rare disease (4–16 cases/million), characterized by us were excluded, and due to the refusal of the patient increased hematocrit and, consequently, blood viscosity. to undergo endomyocardial biopsy, only two options The relationship of ventricular function seems to show would remain for the diagnostic conclusion: idiopathic a paradoxical behavior in respect to polycythemia vera. A nonischemic HF in a patient with polycythemia vera or HF study conducted in the 1960s examined invasively examined secondary to polycythemia vera. the hemodynamic profile of 10 patients with polycythemia Considering the rarity of polycythemia vera,5,11 the vera and compared them with those of healthy individuals,6 existence of previous reports pointing to the possible concluding that patients with polycythemia vera exhibited development of HF in patients with this hematological hypervolemia, which translated into an increased cardiac disease,6,10 the absence of other causes for the HF in this output and oxygen consumption. Taking into account patient, and the medical reasoning of always trying the pathophysiological mechanisms, the occurrence of to explain a patient's complaints with the lowest Cardoso et al. Int J Cardiovasc Sci. 2016;29(6):517-519 519 Heart failure and polycythemia vera Case Report

possible number of diagnoses, we believe that the most Cardoso MB, Albuquerque MF, Pais RRS, Castro RRT. reasonable option to consider is that of polycythemia Writing of the manuscript: Cardoso MB, Castro RRT. vera as the cause of this patient’s HF syndrome, and Critical revision of the manuscript for intellectual content: not as an associated diagnosis in this case. Albuquerque MF, Pais RRS, Castro RRT.

Conclusion Potential Conflict of Interest We present a case in which a causal association No potential conflict of interest relevant to this article between polycythemia vera and HF was made by was reported. exclusion of other diagnoses. The etiology of HF must be

sought in all new cases, because its recognition can direct the therapy and influence the prognosis of these patients. Sources of Funding There were no external funding sources for this study. Author contributions

Conception and design of the research: Castro RRT. Study Association Acquisition of data: Cardoso MB, Albuquerque MF, Pais This study is not associated with any thesis or RRS, Castro RRT. Analysis and interpretation of the data: dissertation work.

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