Download Polycythemia Vera: A Rare Ethiology of Heart Failure

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
dysfunction exceeds 20 million patients worldwide,
which corresponds to 2% of the Western population.1
Over the past two decades, there have been major
advances in the outpatient treatment of individuals with
HF, including the use of not only pharmacotherapy (use
of beta-blockers and aldosterone receptor blockers),2 but
also implantable devices (resynchronization devices and
implantable defibrillators).3 Despite all these advances,
the mortality of patients with HF remains high, exceeding,
for example, many oncological diseases. Another major
treatment challenge in this area refers to the outcomes
considered as secondary but also important, such as the
improvement in quality of life and the reduction in the
number of hospitalizations in these patients.1
The patient is a 47-year-old dark-skinned man, born
in Belo Horizonte (MG) and currently working as a
gardener in the state of Rio de Janeiro. He sought the
emergency service reporting dyspnea for 2 months,
initially to moderate effort, which had progressed to
dyspnea at rest in the previous week. Associated with
the presentation, he reported orthopnea and paroxysmal
nocturnal dyspnea. He denied chest pain, fever, weight
loss, or recent infections. In his past medical history, he
had no reports of comorbidities, hospitalizations, previous
diseases, or surgeries. He denied allergies, smoking, and
alcoholism. Despite working as a gardener, the patient
was a retired military and had worked with ship boilers
for several years. He was unaware of the cause of death
of his parents and had two sisters who were alive and
apparently healthy. There were no other relevant data in
his medical history.
With regard to the etiology, there are different causes
of HF, and their recognition can be crucial for treatment
optimization. Ventricular dysfunction secondary to
myocardial ischemia, arrhythmias, and hypothyroidism,
among others, should be treated with a focus on the
underlying disease.2 Thus, the identification of the HF
cause must be pursued until possibilities are exhausted,
so that its treatment can be individualized. In fact, the
so-called cardiomyopathies of idiopathic origin have
a worse prognosis when compared with those whose
etiology is known.4
The objective of this study was to report a case of HF
secondary to polycythemia vera, a rare hematologic disease.
Keywords
Heart Failure / etiology; Polycythemia Vera /
etiology; Microcirculation; Hematologic Diseases /
physiopathology.
On physical examination, the patient was eupneic at
rest. Had no pallor of conjunctiva or mucous membranes,
was anicteric, acyanotic, hydrated, and presented lower
extremities edema. He presented jugular venous distension
at 45°, a two-paced regular cardiac rhythm, normal cardiac
sounds, a heart rate of 92 bpm, and blood pressure of 110 x
60 mmHg. There were no murmurs or pericardial friction.
On pulmonary auscultation, he presented decreased
breath sounds at both lung bases, with crackles in the
lower two-thirds of both lungs. His abdomen was soft,
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
to palpation. His Traube's space was occupied, but his
spleen was not palpable. There was no peripheral edema.
Considering a diagnosis of HF, treatment with
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
Case Report
and a diuretic (furosemide) was initiated and tests were
requested. Since the patient lived far from the hospital, it
was decided to admit him to better evaluate and stabilize
his clinical condition.
His tests revealed polycythemia (with a hematocrit
of 50.7% and a hemoglobin level of 17.1 g%) with
normocitosis. Leukogram, biochemistry, and liver
and thyroid function tests were normal. Transthoracic
echocardiography revealed dilated cardiomyopathy with
a left ventricular ejection fraction of 24%.
Despite the patient's clinical improvement, his medical
history precluded an identification with a certainty of the
cause of his severe ventricular dysfunction, considering
that he was young and had no classic risk factors for HF. To
search for his condition's etiology, myocardial scintigraphy
with dipyridamole was performed, blood cultures were
collected, and tuberculosis, HIV, Chagas disease, and
collagen diseases were investigated. All these tests were
negative. Considering the increased hematocrit, we chose
to perform a pulmonary evaluation with spirometry at
rest, which was also normal.
Finally, his increased hematocrit in the absence
of smoking or lung disease led to an investigation of
polycythemia vera, which was confirmed. The patient was
offered the option to undergo endomyocardial biopsy but
refused it. Cardiac magnetic resonance was not available;
therefore it was not performed.
With the diagnosis of polycythemia vera, aspirin was
added to the patient's treatment. After hospital discharge,
the patient was referred to a cardiology and hematology
service at his city for follow-up.
Discussion
Polycythemia vera, a myeloproliferative disease, was
first described by Vasquez in 1892 and, subsequently, by
Osler in 1903, and is also known as Osler-Vaquez disease.5
It is a rare disease (4–16 cases/million), characterized by
increased hematocrit and, consequently, blood viscosity.
The relationship of ventricular function seems to show
a paradoxical behavior in respect to polycythemia vera. A
study conducted in the 1960s examined invasively examined
the hemodynamic profile of 10 patients with polycythemia
vera and compared them with those of healthy individuals,6
concluding that patients with polycythemia vera exhibited
hypervolemia, which translated into an increased cardiac
output and oxygen consumption. Taking into account
the pathophysiological mechanisms, the occurrence of
Cardoso et al.
Heart failure and polycythemia vera
hypervolemia and the increase in hematocrit may justify
the higher cardiac output and aerobic capacity. However,
this statement is true up to certain limits.
In fact, extreme increases in hematocrit may increase the
blood viscosity to levels that lead to higher blood viscosity
levels to hypercoagulability. However, the thrombophilic
mechanism in patients with polycythemia vera is, in fact,
even more complex. It includes not only the increased
hematocrit and platelet count rates, but also the interactions
among platelets, leukocytes, and cellular products and a
reduction in endogenous anticoagulants.7 Most of the few
cases found in the literature in which polycythemia vera has
led to HF occurred due to hypercoagulability. These cases
have reported massive intraventricular thrombi causing
obstructive HF, with no direct damage to the mechanisms
of ventricular contraction.8,9
In the international literature, we found only one case of
polycythemia vera that evolved to ventricular dysfunction
due to cardiomyocyte necrosis and slow blood flow in the
coronary microcirculation, probably due to hyperviscosity.10
In spite of our patient having refused to undergo
endomyocardial biopsy, the absence of intracardiac thrombi
on echocardiography makes the hypothesis of microinfarcts
the only possible pathophysiological mechanism for
his ventricular dysfunction. It is worth noting that the
negative outcome for myocardial ischemia on myocardial
scintigraphy does not exclude our pathophysiological
hypothesis since this method does not have adequate
sensitivity to identify microinfarcts.
As illustrated in this case, the certainty of the etiological
association in cases of HF is not always simple and
straightforward. In fact, the reasonable pathophysiological
implication and the absence of other possible etiologies
are the main factors that lead to the etiological definition
(or strong suspicion) in most cases of nonischemic HF in
clinical practice.
In the present case, once the main HF causes among
us were excluded, and due to the refusal of the patient
to undergo endomyocardial biopsy, only two options
would remain for the diagnostic conclusion: idiopathic
nonischemic HF in a patient with polycythemia vera or HF
secondary to polycythemia vera.
Considering the rarity of polycythemia vera,5,11 the
existence of previous reports pointing to the possible
development of HF in patients with this hematological
disease,6,10 the absence of other causes for the HF in this
patient, and the medical reasoning of always trying
to explain a patient's complaints with the lowest
518
Cardoso et al.
519
Int J Cardiovasc Sci. 2016;29(6):517-519
Case Report
Heart failure and polycythemia vera
possible number of diagnoses, we believe that the most
reasonable option to consider is that of polycythemia
vera as the cause of this patient’s HF syndrome, and
not as an associated diagnosis in this case.
Cardoso MB, Albuquerque MF, Pais RRS, Castro RRT.
Writing of the manuscript: Cardoso MB, Castro RRT.
Critical revision of the manuscript for intellectual content:
Albuquerque MF, Pais RRS, Castro RRT.
Conclusion
We present a case in which a causal association
between polycythemia vera and HF was made by
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.
Potential Conflict of Interest
No potential conflict of interest relevant to this article
was reported.
Sources of Funding
There were no external funding sources for this study.
Author contributions
Conception and design of the research: Castro RRT.
Acquisition of data: Cardoso MB, Albuquerque MF, Pais
RRS, Castro RRT. Analysis and interpretation of the data:
Study Association
This study is not associated with any thesis or
dissertation work.
References
1.
2.
3.
Mozaffarian D, Benjamin EJ, Go AS, Arnett DK, Blaha MJ, Cushman M,
et al; American Heart Association Statistics Committee.; Stroke Statistics
Subcommittee. Heart disease and stroke statistics-2016 update: a report
from the American Heart Association. Circulation. 2016;133(4):e38-360.
Erratum in: Circulation. 2016;133(15):e599.
5. Stone MJ. Polycythaemia vera: Osler-Vaquez disease. J Med Biogr.
2001;9(2):99-103.
McMurray JJ, Adamopoulos S, Anker SD, Auricchio A, Böhm M, Dickstein
K, et al; ESC Committee for Practice Guidelines. ESC guidelines for the
diagnosis and treatment of acute and chronic heart failure 2012: The Task
Force for the Diagnosis and Treatment of Acute and Chronic Heart Failure
2012 of the European Society of Cardiology. Developed in collaboration
with the Heart Failure Association (HFA) of the ESC. Eur J Heart Fail.
2012;14(8):803-69. Erratum in: Eur J Heart Fail. 2013;15(3):361-2.
7. Tefferi A. Polycythemia vera: a comprehensive review and clinical
recommendations. Mayo Clin Proc. 2003;78(2):174-94.
Kuck KH, Bordachar P, Borggrefe M, Boriani G, Burri H, Leyva F, et al.
New devices in heart failure: an European Heart Rhythm Association
report: developed by the European Heart Rhythm Association; endorsed
by the Heart Failure Association. Europace. 2014;16(1):109-28.
4. Shore S, Grau-Sepulveda MV, Bhatt DL, Heidenreich PA, Eapen
ZJ, Hernandez AF, et al. Characteristics, treatments, and outcomes
of hospitalized heart failure patients stratified by etiologies of
cardiomyopathy. JACC Heart Fail. 2015;3(11):906-16.
6. Cobb LA, Kramer RJ, Finch CA. Circulatory effects of chronic
hypervolemia in polycythemia vera. J Clin Invest. 1960;39:1722-8.
8.
Ali M, Fayemi AO, Malcolm D, Braun EV. Intraventricular thrombosis
in polycythemia vera: a cause of intractable cardiac failure. Am Heart J.
1980;100(4):520-2.
9.
Yuan SM, Shinfeld A, Raanani E. Massive intraventricular thrombus in
polycythemia vera. J Card Surg. 2009;24(2):110-2.
10. Konopka A, Stepinska J, Banaszewski M, Szajewski T. [A case of dilated
cardiomyopathy caused by myocardial microinfarcts in the course of
polycythemia vera]. Pol Merkur Lekarski. 1998;4(19):26-8.
11. Ma X, Vanasse G, Cartmel B, Wang Y, Selinger HA. Prevalence of
polycythemia vera and essential thrombocythemia. Am J Hematol.
2008;83(5):359-62.