[Downloaded free from http://www.j-pcs.org on Thursday, June 29, 2023, IP: 251.73.208.

165]

Case Report

A Case of Heart Failure and Polycythemia: Treated with

Phlebotomy
Ashwin Kodliwadmath, Dibbendhu Khanra
Department of Cardiology, All India Institution of Medical Sciences, Rishikesh, Uttarakhand, India

Abstract
Polycythemia in heart failure (HF) is known but rarely encountered. A 76‑year‑old male presented with New York Heart Association Class III
HF symptoms leading to secondary polycythemia. He had underlying ischemic cardiomyopathy not amenable to revascularization and thus
was put on optimal medical therapy. However, after being refractory to medical management, he was treated with phlebotomy showing a
significant improvement in his symptoms as well as a drop in hemoglobin level. Diagnostic algorithm and management of polycythemia
related to HF are discussed.

Keywords: Heart failure, phlebotomy, polycythemia

Introduction
Polycythemia in heart failure (HF) ranges from 1.2% to 5.9%
in the literature but is largely is underreported.[1] Polycythemia
in HF has been found to be associated with poor quality of life,
more symptoms, and even higher mortality.[2] The presence of
polycythemia in a HF patient imposes diagnostic as well as
therapeutic challenges and role of phlebotomy in the treatment
of polycythemia in patients of HF is not well established.
Here, we describe a patient with HF secondary to ischemic
cardiomyopathy, which was complicated by secondary
polycythemia and successfully managed by phlebotomy.
Case Report
A   76‑year‑old  gentleman resident of a plain area presented
with dyspnea on exertion progressing from New York Heart
Association (NYHA) Class II to Class III over the past 6 months
with episodes of paroxysmal nocturnal dyspnea (PND) for the
past 1 month. His hypertension and diabetes mellitus were
well controlled with tablet telmisartan 40 mg once daily, tablet
metformin 500 mg twice daily, and tablet glimepiride 1 mg
twice daily. He never smoked or drank alcohol. He suffered
myocardial infarction 10 years ago and was on tablet aspirin
150 mg once daily, tablet clopidogrel 75 mg once daily, tablet
atorvastatin 40 mg once daily, and tablet metoprolol tartrate
Access this article online
Quick Response Code:
Website:
www.j‑pcs.org
25 mg twice daily. The patient lives in a kutcha house with
overcrowding with no adequate ventilation and biomass fuel
is used for cooking. His pulse rate was 80 beats/min, blood
pressure of 120/70 mmHg, and normal jugular venous pressure
with positive hepatojugular reflux. He had a noticeably ruddy
complexion with SpO2 of 90% on room air which increased
to 97% on administrating oxygen at 2 l/min by nasal prongs.
The SpO2 measured after a 6‑min walk test was 82% on room
air. Systemic examination revealed left ventricular (LV) S3
and bilateral basal crepitations without any organomegaly.
Electrocardiogram showed sinus rhythm with poor R
progression. Transthoracic echocardiography showed LV
global hypokinesia with ejection fraction  (EF) of 30% by
the biplane Simpson’s method. His N‑terminal fragment
of pro‑B‑type natriuretic peptide was 165.5 pmol/L
(normal: ≤50 pmol/L). Hematological parameters revealed
Address for correspondence: Dr. Dibbendhu Khanra,
Department of Cardiology, All India Institution of Medical Sciences,
Rishikesh, Uttarakhand, India.
E‑mail: ddk3987@gmail.com
Submitted: 24‑Jan‑2020 Revised: 21-Feb-2020
Accepted: 14-Mar-2020 Published: 27-Aug-2020
This is an open access journal, and articles are distributed under the terms of the Creative
Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to
remix, tweak, and build upon the work non‑commercially, as long as appropriate credit
is given and the new creations are licensed under the identical terms.
For reprints contact: WKHLRPMedknow_reprints@wolterskluwer.com

DOI: How to cite this article: Kodliwadmath A, Khanra D. A case of heart

10.4103/jpcs.jpcs_6_20 failure and polycythemia: Treated with phlebotomy. J Pract Cardiovasc
Sci 2020;6:169-71.

© 2020 Journal of the Practice of Cardiovascular Sciences | Published by Wolters Kluwer - Medknow 169
[Downloaded free from http://www.j-pcs.org on Thursday, June 29, 2023, IP: 251.73.208.165]
Kodliwadmath and Khanra: HF_polycythemia
hemoglobin of 213 g/L (normal: 130–165 g/L), hematocrit
65% (normal: 45%–52%), total leukocyte count
6.92 × 10 9 /L (normal: 4–11 × 10 9 /L), platelet count
163 × 109/L (normal: 150–450 × 109/L), and peripheral
blood film showing increased density of red blood cells.
Workup for polycythemia revealed red cell mass of 42 mg/
kg (normal: ≤35 mg/kg), serum erythropoietin level 55 IU/L
(normal: 3.6–36 IU/L), negative JAK STAT mutation, and
absence of splenomegaly on abdominal ultrasonography.
Pulmonary function test was normal and no abnormality
was detected in chest X‑ray. Carboxyhemoglobin level was
14% (normal in nonsmokers ≤3%; range in smokers 10%–
15%), probably due to the biomass fuel used for cooking. The
serum iron was 32 µmol/L (normal: 11–33 µmol/L) and serum
ferritin was 0.64 nmol/L (normal: 0.027–0.6742 nmol/L).
Workup for HF included coronary angiography which showed
triple‑vessel disease with a calculated SYNTAX score of 26.5.
As per heart team opinion, gadolinium‑enhanced cardiac
magnetic resonance imaging was done which revealed late
gadolinium enhancement extending from the subendocardial
region to involve 50% of wall thickness in the left anterior
descending and right coronary artery region territory with LV
EF of 30%, and thus, the patient was kept on optimal medical
therapy.
After 1 week of treatment with intravenous furosemide and
tablet eplerenone, the patient remained in NYHA Class III
with resting SpO2 at 90%. As advised by hematology and
transfusion medicine team, therapeutic phlebotomy was done
from the right femoral vein and 300 ml of blood was removed.
There were no complications such as hematoma or syncope.
The patient had improvement in his symptoms within 24 h of
phlebotomy with no more episodes of PND and regression
to NYHA Class II with improvement in the SpO2 on room
air to 94% and he was started on tablet carvedilol 3.125 mg
12 hourly and tablet nitroglycerine 2.6 mg 12 hourly. Repeat
echocardiography still revealed an EF of 30% by the biplane
Simpson’s method.
The patient was followed up after 1 week in the outpatient
department. He had changed his place of residence to a
well‑ventilated house and started using the liquefied petroleum
gas for cooking to decrease the ill effects of overcrowding and
indoor air pollution as advised. His ruddy complexion had
recovered remarkably and he remained in NYHA Class II.
Complete hemogram showed hemoglobin of 189 g/L and
hemtocrit of 57.18%. Screening echocardiography still showed
Table 1: Clinical status of the heart failure‑related secondary polycythemia patient before and after phlebotomy
Parameter Presentation After After
OMT phlebotomy
NYHA class III III II
SpO2 (%) in room air 90 90 94
LVEF (%) by echocardiography 30 30 30
LVEF was calculated using the biplane Simpson’s method. OMT: Optimal medical therapy, LVEF: Left ventricular ejection fraction, NYHA: New York
Heart Association
170 Journal of the Practice of Cardiovascular Sciences  ¦  Volume 6  ¦  Issue 2  ¦  May-August 2020
severe LV systolic dysfunction with EF of 30% by the biplane
Simpson’s method and resting SpO2 was 95%. Dietary and
medical advice was reinforced and he was sent home. He
was followed up again after 1 month and hemoglobin was
151 g/L and hematocrit was 44.14%. Significant improvement
in the functional capacity and quality of life was noted with
the patient in NYHA Class I with a resting SpO2 of 96%.
The echocardiography still revealed an EF of 30% by the
biplane Simpson’s method. Pulmonary function tests and
polysomnography were done which were normal, thus ruling
out chronic obstructive pulmonary disease and obstructive
sleep apnea, respectively. Follow‑up at 3 months and 6 months
showed a significant improvement in SpO2 and NYHA class
but persistent EF of 30% [Table 1].
Discussion
Patients with HF and polycythemia are at risk of stroke and
myocardial ischemia, as high hemoglobin level can promote
systemic vasoconstriction by trapping nitric oxide and by
generation of oxygen‑derived free radicals.[2] Although HF
itself can cause polycythemia due to chronic hypoxia, other
causes should always be sought and ruled out. A decreased
serum erythropoietin level and a positive JAK STAT mutation
confirm primary polycythemia. Elevated erythropoietin levels
with low SpO2 dictate evaluation for heart or lung disease. An
increased carboxyhemoglobin level in a smoker clinches a
diagnosis of smoker’s polycythemia. Increased hemoglobin O2
affinity indicates a hemoglobinopathy, while a normal affinity
dictates a search for a tumor secreting erythropoietin.
Unlike anemia in HF, there is no consensus in managing
polycythemia secondary to HF. Therapeutic phlebotomy
is recommended for cyanotic congenital heart disease that
presents with hemoglobin levels of >20 g/dL and hematocrit
levels of >65%.[3] The goal of phlebotomy must be to maintain a
hematocrit <45%.[3] Patients with hypoxemic lung disease who
exhibit symptoms of hyperviscosity syndrome with hematocrit
levels >56% are recommended to undergo phlebotomy to
reduce their hematocrit levels to 50%–52%. However, there
is no consensus for phlebotomy in polycythemia with HF.[4]
Phlebotomy is of three types: allogenic – blood drawn from
donors for storage in hospital blood banks; autologous – where
the donor is the recipient, and therapeutic – where blood
is drawn for therapeutic reduction of iron or red cells.[5]
Therapeutic erthrocytapheresis is an apheresis technique
by which red blood cells are separated from whole blood
At 1 week 1 month 3 months 6 months
discharge follow up follow up follow up follow up
II II I I I
94 95 96 98 99
30 30 30 30 30
[Downloaded free from http://www.j-pcs.org on Thursday, June 29, 2023, IP: 251.73.208.165]
Kodliwadmath and Khanra: HF_polycythemia
extracorporeally and the remaining blood returned to the
circulation. It is useful in polycythemia vera but rarely
recommended for secondary polycythemia.[6] Auchincloss
and Duggan observed increases in functional residual
capacity, residual volume, and total lung capacity but no
significant changes in vital capacity in patients with chronic
pulmonary emphysema and secondary polycythemia who
underwent phlebotomy.[7] Dayton et al. have shown that
phlebotomy in polycythemia secondary to chronic lung
disease improves subjective benefit, especially in patients
with associated HF and hematocrit more than 60%, and the
benefit is probably due to improvement in the blood viscosity,
although a decrease in the blood volume may also play a
role.[8] Segal and Bishop have shown that patients with more
severe lung disease and congestive HF demonstrated a slight
fall in various indices except pulmonary wedge pressure and
cardiac output following repeated phlebotomy.[9]
Although we offered medical therapy for our patient, the
perioperative management of patients of polycythaemia
undergoing cardiopulmonary bypass (CPB) undergoing
coronary artery bypass grafting (CABG) is challenging as
the chances of thromboembolic complications increase. The
perioperative management may include weekly phlebotomy
to reduce hematocrit to <45%, phlebotomy after induction
of anesthesia, liberal perioperative fluid administration,
maintenance of core temperature, and the use of low‑dose
heparin.[10]
Every effort should be made to rule out other causes before
labeling HF as the cause of polycythemia. Hematocrit ≥55%
with symptoms of HF refractory to standard medical therapy
for HF should be an indication of phlebotomy which can have
a significant impact on the patient’s symptoms and functional
capacity, with a goal to maintain hematocrit ≤45%. Usually,
single phlebotomy would suffice, but multiple procedures
may be required depending on the patient’s symptoms and
hematocrit level. CPB for CABG in patients with polycythemia
needs vigilant perioperative management. More case series are
required to standardize these recommendations to patients with
HF and polycythemia.
Journal of the Practice of Cardiovascular Sciences  ¦  Volume 6 ¦ Issue 2 ¦ May-August 2020
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form the patient(s) has/have
given his/her/their consent for his/her/their images and other
clinical information to be reported in the journal. The patients
understand that their names and initials will not be published
and due efforts will be made to conceal their identity, but
anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
1. Taegtmeyer AB, Banner NR. Polycythaemia and chronic heart
failure. Curr Cardiol Rev 2005;1:117‑26.
2. Sharma R, Francis DP, Pitt B, Poole‑Wilson PA, Coats AJ,
Anker SD. Haemoglobin predicts survival in patients with chronic
heart failure: A substudy of the ELITE II trial. Eur Heart J
2004;25:1021‑8.
3. Kim KH, Oh KY. Clinical applications of therapeutic phlebotomy.
J Blood Med 2016;7:139‑44.
4. Barbui T, Passamonti F, Accorsi P, Pane F, Vannucchi AM, Velati C,
et al. Evidence‑ and consensus‑based recommendations for
phlebotomy in polycythemia vera. Leukemia 2018;32:2077‑81.
5. Cook LS. Therapeutic phlebotomy: A review of diagnoses and
treatment considerations. J Infus Nurs 2010;33:81‑8.
6. Valbonesi M, Bruni R. Clinical application of therapeutic
erythrocytapheresis (TEA). Transfus Sci 2000;22:183‑94.
7. Auchincloss JH Jr., Duggan JJ. Effects of venesection on pulmonary
and cardiac function in patients with chronic pulmonary emphysema
and secondary polycythemia. Am J Med 1957;22:74‑82.
8. Dayton LM, McCullougy RE, Scheinhorn DJ, Weil JV. Symptomatic
and puomonary response to acute phlebotomy in secondary
polycythemia. Chest 1975;68:785‑90.
9. Segel N, Bishop JM. The circulation in patients with chronic
bronchitis and emphysema at rest and during exercise, with
special reference to the influence of changes in blood viscosity
and blood volume on the pulmonary circulation. J Clin Invest
1966;45:1555‑68.
10. Arora D, Juneja R, Pendarkar D, Mehta Y, Trehan N. Off‑pump
coronary artery bypass grafting in a polycythaemic patient–Case
report and review of literature. Ann Card Anaesth 2007;10:54‑7.
171