|Year : 2020 | Volume
| Issue : 2 | Page : 169-171
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
|Date of Submission||24-Jan-2020|
|Date of Decision||21-Feb-2020|
|Date of Acceptance||14-Mar-2020|
|Date of Web Publication||27-Aug-2020|
Dr. Dibbendhu Khanra
Department of Cardiology, All India Institution of Medical Sciences, Rishikesh, Uttarakhand
Source of Support: None, Conflict of Interest: None
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
|How to cite this article:|
Kodliwadmath A, Khanra D. A case of heart failure and polycythemia: Treated with phlebotomy. J Pract Cardiovasc Sci 2020;6:169-71
|How to cite this URL:|
Kodliwadmath A, Khanra D. A case of heart failure and polycythemia: Treated with phlebotomy. J Pract Cardiovasc Sci [serial online] 2020 [cited 2020 Sep 20];6:169-71. Available from: http://www.j-pcs.org/text.asp?2020/6/2/169/293593
| Introduction|| |
Polycythemia in heart failure (HF) ranges from 1.2% to 5.9% in the literature but is largely is underreported. Polycythemia in HF has been found to be associated with poor quality of life, more symptoms, and even higher mortality. 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 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 hemoglobin of 213 g/L (normal: 130–165 g/L), hematocrit 65% (normal: 45%–52%), total leukocyte count 6.92 × 109/L (normal: 4–11 × 109/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 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].
|Table 1: Clinical status of the heart failure-related secondary polycythemia patient before and after phlebotomy|
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| 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. 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%. The goal of phlebotomy must be to maintain a hematocrit <45%. 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.
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. Therapeutic erthrocytapheresis is an apheresis technique by which red blood cells are separated from whole blood extracorporeally and the remaining blood returned to the circulation. It is useful in polycythemia vera but rarely recommended for secondary polycythemia. 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. 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. 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.
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.
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.
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.
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Conflicts of interest
There are no conflicts of interest.
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