Cardiac hypertrophy in polycythemia vera: A case report and review of literature

Table 1 Blood test results of the patient at successive hospitalizations

Parameters	March 2021	January 2022	August 2022	October 2023	December 2023	Reference range
Hemoglobin	200 g/L	200 g/L	178 g/L	181 g/L	155 g/L	120-160 g/L
Hematocrit	0.595 L/L	0.569 L/L	0.516 L/L	0.524 L/L	0.449 L/L	0.4-0.5 L/L
Erythrocyte count	6.79 × 1012/L	6.63 × 1012/L	6.01 × 1012/L	5.99 × 1012/L	4.63 × 1012/L	4 × 1012-5.5 × 1012/L
White blood cells	9.22 × 109/L	7.49 × 109/L	8.14 × 109/L	9.16 × 109/L	8.7 × 109/L	4 × 109-10 × 109/L
Platelet count	197 × 109/L	227 × 109/L	209 × 109/L	223 × 109/L	257 × 109/L	100 × 109-300 × 109/L

Table 2 Results of cardiac color Doppler ultrasound

Parameters	March 2021	January 2022	August 2022	October 2023
IVS (mm)	22	20.6	20.2	22
LVPW (mm)	21.9	28.9	18	16.4
EF%	75.4	75.8	67.2	68.4

IVS: Interventricular; LVPW: Left ventricular posterior wall; EF: Ejection fraction.

Table 3 Case reports of polycythemia vera complicated with cardiac disease

Ref.	Biographical information	Diagnosis	Therapy	Prognosis	Pivot
Bahbahani et al[9]	Egyptian woman aged 37 years	Acute myocardial infarction, PV	Thrombolysis, hydroxyurea 15 mg/kg, aspirin 81 mg	After 4 weeks, myocardial perfusion imaging of the patient revealed no evidence of myocardial ischemia. Coronary CT angiography showed normal findings	Young individuals without atherosclerosis and its associated risk factors may experience cardiovascular thrombotic events due to PV
Zaman et al[7]	61-year-old female	Heart failure, microcirculatory disorder, PV	Normally treated with bloodletting, aspirin, and clopidogrel after diagnosis	During follow-up, the patient did not experience any new episodes of chest pain	PV can lead to microembolism in the cardiac microcirculation, resulting in impaired cardiac function
Duran Luciano and Sabella-Jiménez[31]	52-year-old Hispanic male	Acute myocardial infarction, JAK2 negative PV	Antiplatelet, anticoagulation, and PCI therapy	Follow-up revealed improvement in cardiac function compared to previous assessments	JAK2-negative PV can also lead to cardiovascular thrombotic events
Inami et al[32]	64-year-old male	Acute myocardial infarction, recurrence of myocardial infarction after PCI, PV	PCI treatment, phlebotomy, and hydroxyurea for PV	No complications occurred	Patients with PV have a high risk of intrastent thrombosis following PCI
D'Onofrio et al[33]	86-year-old female	Severe stenotic aortic valve, pulmonary edema, post aortic valve replacement, respiratory circulatory failure	Aortic valve replacement, ECMO, CPR	The patient died	PV accompanied by severe thrombocytosis precluded antiplatelet and anticoagulant therapy, resulting in death from cerebral hemorrhage. Autopsy revealed extensive white thrombi formation in both the aortic valve and ventricles
Butt and Latif[34]	49-year-old male	Dilated cardiomyopathy, New York Classification III	Aspirin 100 mg, ramipril and bisoprolol in an increasing dose titration regimen. Furosemide 40 mg	During follow-up, the ejection fraction improved from 18% to 42%	Microvascular myocyte necrosis is considered the sole plausible pathophysiology of the cardiomyopathy
Haroun et al[35]	71-year-old Ethiopian man	PV, pericardial effusion, post-PV myelofibrosis	Discontinuation of hydroxyurea, pericardiocentesis	At 8 weeks following the initial consultation, during outpatient follow-up, complete blood cell counts revealed a leukocyte count of 13.6 × 109 cells/L, hemoglobin level of 9.9 g/dL, and platelet count of 556000/L	PV progressed to bone marrow fibrosis, resulting in extramedullary hematopoiesis and the formation of pericardial effusion

CT: Computed tomography; PV: Polycythemia vera; PCI: Percutaneous coronary intervention; JAK2: Janus kinase 2; ECMO: Extracorporeal membrane oxygenation; CPR: Cardiopulmonary resuscitation.

Table 4 Characteristics of different cardiac hypertrophy diseases

Name of disease	Typical features	Means of identification
HCM	Asymmetric septal hypertrophy, often accompanied by left ventricular outflow tract obstruction	Genetic testing and cardiac MRI
Hypertensive heart disease	Symmetrical myocardial hypertrophy, generally, ventricular wall thickness is ≤ 15 mm	History of hypertension for many years
Fabry disease	Concentric hypertrophy	α-Galactosidase A activity assay, GLA gene test
Myocardial amyloidosis	Symmetrical myocardial hypertrophy, characterized on electrocardiography by low voltage or normal voltage	Radionuclide imaging, cardiac biopsy line histology and amyloid staining
Physiological hypertrophy	In athletes, the unique condition of mild, uniform left ventricular wall thickening may be accompanied by an increase in left ventricular cavity diameter	Cardiopulmonary exercise test

HCM: Hypertrophic cardiomyopathy; MRI: Magnetic resonance imaging.

Table 5 World Health Organization criteria for polycythemia vera

Major criteria	Minor criterion
Hemoglobin 16.5 g/dL in men Hemoglobin 16.0 g/dL in women, or Hematocrit 49% in men Hematocrit 48% in women, or increased RCM1	Subnormal serum erythropoietin level
BM biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic, mature megakaryocytes (differences in size)
Presence of JAK2 V617F or JAK2 exon 12 mutation

¹Diagnosis of polycythemia vera requires meeting either all 3 major criteria, or the first 2 major criteria and the minor criterion.

RCM: Red cell mass; JAK2: Janus kinase 2.