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Pulmonary hypertension in patients with myelofibrosis due to myeloproliferative syndromes: a mechanism unraveled

Session Poster session 1 Saturday 08:30 -17:30

Speaker Javier Segovia Cubero

Event : Heart Failure 2015

  • Topic : valvular, myocardial, pericardial, pulmonary, congenital heart disease
  • Sub-topic : Pulmonary Hypertension
  • Session type : Poster Session

Authors : J Segovia (Madrid,ES), M Gomez-Bueno (Madrid,ES), A Jaramillo (Madrid,ES), E Ojeda (Madrid,ES), J De Haro (Madrid,ES), F Hernandez (Madrid,ES), I Sayago (Madrid,ES), S Cuenca (Madrid,ES), A Restrepo (Madrid,ES)

Authors:
J Segovia1 , M Gomez-Bueno1 , A Jaramillo1 , E Ojeda1 , J De Haro1 , F Hernandez1 , I Sayago1 , S Cuenca1 , A Restrepo1 , 1University Hospital Puerta de Hierro Majadahonda, Advanced Heart Failure - Madrid - Spain ,

Citation:
European Journal of Heart Failure Abstracts Supplement ( 2015 ) 17 ( Supplement 1 ), 116

Background: Pulmonary hypertension (PH) is a well-known complication of myeloproliferative syndromes (MPS) such as polycythemia vera (PV), essential thrombocythemia (ET) and agnogenic myelofibrosis (AM) in advanced stages. Although initially attributed to increased pulmonary vascular resistance (WHO group 1 PH), this entity was reclassified in 2009 in group 5 (unknown mechanism and miscellaneous PH). Our aim was to describe the prevalence and possible causes of PH in a series of patients MPS and myelofibrosis.

Methods: We studied patients with myelofibrosis secondary to MPS using echocardiogram, right heart catheterization and scintigraphy following intraarterial infusion of Tc99-labeled albumin macroaggregates.

Results: We included 11 patients with MPS and myelofibrosis (7 male, mean age 58 years, 4 with PV, 4 TE and 3 AM) during the period 2009-2014. All had JAK-2 gene mutations, bone marrow fibrosis and visceromegaly. Median NT-proBNP levels were 4597 pg/ml (range 175-5700). Echocardiograms showed pulmonary hypertension in most cases, with a mean pulmonary systolic pressure of 54 ± 17 mmHg (range 35-80). Right heart catheterization disclosed high cardiac output (HCO) in all patients. After ruling out other causes of HCO, a scintigraphy was performed after administrating Tc99-labeled albumin macroaggregates in the descending thoracic aorta. In every case, a percentage (6.1 ± 2.0% of the radioactivity) of the labeled macroaggregates were plugged in the pulmonary capillary bed, which makes the diagnosis of microfistulas in the infradiaphragmatic territory.

Conclusion: Most patients with MPS and myelofibrosis show pulmonary hypertension associated with high cardiac output caused by microfistulas without a significant increase in pulmonary resistance. This finding has important clinical implications because pulmonary vasodilators (once recommended) should be contraindicated since they may worsen the clinical presentation.

Patient

1

2

3

4

5

6

7

8

9

10

11

Pulmonary Artery Pres.

(S/D/Mean)

58/38/ 42

69/41/ 50

48/29/ 40

84/26/ 45

57/17/ 30

22/17

/ 19

51/16

/ 28

22/9

/ 13

25/8

/ 14

71/23

/ 39

30/7

/ 15

Pulmonary wedge pres.

28

37

27

13

9

7

13

7

8

12

6

Cardiac output (l/min)

12.5

8.0

11

7.1

8

7.3

8.7

6.7

7.45

6

7.8

Cardiac index (l/m/m2)

5.5

4.0

6.6

3.6

5

4

4.8

4.0

4.5

3.9

5.1

Pulmonary vascular resistance (Wood U.)

1.2

1.4

1.2

4.5

2.6

3

1.7

0.9

0.8

4.5

1.1

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