▪ CTEPH is symptomatic PH with persistent perfusion defects after 3-6 months of adequate anticoagulation

▪ CTEPH is a disease with

▪ a mechanical component judged amenable to surgery or BPA (obstruction of elastic PA)

▪ and variable small vessel disease in non-occluded areas (remodelling in small muscular PA)

Remodelling

Obstruction

Chronic thromboembolic pulmonary hypertension (CTEPH)

Diagnostic algorithm for CTEPH

Brief Medical History

▪ Female born in 9/1947

▪History of hypertension, hyperlipidaemia

▪ Dyspnea NYHA II since 7/2016

▪ 7-15/08/2016: Admission in a Hospital due to syncope and cranial injury.Diagnosis of Pulmonary embolism

▪ No evidence of DVT. TTE: RV with dysfunction, TR 2/4, RVSP 70 mmHg. Treated withheparin and rivaroxaban

▪Persistent symptoms (WHO III). Unchanged findings in cardiac u/s 3 months later

▪Referred for PH evaluation one year later (7/2017)

Diagnostic evaluation

• Echo (TTE): No congenital heart disease, RVEDD:45 mm, RVSP: 88 mmHg, LV: D-shape (systole)

• V/Q scan: Perfusion defects in both, but especially in right lung

• CTPA: Perfusion defects mainly in subsegmental branches in both PAs. (net-like)

Mosaic appearance

V/Q Scan

ANTERIOR POSTERIOR

HRCT

CTPA

Pulmonary Angiography (2/2018)

RHC

10/2017

RA 9

PA 104/35/62

PCW 13

CI 1.85

PVR 14.4

SAO2 90.8

SVO2 61.7

HR 84

• NT-proBNP: 1349 pg/ml

• A referral as a potential candidate for PEA was proposed but she refused any surgical intervention

• Consider BPA

• Treatment with accenocoumarol, no specific PH drug

BPA sessionsDate PA SEGMENTS LESIONS BALLOONS (mm)

1 11/2017 RPA RA7a, RA7b, RA6b,RA6a, RA8, RA9, RA10a, RA10b 8 1.5, 2.0

2 2/2018 LPA LA1b, LA1a, LA10, LA8, LA5a, LA5b, LA8b 7 1.5, 2.0

3 5/2018 RPA RA1, RA3, RA10, RA10a, RA10b, RA10c 6 2.0, 3.0

4 6/2018 RPA RA4, RA2, RA5 3 2.0

5 7/2018 LPA LA5, LA5a, LA6, LA8a, LA8b, LA10, LA10a 7 2.0, 3.5

6 8/2018 LPA+RPA LA3, LA1, LA1a, LA8a, LA4a, LA4b, LA5, LA5a, RA5a, RA5b, RA5c

11 2.0, 3.0

7 9/2018 RPA RA10a, RA10b, RA8, RA8a, RA6, RA6a 6 2.5, 4.0, 5.0

8 1/2019 RPA RA4, RA1a, RA1b, RA3, RA3a, RA3b, RA5, RA10 8 2.0, 2.5, 4.0

9 2/2019 LPA LA10a, LA10b, LA8, LA8a, LA5, LA5a, LA4, LA9, LA9a, LA9b, LA9c, LA6, LA3, LA1, LA1a, LA2

16 3.5, 4.0, 5.0

10 4/2019 RPA RA1, RA3, RA3a, RA3b, RA5, RA8 6 2.0, 3.0, 5.0

TOTAL 40 RPA, 38LPA 78

RA1

RA10

LA9

LA8

Hemodynamics and NT-proBNP before and after BPA

104

86 90

81

94

74 76

5446

40

62

5054

48

57

43 45

31 30 28

14.4 13.7 12.9 11.1 148.2 9.8

5.1 5.5 3.4

0

10

20

30

40

50

60

70

80

90

100

110

RHC 1 RHC 2 RHC 3 RHC 4 RHC5 RHC6 RHC7 RHC8 RHC9 RHC10

PAP m

PVR

1349

1234

924

620

462574

422

119

0

200

400

600

800

1000

1200

1400

1600

Nov-17 Feb-18 May-18 Jun-18 Jul-18 Sep-18 Jan-19 Apr-19

ΝΤ-proΒΝΡ

BPA Results (after 9 sessions)

Baseline Post BPA Change %

sPAP 104 40 -62%

mPAP 62 28 - 55%

PVR 14.4 3.4 - 76%

CI 1.85 2.84 + 35%

RA 9 6 - 33%

HR 84 59 - 30%

NT-proBNP 1349 119 - 91%

Conclusion

▪ Imaging plays a central role in the diagnosis of CTEPH and patient selection for PEA and BPA

▪ Various imaging tools are used in concert, with techniques such as computed tomography (CT) and conventional pulmonary angiography providing detailed structural information, and tests such as ventilation-perfusion (V/Q) scanning and cardiac u/s providing functional data, helping us in diagnosis and also follow-up.

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