Increased Arterial Stiffness in Chronic Thromboembolic Pulmonary Hypertension Was Improved with Riociguat and Balloon Pulmonary Angioplasty: A Case Report

Figures & data

Figure 1 Pulmonary angiography. (A) Before BPA, (B) during balloon dilatation, (C) after BPA. Organized thrombi in the segmental and subsegmental pulmonary arteries (arrow).

Abbreviation: BPA, balloon pulmonary angioplasty.

Table 1 Changes in Clinical Parameters from Diagnosis of CTEPH Until 12 Months

	Time (Months)
	Diagnosis of CTEPH (Before Riociguat Administration)	1	2	3	4	5	6	12 (6m After BAP)
Riociguat		3 mg	3 mg	3 mg	3 mg	3 mg	3 mg	3 mg
BPA session			①	②	③	④	⑤
CAVI	10.0	7.3	9.1	7.8	8.2	7.8	7.0	5.8
sBP (mmHg)	124	126	134	128	127	129	121	117
dBP (mmHg)	76	74	79	77	79	87	80	67
mBP (mmHg)	92.0	91.3	97.3	94.0	95.0	101.0	93.7	83.7
HR (beat/min)	72	72	58	63	72	62	70	67
sPAP (mmHg)	86		69	54	45	45	36	32
dPAP (mmHg)	35		13	15	12	10	11	8
mPAP (mmHg)	51		41	30	26	26	21	19
mRAP (mmHg)	5		10	6	6	6	7	5
PCWP (mmHg)	6		15	11	11	13	9	10
CI (L/min/m^2)	1.82		2.62	4.05	2.55	2.78	2.90	2.57
6MWD (m)	322		339	355	390	405	450	510

Abbreviations: CTEPH, chronic thromboembolic pulmonary hypertension; BPA, balloon pulmonary angioplasty; CAVI, cardio-ankle vascular index; sBP, systolic blood pressure; dBP, diastolic blood pressure; mBP, mean blood pressure; HR, heart rate; sPAP, systolic pulmonary artery pressure; dPAP, diastolic pulmonary artery pressure; mPAP, mean pulmonary artery pressure; mRAP, mean right atrium pressure; PCWP, pulmonary capillary wedge pressure; CI, cardiac index; 6MWD, 6-minute walk distance.

Figure 2 Lung perfusion scintigraphy before treatment (A) and after treatment (B).

Figure 3 Clinical course of the patient and changes in various circulation parameters and CAVI.

Abbreviations: BPA, balloon pulmonary angioplasty; WHO-FC, World Health Organization functional class; SVR, systemic vascular resistance; mPAP, mean pulmonary artery pressure; PVR, pulmonary vascular resistance; CAVI, cardio-ankle vascular index; sBP, systolic blood pressure; BNP, brain natriuretic peptide; CO, cardiac output.

Table 2 Changes in Echocardiographic Findings

	Before Riociguat Administration	After Riociguat Administration (Before BPA)	After BPA (6M After BPA)
LVEF (%)	73	79	65
e’ (cm/sec)	2.3	4.2	4.2
E/e’	16.5	14.0	14.0
GLS-LV (%)	−10.3	−17.2	−19.3
LAD (mm)	30	30	30
TRPG (mmHg)	78	53	18
TAPSE (mm)	14.3	14.0	17.0

Abbreviations: BPA, balloon pulmonary angioplasty; LVEF, left ventricular ejection fraction; E, peak early diastolic transmitral flow velocity; e’, peak early diastolic annular velocity; E/e’, ratio of E to e’; GLS, global longitudinal strain; LV, left ventricle; LAD, left atrial diameter; TRPG, tricuspid regurgitation pressure gradient; TAPSE, tricuspid annular plane systolic excursion; 6MWD, 6-minute walk distance.

Figure 4 Changes in echocardiographic findings during treatment. Apical 4-chamber view, short axis view and bull’s-eye showing segments of the left ventricle to determine GLS are shown in order. Before riociguat administration (A–C), after riociguat administration (D–F) and after BPA (G–I).

Abbreviations: BPA, balloon pulmonary angioplasty; GLS, global longitudinal strain.