„ Clinical vignette

Chronic thromboembolic pulmonary hypertension complicated by left main compression syndrome

Sylwia Sławek-Szmyt1Aleksander Araszkiewicz1Marek Grygier1Dariusz Zieliński2Maciej Lesiak1Tatiana Mularek-Kubzdela1
11stDepartment of Cardiology, Poznan University of Medical Sciences, Poznań, Poland
2Department of Cardiac Surgery, Medicover Hospital, Warszawa, Poland

Correspondence to:

Sylwia Sławek-Szmyt, MD, PhD,

1st Department of Cardiology,

Poznan University of Medical Sciences,

Długa 1/2, 61–848 Poznań, Poland,

phone: +48 61 854 91 46,

e-mail: sylwia.slawek@skpp.edu.pl

Copyright by the Author(s), 2022

DOI: 10.33963/KP.a2022.0067

Received: December 5, 2021

Accepted: March 8, 2022

Early publication date: March 8, 2022

A 55-year-old-female was admitted with exertional dyspnea (World Health Organization functional class [WHO-FC] III/IV). Clinical evaluation revealed increased N-terminal-pro--brain-type natriuretic peptide (NT-proBNP, 3994 pg/ml) and reduced the six-minute walk test (6MWT, 330 meters). Echocardiography showed enlargement of the right atrium (area, 26.4 cm2) and right ventricle (RV, 54 mm) with high-velocity tricuspid regurgitation (4.7 m/s), pulmonary artery (PA) dilatation (80 mm), elevated estimated RV systolic pressure (96 mm Hg) and mean pulmonary arterial pressure (mPAP, 56 mm Hg). Electrocardiogram indicated RV hypertrophy and overload (Supplementary material, Figure S1). Magnetic resonance imaging ruled out congenital heart defects. The right heart catheterization (the thermodilution method) indicated: mPAP of 58 mm Hg, PA wedge pressure of 12 mm Hg, pulmonary vascular resistance (PVR) of 10.8 Wood units (Wu), and cardiac index of 2.6 l/min × m2. The patient’s pulmonary angiography (Axiom Artis Zee, Siemens, Germany) and computed tomography scan (LightSpeed VCT 64 scanner GE, Chicago, IL, US) revealed a large main PA aneurysm (PAA, 86 mm), along with right and left PAAs (69 mm), and multiple organized thromboembolic lesions in lobar and segmental arteries, which allowed the diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) (Figure 1AD). Coronary angiography demonstrated severe left main coronary artery (LMCA) stenosis due to compression by PAA, confirmed by intravascular ultrasound (IVUS, Eagle Eye Platinum, Philips, Netherlands) (Figure 1E, Supplementary material, Video S1).

Figure 1. A, B. Angiography of the left (A) and right (B) pulmonary artery (PA) showing dilatation and calcified thromboembolic lesions. C, D. Computed tomography scan (C) with volume rendering reconstruction (D) showing a pulmonary artery aneurysm (PAA) (the arrow). E. Coronary angiography (CA) with an intravascular ultrasound (IVUS) showing critical stenosis of the left main coronary artery (LMCA) attributed to the PAA compression (the arrow). LMCA minimal lumen area (MLA) was 4.9 mm2. F. CA with IVUS after LMCA stenting, with normal LMCA shape and size (the arrow). LMCA MLA after stenting was 15.9 mm2

The case was carefully analyzed by the multidisciplinary CTEPH team, and the patient was not qualified for endarterectomy with PA reconstruction due to high risk. The combined pulmonary vasodilators, along with LMCA percutaneous intervention (PCI), were offered for the patient as bridging therapy before lung transplantation (LTx). PCI was performed and the LMCA ostium was expanded with a 4.0 mm × 28 mm drug-eluting stent (Synergy MegatronTM, Boston Scientific, Marlborough, MA, US) and post-dilated with a 5.0 mm × 15 mm balloon (Pantera Pro, Biotronik, Berlin, Germany). The final angiogram and IVUS showed good stent expansion with relief of LMCA extrinsic compression (Figure 1F, Supplementary material, Video S2). The combined specific treatment was also subsequently introduced. The patient was started on oral riociguat titrated from 3 mg to 7.5 mg daily, along with a continuous subcutaneous infusion of treprostinil titrated to the maximum tolerated dosage (30 ng/kg/min).

A six-month follow-up examination demonstrated a significant reduction of mPAP (47 mm Hg) and PVR (6.25 Wu) without PAA diameter progression. The patient improved to II/III WHO-FC with a 390 m distance at 6MWT and NT-proBNP of 1007 pg/ml.

We describe a rare case of CTEPH-related PAA causing LMCA compression. In CTEPH, PAA seems to be associated with mural thickening, webs, or intramural calcified thrombi [1]. PAA may lead to life-threatening complications, including PAA dissection or acute coronary syndrome [2, 3]. To date, the optimal treatment of PAA has not been established. There are no criteria indicating urgent reconstructive surgery or waiting for LTx, especially in asymptomatic patients [1, 3]. In the case of LMCA, the accumulated experience suggests stenting might be a first-choice strategy [3, 4]. Recently, treprostinil was proven to increase the exercise capacity in CTEPH [5]. In the presented case, LMCA stenting and pulmonary vasodilators led to the improvement of the patient’s hemodynamic status.

Supplementary material

Supplementary material is available at https://journals.viamedica.pl/kardiologia_polska.

Article information

Conflict of interest: None declared.

Open access: This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially. For commercial use, please contact the journal office at kardiologiapolska@ptkardio.pl.


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