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Case Reports
. 2022 Mar 5;22(1):89.
doi: 10.1186/s12872-022-02524-w.

Eisenmenger syndrome with left main compression syndrome: a case report

Charlotte Johanna Cool  1 , Fathy Fathini  2 , Ibnu Adams  3 , Aninka Saboe  3 , Nuraini Yasmin Kusumawardhani  4 , Astri Astuti  4 , Achmad Fauzi Yahya  3
Affiliations
Case Reports

Eisenmenger syndrome with left main compression syndrome: a case report

Charlotte Johanna Cool et al. BMC Cardiovasc Disord. .

Abstract

Background: Left main coronary artery disease secondary to pulmonary artery compression related to Eisenmenger syndrome is an under-suspected condition that can cause fatal outcomes if left untreated. It presents with typical angina but is frequently mistaken for pulmonary hypertension (PH) symptoms. It is now recognized as one of the few important causes of angina in PH.

Case presentation: A 37-year-old man with a history of unoperated atrial septal defect and Eisenmenger syndrome came to the outpatient department with a chief complaint of angina on exertion. Electrocardiogram showed regular sinus rhythm with right axis deviation, right ventricular hypertrophy, deep T-wave inversion in inferior and anterior leads suggestive of ischemia or strain, and incomplete right bundle branch block. Cardiac CT showed compression of the left main coronary artery due to a dilated main pulmonary artery. Therefore, this patient was diagnosed with Eisenmenger syndrome with left main compression due to dilated pulmonary artery. He was treated successfully with IVUS-guided stent implantation. The patient experienced marked improvement in regular activities, with no recurrence of angina symptoms. Angiography 3 months after the procedure revealed good patency of the stent, without significant stenosis.

Conclusions: Left main coronary artery compression is a complication that should be suspected in patients with Eisenmenger syndrome presenting with angina symptoms. Non-invasive modalities are recommended for diagnostic evaluation, but the gold-standard technique remains coronary angiography. The best treatment is not well-established, with either myocardial revascularization or PH treatment, but a left main coronary artery stenting procedure is considered an ideal emergent treatment to provide a better quality of life for patients in this condition.

Keywords: Coronary artery compression; Eisenmenger syndrome; Left main coronary artery disease; Pulmonary hypertension.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
ECG showed regular sinus rhythm with RAD, RVH, deep T-wave inversion in inferior and anterior leads suggestive of ischemia or strain, and incomplete RBBB
Fig. 2
Fig. 2
Thorax X-ray showed cardiomegaly with prominent pulmonary conus and increased pulmonary vascularity
Fig. 3
Fig. 3
Transthoracic echocardiography images showed high probability of PH, (upper) with peak TR velocity of 5.1 m/s, (lower left) dilated MPA of 48 mm, (lower right) dilated RV of 51 mm
Fig. 4
Fig. 4
A Angiography showed severe LMCA stenosis in LAO-caudal view; B, C IVUS evaluation showed dynamic compression of LMCA
Fig. 5
Fig. 5
Contrast-enhanced cardiac CT images, (upper) images showed dilated MPA of 56.8 × 51.9 mm, (bottom left) black arrows showed severe compression of LMCA and (bottom right) LM take-off angle of 32°
Fig. 6
Fig. 6
A Successful PCI of the LMCA in LAO-caudal view; B IVUS evaluation post-PCI of LM
See this image and copyright information in PMC

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