Eisenmenger syndrome with left main compression syndrome: a case report
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)...
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2022-03-01
|
| Series: | BMC Cardiovascular Disorders |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12872-022-02524-w |
| _version_ | 1818919650807775232 |
|---|---|
| author | Charlotte Johanna Cool Fathy Fathini Ibnu Adams Aninka Saboe Nuraini Yasmin Kusumawardhani Astri Astuti Achmad Fauzi Yahya |
| author_facet | Charlotte Johanna Cool Fathy Fathini Ibnu Adams Aninka Saboe Nuraini Yasmin Kusumawardhani Astri Astuti Achmad Fauzi Yahya |
| author_sort | Charlotte Johanna Cool |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-20T01:09:14Z |
| format | Article |
| id | doaj.art-5c68f66be30c4ba9b2ff72a85621ad80 |
| institution | Directory Open Access Journal |
| issn | 1471-2261 |
| language | English |
| last_indexed | 2024-12-20T01:09:14Z |
| publishDate | 2022-03-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Cardiovascular Disorders |
| spelling | doaj.art-5c68f66be30c4ba9b2ff72a85621ad802022-12-21T19:58:43ZengBMCBMC Cardiovascular Disorders1471-22612022-03-0122111110.1186/s12872-022-02524-wEisenmenger syndrome with left main compression syndrome: a case reportCharlotte Johanna Cool0Fathy Fathini1Ibnu Adams2Aninka Saboe3Nuraini Yasmin Kusumawardhani4Astri Astuti5Achmad Fauzi Yahya6Congenital Heart Disease Division, Department of Cardiology and Vascular Medicine, University of Padjadjaran, Hasan Sadikin General HospitalDepartment of Cardiology and Vascular Medicine, University of Padjadjaran, Hasan Sadikin General HospitalCardiology Intervention Division, Department of Cardiology and Vascular Medicine, University of Padjadjaran, Hasan Sadikin General HospitalCardiology Intervention Division, Department of Cardiology and Vascular Medicine, University of Padjadjaran, Hasan Sadikin General HospitalCardiovascular Imaging Division, Department of Cardiology and Vascular Medicine, University of Padjadjaran, Hasan Sadikin General HospitalCardiovascular Imaging Division, Department of Cardiology and Vascular Medicine, University of Padjadjaran, Hasan Sadikin General HospitalCardiology Intervention Division, Department of Cardiology and Vascular Medicine, University of Padjadjaran, Hasan Sadikin General HospitalAbstract 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.https://doi.org/10.1186/s12872-022-02524-wPulmonary hypertensionEisenmenger syndromeLeft main coronary artery diseaseCoronary artery compression |
| spellingShingle | Charlotte Johanna Cool Fathy Fathini Ibnu Adams Aninka Saboe Nuraini Yasmin Kusumawardhani Astri Astuti Achmad Fauzi Yahya Eisenmenger syndrome with left main compression syndrome: a case report BMC Cardiovascular Disorders Pulmonary hypertension Eisenmenger syndrome Left main coronary artery disease Coronary artery compression |
| title | Eisenmenger syndrome with left main compression syndrome: a case report |
| title_full | Eisenmenger syndrome with left main compression syndrome: a case report |
| title_fullStr | Eisenmenger syndrome with left main compression syndrome: a case report |
| title_full_unstemmed | Eisenmenger syndrome with left main compression syndrome: a case report |
| title_short | Eisenmenger syndrome with left main compression syndrome: a case report |
| title_sort | eisenmenger syndrome with left main compression syndrome a case report |
| topic | Pulmonary hypertension Eisenmenger syndrome Left main coronary artery disease Coronary artery compression |
| url | https://doi.org/10.1186/s12872-022-02524-w |
| work_keys_str_mv | AT charlottejohannacool eisenmengersyndromewithleftmaincompressionsyndromeacasereport AT fathyfathini eisenmengersyndromewithleftmaincompressionsyndromeacasereport AT ibnuadams eisenmengersyndromewithleftmaincompressionsyndromeacasereport AT aninkasaboe eisenmengersyndromewithleftmaincompressionsyndromeacasereport AT nurainiyasminkusumawardhani eisenmengersyndromewithleftmaincompressionsyndromeacasereport AT astriastuti eisenmengersyndromewithleftmaincompressionsyndromeacasereport AT achmadfauziyahya eisenmengersyndromewithleftmaincompressionsyndromeacasereport |