Open Access

CASE REPORT

“Treat-Repair-Treat”: Management of Left Main Coronary Compression by a Pulmonary Artery Aneurysm in a Patient with Atrial Septal Defect and Significant Pulmonary Hypertension

Andrei George Iosifescu^1,2,*, Roxana Enache^1,3, Ioana Marinică⁴, Corina Radu², Vlad Anton Iliescu^1,2

1 Carol Davila University of Medicine and Pharmacy, Bucharest, 020021, Romania
2 Department of Cardiac Surgery, C. C. Iliescu Emergency Institute for Cardiovascular Diseases, Bucharest, 022322, Romania
3 Department of Cardiology, C. C. Iliescu Emergency Institute for Cardiovascular Diseases, Bucharest, 022322, Romania
4 Department of Cardiovascular Anesthesia and Intensive Care, C. C. Iliescu Emergency Institute for Cardiovascular Diseases, Bucharest, 022322, Romania

* Corresponding Author: Andrei George Iosifescu. Email:

(This article belongs to this Special Issue: Nightmare Case Reports in Congenital Heart Disease)

Congenital Heart Disease 2023, 18(1), 67-72. https://doi.org/10.32604/chd.2023.026598

Received 14 September 2022; Accepted 07 November 2022; Issue published 09 January 2023

Abstract

Left main coronary compression syndrome (LMCS) may complicate pulmonary artery aneurysms (PAA), usually developed in the context of pulmonary arterial hypertension (PAH). We report the case of a 51-year-old female patient with an atrial septal defect (unsuitable for device closure) complicated by a PAA generating a 90% left main stenosis. The significant PAH held us back from immediate surgery. After specific dual PAH-targeted therapy (sildenafil and bosentan), the atrial septal defect could be closed with a unidirectional valved patch; the PAA-induced LMCS was treated by reductive arterioplasty. The postoperative course was uneventful. Follow-up showed clinical improvement, but PAH treatment was still needed. After three months, coronary angiography showed only an insignificant residual left main stenosis, proving that reductive pulmonary arterioplasty was effective in treating LMCS. Any PAA requires further evaluation for LMCS, a dangerous but treatable complication. The “treat-repair-treat” strategy and shunt-closure with a unidirectional valved patch can both improve surgical prospects of LMCS with shunt-related PAH.

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Graphical Abstract

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