| Summary: | Compression of left main coronary artery is a severe complication in PAH and the treatment strategy is tricky and should be individualized. We presented a patient with large atrial septal defect (ASD) and non-Eisenmenger pulmonary arterial hypertension (PAH). Right heart catheterization (RHC) showed the defect was non-correctable due to a pulmonary vascular resistance (PVR) of 9.49 Wood Units. EKG suggested myocardial ischemia and coronary angiography and coronary CTA confirmed proximal left main coronary artery was compressed by extremely dilated pulmonary arterial trunk, which is named left main coronary compression syndrome (LMCS). Percutaneous coronary intervention (PCI) using drug-eluting stent was performed and combined PAH-specific medications was prescribed simultaneously. At 6-month following the PCI procedure, PVR had decreased to 3.43 Wood Units, and percutaneous ASD closure procedure was performed. A 6-month follow-up the patient was asymptomatic and had significantly improved pulmonary hemodynamics. LMCS is rare among patients with ASD-PAH. PCI combined with PAH-specific medications allowed the closure of ASD, resulting in clinical improvement.
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