Treat-and-repair Strategy for Atrial Septal Defect with Eisenmenger Syndrome due to Pulmonary Arterial Hypertension.
Muneyuki Kadota, Shusuke Yagi, Satoshi Akagi et al.
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Case in brief
A 53-year-old man with Eisenmenger syndrome from a large secundum atrial septal defect had severe pulmonary arterial hypertension. After escalation of PAH-specific therapy, his hemodynamics improved markedly, with substantial reductions in pulmonary pressure and vascular resistance. He then underwent successful surgical ASD closure with a fenestrated patch. The report describes symptomatic and hemodynamic improvement with this treat-and-repair approach in a patient who responded favorably to medical therapy.
What made this case unusual
Despite Eisenmenger syndrome and severe PAH, the patient’s favorable response to intensified medical therapy allowed fenestrated surgical closure of the ASD.
Diagnostic / clinical pearl
In this case, marked hemodynamic improvement on PAH-specific therapy preceded fenestrated ASD closure; the treat-and-repair approach was used in a patient with Eisenmenger syndrome and severe PAH.