Secundum Atrial Septal Defect with Significant Pulmonary Arterial Hypertension: Treat And Repair Strategy

Author : N.G. Liman, R. Prakoso
Upload Date : 19-04-2018

Background: The therapeutic strategy for patients with atrial septal defect (ASD) and significant pulmonary arterial hypertension (PAH) remains controversial. This case report aimed to assess the effect of PAH-specific medications and subsequent shunt closure (i.e., a treat and repair strategy) in such patients.

Methods and Results: Patient 1 is a 45-year-old female who had prior indexed pulmonary vascular resistance (PVRi) of 10 Wood units (WU).m2 and PVR/SVR 0.28. After ten months of treatment with sildenafil, her PVRi and PVR/SVR decreased to 5.14 WU.m2 and 0.21, respectively, then with oxygen reactivity test they decreased to 2.64 WU.m2 and 0.13, respectively. She underwent successful surgical ASD closure. Patient 2 is a 25-year-old female who had prior six-minute walk test result of 310 m. After six months of therapy with sildenafil, evaluation of PVRi and PVR/SVR resulted in 7.2 WU.m2 and 0.49, respectively, which decreased to 5.5 WU.m2 and 0.36 with oxygen reactivity test. The trans-catheter closure was performed successfully. The left ventricular ejection fraction of patient 1 increased from 48% to 54% acutely after the surgery. The six-minute walk test of patient 2 increased from 310 m to 480 m six months after the ASD closure. Surgical and device secundum ASD closure in adults are similarly effective. However, the complication rate was lower, the length of hospital stay is shorter, the cost is less expensive for device closure than surgical repair.

Conclusion: Treat and repair strategy provided substantial improvement, even in patients with ASD and significant PAH. Long-term hemodynamic follow-up is mandatory to evaluate the ultimate efficacy and safety of this new strategy.

KEYWORDS : atrial septal defect; pulmonary hypertension; treat and repair strategy

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