Lutembacher Syndrome Guidelines

Updated: Dec 15, 2020
  • Author: Kamran Riaz, MD; Chief Editor: Yasmine S Ali, MD, MSCI, FACC, FACP  more...
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Guidelines

Guidelines Summary

The European Society of Cardiology (ESC) updated their 2010 guidelines on the management of adult congenital heart disease (ACHD) in 2020. [15, 16]  Class I and III recommendations regarding ACHD and atrial septal defect (ASD) are outlined below.

Refer CHD patients with documented arrhythmias or at high risk for postprocedural arrhythmias (eg, atrial septal defect [ASD] closure at older age) considered for percutaneous or surgical (re)interventions to a center with a multidisciplinary team with expertise in these interventions and in invasive treatment of arrhythmias.

ASD (native and residual)

ASD closure is recommended regardless of symptoms in those with evidence of right ventricular (RV) volume overload without pulmonary artery hypertension (PAH) (no noninvasive signs of pulmonary arterial pressure [PAP] elevation or invasive proof of pulmonary vascular resistance [PVR] < 3 Wood units [WU] in case of such signs) or left ventricular (LV) disease.

Device closure is recommended as the method of choice for secundum ASD closure when technically suitable. In seniors not candidates for device closure, carefully weigh the surgical risk against the potential benefit of ASD closure.

In patients with noninvasive signs of PAP elevation, invasive PVR measurement is required. In patients with LV disease, perform balloon testing, and carefully weigh the benefit of eliminating left-to-right (LR) shunt against the potential negative impact of ASD closure on outcome due to an increase in filling pressure (when considering closure, fenestrated closure, and no closure).

ASD closure is not recommended in those with Eisenmenger physiology, those with PAH and PVR ≥5 WU despite targeted PAH treatment, or exercise desaturation.