Ventricular septal defect
ICD-10 Q21.0 · ICD-11 LA88.4

Treatment of Ventricular Septal Defect with Pulmonary Arterial Hypertension and Significant Left-to-Right Shunt

Clinical Scenario

This protocol targets patients with a ventricular septal defect who have developed pulmonary arterial hypertension (PAH) at a pulmonary vascular resistance (PVR) of 3–5 Wood Units, while a significant left-to-right shunt — defined by a Qp:Qs ratio above 1.5 — remains present.

Comorbidity in Focus: Pulmonary Arterial Hypertension

The presence of PAH at this specific PVR range alongside a haemodynamically relevant shunt defines a narrow, carefully characterised management window. Both the degree of pulmonary vascular resistance and the magnitude of the shunt are central to determining the appropriate intervention.

Approach — partial overview

VSD closure is a consideration in this setting. The complete protocol outlines when closure is indicated and which approach — surgical or transcatheter — applies to which anatomical and procedural circumstances.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1093/eurheartj/ehaa554

In patients who have developed PAH with PVR 3-5 WU, VSD closure should be considered when there is still significant LR shunt (Qp:Qs >1.5).

Surgical closure can be performed with low operative mortality (1-2%) and good long-term results.

Transcatheter closure has become an alternative, particularly in residual VSDs, in VSDs that are poorly accessible for surgical closure, and in muscular VSDs that are located centrally in the interventricular septum.

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