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

Ventricular Septal Defect with Developed Pulmonary Arterial Hypertension (PVR ≥5 WU) and Significant Left-to-Right Shunt

This protocol applies to patients with a ventricular septal defect who have developed pulmonary arterial hypertension (PAH) with a pulmonary vascular resistance (PVR) of 5 Wood Units or greater, yet still demonstrate a significant left-to-right shunt (Qp:Qs >1.5). Eisenmenger physiology is absent and there is no desaturation on exercise.

Key finding: Elevated PVR (≥5 WU) alongside a preserved left-to-right shunt — without Eisenmenger physiology or exercise-induced desaturation — defines a specific, high-stakes subgroup in which the question of defect closure requires careful individual assessment at an expert centre.
VSD closure may be considered in this setting — both surgical and transcatheter approaches exist — but the decision requires careful, individualised evaluation in an expert centre.
The complete closure criteria, approach selection, and management algorithm are in the full protocol.

References

In patients who have developed PAH with PVR ≥5 WU, VSD closure may be considered when there is still significant LR shunt (Qp:Qs >1.5), but careful individual decision in expert centres is required.

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.

DOI: 10.1093/eurheartj/ehaa554

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