Atrial Septal Defect Closure With Persistently Elevated Pulmonary Vascular Resistance

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Secundum atrial septal defect with pulmonary arterial hypertension (n=1). Intervention: Treat-to-repair strategy (PAH-targeted therapy and fenestrated surgical closure). Primary outcome: Clinical, echocardiographic, biomarker, and hemodynamic recovery.

Q: What does this research mean for the field?

A treat-to-repair strategy combining targeted pulmonary arterial hypertension therapy with fenestrated defect closure achieves marked clinical and hemodynamic recovery in patients with atrial septal defect and persistently elevated pulmonary vascular resistance. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

D Cazeiro; Rui Plácido; Sofia Esteves; Ana Rita Francisco; Manuel Abecasis; Tatiana Guimarães; Ângelo Nobre; Fausto J. Pinto; Ricardo Ferreira

doi:10.1016/j.jaccas.2026.108916

June 1, 2026JACC Case ReportsOpen Access

Atrial Septal Defect Closure With Persistently Elevated Pulmonary Vascular Resistance

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Key result

Treat-to-repair strategy with fenestrated ASD closure achieves marked hemodynamic recovery in severe pulmonary hypertension.

n=1

Figures

Graphical abstract published by JACC Case Reports — Graphical abstract · Published by JACC Case Reports · CC BY-NC-ND

Why the study?

Does a treat-to-repair strategy involving PAH-targeted therapy followed by fenestrated surgical closure improve hemodynamics and clinical status in a patient with a secundum ASD and elevated pulmonary vascular resistance?

Key Points

To evaluate diagnostic, medical, and surgical management strategies for repairing a large secundum atrial septal defect in the presence of severe pulmonary arterial hypertension and persistently elevated pulmonary vascular resistance.
Clinical and hemodynamic evaluation of a 32-year-old man presenting with progressive exertional dyspnea, secundum atrial septal defect, and precapillary pulmonary hypertension.
Work-up included transthoracic and transesophageal echocardiography, right heart catheterization, cardiopulmonary exercise testing, and genetic testing.
Treatment involved upfront dual targeted medical therapy (tadalafil and bosentan) followed by multidisciplinary reassessment and surgical repair using a fenestrated bovine pericardial patch.
Baseline right heart catheterization revealed precapillary pulmonary arterial hypertension with a mean pulmonary arterial pressure of 56 mm Hg, pulmonary vascular resistance of 8.56 Wood units, and pulmonary/systemic flow ratio of 1.8.
Dual targeted therapy improved functional status to WHO FC II, reduced NT-proBNP from 1,500 pg/mL to 898 pg/mL, and decreased pulmonary vascular resistance to 6.39 Wood units.
Following fenestrated surgical closure, the patient achieved marked clinical, echocardiographic, biomarker, and hemodynamic recovery at 6-month follow-up despite transient early postoperative right ventricular dysfunction.

Study Design

Type

Case Report (n=1)

Multicenter

No

Structured PICO

Does a treat-to-repair strategy involving PAH-targeted therapy followed by fenestrated surgical closure improve hemodynamics and clinical status in a patient with a secundum ASD and elevated pulmonary vascular resistance?

P

Population

A 32-year-old man with a secundum atrial septal defect and severe pulmonary arterial hypertension who underwent a treat-to-repair strategy with 6 months of postoperative follow-up.

I

Intervention

Dual pulmonary arterial hypertension (PAH)-targeted therapy (tadalafil and bosentan) for 4 months, followed by fenestrated surgical ASD closure using a bovine valved patch, with resumption of dual PAH therapy postoperatively.

O

Outcome

Clinical (WHO functional class), echocardiographic (RV function, RV-PA coupling), biomarker (NT-proBNP), and hemodynamic (PVR, mPAP, cardiac index) response at 6-month follow-up.

Limitations

Findings are based on a highly selected single patient and do not support liberal closure in ASD-associated PAH.
Supporting evidence for fenestrated/valved closure in this setting remains limited to observational series with small sample sizes and short follow-up.
Single patient case report; findings cannot be generalized to support liberal closure in all ASD-associated PAH.
Current evidence for this approach is limited to small observational series with short follow-up.

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Implication

A physiology-guided treat-to-repair strategy combining targeted medical therapy and fenestrated closure can safely improve clinical and hemodynamic outcomes in highly selected ASD patients with elevated pulmonary vascular resistance.

Abstract

CLINICAL CONDITION: A 32-year-old man presented with a secundum atrial septal defect (ASD) complicated by pulmonary arterial hypertension (PAH) with elevated pulmonary vascular resistance (PVR), creating a major operability dilemma. KEY QUESTIONS: What is the diagnostic work-up in suspected pulmonary hypertension? Should genetic testing be performed in congenital heart disease-associated PAH? How should ASD patients with elevated PVR be managed? Should closure be pursued despite persistent PVR elevation? What is the surgical strategy in this setting? OUTCOME: PAH-targeted therapy improved symptoms and reduced PVR, although values remained elevated. After multidisciplinary reassessment, a "treat-to-repair" strategy with fenestrated surgical closure was pursued. Despite transient postoperative right ventricular dysfunction, the patient achieved marked clinical, echocardiographic, biomarker, and hemodynamic recovery. TAKE-HOME MESSAGES: Operability in ASD-associated pulmonary hypertension requires individualized, multidisciplinary decision-making beyond fixed PVR cutoffs. Fenestrated ASD closure after targeted PAH therapy can balance shunt reduction and right ventricular protection in patients with elevated PVR.

Discussion

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Cite This Study

Cazeiro et al. (2026) conducted a case report in Secundum atrial septal defect with pulmonary arterial hypertension (n=1). Treat-to-repair strategy (PAH-targeted therapy and fenestrated surgical closure) was evaluated on Clinical, echocardiographic, biomarker, and hemodynamic recovery. A treat-to-repair strategy with PAH-targeted therapy and fenestrated closure achieved marked hemodynamic recovery (PVR decreased to 3.5 WU) in a patient with ASD and severe pulmonary hypertension.

synapsesocial.com/papers/6a3721e201fe431c25188925 https://doi.org/10.1016/j.jaccas.2026.108916

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Atrial Septal Defect Closure With Persistently Elevated Pulmonary Vascular Resistance

Figures

Key Points

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

Atrial Septal Defect Closure With Persistently Elevated Pulmonary Vascular Resistance

Figures

Key Points

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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