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Successful of High Risk Pulseless-Balloon Aortic Valvuloplasty Procedure in Uncorrected Pulmonary Atresia with Severe Congenital Aortic Stenosis and Low Left Ventricular Function

1Doctoral Study Program of Medical and Health Science, Universitas Diponegoro, Semarang, Indonesia, Indonesia

2Department of Cardiology, Faculty of Medicine, Universitas Diponegoro, Semarang, Indonesia, Indonesia

3Department of Surgery, Faculty of Medicine, Universitas Diponegoro, Semarang, Indonesia, Indonesia

4 Department of Anesthesiology and Intensive Care, Faculty of Medicine, Universitas Diponegoro, Semarang, Indonesia, Indonesia

5 Harapan Kita National Heart Center, Jakarta, Indonesia, Indonesia

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Received: 10 Apr 2025; Revised: 19 May 2025; Accepted: 26 Jun 2025; Available online: 7 Jul 2025.
Open Access Copyright 2021 JAI (Jurnal Anestesiologi Indonesia)
Creative Commons License This work is licensed under a Creative Commons Attribution 4.0 International License.

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Abstract

Background: Pulmonary atresia with ventricular septal defect (PA-VSD) and severe bicuspid aortic stenosis (AS) is an uncommon condition that presents significant surgical challenges. The dual obstruction leads to chronic pressure overload, resulting in ventricular hypertrophy and decreased systolic function. A low left ventricular ejection fraction (LVEF) increases the risk of mortality during surgical interventions. Percutaneous balloon aortic valvuloplasty (BAV) poses additional risks due to the intentional induction of pulselessness during balloon inflation. This case report aims to detail the perioperative management strategies employed during BAV in a patient with PA-VSD, severe AS, and low LVEF.

Case: We present a 19-year-old female patient weighing 45 kg, who presented with shortness of breath and fatigue. Her oxygen saturation was measured at 90% across all extremities. Electrocardiogram findings indicated sinus rhythm with biventricular hypertrophy and incomplete left bundle branch block. Chest X-ray revealed cardiomegaly and pulmonary artery dilation, while echocardiography confirmed PA-VSD, severe AS with a mean pressure gradient (mPG) of 55 mmHg, and an LVEF of 41.3%. A percutaneous pulseless-BAV was performed using a Tyshak balloon via an antegrade transvenous femoral approach under fluoroscopy and transesophageal echocardiography guidance.

Discussion: Anesthesia management focused on gradual medication titration, minimizing patient movement during the procedure, and ensuring comfort due to TEE probe insertion. A temporary pacemaker was placed in the right ventricle apex for pacing at 220 beats per minute until cardiac arrest occurred post-balloon inflation; defibrillation successfully restored spontaneous circulation.

Conclusion: This case illustrates that percutaneous BAV in uncorrected PA-VSD with severe AS and low LVEF is feasible despite its inherent risks when conducted by a skilled team utilizing careful judgment throughout the procedure.

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Keywords: Cardiovascular anesthesia; congenital heart disease; balloon aortic valvuloplasty; pulmonary atresia; aortic stenosis

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