Immediate and Short-Term Outcomes of Pulmonary Valvuloplasty in a Fetus with Pulmonary Atresia

Introduction

Pulmonary atresia with intact ventricular septum (PA/IVS) accounts for approximately 1.9% of congenital heart defects. A severe manifestation of this condition is hypoplastic right heart syndrome (HRHS), characterized by underdevelopment of the right ventricle (RV), tricuspid valve, and pulmonary arteries. HRHS, combined with low birth weight, is a major risk factor for fetal mortality. Fetal pulmonary valvuloplasty (FPV) has emerged as a prenatal intervention to relieve right ventricular outflow tract obstruction, promote RV growth, and increase the likelihood of achieving biventricular circulation postnatally. Despite its potential, fewer than 70 cases of HRHS treated with FPV have been reported globally, and postnatal management strategies remain underdeveloped. This report details a successful case of FPV performed at 26 weeks’ gestation in China, followed by postnatal percutaneous interventions, highlighting technical considerations and outcomes.

Case Presentation and Prenatal Intervention

A fetus diagnosed with PA/IVS and HRHS at 26 weeks’ gestation underwent FPV at Qingdao Women and Children’s Hospital. Prenatal echocardiography confirmed pulmonary valve atresia, absence of forward flow across the pulmonary valve, and a hypoplastic RV with myocardial hypertrophy [Figure 1A–C]. The procedure involved ultrasound-guided transuterine access, where a 22-gauge needle was advanced through the maternal abdomen and uterine wall into the fetal chest. The pulmonary valve was perforated at its midpoint, and a 3.5 mm × 12.0 mm coronary balloon catheter (Boston Scientific, USA) was inflated to dilate the valve [Figure 1D–E]. Post-procedural Doppler imaging demonstrated restored pulmonary valve forward flow [Figure 1F], and serial echocardiograms showed qualitative improvement in RV size. Tricuspid regurgitation velocities ranged between 2.8 and 3.3 m/s, indicating persistent but manageable right ventricular hypertension.

The pregnancy progressed without complications, and a female infant weighing 3050 g was delivered via cesarean section at 38 weeks. The newborn’s initial pulse oxygen saturation (SpO2) was 90%, prompting immediate prostaglandin E1 (PGE1) infusion to maintain ductus arteriosus patency. Neonatal echocardiography revealed critical pulmonary stenosis, moderate RV hypoplasia, and a 3 mm ductus arteriosus without evidence of RV-dependent coronary circulation.

Postnatal Management and Interventions

At 24 hours of life, the infant underwent percutaneous balloon pulmonary valvuloplasty (PBPV) under general anesthesia. Gradual dilation of the pulmonary valve was achieved using balloons ranging from 1.5 mm to 4.5 mm in diameter [Supplementary Figure 1A]. Post-procedural SpO2 improved to 95–100% with continued PGE1 infusion. However, at one week post-PBPV, SpO2 declined to 85%, suggesting pulmonary valve restenosis or inadequate RV adaptation. A second PBPV was performed using an 8 mm × 20 mm balloon (Numed, Germany), followed by implantation of a 3.5 mm × 18.0 mm ductus arteriosus stent (Lepu Medical Technology, China) [Supplementary Figure 1B]. This intervention stabilized SpO2 at 95%, and subsequent weaning from PGE1 was achieved.

Follow-Up and Outcomes

Four-month follow-up demonstrated sustained SpO2 above 99%, with echocardiographic evidence of adequate RV growth and function. The RV supported pulmonary circulation independently, confirming successful establishment of biventricular physiology. The absence of reintervention requirements or complications highlighted the efficacy of the staged approach combining FPV, neonatal PBPV, and ductal stenting.

Discussion

Technical Success and Challenges of FPV

FPV success rates approximate 70%, with biventricular repair achieved in 40% of cases. Intraprocedural complications, particularly fetal bradycardia (31% incidence), remain significant risks. This case underscores the importance of meticulous patient selection using validated scoring systems. The Gómez Montes criteria, which integrate echocardiographic morphologic (tricuspid valve size, RV morphology) and functional (tricuspid regurgitation velocity) indices, were applied preoperatively to predict a single-ventricle outcome with 100% sensitivity and 98% specificity. The fetus in this report met criteria favoring postnatal biventricular repair, justifying FPV eligibility.

Postnatal Strategy for RV Hypoplasia

Neonates with HRHS face challenges due to RV noncompliance and fixed stroke volume. Immediate PBPV, while effective in relieving stenosis, may not address ductal dependency. This case illustrates the necessity of combining PBPV with ductus arteriosus stenting to ensure pulmonary blood flow during RV adaptation. The stent’s 90% success rate and <10% mortality compare favorably with surgical systemic-to-pulmonary shunts, offering a less invasive alternative.

Pulmonary Valve Restenosis and Reintervention

Tricuspid regurgitation velocity trends post-FPV serve as early indicators of pulmonary valve restenosis. In this case, velocity increases prompted timely reintervention, emphasizing the role of serial echocardiographic monitoring. The second PBPV utilized a larger balloon to accommodate annular growth, while ductal stenting mitigated hypoxemia during RV remodeling.

Broader Implications

This case represents the youngest gestational age (26 weeks) for FPV in Asia. The outcomes align with international data, demonstrating that prenatal intervention coupled with postnatal catheter-based strategies can circumvent neonatal thoracotomy and promote biventricular circulation. The staged approach reduces cumulative procedural risk, hospital stays, and family burden.

Conclusion

This report highlights the feasibility of FPV in mid-gestation for severe PA/IVS with HRHS, supported by strategic postnatal interventions. Key elements for success include stringent patient selection using predictive scoring, proactive management of restenosis, and integration of ductal stenting to stabilize pulmonary flow. The case contributes to growing evidence that early fetal intervention, combined with minimally invasive neonatal procedures, optimizes long-term outcomes in complex congenital heart disease.

doi.org/10.1097/CM9.0000000000000322

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