Balloon pulmonary valvuloplasty as a palliative strategy for late presenting adolescents and adult patients with cyanotic congenital heart disease.

BACKGROUND: Management of late presenters with cyanotic congenital heart disease (CCHD) and reduced pulmonary blood flow is challenged by multi-system derangements from longstanding hypoxia in addition to anatomic factors. Balloon pulmonary valvuloplasty (BPV) is a potentially viable palliative option for selected patients with severely stenotic pulmonary valve and are unsuitable for definitive surgical repair METHODS: A retrospective chart review was conducted (2002-2024, age >12 years). BPV was considered for those with a significant component of valvular pulmonary stenosis (PS) on echocardiography. A strategy of stepwise dilation using progressively larger balloon sizes, andmonitoring pulmonary artery pressures between each dilation, was adapted.

RESULTS: Thirty patients [TOF (n = 13), DORV/DILV (n = 7/2), single ventricle/Unbalanced AVCD (n = 3), and corrected transposition (n = 5)] with median age: 20.5 years (IQR: 14.0-26.8), weight: 49 kg (IQR: 44.1-57.7) underwent BPV. The fluoroscopy and procedure times were 17.8 min (IQR:12.7-25.7) and 71 ± 26 min (mean ± SD), respectively. The balloon-to-annulus ratio was 1.01 (0.96-1.2). Saturation improved significantly from 76.7 ± 9.6 % to 90.2 ± 4.4 %. Complications included transient arrhythmias in five patients and fatal re-perfusion pulmonary edema during repeat BPV in one. At a median follow-up of 51 months (IQR: 26.75-139.25 months), majority of patients demonstrated sustained symptomatic relief. Seven patients underwent corrective surgery, and four required repeat BPV.

CONCLUSION: In a selected subgroup of anatomically suited patients with CCHD and PS, stepwise dilatation of the pulmonary valve resulted in improved oxygenation and symptomatic relief, which was sustained on follow-up, making it an attractive low-resource option for palliation, particularly in resource constrained LMIC settings.