Peripheral pulmonary stenosis with Noonan syndrome treated by balloon pulmonary angioplasty

Balloon Pulmonary Angioplasty for Takayasu Arteritis and Peripheral Pulmonary Artery Stenosis Mimicking Chronic Thromboembolic Pulmonary Hypertension

“…In 2020, Kamada et al advocated that performing BPA in a staged manner of balloon angioplasty followed by subsequent stent placement may result in gradually reduced mean PAP in a patient with suspected TA-PH, in order to avoid significant RPE [19]. Ko et al reported that they used scoring balloons in a graded approach instead of stents or cutting balloons in an adult patient with PPS and Noonan syndrome, in order to avoid vessel injuries caused by excessive dilation of EEM, which may further lead to maintained dilatation of the lesion without restenosis at follow-up [20]. In 2021, Kim et al reported in their review paper the treatment for peripheral pulmonary artery stenosis; PAS (PPS) that simple balloon angioplasty is the most basic therapeutic option for proximally located PPS [23].…”

“…Mean PAP decreased from 45 to 22 mmHg and a concomitant increase in overall pulmonary blood flow, evaluated by 4D-flow cardiovascular magnetic resonance [19]. Ko et al reported an adult patient with PPS complicated with Noonan syndrome, who was treated with total of four sessions of BPA [20]. After BPA, the mPAP improved from 27 to 17 mmHg, and the PVR dropped from 183 to 159 dyne s/cm 5 with symptom alleviation [20].…”

“…Ko et al reported an adult patient with PPS complicated with Noonan syndrome, who was treated with total of four sessions of BPA [20]. After BPA, the mPAP improved from 27 to 17 mmHg, and the PVR dropped from 183 to 159 dyne s/cm 5 with symptom alleviation [20]. In 2022, Huang et al reported 32 consecutive patients (28 females, mean age, 42.8 ± 11.9 years) with TA-PH who underwent PTPA (BPA) [21].…”

The treatment dilemma in adult patients with peripheral pulmonary artery stenosis of diverse etiologies

“…A high index of suspicion during the initial evaluation of patients with PH is essential for the precise diagnosis and treatment of PPAS bearing in mind that the treatment for PPAS is BPA, not PH-specific therapies [5,7].…”

“…The most frequent congenital heart diseases with concomitant PPAS are the tetralogy of Fallot, the double-outlet right ventricle (RV), the patent ductus arteriosus, the atrial septal defect, and the pulmonary atresiaventricular septal defect. Most often, they are diagnosed in syndromic cases such as Williams-Beuren syndrome, Alagille syndrome, Noonan syndrome, congenital rubella syndrome, Ehlers-Danlos syndrome, cutis laxa, and Russell-Silver syndrome [5].…”