Treatment of Peripheral Pulmonary Artery Stenosis

Abstract

Peripheral pulmonary artery stenosis (PAS) is an abnormal narrowing of the pulmonary vasculature and can form anywhere within the pulmonary artery tree. PAS is a congenital or an acquired disease, and its severity depends on the etiology, location, and number of stenoses. Most often seen in infants and young children, some symptoms include shortness of breath, fatigue, and tachycardia. Symptoms can progressively worsen over time as right ventricular pressure increases, leading to further complications including pulmonary artery hypertension and systolic and diastolic dysfunctions. The current treatment options for PAS include simple balloon angioplasty, cutting balloon angioplasty, and stent placement. Simple balloon angioplasty is the most basic therapeutic option for proximally located PAS. Cutting balloon angioplasty is utilized for more dilation-resistant PAS vessels and for more distally located PAS. Stent placement is the most effective option seen to treat the majority of PAS; however, it requires multiple re-interventions for serial dilations and is generally reserved for PAS vessels that are resistant to angioplasty.

Ultra-high pressure balloon angioplasty for pulmonary artery stenosis in children with congenital heart defects: Short- to mid-term follow-up results from a retrospective cohort in a single tertiary center

“…Later, the advent of relatively high-pressure balloons ( 36 , 37 , 45 , 46 ) (rated with burst pressures >8 atm but no more than 15–22 atm) further increased the success rate to ~50–70%, making them the preferred choice to date. However, there are still a significant proportion of lesions that do not respond to simple BA ( 2 ).…”

“…The indications for BA were in line with universally accepted criteria (1,3,24,33) with slight modifications. Specifically, patients who met one of the following criteria were enrolled for UHP BA: (1) patients who were symptomatic; (2) patients with the pressure gradient across the stenotic segment ≥20 mm Hg; and (3) patients with either the right ventricular systolic pressure ≥60 mm Hg or the ratio of right to left ventricular systolic pressure ≥0.5. Imbalanced bilateral lung perfusion for assessment of unilateral PAS was not adopted as one of the criteria because radionuclide lung perfusion imaging was not available in our center.…”

“…Traditional surgical correction is more invasive and has a higher complication rate, which has made balloon angioplasty (BA) the cornerstone of current PAS treatment since its introduction in the 1980's (1,3,4). However, there remains a significant proportion of lesions that do not respond to simple BA (2). Cutting BA (5)(6)(7)(8)(9)(10)(11) and endovascular stenting (12)(13)(14)(15)(16)(17)(18) offer promising options for these lesions.…”

“…Most cases of PAS become complicated by congenital heart defects. Patients with pulmonary atresia (PA), ventricular septal defect (VSD), and tetralogy of Fallot (ToF) have been reported to have a 70, 20, and 10% chance, respectively, of developing branch PAS throughout the natural history of the disease ( 1 , 2 ). PAS increases right ventricular pressure, leading to ventricular hypertrophy and restriction of filling, which eventually progresses to heart failure or malignant arrhythmias.…”

Commentary: Surgical correction of peripheral pulmonary artery stenosis appears to be a safe and durable option

“…It is well known that the severity of PPAS mainly depends on etiology, anatomic location, and number of stenotic segments, and that early intervention is important to prevent complications, including pulmonary artery hypertension and systolic and diastolic dysfunction. 3 Yet, many centers debate which options are best suited for the youngest of patients. Often balloon or cutting balloon angioplasty are initial therapies offered, whereas stenting is often reserved for more recalcitrant PPAS.…”