Pulmonary vein stenosis: Anatomic considerations, surgical management, and outcomes

Partially unroofed coronary sinus in an infant with pulmonary vein stenosis: mixed blessing

“…Primary pulmonary vein stenosis in patients with associated CHD is usually either identified prior to or immediately after the surgical repair and is usually attributed to the technique of repair. 2,3 Furthermore, the presence of an unroofed coronary sinus allowed for safe access to the left atrium without the need to create a new atrial septal defect. To our knowledge, this is the first time such an approach has been described.…”

Management of Pediatric Pulmonary Vein Stenosis

“…13 Further, surgery is preferred for geometrically distorted veins with neointimal proliferation not amenable to balloon dilation only. 12 In patients with pulmonary venous obstruction following anomalous pulmonary venous connection repair, reoperation is performed if the obstruction is localized to the confluence anastomosis or secondary to residual atrial tissue, as seen in the cardiac subtypes (coronary sinus or superior vena cava/right atrium junction). The sutureless technique is the most common technique used for pulmonary venous obstructions, is superior to conventional repairs, and has a lower incidence of restenosis if performed at the time of the neonatal anomalous venous connection repair.…”

“…The sutureless technique is the most common technique used for pulmonary venous obstructions, is superior to conventional repairs, and has a lower incidence of restenosis if performed at the time of the neonatal anomalous venous connection repair. 2,10,13,51,52 Feins et al 12 reported a single-center experience with the "anatomic repair," which extends the left atrium laterally to the pulmonary veins distal to the stenosis, initially with homograft patch plasty but more recently using an autologous atrial flap. The technique demonstrated a survival benefit compared with sutureless techniques but without a decrease in postoperative reinterventions.…”

“…[7][8][9] This fibromyxoid proliferation causing luminal narrowing is commonly seen at the location of vein distortion by the surrounding anatomy, leading some investigators to hypothesize that disturbed flow and elevated wall shear stress (WSS) may play a role in the development of PVS. [10][11][12][13] Although the exact incidence of patients with PVS is unknown, there is a perception of increasing numbers potentially due to an increase in awareness, improved survival of premature infants, and evolving definitive and palliative surgeries for complex congenital heart disease. The incidence of ex-full-term patients with structurally normal hearts presenting in the first few months of life with PVS appears to be stable.…”

“…The distortion progresses secondarily as thoracic conditions worsen: right pulmonary artery dilation in worsening PH, left bronchial dilation in chronic ventilation, and heart rotation stretching left lower pulmonary vein over aorta in chronic dependent atelectasis. 10,12 "Secondary" or "postoperative" PVS is a well-known phenomenon occurring in patients born with anomalous pulmonary venous connections or obstructive atrial tissue (ie, cor triatriatum) requiring surgical repair. 22 Delineating the mechanism of postoperative pulmonary venous obstruction is critical, as the neointimal proliferation pathognomonic for PVS within the individual vessels does not always occur.…”