Premature closure of the foramen ovale and congenital pulmonary cystic lymphangiectasis in aortic valve atresia or in severe aortic valve stenosis

Abstract

Because hypoplasia of the left heart is considered inoperable, few studies deal with detailed surgical anatomy and the state of the pulmonary vessels. 1–3 Recently, surgical techniques derived from the Fontan operation have been developed that separate the pulmonary and systemic circulations, thus achieving a physiologic “correction.” 4 However, this type of palliative procedure is unlikely to succeed in infants with severe pulmonary venous congestion and secondary congenital pulmonary cystic lymphangiectasis (CPCL), which has been described in some cases of hypoplastic left heart syndrome. 5–15 Alerted by the index case, which showed severe CPCL and a sealed foramen ovale (FO), we focused our attention on the state of the atrial septum and the anatomic and histologic aspects of the lung tissue and pulmonary vasculature, particularly the pulmonary veins and lymphatic system, in 7 other consecutive cases of hypoplastic left heart syndrome. This study reports on the association of premature closure of the FO and CPCL.