Infantile scimitar syndrome with severe pulmonary hypertension: successful treatment with coil embolization of the systemic arterial supply to the sequestered lung

Abstract

Scimitar syndrome is a rare cardiopulmonary malformation. Presentation in infancy is usually associated with pulmonary hypertension and severe symptoms. We discuss treatment of such an infant. Two abnormal systemic vessels supplying the sequestered lower part of the right lung were embolized using catheter-inserted coils. The patent arterial duct was surgically ligated. These procedures resulted in a significant reduction of the shunt and the level of pulmonary hypertension, as well as in an impresssive improvement of symptoms.

The impact of interruption of anomalous systemic arterial supply on scimitar syndrome presenting during infancy

“…There are contradictory reports in the literature about the impact of interruption of ASAS in the early management of symptomatic infants diagnosed with SS [4, 5, 7, 9, 10]. Some studies demonstrated favorable effect to alleviate symptoms [4, 7, 8, 11, 12]. Yet others suggested an insignificant impact because ASA supplied relatively small segments of the lung to influence the shunt and PA pressure [5, 9, 10].…”

The bronchopulmonary foregut malformation complex

“…Congestive heart failure and pulmonary arterial hypertension are particularly common in symptomatic neonates and young infants. [160][161][162]183,[189][190][191][192][193][194][195][196][197][198][199][200][201] The reasons for these features include pulmonary venous obstruction of both right or left-sided pulmonary veins, left-to-right shunting through the anomalous systemic artery or atrial septal defect, or from other coexisting cardiac lesions, or persistent pulmonary arterial hypertension of the newborn. 86,87,[160][161][162]183,[188][189][190][191][192][193][194][195][196][197][198][199][200][201][202] Rarely, the inferior caval vein may be congenitally stenotic, as in the patient reported by Tumbarello et al, 203 and others, 204 this feature also contributing to pulmonary venous obstruction (Fig.…”

“…[183][184][185][186] The observations about the causes for congestive heart failure have been confirmed by clinical and surgical reports emanating from Toronto and elsewhere. 86,87,154,162,[164][165][166]188,189,[191][192][193][194][195][196][197] The so-called scimitar sign on the frontal chest radiograph, while characteristically representing the anomalously draining right pulmonary veins, can be mimicked by other structures, including right pulmonary veins meandering through a tortuous course to the left atrium, a large anomalous systemic artery, or anomalous low insertion of the right superior caval vein. 156,182,205,206 In at least one patient with a falsepositive scimitar sign, azygos continuation of the inferior caval vein was noted, and was likely responsible for the abnormal silhouette.…”

“…228 The medical, surgical, and catheter-based management of any given patient may vary considerably, with important differences in outcomes, both morbidity and mortality. [159][160][161][162][193][194][195][196][197][198][199][200][201]229 Therapy has ranged from no intervention for the older asymptomatic adult, to lobectomy or pneumonectomy, this latter operation as a primary or secondary procedure. 193,200,230,231 The critically ill infant continues to remain a challenge, because there are potentially numerous issues contributing to the congestive heart failure and pulmonary arterial hypertension in this group.…”

“…189,197 Other patients will require more demanding procedures to relieve pulmonary venous obstruction, with re-routing of the anomalously connected right pulmonary veins, as well as attention to coexisting cardiac lesions. [159][160][161][162][193][194][195][196][197][198][199][200][201]229 Any number of procedures has been employed to baffle the right pulmonary veins through the atrial septal defect. In the neonate and infant, all too frequently, the baffle becomes obstructive, resulting in severe right-sided pulmonary venous obstruction, albeit abolishing the left-to-right shunt at atrial level.…”