Current outcomes of the Glenn bidirectional cavopulmonary connection for single ventricle palliation

Abstract

Despite established selection criteria and improved surgical technique and medical management, there is a continuous failure and attrition risk following BCPC. Outcomes are influenced by underlying cardiac anomaly; patients with dominant left ventricle (i.e. tricuspid atresia, double inlet left ventricle) having the best survival while those with dominant right ventricle (i.e. hypoplastic left heart syndrome, double outlet right ventricle with heterotaxy) having the worst survival. Increased PVR remains a significant factor affecting mortality.

Single Ventricle Palliation in Low Weight Patients Is Associated With Worse Early And Midterm Outcomes

“…In addition to early death, low weight at time of firststage surgery was suggested to have an adverse effect on late survival [3,11,12]. Gelehrter and colleagues [11] reviewed outcomes of 47 neonates with HLHS patients who were 2.5 kg or less at time of Norwood palliation.…”

“…The increased death hazard in low weight SV patients persists for almost 1 year after initial palliation, suggesting the need for more vigilant monitoring and outpatient care in those high-risk patients. The anatomy of SV anomalies varies and affects the mode of presentation and subsequently the timing and type of initial palliation [1][2][3]. Anatomic factors that influence presentation and subsequent intervention are largely related to the degree of obstruction of the pulmonary venous return to the heart, pulmonary outflow tract, systemic outflow tract, or aortic arch [1][2][3].…”

“…The anatomy of SV anomalies varies and affects the mode of presentation and subsequently the timing and type of initial palliation [1][2][3]. Anatomic factors that influence presentation and subsequent intervention are largely related to the degree of obstruction of the pulmonary venous return to the heart, pulmonary outflow tract, systemic outflow tract, or aortic arch [1][2][3]. Occasionally, SV patients present with a balanced physiology allowing deferment of first palliation surgery for a few months with the Glenn operation, traditionally the second stage surgery, becoming the first surgery performed [3].…”

“…The principles of first-stage palliation are to achieve unobstructed systemic cardiac output, a controlled and reliable source of pulmonary blood flow, and unobstructed pulmonary venous return to the heart. The type of initial palliation is dictated by the SV anatomy and involves placement of a modified Blalock-Taussig shunt (BTS), placement of a pulmonary artery band (PAB) with or without concomitant arch repair, or a Norwood-type operation [1][2][3].…”

Specific Injuries Management in the Postoperative of Congenital Heart Diseases (II): Univentricular Hearts

“…When systemic flow is dependent on the flow through a ventricular septal defect or bulbo-ventricular foramen, sharp decreases in ventricular dimensions may precipitate the onset of subaortic stenosis. The emergence of an ejection murmur in a patient with susceptible anatomy after the completion of a bidirectional Glenn should lead to screening for this injury [10].…”