Pulsatile Glenn as long-term palliation for single ventricle physiology patients

• Published in: Congenital heart disease Vol. 13; no. 6; p. 927
• Main Authors: Chacon-Portillo, Martin A, Zea-Vera, Rodrigo, Zhu, Huirong, Dickerson, Heather A, Adachi, Iki, Heinle, Jeffrey S, Fraser, Jr, Charles D, Mery, Carlos M
• Format: Journal Article
• Language: English
• Published: United States 01.11.2018
• Subjects: Cardiac Catheterization; Echocardiography; Female; Follow-Up Studies; Fontan Procedure - methods; Heart Defects, Congenital - diagnosis; Heart Defects, Congenital - surgery; Heart Ventricles - abnormalities; Heart Ventricles - diagnostic imaging; Heart Ventricles - surgery; Humans; Incidence; Infant; Male; Palliative Care - methods; Postoperative Complications - epidemiology; Retrospective Studies; Risk Factors; Texas - epidemiology; Treatment Outcome; Texas; cavopulmonary connection; congenital heart disease; single ventricle
• ISSN: 1747-0803
• DOI: 10.1111/chd.12664

There are limited studies analyzing pulsatile Glenn as a long-term palliation strategy for single ventricle patients. This study sought to determine their outcomes at a single institution. A retrospective review was performed. Study performed at a single pediatric hospital. All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included. Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk factors were assessed by Cox multivariable competing risk analyses. Seventy-eight patients underwent pulsatile Glenn at age 9 months (interquartile range, 5-14). In total, 28% had heterotaxy, 18% had a genetic syndrome, and 24% had an abnormal inferior vena cava. There were 3 (4%) perioperative mortalities. Further palliation was performed in 41 (53%) patients with a median time-to-palliation of 4 years (interquartile range, 3-5). Pulsatile Glenn failure occurred in 10 (13%) patients with 8 total mortalities. Five- and 10-year transplant-free survival were 91% and 84%, respectively. At a median follow-up of 6 years (interquartile range, 2-8), 27 patients (35%) remained with PG (age 7 years [interquartile range, 3-11], oxygen saturation 83% ± 4%). Preoperative moderate-severe atrioventricular valve regurgitation (AVVR) (hazard ratio 7.77; 95% confidence interval 1.80-33.43; P =.005) and higher pulmonary vascular resistance (hazard ratio 2.59; 95% confidence interval 1.08-6.15; P =.031) were predictors of pulsatile Glenn failure after adjusting for covariates. Reaching further palliation was less likely in patients with preoperative moderate-severe AVVR (hazard ratio 0.22, 95% confidence interval 0.08-0.59; P =.002). Pulsatile Glenn can be an effective tool to be used in challenging circumstances, these patients can have a favorable long-term prognosis without reducing their suitability for further palliation.