scholarly journals A shunt decision-making protocol in the surgical palliation of hypoplastic left heart syndrome from 2004 to 2016

2020 ◽  
Vol 58 (1) ◽  
pp. 153-162
Author(s):  
Thomas John Kelly ◽  
Diana Zannino ◽  
Johann Brink ◽  
Igor E Konstantinov ◽  
Michael M Cheung ◽  
...  

Abstract OBJECTIVES The aim of this study was to study the impact of a decision-making protocol for shunt type in the Norwood procedure for hypoplastic left heart syndrome. Our cohort extends from 2004 to 2016. In era 1 (pre-2008), there was no policy for the choice of Norwood shunt. In era 2 (post-2008), a standard protocol was implemented. The right ventricle (RV)-to-pulmonary artery conduit was utilized for low-birth weight patients (<2.5 kg). The right modified Blalock–Taussig Shunt (RBTS) was constructed for normal birth weight patients. METHODS The records of 133 consecutive operative patients with hypoplastic left heart syndrome anatomy between 2004 and 2016 were retrospectively reviewed. Survival risk factors were analysed using the Cox proportional hazards risk model. RESULTS The Norwood procedure was performed at a mean age of 2.9 ± 1.9 days. Bidirectional cavopulmonary shunt was performed at a median age of 99 days (interquartile range 91–107). In era 1, 38.6% (22/57) of patients received the RBTS and 61.4% (35/57) of patients received the RV-to-pulmonary artery conduit. In era 2, 86.8% (66/76) of patients received the RBTS and 13.2% (10/76) of patients received the RV-to-pulmonary artery conduit. The actuarial survival to Fontan was 72.2% (96/133). Era 1 patients were more likely to die within the 1st year (hazard ratio = 2.310, P = 0.025). CONCLUSIONS The shunt protocol may improve outcomes in high-risk patients, and we have demonstrated the reliability of the RBTS in low-risk patients. The short- and mid-term outcomes of our Norwood population justify the continued efforts to improve surgical and perioperative management.

2020 ◽  
pp. 021849232095506
Author(s):  
Ashish Mishra ◽  
Bhavik Champaneri ◽  
Yashpal Rana ◽  
Gaurav Singh ◽  
Senthilraj Thangasami ◽  
...  

Classic hypoplastic left heart syndrome is a rare but fatal congenital heart disease associated with variable underdevelopment of the left side of the heart. Complex forms of hypoplastic left heart syndrome have been reported to coexist with anomalous pulmonary venous drainage, transposition of the great arteries, or pulmonary valve dysplasia. We report a case of hypoplastic left heart syndrome with anomalous origin of the right pulmonary artery from the ascending aorta a rare association not reported in the literature. Preoperative comprehensive echocardiography is essential for diagnosis and accurate recognition of such rare anatomic variations.


Sign in / Sign up

Export Citation Format

Share Document