Hypoplastic left heart syndrome: from fetus to fontan

AbstractThe care of children with hypoplastic left heart syndrome is constantly evolving. Prenatal diagnosis of hypoplastic left heart syndrome will aid in counselling of parents, and selected fetuses may be candidates forin uterointervention. Following birth, palliation can be undertaken through staged operations: Norwood (or hybrid) in the 1st week of life, superior cavopulmonary connection at 4–6 months of life, and finally total cavopulmonary connection (Fontan) at 2–4 years of age. Children with hypoplastic left heart syndrome are at risk of circulatory failure their entire life, and selected patients may undergo heart transplantation. In this review article, we summarise recent advances in the critical care management of patients with hypoplastic left heart syndrome as were discussed in a focused session at the 12th International Conference of the Paediatric Cardiac Intensive Care Society held on 9 December, 2016, in Miami Beach, Florida.

Download Full-text

Use of Computational Fluid Dynamics to Estimate Hemodynamic Effects of Respiration on Hypoplastic Left Heart Syndrome Surgery: Total Cavopulmonary Connection Treatments

The Scientific World JOURNAL ◽

10.1155/2013/131597 ◽

2013 ◽

Vol 2013 ◽

pp. 1-12 ◽

Cited By ~ 9

Author(s):

Jinlong Liu ◽

Yi Qian ◽

Qi Sun ◽

Jinfen Liu ◽

Mitsuo Umezu

Keyword(s):

Fluid Dynamics ◽

Computational Fluid Dynamics ◽

Hypoplastic Left Heart Syndrome ◽

Total Cavopulmonary Connection ◽

Left Heart ◽

Hemodynamic Effects ◽

Hypoplastic Left Heart ◽

Left Heart Syndrome ◽

Cavopulmonary Connection

Download Full-text

Impact of age at bidirectional cavopulmonary anastomosis on haemodynamics after Fontan operation

Cardiology in the Young ◽

10.1017/s1047951118001543 ◽

2018 ◽

Vol 28 (12) ◽

pp. 1436-1443 ◽

Cited By ~ 3

Author(s):

Shuichi Shiraishi ◽

Toshihide Nakano ◽

Shinichiro Oda ◽

Hideaki Kado

Keyword(s):

Pulmonary Artery ◽

Hypoplastic Left Heart Syndrome ◽

Chest Tube ◽

Total Cavopulmonary Connection ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Cavopulmonary Anastomosis ◽

Bidirectional Cavopulmonary Anastomosis ◽

Left Heart Syndrome ◽

Cavopulmonary Connection

AbstractBackgroundsThe aim of this study was to assess the impact of age at bidirectional cavopulmonary anastomosis on haemodynamics after total cavopulmonary connection.MethodsWe conducted a retrospective analysis of 100 consecutive patients who underwent total cavopulmonary connection from 2010 to 2014. All patients had previously undergone bidirectional cavopulmonary anastomosis. These patients were classified into two groups according to age at bidirectional cavopulmonary anastomosis: younger group, <6 months (n=33), and older group, >6 months (n=67).ResultsThe proportion of hypoplastic left heart syndrome was higher in the younger group (48 versus 4%). After total cavopulmonary connection, the chest tube period was longer in the younger group (10.1±6.6 versus 6.7±4.5 days; p=0.009). Catheterisation 6 months after total cavopulmonary connection revealed that pulmonary artery pressure was higher (11.5±1.9 versus 10.4±2.1 mmHg; p=0.017) and Nakata index was lower (219±79 versus 256±70 mm2/m2; p=0.024) in the younger group. In patients with a non-hypoplastic left heart syndrome, there was no difference in post-operative haemodynamics between two groups, but the total amount of chest drainage after total cavopulmonary connection was larger in the younger group (109±95 versus 55±40 ml/kg; p=0.044).ConclusionsEarly bidirectional cavopulmonary anastomosis did not affect the outcome of total cavopulmonary connection. Longer chest tube period, smaller pulmonary artery, and higher pulmonary artery pressure after total cavopulmonary connection were recognised in early bidirectional cavopulmonary anastomosis patients, especially in hypoplastic left heart syndrome.

Download Full-text

Outcomes for the superior cavopulmonary connection in children with hypoplastic left heart syndrome: a 30-year experience

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezaa117 ◽

2020 ◽

Vol 58 (4) ◽

pp. 809-816

Author(s):

Mallory L Hunt ◽

Richard F Ittenbach ◽

Michelle Kaplinski ◽

Chitra Ravishankar ◽

Jack Rychik ◽

...

Keyword(s):

Risk Factors ◽

Hospital Mortality ◽

Hypoplastic Left Heart Syndrome ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Logistic Regression Models ◽

Genetic Anomaly ◽

Left Heart Syndrome ◽

Cavopulmonary Connection ◽

Support Time

Abstract OBJECTIVES The objective of this study was to estimate hospital mortality and length of stay (LOS) for children with hypoplastic left heart syndrome undergoing superior cavopulmonary connection (SCPC). METHODS All hypoplastic left heart syndrome interstage survivors who underwent SCPC between 1 January 1988 and 31 December 2017 were included. The study period was divided into 4 eras based on changes in operative or medical management. Mortality rates were estimated using standard binomial proportions. Adjusted and unadjusted logistic regression models were used to identify risk factors for mortality and LOS. RESULTS The most common procedures for the cohort (n = 958) were Hemi-Fontan (57.3%) or Bidrectional Glenn shunt (35.7%). The mortality was 4.1% overall and decreased in all 3 later eras compared to era 1. Factors associated with mortality in a multiple covariate model included longer total support time, earlier gestational age, longer LOS at the Norwood Procedure and need for additional procedures. Overall, the median LOS was 7.0 days with a decrease from eras 1 to 2 and plateaued in eras 3 and 4. Predictors of longer LOS included genetic anomaly, longer Norwood LOS, additional procedures, lower weight at surgery and longer total support time. The type of SCPC was not associated with mortality or LOS. CONCLUSIONS In this large cohort of patients with hypoplastic left heart syndrome undergoing SCPC, hospital mortality has decreased significantly. LOS initially declined but plateaued in recent eras. The risk factors for mortality and longer LOS are related to patient and procedural complexity, especially the need for additional procedures at the time of SCPC.

Download Full-text