Understanding the Hybrid Stage I Approach for Hypoplastic Left Heart Syndrome

2016 ◽  
Vol 36 (5) ◽  
pp. 48-55
Author(s):  
Sharon L. Cheatham ◽  
Grace M. Deyo
Keyword(s):  
Blood Flow ◽  
Pulmonary Artery ◽  
Hypoplastic Left Heart Syndrome ◽  
Main Pulmonary Artery ◽  
Median Sternotomy ◽  
Stage I ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Cavopulmonary Anastomosis ◽  
Left Heart Syndrome

Hybrid stage I palliation combines cardiothoracic surgery and interventional transcatheter procedures for treatment of hypoplastic left heart syndrome. The approach is an alternative to the Norwood procedure, the traditional first stage of surgical palliation. Hybrid stage I palliation involves placing bilateral branch pulmonary artery bands and a patent ductus arteriosus stent through a median sternotomy, performed without cardiopulmonary bypass. The purpose of the bands is to control blood flow to the lungs and protect the pulmonary bed while the stent sustains systemic cardiac output. A balloon atrial septostomy is performed to create an atrial septal defect for unobstructed blood flow from the left atrium to the right atrium. The second stage of palliative surgery is the comprehensive stage II, which incorporates removal of the stent and pulmonary artery bands, atrial septectomy, anastomosis of the diminutive ascending aorta to the main pulmonary artery, aortic arch augmentation, and bidirectional cavopulmonary anastomosis. The traditional Fontan procedure completes the series of palliation.

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

2018 ◽  
Vol 28 (12) ◽  
pp. 1436-1443 ◽  
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.

Norwood Procedure for Palliation of Hypoplastic Left Heart Syndrome: Right Ventricle to Pulmonary Artery Conduit vs Modified Blalock-Taussig Shunt

2016 ◽  
Vol 36 (6) ◽  
pp. 42-51 ◽  
Author(s):  
Dorothy M. Beke
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricle ◽  
Hypoplastic Left Heart Syndrome ◽  
Stage I ◽  
Left Heart ◽  
Norwood Operation ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome ◽  
Artery Conduit ◽  
Blalock Taussig Shunt

Patients with hypoplastic left heart syndrome undergo a series of operations to separate the pulmonary and systemic circulations. The first of at least 3 operations occurs in the newborn period, with a stage I palliation. The goal of stage I palliation is to provide pulmonary blood flow and create an unobstructed systemic outflow tract. Advances in surgical techniques and intraoperative and postoperative care have helped decrease morbidity and mortality for patients with hypoplastic left heart syndrome who have the stage I Norwood operation, but the patients continue to be at increased risk for hemodynamic collapse and adverse outcomes. This article discusses risk factors, surgical approach, postoperative nursing and medical management strategies, differences between and outcomes for the Norwood operation with the right ventricle to pulmonary artery conduit and the Norwood operation with a modified Blalock-Taussig shunt.

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