Standardized Approach to Intervention for Intestinal Malrotation in Single Ventricle Patients with Heterotaxy Syndrome: Impact on Interstage Attrition and Time to Superior Cavopulmonary Connection

The superior cavopulmonary connection (SCPC) or “bidirectional Glenn” is an integral, intermediate stage in palliation of single ventricle patients to the Fontan procedure. The procedure, normally performed at 3 to 6 months of life, increases effective pulmonary blood flow and reduces the ventricular volume load in patients with single ventricle (parallel circulation) physiology. While the SCPC, with or without additional sources of pulmonary blood flow, cannot be considered a long-term palliation strategy, there are a subset of patients who require SCPC palliation for a longer interval than the typical patient. In this article, we will review the physiology of SCPC, the consequences of prolonged SCPC palliation, and modes of failure. We will also discuss strategies to augment pulmonary blood flow in the presence of an SCPC. The anesthetic considerations in SCPC patients will also be discussed, as these patients may present for noncardiac surgery from infancy to adulthood.

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Simultaneous complex single ventricle palliation and tracheoplasty for heterotaxy syndrome

Interactive Cardiovascular and Thoracic Surgery ◽

10.1093/icvts/ivs229 ◽

2012 ◽

Vol 15 (3) ◽

pp. 523-524 ◽

Cited By ~ 3

Author(s):

H. Matsuhisa ◽

Y. Oshima ◽

T. Kadowaki ◽

A. Yokoi

Keyword(s):

Single Ventricle ◽

Heterotaxy Syndrome ◽

Single Ventricle Palliation

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Fontan completion in reverse order out of necessity: secondary Glenn after primary extracardiac inferior cavopulmonary artery connection

Cardiology in the Young ◽

10.1017/s1047951116001839 ◽

2016 ◽

Vol 27 (5) ◽

pp. 925-928 ◽

Cited By ~ 1

Author(s):

Jannika Dodge-Khatami ◽

Avichal Aggarwal ◽

Mary B. Taylor ◽

Douglas Maposa ◽

Jorge D. Salazar ◽

...

Keyword(s):

Single Ventricle ◽

Upper Body ◽

Reverse Order ◽

Venous Anatomy ◽

Caval Vein ◽

Single Ventricle Physiology ◽

Superior Caval Vein ◽

Good Outcome ◽

Cavopulmonary Connection

AbstractThe primary extracardiac inferior cavopulmonary connection is an unusual novel palliation for single-ventricle physiology, which we first performed in the setting of unfavourable upper-body systemic venous anatomy for a standard bi-directional Glenn, and in lieu of leaving our patient with shunt-dependent physiology. After an initial 16-month satisfactory follow-up, increasing cyanosis led to the discovery of a veno-venous collateral that was coiled, but, more importantly, to impressive growth of a previously diminutive superior caval vein, which allowed us to perform completion Fontan with a good outcome. Performing the single-ventricle staging in a reverse manner, first from below with a primary inferior cavopulmonary connection, followed by Fontan completion from above with a standard superior caval vein bi-directional Glenn, is also possible when deemed necessary.

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Total cavopulmonary connection for functionally single ventricle with pulmonary atresia and abnormal arborization of pulmonary arteries - exclusion of overwhelmed area by collateral arteries from Fontan circulation

Interactive Cardiovascular and Thoracic Surgery ◽

10.1510/icvts.2007.174367 ◽

2008 ◽

Vol 7 (6) ◽

pp. 1180-1182 ◽

Cited By ~ 2

Author(s):

M. Nagashima ◽

N. Hibino ◽

E. Yamamoto ◽

T. Higaki

Keyword(s):

Single Ventricle ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Fontan Circulation ◽

Total Cavopulmonary Connection ◽

Collateral Arteries ◽

Cavopulmonary Connection

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Superior Vena Cava Banding to Facilitate Unilateral Bidirectional Glenn Operation in Patients With Single Ventricle Heart Disease and Bilateral Superior Caval Veins

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135116679824 ◽

2016 ◽

Vol 8 (2) ◽

pp. 215-219 ◽

Cited By ~ 1

Author(s):

Matthew C. Schwartz ◽

David Nykanen ◽

William DeCampli ◽

Kamal Pourmoghadam

Keyword(s):

Heart Disease ◽

Congenital Heart ◽

Single Ventricle ◽

Superior Vena ◽

Vena Cava ◽

Novel Technique ◽

Bidirectional Glenn ◽

Increased Risk ◽

Bridging Vein ◽

Cavopulmonary Connection

Staged palliation to achieve a total cavopulmonary connection is a common treatment strategy in patients with single ventricle congenital heart disease. Patients with bilateral superior caval veins (bilateral SVC) often require the creation of bilateral superior cavopulmonary connections as part of the staged palliation, and these patients are at increased risk of morbidity. We describe a novel technique used in two patients with bilateral SVC and very small (1-2 mm) bridging vein that encouraged bridging vein growth and facilitated creation of a unilateral superior cavopulmonary connection.

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