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Fontan and Cavopulmonary Connections


Conotruncal Development and Pulmonic Stenosis/Atresia
Surgical Principles of Cavopulmonary Connection
Intra-atrial Conduit with ASD
Rastelli Procedure in DORV with PS
(Figures from Child, J. Echocardiographic evaluation of the adult with postoperative congenital heart disease. In Otto CM, ed. The practice of clinical echocardiography [3. ed]. p. 1097 ©Elsevier, 2007)

Conotruncal Development and Pulmonic Stenosis/Atresia

malalignment
Septation of the conus/truncus into recognizable aorta and pulmonary trunk requires four separate processes: looping of the conus/truncus to the interventricular position; septation of the conus cordis into two roughly equal tubes; rotation of the truncal septal plane 90 degrees from that of the interventricular septal plane; and alignment of the conotruncal septum with the interventricular septum.

Pulmonic stenosis results when conotruncal septation results in a smaller diameter for the future pulmonary trunk. It may also occur at the level of the infundibulum if the conotruncal septum is not aligned with the bulboventricular septum, resulting in a more prominent supraventricular crest or a subpulmonic ridge. A membranous VSD may also occur.

(Figures from Child, J. Echocardiographic evaluation of the adult with postoperative congenital heart disease. In Otto CM, ed. The practice of clinical echocardiography [3. ed]. p. 1097 ©Elsevier, 2007)

Surgical Principles of Cavopulmonary Connection

fontan
Beginning with the Blalock-Taussig shunt in the 1940's, cardiopulmonary or venopulmonary shunts are created using a variety of different approaches, each with its own profile of candidates and benefits. The overall goal is to create a one-way conduit from low-pressure venous to low-pressure pulmonary system. The Fontan procedure is a way of connecting the systemic veins to the pulmonary artery or its branches. Direct connection with the pulmonary trunk is not always feasible due to stenosis or accessibility.


Intra-Atrial Fontan with ASD and Vented Conduit

intrafont
The conduit is anchored at the SVC-RA junction and the IVC-RA junction through an anterolateral atriotomy. The vent is cut to overlap the size of an ASD. (Figures from Child, J. Echocardiographic evaluation of the adult with postoperative congenital heart disease. In Otto CM, ed. The practice of clinical echocardiography [3. ed]. p. 1097 ©Elsevier, 2007)

Rastelli Procedure for DORV with PS

rastcolr
DORV (double outlet right ventricle) is a type of malalignment syndrome in which the truncus fails to seat itself directly in the interventricular sulcus and instead continues anterior and rightward. The two great vessels thus arise side-by-side from the right ventricle. As the left ventricle now has no outflow, a VSD is usually present. The Rastelli procedure patches the VSD so that the aortic valve is included on the left ventricular side; it adds a valved conduit between the RVOT and the right pulmonary artery and closes the (often stenotic) left-sided pulmonary trunk.

(Figures from Child, J. Echocardiographic evaluation of the adult with postoperative congenital heart disease. In Otto CM, ed. The practice of clinical echocardiography [3. ed]. p. 1097 ©Elsevier, 2007)


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All images, text, and captions ©2000 - 2009 Starr Kaplan, except where otherwise noted.