Learning objectives
To know
•Various types of single ventricle physiology hearts, their anatomy and imaging findings
•Various palliative surgeries, their timing and pathophysiology
•How CT and MRI are useful for assessment and follow up
Background
•
•Single ventricle(SV) heart is “Defined as entire AV junction is connected to only one chamber in the ventricular mass
•SV heart is a rare condition with estimated prevalence of 1:10000.
Different classifications of SV given in tables 1, 2 & 3
Pathophysiology
•Mixing of pulmonary and systemic circulation
•SV bears the burden of pumping blood to both systemic and pulmonary circulation
•Presence of either systemic or pulmonary outflow tract obstruction
•Atrial septal defect – open or restrictive(Restrictive defect may cause increase in CVP or...
Findings and procedure details
Fontan repair has to be done under three stages
Stage 1- Neonatal palliation
•In immediate postnatal period
•Goal - To maintain unobstructed systemic outflow and pulmonary venous return to the ventricle and create a stable and regulated source of pulmonary blood flow
Principles in neonatal palliation
•Protect the lung: prevent pulmonary HTN with PA banding(fig 4)
•Correct cyanosis: BT shunt(fig 5,fig 6b)/sano shunt
•Atrial septostomy/septectomy- to allow mixing of blood at atrial level
•Relieve systemic outflow tract obstruction – Norwood procedure(fig 6) , Damus-Kaye-Stansel (DKS)...
Conclusion
For a radiologist, knowledge of anatomy and hemodynamics of the single ventricle heart both before and after different palliative surgeries along with different CT and MRI protocols and techniques is essential to correctly report the imaging findings and also to get an optimal answer for the clinical question asked by the clinician.
Personal information and conflict of interest
A. Alex; Trivandrum/IN - nothing to disclose M. R. Bursupalle; Trivandrum, KERALA/IN - nothing to disclose A. Ayyappan; Trivandrum/IN - nothing to disclose V. Jineesh; Trivandrum/IN - nothing to disclose
References
[1]A. M. Gaca, J. J. Jaggers, L. T. Dudley, and G. S. Bisset, “Repair of congenital heart disease: a primer-part 1,” Radiology, vol. 247, no. 3, pp. 617–631, Jun. 2008, doi: 10.1148/radiol.2473061909.
[2]M. Gewillig, “The Fontan circulation,” Heart Br. Card. Soc., vol. 91, no. 6, pp. 839–846, Jun. 2005, doi: 10.1136/hrt.2004.051789.
[3]S. Khambadkone, “The Fontan pathway: What’s down the road?,” Ann. Pediatr. Cardiol., vol. 1, no. 2, pp. 83–92, 2008, doi: 10.4103/0974-2069.43872.
[4]A. A. Ghaferi and G. M. Hutchins, “Progression of liver pathology in patients...