2022
DOI: 10.4103/apc.apc_34_22
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Abstract: Congenital portosystemic venous connections are rare vascular anomalies that connect the portal and systemic venous circulations. These vascular lesions can lead to complex and varied physiologic manifestations in single-ventricle patients as they progress through the various stages of palliation in the Fontan pathway. Specifically, these connections may be unmasked after a superior cavopulmonary anastomosis operation, but then “re-masked” after Fontan completion. We describe the complex physiology with an ill… Show more

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“…This technique reverses the shunt's hepatofugal flow (ie, away from the liver) thereby making it visible with angiography in the IVC/azygos vein. 5 However, seven months passed with worsening symptoms including difficulty walking 100 yards and baseline saturations of 60% to 65% requiring continuous NCO2, and Hb 21.4 gm/dL. Noninvasive evaluations—including computed tomography angiography (CTA), pulmonary function, and cardiorespiratory exercise testing among others, did not demonstrate another cause for her clinical condition.…”
Section: Patient Casementioning
confidence: 93%
“…This technique reverses the shunt's hepatofugal flow (ie, away from the liver) thereby making it visible with angiography in the IVC/azygos vein. 5 However, seven months passed with worsening symptoms including difficulty walking 100 yards and baseline saturations of 60% to 65% requiring continuous NCO2, and Hb 21.4 gm/dL. Noninvasive evaluations—including computed tomography angiography (CTA), pulmonary function, and cardiorespiratory exercise testing among others, did not demonstrate another cause for her clinical condition.…”
Section: Patient Casementioning
confidence: 93%