Progressive cyanosis following Kawashima operation: slow resolution after redirection of hepatic veins

Larsen, Steffen Falgreen; Emmertsen, K; Bjerre, Jesper Vandborg; Hjortdal, Vibeke E.

doi:10.1186/1749-8090-8-67

Cited by 5 publications

(5 citation statements)

References 22 publications

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“…Moreover, Larsen et al described that improvement in SpO 2 may take several months to occur, as they observed arterial oxygen saturations that improved over nine months to 90-93% and then eventually to 95-100% after three years. 3 Despite the late referral of our patient, recovery was noticed after completion of the HV inclusion as demonstrated by the increase in peripheral saturation up to 94% at one-year follow-up, regression of symptoms, and improvement in functional class.…”

Section: Discussionmentioning

confidence: 60%

“…Knowledge on the optimal timing and extent of resolution of PAVFs following hepatic venous redirection is sparse and controversial. 3 It has been reported that patients with the polysplenia form of heterotaxy syndrome (left isomerism) seem to be more prone to develop PAVFs after a Kawashima procedure, in which all systemic venous return except HV and coronary venous effluent is emptying to the pulmonary artery circulation, compared with patients with other forms of heart disease who undergo superior cavopulmonary connections. 1 Given the increasing evidence that the flow of HV blood to the lungs is critical to prevent PAVFs after superior cavopulmonary connection, one of the main priorities is constructing a pathway that minimizes the chances of significant streaming of HV flow toward one lung at the expense of the other.…”

Section: Discussionmentioning

confidence: 99%

“…Knowledge on the optimal timing and extent of resolution of PAVFs following hepatic venous redirection is sparse and controversial. 3…”

Section: Discussionmentioning

confidence: 99%

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Percutaneous Transhepatic Fontan-Kreutzer Completion of Hepatic Vein Inclusion

Juaneda

Peirone

Guadagnoli

et al. 2017

World J Pediatr Congenit Heart Surg

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We report the case of an 11-year-old girl with heterotaxy syndrome, dextrocardia, and azygos continuation of an interrupted inferior vena cava who had developed pulmonary arteriovenous fistulas after a Kawashima procedure consisting of bilateral superior cavopulmonary anastomoses. She presented with profound cyanosis, fatigue, and failure to thrive. An operative procedure to direct hepatic vein effluent to the pulmonary circulation was performed with placement of an extracardiac conduit between the hepatic veins and the left pulmonary artery. Persistence of cyanosis led to investigation, which led to the discovery of an unintentionally excluded right hepatic vein. A percutaneous transhepatic catheter intervention was performed in which a vascular plug was implanted to occlude the "missed" right hepatic vein, redirecting the flow through intrahepatic venovenous channels to the conduit. Clinical condition and arterial oxygen saturation were substantially improved one year after the two-step hepatic vein inclusion procedure.

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Section: Discussionmentioning

confidence: 60%

Section: Discussionmentioning

confidence: 99%

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Percutaneous Transhepatic Fontan-Kreutzer Completion of Hepatic Vein Inclusion

Juaneda

Peirone

Guadagnoli

et al. 2017

World J Pediatr Congenit Heart Surg

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“…All the included studies were either case studies or case reports. [ 7 8 9 11 12 13 14 15 16 ] No manuscripts were excluded from this analysis due to concerns with study quality based on assessment using the National Heart, Lung, and Blood Institute Quality Assessment Tool for Case Series Studies. A total of 41 patients were included in the analysis.…”

Section: Resultsmentioning

confidence: 99%

Arterial desaturation due to pulmonary arteriovenous malformations after the Kawashima Operation

Loomba

2016

Ann Pediatr Card

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Arterial desaturation may occur after the Kawashima procedure and, in the absence of venovenous collaterals is usually due to pulmonary arteriovenous malformations. Stenting of the pulmonary arteries, oxygen therapy, and pulmonary vasodilators such as sildenafil have not been able to resolve the arterial desaturation and the only way to do this has been Fontan completion. The time course of the formation of these malformations after the Kawashima and the progression of cyanosis and its resolution after the Fontan has only been demonstrated in case reports and small case series. We pool the available data to model arterial saturations in patients with pulmonary arteriovenous malformations after the Kawashima procedure.

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“…1 Larsen et al reported an interval of nearly three years following HVI before saturations normalized 21 and also underscored the need for potentially earlier HVI that could accelerate resolution of cyanosis. Earlier HVI has similarly been suggested by Vollebregt et al 3 and others (McElhinney et al, 18 Larsen et al 21 ) to facilitate more expeditious resolution of PAVMs, though the optimal timing of HVI is not yet defined. The degree of hypoxia was only moderate in our cohort, reflecting our philosophy to offer HVI in the presence not only of profound desaturations but with exercise limitation or reduced functional status.…”

Section: Commentmentioning

confidence: 99%

Hepatic Vein Incorporation Into the Azygos System in Heterotaxy and Interrupted Inferior Vena Cava

Montesa

Karamlou

Ratnayaka

et al. 2019

World J Pediatr Congenit Heart Surg

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Background: Patients with heterotaxy, single ventricle and interrupted inferior vena cava are at risk of developing significant pulmonary arteriovenous malformations and cyanosis, and inequitable distribution of hepatic factor has been implicated in their development. We describe our experience with a technique for hepatic vein incorporation that reliably provides resolution of cyanosis and presumably equitable hepatic factor distribution. Methods: A retrospective review of a single-surgeon experience was conducted for patients who underwent this modified Fontan operation utilizing an extracardiac conduit from the hepatic veins to the dominant superior cavopulmonary connection. Preoperative characteristics and imaging, operative details, and postoperative course and imaging were abstracted. Results: Median age at operation was 5 years (2-10 years) and median weight was 19.6 kg (11.8-23 kg). Sixty percent (3/5) of patients had Fontan completion without cardiopulmonary bypass, and follow-up was complete at a median of 14 months (range 1-20 months). Systemic saturations increased significantly from 81% + 1.9% preoperatively to 95% + 3.5% postoperatively, P ¼ .0008. Median length of stay was 10 days (range: 7-14 days). No deaths occurred. One patient required reoperation for bleeding and one was readmitted for pleural effusion. Postoperative imaging suggested distribution of hepatic factor to all lung segments with improved pulmonary arteriovenous malformation burden. Conclusions: Hepatic vein incorporation for patients with heterotaxy and interrupted inferior vena cava should optimally provide equitable pulmonary distribution of hepatic factor with resolution of cyanosis. The described technique is performed through a conventional approach, is facile, and improves cyanosis in these complex patients.

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Progressive cyanosis following Kawashima operation: slow resolution after redirection of hepatic veins

Cited by 5 publications

References 22 publications

Percutaneous Transhepatic Fontan-Kreutzer Completion of Hepatic Vein Inclusion

Percutaneous Transhepatic Fontan-Kreutzer Completion of Hepatic Vein Inclusion

Arterial desaturation due to pulmonary arteriovenous malformations after the Kawashima Operation

Hepatic Vein Incorporation Into the Azygos System in Heterotaxy and Interrupted Inferior Vena Cava

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