Outcomes of Single Ventricle Patients Undergoing the Kawashima Procedure: Can We Do Better?

Objectives: Current technology advances in virtual surgery modeling and computational flow dynamics allow preoperative individualized computer-based design of Fontan operation. To determine potential role of those innovations in patients undergoing hepatic vein incorporation (HVI) following Kawashima operation, we retrospectively examined historic cohort of patients who underwent HVI following Kawashima with focus on regression of pulmonary arteriovenous malformations (PAVMs). Methods: Twenty-two children with single ventricle and interrupted inferior vena cava underwent Kawashima operation (2002-12). Twenty-one (96%) patients had left atrial isomerism and 21 (96%) had undergone prior first-stage palliation. Clinical outcomes were examined. Results: Mean O2 saturation (SaO2) increased from 77% ± 8% to 85% ± 6% ( P = .002) after Kawashima. Fifteen (68%) patients developed PAVMs. Eighteen patients underwent HVI (median age and interval from Kawashima: 4.4 and 3.7 years, respectively). Mean SaO2 prior to HVI was 77% ± 8% and increased to 81% ± 10% at the time of hospital discharge ( P = .250), with five patients requiring home oxygen. On follow-up, mean SaO2 increased to 95% ± 4% ( P < .001). Overall ten-year survival following Kawashima was 94%. Conclusions: A large number of patients develop PAVMs and subsequent cyanosis after Kawashima operation. Early following HVI, SaO2 is commonly low and insignificantly different from that prior to HVI. Although SaO2 will improve on follow-up in most patients, a number of patients continue to have low saturations, indicating incomplete resolution of PAVMs. Given the heterogeneity of those patients and lack of preoperative predictors for complete PAVM regression, our findings suggest a role for virtual surgery to determine optimal individual procedure design that would provide even distribution of hepatic blood flow to both pulmonary arteries.

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Cerebral arterial gas embolism in a patient with hypoplastic left heart syndrome treated with emergent hyperbaric oxygen: case report

Undersea and Hyperbaric Medicine ◽

10.22462/03.20.2020.13 ◽

2020 ◽

pp. 431-434

Author(s):

Mark Binkly ◽

◽

Matthew Kelly ◽

Kevin Hardy ◽

◽

...

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Hyperbaric Oxygen ◽

Single Ventricle ◽

Case Reports ◽

Pulmonary Arteries ◽

Vena Cava ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Small Areas ◽

Left Heart Syndrome

A 30-year-old female with a history of seizure disorder and hypoplastic left heart syndrome treated with a Norwood procedure in 1986 followed by a modified non-fenestrated Fontan (Left SVC to IVC to pulmonary arteries) with a known baffle leak presented to the emergency department. On day of presentation, the patient became unresponsive, with perioral cyanosis, rightward gaze and a left facial droop near the end of a platelet transfusion. An emergent non-contrast head CT revealed intracranial air in the right MCA distribution. She was taken to the hyperbaric chamber and was treated with a U.S. Navy Table 6 in a multiplace chamber with no extensions. Ten minutes into the treatment patient became more alert and spontaneously asked questions. The following day she was treated with a U.S. Navy Table 5. Patient had repeat CT of the head, which showed resolution of intracerebral gas and small areas of ischemia in right frontal lobe and right caudate. On hospital day five neurologic exam was normal, with 5/5 strength and no residual deficits. Treating the patient was a concern because patient has a single ventricle, in which the pulmonary artery is connected directly to the vena cava. There is very little data regarding the effects of hyperbaric oxygen (HBO2) therapy on single-ventricle physiology. Only two case reports of three pediatric patients treated with HBO2 for CAGE in a similar setting are known. In these cases the patients had improvements in their symptoms following HBO2. These cases and ours indicate HBO2 is feasible and indicated for CAGE in patients with cyanotic congenital heart disease.

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Pulmonary arteriovenous malformations in a patient with single ventricle and polysplenia syndrome

BMJ Case Reports ◽

10.1136/bcr-2019-229491 ◽

2019 ◽

Vol 12 (7) ◽

pp. e229491

Author(s):

Shinichiro Sakaki ◽

Taiyu Hayashi ◽

Hiroshi Ono

Keyword(s):

Arteriovenous Malformations ◽

Single Ventricle ◽

Vena Cava ◽

Portosystemic Shunt ◽

Pulmonary Arteriovenous Malformations ◽

Polysplenia Syndrome ◽

Abdominal Angiography ◽

Venous Filling ◽

Congenital Extrahepatic Portosystemic Shunt ◽

Kawashima Operation

A 5-month-old girl with single ventricle, interrupted inferior vena cava and polysplenia syndrome palliated with bilateral Blalock-Taussig shunts developed severe cyanosis despite apparently increased pulmonary blood flow. Angiography revealed diffuse pulmonary arteriolar capillary dilatation and early pulmonary venous filling, suggesting the presence of pulmonary arteriovenous malformations. Abdominal angiography at 6 months demonstrated a large extrahepatic portosystemic shunt, which was percutaneously closed with a vascular plug. Kawashima operation was performed 2 weeks after portosystemic shunt closure. Although cyanosis improved temporarily, the patient suffered from deteriorating cyanosis at 9 months of age and underwent Fontan completion. Thereafter, her oxygen saturation gradually improved to 95% over the course of 3 weeks. Both the congenital extrahepatic portosystemic shunt and Kawashima operation contributed to the development of pulmonary arteriovenous malformations.

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In search of “hepatic factor:” Lack of evidence for ALK1 ligands BMP9 and BMP10

10.1101/2020.07.09.20148320 ◽

2020 ◽

Author(s):

Teresa L. Capasso ◽

Sara M. Trucco ◽

Morgan Hindes ◽

Tristin Schwartze ◽

Jamie L. Bloch ◽

...

Keyword(s):

Single Ventricle ◽

Venous Return ◽

Superior Vena ◽

Plasma Concentrations ◽

Pulmonary Arteries ◽

Vena Cava ◽

Systemic Circulation ◽

Pulmonary Vasculature ◽

Morphogenetic Protein ◽

Bone Morphogenetic Protein 9

AbstractIn children with single ventricle physiology, the Glenn procedure is performed to redirect venous return from the superior vena cava directly to the pulmonary arteries and route venous return from the inferior vena cava exclusively to the systemic circulation. Although this surgery successfully palliates the hemodynamic stress experienced by the single ventricle, patients frequently develop pulmonary arteriovenous malformations (PAVMs). Interestingly, PAVMs may regress upon rerouting of hepatic venous effluent to the pulmonary vasculature, suggesting the presence of a circulating “hepatic factor” that is required to prevent PAVMs. Here, we test the hypothesis that hepatic factor is bone morphogenetic protein 9 (BMP9) and/or BMP10. These circulating ligands are produced by the liver and activate endothelial endoglin (ENG)/ALK1 signaling, and mutations in ENG and ALK1 cause hereditary hemorrhagic telangiectasia, a genetic disease associated with AVM development. However, we found no within-subject variation in BMP9, BMP10, or BMP9/10 plasma concentrations when sampled from five cardiovascular sites, failing to support the idea that the Glenn would limit access of these ligands to the lung vasculature. Unexpectedly, however, we found a significant decrease in all three ligand concentrations in Glenn cases versus controls. Our findings suggest that BMP9/BMP10/ENG/ALK1 signaling may be decreased in the Glenn vasculature but fail to implicate these ligands as hepatic factor.

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

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135119842869 ◽

2019 ◽

Vol 10 (3) ◽

pp. 330-337

Author(s):

Christine Montesa ◽

Tara Karamlou ◽

Kanishka Ratnayaka ◽

Stephen G. Pophal ◽

Justin Ryan ◽

...

Keyword(s):

Inferior Vena Cava ◽

Hepatic Vein ◽

Inferior Vena ◽

Fontan Operation ◽

Vena Cava ◽

Hepatic Veins ◽

Extracardiac Conduit ◽

Complex Patients ◽

Surgeon Experience

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 = .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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A Study of TCPC-Stent Conjunction for Cavopulmonary Assist in Fontan Patients With Right Ventricular Dysfunction

Volume 7: Fluids Engineering ◽

10.1115/imece2016-68760 ◽

2016 ◽

Cited By ~ 2

Author(s):

Jakin Jagani ◽

Alexandrina Untaroiu

Keyword(s):

Energy Loss ◽

Power Loss ◽

Therapeutic Option ◽

Superior Vena ◽

Fontan Operation ◽

Pulmonary Arteries ◽

Vena Cava ◽

Paediatric Population ◽

Circulatory Support ◽

Blood Distribution

Mechanical circulatory support devices have gained significant importance in recent years as a viable therapeutic option to support paediatric population and children with single functional ventricle. The Fontan operation helps to reroute the deoxygenated blood to the lungs by bypassing the dysfunctional right ventricle. Total Cavopulmonary Connection (TCPC) is usually a method opted by the clinicians to connect the superior vena cava (SVC) and inferior vena cava (IVC) to the left and right pulmonary artery (LPA and RPA). However, the non-physiologic flow patterns created by the Fontan procedure leads to an increase in chances of platelet deposition and pressure loss which calls for heart transplantation to prevent early and late stage pathophysiology. This had led to modification of TCPC geometry to reduce the pressure and energy loss and thereby unload the single functional ventricle to ensure longer survival period. A study on mechanical circulatory device in conjunction with the modified TCPC geometry has seen little exposure and has opened new gates to develop a variety of state-of-art cavopulmonary assist devices. This study is focused on the selection of optimal TCPC to reduce energy loss and the effect of stent inside the modified TCPC on hemodynamics and flow structures. Four TCPC connections, developed for a particular age group of children, were studied for the velocity field, overall pressure and energy loss. In addition, the four TCPC connection geometries were also studied for distribution of hepatic blood from the IVC to both pulmonary arteries, and hence the lungs, to prevent development of any arteriovenous malformations. The entire stent assembly mounted inside the two best performing TCPC connections was examined for the hemodynamic effects using a series of 3D-CFD simulations. The curved-type connection for the TCPC proved to provide minimum pressure and energy loss along with reduced traces of vortex and recirculation. However, it was not efficient in terms of hepatic blood distribution. The flared geometry performed second best in terms of both minimum power loss and even hepatic blood distribution. There was a slight difference in power loss between the flared and the curved TCPC configuration with stent but the flared geometry had better hepatic blood distribution. This study demonstrated that a stent in conjunction with a TCPC leads to development of a helical flow pattern which provides better mixing of blood and even distribution to both the pulmonary arteries. The design of a stent with the best performing flared TCPC configuration can be optimized to reduce the amount of power loss and vortex generation and can be used to design similar scaled models for paediatric population of various age groups.

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Individualized computer-based surgical planning to address pulmonary arteriovenous malformations in patients with a single ventricle with an interrupted inferior vena cava and azygous continuation

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/j.jtcvs.2010.11.032 ◽

2011 ◽

Vol 141 (5) ◽

pp. 1170-1177 ◽

Cited By ~ 34

Author(s):

Diane A. de Zélicourt ◽

Christopher M. Haggerty ◽

Kartik S. Sundareswaran ◽

Brian S. Whited ◽

Jarek R. Rossignac ◽

...

Keyword(s):

Inferior Vena Cava ◽

Arteriovenous Malformations ◽

Surgical Planning ◽

Inferior Vena ◽

Single Ventricle ◽

Vena Cava ◽

Pulmonary Arteriovenous Malformations ◽

Computer Based

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Incidence and predictors for the development of significant supradiaphragmatic decompressing venous collateral channels following creation of Fontan physiology

Cardiology in the Young ◽

10.1017/s1047951101000312 ◽

2001 ◽

Vol 11 (3) ◽

pp. 289-294 ◽

Cited By ~ 19

Author(s):

Howard S. Weber

Keyword(s):

Fontan Operation ◽

Cardiac Physiology ◽

Number Of Patients ◽

Coil Occlusion ◽

Angiographic Data ◽

Fontan Physiology ◽

Cavopulmonary Connection ◽

Cavopulmonary Shunt ◽

Bidirectional Cavopulmonary Connection

The occurrence of supradiaphragmatic decompressing venous collateral channels following construction of a bidirectional cavopulmonary connection or completion of the Fontan operation resulting in abnormal systemic hypoxemia has been infrequently described. In addition, the incidence and predictors of these channels have not been well delineated, especially in those patients without formation of such structures preoperatively. I evaluated, retrospectively, 40 patients who had undergone either construction of a bidirectional cavopulmonary shunt or completion of the Fontan operation, and who had complete pre and postoperative hemodynamic and angiographic data. Of the patients, 17 (43%) had developed a total of 21 decompressing venous collateral channels, of which 7 (18%) were considered to be hemodynamically significant requiring transcatheter coil occlusion. Of all variables examined, seven patients with significant decompressing collaterals had a greater transpulmonary gradient at follow-up catheterization (8 + /− 2 vs 5 + /− 2 mmHg, p= .01) and lower systemic saturations at routine clinical follow-up visits (82 + /− 5 vs 89 + /− 5 mmHg, p= .007) in comparison to the 33 others. When not evident preoperatively, decompressing venous collateral channels develop in a significant number of patients following conversion to Fontan physiology. If sufficiently large, they may produce lower than expected systemic saturations for the observed cardiac physiology. The larger decompressing channels are more likely to occur when a greater transpulmonary gradient exists postoperatively, which may require cardiac catheterization and transcatheter coil occlusion.

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Numerical Modeling of Pulsating Inflow to the Pulmonary Arteries in TCPC Morphology Using FSI Approach

Volume 3A: Biomedical and Biotechnology Engineering ◽

10.1115/imece2013-63776 ◽

2013 ◽

Cited By ~ 1

Author(s):

Hamid Reza Rajabzadeh ◽

Bahar Firoozabadi ◽

Mohammad Said Saidi ◽

Salman Sohrabi ◽

Seyyed Mahdi Nemati Mehr

Keyword(s):

Blood Flow ◽

Single Ventricle ◽

Pulmonary Stenosis ◽

Fontan Operation ◽

Pulmonary Arteries ◽

Rigid Wall ◽

New Approach ◽

Pulmonary Flow ◽

Single Ventricle Heart Defect ◽

Fontan Surgery

The Fontan surgery is performed on patients with a single ventricle heart defect to prevent the combination of highly-oxygenated and poorly-oxygenated blood. Blood flow in total cavopulmonary connection (TCPC) which culminates an ordinary Fontan operation is practically steady-state but this flow is not appropriate for respiratory systems. This article investigates an approach in Fontan surgery that has been recently proposed in order to make the pulmonary blood flow pulsating. Moreover, for investigating the compliance of vessels and its effects on blood flow in TCPC, we have used the FSI (Fluid Structure Interaction) method as well as rigid wall assumption for comparison purposes. Our TCPC model structure has obtained from CT Angiography (CTA) scan of a single ventricle patient who has undergone a normal Fontan surgery. In this new procedure, pulmonary stenosis (PS), containing high pressure and pulsating flow, has been added to the original geometry for pulsating the main flow and then its effect on the general flow in left and right pulmonary arteries has been studied by increasing the inlet velocity to PS. In overall, our results show that this new approach increases the pulsations of pulmonary flow.

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