Transcatheter occlusion of a post-Fontan residual hepatic vein to pulmonary venous atrium communication using the Amplatzer septal occluder

A residual hepatic vein to left atrial communication may result in progressive cyanosis after the Fontan procedure. This problem has usually been treated surgically by ligation or re-inclusion of the residual hepatic vein in the Fontan circulation. Previous attempts at transcatheter closure of such veins have been unsuccessful. An Amplatzer septal occluder was successfully used for transcatheter closure of a post-Fontan hepatic vein to pulmonary venous atrium fistula in an 8 year old boy.     

Transcatheter closure of large persistent left superior vena cava causing cyanosis in two patients post-Fontan operation utilizing the Gianturco Grifka vascular occlusion device.

We report the successful transcatheter closure of a large persistent left superior vena cava draining into the pulmonary venous circulation causing cyanosis in two patients who had previously undergone the Fontan operation utilizing the Gianturco Grifka vascular occlusion device. Cathet Cardiovasc Intervent 2001;53:398-404.     

Interventional occlusion of interatrial communication after modified Fontan operation

After modified Fontan operations various communications between the systemic and pulmonary venous returns may cause persistent or increasing postoperative cyanosis. Interventional closure of these right-to-left shunts may be necessary to eliminate hypoxemia and to reduce the risk of paradoxical embolic complications. PATIENTS AND METHODS: Eighteen patients with a mean age of 5.6 +/- 4.1 (2.5-17.5) years underwent interventional closure of a right-to-left shunt 17.4 +/- 15.8 (3-60) months after a modified Fontan operation. After test balloon occlusion fenestrations were closed in 13 patients using an Amplatzer Septal occluder (n = 7), a Rashkind PDA occluder (n = 3), a CardioSeal umbrella (n = 1) and detachable coils (n = 2). Residual leaks at the suture lines between the interatrial patch and the right atrial wall were closed using detachable coils and a Rashkind PDA occluder in 2 and 1 patients, respectively. In 3 patients intracardiac venous collateral channels were closed by means of detachable coils. RESULTS: The mean aortic oxygen saturation increased from 85 +/- 4.5 (70-89)% to 91.4 +/- 2.8 (83-95)% (p < 0.001) breathing room air and the mean tunnel pressure rose from 10.7 +/- 1.8 (6-14) mmHg to 12.1 +/- 2.4 (6-16) mmHg (p < 0.001). Calculated Qs decreased from 5.15 +/- 2.1 (2.1-11.3) l/min/m2 to 3.6 +/- 1.0 (1.8-5.6) l/min/m2 (p < 0.001). Mixed venous saturation (66.4 +/- 7.4% vs 65 +/- 7%) and mean systemic arterial pressure (73 +/- 8 mmHg vs 73 +/- 9 mmHg) remained unchanged. In one patient an additional leak of the tunnel could not be closed because of an increase to more than 18 mmHg of the mean pressure in the lateral tunnel during balloon test occlusion. In 2 patients residual leaks after umbrella and coil occlusion of a fenestration and an additional venous collateral channel were closed by means of coils after 16 and 21 months, respectively. At a follow-up of 42 +/- 23 (7-99) months, mean oxygen saturation measured by pulse oxymetry was 93 +/- 2 (90-97)%. In 2 patients color-coded Doppler echocardiography revealed a minimal residual right-to-left shunt. In 2 patients contrast echocardiography demonstrated the additional presence of intrapulmonary fistulas. All patients remained free from device migration, thromboembolic events and hemolysis. CONCLUSION: After modified Fontan operations various right-to-left shunts between the systemic and pulmonary venous returns can be successfully closed using umbrella devices or coils to eliminate cyanosis and to reduce the risk of paradoxical embolism.     

Transcatheter umbrella closure of congenital heart defects

Between October 1984 and September 1986, we attempted transcatheter umbrella closure, using the Rashkind PDA occluder, of 12 congenital or postoperative cardiovascular defects (other than patent ductus arteriosus [PDA]) in 11 patients. In each, we used the umbrella for closure because the defect was too short and/or too large to close with conventional transcatheter methods. The defects included three post-Glenn venous communications (superior vena cava-right atrium, n = 2; azygos vein to inferior vena cava), four congenital "interatrial defects" producing cyanosis ("coronary sinus" septal defect, left superior vena cava to left atrium, patent foramen ovale, left inferior vena cava to left atrium), and five non-PDA systemic-to-pulmonary arterial communications (two congenital and three postoperative). Ten of 12 defects were embolized successfully; nine had complete or subtotal closure, and one was partially closed. The first attempted closure resulted in embolization of a 12 mm device to a lower-lobe pulmonary artery, without clinical sequelae. No other complications occurred. Clinical improvement was most dramatic in those patients whose cyanosis was relieved and less obvious when pulmonary blood flow was reduced. The Rashkind umbrella device, originally designed for closure of PDA, considerably expands the list of congenital or operative defects that can be closed nonsurgically.     

Bilateral superior vena cava with right superior vena cava draining into left atrium

Anomalies of systemic venous return are extremely heterogeneous congenital malformations with variable ranges from completely normal physiology to severe forms of right to left shunting requiring surgical treatment. Anomalous drainage of a right-sided superior vena cava (SVC) to the left atrium (LA) is one of the rarest variants of systemic venous return anomalies, characterized by right-to-left shunt physiology and cyanosis. Here we report a 2 years old girl presented with cyanosis which was observed shortly after birth by her parents but not further investigated. She is otherwise active girl and with normal growth and development. Her clinical examination was unremarkable apart from mild clubbing of the fingers and low oxygen saturation of 88–90% in room air. Her ECG and chest X-ray were unremarkable. Echocardiography showed bilateral SVC connected by a small innominate vein. The right SVC drains directly into the LA while the left SVC drains into the right atrium (RA) via a dilated coronary sinus. There is a small superior sinus venosus type atrial septum defect (ASD) with left to right shunt. Also, there is partial anomalous pulmonary venous return with right upper and right middle pulmonary veins draining directly into the right SVC, which is connected to LA. The right lower pulmonary vein and left pulmonary veins drain directly to LA. The rest of her echocardiography demonstrated normal heart structures and function. This patient was referred for surgical correction, including baffling of the right SVC to the RA and closure of the ASD. We describe this case to highlight the importance of recognizing this rare anomalous systemic venous connection as one of the very rare causes of cyanosis in the pediatric age group as well as at older age.     

Use of Amplatzer occlusion devices to occlude Fontan baffle leaks during fenestration closure procedures

The authors describe the use of Amplatzer occlusion devices to successfully occlude Fontan baffle leaks in three patients. These additional right to left shunts were recognized at the time of elective transcatheter closure of surgically created fenestrations. A common site of baffle leak is described at the base of the right atrial appendage. This suture line serves to exclude the right atrial appendage from Fontan baffle flow and lies within the corrugated surface of the pectinate muscles, making it particularly vunerable to baffle leaks over time. © 2008 Wiley‐Liss, Inc.     

Percutaneous management of cyanosis in Fontan patients using Amplatzer occluders

Objectives : To determine causes of cyanosis and to evaluate percutaneous management of cyanosis in a group of consecutive Fontan patients. Background : A variety of communications allow a right‐to‐left shunt in Fontan circulation causing cyanosis and these communications are amenable to percutaneous closure. Methods : Between November 1997 and November 2007, 45 consecutive patients ranging in age from 2.5 to 26 years (median 8 years) with Fontan circulation and cyanosis underwent cardiac catheterization and percutaneous closure of superfluous communications using different types of Amplatzer occluders. Results : Altogether, 51 communications were detected and 50 of them were closed. In 5 patients (11.1%), multiple communications were present and were closed. Fenestration was detected in 37 of 41 patients (90.2%) after total cavopulmonary connection and all were closed by Amplatzer septal occluders. Five venous collaterals were revealed in 3 of 41 patients (7.3%) with total cavopulmonary anastomosis and were closed by Amplatzer vascular plugs. In 3 patients, lateral tunnel leaks were detected and were closed by Amplatzer PFO occluders. In 4 patients after Kawashima operation, 3 major pulmonary arterio‐venous malformations and single venous collateral contributed to the cyanosis and all were closed using Amplatzer vascular plugs. Conclusions : Different communications cause cyanosis in Fontan patients. Progressive decline of percutaneous oxygen saturation is suggestive of development of venous collaterals or pulmonary arteriovenous malformations. Superfluous communications are amenable to percutaneous closure using various types of Amplatzer occluders. A novel use of an Amplatzer PFO occluder for the percutaneous closure of a lateral tunnel leak is described. © 2008 Wiley‐Liss, Inc.     

Double superior vena cava with drainage of the right superior vena cava into the left auricle. Presentation as a cerebral abscess in an adult

A 44 year old man presented with a cerebral abscess, the location of which suggested a septicaemic origin. Although the patient was not cyanosed, a cardiological work-up was requested to exclude a right-to-left shunt. This showed a double abnormality of the systemic venous drainage: presence of an abnormal left superior vena cava draining into the coronary sinus and of a right superior vena cava draining into the left atrium. These two vena cava intercommunicated by anastomoses. Angiography in the right superior vena cava after occlusion by balloon catheter at its junction with the left atrium showed flow from the right to the left superior vena cava and to the azygos system. Simple ligature of the right superior vena cava was therefore performed to prevent recurrence of cerebral abscess. This case is rare and of interest because of the presence of two superior vena cavae, one on the right draining into the left atrium and the other on the left draining into the coronary sinus, with anastomoses between the two superior vena cavae. This double abnormality of systemic venous drainage explains the absence of cyanosis and therefore the relatively late detection of this malformation.     

Feasibility and clinical impact of transcatheter closure of interatrial communications after a fenestrated Fontan procedure: medium-term outcomes.

OBJECTIVE: This study was to review an institutional experience with transcatheter closure of Fontan fenestrations and its impact on clinical care. BACKGROUND: An interatrial fenestration improves postoperative outcomes in high-risk children undergoing a Fontan repair. While technical feasibility has been well defined, the clinical impact of subsequent closure is not well defined. METHODS: Transcatheter closure of a surgically created or additional interatrial communication was attempted in 152 children at a median interval of 13.8 months after surgery. The clinical records were reviewed for demographic and anatomical characteristics, previous surgeries; catheterization data, and status at latest follow-up. RESULTS: Mean oxygen saturation and right atrial pressure increased acutely from 87% +/- 5% to 96% +/- 3% (P < 0.001) and 12 +/- 2 mm Hg to 13 +/- 3 mm Hg (P < 0.001), respectively. Higher systemic venous atrial pressures after occlusion correlated with higher pulmonary artery pressures (P = 0.05) before the Fontan procedure and with higher right (P < 0.001) and left atrial (P = 0.001) and ventricular end-diastolic pressures (P < 0.001) immediately before occlusion. Complications included device malposition in 2 children, 1 child each had an air embolism and post-procedural bleeding, and each self-limiting and 1 child had acute ST elevation in inferior ECG leads because of occlusion of the acute marginal branch which was treated with angioplasty and placement of a stent. At follow-up (median 4.5 years), the mean oxygen saturation was 95% +/- 3%. Residual interatrial leaks were noted echocardiographically in 9%. Two children developed protein-losing enteropathy after fenestration closure. No deaths or strokes were observed in follow-up. CONCLUSIONS: Transcatheter occlusion of Fontan fenestrations is safe with acute and persistent improvements in oxygen saturations.     

Transcatheter closure of dilated left superior vena cava for resolution of late cyanosis following fontan palliation

We report two adolescents with late cyanosis post-Fontan palliation arising from a persistent and dilated left superior vena cava (LSVC) draining into the pulmonary venous circulation. One has undergone successful transcatheter closure of the left superior vena cava while the other is waiting closure. There is a need for long-term follow-up of Fontan patients including adequate clarification of increasing cyanosis which may be correctable.     

Transcatheter closure of fenestrations and excluded hepatic veins after fontan: versatility of the Amplatzer device

BACKGROUND: The aim of this study was to evaluate the Amplatzer septal occluder (ASO) for transcatheter closure of fenestrations and excluded hepatic veins in patients after modified Fontan operations. Residual right-to-left shunts have improved surgical results of the Fontan operation. Shunt closure may eventually be desirable to eliminate hypoxemia and reduce risk of embolic complications. METHODS AND RESULTS: Ten patients with hypoxemia caused by residual shunts after Fontan procedures were evaluated for closure. After favorable results of test occlusion, all shunts were closed with the use of the ASO. Eight ASOs were used to close fenestrations in 7 patients with 6F transvenous sheaths. Three ASOs were used to close excluded hepatic veins in 3 patients with 6F venous sheaths and transbaffle punctures. Fluoroscopy and transesophageal echocardiography were used to guide device placement. Device placement in all patients was successful. All shunts were closed by angiography after device placement. While breathing room air, systemic oxygen saturation rose from 87.9% +/- 3.0% to 96.3% +/- 0. 9% (P <.001) in the patients. There were no complications of the implant procedures and none noted in outpatient follow-up. CONCLUSIONS: This experience suggests that the ASO is safe and effective for closing surgical shunts after Fontan procedures. The ASO design allows closure of excluded hepatic veins and has advantages over other devices in closure of fenestrations.     

The adult with a Fontan operation

Since 1971, the Fontan operation has been performed for the repair of single-ventricle physiology. This ingenious operation commits a single ventricle to the systemic circulation and takes advantage of cardiovascular and respiratory physiology to propel deoxygenated blood to the lungs, thus minimizing right-to-left shunting and cyanosis. Initially performed as a right atrial to pulmonary artery anastomosis, the Fontan operation has gone through evolutionary steps that have resulted in progressive improvements in mortality, morbidity, and outcomes. Inclusion of the right atrium in the slow-flowing Fontan circuit results in progressive dilation and incessant arrhythmias. This spurred forth efforts to create modifications that partially or completely exclude the atrium from the Fontan circuit. The transcatheter completion of the Fontan operation has been performed in a small number of patients and we expect minimally invasive, transcatheter, and hybrid interventions to play an important role in the future management of these patients.     

Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk

Ventricular dysfunction, elevated pulmonary vascular resistance, and residual distal pulmonary artery distortion contribute to early mortality after a Fontan operation; they may be transient or reversible. A baffle fenestration, allowing right-to-left shunting, maintains cardiac output and limits right atrial pressure. A baffle fenestration was surgically created at the time of a modified Fontan repair in 20 consecutive patients. Risk factors included pulmonary artery pressure of 18 mm Hg or more, end-diastolic pressure of 12 mm Hg or more, valvar regurgitation, pulmonary artery distortion, pulmonary vascular resistance of 2 Woods' units or more, ventricular outflow obstruction, and complex anatomy. Nineteen of 20 patients survived. After the operation, mean arterial oxygen saturation was 86%, mean right atrial pressure was 15 mm Hg, and mean duration of pleural effusions was 6 days. Twelve of 19 survivors tolerated early test occlusion and had permanent transcatheter umbrella closure. Four patients failed early test occlusion, with a significant decrease in venous O2 saturation and a rise in central venous pressure, due to ventricular dysfunction, pulmonary artery distortion, or aortopulmonary collaterals. Three of four had successful late closure of the fenestration after correction of these abnormalities.     

Transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium

Background

A persistent anastomosis between the pulmonary veins that connect with the left atrium and the systemic vein that drains into the right atrium has occasionally been reported. We report characteristics and transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium.

Methods

We retrospectively studied such patients in 5 institutions.

Results

Ten patients (6 girls) presented at a median age of 8 (0.1 to 54) years with 2 anatomic types: 8 vertical vein types with drainage of the left upper lobe to the innominate vein via a large vertical vein (left superior cardinal vein) and to the left atrium via the left upper pulmonary vein; and 2 scimitar vein (SV) types with drainage of the right middle and lower pulmonary veins into the inferior vena cava and to the left atrium via an anomalous connecting vein. Associated malformations were aortic coarctation (n = 2) and secundum atrial septal defects (n = 3). Two patients of the vertical vein type were operated. Transcatheter occlusion of the abnormal pulmonary venous return was performed in 7 cases, associated with occlusion of systemic arterial supply (n = 2), secundum atrial septal closure (n = 2), left upper pulmonary vein stenosis stenting (n = 1), and coarctation stenting (n = 1). Including previously published cases, 18 patients (13 vertical veins and 5 scimitar veins) underwent transcatheter repair. Patients over 40 years of age tend to be symptomatic at presentation (p = 0.056).

Conclusion

In partially abnormal pulmonary venous return with dual drainage, transcatheter therapy can be offered in the majority of patients.     

Transhepatic approach to create stent fenestration in the extracardiac fontan conduit in a child with dextrocardia and interrupted inferior vena cava with azygos continuation

Plastic bronchitis is a rare life‐threatening complication of Fontan operation. When medical treatment is ineffective in the setting of high systemic venous pressures, Fontan fenestration may be considered to decompress venous pressures and improve cardiac output by creation of the right‐to‐left shunting. However, transcatheter approach can be difficult in patients with complex venous anatomy. We report a 4‐year‐old girl born with hypoplastic left ventricle and heterotaxy syndrome, who developed plastic bronchitis following extracardiac Fontan procedure. Her venous anatomy was complex with dextrocardia and interrupted inferior vena cava with azygos continuation. Stent fenestration was successfully performed via transhepatic approach, which was selected based on the anatomical relationship (between extracardiac conduit, left atrium, and hepatic veins) delineated by pre‐catheterization cardiac MRI. Simultaneous transesophageal echocardiography guided the intervention. Her plastic bronchitis improved significantly in 3 months but slowly progressed after the stent fenestration. At her 8‐month follow‐up, stent fenestration remains open and she is currently under heart transplantation evaluation due to persistent plastic bronchitis. Treatment of plastic bronchitis can be undertaken with Fontan fenestration, with pre‐procedural MRI playing an essential role in patients with complex venous anatomy. © 2012 Wiley Periodicals, Inc.     

Isolated atrial inversion Visceral situs solitus, visceroatrial discordance, discordant ventricular d loop without transposition, dextrocardia: Diagnosis and surgical correction

After cardiac catheterization and angiocardiographic studies an infant with cyanosis from birth was found to have visceral situs solitus, visceroatrial discordance without venoatrial discordance, a discordant ventricular d loop without transposition, and dextrocardia. The circulatory pathway was from the venae cavae to right atrium to left ventricle to aorta, and from the pulmonary veins to left atrium to right ventricle to pulmonary artery. An atrial defect was present allowing some mixing between the pulmonary and systemic circulations, but this appeared inadequate and the defect was enlarged surgically. Subsequently, a corrective operation using an atrial baffle to direct the pulmonary venous return to the left ventricle and the systemic venous return to the right ventricle was successfully undertaken.     

Left‐sided Scimitar Vein Causing Cyanosis after Fontan Operation: Successful Transcatheter Device Occlusion Using Magnetic Resonance Imaging X‐ray Fusion

We present the case of a 3‐year‐old boy with asplenia‐type heterotaxy syndrome and functionally single ventricle congenital heart disease who developed cyanosis early after the Fontan operation. Combined cardiac magnetic resonance imaging (MRI) and catheterization identified a large hepatic vein to pulmonary vein connection as the source of right to left shunt. The anatomy was quite unusual, suggesting an underlying diagnosis of mixed total anomalous pulmonary venous connection with left‐sided scimitar vein. This pattern of pulmonary venous return has not been previously reported in a patient with asplenia. MRI x‐ray fusion was used to guide transcatheter device occlusion of the scimitar vein, resulting in marked clinical improvement.     

Alternate pathways to pulmonary venous flow in left-sided obstructive anomalies.

In cardiac anomalies causing severe obstruction in the left side of the heart, such as aortic atresia, mitral atresia, or occasionally severe aortic stenosis, maintenance of circulation depends upon shunting of pulmonary venous blood into the right atrium. The usual pathway by which the shunt is achieved is across the atrial septum through the foramen ovale. When this route is closed or severely narrowed, alternate but less common pathways may exist. These involve either anomalous connections of pulmonary veins to systemic veins or communications with the coronary venous system. In the latter, as commonly occurs in aortic atresia, left ventricular myocardial sinusoids carry pulmonary venous blood from the left ventricular cavity and into the cardiac veins. In other instances of severe left-sided obstruction, a direct communication may exist between the left atrium and the coronary sinus.