Congenital Intervention – Systemic Venous

General description of procedure, equipment, technique

Venovenous collateral occlusion

Venovenous collaterals can occur in patients with elevated systemic venous pressure, such as in the Fontan physiology. These vessels can result in connections from the systemic veins to the pulmonary venous return. Consequently, they can cause systemic hypoxemia and thus contribute to the polycythemia seen in this patient population.

Superior vena cava obstruction

Obstruction of the superior vena cava (SVC) can occur in patients that underwent a Mustard or Senning operation for transposition of the great arteries. This surgical procedure is much less common now that these patients, starting in the mid to late 1980s, have undergone the arterial switch operation.

SVC obstruction can also occur after bicaval heart transplantation, secondary to trauma, due to external compression; after sinus venosus atrial septal defect repair; and even after vascular access of the SVC for surgical cannulation, interventional procedures, pacemaker wires, or indwelling catheters.

Inferior vena cava obstruction

Obstruction of the inferior vena cava (IVC) can occur in patients with history of Mustard or Senning operation for transposition of the great arteries due to trauma, external compression, after inferior sinus venosus atrial septal defect repair, indwelling catheters, and history bicaval heart transplantation. Another unusual cause is a pacemaker wire adhering to the eustachian valve.

Systemic venous baffle leak

Unfortunately, most adults with transposition of the great arteries older than 25 years of age were surgically treated with an atrial rather than arterial switch operation.

These operations are known as a Mustard or Senning procedure and involve baffling the superior and inferior vena cava to the left-sided atrioventricular valve to allow systemic venous return to enter the left-sided pulmonary ventricle circulation. In addition, the pulmonary veins were baffled from their entry into the left atrium to the right-sided atrioventricular valve to allow pulmonary venous return to enter the right-sided systemic ventricle circulation.

Fontan pathway obstruction

Obstruction to the Fontan baffle can occur in patients with a history of surgery for single ventricle physiology. Obstruction can occur due to a suture line stenosis at the inferior vena cava, or pulmonary artery anastomosis, or secondary to external compression due to other comorbidities. Also, a history of indwelling catheters or pacemaker wires can lead to obstruction.

Fontan pathway fenestration closure

Fontan fenestration was reported initially by Dr. Nancy Bridges in Boston. Today, most Fontan operations do not include a fenestration.

High-risk patients often get a fenestration due to concern for poor hemodynamics during the early postoperative period. These fenestrations, long term, can result in systemic hypoxemia below 88% saturation; paradoxical embolic events, such as stroke; and early exercise fatigue.

Patients with persistent fenestration often proceed to the catheter laboratory for closure. This is uncommon in the adult congenital patient population.

Indications and patient selection

Venovenous collateral occlusion

• Collateral diameter greater than 3 mm

• Systemic hypoxemia with saturations less than 88% or clinical symptoms

• Potential risk for paradoxical embolic events

Superior vena cava obstruction

• Superior vena cava syndrome

• Severe headaches or signs of cerebral venous distention

Inferior vena cava obstruction

• Abdominal distention and/or ascites

• Low cardiac output due to diminished venous return

• Significant liver congestion

Systemic venous baffle leak

• Baffle leak diameter greater than 3 mm

• Systemic hypoxemia with saturations less than 88% or clinical symptoms

• Potential risk for paradoxical embolic events, such as history of an event or presence of pacemaker wires in the venous pathway

Fontan pathway obstruction

• Lower extremity edema, ascites, hepatosplenomegaly

• Superior vena cava syndrome

• Severe headaches or signs of systemic venous distention, especially on the chest wall and abdomen.

Fontan pathway fenestration closure

To prevent systemic hypoxemia; paradoxical embolic events, such as stroke; and early exercise fatigue symptoms

Contraindications

Venovenous collateral occlusion

• Hemodynamic insignificant collaterals if deemed to not be contributory to hypoxemia or systemic embolic risk

• Severely elevated systemic venous pressure due to failing Fontan physiology or other forms or right heart failure because the pop-off physiology may often be needed to maintain adequate cardiac output

Superior vena cava obstruction

No significant contraindications other than length of the total occlusion, deemed unable to cross typically once the lesion extends beyond the superior vena cava and into the bilateral innominate veins. The only other contraindication would be patients at higher risk for bleeding complications, such as taking blood thinners or coagulopathy.

Inferior vena cava obstruction

No significant contraindications other than length of the total occlusion, deemed unable to cross usually once the lesion extends beyond the hepatic portion of the IVC. The only other contraindication would be patients at higher risk for bleeding complications, such as taking blood thinners or coagulopathy.

Systemic venous baffle leak

Presence of pacemaker wires should be a contraindication for covered stent angioplasty unless a staged approach is used to place a transfemoral temporary pacing lead, then remove the pacemaker wires, place the covered stent, then replace the SVC pacing leads and remove the transfemoral temporary pacing lead.

Fontan pathway obstruction

No significant contraindications other than length of a total occlusion, deemed unable to cross, which is usually not an issue. The only other contraindication would be patients at higher risk for bleeding complications, such as taking blood thinners or coagulopathy.

Fontan pathway fenestration closure

Abnormal Fontan hemodynamics and/or clinical findings consistent with protein-losing enteropathy or plastic bronchitis.

Details of how the procedure is performed

Venovenous collateral occlusion

Transcatheter closure can be performed using Gianturco coils, Amplatzer vascular plugs, or the Amplatzer PDA device.

Superior vena cava obstruction

Access above and below. Crossing from the heart into the vena cava is preferred due to lower risk of a vascular complication with the pulmonary veins or right pulmonary artery.

Transseptal needle or the stiff end of a wire can be used to perforate the lesion. A 5- to 15-mm Snare catheter can be placed in the superior vena cava location to function as a target using biplane fluoroscopy and angiography.

Inferior vena cava obstruction

Access above and below. Crossing from the IVC into the right atrium is preferred due to a better angle of approach, as well as a lower risk of a vascular complication from puncture of the liver.

Transseptal needle or stiff end of a wire can be used to perforate the lesion. A 5- to 15-mm Snare catheter can be placed from the internal jugular approach to the right atrium along the IVC junction as a target using biplane fluoroscopy and angiography.

Systemic venous baffle leak

The procedure is performed via Gore-Tex covered stent angioplasty.

Fontan pathway obstruction

Access above and/or below depending on the anatomy. Total occlusion is rare. Stenosis can be difficult to appreciate, thus biplane imaging or 3-D imaging is very helpful. Stenosis is usually along the suture anastomosis.

If the inferior vena cava anastomosis is obstructed using an open cell, a stent helps prevent obstruction of the hepatic venous return. Stent angioplasty is almost always needed; take care not to dislodge an endothelial peal of tissue that can become a pulmonary embolic event, a systemic embolic event if there is a fenestration, or can occlude an important fenestration in the presence of abnormal hemodynamics.

If the superior anastomosis is the area of concern, a short and strong stent is helpful as this area often requires a high-pressure angioplasty to be successful.

Fontan pathway fenestration closure

The femoral approach is preferred. Closure can be accomplished with a 4 to 6 mm Amplatzer ASD device, 15-mm Gore Helex Septal Occluder, or in some cases a Gore-Tex covered stent approach is used

Interpretation of results

Venovenous collateral occlusion

Transcatheter closure can be performed using Gianturco coils, Amplatzer vascular plugs, or the Amplatzer PDA device.

Superior vena cava obstruction

• Generally good but stent angioplasty can result in restenosis

• Recommend dilation of venous stents to at least 12 mm and prefer 14 to 18 mm

Inferior vena cava obstruction

Follow-up echocardiogram, CT angiography, or MRA within 6 months to determine if redilation of the stent is required. Monitor for clinical symptoms or findings of abdominal distention or ascites.

Systemic venous baffle leak

Results are determined by angiography and pressure data in the catheterization laboratory. Follow-up results can be assessed by absences of clinical symptoms, 6-minute walk test to assess exercise tolerance and systemic hypoxemia, and imaging if needed by echocardiography, cardiac magnetic resonance imaging (MRI), or cardiac CT.

Fontan pathway obstruction

• Follow-up echocardiogram, CT angiography, or MRA within 6 months to determine if redilation of the stent is required

• Recurrence of symptoms, clinical findings

Fontan pathway fenestration closure

Angiography in the in the inferior vena cava or microcavitation injection in the inferior vena cava along with transesophageal or intracardiac echocardiography to determine if there is any residual shunting to the atrium from the Fontan pathway. In addition, the systemic saturations will increase if the fenestration was a large shunt.

Outcomes (applies only to therapeutic procedures)

Venovenous collateral occlusion

Based on systemic saturations by 6-minute walk test and distance results, comparing preclosure and postclosure results.

Superior vena cava obstruction

Bleeding and cardiac perforation

Inferior vena cava obstruction

Generally outcome is good, but stent angioplasty can result in restenosis.

Systemic venous baffle leak

Systemic baffle leaks can be difficult to close in the catheterization laboratory. Outcomes are determined by elimination of systemic hypoxemia, improvement in exercise tolerance, and prevention of paradoxical embolic events.

Fontan pathway obstruction

• Generally outcome is good, but stent angioplasty can result in restenosis.

• Recommend dilation of venous stents to at least 12 mm and preferably 14 to 18 mm.

Fontan pathway fenestration closure

Generally outcome is good but closure can result in protein-losing enteropathy during long-term follow-up if the Fontan physiology is poor.

Alternative and/or additional procedures to consider

Superior vena cava obstruction

Surgical reconstruction using pericardial tissue or a tube graft.

Inferior vena cava obstruction

Surgical reconstruction using pericardial tissue or a tube graft.

Systemic venous baffle leak

Surgical closure is a reasonable option for patients who are not able to be successfully closed with a device. It also is used when covered stent options are not adequate, such as when the only available stent graft is an inappropriate length that could result in pulmonary artery or systemic venous return obstruction, or if the graft is not the correct diameter for an irregular-shaped baffle.

Fontan pathway obstruction

Surgical revision is a significantly higher risk for morbidity or mortality. This is usually reserved for when the entire baffle is abnormal, such as if there are untreatable atrial arrhythmias in a right atrial baffle anatomy or a conduit that is too small (<18 mm) in an older child or adult.

Fontan pathway fenestration closure

Surgical closure of the Fontan fenestration is nearly never considered due to the safety and efficacy of transcatheter techniques compared to surgical risk.

Complications and their management

Serious adverse events and management

Venovenous collateral occlusion

Embolization of the closure device into the systemic circulation. Snare catheter through a coronary artery catheter to allow manipulation of the catheter tip should be available to retrieve the embolized coil or device.

Superior vena cava obstruction

Bleeding and cardiac perforation

Inferior vena cava obstruction

• Bleeding

• Liver laceration

• Cardiac puncture

Systemic venous baffle leak

The main serious adverse event that can occur during transcatheter repair of a systemic baffle leak is paradoxical embolic events related to thrombus, endothelial tissue dislodgment, or air emboli. This should be avoided in most patents when the interventionalist recognizes the risk.

First, use systemic heparin and monitor the advanced clotting time (ACT) to avoid thrombosis. Avoid leaving catheters across or near the baffle leak for extended periods of time, using transesophageal or intracardiac echocardiography (TEE or ICE) imaging for assessment of the leak and early recognition of a thrombus or a flap of endothelial tissue, and prudent flushing technique of the sheaths, catheters, and IVs to avoid air entry. When the long delivery sheath is across the leak or positioned to place a covered stent, this is a critical time to avoid air entry.

Fontan pathway obstruction

Bleeding and cardiac perforation

Fontan pathway fenestration closure

Although there is a small risk of device dislodgement or entrapment of the atrioventricular valve apparatus during transcatheter Fontan fenestration closure, the main serious adverse events to avoid are paradoxical embolic events related to thrombus, endothelial tissue dislodgement, or air emboli.

This should be avoided in most patents when the interventionalist recognizes the risk. First, use systemic heparin and monitor the ACT to avoid thrombosis; avoid leaving catheters across or near the baffle leak for extended periods of time; use TEE or ICE imaging for assessment of the fenestration and early recognition of a thrombus or a flap of endothelial tissue; and perform prudent flushing technique of the sheaths, catheters, and IVs to avoid air entry. When the long delivery sheath is across the leak or positioned to place a covered stent, this is a critical time to avoid air entry.

What’s the evidence?

These are the key studies regarding transcatheter systemic venous interventions:

Patel, S, Shah, D, Chintala, K, Karpawich, PP. “Atrial baffle problems following the Mustard operation in children and young adults with dextro-transposition of the great arteries: The need for improved clinical detection in the current era”. Congenit Heart Dis. vol. 6. 2011. pp. 466-74. (This is a nice review of problems after the Mustard operation.)

Bentham, J, English, K, Hares, D, Gibbs, J, Thomason, J. “Effect of transcatheter closure of baffle leaks following Senning or Mustard atrial redirection surgery on oxygen saturations and polycythemia”. Am J Cardiol. vol. 110. 2012. pp. 1046-50. (This is a review of the outcome for Mustard baffle leak closure.)

Imielski, BR, Woods, RK, Mussatto, KA. “Fontan fenestration closure and event-free survival”. J Thorac Cardiovasc Surg. vol. 145. 2013. pp. 183-7. (This article evaluates follow-up after Fontan fenestration closure.)

Atz, AM, Travison, TG, McCrindle, BW. “Late status of Fontan patients with persistent surgical fenestration”. J Am Coll Cardiol. vol. 57. 2011. pp. 2437-43.

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