Late catheter interventions in hypoplastic left heart syndrome

Abstract

Interventional cardiology plays a key role in the diagnosis and management of patients with functionally univentricular physiology after the various stages of surgical palliation. The interventions performed are widely variable in type, including angioplasty of stenotic vessels and implantation of stents in stenotic vessels; closure of defects such as collaterals, leaks in baffles, and fenestrations; creation of fenestration; and more. In the setting of venous hypertension associated with stenosis at the Fontan baffle, conduit, or pulmonary arteries, stent implantation is often preferred, as the aim is to eliminate completely the narrowing, given that relatively mild stenosis can have a significant detrimental hemodynamic effect in patients with functionally univentricular circulation. The procedure is highly successful. In patients who fail after Fontan procedure, creation of a fenestration is often performed, with variable technique depending on the underlying anatomic substrate. To increase chances of patency of the fenestration, implantation of a stent is often required, particularly in the setting of an extracardiac conduit. For those patients with cyanosis and favorable Fontan hemodynamics, closure of the fenestration is performed using atrial septal occluder devices with high success rate. Coils compatible with magnetic resonance imaging are used widely to treat collateral vessels, although on occasion other specific embolization tools are required, such as particles or vascular plugs. Postoperative arch obstruction is successfully managed with angioplasty at a younger age, while implantation of a stent in the aorta is reserved for older patients. Specifics of these interventional procedures as applied to the population of patients with functionally univentricular hearts are described in this manuscript.

Extracardiac Fontan fenestration using the SafeSept transseptal guidewire and snare‐controlled diabolo‐shaped covered‐stent placement

“…Late catheter interventions have been described for the failing Fontan . Right to left shunts created by transcatheter fenestration in both lateral tunnel and extra‐cardiac Fontan baffles have been shown to improve cardiac output and pleural effusions, though at the expensive of lowering arterial oxygen saturation .…”

“…Stents have also been shown to provide a reliable communication for decompression in a variety of Fontan conduits . In the extracardiac Fontan conduit, covered stents can provide a tunnel to the atrium, reducing the risk of exsanguination into the pericardial space .…”

Fontan fenestration closure with Amplatzer Duct Occluder II device

“…Device closure of Fontan fenestration is a widely adopted procedure which is safe and associated with acute and persistent improvements in oxygen saturations, somatic growth, exercise capacity, and reduction in anticoagulation requirements . The most commonly used device is the ASO , probably due to long‐standing usage and that the fenestration may be considered as a neo‐atrial septal defect (ASD) (circular defect surrounded by flat, thin walls). However, the Fontan fenestration differs significantly from a secundum ASD for obvious reasons.…”

“…However, in the long‐term, the Fontan fenestration is a source of continuing systemic desaturation and a potential conduit for paradoxical thrombo‐embolization . Device closure of Fontan fenestration is a widely accepted strategy to eliminate these potential complications . The most common device deployed is the Amplatzer Septal Occluder (ASO).…”

Przetoki naczyniowe w praktyce kardiologicznej — wspólny obszar zainteresowania współczesnej kardiologii interwencyjnej i kardiochirurgii

“…3), różnych połączeń żyły nieparzystej czy połączeń między żyłą bezimienną lub żyłą główną górną a żyłami płucnymi [17][18][19]. Chociaż istotne przetoki żylno-żylne są zazwyczaj zamykane przed końcowym etapem leczenia paliatywnego, to po operacji Fontana, w wyniku różnicy ciśnień między żyłami systemowymi i płucnymi, mogą się otwierać nowe połączenia, najczęściej drenujące bezpośrednio do żył płucnych lub przedsionka żył płucnych [19]. Podstawowym badaniem jest doplerowskie badanie USG, w którym potwierdza się obecność przetoki tętniczo-żylnej dzięki ocenie charakteru i prędkości przepływów naczyniowych.…”