Clinical Outcome of Fenestrated Fontan Patients After Closure

Abstract

Background —The late clinical status of Fontan patients after fenestration closure is unknown. Data are now available on all patients who underwent closure from 1989 to 1999. Methods and Results —All patients who underwent catheter closure of a Fontan fenestration were enrolled in either the Clamshell (1989 to 1994) or CardioSEAL (1996 to 1999) regulatory trials. Physiological values obtained at catheterization helped assess the hemodynamic effects of fenestration occlusion. In addition to survival, outcomes assessed included O 2 saturations, medication use, significant clinical findings (eg, heart failure, protein-losing enteropathy, or new arrhythmias), and somatic growth. Of 181 patients who underwent closure, 27 had additional significant leaks. The remaining 154 patients constituted the study group. Median time from closure to latest follow-up was 3.4 years (range 0.4 to 10.3 years). Fenestration closure increased O 2 saturation 9.4% on average ( P <0.001). The numbers of patients receiving digoxin or diuretics decreased at the most recent follow-up compared with baseline ( P <0.001), but use of antiarrhythmic agents increased marginally ( P =0.05). Height and weight percentiles rose (medians of 2 and 4, respectively; P <0.001). Clinical decompensation during follow-up of 154 patients was rare (4.5%), with 2 deaths, 3 Fontan revisions, and 1 patient each with protein-losing enteropathy and ascites. No other patient developed chronic congestive symptoms; 21 patients developed new arrhythmias, and 2 had a stroke or transient ischemic attack. Conclusions —Fenestration closure in Fontan patients was followed by improved oxygenation, reduced need for anticongestive medication, and improved somatic growth at latest follow-up. Death (1.3%) or chronic decompensation (3.2%) was rare.

Percutaneous management of a Fontan fenestration: In search for the ideal restriction—occlusion device

“…Location, size, and type of fenestrations vary widely. Consequently, numerous devices have been used for fenestration closure, including Gianturco coils [14], detachable coils [20], Raskind duct occluders [13], Clamshell devices [4], CardioSeal devices [17], Amplatzer septal occluder [15,16,22], Amplatzer duct occluder [18], AngelWings devices [19], and covered stents [21]. Choice of device to close a fenestration is guided by several factors, including the type of Fontan circulation (intracardiac versus extracardiac), size and location, geometry of the fenestration, likelihood of placing a long sheath in the systemic atrium, ease of availability and atrial size [21].…”

“…The majority of patients had an extracardiac Fontan conduit (n ¼ 63) with only five (n ¼ 5) having lateral tunnel intracardiac conduits. The median size of the fenestration was 5.0 mm (range: 4-6) in a 20 mm (range [14][15][16][17][18][19][20][21][22] conduit. Patients were divided into two groups: The aim in the first group (n ¼ 50) was to obtain complete closure of the fenestration.…”

“…Persistent cyanosis may lead to decreased exercise tolerance and a risk of paradoxical embolic events if spontaneous closure of the fenestration does not occur [5][6][7][8][9][10]. In addition to the use of surgically placed abdominal snares [11,12], a variety of transcatheter devices have been used for fenestration closure [1,[13][14][15][16][17][18][19][20][21][22]. However, most of these devices lack desirable criteria such as ease of deployment, low profile with minimal material, nonthrombogenicity and a perfect closure rate.…”

Right Ventricular Cardiomyopathy Accompanied by Protein-Losing Enteropathy and Chylous Effusion.

“…Protein-losing enteropathy is also frequently mentioned as occurring after the Fontan operation and in cases of congenital heart diseases, such as tricuspid hypoplasia. 6 However, only a few cases of proteinlosing enteropathy associated with cardiomyopathy have been reported. 7 It is conceivable that severe right-side heart failure induces chronic venous congestion and lymphangiectasia, and, in the present case, abnormal connection of the thoracic duct might have exacerbated lymphangiectasia and induced both protein-losing and poor absorption of fat.…”

Fenestration Improves Clinical Outcome of the Fontan Procedure

“…18 Furthermore, multiple centers have shown that a fenestration can be safely closed with a device in the cardiac catheterization laboratory 18 -20 and that such closures are welltolerated. 21 At our institution, we have not routinely occluded fenestrations unless there is significant cyanosis without an alternative cause. Follow-up of this randomized cohort may provide additional insights into effective long-term treatment.…”