Arezou Saedi

Persistent fenestration may be a marker for physiologic intolerance after Fontan completion

BACKGROUND We sought to evaluate the medium-term implications of fenestration status. METHODS Between 1994 and 2012, 326 patients received an extracardiac Fontan (hospital mortality n = 6, 1.8%). A fenestration was routinely created (n = 306, 94%) unless there was technical difficulty. Three hundred patients discharged with an open fenestration were included. The primary end points were death and Fontan failure. Secondary outcomes were Fontan complications such as venovenous collaterals, protein-losing enteropathy, pacemaker requirement, and arrhythmias. RESULTS The fenestration was closed in 260 patients: 185 as a catheter intervention (62%) and 75 (25%) spontaneously. Forty patients (13%) had the fenestration open at a median follow-up period of 5.05 years. Of these patients, catheter-based closure failed in 10 (3%). There was no statistically significant difference in pre-Fontan hemodynamic parameters, such as pulmonary artery pressure and pulmonary vascular resistance between the patients with open fenestration and the ones with closed fenestration. Patients with an open fenestration had significantly more late deaths (P < .001), Fontan failure (P = .021), and Fontan complications (P = .011) compared with those with a closed fenestration. Multivariable Cox regression revealed open fenestration (P < .001) and indeterminate ventricular morphology (P = .002) as risk factors for death/Fontan failure, and ventricular dysfunction (P = .014) and open fenestration (P = .009) as risk factors for Fontan complications. CONCLUSIONS Persistent fenestration was a marker for physiologic intolerance as noted by increased rates of mortality and a higher incidence of Fontan failure/complications. The specificity of pre-Fontan physiologic data for fenestration status may not have the fidelity needed for long-term care and thus, the consequences of decision making regarding fenestration status may not be determined until well after the operation.

Geometry and growth of the reconstructed aorta in patients with hypoplastic left heart syndrome and variants

Objective: The interdigitating technique in aortic arch reconstruction in hypoplastic left heart syndrome and variants (HLHS) reduces the recoarctation rate. Little is known on aortic arch growth characteristics and resulting clinical impact. Methods: A total of 139 patients with HLHS underwent staged palliation between 2007 and 2014; 73 patients underwent arch reconstruction. Dimensions of ascending aorta, transverse arch, interdigitating anastomosis, and descending aorta in pre‐stage II and pre‐Fontan angiograms were measured. Aortic arch dimensions were analyzed. Ventricular and atrioventricular valve function were assessed. Results: Diameters increased in all segments between pre‐stage II and pre‐Fontan (P < .0005). The z scores remained unchanged in all segments but the descending aorta that was significantly larger pre‐Fontan (P = .039). Dimensions and z scores between pre‐stage II and pre‐Fontan correlated in proximal segments, but not at and distal to the interdigitating anastomosis. Pronounced tapering occurred between the transverse arch and the interdigitating anastomosis. Arch intervention of any type was performed in 7 (9.6%), and intervention for recoarctation in 3 (4.1%) patients. Conclusions: The aortic arch after reconstruction with the interdigitating technique differs from normal. Growth was proportional with no further geometrical distortion. Recoarctation and reintervention rate is low. Further improvement may be achieved by optimizing patch configuration and material.

Chylothorax and pleural effusion in contemporary extracardiac fenestrated fontan completion

Background: We hypothesized that chylothorax could be a sign of intolerance to the Fontan physiology, and thus patients who develop chylothorax or pleural effusion have worse medium‐term to long‐term survival. Methods: A total of 324 patients who underwent the Fontan operation between 2000 and 2013 were included. Chylothorax was defined as ≥5 mL/kg/day of chylomicron‐positive chest drainage fluid no earlier than postoperative day 5 or drainage with >80% lymphocytes. Outcomes were compared between the chylothorax and non‐chylothorax groups by the Kaplan–Meier method and log‐rank test. Independent predictors of chylothorax and number of days of any chest drainage were analyzed with multivariable logistic regression and multivariable generalized negative binomial regression for count data, respectively. Results: Chylothorax occurred in 78 patients (24%). Compared with the non‐chylothorax group, the chylothorax group had a longer duration of chest tube requirement (median, 18 days vs 9 days; P < .000) and a longer length of hospital stay (median, 19 days vs 10 days; P < .000). Eight patients (10.3%) required thoracic duct ligation. The chylothorax group had lower freedom from death (P = .013) and from composite adverse events (P = .021). No predictor was found for chylothorax. Pulmonary atresia (P = .031) and pre‐Fontan pulmonary artery pressure (P = .01) were predictive of prolonged pleural effusion (>14 days). Conclusions: Occurrence of chylothorax following the Fontan operation can be a marker of poorer medium‐term clinical outcomes. It is difficult to predict occurrence of chylothorax owing to its multifactorial nature and involvement of lymphatic compensatory capacity that is unmasked only after the Fontan operation.