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PubMed2001

The bronchial cast syndrome after the fontan procedure: further evidence of its etiology.

We describe a three-year-old boy who presented with recurrent expectoration of bronchial casts six months following creation of a fenestrated lateral tunnel Fontan circulation for pulmonary atresia with intact ventricular septum. Cardiac catheterization demonstrated elevated central venous pressure with two areas of stenosis within the Fontan circuit, specifically at the junction of the right superior caval vein and the right pulmonary artery, and between the atrial baffle and the right superior caval vein. Insertion of Palmaz stents in these areas resulted in a reduction in central venous pressure, and a transient reduction in production of casts. Eight weeks after catheterization, however, he produced further casts, which resulted in hypoxia, respiratory arrest and death. We reviewed the autopsied specimens obtained from patients with the Fontan circulation over an eleven-year period at our institution in order to ascertain the prevalence of subclinical production of bronchial casts. We found no casts in the thirteen patients examined. Hemodynamic assessment is vital in all patients who develop this syndrome, and should be the primary focus of investigation, rather than solely directing efforts at lysis of casts.

Bronchogenic Cyst↗
PubMed1983

The Fontan procedure in the absence of the interatrial septum. Failure of its principle?

The Fontan principle, defined as a procedure in which the right ventricle is bypassed in order to convey desaturated venous blood from the right atrium to the lungs, is presently applied for a wide variety of congenital heart malformations including those in which there is no suitable ventricular pumping chamber. Recently, the procedure has also been advocated for complex malformations that require atrial septation or intra-atrial rerouting. The present report evaluates our experience in four patients with such complex malformations. Three had a complete form of atrioventricular septal defect with double-outlet right ventricle and one patient had left atrioventricular valve atresia. The common atrium was morphologically right in two patients and morphologically left in one. In each of these instances anomalous pulmonary venous connections were present, together with abnormal systemic venous connections. The results were unsatisfactory. Three of the four patients died. The only survivor had no pulmonary or systemic venous abnormalities. Severe impairment of pulmonary blood flow was one of the most important postoperative complications. The findings suggest that the complexities of rerouting the blood within the atria play an important role. The excess proportion of prosthetic material to atrial myocardium may result in excessive loss of contractile atrial myocardium and, probably equally significant, in a complex intra-atrial geometry of pathways that may cause a critical pressure gradient. The results clearly show that with an expanding horizon of the application of the Fontan principle, new pitfalls may arise which presently appear to be the prevailing factors limiting its success.

Adolescent↗
PubMed2004

Modification to the Fontan procedure for the prophylaxis of intra-atrial reentrant tachycardia: short-term results of a prospective randomized blinded trial.

OBJECTIVES: We evaluated the feasibility, safety, and short-term efficacy of an interventional atrial incision placed at the time of the Fontan operation to reduce the development of intra-atrial reentrant tachycardia. METHODS: This prospective randomized blinded trial was conducted in patients with congenital heart disease undergoing an initial lateral tunnel Fontan. Intervention patients underwent a lateral tunnel Fontan with an interventional atrial incision/cryoablation from the atriotomy to the right atrioventricular annulus. Controls underwent a standard lateral tunnel Fontan. Safety of the intervention was monitored. Short-term efficacy was determined by comparisons of conduction block across the incision area and spontaneous or inducible atrial arrhythmias. RESULTS: There were no significant differences between intervention (n = 21, median 2.4 years, range 0.8-3.9) and controls (n = 21, median 2.7 years, range 1.5-13.9) in age, type of heart disease, surgical parameters, or postoperative outcomes. Safety parameters showed no difference between groups in number or severity of adverse events. Short-term efficacy included evidence of conduction block with a longer conduction time across the incision area in intervention patients (median 97 ms, range 35-160) compared with controls (median 40 ms, range 8-77, P =.0001). No intervention patients had spontaneous or inducible intra-atrial reentrant tachycardia versus 2 controls (0/21 versus 2/21, P = NS). CONCLUSIONS: An interventional atrial incision to reduce intra-atrial reentrant tachycardia in the Fontan operation was feasible and safe. The intervention changed the atrial substrate as shown by an increase in conduction time. Short-term results showed a low incidence of intra-atrial reentrant tachycardia in all patients. Longer follow-up is necessary to assess clinical efficacy.

Adolescent↗