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Hemi-Fontan procedure for hypoplastic left heart syndrome: outcome and suitability for Fontan.

BACKGROUND: Following the Norwood procedure for hypoplastic left heart syndrome (HLHS), pulmonary artery distortion and hypoplasia are common and may negatively impact late outcome. The hemi-Fontan procedure (HFP) augments the central pulmonary arteries and establishes a connection between the right atrial/superior vena cava junction and the pulmonary arteries, while excluding the inferior vena cava. METHODS: The hospital records of all 114 patients undergoing a HFP for HLHS between August 1993 and April 1998 were reviewed to assess patient, procedural, and morphologic determinations of outcome. The results of cardiac catheterization, Doppler/echocardiography, 12 lead electrocardiograms, hospital and subsequent course, as well as suitability and outcome for the Fontan procedure were analyzed. RESULTS: Mean age was 5.4 months (range 1.5 to 15 months). Right ventricular function was normal in 95 patients, moderately depressed in 14, and severely depressed in five. Tricuspid regurgitation was absent or mild in 91 patients, moderate in 13, and severe in 10. Concomitant procedures included left superior vena cava to pulmonary artery anastomosis (12), tricuspid valve repair (10), pulmonary artery stent placement (3), coarctation repair (2), and aortic pseudoaneurysm repair (1). Hospital survival was 112/114, 98% (95% confidence interval [CI]: 95% to 100%). There were two late deaths, one noncardiac. Sinus rhythm is present in 105 patients (92%, 95% CI: 87% to 97%). To date, 79 of these patients have undergone the Fontan procedure with 74 survivors (94%, 95% CI: 89% to 99%). CONCLUSIONS: The HFP may be performed with excellent results for HLHS. It effectively augments the central pulmonary arteries while preserving sinus rhythm in the majority. In addition, the HFP facilitates the subsequent Fontan procedure and has significantly improved the overall outcome.

Angiography↗

Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure: risk of development of arteriovenous fistulae.

Since the Fontan procedure results in low pulsatile pulmonary blood flow similar to that seen in patients with a Glenn shunt, it may also be associated with abnormal distribution of flow to the lower lung lobes and with the development of pulmonary arteriovenous fistulae (PAVF). In 12 patients 0.8 to 4.5 years after Fontan procedure and in 20 patients 0.2 to 18 years after receipt of Glenn shunts we assessed ventilation (with 133Xe) and perfusion (after a peripheral injection of 99mTc-macroaggregated albumin) to compare upper to lower lobe distribution of blood flow with that in a control group. The presence of PAVF was assessed by radionuclide activity in kidneys and the brain and by a two-dimensional echocardiographic contrast study. A decreased upper/lower lobe perfusion ratio was noted in 13 of 20 patients with Glenn shunts (65%) and correlated with the time after surgery (p less than .05). Despite the shorter follow-up period, two of 12 (16%) patients who had undergone the Fontan procedure also had a decreased upper/lower lobe perfusion ratio, and one of these developed right heart failure. Brain and kidney radionuclide counts above control values were observed in all patients with Glenn shunts and in 11 of 12 patients who had the Fontan operation. However, in only five of 20 (25%) patients with Glenn shunts were PAVF confirmed by the two-dimensional echocardiographic contrast study. Three of the five patients with PAVF had Glenn shunts of long duration.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[A modified Damus-Kaye-Stansel anastomosis for restrictive bulvoventricular foramen developed 5 years after Fontan procedure].

An one-year old girl, who had pulmonary artery (PA) banding and balloon atrioseptostomy done in the newborn period, underwent modified Fontan procedure on an emergency basis without cardiac catheterization, because of right heart failure. Echocardiogram showed no subaortic stenosis at Fontan procedure. Five years later, subaortic stenosis was documented with a 65 mmHg of ventricular-aortic pressure gradient at catheterization. A modified Damus-Kaye- Stansel (D-K-S) procedure was successfully performed using an end-to-end anastomosis of the distal aorta and a common stump, consisting of previously suture-closed main PA stump and the proximal aorta. At postoperative catheterization, the pulmonary valve was functioning well at LV outlet portion without regurgitation and stenosis. Significant subaortic stenosis may develop after Fontan procedure in patients with morphological narrowing without demonstrable pressure gradient. Such patients should have surgical intervention to the narrowing at the time of Fontan procedure. The modified D-K-S procedure can be an effective procedure to relieve subaortic stenosis after Fontan procedure.

Anastomosis, Surgical↗

Similarities and differences in the exercise performance of patients after a modified Fontan procedure compared to patients with complete transposition following a Senning operation.

OBJECTIVE: To investigate cardiopulmonary performance in patients after a Fontan procedure, comparing it to patients following a Senning operation. METHODS: We studied 21 children, with a mean age of 11.1 years, after a total cavopulmonary anastomosis, comparing them to 13 with complete transposition after a Senning procedure, having a mean age of 11.8 years, and 21 control subjects with a mean age of 11.2 years. All were tested on a bicycle ergospirometer. RESULTS: Peak consumption of oxygen, maximal work rate, peak oxygen pulse and endexpiratory pressure of carbon dioxide at a work rate of 1.5 Watt/kg were lowest in patients with a modified Fontan procedure, and highest in the control group (p < or = 0.0278). Production of carbon dioxide, and minute ventilation at a work rate of 1.5 Watt/kg, was highest in the patients after Fontan procedure, and lowest in the healthy subjects (p < or = 0.0163). Production of carbon dioxide per single breath was lower in those having a Fontan procedure (28.9 ml) than in the two other groups (35.1 ml; p = 0.0243). The tidal volume showed no significant differences between the three groups. CONCLUSIONS: The reaction to exercise was identical qualitatively in both groups of patients, and comparable to the behaviour of patients with chronic heart failure. Quantitatively, the results of the patients following a Senning procedure lay between those of control subjects and those who had undergone a Fontan operation. The only exception was dead space ventilation, where the patients after a Fontan procedure differed from the two other groups because of their increased ventilation-perfusion mismatch.

Anastomosis, Surgical↗

Anticoagulation and incidence of late cerebrovascular accidents following the Fontan procedure.

Thromboembolic events are a well-reported complication following the Fontan procedure, but no previous studies have compared the incidence of thromboembolic events relative to the prophylactic anticoagulation strategy utilized. We examined the time-adjusted incidence of late thromboembolic events relative to chronic anticoagulation strategy. All patients who have undergone Fontan palliation and are followed at our institution were reviewed. All thromboembolic and major bleeding events were recorded and compared among different subgroups (anticoagulant medication utilized, Fontan variant, and the presence of a residual right-to-left shunt). The incidence of late cerebrovascular accidents (CVAs) per patient-year was calculated for each subgroup. The records of 132 patients were analyzed (median follow-up, 7.6 years; 1066.5 total patient-years). There were no major bleeding complications. One patient receiving no anticoagulation therapy developed a symptomatic thrombus 6 months after Fontan. Three patients suffered late CVAs (range, 3-7 years); 2 were receiving aspirin, and the other received no anticoagulation therapy. All 3 had lateral tunnel Fontan and a residual right-to-left shunt. The overall incidence of late CVA was 2.3%, with an event rate of 0.28% per patient-year. Late CVA was not related to anticoagulation strategy or time from Fontan procedure but was associated with a residual right-to-left shunt and lateral tunnel-type Fontan palliation (p < 0.001). Regardless of anticoagulation strategy utilized, symptomatic CVA is a rare long-term complication following the Fontan procedure.

Adolescent↗

Is an increase in lactate concentration associated with cardiac dysfunction after the Fontan procedure?

PURPOSE: To investigate how blood lactate concentration changes in the early postoperative course after the Fontan procedure, and whether such a change is associated with postoperative hemodynamics. MATERIALS AND METHODS: Eight pediatric patients who underwent the Fontan procedure for congenital heart disease were included. Enrollment criteria were body weight >10 kg and staged Fontan procedure following the bidirectional Glenn procedure. Blood lactate concentration and central venous oxygen saturation (SvO2) were measured at five points: before skin incision, upon intensive care unit (ICU) admission, 15 minutes before, 15 minutes after, and 2 hours after extubation. Cardiac index (CI) was continuously monitored using the pulse contour technique. RESULTS: Lactate concentration increased in the ICU, peaking from 15 minutes before (2.7+/-1.3 mmol/L) to 15 minutes after (3.0+/-1.3 mmol/L) extubation. Values returned to normal parameters (1.0+/-0.1 mmol/L) within 48 hours. The CI values were also higher immediately after extubation (4.0+/-0.6 L/min/m2) than at ICU admission (3.3+/-0.6 L/min/m2) and before extubation (3.6+/-0.6 L/min/m2) (p=0.012). No significant change in central SvO2 or blood pressure (BP) was evident during the study period. CONCLUSION: In the early postoperative period after the Fontan procedure, blood lactate concentration increased temporarily around the time of extubation but the increase was not associated with hemodynamic deterioration.

Adolescent↗

Early results of extracardiac fontan procedure with autologous pericardial tube conduit.

BACKGROUND: Different types of conduits are used for extracardiac Fontan procedure. The use of autologous pericardial tube as an alternative conduit for connecting the inferior vena cava to the pulmonary artery is investigated. METHODS: We performed 25 extracardiac Fontan procedures by using pericardial tube between June 2000 and October 2003. Fifteen patients were male. Mean age and weight were 7.6 +/- 4.9 years (range 3 to 24 years) and 22.8 +/- 10.4 kg (range 12 - 50 kg), respectively. RESULTS: All patients survived after extracardiac Fontan procedure. Prolonged chest tube drainage (> 7 days) was required in 9 (36 %) patients. Follow-up was complete and mean follow-up was 24.6 +/- 11.5 months (1 - 39 months). One patient died 3 months postoperatively. Routine serial postoperative echocardiographic examinations in all patients and magnetic resonance angiography in 6 patients with suspect flow patterns in echocardiography did not reveal any problems with the Fontan circuit. CONCLUSIONS: Fresh autologous pericardial tube conduit is a suitable and safe alternative for extracardiac Fontan procedures. Major advantages are availability, no cost, easy handling and hemostasis, low risk of thrombosis and emboli, and growth potential.

Adolescent↗

Elevated atrial natriuretic peptide after the Fontan procedure.

To assess the response of atrial natriuretic peptide (ANP) to rapidly changing right atrial pressures in vivo, we measured ANP levels in 15 patients undergoing the Fontan procedure and compared them with control levels in nine patients undergoing cardiac surgery for lesions not associated with atrial hypertension. There were no significant differences in preoperative ANP levels: 57 +/- 15 pg/ml for patients undergoing the Fontan procedure, 43 +/- 8 pg/ml for control patients. There was no significant change in ANP during surgery or in the postoperative period in control patients. In contrast, ANP increased significantly to 333 +/- 70 pg/ml (p less than .0025) after establishment of right atrial-pulmonary artery continuity with the Fontan procedure and was related to right atrial pressure, which increased from 5 mm Hg before to 14 mm Hg after the Fontan procedure (p less than .001). There was no significant change in left atrial pressure. During the first postoperative day, ANP levels fell to 141 +/- 34 pg/ml (p less than .01) but later increased to 290 +/- 80 pg/ml (p less than .025), a finding that may suggest depletion of a readily releasable intracellular pool of ANP before mobilization of a storage pool. There was no direct relationship between ANP levels and effusions in patients undergoing the Fontan procedure. Pharmacologically significant increases in ANP occur in patients undergoing the Fontan procedure, correlating with increased right atrial pressures, but the relationship between ANP and the fluid derangements after the procedure remains unclear.

Atrial Natriuretic Factor↗

Modified Fontan procedure in patients less than 4 years of age.

BACKGROUND: Initial experience with the modified Fontan procedure in patients < 4 years of age has met with variable success, with early reports showing a high mortality. More recently, improved results with the modified Fontan procedure in younger patients have been achieved. Important advantages of an early Fontan include protection of the pulmonary vascular bed and preservation of ventricular function by reduction in ventricular volume overload and chronic hypoxemia. METHODS AND RESULTS: From 1982 through May 1991, 90 patients < 4 years of age underwent a modified Fontan procedure. The average age was 29 months (range, 7-48 months), and 30 patients were < 2 years of age. Diagnoses included tricuspid atresia in 36, single ventricle in 45, pulmonary atresia intact septum in seven, and hypoplastic left heart syndrome in two patients. An adjustable ASD was placed in 21. Early mortality was 8.9% (eight of 90), which is only slightly higher than our overall Fontan early mortality of 7.4% in 242 patients. The early mortality in the 30 patients < 2 years of age was 6.7% (two of 30). In a mean follow-up of 38 months (range, 7-89 months), there have been three late deaths (3.7%), and one patient has undergone cardiac transplantation. CONCLUSIONS: Early Fontan should be undertaken in patients > 6 months old who present with increasing cyanosis provided good hemodynamics are present. A modified Fontan procedure can be performed safely with good clinical results in patients < 4 years old.

Anastomosis, Surgical↗

Management of atrial flutter after the Fontan procedure.

OBJECTIVES: The purpose of this study was to review the management of atrial flutter occurring after the Fontan procedure. BACKGROUND: Atrial flutter occurs frequently after the Fontan procedure and is often hemodynamically poorly tolerated. METHODS: The patients' charts were reviewed for relevant information. RESULTS: Between 1984 and 1992, 18 patients had atrial flutter after the Fontan procedure. The underlying heart defect was tricuspid atresia in nine, mitral atresia in six and double inlet left ventricle in three. All but three patients had undergone previous palliative surgery. The time interval from Fontan operation to atrial flutter was < 1 day to 16 years (mean 3.7 years). Seven had early atrial flutter before leaving the hospital. Electrophysiologic study in 15 showed sinus node dysfunction in 12. Atrial flutter was inducible in all patients, and 13 had > 1 flutter configuration. Digoxin and a variety of other antiarrhythmic agents (mean 2.7 drugs/patient) were tried with poor results. Only digoxin, amiodarone, flecainide and propafenone showed some benefit when used alone or in combination. Antitachycardia pacemakers were implanted in 16 patients (endocardial 14, epicardial 2) and, with drugs, were useful in 8 (50%). Because atrial flutter was resistant to treatment, right atriectomy was performed in three patients (with benefit in two, one death), successful radiofrequency catheter His bundle ablation in one patient and catheter ablation of atrial flutter in three patients (two failed, one partial success). One patient underwent heart transplantation, and two died suddenly. Another died of complications after an elective epicardial pacemaker replacement procedure. CONCLUSIONS: Atrial flutter after the Fontan procedure is difficult to control. Aggressive drug and antitachycardia pacemaker therapy help about half of the patients. When these measures fail, other options, such as atriectomy, His bundle ablation or catheter ablation of atrial flutter, need consideration. The risk of sudden death justifies the use of such aggressive treatment methods.

Adolescent↗

Management of late complications after classic Fontan procedure by conversion to total cavopulmonary connection.

Classic Fontan procedures, such as atriopulmonary connection or atrioventricular connection, are associated with right atrial dilatation and several late complications. Seven patients with univentricular heart who presented with exercise intolerance (n = 7), severely dilated right atrium (n = 7) with pulmonary vein compression (n = 3), atrial arrhythmias resistant to medical treatment (n = 4), cyanosis (n = 4), diffuse effusions and oedema (n = 1), and protein-losing enteropathy (n = 1), underwent conversion to total cavopulmonary connection 5.8-14.4 years after a previous atriopulmonary connection (n = 6) or atrioventricular connection (n = 1). A 14-year-old boy who, preoperatively, was in ventricular failure and a very poor state died early after conversion because of low cardiac output. All survivors had either marked or partial clinical improvement with regression of cardiomegaly, absence of pulmonary vein compression or cyanosis, and recovery of sinus rhythm. Conversion to total cavopulmonary connection appears to be effective in the treatment of late complications after classic Fontan procedures. It should be considered early in symptomatic patients, before significant ventricular dysfunction and clinical deterioration ensue.

Adolescent↗

Impact of the off-pump Fontan procedure on complement activation and cytokine generation.

BACKGROUND: To investigate whether the Fontan procedure in an off-pump fashion is less invasive in terms of the systemic inflammatory reaction. METHODS: Plasma levels of complement and cytokines were measured during and after the Fontan procedure in consecutive 38 patients. Of these, 16 underwent the extracardiac method without use of cardiopulmonary bypass (off-pump group), while the machine was used in the remaining 22 because of intracardiac maneuvers concomitantly needed (CPB group). RESULTS: There was no difference, between these two groups, in any value of plasma complement or cytokines after anesthetic induction. Immediately after commencement of the Fontan circulation, however, plasma concentration was significantly lower in the off-pump group for activated complement 3 (C3a), interleukin-6, interleukin-8, and polymorphonuclear elastase. The C3a value was also lower at 2 hours later in the off-pump group than in the CPB group. Furthermore, the values even stayed within the normal ranges, in the off-pump group, for tumor necrosis factor alpha (TNFalpha) and thrombomodulin. A pulmonary venous oxygen tension divided by an inspired oxygen tension (PpvO2/FiO2) ratio immediately after commencement of the Fontan circulation was 528 +/- 93 mm Hg (410 to 580 mm Hg) in the off-pump group, and 258 +/- 167 mm Hg (86 to 540 mm Hg) in the CPB group (p = 0.01). Duration of drainage for fluid sequestration was shorter in the former group (6.3 +/- 0.7 days versus 13.9 +/- 2.5 days, p = 0.02). CONCLUSIONS: Inflammatory reactions were attenuated when the Fontan procedure was employed in an off-pump fashion compared with the usual procedure on bypass.

Adolescent↗

Time course of endothelin-1 and adrenomedullin after the Fontan procedure.

BACKGROUND: The endothelium-derived vasoconstrictor endothelin (ET)-1 might contribute to the physiology of blood flow regulation and play a role in cardiovascular disease. Adrenomedullin (AM) is a potent vasodilator peptide that has major effects on cardiovascular function and has multiple biologic effects involved in cardiovascular homeostasis. Although pulmonary vascular resistance is known to be one of the most important factors to determine the indications for a Fontan procedure, the time course of the plasma cytokine before and after the Fontan procedure is not known. METHODS: Sixteen patients were divided into two groups, 8 patients (1 to 14 years) who had the Fontan procedure (atriopulmonary connection) and 8 age-matched controls who had biventricular repair with normal central venous pressure. Plasma ET-1 and adrenomedullin levels were measured in both groups immediately before cardiopulmonary bypass, immediately after cardiopulmonary bypass, and 6 and 24 hours after cardiopulmonary bypass. A thermodilution catheter was inserted during the operation, and mean pulmonary arterial pressure, pulmonary wedge pressure, and cardiac output were measured, and pulmonary vascular resistance was calculated at the same time points. Correlation between the plasma ET-1 levels and pulmonary vascular resistance data were obtained in the Fontan group. RESULTS: Plasma ET-1 levels in the Fontan group were elevated after operation and were higher than the control group at 6 and 24 hours after cardiopulmonary bypass. Plasma adrenomedullin in the Fontan group was lower than in the control group at 6 and 24 hours after cardiopulmonary bypass. A significant positive correlation was obtained between the plasma ET-1 and pulmonary vascular resistance data (r = 0.475). CONCLUSIONS: Imbalance between increased ET-1 and relatively decreased adrenomedullin after cardiopulmonary bypass in the Fontan procedure could contribute to dominant effects of ET-1, which might induce vasoconstriction after the Fontan procedure. ET-1 might play an important role in maintaining vasoconstriction after the Fontan procedure.

Adolescent↗

A model to simulate the haemodynamic effects of right heart pulsatile flow after modified Fontan procedure.

The effect of pulsatile pulmonary flow after the modified Fontan procedure was examined in a model that simulated the right heart. An inlet overflow tank (preload), axial pulsatile pump, Wind-Kessel model (afterload), and an outlet overflow tank were connected in series. The standard conditions were flow 2.00 l/min with 12 mm Hg preload pressure, 3.0 Wood units resistance, and an outlet overflow tank pressure at 6 mm Hg. The pump rate was set at 80 beats/min. The simulated pulmonary arterial pressure and pulmonary flow waves produced by this model closely resembled those obtained from patients who had undergone the modified Fontan procedure. All variables except the preload were fixed and changes in pulmonary flow were examined at preload pressures of 8, 12, 15, and 17 mm Hg. As the peak pulmonary arterial pressure increased so did pulmonary flow, until it was greater than during the non-pulsatile state. Because the afterload of this model was fixed, this result suggests that there was a concomitant decrease in resistance. This model indicates that pulsatile pulmonary blood flow is likely to have a beneficial effect on the pulmonary circulation after the modified Fontan procedure.

Blood Pressure↗

Right to left interatrial communications after the modified Fontan procedure: identification and management with transcatheter occlusion.

OBJECTIVE: To describe unusual venous communications from the right to the left atrium resulting in cyanosis after the modified Fontan procedure, and their management with transcatheter occlusion. METHODS: Between September 1992 and November 1994, eight patients were assessed for persistent cyanosis after a modified Fontan procedure. Desaturation was found to be caused by unusual venous shunts originating at atrial level, and transcatheter occlusion with either a double umbrella or coil was attempted. RESULTS: Three types of venous channels were identified. The first type of communication consisted of thin long tortuous channels originating from the right atrial wall, and draining into the left atrium through a capillary network. The second type of communication was in the superior anterior portion of the atrial baffle, incorporating the pectinate muscles of the right atrium, draining into the neoleft atrium. These channels were shorter and often fanned out into small vessels toward the right atrial appendage. In each instance, the shunts were in the superior suture line of a lateral tunnel modification of the Fontan procedure. The third type of communication originated from the inferior vena cava, connecting inferior phrenic veins to pericardial veins and subsequently to the left atrium, at or close to the ostium of the left pulmonary veins. Before device occlusion, the room air aortic oxygen saturation was 88(SD 4)% (range 84% to 94%), and increased to 95(3)% (range 91% to 100%) following occlusion (PL << 0.001). The mean right atrial pressure was 14(4)mm Hg and remained unchanged after occlusion. In six patients there was complete shunt obliteration, while in two both occluded with umbrella devices, a small residual leak persisted. No complication occurred during or immediately after catheterisation. CONCLUSIONS: Unusual venous communications can evolve after the Fontan procedure, resulting in the development or persistence of cyanosis. Some of these communications may be present preoperatively as normal veins draining into the right atrium, enlarging with the increased atrial pressure after surgery. These observations affect long term function after the Fontan procedure. Transcatheter occlusion of these communications is technically feasible and effective, although recurrence may occur.

Adolescent↗

Morphological findings contributing to a failed Fontan procedure. Twelve-year experience.

A group of 37 patients (age range, 3 months to 29 years) who died after the modified Fontan procedure (within 2 months), representing 15% of the 245 patients undergoing this procedure from 1976 through 1988, was reviewed to determine the causes of death. The three morphological groups were 1) univentricular atrioventricular connection (n = 19), 2) tricuspid atresia (n = 9), and 3) other complex malformations (n = 9). Subaortic stenosis was present in 15 patients (40%) in this group compared with 31 of 208 early survivors (15%) (p = 0.008). Pulmonary artery banding, identified as a risk factor in our previous experience, was performed in 14 patients, nine of whom had subaortic stenosis. Nine palliative procedures for subaortic stenosis were performed in eight patients--before the Fontan procedure in three patients and concurrently with the Fontan procedure in five patients. Myocardial hypertrophy and signs of acute ischemic injury were common findings at autopsy (n = 25) and were particularly prominent in all patients with univentricular heart of left ventricular morphology associated with subaortic stenosis and previous pulmonary artery banding (n = 7). We conclude that the present majority of deaths occurring after the modified Fontan procedure are myocardial in nature and attributable to advanced myocardial hypertrophy that is potentiated by previous pulmonary artery banding and subaortic stenosis.

Aortic Valve Stenosis↗

Fenestration improves clinical outcome of the fontan procedure: a prospective, randomized study.

BACKGROUND: The Fontan procedure is the definitive operation for palliation of complex congenital heart disease with single-ventricle physiology. Fenestration of the Fontan circuit allows for shunting of deoxygenated blood to the systemic circulation. This procedure improved the clinical outcomes of patients who are at high risk for poor Fontan results. However, it is controversial whether fenestration is beneficial for standard-risk patients. METHODS AND RESULTS: This prospective, randomized trial evaluated the clinical utility of fenestration in patients with standard preoperative risk profiles for Fontan surgery. Forty-nine consecutive patients were assigned to undergo either a fenestrated (25 patients) or nonfenestrated (24 patients) Fontan procedure. The fenestrated and nonfenestrated groups were comparable with respect to age (P=0.944), body surface area (P=0.250), number of preoperative risk factors for poor outcome (P=0.681), cardiopulmonary bypass time (P=0.302), number of patients who required aortic cross-clamping (P=0.240), preoperative oxygen saturation (P=0.101), and number of patients with dominant left ventricular morphology (P=0.534). Patients in the fenestrated group had 55% less total chest tube drainage (P=0.036), 41% shorter total hospitalization (P=0.018), and 67% fewer additional procedures in the postoperative period (P=0.006) than those in the nonfenestrated group. CONCLUSIONS: Baffle fenestration performed at the time of Fontan surgery improves short-term outcome in standard-risk patients by decreasing pleural drainage, hospital length of stay, and need for additional postoperative procedures.

Child↗

Imaging of pulmonary venous pathway obstruction in patients after the modified Fontan procedure.

To evaluate the efficacy of chest roentgenograms and echocardiograms in identifying pulmonary venous pathway obstruction in patients after the modified Fontan procedure, the records of 297 patients who underwent 307 Fontan procedures between 1984 and 1990 were reviewed. Twelve cases of pulmonary venous pathway obstruction documented by autopsy (3 cases) or cardiac catheterization (9 cases with an A wave gradient greater than or equal to 4 mm Hg) were found in 10 patients (mean age 43 +/- 28 months). The mechanisms of obstruction included narrow pulmonary vein ostia in six cases, narrow left atrial outlet in four and atrial baffle obstruction in three. Two causes of obstruction were present in one case. No patient had pulmonary venous congestion on chest roentgenograms. Pathway diameters indexed to 3 square root of body surface area with two-dimensional echocardiography were found to be 6.8 +/- 1.2 mm/3 square root of m2 in the subcostal frontal view, 7.9 +/- 0.6 mm/3 square root of m2 in the subcostal sagittal view and 6.5 +/- 1.7 mm/3 square root of m2 in the apical "four-chamber" view. These values differed significantly from those in 11 age-matched patients undergoing the Fontan procedure without pulmonary venous pathway obstruction documented by catheterization (p = 0.001). With pulsed Doppler ultrasound, there was a relatively narrow range of velocities distal to the obstruction (1.3 to 2.5 m/s). In five of the seven cases with pulsed Doppler measurements, flow was continuous and the Doppler spectral recordings were not phasic. Thus, in patients who have undergone the Fontan procedure and have pulmonary venous pathway obstruction 1) chest roentgenography cannot be used as a screening tool; 2) distal velocities as low as 1.3 m/s occur, usually with nonphasic, continuous forward flow; and 3) pathway diameters indexed to 3 square root of body surface area may be used as an output-independent parameter to cross-check Doppler data.

Cardiac Surgical Procedures↗