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PubMed2000

Extracardiac conduit fontan procedure: early and intermediate results.

OBJECTIVE: The extracardiac Fontan procedure, as compared with classic atriopulmonary connections, may have the potential for optimizing ventricular and pulmonary vascular function by maximizing the laminar flow principle, by the avoidance of intra-atrial suture lines and cardiac manipulation, and by minimizing cardiopulmonary bypass time. In this study the clinical results of this procedure are assessed. METHODS: From January 1990 until January 1997, 45 patients (33 males and 12 females) with a median age of 4.0 years (range 2.7-38 years) underwent an extracardiac Fontan procedure for univentricular physiology. The underlying diagnoses included tricuspid atresia (n=19), double-inlet left ventricle (n=11), and complex anomalies (n=15). Forty patients (89%) were in sinus rhythm. The median ventricular ejection fraction was 60%. In 37 patients (82%) the procedure was staged. RESULTS: Median cardiopulmonary bypass time was 72 min, with a decrease to a median time of 24 min in the last ten patients. Aortic cross-clamping was avoided in 33 patients (73%). The intraoperative Fontan pressure and transpulmonary gradient were low: 13.6+/-3.2 and 8.5+/-3.9 mmHg, respectively. Transient supraventricular tachyarrhythmias were observed in six patients (13%). There was no early or late mortality. At a median follow-up of 64 months (range 26-105 months), 39 patients (87%) were in NYHA class I, four (9%) were in NYHA class II, and two (4%) were in class III. Forty patients (89%) remained in sinus rhythm. The median ventricular ejection fraction was 59%. The median arterial oxygen saturation raised from 82% preoperatively to 97%. Functional class (P=0.02), maintenance of sinus rhythm (P=0.04), and preservation of ventricular function (P=0.05) was superior in patients who were appropriately staged. None of the patients had atrial thrombus, chronic pleural effusions, or protein losing enteropathy. CONCLUSIONS: In the majority of patients, the extracardiac Fontan procedure, when performed as a staged procedure, provides excellent early and midterm results in terms of quality of life, maintenance of sinus rhythm, and preservation of ventricular function.

Adolescent↗
PubMed2005

Long-term anticoagulation therapy and thromboembolic complications after the Fontan procedure.

BACKGROUND: The necessity for chronic anticoagulation of Fontan patients remains controversial. We determined the prevalence of thromboembolic complications after the Fontan procedure in relation to different long-term anticoagulation strategies. METHODS: The clinical outcomes, postoperative anticoagulation strategies and occurrence of thromboembolic complications in 102 ethnic Chinese patients who had undergone Fontan procedure between 1980 and 2002 were reviewed. RESULTS: The early and late surgical mortalities, all unrelated to thromboembolism, were 10.8% (11/102) and 5.8% (6/104), respectively. Of the 85 survivors, 46 (54%) were maintained on long-term warfarin therapy, 8 (9%) on aspirin prophylaxis while 31 (37%) were not on chronic anticoagulation. Four (4.5%) patients, two with and two without warfarin prophylaxis, developed thromboembolic complications at 0.14 to 7.7 years after the Fontan procedure (0.74%/patient-year). Three had a grossly dilated right atrium after atriopulmonary connection, two of whom had atrial fenestrations. The other had atrial tachycardia. Freedom from development of thromboembolic complications (mean+/-S.E.) at 1, 5 and 10 years after surgery was 97+/-19%, 96+/-2.5% and 92+/-4.2%, respectively. When compared with those on long-term warfarin therapy, patients without chronic anticoagulation were followed-up longer (p=0.001), more likely to have undergone atriopulmonary connection (p<0.001), less likely to have fenestrations (p=0.02) and cardiac arrhythmias (p=0.02) but not predisposed to increased risk of thromboembolism (p=1.00). CONCLUSION: The study supports the contention that chronic anticoagulation may not be required for majority of ethnic Chinese Fontan patients. Nonetheless, it may perhaps be considered in those with grossly dilated right atrium, cardiac arrhythmias and residual right-to-left shunts.

Anticoagulants↗
PubMed1998

Clinical results of the staged Fontan procedure in high-risk patients.

BACKGROUND: For high-risk Fontan candidates, the introduction of a bidirectional Glenn shunt before total cavopulmonary connection (a two-staged strategy) may extend the indications for the Fontan procedure. The clinical results of the two-staged and one-staged Fontan procedure were thus reviewed and compared. METHODS: Between November 1991 and July 1996, the two-staged strategy was performed in 40 high-risk Fontan candidates with a mean interval of 17.2 months after introducing the bidirectional Glenn shunt (staged group). We considered a young age (<2 years), high mean pulmonary arterial pressure (> or =20 mm Hg), high pulmonary vascular resistance (> or =3 Wood units), small pulmonary artery (Nakata index <200 mm2/m2), atrioventricular valve incompetence (> or = moderate), distortion of pulmonary artery, anomalous pulmonary venous return, and poor ventricular function as risk factors for the successful completion of Fontan circulation. During the same period, 68 patients underwent the modified Fontan procedure in a one-step fashion (primary group). RESULTS: In the staged group after the bidirectional Glenn shunt, the mean pulmonary arterial pressure and ventricular end-diastolic pressure were both found to have decreased significantly to the same level as those in the primary group, whereas the pulmonary artery demonstrated a significantly smaller size than that in the primary group. Operative morbidity was similar in both groups. Operative mortality was also similar and low in both groups (1.5% in the primary group and 0% in the staged group). CONCLUSIONS: A bidirectional Glenn shunt was found to be a useful interim palliation in high-risk Fontan candidates. This two-staged strategy may extend the operative indications for the Fontan procedure.

Adolescent↗
PubMed2006

Transcatheter closure of ventriculopulmonary artery communications in staged Fontan procedures.

BACKGROUND: Ventricle-pulmonary artery connections in patients after the Fontan procedure lead to ineffective volume loading and can cause long term problems. In patients with a cavopulmonary shunt anterograde pulmonary blood flow is frequently maintained, but can cause significant volume loading of the heart or complicate the subsequent Fontan procedure. OBJECTIVE: To evaluate the use of transcatheter closure of a ventricle-pulmonary artery communication in the setting of a cavopulmonary shunt or after the Fontan procedure. PATIENTS AND METHODS: Retrospective study at a tertiary referral centre. Eight patients (age 1.5-18 years, mean 7.8 years). INDICATIONS: cardiac failure or persistent pleural effusions after cavopulmonary shunt (n = 2) or after Fontan (n = 3) and abolishing the volume load of the single ventricle prior to Fontan completion (n = 3). RESULTS: Devices used: Rashkind Umbrella (n = 1), Amplatzer PDA (n = 7) and Amplatzer ASD (n = 1). One patient required two devices. There were no procedural complications. All 3 patients with prolonged pleural effusions (1 post CP shunt and 2 post Fontan) showed complete resolution between 4 and 10 days after catheter closure. Two patients underwent transcatheter occlusion for progressive ventricular dilatation and cardiac failure. The first patient was post Fontan and showed gradual improvement in ventricular function. The second patient (post CP shunt) was in end stage cardiac failure due to severe AV valve regurgitation. The patient died 48 hours after an uncomplicated procedure due to ventricular failure and electromechanical dissociation (non-procedure-related cardiac death). Three patients underwent catheter closure to off-load the systemic ventricle prior to the Fontan procedure. The device had to be removed prior to release in one patient, due to unsatisfactory position. CONCLUSIONS: Transcatheter closure of ventricle-pulmonary artery communication is a safe and effective technique in the treatment of selected patients after cavopulmonary shunt or Fontan procedure with early or late complications due to inappropriate pulmonary blood flow. This intervention should also be considered in the preparation for the Fontan procedure in selected patients with ventricular overload.

Adolescent↗
PubMed1995

Effect of age and surgical technique on symptomatic arrhythmias after the Fontan procedure.

The purpose of this study was to determine the effects of newer Fontan modifications (lateral tunnel with or without fenestration) and patient's age at surgery on the incidence and impact of symptomatic postoperative early and intermediate arrhythmias. Modifications to the Fontan procedure are used to decrease postoperative complications, and the Fontan procedure is now being performed on younger patients to reduce age-related changes in ventricular function. A retrospective review was done of the medical records of 151 consecutive patients, ranging in age from 1 to 49 years, who underwent a Fontan procedure at Texas Children's Hospital between 1987 and 1993. Risk factors were identified for early and intermediate arrhythmias. Age at time of the procedure was an independent predictor of early atrial arrhythmias (p = 0.03), ventricular arrhythmias (p = 0.003), and junctional ectopic tachycardia (JET) (p = 0.05). We found that the older the patient at surgery, the higher the incidence of atrial and ventricular arrhythmias, whereas the younger the patient, the higher the incidence of JET. Using Cox's proportional-hazards model, the risk of intermediate atrial arrhythmias after lateral tunnel modification was 1/3 that after atriopulmonary connection. Younger patients who underwent the Fontan procedure had a lower risk for early atrial and ventricular arrhythmia but an increased risk for JET. The lateral tunnel modification can be performed in order to reduce the risk of intermediate atrial arrhythmias.

Adolescent↗
PubMed1989

[Modified Fontan procedure on 106 cases: indication and surgical results].

Since 1974, we have performed modified Fontan procedure on 106 patients, ranging in ages from 1 to 32 years, consisting of 44 cases of tricuspid atresia (TA), 21 with univentricular heart (UVH) of right ventricular type, 18 with UVH of left ventricular type, for which ventricular partition was unfeasible, and 23 with various complex anomalies. Hospital mortality rates for TA and other complex anomalies were 11.4 and 11.3%, respectively. Surgical results have markedly improved recently. Since 1986, 50 cases underwent Fontan procedure with 3 hospital deaths (6.0%). Late death occurred in 4 cases in a mean follow-up period of 49 months. Regarding the indication for operation, majority of patients had 2 to 3 parameters which were out of 10 criteria for Fontan procedure. Regurgitation of atrioventricular valve was repaired by annuloplasty in 19 patients underwent Fontan procedure and 17 survived. Abnormal systemic venous connection was seen in 11 cases and all survived. Association of total anomalous pulmonary venous connection is still a difficult problem and 2 of 5 cases died. Fontan procedure was performed in 8 patients following palliative right ventricular outflow reconstruction for poor development of pulmonary artery and 7 survived. Cumulative mortality rate for the entire series was relatively well at 15.1%.

Adolescent↗
PubMed1996

Conversion of modified Fontan procedure to lateral atrial tunnel cavopulmonary anastomosis.

After modified Fontan procedures with atriopulmonary anastomoses or right atrium-right ventricle conduits, some patients have progressive exercise intolerance, effusions, arrhythmias, or protein-losing enteropathy. Theoretic advantages of a lateral atrial tunnel cavopulmonary anastomosis and published clinical results suggest that conversion of other Fontan procedures to the lateral atrial tunnel may afford clinical improvement for some patients. Eight patients (8 to 25 years old) with tricuspid atresia (n =4), double-inlet left ventricle (n = 3), and double-outlet right ventricle (n=1) underwent conversion to a lateral tunnel procedure between December 1990 and November 1994. An arbitrary clinical score was assigned before the lateral tunnel procedure and at follow-up. Before conversion, patients had decreased exercise tolerance (n = 8), arrhythmias (n = 6), effusions (n = 4), and protein-losing enteropathy (n = 8). At catheterization, all had a low cardiac index (1.9 +/- 0.7 L x min(-1) x M(-2), five had elevated pulmonary vascular resistance (>3 Wood units), and three had right pulmonary venous return obstruction by compression of an enlarged right atrium. Fenestrated lateral tunnel construction was undertaken 7.3 +/- 3.6 years after atriopulmonary anastomosis, with one early death related to low cardiac output. After the lateral tunnel procedure, two patients had no clinical improvement (no change in clinical score) but five patients had either marked or partial improvement. The right pulmonary vein compression present in three patients was resolved after conversion. The mean clinical scores improved from 4.5 +/- 1 to 3.0 +/- 2 (p < 0.04). In conclusion, conversion to a lateral tunnel procedure led to clinical improvement in five of eight patients at short-term follow-up and may be particularly indicated for patients with giant right atria or pulmonary vein compression who have symptoms. Pulmonary vein compression should be looked for in patients after modified Fontan procedures and can be relieved by conversion to the lateral tunnel procedure.

Adolescent↗
PubMed2002

[Single-stage Fontan procedure: early and late outcome in 124 patients].

INTRODUCTION AND OBJECTIVES: The Fontan procedure was designed to palliate complex congenital heart disease with univentricular physiology. A retrospective study was made to document the determinants of early (</= 30 days) and late (>/= 31 days) mortality with the modified Fontan procedure performed in one-stage over a 22-year period. MATERIAL AND METHODS: Between 1978 and 2000, 102 atriopulmonary, 16 cavopulmonary, and 6 Kawashima type anastomoses were performed to palliate complex congenital heart defects in 124 patients with a mean age of 7.3 4.7 years. Forty-five patient and procedure-related variables were analyzed in relation to mortality. All events were verified. RESULTS: There were 29 early (23%) and 20 late (16%) deaths. Estimated survival at 30 days, 2 years, 5 years, and 20 years was 78, 75, 66, and 50%, respectively. Subaortic stenosis, protein-losing enteropathy, and arrythmia were observed in 8, 5 and 33 patients, respectively, after surgery. Univariate and multivariable analysis indicated that left ventricular end-diastolic pressure (>/= 13 mmHg), mean pulmonary pressure (>/= 19 mmHg), mitral stenosis/atresia, atrioventricular valve regurgitation, visceral heterotaxia, absence of fenestration, risk factors criteria, duration of extracorporeal circulation, and operative technique were associated with early mortality. Reoperation, arrhythmia, and pacemaker implantation were predictors of late death. Forty percent remained free from surgical or catheter reintervention after Fontan operation at 20 years. CONCLUSIONS: The outcome of Fontan procedure is profoundly affected by patient-related variables (ventricular function and pulmonary circulation). Postoperative arrhythmia and reoperation shortened the lifespan of the Fontan circulation model in patients with atriopulmonary connections. Total cavopulmonary anastomosis improves the physiology of univentricular circulation. In the light of our findings, the modified Fontan procedure (one or two stages) should be performed early in life to better preserve ventricular and pulmonary vascular function.

Adolescent↗
PubMed1994

Spontaneous closure of fenestrations in an interatrial Gore-Tex patch: application to the Fontan procedure.

The concept of the partial Fontan procedure, first described with the adjustable atrial septal defect (ASD) and more recently with the fenestrated technique, has become an accepted approach for the management of high-risk patients undergoing the Fontan procedure. Experience with both techniques has shown that a patent ASD placed in a prosthetic interatrial baffle may close spontaneously over a period of weeks to months. The mechanism and timing of spontaneous closure, as well as the effect of antiplatelet therapy on this process, are poorly understood. To better define this process, the interatrial septum of 15 mongrel dogs was excised and replaced with a fenestrated Gore-Tex (W.L. Gore, Flagstaff, AZ) patch. Postoperative echocardiography confirmed the patency of the ASD and left-to-right shunting. Animals were sacrificed 4 to 6 weeks postoperatively, or sooner if infection or other postoperative complications developed. Eight animals underwent no antiplatelet or anticoagulation therapy postoperatively, and 7 received antiplatelet therapy with aspirin. Patches were removed at the end of the study period and analyzed. By 6 weeks, all 2.7-mm and 4-mm holes had closed spontaneously in all animals that had not received antiplatelet therapy. The earliest closure occurred at 1 week. With antiplatelet therapy, hole closure was found to be delayed but not prevented, and was complete by 6 weeks in all but 1 animal. Histologic examination of the explanted patches revealed that closure was accomplished primarily through the ingrowth of fibrous tissue, accompanied by an inflammatory cell infiltrate.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed2005

Intermediate outcomes after the Fontan procedure in the current era.

OBJECTIVE: This study was undertaken to investigate the intermediate outcomes after the Fontan operation in the current era. METHODS: A cross-sectional analysis was performed by using data gathered between October 13, 2003, and February 22, 2005, on all 310 survivors of the Fontan procedure between January 1, 1992, and December 31, 1999. Medical records were reviewed, and a customized questionnaire was administered to caregivers. Outcome variables included failure of the Fontan operation (ie, death, takedown of Fontan, or transplantation), number of cardiac-related rehospitalizations, parental assessment of health, school performance, and cardiac functional status. RESULTS: A total of 332 patients underwent the Fontan procedure during the study period. A lateral tunnel was constructed in 281 (85%), and an extracardiac Fontan procedure was performed in 51 (15%). A total of 210 (63%) children had undergone previous stage I reconstruction, and 162 (49%) had received an initial diagnosis of hypoplastic left heart syndrome. There were 310 hospital survivors of the Fontan procedure (93.4%), with an additional 16 deaths (5.2%) during follow-up and 1 living heart transplant patient before the cross-sectional period. For initial hospital survivors, Kaplan-Meier estimates of freedom from death or transplantation were 98.0% (95% confidence interval, 95.6%-99.1%) at 1 year after the Fontan procedure, 94.9% (91.6%-97.0%) at 5 years, and 93.9% (90.1%-96.2%) at 8 years. Questionnaires were completed for 240 (81.9%) of the 293 available patients. By parental recollection, a total of 130 (54.2%) children required cardiac-related rehospitalization at some point during the follow-up period. At a median follow-up of 8.6 years (range, 4.1-12.8 years), 94.6% of guardians described their child's overall health as excellent or good, and 5.4%, as fair or poor. School performance was described as above average in 30.2%, average in 39.9%, and below average in 29.8%. With regard to cardiac functional status, 34.2% responded that their child had no limitations to physical activity, 52.5% reported a slight limitation, 12.1% reported a significant limitation, and 1.2% reported a severe limitation. CONCLUSIONS: Acceptable survival outcomes have been observed at intermediate follow-up of the Fontan operation in this cohort; a significant proportion of patients had hypoplastic left heart syndrome. Although cardiac-related rehospitalization is common, the parents and guardians believed that overall health, school performance, and cardiac functional status were good to excellent for most patients.

Child↗
PubMed1984

Dynamics of right heart flow in patients after Fontan procedure.

In seven patients who underwent Fontan procedures but in whom no valves were inserted, dynamics of right heart flow were evaluated with the use of a catheter-tipped velocity transducer, pulsed Doppler echocardiography, and angiocardiography. Right atrial (RA) contraction caused a forward flow to the pulmonary artery (PA) and a backward flow to the inferior vena cava (IVC). Backward flow to the superior vena cava (SVC) was minimal. As the right atrium relaxed, a rapid forward flow occurred at the IVC and SVC that filled the atrium and a small amount of pulmonary regurgitant flow was observed. Subsequently, a forward flow was observed at the IVC, SVC, and PA during RA diastole. Angiographically determined RA stroke volume (SV) was less than 40% of the left ventricular (LV) SV in three patients in whom the postoperative increase in atrial "a" wave pressure (delta p) was greater than 8 mm Hg, while it was similar to or greater than LVSV in four patients in whom delta p was 6 mm Hg or less. In all patients LV end-diastolic volume was 107 +/- 27(SD)% of normal but LV ejection fraction was 0.53 +/- 0.07, resulting in the reduced cardiac output (2.8 +/- 0.7 l/min/m2). There was no correlation between the RASV or RA ejection fraction and cardiac output. These data show that the RA contraction causes a forward flow to the PA and that pulmonary regurgitation is not significant after Fontan procedure even when valves are not inserted. Also, the postoperative increase in the RA afterload may depress RA function.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2002

Effects of dilutional and modified ultrafiltration in plasma endothelin-1 and pulmonary vascular resistance after the Fontan procedure.

BACKGROUND: Pulmonary vascular resistance (PVR) is closely related with patients' hemodynamics after the Fontan procedure and endothelin-1 (ET-1) may play an important role in pulmonary circulation. Modified ultrafiltration (MUF) is known to remove inflammatory mediators after cardiopulmonary bypass (CPB) surgery. The time courses of plasma ET-1 and PVR were examined before and after the Fontan procedure with MUF. METHODS: Twenty-two patients who underwent the Fontan procedure were divided into two groups: a dilutional ultrafiltration/modified ultrafiltration (DUF/MUF) group (n =11) and a control group (n = 11). Conventional ultrafiltration was performed during CPB in the control group. DUF was performed semicontinuously during CPB and MUF was continued until 15 to 20 minutes after the CPB with polyacrylonitonile membrane in the DUF/ MUF group. The plasma ET-1 concentration was measured before and after CPB, after MUF in the DUF/MUF group, and 6 and 24 hours after CPB. PVR was calculated simultaneously using a thermodilutional catheter. RESULTS: Plasma ET-1 levels increased significantly after CPB in the control group but they did not increase immediately after CPB in the DUF/MUF group. Similarly, PVR increased significantly after CPB in the control group but it did not increase after CPB in the DUF/MUF group and remained low at 6 and 24 hours after CPB. CONCLUSIONS: DUF and MUF suppress the increase in the plasma ET-1 concentration that occurs immediately after the completion of the Fontan procedure and may be an effective intervention for maintaining low PVR after the procedure.

Cardiopulmonary Bypass↗
PubMed1992

Outcome and assessment after the modified Fontan procedure for hypoplastic left heart syndrome.

BACKGROUND: We reviewed the outcome of 76 consecutive patients (age range, 5 months to 6 years; median age, 19 months) who underwent a modified Fontan procedure after initial palliative surgery for hypoplastic left heart syndrome (HLHS) between January 1984 and December 1989. METHODS AND RESULTS: Modifications of the Fontan procedure included transatrial baffle of pulmonary venous return to the tricuspid valve (n = 10) or inferior vena cava baffle within the right atrium to the superior vena caval-pulmonary artery anastomosis, with pulmonary artery augmentation (n = 66). Actuarial survival rates were 74% (1 month), 58% (12 months), 56% (2 years), and 52% (4 years). Of the 43 survivors, 25 patients have returned for postoperative cardiac catheterization at a medium of 13 months after the Fontan procedure. Mean +/- SD hemodynamic values were cardiac index, 2.8 +/- 0.6 l/min/m2; right arterial pressure, 11 +/- 2 mm Hg; pulmonary artery wedge pressure, 6 +/- 3 mm Hg; and arterial oxygen saturation, 94 +/- 3%. No patient had significant tricuspid or native pulmonary valve insufficiency. CONCLUSIONS: Survival after the Fontan procedure in patients with HLHS is comparable to survival after a Fontan procedure in patients with other complex congenital heart lesions. In the subgroup of patients with HLHS who survived both reconstructive surgery and a Fontan procedure and have been evaluated by cardiac catheterization after a Fontan procedure, the use of the right ventricle as the systemic ventricle yielded excellent intermediate results for Fontan physiology.

Cardiac Catheterization↗
PubMed1995

Comparison of cardiopulmonary adaptation during exercise in children after the atriopulmonary and total cavopulmonary connection Fontan procedures.

BACKGROUND: There are several potential physiological differences between the atriopulmonary (AP) and the total cavopulmonary connection (TCPC) Fontan circulations. Studies suggest that the TCPC reduces energy loss due to turbulence and may have more dependence on respiratory movement for pulmonary blood flow. We compared cardiopulmonary physiology during rest and exercise in patients who had undergone the AP Fontan procedure with those who had undergone the TCPC Fontan procedure. METHODS AND RESULTS: Forty-three children were studied more than 6 months after undergoing a Fontan procedure (23 AP and 20 TCPC); 106 healthy children were also studied as a control group. Measurements of effective pulmonary blood flow, stroke volume, arteriovenous oxygen difference, minute ventilation, heart rate, and oxygen and carbon dioxide consumption were made with an Innovision quadrupole mass spectrometer. Data from the control group allowed calculation of z scores for the Fontan groups matched for age, sex, pubertal stage, and body surface area. Maximal exercise performance was equal in the two Fontan groups, but it was below normal. However, adaptation to exercise was different in the Fontan groups. After 9 minutes of exercise, pulmonary blood flow rose less in the AP group than in the TCPC group (P < .01), and the stroke volume in the AP group also tended to be lower (P = .057) and their arteriovenous oxygen difference was significantly greater (P < .01). Although minute ventilation per unit of carbon dioxide production was similar in the Fontan groups at this level of exercise, children in the TCPC group breathed faster by approximately 10 breaths per minute (P < .005). CONCLUSIONS: At submaximal exercise, children who had undergone the TCPC Fontan procedure had pulmonary hemodynamics superior to those of children who had undergone the AP procedure, largely because of respiratory adaptation that permitted blood to be "sucked" into the lungs. To achieve the same maximal exercise performance, children who had undergone the AP procedure had a superior metabolic adaptation to exercise stress.

Adaptation, Physiological↗
PubMed1995

Effect of late postoperative atrial septal defect closure on hemodynamic function in patients with a Lateral tunnel Fontan procedure.

OBJECTIVES: The aim of this study was to evaluate prospectively the effect of late atrial septal defect closure on cardiac output and oxygen delivery in patients who have undergone the Fontan procedure. BACKGROUND: An adjustable atrial septal defect is incorporated in patients undergoing the Fontan procedure who have increased pulmonary vascular resistance or poor ventricular function, or both. After the Fontan procedure, the atrial septal defect is test occluded. Patients with mean right atrial and pulmonary artery pressures > 15 mm Hg are discharged with the atrial septal defect open. METHODS: Twelve patients (20 months to 12 years old) underwent evaluation and closure of the atrial septal defect at a mean interval of 3.8 months (range 1 to 18) after the Fontan procedure. Each patient underwent full right and left heart catheterization. Cardiac output was obtained using the cine-volume method. The study included six patients with a high transpulmonary gradient or poor ventricular function preoperatively, or both (high risk group) and six who had only borderline increased pulmonary vascular resistance (low risk group). Patients in both groups had a mean right atrial pressure > 15 mm Hg when the atrial defect was test occluded in the first week after the Fontan procedure. RESULTS: All results are given as mean value +/- SD. Ventricular end-diastolic pressure was significantly lower (p = 0.03) with the atrial septal defect open in low risk patients (6 +/- 3 mm Hg) than in high risk patients (10 +/- 3 mm Hg). With the atrial septal defect open, low risk patients had a significantly higher (p = 0.04) cardiac index (4.87 +/- 0.81 liters/min per m2) than the high risk patients (3.96 +/- 0.47 liters/min per m2). There was no significant difference (p = 0.14) in cardiac index between the two groups with occlusion of the atrial septal defect. Oxygen delivery was also significantly higher (p < 0.05) with the atrial septal defect open in low risk patients (836 +/- 99 ml/min per m2) than in high risk patients (704 +/- 106 ml/min per m2). There was no significant difference (p = 0.89) in oxygen delivery between the two groups with occlusion of the atrial septal defect. With the atrial septal defect open, the interatrial gradient was not significantly different in low risk patients (4 +/- 1 mm Hg) from that in high risk patients (4 +/- 1 mm Hg). CONCLUSIONS: These data show that an interatrial communication results in increased postoperative systemic perfusion and oxygen delivery in patients with good diastolic ventricular function after the Fontan procedure.

Atrial Function↗
PubMed2005

Systemic oxygen saturation and coagulation factor abnormalities before and after the fontan procedure.

Coagulation factor abnormalities are believed to predispose to increased risk for thromboembolism after the Fontan procedure. Recent data, however, suggest that coagulation abnormalities may precede the operation. This study determined coagulation factor abnormalities in patients with single-ventricle congenital heart defects before the Fontan procedure and compared the findings with age-matched post-Fontan patients and control subjects. Liver function, coagulation factor levels, and pulse oximetry readings were determined in 11 pre-Fontan patients aged 9.3+/-5.6 years. The results were compared with those of 11 age-matched post-Fontan patients, at 9.1+/-2.4 years after the Fontan procedure, and 11 age-matched controls by simple analysis of variance and post hoc Tukey's test. Liver function was normal in patients before and after surgery apart from mildly elevated bilirubin levels in post-Fontan patients (p=0.027). When compared with controls, pre-Fontan patients had significantly lower levels of protein C; protein S; antithrombin III; and factors II, V, VII, and X and longer prothrombin times (all with p<0.05). When compared with post-Fontan patients, pre-Fontan patients had similarly lower levels of free protein S (p<0.001), protein C (p=0.06), and antithrombin III (p=0.001). Systemic oxygen saturation correlated positively with the levels of anticoagulants (protein C, protein S, antithrombin III) and procoagulants (factors II, V, VII, and X) (all with p<0.05, r=0.33 to 0.61). In conclusion, the findings suggest that coagulation factor abnormalities precede the Fontan procedure and tend to normalize after the operation, relating perhaps to improved systemic oxygenation.

Biomarkers↗