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PubMed1997

Dyspnoea and exercise intolerance during cardiopulmonary exercise testing in patients with univentricular heart. The effects of chronic hypoxaemia and Fontan procedure.

BACKGROUND: Patients with univentricular hearts have decreased exercise tolerance and may demonstrate exertional dyspnoea. It is not known if chronic hypoxaemia exacerbates exercise intolerance and contributes to symptomatic limitation. The extent to which surgical correction of a right-to-left shunt by a Fontan-type procedure can increase exercise tolerance by reducing arterial deoxygenation is not well documented. The cardiopulmonary exercise responses and the symptomatic status in two groups of univentricular patients, those who are cyanotic and those who are acyanotic with Fontan-type circulation, were compared. METHODS AND FINDINGS: Cardiopulmonary exercise testing was performed in 10 univentricular patients with rest or stress-induced cyanosis (age 30.5 +/- 2.3 [SE] years; 5 men) who had palliative or no surgery and eight patients (age 29.4 +/- 1.5 years; 4 men) with Fontan-type circulation. Peak oxygen consumption was comparable in both groups of univentricular patients (21.7 +/- 2.5 vs 21.0 +/- 1.9 ml.kg-1.min-1, P = 0.85) but was less than an age-matched group of 10 healthy subjects (34.7 +/- 1.9 ml.kg-1.min-1, P < 0.001 for both). Arterial oxygen saturation was 90.6% at rest in the cyanotic patients compared with 95.1% in the Fontan patients (P < 0.001) and at peak exercise, 66.2% compared with 90.5% (P < 0.001). Using a modified Borg scale (0-10), the symptoms of dyspnoea and fatigue were also assessed during exercise in the patient groups. The Borg scores for dyspnoea in the cyanotic and the corrected univentricular patients were, respectively, as follows: Stage 1: 0.5 vs 1.7; P= 0.04; Stage 2: 1.8 vs 2.3, P = 0.5; Stage 3: 3.0 vs 3.5, P = 0.7; Peak Exercise: 4.9 vs 4.8, P = 0.9. In addition, the Borg scores for fatigue were: Stage 1: 0.4 vs 1.6, P = 0.08; Stage 2: 2.0 vs 2.2, P = 0.9; Stage 3: 3.0 vs 4.3, P = 0.5; Peak Exercise: 4.9 vs 5.4, P = 0.5. The major limiting symptom at peak exercise was dyspnoea in four cyanotic patients compared with one in the Fontan group (Chi-square 0.982, P > 0.10). The arterial oxygen desaturation at peak exercise in the cyanotic patients limited by dyspnoea was not different from those limited by fatigue (67.5 +/- 10.1% vs 66.7 +/- 13.7%, P = 0.92). Exercise tolerance was also not related to the arterial oxygen saturation at peak exercise (r = 0.47, P = 0.17) in these patients. CONCLUSIONS: Despite correction with Fontan-type surgery, the exercise tolerance and symptoms of these univentricular patients remained similar to those who were cyanosed. Cyanotic patients have adjusted to chronic hypoxaemia and it does not appear to determine the exercise tolerance or the genesis of dyspnoea in these patients. Further randomized prospective studies are required to investigate the long-term benefits of Fontan-type procedures in these patients on exercise tolerance, symptoms and prognosis.

Adult↗
PubMed1992

Arrhythmias after the Fontan procedure.

OBJECTIVE: To study the determinants and outcome of arrhythmias after the Fontan type operation. DESIGN: Retrospective analysis of data in patients operated on between 1972 and 1986 (follow up 5-19 years (mean 12 years)). PATIENTS: All 60 patients undergoing a Fontan type procedure at the National Heart Hospital, London, during the study period (mean age (SD) 12.3 (6.8) years). RESULTS: Postoperative arrhythmias occurred in 34 patients (57%), and 11 (58%) of 19 early postoperative deaths (within seven days) were related to arrhythmias. Early arrhythmias occurred in 19 (32%) patients of whom 11 (58%) died. All patients with early atrial fibrillation and His bundle tachycardia died and only preoperative atrial fibrillation recurred early. There was a higher incidence of early arrhythmias, which were less well tolerated, in double inlet single ventricle patients (9/19) than in those with tricuspid atresia (8/37). There were no other preoperative determinants of early arrhythmias or deaths from early arrhythmia. Late (after seven days) arrhythmias occurred in 15 (37% of hospital survivors). They had higher right atrial (RA) pressures both early and late after operation and had lower ventricular ejection fractions late after operation. Of those with atrial arrhythmias 86% had RA obstruction and 57% had an RA thrombus or pulmonary embolism at presentation; this was also confirmed in two patients in whom late sudden deaths occurred. Atrial fibrillation early after reoperation for RA obstruction was fatal. The actuarial arrhythmia free survival for hospital survivors was 60% at 10 years. CONCLUSIONS: Early postoperative arrhythmias were poorly tolerated, particularly atrial fibrillation and His bundle tachycardia. Previous atrial fibrillation was a relative contraindication to this procedure. Late postoperative arrhythmias were associated with higher RA pressures measured both early and late after operation and worse late ventricular function. Late arrhythmias may be the first manifestation of RA obstruction, which must be sought. RA thrombus was common in patients with atrial arrhythmias and should be treated early with anticoagulants.

Adolescent↗
PubMed2002

Reverse flow in compliant vessels and its implications for the Fontan procedure: numerical studies.

The Total Cavopulmonary Connection (TCPC), a variant of the Fontan operation used for palliative cardiovascular repair of patients with single ventricle physiology, creates a passive system of blood flow into the pulmonary circulation for which energy efficiency may be critical to long term outcome. Clinical studies have shown that reverse flow in the TCPC is an indication of poor clinical status in these patients. Using numerical simulations, we demonstrate that reverse flow leads to increased energy losses in compliant vessels. Such an effect can potentially set off a series of spiraling negative events with decreased ventricular function leading to reverse flow, which causes decreased energy efficiency, which in turn leads to worsening function, and so forth, thereby suggesting one cause of progressive heart failure in this patient group.

Compliance↗
PubMed2002

Coagulation factor abnormalities in patients with single-ventricle physiology immediately prior to the Fontan procedure.

BACKGROUND: Coagulation abnormalities have been reported following the Fontan operation and have been attributed to various aspects of Fontan-associated physiology. Using age-matched controls, this study evaluated coagulation abnormalities in children who had undergone a bidirectional Glenn procedure to test the hypothesis that coagulation abnormalities are present before the Fontan operation. METHODS: Coagulation factors were assayed in 38 children (mean age 34.4 +/- 15 months) immediately before the Fontan operation; 37 healthy children (mean age 33 +/- 17 months) were assayed as controls. Concentration of factors II, V, VII, VIII, IX, and X and of antithrombin III, plasminogen, proteins C and S, fibrinogen, serum albumin, and liver enzymes were measured. Normal reference intervals based on the control patients were determined using 95% confidence limits. Patient demographic data, hemodynamic variables, and elapsed time after the Glenn procedure were evaluated as possible predictors of coagulation abnormalities. RESULTS: Concentrations of protein C; factors II, V, VII, and X; plasminogen; and antithrombin III were significantly lower before the Fontan operation compared with age-matched controls (p < 0.01); no specific hemodynamic variables were predictive of a pro- or anticoagulant deficiency. There were significant positive correlations between patients who had abnormally low factor VII, protein S, and protein C levels and a longer interval between the bidirectional Glenn procedure and the Fontan operation (p < 0.001). CONCLUSIONS: Coagulation abnormalities that could predispose patients to increased risk for clotting or bleeding are evident early in the course of staged single-ventricle repair.

Blood Coagulation Factors↗
PubMed1992

The modified Fontan procedure: physiology and anesthetic implications.

The modified Fontan operation has gained wide acceptance as a functional corrective procedure for patients with CHD with single ventricle physiology. Long-term survival and palliation of symptoms are excellent with most patients able to lead normal lives. The absence of a pulmonary contractile ventricle means that the single ventricle is responsible for perfusion of both the pulmonary and systemic circulations. Elevated systemic venous pressure is required to overcome PVR and this state of systemic venous hypertension has a significant impact on the anesthetic and postoperative care of these patients.

Anastomosis, Surgical↗
PubMed1979

Persistence and effects of sinus rhythm after Fontan procedure for tricuspid atresia.

Four patients who had had a Fontan type of procedure for tricuspid atresia 23, 6, 6, and 11 months previously were investigated by ambulatory electrocardiographic recording and simultaneous recording of the jugular venous pressure and echocardiogram of the conduit or pulmonary valve. All had been considerably improved by the operation. In 1 patient episodes of supraventricular tachycardia were recorded but no rhythm disturbance was detected in the other 3. Pulmonary blood flow was shown to be pulsatile and atrial systole is an important factor in this. The conduit valve showed delayed opening and slow closure suggesting that its presence in the pulmonary circuit may be unnecessary.

Adolescent↗
PubMed2004

Left ventricular outflow obstruction after Fontan procedure involving a patient with complete atrioventricular septal defects complicated by a small left ventricle.

We report a 1-year-old girl who developed ventricular outflow tract obstruction early after a Fontan operation, necessitating surgical relief using the Damus-Kaye-Stansel procedure. The patient had a complete atrioventricular septal defect complicated by a muscular ventricular septal defect (VSD) and a small left ventricle, a morphology that has not previously been reported in cases of systemic outflow tract obstruction after the Fontan procedure. Postoperative systemic outflow obstruction must be considered as a possible sequela following Fontan surgery in patients with an atrioventricular septal defect and a small left ventricle.

Aftercare↗
PubMed2006

Laparoscopic surgery in the pediatric patient post Fontan procedure.

We present two case reports describing laparoscopic surgery in patients who have undergone previous Fontan surgery and discuss the theoretical implications of laparoscopic surgery in these patients. A brief discussion of the late complications of Fontan surgery is included.

Child↗
PubMed1998

Oxygen utilization and hemodynamic response during exercise in children after Fontan procedure.

Eight patients, 9.1 to 16.5 years of age, were studied 2.8 to 8.5 years after Fontan operation. Oxygen utilization was determined during upright bicycle exercise. The cardiac index and stroke index were measured by echocardiography and the anaerobic threshold was determined. The results were compared with 10 patients after surgical closure of the atrial septal defect. Anaerobic threshold (AT) in Fontan patients was lower than in the control subjects. Oxygen consumption at each stage of exercise was significantly lower in the Fontan group compared with the control subjects. From the beginning of exercise until AT, the increase in stroke index was lower in the Fontan patients than in the control subjects. After that point, the stroke index decreased significantly in the Fontan patients while it remained almost at the same level in the control subjects. Significant correlations were observed between the oxygen pulses and the stroke index at AT both in the control and Fontan groups. These results suggest that impaired exercise capacity in Fontan patients is mainly due to a subnormal response of the stroke index at AT and to the decreased response of the stroke index and the heart rate at the maximal workload.

Adolescent↗
PubMed2002

Coagulation abnormalities in patients with single-ventricle physiology precede the Fontan procedure.

OBJECTIVE: Thromboembolic events in patients who have undergone the Fontan operation have been reported to be as high as 20% to 33%. A hypercoagulable state with deficiencies in proteins C and S has been implicated. Using age-matched control subjects, we evaluated whether an altered coagulation state is present earlier in the course of staged single-ventricle repair. METHODS: After informed consent had been obtained, coagulation factors were assayed in 36 infants (mean age, 7.7 +/- 3.6 months) with single-ventricle cardiac defects immediately before undergoing the bidirectional Glenn procedure; 34 infants (mean age, 8.4 +/- 2.6 months) without cardiac disease were assayed as control subjects. Concentration of factors II, V, VII, VIII, IX, and X; antithrombin III; plasminogen; proteins C and S; fibrinogen; serum albumin; and liver enzymes were measured. Normal reference intervals on the basis of the control subjects were determined by using 95% confidence limits. Patient demographic and hemodynamic variables were evaluated as possible predictors of coagulation abnormalities. RESULTS: Concentrations of protein C; factors II, V, VII, IX, and X; plasminogen; fibrinogen; and antithrombin III were significantly lower in the pre-Glenn infants compared with the age-matched control subjects (all P <.001, Student t test). On the basis of multiple logistic regression, no specific hemodynamic variables were predictive of a procoagulant or anticoagulant deficiency. Ventricular dysfunction did predict the presence of multiple coagulation abnormalities (P <.001). CONCLUSION: Procoagulant and anticoagulant factor abnormalities occur early in the course of single-ventricle repair and precede the cavopulmonary connection.

Blood Coagulation Factors↗