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PubMed1988

Cardiorespiratory response to exercise after the Fontan procedure for tricuspid atresia.

Noninvasive exercise testing was used to assess gas exchange in 13 patients age 6-25 yr who had undergone Fontan procedures for tricuspid atresia, five of whom had preexisting Glenn shunts. The results were compared to 28 age- and sex-matched controls. Oxygen saturation was measured by ear oximetry at rest and after exercise. Ventilation, oxygen consumption (VO2), carbon dioxide production (VCO2), and heart rate were measured during progressive exercise. The ventilatory equivalents for oxygen (VE/VO2) and carbon dioxide (VE/VCO2), mixed expired pCO2 (PECO2) end-tidal pCO2 (PETCO2), and dead space to tidal volume ratio (VD/VT) were determined during steady state exercise on a cycle ergometer. Heart rate was higher for VO2 by 15% (p less than 0.02) and ventilation was higher for both VO2 (by 37%, p less than 0.001) and VCO2 (by 27%, p less than 0.002) in the patients than the controls. Mean VE/VO2 was 35.4 +/- 7.8 (SD) compared to 25.8 +/- 3.1 (p less than 0.001) and mean VE/VCO2 was 41.7 +/- 9.0 compared to 31.6 +/- 4.3 (p less than 0.001). Mean PECO2 was 21.4 +/- 4.4 torr with controls at 27.9 +/- 3.8 (p less than 0.001) and mean PETCO2 was 33.0 +/- 5.3 torr compared to 40.0 +/- 3.3 (p less than 0.001). The patients had a mean oxygen saturation of 92 +/- 5% at rest and abnormal saturation after exercise (87 +/- 9, p less than 0.005).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

Early and late results of the modified Fontan procedure for double-inlet left ventricle: the Mayo Clinic experience.

Between May 1974 and March 1989, 155 patients with double-inlet left ventricle had the Fontan procedure performed at the Mayo Clinic. Age at operation ranged from nearly 2 to 41 years (median 10). The operative mortality rate from 1974 through 1980 (39 patients) was 21%, but from 1981 through 1989 (116 patients) it was reduced to 9%. The 17 late deaths were secondary to reoperation (n = 8), progressive myocardial failure (n = 5), sudden arrhythmia (n = 3) and bleeding varices (n = 1). Neither operative nor late mortality rate was significantly related to age at operation. At follow-up of 6 months to 11 years (mean 4.9 years) in 111 patients, 88% were in good or excellent condition and 12% were in fair or poor condition. The Fontan operation can be performed with a mortality risk of less than 10% in properly selected patients with double-inlet left ventricle. Late results are encouraging when contrasted with the clinical course of patients before this operative approach was utilized.

Academic Medical Centers↗
PubMed1985

Response to the Valsalva maneuver after the Fontan procedure for tricuspid valve atresia, single ventricle or pulmonic valve atresia.

The Valsalva maneuver was performed during cardiac catheterization in 9 patients who had undergone a Fontan operation (Fontan group) and in 10 control subjects. The Fontan group had higher right atrial (RA) pressure (16 +/- 1 vs 4 +/- 0.5 mm Hg, p less than 0.001), lower cardiac index (2.5 +/- 0.1 vs 3.7 +/- 0.2 liters/min/m2, p less than 0.05), lower stroke index (32 +/- 2 vs 44 +/- 2 ml/beat/m2, p less than 0.05), and higher systemic vascular resistance (31 +/- 1 vs 24 +/- 1 units, p less than 0.05) than the control group. Four patients in the Fontan group had a normal 4-phase Valsalva response, and 5 did not (p less than 0.05). RA pressure was similar in those who responded normally and those who did not (16 +/- 0.5 vs 17 +/- 1 mm Hg), whereas in those who responded normally cardiac index was higher (2.9 +/- 0.2 vs 2.2 +/- 0.1 liters/min/m2, p less than 0.05), stroke index was higher (36 +/- 1 vs 28 +/- 1 ml/beat/m2, p less than 0.05), and systemic vascular resistance was lower (28 +/- 1 vs 31 +/- 1, p less than 0.05). It is concluded that cardiac output is a better predictor of a normal response to the Valsalva maneuver than RA pressure in patients after the Fontan procedure. A normal response to the supine Valsalva maneuver suggests a cardiac index greater than 2.4 liters/min/m2 and stroke index greater than 31 ml/beat/m2.

Aorta↗
PubMed2006

Improving results of the Fontan procedure in patients with heterotaxy syndrome.

BACKGROUND: The Fontan operation in patients with heterotaxy syndrome has been associated with a poor prognosis. We studied whether the outcome of those patients with heterotaxy syndrome improved compared with those who did not have the syndrome after an extracardiac Fontan operation. METHODS: A retrospective review was performed on 185 patients who had undergone an extracardiac Fontan operation between 1996 and 2005. RESULTS: Sixty-two of the patients had heterotaxy syndrome (33.5%). Forty-one had right isomerism and 21 had left isomerism. Heterotaxy syndrome was commonly associated with a morphologic right ventricle (59.7%), a common atrioventricular valve (72.6%), an interrupted inferior vena cava (25.8%), a separate hepatic vein (30.6%), and extracardiac pulmonary venous drainage (16.1%). The hospital mortality rate was higher in the heterotaxy syndrome than nonheterotaxy (4.8% vs 2.4%; p = 0.05). Eight-year survivals were 91.9 +/- 3.2% in the nonheterotaxy group and 89.3 +/- 4.2 % in the heterotaxy group (p = 0.39). At 8 years, freedom from reoperation was 90.2 +/- 3.2% in the nonheterotaxy group and 78.5 +/- 6.2% in the heterotaxy group (p = 0.15). The outcomes (other than those of arrhythmia) were no different between the two groups. The incidences of early and late postoperative arrhythmia were 29.0% and 25.4%, respectively, in heterotaxy patients, and 15.4% and 10.8% in nonheterotaxy patients (p < 0.05). Bradyarrhythmia was found to be more common. During follow-up, atrioventricular valve regurgitation of more than mild was more common in heterotaxy patients (33.9% vs 18.9%; p = 0.05). CONCLUSIONS: Midterm outcomes after an extracardiac Fontan operation in heterotaxy and nonheterotaxy patients are similar, except arrhythmia and atrioventricular valve regurgitation.

Arrhythmias, Cardiac↗
PubMed1999

Valvular repair for atrioventricular regurgitation in complex anomalies in modified Fontan procedure with reference to a single ventricle associated with a common atrioventricular valve.

Between January 1985 and October 1998, 169 of 372 patients who underwent a modified Fontan procedure had atrioventricular valve regurgitation (AVVR) that ranged in degrees from 1 to 4. Concomitant repair for AVVR was performed in the majority of cases. All but 12 patients had the AV valve repaired, mainly by circular annuloplasty; none had valve replacement. Although mortality was significantly higher in the AVVR cases (21 of 169 [12%]) than in the cases without AVVR (eight of 203 [3.9%]; P <.007, chi-square), actuarial survival in the AVVR cases was 83% at 5 years, 81% at 10 years, and 73% at 12 years. The degree of AVVR before surgery was 1.62 +/- 0.73 on average; 82 cases had more than grade 2 regurgitation. There was a significant decrease to 0.54 +/- 0.61 (P <.0001) after surgery in long-term survivors. Cases with AVVR can be treated with reasonable risk provided proper repair of the valve is performed. Circular annuloplasty is a simple and uniformly effective method with which to control regurgitation, even for the common AV valve. Copyright 1999 by W.B. Saunders Company

Journal Article↗
PubMed1985

Pulmonary artery thrombosis associated with anomalous pulmonary venous connection: an unusual complication following the modified Fontan procedure.

The presence of unrecognized partial anomalous pulmonary venous connection may cause complications of pulmonary infarction and right-to-left shunting in patients undergoing the modified Fontan procedure. These complications can be prevented by identification of any anomalous pulmonary venous connection at preoperative cardiac catheterization.

Adult↗
PubMed1984

Hemodynamic response to positive end-expiratory pressure following right atrium-pulmonary artery bypass (Fontan procedure).

Thirteen patients were studied in the early postoperative period to determine the hemodynamic response to increasing levels of positive end-expiratory pressure (PEEP) following right atrium-pulmonary artery bypass (Fontan procedure). Hemodynamic data and arterial oxygen and carbon dioxide tensions were measured without PEEP and with PEEP = 3, 6, 9, and 12 cm H2O. Cardiac index decreased progressively with increasing levels of PEEP compared to PEEP = 0 (cardiac index = 2.7 +/- 1.2 L/min/m2), and the decrease was significant at PEEP = 9 (cardiac index = 2.2 +/- 0.8 L/min/m2, p less than 0.05) and 12 cm H2O (cardiac index = 2.0 +/- 0.7 L/min/m2, p less than 0.05). Both arterial oxygen tension and pulmonary vascular resistance index increased significantly at all levels of PEEP studied compared to PEEP = 0. Significant positive trends were demonstrated for arterial oxygen tension and pulmonary vascular resistance index and a significant negative trend was shown for cardiac index with increasing PEEP. Heart rate, right atrial pressure, left atrial pressure, mean arterial blood pressure, and arterial carbon dioxide tension did not change significantly nor consistently with increasing PEEP. From these data it appears that PEEP is an effective means of raising arterial oxygen tension after right atrium-pulmonary artery bypass. A progressive fall in cardiac index occurs with increasing PEEP, and the fall becomes significant at PEEP greater than 6 cm H2O. The fall in cardiac index appears to be mediated by a significant rise in pulmonary vascular resistance index.

Adolescent↗
PubMed1984

Surgical repair of cyanotic cardiac defects in young adults. The Fontan procedure and use of the extracardiac valved conduit.

Improvement in the health status of young adults with complex cyanotic congenital cardiac defects has been realized with recent advances in cardiovascular surgical techniques. The Fontan procedure and its modifications have allowed physiologic correction of tricuspid atresia by separating the pulmonary and systemic circulations and creating a direct connection from right atrium to pulmonary artery. The extracardiac valved conduit has been successfully used in the repair of pulmonary atresia with ventricular septal defect, and the triad of transposition of the great vessels, ventricular septal defect, and pulmonary stenosis. Nursing implications for the care of these patients in the postoperative period and their long-term management has been addressed. Advances in surgical techniques have brought these anomalies into the realm of surgical repair and have increased the life expectancy of these patients, and thus more patients with these cardiac defects will be seen in adult cardiology settings.

Adult↗
PubMed1994

Regional ventricular wall motion abnormalities in tricuspid atresia after the Fontan procedure: flawed methodology may lead to a spurious finding of hypokinesia.

We believe that the two-frame method described by Akagi et al. cannot adequately describe the highly abnormal wall motion characteristics of these post-Fontan ventricles, and the systolic hypokinesia they describe may be spurious. Our data show that the predominant abnormality is incoordinate relaxation of the ventricular wall, which in turn prolongs the time constant of relaxation and the isovolumetric relaxation time and leads to reduced early rapid filling. Indeed, it was these abnormalities of diastolic, not systolic, function that were the strongest predictor of poor exercise performance in our study of patients late after the Fontan procedure. We strongly believe that the analysis of ventricular wall motion requires sequential data throughout the cardiac cycle, with well defined reference points concerning the timing of cardiac events, so that misinterpretation can be avoided.

Heart↗
PubMed1990

Early diastolic clicks after the Fontan procedure for double inlet left ventricle: anatomical and physiological correlates.

M mode echocardiograms and simultaneous phonocardiograms were recorded in four patients with early diastolic clicks on auscultation. All had double inlet left ventricle and had undergone the Fontan procedure with closure of the right atrioventricular valve orifice by an artificial patch. The phonocardiogram confirmed a high frequency sound occurring 60-90 ms after aortic valve closure and coinciding with the time of maximal excursion of the atrioventricular valve patch towards the ventricular mass. One patient had coexisting congenital complete heart block. The M mode echocardiogram showed "reversed" motion of the patch towards the right atrium during atrial contraction. Doppler flow studies showed that coincident with this motion there was forward flow in the pulmonary artery with augmentation when atrial contraction coincided with ventricular systole. The early diastolic click in these patients was explained by abrupt cessation of the motion of the atrioventricular valve patch towards the ventricular mass in early diastole. In one patient atrial contraction led to a reversal of this motion and was associated with forward flow in the pulmonary artery.

Adolescent↗
PubMed1991

Transesophageal echocardiography in evaluation and management after a Fontan procedure.

Transesophageal echocardiography was used in 18 patients (aged 1.6 to 34 years, mean age 12.6) to assess the immediate (5 patients) or intermediate (13 patients) results after a Fontan-type procedure. The findings were correlated with precordial echocardiographic (all patients) and cardiac catheterization (11 patients) data. Atrial shunting was documented by transesophageal studies in three patients (precordial in one patient). In two patients it was confirmed by cardiac catheterization; the third underwent reoperation based on the transesophageal study alone. Pulmonary artery obstruction was documented in three patients (precordial in one patient) and was confirmed by subsequent cardiac catheterization in all. Evaluation of anterior Fontan connections was successful in 5 of 8 patients (precordial in 6 of 8), and posterior connections in 10 of 10 patients (precordial in 5 of 10). A Glenn shunt could be evaluated in eight of nine patients (precordial in three of nine). Thrombus formation was detected by transesophageal studies in three patients (precordial in one patient); repeat studies were used to evaluate thrombolytic therapy in two. Atrioventricular valvular regurgitation (11 of 18 patients) was better defined by transesophageal than by precordial studies (5 of 18). A coronary artery fistula was identified in two cases (precordial in none). Transesophageal pulsed Doppler interrogation of pulmonary artery and pulmonary vein flow patterns consistently allowed a detailed evaluation of the Fontan circulation. Transesophageal echocardiography is an important diagnostic and monitoring technique after the Fontan procedure. In this series, it was far superior to precordial ultrasound evaluation and of substantial additional value to cardiac catheterization.

Adolescent↗
PubMed1992

Pre-operative assessment of ventricular function in patients considered for Fontan procedure.

In 1978 Choussat and Fontan established ten criteria, which should be fulfilled to achieve a successful outcome in Fontan operation. Recent data suggest that while some of these ten criteria need not be necessarrily be fulfilled, new criteria should be added. These include 1. good diastolic function, 2. normal or only slightly increased ventricular mass, and 3. absence of systemic outflow obstruction. In addition the morphology of the single ventricle may be important as long-term results in patients with single ventricle of right ventricular morphology may be worse than results in patients with single ventricle of left ventricular morphology. Ventricular size and pump function can be assessed by cardiac catherization, echocardiography or magnetic resonance imaging. Estimation of ejection fraction under stress by nuclear angiography may be indicated. Diastolic function can be examined using Doppler echocardiography or nuclear angiography. Myocardial mass may be assessed by echocardiography or magnetic resonance imaging. Normal reference values for different parameters of systolic and diastolic function are listed in the enclosed tables. Patients scheduled for a Fontan operation should have an ejection fraction less than 50%. Patients with borderline ejection fraction should be examined by echocardiography to determine the end-systolic wall stress, a parameter of ventricular contraction, which is independent of pre- and afterload. As afterload may decrease after a Fontan operation some patients with reduced ejection fraction but normal end-systolic wall stress may still be suitable candidates for Fontan operation.(ABSTRACT TRUNCATED AT 250 WORDS)

Heart Defects, Congenital↗
PubMed1991

[Fontan procedure for DORV with mitral atresia and anomalous hepatic vein connection to the left atrium--advantage of leaving right to left shunt in situ].

A 5-year-old boy with double outlet right ventricle, mitral atresia, pulmonary stenosis and anomalous hepatic vein drainage into left atrium underwent Fontan procedure leaving hepatic venous-left atrial connection. Systemic venous-left atrial connection provided enough filling volume of the systemic ventricle despite of high pulmonary resistance. Extra cardiac shunt between the systemic vein and left atrium would be the second best in fontan operation for cases with high pulmonary resistance.

Abnormalities, Multiple↗
PubMed1988

[Assessment of pulmonary blood flow after total cavopulmonary shunt operation and the modified Fontan procedure for univentricular heart].

Pulsed Doppler echocardiography was utilized to elucidate the characteristics of pulmonary arterial (PA) blood flow in five patients without apparent pumping chambers in their right heart circulation after right heart bypass surgery for univentricular heart. Two of these patients underwent total cavopulmonary shunt operation, in which the total systemic venous return drained directly into the PA, bypassing the right atrium and ventricle. Three underwent the modified Fontan procedure with atrial partition, in which the right-sided atrium was reconstructed merely as a pathway from the vena cava to the PA, and atrial contraction was nearly entirely excluded. The flow pattern in the PA was biphasic and forward in all five patients. Pulmonary regurgitation was not observed in any of the patients. The first phase of PA flow had its peak during atrial systole; the second, during ventricular systole. Simultaneous observation of PA flow and pressures demonstrated an inverse relation between PA flow and pressure. Pulmonary venous (PV) blood flow pattern was also biphasic and similar to the PA blood flow pattern with time lags. In conclusion, in cases without pumping right heart chambers, PA flow reflects PV flow resulting from contraction and relaxation of the left atrium and ventricle.

Adult↗
PubMed1992

Modified Fontan procedure for severe Ebstein's malformation with predominant tricuspid stenosis.

Two children, aged 7 and 6 years, had severe Ebstein's malformation of the tricuspid valve and underwent operation because of progressive cyanosis (saturation, 70%). Preoperative angiography demonstrated linear attachment of the distal edges of the displaced anterosuperior and mural leaflets, leaving only a "keyhole" communication between the atrialized and functional portions of the right ventricle. Operation was directed toward completely excising these dysplastic leaflets, which were obstructing the flow of blood. The continuity between the functional right ventricle and pulmonary trunk was left intact. In addition, an atriopulmonary truncal connection was established. Postoperatively, both children were in functional class I with arterial saturation of 95%. Angiography and Doppler echocardiography demonstrated that there was a dominant phase of systolic flow of blood up the pulmonary trunk from the right ventricle, and also diastolic filling of the pulmonary arteries through the atriopulmonary truncal connection. The filling patterns did not change over time for the follow-up period of 4 and 1.5 years, respectively. This modified Fontan procedure appears to be an effective surgical alternative for some patients with severe Ebstein's malformation and predominant tricuspid stenosis.

Child↗
PubMedDate not supplied

Cardiac catheterization is necessary before bidirectional Glenn and Fontan procedures in single ventricle physiology.

There are significant mortality and morbidity in bidirectional Glenn (stage II) and Fontan (stage III) procedures for congenital heart diseases with single ventricle physiology. In order to minimize the mortality and morbidity, the presence or absence of hemodynamic and anatomical abnormalities, such as poor ventricular function, coarctation of the aorta, pulmonary artery distortion, high pulmonary resistance, and abnormal collateral vessels, should be evaluated. Echocardiography and magnetic resonance imaging (MRI) can visualize ventricular size and coarctation of the aorta but may fail to visualize pulmonary artery distortion. Furthermore, cardiac catheterization is needed to measure pulmonary resistance. If the pulmonary resistance is > 3 Wood units/m2 before stage II, the risk for the bidirectional Glenn operation may be high. Abnormal aortopulmonary collateral vessels may develop after the stage II procedure and echocardiography and MRI may not visualize these vessels. Coil embolization may be required to treat these vessels. In conclusion, cardiac catheterization is required to evaluate abnormalities, which may be treated by interventional catheterization, and to reduce mortality and morbidity of stage II and III procedures.

Cardiac Catheterization↗
PubMedDate not supplied

Fontan procedure for univentricular hearts: have changes in design improved outcome?

The Fontan operation has undergone numerous design changes in the last 30 years, which have resulted in an improved morbidity and mortality of patients with a univentricular heart. However, good patient selection and a staged approach to the Fontan operation have also contributed greatly to a better outcome in these patients.

Arrhythmias, Cardiac↗