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PubMed2005

Performance of the Fontan procedure using extra-cardiac direct anastomosis on patients treated for penicillin-resistant Streptococcus pneumoniae endocarditis.

Streptococcus pneumoniae is now a rare cause of endocarditis in humans. We report a patient with a double outlet right ventricle and mitral atresia, who underwent the Fontan procedure without prosthetic materials after treatment for penicillin-resistant Streptococcus pneumoniae endocarditis. Postoperative infectious endocarditis was not found. In patients with a history of infectious endocarditis, direct anastomosis of the main pulmonary artery and inferior vena cava would reduce the risk of recurrent infectious endocarditis.

Abnormalities, Multiple↗
PubMed2000

Pharmacokinetic evaluation of triiodothyronine supplementation in children after modified Fontan procedure.

BACKGROUND: Triiodothyronine (T(3)) supplementation may be a useful adjunct in the management of patients after cardiopulmonary bypass. Limited data are available regarding the use and pharmacokinetics of T(3) in children. The present study was performed to evaluate T(3) pharmacokinetics in a cohort of children undergoing the modified Fontan procedure. METHODS AND RESULTS: A total of 28 patients were enrolled in this randomized, prospective study. The patients were divided into 4 groups: 1 group received a placebo and 3 groups received intravenous T(3) at dosages of 0.4, 0.6, and 0.8 microg/kg, respectively. All 28 patients survived their operative procedures. Two patients developed low cardiac output, and 3 patients had pleural effusions. The median length of hospital stay was 7 days. The mean free T(3) level was 316+/-67 pg/dL after then administration of a placebo. Patients who received T(3) had mean peak free T(3) levels of 972+/-88, 1351+/-299, and 1869+/-281 pg/dL for the dosages of 0.4, 0.6, and 0.8 microg/kg, respectively. The calculated half-life of T(3) was 7 hours. CONCLUSIONS: The half-life of intravenous T(3) in children is approximately one-third of that reported for adults. These results provide a framework for studying the efficacy of T(3) supplementation in children undergoing open-heart surgery.

Cardiac Output↗
PubMed1982

The hemodynamic response to dopamine and nitroprusside following right atrium-pulmonary artery bypass (Fontan procedure).

Cardiac output is critically dependent upon pulmonary vascular resistance after right atrium-pulmonary artery bypass (Fontan procedure), since there is no pulmonary ventricle in the circulation. Inotropic agents, including dopamine, may increase pulmonary vascular resistance and, therefore, might have an adverse effect on cardiac output. The present study determined the hemodynamic responses to dopamine and nitroprusside of 9 patients following right atrium-pulmonary artery bypass. Particular attention was given to effects on cardiac output (CI), pulmonary vascular resistance, and right atrial pressure (RAP). Baseline hemodynamic data were measured without drugs, with dopamine at 7.5 micrograms/kg/min, with sodium nitroprusside up to 5.0 micrograms/kg/min, and with a combination of dopamine, 7.5 micrograms/kg/min, and sodium nitroprusside, 1.0 micrograms/kg/min. Right and left atrial pressures (LAP), mean arterial blood pressure (BP), heart rate (HR), and CI were measured. Stroke volume index and pulmonary arteriolar resistance index were calculated. The increase in CI from baseline (1.98 +/- 0.86 liters per minute) was significant for infusions of dopamine (2.75 +/- 1.05, p less than 0.001), sodium nitroprusside (2.57 +/- 0.78, p less than 0.001), and both drugs (2.74 +/- 0.84, p less than 0.001). The increased CI was achieved primarily by a significant increase in HR with dopamine and by an increase in stroke volume index with sodium nitroprusside. With a similar increment in CI, the RAP was significantly decreased from baseline (21 +/- 4 torr) with sodium nitroprusside (15 +/- 3, p less than 0.001) but was unchanged with dopamine. Pulmonary arteriolar resistance index decreased significantly from baseline (375 +/- 230 dynes sec cm-5/m2) with sodium nitroprusside (169 +/- 132, p less than 0.001), and, interestingly, with dopamine as well (273 +/- 165, p less than 0.05). Both dopamine and sodium nitroprusside in these dosages have favorable effects on CI and pulmonary arteriolar resistance index in patients after right atrium-pulmonary artery bypass. Whenever feasible, sodium nitroprusside is preferred for increasing CI after such a bypass procedure, since lower RAP decreases the severity of fluid retention, ascites, and chest tube drainage.

Adolescent↗
PubMed1988

Modified Fontan procedure: atrial baffle and systemic venous to pulmonary artery anastomotic techniques.

Two technical modifications to the modified Fontan procedure are presented. Systemic venous to pulmonary artery continuity is achieved by superior vena cava (SVC) division and end to side anastomosis to the right pulmonary artery (RPA), particularly following a right Blalock shunt, or by RPA division and anastomosis to the SVC, particularly in the presence of RPA stenosis. Intra-atrial partitioning is achieved by a systemic venous baffle rather than a pulmonary venous baffle. This is particularly useful in the presence of left atrioventricular valve atresia, but may be a preferable technique with double-inlet single ventricle or single ventricle with common AV valve. These techniques were applied successfully to 10 of 12 children with various forms of single ventricle, including 5 with left AV valve atresia or stenosis.

Adult↗
PubMed1993

Regional ventricular wall motion abnormalities in tricuspid atresia after the Fontan procedure.

OBJECTIVES: The purpose of this study was to determine whether wall motion abnormalities are present before or after the Fontan procedure in patients with a univentricular heart of the left ventricular type with an absent right atrioventricular valve connection (tricuspid atresia) and to assess the impact of such abnormalities on ventricular performance and clinical outcome. BACKGROUND: Normal systolic and diastolic ventricular function is critical for a successful Fontan repair. However, there have been no previous studies addressing the relation between regional ventricular function and hemodynamic factors. METHODS: Thirty-seven pediatric patients were studied with biplane ventricular cineangiography. There were 20 male and 17 female patients whose mean age at the time of the Fontan operation was 6.5 +/- 3.5 years (range 2.5 to 15.6). Eighteen patients were studied preoperatively, 25 at > 1 year postoperatively and 6 serially. Wall motion was assessed by a centerline method. Normal ranges for wall motion and other variables were established from 25 normal subjects. RESULTS: Wall motion abnormalities were observed in 2 of 18 patients preoperatively and in 11 of 25 patients postoperatively. Age at operation and ventricular volumes did not differ between postoperative patients who had normal (group I, 14 patients) or abnormal (group II, 11 patients) wall motion. However, ventricular mass and the mass/volume ratio were significantly greater and systolic variables and cardiac index were significantly lower in group II versus group I. Two patients in group I were considered to have a clinically poor outcome (persistent heart failure), and five in group II had heart failure, including one who died late. CONCLUSIONS: These observations suggest that postoperative regional wall motion abnormalities in this setting are not rare, may be related to excessive hypertrophy and may contribute to cardiac dysfunction and a poor clinical outcome.

Adolescent↗
PubMed1989

Relation between preoperative left ventricular muscle mass and outcome of the Fontan procedure in patients with tricuspid atresia.

The relation between preoperative left ventricular muscle mass and clinical outcome of the Fontan procedure was evaluated retrospectively in 22 patients with tricuspid atresia who were selected for this physiologic surgical correction by conventional hemodynamic criteria. Patients were divided into two groups: group A (excellent or good outcome) and group B (poor outcome or death) based on the clinical course assessed up to 9.5 years postoperatively. Thirteen of 22 group A patients did not have prolonged, clinically significant, systemic venous hypertension and were not on long-term diuretic drug therapy. Nine of 22 group B patients either had clinically significant systemic venous hypertension, required long-term diuretic drug therapy or died (3 patients). Age at surgery, pulmonary arteriolar resistance, left ventricular ejection fraction, end-diastolic volume, end-diastolic pressure, systemic oxygen saturation and pulmonary to systemic blood flow ratio (Qp/Qs) were not statistically different between the two groups. Left ventricular muscle mass, both in group A patients (92 +/- 31 g/m2) and in group B patients (146 +/- 61 g/m2), was greater than the normal mean value (p less than 0.01 and p less than 0.001, respectively). Left ventricular muscle mass in group B was significantly greater than in group A (p less than 0.01). Furthermore, left ventricular muscle mass/end-diastolic volume (mass/volume) ratio, reflecting the extent of left ventricular hypertrophy relative to volume overload, was significantly greater in group B (1.1 +/- 0.28) than in group A (0.84 +/- 0.21) (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

The outcome of antibiotic sterilised aortic homografts used in the Fontan procedure.

Between 1977 and 1988, 27 patients between the ages of 4 and 22 years (mean 8.9) underwent a Fontan procedure with the use of an antibiotic sterilised aortic homograft. There were 15 patients with tricuspid atresia, 9 with univentricular heart and 3 others. The homograft was anastomosed to a right-sided pulmonary artery in ten, to a left-sided main pulmonary artery in eight and in nine patients the homograft connected the right atrium to the right ventricle. There were five early deaths (18.5%) unrelated to the homograft and two late deaths at 7 and 10 years postoperatively. Five patients have required removal of the calcified obstructed homograft with no death. In three patients patches were inserted, but in two patients with good right ventricles a second homograft was inserted. In three of the re-operated patients the homograft lay directly behind the sternum and the femoral artery was exposed and in two of them the femoral artery was cannulated before the chest was opened to control haemorrhage. Of the patients 74% are alive up to 15 years later, 15 with their original homograft. Eight (57%) of those still have their original homograft more than 10 years post-operatively.

Adolescent↗
PubMed2004

Altered dystrophin expression in the right atrium of a patient after Fontan procedure with atrial flutter.

Underlying mechanisms in the development of atrial flutter or intra-atrial re-entry tachycardia in patients with structural cardiac abnormalities remain poorly defined. The right atrial myocardium from two patients with congenital heart disease was evaluated, of whom one presented with severe right atrial dilation and arrhythmia and the other with a normal right atrium, to assess whether increased right atrial pressure and volume overload give rise to sarcolemmal alteration. N-terminus dystrophin staining in the atrium from the patient who had undergone a Fontan procedure showed a normally distributed but significantly reduced staining signal compared with the second patient. This is the first report that patients with severe right atrial dilation and atrial flutter have marked reduction in atrial dystrophin expression.

Atrial Flutter↗
PubMed1981

Separation of neointima from Dacron graft causing obstruction. Case following Fontan procedure for tricuspid atresia.

A nine-year-old patient with tricuspid atresia and normally related great vessels underwent a Fontan procedure, in which a porcine valved Dacron conduit was interposed between the right atrium and pulmonary trunk. After death, 6 months postoperatively, pathological examination revealed a thick layer of neointima (peel) lining the conduit. The peel was thicker in the segment of the conduit proximal to the valve, where it measured up to 4.5 mm in thickness. Proximal to the valve, the peel had separated from the wall of the graft and a hematoma had developed between the separated peel and graft. The result was reduction in the caliber of the conduit to about half the original. The patient's final illness was complicated, being associated with acute pancreatitis. Reduction in the caliber of the graft may have contributed to the death.

Acute Disease↗
PubMed2005

[Criss-cross heart: anesthetic considerations for total cavopulmonary shunt (Fontan procedure)].

We report the anesthetic technique used for interatrial septal defect a 2-year-old girl weighing 11 Kg who presented with crossed atrioventricular connection (criss-cross heart), transposition of the great vessels, interatrial and interventricular septal defects, and subpulmonary and pulmonary valve stenoses. The patient was proposed for total cavopulmonary anastomosis with basal arterial oxygen saturation (SapO2) at 65%. Anesthesia was induced with inhaled agents and after intubation, hyperventilation was induced to achieve an end-tidal carbon dioxide pressure around 27 mm Hg. General anesthesia was provided in combination with spinal infusion of morphine chloride (100 microg Kg(-1)) for pain control. A Fontan procedure was carried out uneventfully with cardiopulmonary bypass. Milrinone was used at the extracorporeal circuit pump outlet. The patient was transferred to the pediatric intensive care unit where she was extubated without complications 90 minutes after admission. Anesthetic management is based on maintaining adequate preloading doses by administering volume, inhaled and/or intravenous agents, or use of vasoconstrictors and adjustment of ventilator parameters to modify pulmonary or systemic vascular resistance. We were able to maintain normal arterial pressure in our patient and provide adequate preloading through hyperventilation to reduce pulmonary vascular resistance.

Anesthesia↗
PubMed1995

Modified Fontan procedure for Ebstein's anomaly of the tricuspid valve--an alternative surgical approach preserving Ebstein's anatomy.

In young patients with Ebstein's anomaly and symptoms of congestive heart failure or severe cyanosis tricuspid valve repair may be unsuccessful and prosthetic valve replacement is needed, which is associated with a high operative mortality. This report describes the implantation of a valved aortic homograft as a conduit between right atrium and pulmonary artery to at least postpone valve surgery in three symptomatic children (4.1, 8.3, and 10 years of age) with an arterial oxygen saturation of 75 to 82%. The intracardiac anatomy was left untouched except for closure of the associated secundum atrial septal defect. One patient with stable postoperative hemodynamics died of severe endobronchial bleeding on the 2nd postoperative day. Due to the increase of antegrade pulmonary blood flow via the conduit the arterial oxygen saturation rose to 92% in the two surviving patients. Postoperative hemodynamic evaluation revealed low right atrial and mean pulmonary artery pressures (12 mmHg). Pulsed Doppler ultrasound sampling of flow in the conduit demonstrated antegrade flow during atrial and ventricular systole. In symptomatic patients with severe malformation of the tricuspid valve a partial modified Fontan procedure offers a promising palliation avoiding tricuspid valve surgery in early childhood.

Aortic Valve↗
PubMed1993

Influence of ventricular morphology on diastolic filling performance in double-inlet ventricle after the Fontan procedure.

OBJECTIVES: The purpose of this study was to define the influence of dominant chamber morphology on ventricular performance after the Fontan procedure in patients with double-inlet ventricle. BACKGROUND: Previous studies have reported the impact of ventricular morphology on preoperative ventricular performance and surgical outcome. However, the influence on postoperative ventricular performance has not been addressed. METHODS: Twenty-six clinically asymptomatic patients > 1 year after repair (mean age at procedure 6.1 +/- 3.7 years) were evaluated with ventricular cineangiography and radionuclide blood pool studies (18 with a dominant left ventricular morphology [LV group], 8 with a dominant right ventricular morphology [RV group]) and compared with normal control subjects. RESULTS: Ventricular volume, mass and systolic variables were similar between patient groups. In the LV group, however, the mass/volume ratio was significantly elevated compared with values in control subjects (1.11 +/- 0.28, 0.97 +/- 0.19, p < 0.05), whereas this ratio in the RV group (0.90 +/- 0.11) was within the normal range and significantly lower than that in the LV group (p < 0.05). Mean right atrial and pulmonary artery pressures in the RV group were significantly higher than those in the LV group (p < 0.05). Peak filling rates (2.87 +/- 0.70, 2.41 +/- 1.15 and 3.84 +/- 0.51 end-diastolic volume/s [LV and RV groups and control subjects, respectively]) were significantly lower in both groups than in control subjects (p < 0.001), without intergroup difference. CONCLUSIONS: Ventricular filling abnormalities after atrial to pulmonary anastomosis are common regardless of the type of dominant ventricular morphology, and these abnormalities in patients with dominant right ventricular morphology do not coexist with ventricular hypertrophy. Such diastolic abnormalities may be related to either intrinsic myocardial or acquired factors, not to excessive hypertrophy alone. Those differences may become clinically more apparent with longer follow-up and may raise concerns over the long-term course.

Cardiac Catheterization↗
PubMed1984

Radionuclide studies in postoperative evaluation of the Fontan procedure.

Radionuclide studies were performed on 12 patients who had had a Fontan operation for cyanotic congenital heart disease, six of whom had undergone a prior palliative Glenn procedure. The patients without prior Glenn anastomoses were studied by radionuclide first-pass angiocardiography, using a right antecubital vein injection of 99mTc pertechnetate. The patients with Glenn anastomoses required two injections, one by femoral vein to study the Fontan procedure, using bolus injection of 99mTc pertechnetate or microspheres, and the second by right antecubital vein to study the Glenn anastomosis and right lung, using a bolus of microspheres. Gated cardiac blood-pool scintigraphy was used to measure right atrial and left ventricular ejection fraction in three patients. In nine patients, contrast angiography confirmed that these techniques allowed recognition of residual right-to-left shunts, right atrial stasis, right atrial outflow obstructions, left ventricular dysfunction, and right lung arteriovenous fistulas. Noninvasive radionuclide methods seem to be dependable in the postoperative evaluation of patients after the Fontan procedure. First-pass angiocardiography is most helpful in evaluating the dynamics and distribution of blood flow, especially the right atrial output, and gated blood-pool scintigraphy offers a better evaluation of right atrial and left ventricular contraction, so both supply complementary information.

Adolescent↗
PubMed1983

[Total correction of complete transposition of the great arteries (d-TGA) and Fontan procedure for tricuspid atresia(TA)].

Since 1975, 87 patients underwent total correction consisting of 35 cases of Mustard procedure with 6 early deaths (17.1%), 36 Senning with 2 deaths (5.6%), 12 Rastelli operations with 4 deaths (33.3%), and Jatene procedure on 4 cases without mortality. Sixteen patients out of 63 early survivors following intraatrial repair resulted in fatal outcome. In 17 with a modified senning procedure with a pedicled autologous pericardial patch on the functional left atrium, 8 died of pulmonary venous obstruction caused by thickening and calcification of pericardium. However, no death was seen in 17 patients with original Senning procedure. Right ventricular function remained poor in cases with intra-arterial repair and accounted for relatively poor long-term results. Although Rastelli operation carried high early mortality, late death was seen only in 1. Jatene procedure was performed in 4 ranging in ages from 5 months to 6 years without mortality. Fontan procedure was done on 22 patients with 2 early deaths and no late death in tricuspid atresia. Average cardiac index after surgery was 2.6 +/- 0.6. Cardiac index showed close correlation with preoperative cross sectional area of both pulmonary artery divided by body surface area (PA-index), but had poor correlation with right atrial pressure or left ventricular volume.

Age Factors↗
PubMed1985

Radionuclide assessment of ventricular contraction at rest and during exercise following the Fontan procedure for either tricuspid atresia or single ventricle.

Ventricular contraction was evaluated in 18 patients studied at a mean of 2.6 years after Fontan repair. The diagnosis was tricuspid atresia in 9 patients and single ventricle in 9. Gated first-pass and gated equilibrium radionuclide ventriculography were performed at rest and during exercise. Abnormally low ventricular ejection fraction (EF) at rest was present in 8 of 18 patients by the gated equilibrium technique and 6 of 13 technically adequate gated first-pass studies. An abnormal response to exercise (failure of EF to increase less than or equal to 5% from rest to maximal exercise) was found in 10 of 16 patients by the gated equilibrium technique and in 8 of 12 by the gated first-pass technique. Only 2 patients by each radionuclide technique had both normal EF at rest and normal exercise response. Thus, this study confirms the frequent presence of abnormalities in ventricular contraction after the Fontan procedure at rest or during exercise or both despite absence of symptoms. Both EF response and the hemodynamic response during exercise were more abnormal in the presence of an atriopulmonary than an atrioventricular connection.

Adolescent↗