Fontan Operation For Tricuspid Atresia Research Articles

Fontan operation for tricuspid atresia with absent pulmonary valve: a case series.

Combined tricuspid atresia and absent pulmonary valve with dysplasia of the right ventricular myocardium is a very rare congenital heart anomaly with a poor prognosis. We present three cases of this rare disease that reached the Fontan operation without prior surgical intervention of the right ventricle or pulmonary artery; no right ventriclar dilation was detected. All patients had uneventful post-operative courses.

A Carpentier–Edwards porcine-valved dacron conduit: At twenty-five years

We report the longest duration of implantation of a porcine bioprosthesis in the English language literature — a Carpentier–Edwards valved conduit excised after twenty-five years. This 50-year-old man had undergone a modified Fontan operation for tricuspid atresia. He presented with progressive dyspnea, fatigue and edema. The bioprosthesis' dysfunction was due to the pathologic findings of pannus, significant thrombus (on the sinus surface of a cusp), mild structural valve deterioration and pseudo-intimal formation within the conduit. The bioprosthesis' longevity is likely related to low-pressure circulation in the right heart and to host tissue (pannus) overgrowth that covered the cusps and thereby reduced tissue degeneration.

Atrial pacing: An alternative treatment for protein-losing enteropathy after the Fontan operation

J Thorac Cardiovasc Surg 2001;121:582-3

Iatrogenic atrioventricular bypass tract following a Fontan operation for tricuspid atresia.

A 16 year old female with tricuspid atresia had undergone a Fontan operation at four years of age. Two years later she first presented with a narrow complex tachycardia which...

Acute changes in left ventricular geometry after volume reduction operation

After surgical removal of a volume load, regression of myocardial mass proceeds slowly relative to diminution in ventricular cavity size, resulting in increased wall thickness and decreased cavity dimensions, which may affect the filling properties and performance of the heart. We investigated the acute changes in ventricular geometry that occur after the Fontan operation and hemi-Fontan operation for tricuspid atresia, and compared them with closure of a ventricular septal defect in a two-ventricle heart. We reviewed the results of echocardiography performed before and 8 +/- 7 days after (1) Fontan operation for tricuspid atresia (n = 9), (2) hemi-Fontan operation for tricuspid atresia (n = 10), and (3) closure of a ventricular septal defect (n = 13). Measurements were made from images of the left ventricle at end-diastole: (1) apical, septal, and posterior wall thickness; and (2) long- and short-axis cavity diameters, cross-sectional areas, and ventricular volume. Posterior wall thickness to cavity dimension ratio was calculated. Wall thickness increased in all groups, with the greatest degree of increase after the Fontan operation. Cavity measures decreased most dramatically after the Fontan operation, with less dramatic and equivalent changes noted after the hemi-Fontan operation and ventricular septal defect closure. Posterior wall thickness to cavity diameter ratios were equivalent in all before operation, increased after operation, and were greatest after the Fontan operation. Changes in ventricular geometry identified as an increase in wall thickness and a decrease in cavity dimension are most dramatic after the Fontan operation. Changes seen after the hemi-Fontan operation are of a milder degree, which may in part explain the excellent clinical course after this operation.

Modified fontan operation with reconstruction of the nonconfluent pulmonary artery

A modification of the Fontan operation for tricuspid atresia with a nonconfluent pulmonary artery after a Glenn shunt is reported. Confluence was established posteriorly using an inverted right atrial flap and anteriorly with a xenopericardial patch.

Long-term results after the Fontan operation for tricuspid atresia.

Between 1968 and 1977, 32 patients who underwent the Fontan operation for tricuspid atresia at Bordeaux, France, or Leiden, The Netherlands, survived at least 1 year after surgery. These patients were evaluated clinically 7 to 16 years (mean 8.9 years) after surgery by review of clinical records, questionnaire, or direct examination. There were five deaths: one during arrhythmia, the second sudden and unexplained, and three others after reoperation. Four of the five patients had evidence of obstruction of the atriopulmonary or atrioventricular conduit. There were eight reoperations. Two were for residual atrial septal defects, one for an atrial septal defect and an intrapulmonary arteriovenous fistula, and one for a residual ventricular septal defect. The other four were reoperated for severe conduit or homograft obstruction. One of these four also had a residual shunt, severe mitral regurgitation, and reduced left ventricular function. One patient was found to have left ventricular dysfunction 3 months after surgery, which persisted. Another patient had left ventricular dysfunction unmasked after successful mitral annuloplasty for severe mitral regurgitation. No other patients had clinical evidence of left ventricular dysfunction. Recurrent supraventricular tachycardia occurred in four patients and was eventually fatal in one patient with associated conduit obstruction. Of the 27 survivors, 13 are in NYHA class I, 13 are in class II, and one is in class III. We conclude that clinical results of the Fontan operation remain good in most patients 7 to 16 years after surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

A case of protein-losing enteropathy after Fontan operation for tricuspid atresia

A case of protein-losing enteropathy after Fontan operation for tricuspid atresia

Exercise tolerance and cardiorespiratory response to exercise after the fontan operation for tricuspid atresia or functional single ventricle

To determine the impact of the Fontan operation on exercise tolerance and on the cardiorespiratory response to exercise, we compared the results of graded exercise to maximal effort of 81 patients with tricuspid atresia or single functional ventricle studied preoperatively with those of 29 patients studied postoperatively. Postoperatively, the values for total work performed, duration of exercise and maximal oxygen uptake increased significantly. Regardless of operative status, the maximal heart rate during exercise was reduced. The cardiac output and stroke volume response to exercise were subnormal after operation. Systemic arterial blood oxygen saturation was reduced markedly preoperatively both at rest and during exercise; postoperatively, it was significantly greater than the preoperative value but it remained slightly abnormal. The ventilatory response to exercise (respiratory rate, minute ventilation and ventilatory equivalent for oxygen) decreased toward normal after operation. Exercise tolerance and the cardiorespiratory responses to exercise improve after the Fontan operation. Formal exercise testing is essential to quantitate the degree of improvement.

Ｆｏｎｔａｎおよびその類似手術 三せん弁閉鎖と他の複雑心奇形との比較検討

Ｆｏｎｔａｎおよびその類似手術 三せん弁閉鎖と他の複雑心奇形との比較検討

Liver fibrosis (cardiac cirrhosis) five years after modified Fontan operation for tricuspid atresia

A 15-year-old girl was found to have severe liver fibrosis on liver biopsy at the time of cholecystectomy, 5 1/2 years following a modified Fontan procedure (right atrial-right ventricular conduit) for tricuspid atresia. Postoperative right atrial pressures were consistently elevated above 13 mm Hg and this, in part, may have been due to progressive mild conduit stenosis. Because of increasing symptoms, the patient underwent successful revision of the conduit at the age of 15 years. It is suggested that sustained systemic venous hypertension caused the striking morphologic changes in the liver and that this serious complication may significantly affect the long-term prognosis of patients surviving the Fontan procedure.

Clinical and hemodynamic results of the Fontan operation for tricuspid atresia

Thirty patients aged 3 months to 26 years (median 9 years) with tricuspid atresia or severe tricuspid stenosis underwent a modified Fontan operation at Children's Hospital, Boston, between 1973 and 1980. Thirty-six palliative operations had been performed previously in 20 patients. Preoperative hemodynamic measurements included: mean pulmonary arterial pressure 6 to 18 mm Hg (median 9); pulmonary vascular resistance 0.3 to 2.2 Woods units/m 2 (median 1.5) and left ventricular end-diastolic pressure 3 to 14 mm Hg (median 7). There were five hospital deaths (17 percent), including two in the last 24 operations. The 25 survivors have been followed up for 4 to 85 months (median 23) with no late deaths. Twenty patients are in New York Heart Association class I and four in class II; one infant was not classified. Two patients have required reoperation, at, respectively, 71 and 26 months postoperatively; the former for late-developing conduit obstruction and residual bidirectional shunting; and the latter for a residual right to left shunt at atrial level. Early postoperative monitoring of hemodynamics in the intensive care unit revealed that a mean right atrial pressure of 17 mm Hg or greater, without obstruction of the anastomosis, was invariably associated with serious morbidity or mortality. Fifteen patients underwent elective cardiac catheterization 9 to 21 months (median 13) postoperatively. The mean right atrial pressure ranged between 5 and 12 mm Hg (median 8) and mean pulmonary arterial pressure between 4 and 11 mm Hg (median 7). The cardiac index (measured in 12 patients) was 1.9 to 4.0 liters/min per m 2 (median 3.2). The left ventricular ejection fraction measured in 11 patients did not change significantly postoperatively, remaining normal in 10. Three patients had a residual right to left shunt at atrial level while the arterial oxygen saturation in the remaining 12 patients was 93 to 97 percent. Thus the modified Fontan operation can be performed with excellent clinical and hemodynamic results in selected patients with tricuspid atresia.

179 POST-OPERATIVE ASSESSMENT OF FONTAN OPERATION

From 1975 to 1980, 10 patients (pts.) age 2.5-24 years (y)(mean 13.6±6y) had modified Fontan operation for tricuspid atresia (7), univentricular heart (2), and transposed great vessels (1). Six had right atrial (RA) to pulmonary artery (PA) anastomosis (ANS), 4 with valved conduit and 2 direct ANS. Four had RA to right ventricular (RV) ANS, 1 with a non-valved conduit and 3 direct ANS. Eight pts. also had Glenn shunts. All 9 survivors are in normal sinus rhythm. Seven pts. had cardiac catheterization 2-60 months (m) post-operatively (post-op)(median 8m). RA mean pressures (p) were 3-19mmHg with RA ‘a’ waves of 10-25mmHg. Pts. with RA-PA ANS had narrow PA pulse p of 2-3mmHg, while pts. with RA-RV ANS had wider, biphasic PA pulse p due to RV contractions. No RA-PA p gradients were present. Aortic saturation was 79%±9 pre-operatively (pre-op) and 95%±3 post-op; left ventricular (LV) end diastolic p was 7.0±5.8mmHg pre-op and 6.7±1.9 post-op. RA regurgitation into hepatic veins and coronary sinus was more marked in pts. with RA-PA ANS. Post-op LV contractility was decreased in all (average LV ejection fraction 53.2%±12) except the youngest pt. While all pts. had improved exercise tolerance post-op, treadmill testing in 5 was impaired with average duration of 6.9 minutes, and maximal endurance index of 238±49 (normal 344±65). These data suggest that RA-RV ANS results in better forward flow and less regurgitation than RA-PA ANS. Exercise tolerance and oxygenation improved post-op but most pts. have clinical and angiographic evidence of decreased cardiac function.