• Journal of Chest Surgery
• /
• v.30 no.5
• /
• pp.493-500
• /
• 1997

We performed this study to evaluate hepatic venous drainage in atrial isomerism by MR and the clinical significance of anomalous hepatic venous return in total cavopulmonary shunt operation. Numbers and locations of hepatic veins in twenty-two patients with isomerism(thirteen with right isomerism and nine with left isom rism) were evaluated by MR. Operative procedure of hepatic veins and postoperative arterial oxygen saturation were compared with hepatic vein connection in six patients after total cavopulmonary shunt operation. Among nine patients with left isomerism, hepatic venous return was totally anomalous via a single opening in eight, and via two separate openings in one. Among thirteen patients with right isomerism, partial anomalous hepatic venous connection directly to the atrium was seen in four. One showed total anomalous hepatic venous connection to atrium through one opening. Total cavopulmonary shunt operation was performed in 6 patients. Hepatic veins were connected to pulmonary arteries in four patients who had one atrial opening of hepatic vein andlor IVC, or two ipsilateral atrial opening of hepatic veins and IVC. In conclusion, hepatic vein drainage to atrium is variable in atrial isomerism. MR is useful for evaluation of hepatic vein drainage in atrial isomerism and surgical pla ning.

• Journal of Chest Surgery
• /
• v.38 no.8 s.253
• /
• pp.529-537
• /
• 2005

Background: Historically the Fontan operation in patients with single ventricle and heterotaxy syndrome has been associated with high mortality because of systemic or pulmonary anomalous venous drainage, incompetent common atrioventricular valve, right ventricle type univentricular heart, and arrhythmia. Material and Method: A retrospective review of 62 patients $(age:\;54.79\pm33.97\;months)$ with heterotaxy syndrome who underwent a extracadiac Fontan operation between 1996 and 2005 was performed. Twenty one patients had left atrial isomerism, and 41 had right isomerism. The Fontan procedure was staged in all but 2 patients, and a fenestration was less placed in left isomerism. Result: Left isomerism was associated more with interrupted inferior vena cava and pulmonary arteriovenous fistula, and right isomerism was associated more with anomalous pulmonary venous drainage, common atrioventricular valve and morphologic right ventricle. There were 3 hospital deaths$(4.8\%)$ and 3 late deaths $(5.2\%)$ with a follow-up duration of $48.8\pm31.0$ months. Eight-year survivals were $90.5\pm6.4\%$ in left isomerism and $88.6\pm5.4\%$ in right isomerism (p=0.94). At 8 years, freedom from reoperation was $73.9\pm11.3\%$ in left isomerism, and $82.3\pm6.7\%$ in right isomerism (p=0.87). Atrioventricular valve regurgitation progressed after Fontan operation in heterotaxy syndrome, and reoperation for pulmonary arteriovenous fistula and permanent pacemaker implantation for sinus node dysfunction were required more in left isomerism. Conclusion: The extracardiac Fontan operation can now be performed in patients with heterotaxy syndrome with excellent survival. However, morbidity in terms of postoperative atrioventricular valve regurgitation, arrhythmia, and pulmonary arteriovenous fistula remains significant.

• Journal of Chest Surgery
• /
• v.31 no.7
• /
• pp.650-659
• /
• 1998

Between August 1996 and August 1997, 22 patients underwent extracardiac Fontan operations. The basic diagnoses included univentricular heart of the right ventricular type (n=12); univentricular heart of the left ventricular type (n=4); tricuspid atresia (n=4); left isomerism, transposition of great arteries, ventricular septal defect and pulmonary stenosis (n=1); and criss-cross heart with uneven ventricle (n=1). The median age of the 14 men and 8 women was 29 months (range from 21 months to 26 years). Previous procedures included bidirectional cavopulmonary shunt (n=15, interval=15.6$\pm$3.4 months), Kawashima operation (n=4, interval=37.5$\pm$20 months), and classic Glenn shunt (n=1, interval=14 years). In 2 patients, extracardiac Fontan operations were done without any previous procedures. A 16- to 22-mm flexible Gore-Tex tube graft (n=18), Hemashield graft (n=3), or, alternatively, a nonvalved aortic allograft (n=1) was cut and anastomosed end-to-end between inferior vena cava and undersurface of pulmonary artery using Gore-Tex or Prolene suture in a running fashion. In risk Fontan patients (n=12), a communication between the extracardiac conduit and the right atrium was constructed. In the most 13 recent patients, the procedures were done without cross-clamping of the aorta and with a beating heart. Operative mortality was 9.1% (n=2). Complications included persistent chest tube drainage for more than 7 days (n=5), chorea (n=2), and low cardiac output (n=1). There were no late deaths. Follow-up echocardiogram (mean: 6 months) demonstrated satisfactory hemodynamic results in the surviving 20 patients. Potential advantages of this technique consist of minimization of surgical manipulation of atrial tissue, reduction or elimination of myocardial ischemia, creationof a uniform and stable inferior vena cava-to-pulmonary artery conduit, and increased flexibility and safety in certain high-risk patients such as those with increased pulmonary vascular resistance, pulmonary hypertension, and impaired ventricular function. Further investigations during a longer follow-up are needed to confirm the intermediate and long-term results, especially the reduction of late atrial arrhythmias.

• Journal of Chest Surgery
• /
• v.40 no.8
• /
• pp.569-573
• /
• 2007

A 3 month old female baby, who had been diagnosed with right atrial isomerism associated with total anomalous pulmonary venous return (TAPVR), a functional single ventricle and major aortopulmonary collateral arteries (MAPCA), underwent left MAPCA unifocalization and left Blalock-Taussig shunt (3.5 mm) at 3 months of age. The post-operative course was complicated by pulmonary venous congestion, and the drainage site of the TAPVR was found to be stenotic on echocardiography. We performed sutureless repair of the TAPVR along with unifocalization of the right MAPCA. She was put on an extracorporeal membrane oxygenator for 8 days after the 2nd operation, and she was able to come off the oxygenator with the placement of a central shunt (3 mm). She developed tracheal stenosis, which was presumably due to longstanding endotracheal intubation, and she then underwent tracheostomy. She was discharged to home on day 104 after the 1st operation, and she has been followed up for 2 months in a good clinical condition.