We have studied potential for pulmonary circulational assist by the dynamic Fontan model with a skeletal muscle ventricle (SMV) constructed using the latissimus dorsi muscles of 5 dogs. After 2 weeks of vascular delay, the SMV was electrically preconditioned for 8 weeks. Under cardiopulmonary bypass (CPB), the right heart (RV) bypass model was established with the SMV anastomosed between the right atrium and pulmonary trunk. The SMV was paced at a burst frequency of 25Hz, 60/min, with an asynchronization ratio. The aortic pressure (AoP), pulmonary arterial pressure (PAP), central venous pressure (CVP), and pulmonary flow (PAF) were measured. Just after on-SMV, PAP and PAF increased, CVP decreased. CVP decreased from 17±1.4mmHg to 13.5±0.7mmHg (p<0.05). PAP increased from 20±2.8/19±1.6mmHg (non-pulsatile flow) to 37.5±4.9/18±2.1mmHg (pulsatile flow). After CPB, pulmonary vascular resistance (Rp) showed 5.9±1.5 Wood units corresponding to a high risk factor for the Fontan procedure. On this Rp, under off-SMV the CVP was 18mmHg and severe RV failure was recognized as PAF was 35% of the preoperative value. Under on-SMV, CVP decreased and PAF was almost the same as the preoperative value. On physiological CVP, an RV bypass model with intrathoracic SMV maintained PAF at the preoperative value under high Rp. We concluded that this model may be a viable surgical option for univentricular heart with high Rp, which may not be Fontan candidates.

Several problems have been reported following coarctectomy, especially in cases involving long-segment coarctation (COA). Although residual COA, proximal arch kinking, and airway compression may occur after coarctectomy, avoiding the use of artificial materials provides a better chance for the subsequent growth of the aorta. We successfully performed a modified end-to-end anastomosis with subclavian flap aortoplasty for a two-month-old boy with COA. A two-month-old boy was admitted to our hospital for nocturnal tachypnea and a feeding disorder. The initial echocardiography showed a preductal long COA beyond the left subclavian artery. A perimembranous VSD, a patent foramen ovale, and a patent ductus arteriosus were also noted. The left ventricular function was mildly depressed with an ejection fraction of 59%. Enhanced CT revealed a long-segment COA with a length of 15 mm. The blood pressure gradient between the upper and lower limbs was 40 mmHg. The operation was performed at the age of 2 months. The 4th intercostal space was opened through a posterolateral left thoracotomy incision. The distal end of the isthmus was so ligated as to maintain blood perfusion to the lower body through the PDA. The arch was clamped between the left carotid and the left subclavian artery (LSCA). The LSCA and the isthmus were divided as distally as possible, and the two distal ends were longitudinally incised and sutured to each other in a side-to-side fashion using a 7-0 polypropylene continuous suture. After complete resection of the ductal tissue, a newly created distal arch was anastomosed to the descending aorta. The left ventricular ejection fraction was increased to 74% at discharge. Catheterizations 3 years after the surgery did not reveal any stenosis or deformity in the aorta at normal PA pressure. The patient has been doing well and is free of complications 7 years after the surgery. At present, end-to-end anastomosis and aortic arch advancement with or without cardiopulmonary bypass are widely used procedures for coarctectomy ; however, a modified end-to-end anastomosis is still a viable option for cases involving long-segment coarctation.

Fourteen patients (mean age 17.2 years, range 2 to 39 years) undergoing right ventricular outflow tract reconstruction for a Ross operation were studied between 1998 and 2000. Ten of 14 patients underwent Ross procedures and 4 received Ross-Konno procedures. Echocardiographic examination of the pulmonary homograf t was performed after surgery. The mean follow-up period was 23.1 months, ranging from 14 to 33 months. Mean peak velocity and peak gradient were 1.6±0.4m/s and 11.9±5.2mmHg, respectively. Three patients in whom echocardiography revealed a peak pulmonary gradient of 20mmHg or more were retrospectively analyzed with each catheterization data. All patients had no more than 10mmHg at the distal end of the homograft with no evidence of deformity or shrinkage. Only one patient had a trivial homograft valve regurgitation, however, no patient had more than mild pulmonary regurgitation. Patient age, donor age, and preservation period did not reveal any significant risk factor for homograft stenosis. Pulmonary homograft appears to be an excellent substitute for right ventricular outflow tract reconstruction during the mid-term postoperative period.

A 6-month-old baby boy had undergone the Jatene procedure at 4 days. Four months later, catheter intervention (balloon angioplasty) was performed because of severe stenosis at the bifurcation of the pulmonary arteries. Twenty days later, several episodes of cyanosis occurred and he was readmitted. The existence of shunt flow between the sinus of valsalva and the pulmonary bifurcation was detected by echocardiography and examination by 16-row MDCT revealed 2 holes at this site. Under a diagnosis of aortopulmonary (AP) window, the patient was placed on cardiopulmonary bypass and the pulmonary artery was opened after aortic clamping. There was a ridge between the bifurcation of the pulmonary arteries. After removing it, 2 holes were visualized that resembled the findings on 16-row MDCT. These holes were closed with Xenomedica patches and the main pulmonary artery was also extended with a Xenomedica patch. AP window is a rare complication after balloon angioplasty for pulmonary stenosis, but we must take great care to prevent this complication.

A neonate, presenting with cyanosis, received the diagnosis of persistent truncus arteriosus with truncal valve stenosis with insufficiency. Her disease was classified as persistent truncus arteriosus Van Praagh type A1, or Collett and Edwards type I. At the age of 2 months, she underwent a modified Blalock-Taussig shunt, and her operative team was waiting for adequate body weight gain before performing further surgery. At the age of 1 year, however, she began to have repeated episodes of congestive heart failure due to severe tricuspid valve regurgitation and truncal valve insufficiency. When she reached 18 months of age, she underwent a definitive operation including a truncal valve plasty, VSD patch closure, and a right ventricular outflow tract reconstruction. Postoperative echocardiography 6 months after surgery showed a good truncal valve function with minimal regurgitaion. Truncal valve surgery is a challenging operation ; we report a successfully treated case, though the patient will require extensive follow-up.

A 3-year-old girl was given a diagnosis of coronary arteriovenous fistula associated with a single right coronary artery on cardiac catheterization. The left coronary artery arose from the proximal part of the right coronary artery. The dilated left coronary artery ran in front of the right ventricular outflow tract and then divided into the left anterior descending branch and the left circumflex artery. A coronary arteriovenous fistula was in the left main coronary artery and opened into the right ventricular outflow tract. Under cardiopulmonary bypass and cardiac arrest, a transverse incision was made at the right ventricular outflow tract 1cm below the dilated vessel and the 5-mm oval-shaped orifice of the fistula was identified. This fistula was closed with a pledgetted mattress suture reinforced with over-and-over suture. Catheterization 8 months after surgery demonstrated no residual shunt and she has been doing well.

Preoperative evaluation of cardiac anatomy is essential to determine the correct surgical procedure for congenital heart disease. Multi-detector row CT (MDCT) is a useful alternative imaging modality to cardiac catheterization and echocardiography. Sixteen patients (12 with total anomalous pulmonary venous return (TAPVR) and 4 with aortic arch anomalies) underwent 16-slice multi-detector row CT scanning. Three-dimensional reconstruction by MDCT was useful to determine the type of TAPVR and the presence of pulmonary venous obstruction (PVO) in TAPVR patients, as well as to detect postoperative PVO in patients who underwent intracardiac repair. In 2 patients who had asplenia associated with TAPVR III and I a, MDCT enabled an intra-atrial approach for TAPVR repair by precise preoperative determination of the relationship between the common PV chamber and single atrium. In patients with aortic arch anomalies, MDCT was useful to determine the type of anomaly, the presence of arch hypoplasia, and any associated rare vascular anomalies (including isolated subclavian artery, and the right-sided descending aorta with left aortic arch). In conclusion, MDCT provides reliable preoperative evaluation of pulmonary venous return and aortic arch anatomy, and therefore is extremely useful for surgical management of congenital heart disease.

A 9-month-old boy who had been given a diagnosis of double outlet right ventricle (DORY), partial anomalous pulmonary venous return (PAPVR), ventricular septal defect (VSD), pulmonary hypertension (PH) and polysplenia with azygos connection, underwent pulmonary artery banding at the age of 6 months. At 2 months after surgery, a chest computed tomogram revealed a main pulmonary artery aneurysm and a main pulmonary artery-right pulmonary artery fistula caused by bacterial endocarditis due to a methicillin-resistant Staphylococcus epidermidis. We performed pulmonary arterioplasty and re-pulmonary artery banding for acute aggravation of cardiac insufficiency and obtained good results. This is an extremely rare case that was treated infectious pulmonary artery aneurysm and fistula after pulmonary artery banding.

We describe a case of postoperative aortic valve regurgitation (AR) after arterial awitch operation (ASO) successfully managed by the modified Konno procedure. A 4-year-old girl with complete transposition of the great arteries (TGA, Type II) had undergone the ASO (LeCompte maneuver) at 10 days of age. Because of progression of moderate AR 4 years after ASO, the modified Konno procedure with aortic valve replacement (SJM 21mm) was successfully performed. She remains in good clinical condition at the last follow-up at 5 years.

Pulmonary venous obstruction (PVO) after repair of total anomalous pulmonary venous connection remains a significant problem. Once it occurs, it not infrequently recurs. A 14-month-old boy with recurrent pulmonary venous obstruction after repair of mixed type total anomalous pulmonary venous connection was successfully treated by the method of sutureless in situ pericardial repair and anastomosis of the left pulmonary vein to the left atrial appendage. His postoperative course was uneventful. Cardiac catheterization at 2 years and 9 months after the re-redo operation showed successful relief of PVO with marked reduction of pulmonary hypertension. In addition, multidetector computed tomography (MDCT) performed 3 years and 1 month after the operation showed no pulmonary vein obstruction.