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We report a neonatal case of one-stage repair for transposition of the great arteries with intact ventricular septum (TGA with IVS) and coarctation of the aorta (CoA). The surgery was performed at 27 days of age when the patient, a male, weighed 3408 g. Preoperative cardiac catheterization and angiography confirmed the diagnosis of TGA with IVS, CoA, atrial septal defect, patent ductus arteriosus, persistent left superior vena cava, left sided juxta-positioning of appendages and dextrocardia. The procedure was simultaneous subclavian flap aortoplasty through a left thoracotomy and an arterial switch operation through a median sternotomy. The patient's postoperative course was uneventful and he has grown well. Postoperative cardiac catheterization revealed mild pulmonary stenosis (pressure gradient of 19 mmHg between the right ventricle and the pulmonary artery) and mild aortic arch kinking from the arterial switch maneuver. One-stage repair, the combination of subclavian flap aortoplasty and arterial switch operation, provides a good clinical result in this complex malformation.

We reported a successful case of the modified Norwood operation for a 21-day-old neonate with hypoplastic left heart syndrome (MS and AS) associated with an aberrant right subclavian artery and a persistent left superior vena cava. The modified Norwood operation was performed without total circulatory arrest and Cardiac arrest. A 4 mm Gore-Tex graft, which was anastomosed between the right carotid artery and the right pulmonary artery for systemic-pulmonary shunt, was used for cerebral perfusion during aortic arch reconstruction. Coronary perfusion was performed with a small cannula placed on the relatively large ascending aorta during anastomosis between the main pulmonary artery and the ascending aorta. Equine pericardial patch was used for aortic arch reconstruction and the ascending aorta was directly anastomosed to a part of the main PA. Postoperative course was uneventful and postoperative MRI revealed no stenosis of the aortic arch and the pulmonary artery.

We report an alternative method of repairing partial anomalous pulmonary venous return (PAPVR) wherein anomalous veins drain into the high portion of superior vena cava (SVC). A six year old girl, weighing 19 kg underwent cardiac catheterization for cardiomegaly. The study showed sinus venous defect and pulmonary veins anomalously drained from the right upper and middle lobe into the high SVC. Under cardiopulmonary bypass with aortic and bicaval direct cannulation, a 2-cm-wide pedicled flap made from the middle part of the right atrium (RA), was pulled into the SVC. The atrial flap divided the SVC from the ostium of the uppermost pulmonary vein (PV) to the ASD. This posterior tunnel drained anomalous PV blood into the left atrium. Postoperative echocardiogram and magnetic resonance imaging showed adequate space in the SVC and the PV channel. The patient maintained normal sinus rhythm after surgery. The advantage of this method is to minimize the risk of injuring the sinus node or sinus node artery, and to easily reconstruct both the SVC and PV channel without using prosthetic materials, even in patients with small RA.

We experienced the rare association of Tetralogy of Fallot with cor triatriatum, unroofed coronary sinus and persistent left superior vena cava. Only one case of this association has ever been reported in the literature. We successfully performed a total correction which consisted of a resection of the intra-left atrial fibrous membrane, reconstruction of the LSVC return pathway to the right atrium with PTFE graft, atrial partition with a bovine pericardial patch and the conotruncal repair for Tetralogy of Fallot. Cardiac catheterization 6 months after operation demonstrated the smooth drainage from LSVC into the right atrium without pressure gradient. His postoperative course has been uneventful.

We experienced two cases with uncommon unroofed coronary sinus. The first patient, a 55-year-old man, had a partially unroofed mid-portion of the coronary sinus. His symptoms and physical state were similar to that of atrial septal defect (ASD). Echocardiogram and angiogram revealed that the coronary sinus (CS) communicated with the left atrium (LA). We confirmed that CS had an enlarged orifice and lacked a part of its roof through the right atriotomy and the atrioseptotomy. We directly closed the defect between the LA and the CS. His postoperative course was satisfactory. The second patient, a 46-year-old man, showed mild cyanosis. He had a completely unroofed CS with left superior vena cava (LSVC), absent inferior vena cava (IVC) and hemiazygos continuity. The LSVC directly drained to the LA, and the CS was absent. A part of the posterior wall of the LA was like a groove which laid from the orifice of the LSVC to the coronary sinus ASD. The roof of this groove was covered with fibrous tissue, which was shaped like a network structure. We thought that this network was a residue of the septum between the CS and the LA. Thus we resected this structure, and reconstructed the roof by equine pericardial patch to drain the venous blood from the LSVC to the right atrium. Anomaly in the patient may be the transitional type between the completely unroofed CS and the partially unroofed CS.

A radical correction involving Vargas's method and a direct anastomosis between the left pulmonary vein (PV) and left atrium (LA) was performed in a 29 day-old infant with supracardiac type Ib total anomalous pulmonary venous return (TAPVR) and an unusual form of the left PV. the left upper and lower PVs drained into a left "common" PV that was just behind the LA, and then into the right pleural cavity. The left common PV was located cephalad to the normal course and received blood from the right lower and upper PVs and drained into the supra-vena cava (SVC). The junction of the SVC and the right PV was slightly stenotic. Vargas's method is a useful technique for Darling's classification Ib TAPVR even in cases without the common PV situated behind the LA. But this patient had a left common PV and it was possible either to anastomose the common PV and LA directly or to perform Vargas's technique. We performed both procedures to prevent left PV obstruction (PVO). Cineangiography performed 2 months after surgery showed that a large amount of blood from the right and left PV drained into the LA through the common PV-LA route. These procedures, which create a dual PV channel, reduce the risk of PVO, so they are useful for radical correction of Darling's Ib type TAPVR.

A 10-year-old girl with bilateral partial anomalous pulmonary venous connections (PAPVC) was referred to us. The right upper pulmonary vein (PV) drained to the high superior vena cava (SVC) and the left upper PV to the left innominate vein. The left upper PV was directly anastomosed to the right atrial appendage. The SVC was divided distal to the orifice of the right upper PV and anastomosed to the right atrial appendage. A Xenomedica patch was placed within the right atrium to divert the pulmonary venous blood from the SVC orifice across the surgically enlarged ASD to the left atrium. The postoperative course was satisfactory without arrhythmia, and cineangiography revealed no obstruction at the SVC and the right upper PV pathways. We thought that this method is advantageous in preserving the sinus node and its feeding artery, as well as being applicable to the PAPVC to the high SVC.

We performed the new operative method for bilateral partial anomalous pulmonary connections (PAPVC) without atrial septal defect (ASD). A 11-years-old boy was admitted for operation. The right upper pulmonary vein returned to the superior vena cave (SVC), and left upper pulmonary vein returned to the innominate vein. Atrial septum was intact. The Qp/Qs was 1.94. The right atrium (RA) was opened and ASD was created. Continuity between the distal end of the SVC and RA was re-established by end-to-end anastomosis to the right atrial appendage. The proximal end of the SVC was closed. The right anomalous pulmonary vein remained in situ on the lower segment of SVC, blood being directed to the left atrium (LA) through the created ASD by suturing the posterior flap of RA incision to the atrial septum well away from the sinoatrial node and artery, anomalous pulmonary vein and cavoatrial junction. The anomalous left vertical vein was dissected up to the innominate vein. The left pulmonary vein was mobilized as much as possible, and was anastomosed to the posterior wall of LA by end-to-side fashion. He has normal sinus rhythm and no evidence of pulmonary venous obstructions on both sides. We support the efficacy of this method as an alternative in the management of this disorder.

We report a case of a 24-year-old woman who had atrial septal defect associated with partial anomalous pulmonary venous return. The right upper pulmonary vein returned anomalously to the proximal part of the superior vena cava. Direct anastomosis between the left atrium and the anomalous right upper pulmonary vein was successfully performed.

We recently encountered a case of total anomalous pulmonary venous connection (TAPVC, Darling Ib) and 3 cases of partial anomalous pulmonary venous connections (PAPVC) to the superior vena cava. We surgically treated these 4 cases by different procedures which were selected according to the morphological features of individual cases. In the case of TAPVC (Darling Ib), we created a right atrial wall flap, according to the method of Vargas, so that the blood from the abnormal pulmonary vein could pass the left atrium via the superior vena cava and atrial septal defect. In 3 cases of PAPVC, we modified some techniques of right atrial incision and closure by suturing. Through these surgical procedures, none of the 4 patients developed postoperative stenosis of the pulmonary venous pathway or arrhythmias. Various surgical procedures have been reported for the treatment of anomalous pulmonary venous connection, an anomaly involving direct connection of the pulmonary vein to the superior vena cava or to the right atrial junction of the superior vena cava. However, the incidence of postoperative stenosis or obstruction of the pulmonary venous pathway or postoperative sick sinus syndrome has been high with these procedures.

A successful repair of total right pulmonary venous return and intact atrial septum associated with patent left superior vena cava (PLSVC) is reported. A 31-year-old women was admitted for evaluation of heart failure. A heart murmur had been detected since her childhood without any subjective symptoms. She was diagnosed as partial anomalous venous return of all right pulmonary vein and intact atrial septum (IAS) associated with PLSVC by cardiac catheterization. Operative findings revealed that all right pulmonary vein connected to right atrium (RA) and superior vena cava (SVC), PLSVC drained into coronary sinous (CS), therefore RA, right ventricle and CS were enlarged, but SVC was small due to PLSVC. Through large incision from RA to SVC, atrial septum was opened 30 mm in diameter. Internal conduit using pericardial patch was made in order to drain anomalous pulmonary venous blood into left atrium through the ASD. Finally, SVC and RA were reconstructed by pericardial patch for prevention of conduit obstruction. One month after operation, no stenosis of SVC and right pulmonary vein was evidenced by cardiac catheterization and all through the postoperative course, arrhythmias did not detected. She was discharged with uneventful postoperative course.

A 29-year-old woman, who was diagnosed as congenital mitral stenosis associated with DORV, PDA, PLSVC and severe pulmonary hypertension, was reported. The mitral valve, consisting of leaflet dysplasia, shortened and thickened chordae tendineae, obliteration of interchordal spaces, under-development of papillary muscle, and stenotic supravalvular membrane, was replaced with 25 SJM prosthesis. Simultaneously, patch closure of VSD and intrapulmonary direct closure of PDA was performed. Her postoperative course was uneventful and systolic pulmonary arterial pressure fell down from 95 mmHg to 56 mmHg. She is doing well after correction. To our knowledge, this is very rare corrective case with congenital mitral stenosis in adult to be reported in the literature in Japan.

Surgical repair of anomalous right pulmonary venous drainage to the high segment of the superior vena cava (SVC) with intact atrial septum in a 5-year-old girl by cavoatrioplasty avoiding injury to the sinoatrial (SA) node and its feeding artery is described. The procedure was composed of #1: an oblique incision at the anterior wall of SVC above the anomalous right pulmonary veins (ARPVs) and closure of the lower end of the SVC, making the lower segment of SVC the ARPV channel, #2: a fan-shaped incision at the base of the right atrial (RA) appendage and suturing the cephalad flap over a created atrial septal defect, diverting the ARPV flow through the SVC into the left atrium, and #3: reconstruction of continuity between the cephalad end of the SVC and the RA by suturing the RA appendage flap over the lower segment of SVC. Pulmonary valvotomy was also done for concomitant pulmonary stenosis. Postoperative course of the patient was excellent, with constant normal sinus rhythm. Angiography one month after operation showed no stenosis of the SVC and pulmonary venous pathways. The method reported here was considered to have following advantages: It employs simple and short incisions, avoids injury to the SA node its feeding artery, and achieves wide and nonobstructive pathways of both SVC and pulmonary venous return without using foreign material.

A 42-year-old woman who had a coronary artery fistula, associated with a giant coronary saccular aneurysm was reported. The coronary artery fistula originated from the proximal portion of the right coronary artery drained into the superior vena cava. The chief complaint was heart murmur which was detected at the 2nd intercostal space of the right sternal border. No other symptoms were present. The aneurysm was approximately 5 X 7 cm in size. In the operation using cardiopulmonary bypass, the proximal and distal portions of the coronary artery fistula were ligated successfully without aneurysmorrhaphy. The postoperative conditions was even without any complications. Congenital coronary artery fistulas with a giant saccular aneurysm should be surgically treated as soon as possible because of potential risk of aneurysmal rupture.

A 27-year-old male with a history of congenital heart disease was admitted for pre-operative evaluation of a cardiac malformation. Echocardiography and cardiac catheterization revealed an incomplete endocardial cushion defect with a persistent left superior vena cava which drained into the left atrium but echo-cardiographic evidence of an abnormal intra-atrial septum was not found. The patient was placed on cardiopulmonary bypass and prepared for the surgical correction of his primary cardiac lesion. Intra-cardiac examination during reconstruction of Raghib's syndrome also revealed the presence of cor triatriatum. Reghib's syndrome is characterized by the combination of abnormal drainage from the left superior vena cava into the left atrium, the presence of an atrial septal defect and the absence of a coronary sinus. To prevent secondary complications such as a brain abscess, we redirected blood flow from the left superior vena cava to the right atrium utilizing a trimmed woven dacron vascular graft that was placed in the intra-atrial position. Mitral valvoplasty, excision of the obstructing diaphragm and atrial septation were also performed successfully. Although the literature has described the surgical repair of Raghib's syndrome, its correction in combination with cor triatriatum is considered to be extremely rare.

A 46-day-old infant weighing 2250 g with the coarctation of the aorta, ventricular septal defect, PDA, the anomalous right subclavian artery, and the persistent left superior vena cava initially underwent by the reversed subclavian flap aortoplasty with the anomalous right subclavian artery and pulmonary arterial banding. The reversed subclavian flap aortoplasty was useful for the coarctation complex with the anomalous right subclavian artery. Two months later he gained weight to 2500 g, and then VSD closure and debanding of PAB was performed successfully. This two-staged operation was recommended for the poor risk coarctation complex with the anomalous subclavian artery.