Modified repair of mixed anomalous pulmonary venous connection

A modified repair technique is reported for mixed total or partial anomalous pulmonary venous connection with the right superior pulmonary vein connecting to the superior vena cava, the right inferior pulmonary vein to the right atrium or left atrium, and the left pulmonary veins to the coronary sinus. The superior vena cava is transected above the highest right superior pulmonary vein, its cephalad end is anastomosed to the right atrial appendage, and a pericardial baffle is constructed between the cardiac ostium of the superior vena cava, the ostium of the right inferior pulmonary vein, and the left atrium, including the coronary sinus, which is unroofed. The reported technique may be valuable to avoid pulmonary venous obstruction in complex mixed forms of total or partial anomalous pulmonary venous connection.     

Isolated left superior vena cava to the left atrium with situs solitus and dextrocardia: extracardiac repair facilitated by juxtaposition of the atrial appendages

We describe an extremely unusual case, with isolated left superior vena cava to the left atrium, normal intracardiac anatomy, and left juxtaposition of the atrial appendages. Surgical repair was performed without cardiopulmonary bypass by anastomosing the left superior cava to the superior aspect of the right atrial appendage, and was facilitated by the ectopic location of the appendage.     

Successful repair of total anomalous pulmonary venous connection and incomplete endocardial cushion defect associated with left isomerism

Successful repair of a 8-month-old girl with polysplenia was reported. The cardiovascular anomalies were TAPVC (II b), incomplete ECD, interruption of inferior vena cava with hemiazygos continuation, bilateral superior vena cava, and left superior vena cava draining into the coronary sinus. Cardiopulmonary bypass was established with ascending aortic perfusion and caval cannulation. A left superior vena cava was directly cannulated after establishing partial bypass. In this case the left pulmonary vein drained into the right atrium near the orifice of the coronary sinus, so the atrial septal flap was made and sutured between the orifice of the left pulmonary vein and the coronary sinus in order to avoid late pulmonary vein obstruction. Then, atrium was separated by an intraatrial baffle which was sutured to the atrial septal flap. Recently, it becomes possible to surgical repair of polysplenia syndrome according to the advancements of the diagnostic methods, cardiopulmonary bypass, and the technique of the open heart surgery.     

Clinicopathologic features of dogs with hepatic microvascular dysplasia with and without portosystemic shunts: 42 cases (1991-1996)

A 14 year old girl with double outlet right ventricle, left isomerism, and complicated atrial anatomy had undergone corrective surgery with intra-atrial rerouting at 5 years of age. Cardiac catheterisation eight years after the surgery showed that she had two systemic venous baffle stenoses: between the hepatic vein and caval vein, and the caval vein and right atrium. Two Palmaz stents were successfully implanted percutaneously through a long sheath and a balloon catheter. The stenoses were relieved immediately and her symptoms quickly disappeared.     

Haemodynamics after Mustard's operation for transposition of the great arteries

Cardiac catheterization data from 54 investigations after Mustard's procedure were examined to study the influence of the operation on pressure events in the atria, great veins, and pulmonary circulation. Systemic venous atrial pressure tracings were characterized by a rapid, sharp 'y' descent. Pressure gradients between the venae cavae and systemic venous atrium were invariable, whether or not vena caval pathway obstruction was present, the 'y' trough and 'a' wave gradients being greater than the mean gradient. Pulmonary venous atrial pressure tracings were not different from normal except when tricuspid regurgitation was present. It is suggested that the baffle effectively reduces the size and compliance of the systemic venous atrium, but influences the pulmonary venous atrium to a lesser degree. The systolic pressure gradient from the left ventricle to pulmonary artery was decreased postoperatively, suggesting that it may be flow-related; the greatest changes were seen in the group with preoperative ventricular septal defect. The ratio of pulmonary: systemic vascular resistance did not change significantly after operation, and it is suggested that both the pre- and postoperative values were higher than normal. Examination of the left ventricular or pulmonary arterial mean pressure postoperatively should raise the suspicion of a complication, e.g. pulmonary venous obstruction or tricuspid regurgitation.     

Progresses in the study of general surgery in China, 1997

Late systemic venous baffle obstruction after Mustard repair for complete transposition of the great arteries is a recognized complication. Balloon-expandable intravascular stents have previously been used to relieve systemic baffle narrowing in children. We report a successful stent implantation to relieve symptomatic superior vena cava obstruction and baffle dehiscence after Mustard repair in an adult patient. She had been turned down for surgery due to right ventricular dysfunction. Eighteen months after the procedure, she remains symptomless.     

Atrial septal displacement for repair of anomalous pulmonary venous return into the right atrium

BACKGROUND: In the repair of anomalous connection of the pulmonary veins to the right atrium, the use of a baffle of pericardium to divert the pulmonary venous blood into the left atrium could cause pulmonary venous obstruction as a result of thickening of the pericardial patch. Anomalous pulmonary venous drainage to the right atrium caused by malposition of the atrial septum primum can be repaired by displacing the shifted septum primum to the normal position. METHODS: In 5 patients with total (n=2) or partial (n=3) anomalous pulmonary venous drainage into the right atrium, the septum primum was shifted toward the left atrium and the pulmonary veins drained into the anatomic right atrium despite their normal connection with the posterior wall of the left atrium. This method consisted of incision of the posterior edge of the atrial septum primum and displacement of the incised atrial septum between the anomalous pulmonary veins and both venae cavae. No patch was used. RESULTS: Postoperative echocardiography showed a wide pathway from the pulmonary veins to the left atrium with no stenotic portions. No atrial arrhythmias occurred after the operation. CONCLUSIONS: This technique may be advantageous because it allows for future growth of the route of the pulmonary venous pathway and avoids postoperative supraventricular arrhythmias.     

Systemic venous collateral development after the bidirectional cavopulmonary anastomosis. Prevalence and predictors

OBJECTIVES: To determine the prevalence of systemic venous collaterals after the bidirectional cavopulmonary anastomosis and the factors associated with their development. BACKGROUND: Systemic venous collaterals have been found after cavopulmonary anastomosis. Methods. Cardiac catheterization was performed in 103 patients before and after a bidirectional cavopulmonary anastomosis. RESULTS: After surgery, 51 venous collaterals were identified in 32 patients (31%). Collateral development was associated with an abnormal superior vena caval connection (56% incidence vs. 26% with a single right superior vena cava, p = 0.01) and postoperative factors including pulmonary artery distortion (53% incidence vs. 22% without distortion, p = 0.002); increased superior vena caval mean pressure (14 +/- 5 mm Hg versus 11 +/- 4 mm Hg with no collaterals, p = 0.0002); increased pulmonary artery mean pressure (13 +/- 4 mm Hg vs. 11 +/- 4 mm Hg with no collaterals, p = 0.02); lower right atrial mean pressure (5 +/- 2 mm Hg vs. 6 +/- 3 mm Hg with no collaterals, p = 0.04); and increased mean gradient between superior vena cava and right atrium (8 +/- 3 mm Hg vs. 5 +/- 4 mm Hg with no collaterals, p = 0.0002). Using multiple logistic regression, only this last factor was independently associated with collateral development with an odds ratio per 1 mm Hg of 1.33 (95% CI 1.12-1.58, p = 0.001) for their presence. CONCLUSIONS: Systemic venous collaterals occur frequently after a bidirectional cavopulmonary anastomosis and are found postoperatively when a significant pressure gradient occurs between cava and right atrium.     

Differential atrial filling after Mustard and Senning repairs. Detection by transcutaneous Doppler ultrasound

The dominance of Mustard's operation for transposition of the great arteries has been challenged by the recent revival of Senning's repair because it promises better long-term results in terms of venous obstruction and atrial haemodynamics. These hypotheses were tested by recording jugular venous flow waveforms transcutaneously in 24 postoperative patients with simple complete transposition using a bidirectional Doppler blood velocimeter. Eight patients had undergone Mustard's operation and 16 the Senning alternative; all had previously had a postoperative cardiac catheterisation. Both groups of patients had similar left ventricular, pulmonary arterial, and systemic venous atrial pressures. No child showed any evidence at catheterisation of either mitral regurgitation or of superior vena caval pathway obstruction. These two findings were endorsed by the transcutaneous Doppler recordings. Jugular venous flow in normal children exhibits two maxima, one of atrial filling during ventricular systole, the other of ventricular filling occurs once the tricuspid valve has opened. Both operative procedures diminished the size of the former phase, but the Mustard did so more. After Mustard's operation forward flow during the atrial filling phase was absent in approximately half the cardiac cycles recorded, and severely diminished in the rest. By contrast, there was approximately a 90 per cent appearance of atrial filling waves after Senning's operation which also provided significantly better atrial function than Mustard's procedure in terms of peak velocity of blood entering the atrium and total atrial filling. It is therefore concluded that both procedures compromise atrial volume and compliance but Senning's repair to a much lesser extent.     

Diversion of the inferior vena cava into the left atrium after repair of atrial septal defect with partial anomalous pulmonary venous drainage

An 11-year-old female had operative repair of atrial septal defect associated with partial anomalous pulmonary venous drainage by direct suture at 6-year-old. Postoperatively, cyanosis and nodal bradycardia were noted by physical exercise. During 5 years thereafter, the symptoms were gradually aggravated accompanying. Cardiac catheterization revealed diversion of the inferior vena cava into the left atrium. Oxygen sampling showed right to left shunt of 22% at the atrial level. At reoperation, orifice of the inferior vena cava into the right atrium became narrow 5 mm in diameter. After enlargement of the orifice, the atrial septum was created with a Gore-Tex patch to redirect the inferior vena cava to the right atrium. Post operative course was uneventful with disappearance of the cyanosis. The patient is up and well now 9 years after reoperation.     

Late postoperative hemodynamic results and cineangiocardiographic findings after Mustard atrial baffle repair for transposition of the great arteries

Forty-nine patients with transposition of the great arteries who underwent a Mustard atrial baffle repair between 1964 and 1971 were assessed late postoperatively. There have been five late deaths: two related to baffle obstruction, two from noncardiac causes, and one sudden and unexpected. Hemodynamic data were available in 42 patients and autopsy in four. Obstruction of the lower venous channel was not encountered. Three patients had severe obstruction of the upper venous channel and in four there was mild restriction. Two patients had severe pulmonary venous obstruction resulting in late death; mild asymptomatic obstruction could not be excluded with certainty in six patients. Tricuspid incompetence was infrequently encountered in patients with an essentially intact ventricular septum. Left ventricular outflow tract obstruction was found in eight patients. In three it was present after satisfactory but incomplete surgical relief and in five it had not been recognized prior to operation. In only one of the latter patients was the obstruction important. Seventeen patients were operated on prior to one year of age. While baffle obstruction was confined almost entirely to these patients, the five youngest patients, aged one to nine weeks at operation, had adequate pulmonary and caval compartments at restudy two to three years later.     

Unroofed coronary sinus syndrome

Unroofed coronary sinus syndrome or coronary sinus septal defect is a rare congenital cardiac anomaly. We performed corrective operations in 9 patients with such condition. Of them, seven patients had the completely unroofed coronary sinus and persistent left superior vena cava (PLSVC) directly draining into the left atrium (LA). One patient had PLSVC and the partially unroofed terminal portion of the coronary sinus (CS), or sinus ostium open into LA. Another patient had total anomalous pulmonary vein connection and the partially unroofed mid-portion of CS without PLSVC. The operative methods of the unroofed coronary sinus included: ligating PLSVC and repairing ostium primum or secundum atrial septal defects in 4 cases; creating an intra-atrial tunnel from PLSVC to the right atrium (RA) and repairing the atrial septal defect in 2 cases; reconstructing the intra-atrial septal with a patch as a baffle to guide PLSVC or sinus ostium respectively towards RA in 2 cases; enlarging the defect on the coronary sinus roof and repairing the atrial septal defect of the coronary sinus type in one case. No patient died in this group. Because unroofed coronary sinus syndrome has atypical clinical manifestation and usually complicates varied congenital anomalies, its preoperative diagnosis is very difficult in some times and may draw the reliable support from echocardiography and catheterization. The operative program and method must depend not only upon its type but also upon PLSVC presence of absence, and communication between PLSVC and RSVC.     

Handedness assessment inventory

A 6 year old boy with a large atrial septal defect, partial anomalous pulmonary venous drainage and unrecognized anomalous insertion of the inferior vena cava into the left atrium had cyanosis after closure of the atrial defect. Repeat study revealed direct drainage of the inferior vena cava into the left atrium with moderate arterial oxygen desaturation. At repeat operation an unusual positioning of the inferior vena cava was seen. After reopening of the atrial defect, the pulmonary venous and systemic venous drainage anomalies were identified. A Dacron patch was inserted so as to divert flow to the proper atrium. Repeat catheterization 3 months after operation revealed a normal heart with no obstruction; arterial oxygen saturation was normal. The child has continued to do well 3 years after operation.     

Continuous murmur following Mustard operation for transposition of the great arteries. A sign of pulmonary venous obstruction

Three patients developed severe pulmonary venous obstruction following Mustard operation for transposition of the great arteries. Each patient had a soft continuous murmur with distinct diastolic accentuation at the low left sternal border or xiphoid area. Simultaneous recording of intracardiac sound and pressure in one patient showed that the murmur originated at the site of obstruction in the surgically-constructed pulmonary venous atrium. Selective cineangiograms demonstrated the baffle to be the cause of the obstruction. A continuous murmur in a patient following Mustard operation may suggest significant pulmonary venous obstruction.     

Levocardia with ventricular inversion and a single atrium in normal position

A 12-year child had complete heart block, an abdominal situs inversus with laevocardia. Further investigation demonstrated in addition an inferior vena cava ending into an azygos vein, a shunt at the atrial level and a pulmonary valve stenosis. At operation there was a lone atrium in normal position, a ventricular inversion without transposition of the great vessels. In spite of a corrective operation of the Mustard type, the child died the following day through haemorrhage and low output.     

Carpal tunnel syndrome in amyotrophic lateral sclerosis and late onset cerebellar ataxia

We report two cases of tricuspid valve replacement for tricuspid valve insufficiency as reoperations following mitral valve replacement through midline sternotomy. A right thoracotomy was used to approach the tricuspid valve. To avoid the risk of cardiac laceration, cardiopulmonary bypass was instituted after cannulation of the femoral artery and of superior vena cava through right atrium with balloon caval occlusion and inferior vena cava through the femoral vein with balloon caval occlusion. Without aortic cross clamping under mild hypothermia, right atriotomy was performed through adherent parietal pleura, pericardium, and right atrial wall without dissection. Tricuspid valve was replaced utilizing the bioprosthetic valve with good clinical results. These new measures were expeditiously carried out without dissection of the heart, which has been deemed to be the risk of reoperations.     

Surgical treatment of the massively dilated ureter in children. Part II. Management by primary reconstruction

Bypass of the left ventricle was accomplished in dogs and the entire circulation was supported temporarily by only the right ventricle. The atrial septum was excised, and the atrium was repartitioned so that the pulmonary veins were in continuity with the right ventricle and the venae cavae were connected through the atrium. Anastomosis of the superior vena cava to the right pulmonary artery brought systemic venous return directly to the lungs. The main pulmonary artery was ligated proximal to the bifurcation, preserving distal confluence of right and left pulmonary arteries. A tubular prosthesis between the proximal pulmonary artery and the aorta connected the right ventricle to the systemic circuit. This procedure, or some modification of the principle, may have clinical feasibility in the treatment of patients with hypoplastic left heart syndrome.     

In vitro metabolism of a new 4-hydroxycoumarin anticoagulant. Structure of an unusual metabolite

A case of common atrium with anomalous systemic and pulmonary venous connections and pulmonary stenosis is described. Surgical correction was performed by pulmonary valvotomy and the insertion of an intra-atrial pericardial baffle. To our knowledge this is the first reported case of surgical correction of total anomalous connections of both systemic and pulmonary veins. The embryologic development of this condition is discussed.     

Cardiac function in total anomalous pulmonary venous return before and after surgery

Cardiac performance was evaluated in 12 infants with isolated total anomalous pulmonary venous return. Four had significant pulmonary venous obstruction and severe pulmonary hypertension (group A). Eight had no obvious venous obstruction, and the pulmonary pressures were lower (group B). In all subjects, right ventricular end-diastolic volume was increased (197% of predicted normal) and its ejection fraction was normal. Left ventricular volume was, generally speaking, still in the normal range (87% of predicted normal); however, its ejection fraction was reduced (0.57 vs normal of 0.73) and left ventricular output was low (3.08 L/min/m2 vs normal of 3.98). Left atrial volume was consistently small (53% of predicted normal) with an appendage of normal size. The infants in group A had smaller chamber volumes/m2 BSA than those in group B. Left atrial function was abnormal, characterized by reduced reservoir function and a greater role as "conduit" from right atrium to left ventricle. Left atrial size was not found to be critical in the surgical repair of TAPVR. Cardiac function is restored to normal following surgery.     

A case report of one-stage repair for transposition of the great arteries with intact ventricular septum and coarctation of the aorta in neonate

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.