Results of rapid two-stage arterial switch operation in patients with transposition of the great arteries: one-year postoperation

The rapid two-stage arterial switch operation is an alternative therapy for patients with simple transposition of the great arteries who present beyond the neonatal period and have low left ventricular pressure. It provides normal ventricular function compared to the atrial switch operation. Between July 1994 and February 1997, there were 13 such infants who had rapid two-stage arterial switch operation performed at Siriraj Hospital. There was 1 late death (11 months after the operation). All 12 survivors (mean age 22.4 +/- 5.7 months) were clinically evaluated and had echocardiography performed at 14.8 +/- 4.9 months after the operation. All were asymptomatic. Echocardiogram revealed a residual small atrial septal defect (1 case), small ventricular septal defect (1 case), mild supravalvar neopulmonary stenosis (2 cases), bicuspid neoaortic valve without stenosis (2 cases), dilated neoaortic sinus of Valsalva (6 cases, 50%) and mild neoaortic insufficiency (11 cases, 91.7%). The left ventricular function was hyperdynamic after pulmonary artery banding and significantly decreased to normal level at the time of study (shortening fraction of 43.8 +/- 10.7 vs 29.2 +/- 3.8%, respectively, p = 0.0005). The wall thickness was significantly increased after pulmonary artery banding and decreased overtime (0.48 +/- 0.08 vs 0.32 +/- 0.05 cm, respectively, p < 0.0005). The left ventricular dimension was significantly increased both after pulmonary artery banding and at the time of study (2.06 +/- 0.42 vs 3.32 +/- 0.30 cm, respectively, p < 0.0005). The left ventricular mass was significantly increased after pulmonary artery banding and at the time of study (21.79 +/- 7.79 vs 33.08 +/- 7.40 g/m2, respectively, p = 0.0005). The mortality and morbidity of rapid two-stage arterial switch operation are low. However, long-term follow-up should be monitored.     

Regression of tricuspid regurgitation after two-stage arterial switch operation for failing systemic ventricle after atrial inversion operation

The cases of five patients with previous Senning (n = 4) or Mustard (n = 1) operations and failing systemic ventricles in whom banding of the pulmonary artery was performed as an interim step toward an arterial switch procedure are reported. The rise in the ratio of left to right mean systolic ventricular pressure, from 0.35 before operation to 0.90 during operation and 0.80 on the first postoperative day, caused a shift of the ventricular septum from a leftward to a midline or nearly midline position. This shift was associated with a reduction of tricuspid regurgitation. At a median interval of 5.1 months after pulmonary artery banding, the mean left ventricular posterior wall thickness had increased to 8.2 mm, versus 5 mm before operation, and the mean left ventricular myocardial mass index had increased to 90 gm/m2, versus 55.6 gm/m2 before operation. After the arterial switch operation, which was performed in four patients, the tricuspid regurgitation decreased to a trivial amount (n = 1) or disappeared completely (n = 3).     

Pulmonary artery banding with a novel percutaneously, bidirectionally adjustable device

Pulmonary artery banding is commonly performed as a palliative procedure in complex congenital heart disease, when pulmonary blood flow is increased. However, the hemodynamics may change postoperatively requiring readjustment of the band, which may necessitate a second operation. We report a new system for pulmonary artery banding which allows precise placement of the band intraoperatively, as well as bidirectional percutaneous adjustment of the band postoperatively. Via left lateral thoracothomy the new device was implanted without complications into a neonate with congestive heart failure due to tricuspid atresia (IIc) and coarctation of the aorta. Although optimal placement of the band had been achieved intraoperatively the band had to be tightened 25 h after the operation and released 85 h after the operation in order to optimize hemodynamics. The bidirectionally adjustable device for banding of the pulmonary artery is superior to previously used devices with either no or unidirectional adjustability of the band because it is safe and easy to implant and has the potential to reduce the number of reoperations associated with this type of procedure.     

Atypical stiff-person syndrome with spinal MRI findings, amphiphysin autoantibodies, and immunosuppression

We report a successful two-stage anatomic correction of D-transposition of the great arteries associated with complete atrioventricular canal without pulmonary obstruction, combining an arterial switch procedure and a two-patch repair for complete atrioventricular canal, after pulmonary artery banding in the neonatal period.     

Aortic coarctation and complex heart disease. Surgical treatment during the first year of life

During the five years 1990 through 1994, 70 neonates and infants had surgery for coarctation of the aorta. 30 patients with complex coarctation of the aorta underwent a first stage correction with supplementary ductus ligation and pulmonary artery banding as needed. The mode of surgical repair of coarctation was end-to-end anastomosis in ten patients and patch graft in 20 patient. Mean follow-up was 766 (range 3-1812) days. Mortality after primary procedure was 16.6% (5/30) and after secondary procedure 11.1% (2/18). Three surviving patient developed recoarctation, but only one patients needed re-operation. In spite of improvement in neonatal cardiac surgery, we conclude that a staged approach is still the current treatment in most cases of complex coarctation of the aorta.     

Surgical treatment of coronary artery-pulmonary artery fistula

We experienced 4 cases of left coronary artery-pulmonary artery fistula. Two cases had small fistulas associated with atherosclerotic coronary lesions, and the other 2 had large fistulas with aneurysmal enlargement. In the former 2 cases, ligation of the fistulas and closure of the opening of fistula into the pulmonary artery through pulmonary arteriotomy were performed together with coronary artery bypass grafting and left ventricular aneurysmectomy. In one of the latter 2 cases, the fistula arising from the anterior descending branch was ligated and the opening of fistula draining into the pulmonary artery was closed through pulmonary arteriotomy. In another case, both openings of the fistula into the anterior descending branch and the pulmonary artery were closed from inside through incision of the dilated fistula. In all 4 cases, operations were performed using cardiopulmonary bypass and retrograde coronary perfusion, which could afford good heart protection even in cases with coronary lesions and coronary steal phenomenon. All cases went an uneventful postoperative course. Postoperative angiograms showed disappearance of the fistulas in 3 cases. In one case, however, residual fistula was found because a fine fistula might be overlooked. In such a case with complicated fistulas with aneurysmal enlargement, fistulas should be examined carefully through incision of the enlarged anomalous vessels. In this paper, diagnosis, operative indication and treatment for coronary artery-pulmonary artery fistula were discussed.     

A new operative technique for pulmonary artery banding: adjustment of pulmonary artery bands by mitral valve flow velocity

We developed a new technique to adjust the pulmonary artery band at surgery by monitoring the mitral valve flow velocity, which is indirectly indicative of the pulmonary flow. We employed this technique for 10 consecutive patients aged from 5 days to 5 months (mean, 1 months) weighing from 2.7 to 4.4 kg (mean, 3.3 kg). Underlying disease was aortic coarctation or interrupted+ventricular septal defect in 7 patients, single ventricule in 1 patients and miscellaneous defects in 2 patients. The pulmonary artery was exposed through a left lateral thoracotomy and a 3 mm wide Teflon tape was placed around the main pulmonary artery. The transducer of the Doppler echocardiography was placed along the left sternal border. The band was tightened gradually until the maximum velocity of the mitral valve flow decreased to around 70% of the previous level. During banding procedure, arterial oxygen saturation, heart rate and left ventricular contractility were monitored continuously. If bradycardia, unacceptable hypoxemia or ventricular dysfunction occurred, the band was released. The mitral valve flow velocity decreased rapidly by just a little additional tightness of the band between the range of 50% to 80% of the previous level. This technique enabled a very fine adjustment (less than 0.5 mm plication) and postoperative management has become very easy. Although there is a limitation of this technique that monitoring of the mitral valve flow velocity cannot be applied to the patients with significant interatrial shunt or mitral regurgitation, we conclude that this technique is simple and useful to obtain the optimum constriction of the pulmonary artery with excessive pulmonary blood flow.     

Hemodynamics in sepsis: its relationship to pulmonary complication

Serial clinical observations and hemodynamic measurements were made on seventeen patients with sepsis and ten control cases. Control patients were slightly hypovolemic immediately after operation. In the eleven high flow patients, that is, those with the initial cardiac index higher than 3.0 liters/min/m(2), we observed characteristic hemodynamics with lowered vascular resistance, higher cardiac output and hypotension. The anomalous vascular resistance began to return to normal approximately 48 hr after operation. In three cases pulmonary complication developed at this time and therefore pulmonary artery pressure and/or pulmonary wedge pressure exceeded the normal range. In these cases treatment was aimed at lowering the pulmonary pressure. Pulmonary complication was improved when these pressures returned within normal range. In the seven low flow patients, that is, those with cardiac index lower than 2.9 liters/min/m(2), we observed decreased cardiac index with increased systemic and pulmonary vascular resistance. In these cases pulmonary complication appeared to develop by increased permeability of the pulmonary capillary wall. Elevated pulmonary artery pressure might accelerate the progress of the complication. Monitoring and controlling pulmonary artery pressure and pulmonary wedge pressure were effective in prevention and treatment of pulmonary complication in this group.     

Subaortic obstruction and the Fontan operation

Systemic outflow tract obstruction in the heart with a functional single ventricle promotes myocardial hypertrophy, and this has been shown to be an unequivocal risk factor for poor outcome at Fontan procedure. Such systemic outflow tract obstruction may be congenital or acquired. Those factors contributing to acquired systemic outflow tract obstruction in those patients with a double-inlet left ventricle, a rudimentary right ventricle, and a discordant ventriculoarterial connection include the size of the ventricular septal defect, previous pulmonary artery banding, and other volume unloading surgical procedures. Staging with a bidirectional cavopulmonary connection and construction of a proximal pulmonary artery-aortic connection or ventricular septal defect enlargement has neutralized this factor.     

Variation in the size and distensibility of the pulmonary arteries in d-transposition of the great arteries

The relative size of the main pulmonary artery was determined from cineangiograms of 117 patients with d-transposition of the great arteries by calculating the ration between the diameters of the main pulmonary artery and aorta. The pulmonary artery was largest in patients with ventricular septal defect or patent ductus arteriosus, or both, because of increased pulmonary arterial pressure and flow. In patients with an intact ventricular septum or with left ventricular outflow tract obstruction, or both, the main pulmonary artery was approximately the size of the aorta. Two cases of d-transposition and gross dilatation of the main pulmonary artery and hypoplastic first branch pulmonary arteries are presented. In these cases the ratio between the diameters of the main pulmonary artery and aorta was greater than in any of the other 117 cases studied...     

Coronary-to-pulmonary artery shunts via the bronchial artery: analysis of cineangiographic studies

Coronary-to-pulmonary artery shunts via the bronchial artery (CA-BA-PA shunts) were observed in 16 of 2,922 consecutive patients who underwent selective coronary cineangiography. Underlying diseases included Takayasu arteritis (n = 8), chronic pulmonary inflammatory diseases (n = 4), pulmonary thromboembolism (n = 2), pulmonary artery tumor (n = 1), and tetralogy of Fallot with pulmonary atresia (n = 1). Ventilation-perfusion scans were available in 15 of the 16 patients. Mismatched defects were identified in 11 patients, and matched defects were identified in four. Bronchial-to-pulmonary artery shunts were detected on selective bronchial angiograms and/or thoracic aortograms in 13 patients. Feeding arteries of the CA-BA-PA shunts included left atrial branches (n = 13), right sinus node branches (n = 7), left sinus node branches (n = 2), right conal branch (n = 1), left conal branch (n = 1), and posterolateral branches (n = 2). These coronary branches seemed to serve as collateral vessels from a coronary arterial system with a higher pressure to a pulmonary arterial system with a lower pressure in conditions of decreased pulmonary flow or in cases of chronic pulmonary inflammatory disease. The importance of the coronary artery along with other superior thoracic collateral networks in contributing to the development of shunts to the pulmonary artery is underscored.     

Truncal valvoplasty for post-operative truncal valve regurgitation of truncus arteriosus: a case report

The surgical treatment for truncal valve regurgitation is still controversial in patients with truncus arteriosus. A two-year-old girl with complaints of low weight gain and tachypnea was referred for treatment of truncal valve regurgitation. She had undergone an emergency pulmonary artery banding for severe congestive heart failure due to truncus arteriosus-type I at six months of age. This anomaly had been corrected by Barbero-Marcial method at seven months of age. But the truncal valve regurgitation started appearing at sixteen months of age with the progression of the stenosis of the pulmonary artery orifice and the right ventricular outflow tract regurgitation. Echo cardiography and cineangiography revealed the truncal valve to be bicuspid, and the regurgitation severe, especially through the prolapsed left sided cusp. The truncal valve was repaired by commissural suspension method, and the right ventricular outflow tract reconstructed with patch angioplasty of the pulmonary artery orifice and Carpentier-Edwards pericardial Bioprosthesis (19 mm). The post-operative course was uneventful. One year after, truncal valve regurgitation is small by color Doppler study. We conclude that valvoplasty is to be considered as the first choice of treatment for truncal valve regurgitation.     

Multiple ventricular septal defects: how and when should they be repaired?

BACKGROUND: Congenital heart lesions with multiple ventricular septal defects remain a surgical challenge. Traditional approaches often rely on either ventriculotomy for exposure or palliation with pulmonary artery banding. However, indications for repair versus palliation and for various approaches to surgical exposure are not clearly defined. METHODS: From July 1992 to January 1998, 45 patients with multiple (>/=2) ventricular septal defects (37 with associated lesions) underwent surgery. Median age was 86 days; all but 4 patients were infants. The mean number of defects was 3.7, and almost half of the patients had more than 3 defects. Apical muscular defects were present in 62% of patients. Thirty-one patients underwent primary complete repair through a right atriotomy or trans-semilunar valve approach (group 1), 8 had palliation (group 2), and 6 underwent complete repair after prior palliation elsewhere (group 3). No patient had a ventriculotomy. RESULTS: One early death occurred in a group 1 patient. Four patients who had had palliation (50%) underwent early reoperation for pulmonary artery band revision because of failure to thrive or band removal after spontaneous closure of the defects. At follow-up (median 22 months), there was 1 death in a group 2 patient (palliation) and 1 other group 2 patient required cardiac transplantation. The only late reoperation was for removal of the pulmonary artery band and closure of multiple apical defects in a group 2 (palliation) patient. No patients who underwent repair have hemodynamically significant residual defects. CONCLUSIONS: In our experience, palliation of multiple ventricular septal defects is associated with greater morbidity than primary repair. Multiple defects can almost always be repaired adequately in early infancy without ventriculotomy, although "Swiss-cheese" septum may be an indication for palliation.     

Total cavopulmonary connection for grown diminutive pulmonary artery after staged Blalock-Taussig shunt

We report a two-year-old girl with asplenia, [A, L, L] DORV, pulmonary atresia, common AV valve, PDA, and TAPVC, who successfully underwent total cavo pulmonary connection (TCPC). Deep cyanosis was pointed out since birth. Cardiac catheterization performed on the sixth day after birth revealed a diminutive pulmonary artery tree of which PA index was 41 mm2/m2. Left modified Blalock-Taussig shunt was created at 27 days of age. The PA index increased to 282 mm2/mm2, but disparity in diameter between the left and the right pulmonary artery was yielded by PDA subsidence. Therefore additional contralateral B-T shunt was made at one year of age. Follow-up cardiac catheterization at 28 months of age showed well developed pulmonary artery; PA index of 460 mm2/m2, right pulmonary resistance (Rp) of 3.49 units, left Rp of 2.33 units, and estimated total Rp was 1.39. According to study, bidirectional Glenn procedure or TCPC was indicated. Considering necessity of urgent repair of common pulmonary vein obstruction, regurgitation of the common atrio-ventricular valve and pulmonary artery stenosis, TCPC was performed with concomitant repair of the associated lesions. Severe butterfly-figure stenosis of the central PA was augmented by anastomosing both the left SVC and the left-sided atrium. In conclusion, diminutive pulmonary artery could be adequately grown by phase-in Blalock-Taussig shunts. Pulmonary blood flow scintigraphy was thought to be useful for estimation of pulmonary resistance in such cases with different pulmonary resistance between right and left PA.     

Transposition of the great arteries. Its anatomical correction. Our initial experience

Arterial switch correction of transposition of the great arteries has been performed without mortality on 8 children, 7 neonates and one 6 years old child, at our institution. One infant and the 6 year old child has previous pulmonary artery banding and modified Blalock-Taussig shunts. The coronary cuffs were anastomosed to the neo-aorta using the "trap-door" technique described by Mee and in seven the Lecompte manoeuvre was used. Postoperative management included treatment for persistent pulmonary hypertension and left ventricular dysfunction. All patients were discharged in satisfactory condition, and continue to do so at a maximum follow-up of ten months. These results encourage us to elect the arterial switch procedure as the primary treatment of transposition of the great arteries.     

Chondrosarcoma of the pulmonary artery

Primary sarcomas of the large vessels are rare. They are observed most commonly in the pulmonary artery. Up to now 28 cases of primary sarcoma of the pulmonary artery have been described in the literature. The clinical features of this disease which presents considerable diagnostic difficulties are dominated by signs of cor pulmonale. In the present paper a primary chondrosarcoma of the pulmonary artery is presented.     

Surgical treatment of solitary or common heart ventricle

On the basis of 24 cases reported in the literature and 2 personal observations of the authors the indications for surgical correction of a single ventricle of the heart are discussed. Two different surgical techniques are described: 1) haemodynamic correction of the defect comprising a closure of the venous atrioventricular valve, oversewing the proximal pulmonary artery, and anastomosing the right atrium and the distal pulmonary artery; 2) radical correction--creation of the ventricular septum. The variants of the disease permitting a radical correction are indicated, the results of surgery, the causes of complications and mortality are discussed.     

Blood grouping problems

From 1965 to 1974, 53 children with coarctation of the aorta (COA) and an associated ventricular septal defect (VSD) underwent cardiac catheterization. Thirty-one patients presented with congestive heart failure. Twenty-five of 27 patients (92%) who underwent cardiac catheterization under age 3 months had either systemic hypertension, a systolic gradient across the coarctation greater than 20 mm Hg or both. Pulmonary hypertension was present in all 25 patients. COA repair was performed in 39 patients and there were seven deaths. Of the 32 survivors, 23 have no residual gradient; six are normotensive but have a mild residual gradient; three are hypertensive or have a gradient greater than 20 mm Hg. Repair of the VSD or pulmonary artery banding has been performed in 11 of 44 patients who survived infancy. Spontaneous closure of the VSD has occurred in three cases and 25 patients have a small VSD that does not warrant surgical repair. Surgical repair of COA during infancy may be unavoidable but conservative medical management of the associated VSD is often successful.     

Relevance of width measurements of the right pulmonary artery descending branch for detection of pulmonary hypertension in chronic obstructive lung diseases (author's transl)

363 patients with chronic obstructive lung diseases were examined regarding the relation between pulmonary hypertension and width of the right descending branch of the pulmonary artery (RDB). There was found a high significant correlation between mean pulmonary artery pressure at rest and diameter of the RDB. The width of RDB was not so close correlated with pulmonary artery pressure during exercise. If the diameter of the RDB was 19 mm and more, a manifest pulmonary hypertension was stated nearly always. Missing this radiological sign doesn't exclude pulmonary hypertension. The method will be useful for screening a risk group "pulmonary hypertension". All cases selected by this method aught to be examined by means of right heart catheterization with the floating technic.     

Utility of psychophysiological measurement in the diagnosis of posttraumatic stress disorder: results from a Department of Veterans Affairs Cooperative Study

OBJECTIVE: Predicting postrepair right ventricular/left ventricular pressure ratio has prognostic relevance for patients undergoing total repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. To this purpose, we currently rely on 2 novel parameters: (1) preoperative total neopulmonary arterial index and (2) mean pulmonary artery pressure changes during an intraoperative flow study. METHODS: Since January 1994, 15 consecutive patients (aged 64 +/- 54 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals were managed according to total neopulmonary arterial index. Seven patients with hypoplastic pulmonary arteries and a total neopulmonary arterial index less than 150 mm(2)/m(2) underwent palliative right ventricular outflow tract reconstruction followed by secondary 1-stage unifocalization and ventricular septal defect closure. The other 8 patients with a preoperative index of more than 150 mm(2)/m(2) underwent primary single-stage unifocalization and repair. The ventricular septal defect was closed in all cases (reopened in 1). In 9, such decision was based on an intraoperative flow study. RESULTS: Patients treated by right ventricular outflow tract reconstruction had a significant increase of pulmonary artery index (P=.006) within 22 +/- 6 months. Repair was successful in 14 cases (postrepair right ventricular/left ventricular pressure ratio = 0.47 +/- 0.1). One hospital death occurred as a result of pulmonary vascular obstructive disease, despite a reassuring intraoperative flow study. Accuracy of this test in predicting the postrepair mean pulmonary artery pressure was 89% (95% CI: 51%-99%). At follow-up (18 +/- 12 months), all patients are free of symptoms, requiring no medications. CONCLUSIONS: The integrated approach to total repair of pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals by preoperative calculation of total neopulmonary arterial index, right ventricular outflow tract reconstruction (when required), and intraoperative flow study may lead to optimal intermediate results.