Surgical treatments of nonconfluent pulmonary arteries with congenital cardiac defects]

Pulmonary artery angioplasty or reconstruction was performed in seven patients with nonconfluent pulmonary arteries and congenital cardiac defects. Age of these patients were ranged from 6 months to 41 years old. Five of them had pulmonary truncal atresia and complex cardiac anomalies. Two of these five patients demonstrated nonconfluent pulmonary arteries due to deformities at ductal insertion of pulmonary arteries. Three patients had had previous systemic to pulmonary artery shunt operations which caused pulmonary artery distortions. Other two patients had intrapulmonary arterial obstructions due to pulmonary artery thrombosis. Patch pulmonary artery plasty was carried out in three patients, dilatation of severe stenotic pulmonary artery was done in one patient simultaneously with pulmonary valvotomy. Central shunt operation was added in one patient with the pulmonary artery which was unable to be reconstructed. Last two patients underwent intrapulmonary artery reconstruction with the rolled pericardial graft. Hospital death occurred in one patient with unproperly increased pulmonary blood flow by central shunt. Average follow-up period of these six survivors after operation was 1.4 +/- 0.8 years. As definite repairs, two patients had Fontan operation, two patients had right ventricle to pulmonary artery reconstruction. And remaining two patients are still to be followed until sufficient growth of pulmonary artery suitable for Fontan operation.     

Right pulmonary artery development after modified Blalock-Taussig shunt (MBTS) in infants with pulmonary atresia, VSD and confluent pulmonary arteries

The ideal palliation for infants with pulmonary atresia, ventricular septal defect and confluent pulmonary arteries should promote normal development of the pulmonary artery branches. In 26 patients who survived a modified Blalock-Taussig shunt (MBTS) in the first year of life, the right pulmonary artery was measured before and after operation by two dimensional (2D) echocardiography. In each patient its size was compared to the normal value for the same body surface area. The patients were divided according to this ratio and according to the real size (mm). Although increased in all cases but two, the size of the right pulmonary artery remained remarkably less than normal in patients with an initially small right pulmonary artery. In view of these results, early palliative enlargement of the right ventricular outflow tract is probably advisable in infants with diminutive pulmonary arteries who do not show adequate pulmonary branch development after MBTS.     

Reconstruction of nonconfluent pulmonary artery using the retroaortic innominate vein for a single ventricle

The innominate vein usually courses anterior to the aortic arch, where it joins the right brachiocephalic vein to form the superior caval vein. A retroaortic innominate vein is an uncommon finding in patients with congenital heart disease. We report a patient with a single ventricle, single atrium, pulmonary atresia, nonconfluent pulmonary artery, persistent left superior caval vein, absent inferior caval vein (azygos connection), right aortic arch, and retroaortic innominate vein. His innominate vein took an anomalous course. The right brachiocephalic vein crossed from right to left underneath the aortic arch and formed a left superior caval vein with the left brachiocephalic vein and the azygos vein. We reconstructed the nonconfluent pulmonary artery using the retroaortic innominate vein and then performed a total cavopulmonary shunt when he was 32 months of age. Thereafter, a total cavopulmonary connection was carried out at age 42 months.     

Modified Blalock-Taussig shunt with compensatory properties

Determination of the proper length of the tubular prosthesis is a major issue when performing a systemic-pulmonary artery shunt. The procedure is simplified by using a prosthesis with accordionlike properties. This was demonstrated in 7 consecutive infants with complex congenital heart defects, in whom systemic-pulmonary artery shunts were placed without early or late complications.     

Dilatable banding of a Blalock-Taussig shunt

Dilatable banding has been used in various situations. Sometimes Blalock-Taussig shunt banding is performed to prevent pulmonary overcirculation. Recently several reports have described dilatable pulmonary artery banding. We modified these methods for flow control of a Blalock-Taussig shunt. We report the case of a neonate with truncus arteriosus in which this technique was used.     

Long-term palliation with the classic Blalock-Taussig shunt

Eighty-five patients received a classic Blalock-Taussig shunt between 1973 and 1986. Their age range was 1 day to 9.3 years and their median age was 4 months. Forty-one percent (35/85) were less than 1 month of age. The basic operative technique was unchanged throughout the time period. The subclavian artery opposite the side of the arch was used in 89% (79/88) of the patients. All anastomoses were done with monofilament suture and there was a tendency toward smaller suture material (7-0) in the latter years. All anastomoses except one were done with an interrupted suture technique. The operative mortality rate was 4.7% (4/85) and was not statistically related to age, diagnosis, or year of operation. Palliation was considered to be satisfactory until either a second shunt or a premature corrective operation were necessary. Seven patients required a second shunt and three, a premature corrective operation. The mean time between the initial shunt and the second procedure, either a second shunt or a corrective operation, was 2.9 years and 2.4 years, respectively. Twenty-five patients have had an elective corrective operation and the mean interval to that procedure was 3.9 years. Two years after the operation, 97% of patients older than 1 month of age at operation remain in well-palliated condition, as do 87% of those less than 1 month of age. At 4 years, 87% of those older than 1 month and 54% of those less than 1 month of age continue to be in well-palliated condition. The classic Blalock-Taussig shunt provides excellent palliation at a low operative mortality for virtually all patients for a minimum of 2 years. It will provide adequate pulmonary blood flow for most patients for an extended period of time beyond 2 years.     

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 neccesity 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.     

Nitric oxide: lifesaving measure for pulmonary vasospasm after modified Blalock-Taussig shunt

Pulmonary vasospasm and hypertension may occur after repair or palliation of congenital cardiac defects, and can be fatal in spite of conventional treatment. Nitric oxide has been shown to improve pulmonary hypertension unresponsive to conventional measures after a variety of repairs, but use has infrequently been reported after palliative systemic to pulmonary artery shunts. We report a case of pulmonary hypertension and life threatening desaturation after a modified Blalock-Taussig shunt that responded rapidly to inhaled nitric oxide. Clinical use, further study, and prospective analysis of prophylactic use of nitric oxide appear warranted.     

Modified Fontan operation for a single ventricle with a nonconfluent pulmonary artery]

A 8-year-old boy with a double inlet right ventricle with a non-confluent pulmonary artery was operated on with a modified Fontan operation. He had right isomerism, right aortic arch, bilateral superior caval veins, and left-sided inferior caval vein. Hepatic veins were separately drained to the right-side atrium. Left Blalock-Taussig shunt and right central shunt operations had been previously performed. Firstly, we had reconstructed the central pulmonary artery with a 16 mm porcine pericardial roll to unify the nonconfluent pulmonary arteries. Secondly, about 2 months after the first operation, we performed a modified Fontan operation. Systemic venous return from the inferior caval vein and the hepatic veins were drained to a reconstructed pericardial roll with an intraatrial Gore-Tex graft, and bilateral superior caval veins were also anastomosed to the roll. The structure of the pulmonary arterial system is one of the most important factors to determine the outcome of a modified Fontan operation. Even if the central pulmonary artery is absent, however, a modified Fontan operation is applicable for the patient whose peripheral pulmonary arteries have enough growth.     

An infected pseudoaneurysm following a modified Blalock-Taussig shunt

A two-month-old male infant with tetralogy of Fallot underwent a right-sided modified Blalock-Taussig shunt using a 4 mm expanded polytetrafluoroethylene graft through a right thoracotomy. Five months later, the patient developed otitis media, followed by repeated relapses of pneumonia and fever of unknown origin. Multidetector-row computed tomography and angiography, performed at 12 months of age, revealed a pseudoaneurysm of the subclavian artery at the insertion of the modified Blalock-Taussig shunt. After 20 days of antibiotic therapy, the pseudoaneurysm and infected graft were successfully resected through a median sternotomy approach. This report describes the treatment strategy of this rare but potentially fatal complication after a modified Blalock-Taussig shunt operation.     

Division of modified Blalock-Taussig shunt at correction avoids distortion of the pulmonary artery

BACKGROUND: Modified Blalock-Taussig (BT) shunt causing pulmonary artery distortion has been reported. This distortion may get worse after a corrective operation if the BT shunt is ligated, rather than divided. In this study we examined whether division of modified BT shunt at the time of corrective operation would allow pulmonary artery growth and avoid further distortion. METHODS: Fifteen patients who had modified BT shunts and subsequently had corrective operations performed by one surgeon between January 1980 to December 1990 were analyzed. The median time from the BT shunt to corrective operation was 46.3 months (range, 3 to 119 months). At the time of corrective procedure, the BT shunt was divided and metal clips were used to occlude and mark each end. At follow-up a chest roentgenogram was obtained and the distance between the two clips was measured. RESULTS: In all 15 patients measured sequentially the distances between the two clips increased steadily. CONCLUSIONS: Division of BT shunt at the time of corrective procedure reduces pulmonary artery distortion.     

Blalock-Taussig shunt and pulmonary artery angioplasty for isolated unilateral absence of the right pulmonary artery; report of a case

A 7-month-old girl was referred to us for further examination of absence of the right pulmonary artery. She had no symptom at that time. Diagnosis was made by chest computed tomography (CT) and cardiac catheterization. Though the size of the right pulmonary artery was not apparent, right pulmonary vein wedge angiography revealed the distal portion of right pulmonary artery sufficient for surgical repair. But the distance between the pulmonary trunk and right pulmonary artery was too far to perform direct anastomosis, and some systemic collaterals had already been recognized. Pulmonary vasculature was also not inadequate. Therefore we planned a palliative procedure. At the age of 7 months, right modified Blalock-Tausig shunt using 5mm expand-polytetrafluoroethylene (ePTFE) tube and angioplasty of the distal portion of right pulmonary artery using autologous pericardium roll with Dacron mesh was performed. Postoperative course was uneventful. She has been followed up for 6 months after the palliation. In the near future the completion of the definitive repair will be considered.     

Very small pulmonary arteries: central end-to-side shunt

Between 1980 and 1989, 28 patients with pulmonary atresia, ventricular septal defect, and very small pulmonary arteries with major aortopulmonary collateral arteries underwent direct central end-to-side shunts as staging procedures. Age range was 2 months to 32 years, with 19 patients less than 1 year of age. Pulmonary artery diameters ranged from 1 to 4 mm, with 24 less than 3 mm. Two patients (7%; 70% confidence limits, 2% to 16%) died after the shunt, and there were two further deaths after subsequent staging or correction. Acute shunt complications included congestive cardiac failure (mild to moderate, n = 8; severe, n = 3) and endocarditis (n = 1). Proximal right pulmonary artery stenoses have occurred in 75% of patients and left pulmonary artery stenoses, in 50%. Satisfactory pulmonary artery growth was achieved, however, in 16 of 24 hospital survivors investigated postoperatively. Twelve patients have proceeded through unifocalization to biventricular repair (in 2 the ventricular septal defect patch was subsequently fenestrated) with one death (8.3%; 70% confidence limits, 1% to 25%). Eight patients are still in staging, and 4 have been excluded from the program because of inadequate unifocalization. The direct central end-to-side shunt has proven satisfactory in attaining pulmonary artery growth in patients with very small central pulmonary arteries.