Congenital aortopulmonary artery fistulas combined with bilateral coronary artery fistulas

We describe an extremely rare case of "congenital aorto-pulmonary artery fistulas" combined with bilateral coronary artery fistulas associated with clinically significant left-to-right shunt. A multi-detector row computed tomographic scan revealed that tortuous anomalous vessels arising from the aortic arch, left anterior descending coronary artery, diagonal branch, and right coronary artery were connected to the main pulmonary trunk. After surgical intervention, the systemic-to-pulmonary flow ratio nearly normalized from 2.02 to 1.05. We describe the case and speculate as to the embryological pathogenesis of these unique fistulas.     

Indication for systemic-pulmonary artery shunts guided by two-dimensional and Doppler echocardiography: criteria for patient selection

From June, 1983, to February, 1987, 91 patients with pulmonary outflow tract obstruction underwent a systemic-to-pulmonary artery shunt without an invasive study. The surgical indication was based on two-dimensional (2-D) and Doppler echocardiography only. In 82 patients (90.1%), 2-D and Doppler echocardiography allowed correct assessment of the intracardiac anatomy and of the morphology of the pulmonary arteries, pulmonary veins, and aortic arch. The aortic arch anatomy was misdiagnosed in 6 patients (6.6%) who underwent a modified instead of a classic Blalock-Taussig shunt. In 3 patients (3.3%), the indication for the systemic-pulmonary artery shunt was inappropriate; in 1, operative death resulted from the incomplete diagnosis. After the preselection of patients based on knowledge of the anatomical patterns, a systemic-pulmonary artery shunt may be performed without cardiac catheterization if these rules for definitive patient selection are followed: (1) accurate clinical assessment to identify the patients with restricted pulmonary blood flow; (2) precise intracardiac diagnosis by 2-D and Doppler echocardiography; and (3) definitive 2-D echocardiographic visualization of the pulmonary venous connection and the confluence of the pulmonary arteries.     

Venovenous extracorporeal membrane oxygenation in neonatal systemic artery-to-pulmonary artery shunt operations using a double lumen catheter

The right atrium of the neonate may be too small for direct insertion of 2 venous catheters during intraoperative life support. We inserted a double lumen catheter into the right atrium, and venovenous (V-V) extracorporeal membrane oxygenation (ECMO) was instituted. The patient's arterial oxygen saturation was maintained at 70% to 90%, and hemodynamic stability was obtained during V-V ECMO. V-V ECMO using a double lumen catheter can be easily established in a small neonate, and is an effective support technique for untolerable hypoxemia during systemic-to-pulmonary artery shunt operations.     

Systemic-to-pulmonary artery shunt thrombosis in a neonate with factor V Leiden mutation

We present the case of a newborn male with D-transposition of the great arteries, ventricular septal defect, and subpulmonary stenosis who experienced two episodes of systemic-to-pulmonary artery shunt thrombosis. Hematologic evaluation revealed heterozygous factor V Leiden mutation. This report emphasizes the importance of evaluation for inherited coagulation disorders in pediatric patients with unexpected thrombotic complications.     

First-stage angioplasty for a single ventricle with pulmonary artery coarctation

We report first-stage palliation consisting of pulmonary artery angioplasty and a systemic-to-pulmonary shunt using a minimized cardiopulmonary bypass technique performed with modified ultrafiltration for two patients with a single ventricle, pulmonary atresia, patent ductus arteriosus, and pulmonary coarctation during early infancy. Postoperative early extubation (15 and 18 h) and bilateral pulmonary artery growth before the second stage (Nakata indexes, in mm²/m²: right 94, left 53; and right 209, left 70) were experienced. Less-invasive pulmonary artery continuity reconstruction at an early stage is advantageous for low-weight patients with a single ventricle and pulmonary coarctation.     

Modified Norwood procedure with a handmade down-sizing valved right ventricle-to-pulmonary artery conduit

First-stage palliation of hypoplastic left heart syndrome has been revolutionized by the recent introduction of a right ventricle-to-pulmonary artery (RV-PA) conduit as an alternative to a systemic-to-pulmonary shunt. However, most conduits are unvalved, and the use of valved xenografts was abandoned during the early era of this operation. We performed a successful modified Norwood operation in a 2-month-old infant with aortic atresia and ventricular and atrial septal defects using a hand-made down-sizing valved graft as an RV-PA conduit. The postoperative course was uneventful with well-balanced pulmonary and arterial perfusion. We believe that minimization of the regurgitant volume from an unvalved prosthetic conduit by utilizing this modification might be of benefit in this particular group of patients.     

Primary antiphospholipid syndrome: a cause of catastrophic shunt thrombosis in the newborn.

This is a unique report of systemic-to-pulmonary artery shunt thromboses secondary to primary antiphospholipid syndrome and antithrombin III deficiency in a neonate with cyanotic congenital heart disease. This infant with tricuspid atresia experienced thromboses of two modified Blalock-Taussig shunts en route to a bidirectional cavo-pulmonary shunt and potential future Fontan operation. Chronic warfarin anticoagulation has prevented additional thrombo-embolic events.     

Outcome of patients with cyanotic congenital heart disease undergoing a second systemic-to-pulmonary artery shunt

BACKGROUND: Multiple systemic-to-pulmonary artery shunts often represent a deviation from the initial management strategy for cyanotic congenital heart disease. This study analysed the outcome in patients undergoing a second shunt. METHODS: Between 1965 and 1998, 80 patients required a second shunt. Patient age ranged from 11 days to 11.2 years at the initial shunt, and 4 months to 25.6 years at the second shunt. The interval between shunts ranged from 11 days to 19.6 years. RESULTS: The patient survival rates at 1, 6, 12, 60 and 120 months following the second shunt were 95.0 +/- 2.4%, 92.5+/-3.0%, 87.4+/-3.7%. 82.4+/-4.5% and 74.8+/-5.8%, respectively. Although excess pulmonary flow was not encountered after the second shunt, mode and incidence of morbidity were otherwise similar to the first one. Between 9 months and 7.5 years after the second shunt, 24 patients underwent successful definitive repair. Multivariable analysis identified pulmonary atresia (p=0.027) and a short (<1 year) interval between the two shunts (p=0.034) as the independent risk factors for long-term shunt failure. Single ventricle physiology (p=0.002) and a central approach for the second shunt (p=0.016) were independent risk factors for lack of application, or failure of intracardiac definitive repair. CONCLUSIONS: A significant limitation in longevity and quality of life is common in patients requiring a second shunt, especially those associated with pulmonary atresia, the single ventricle physiology heart, or ungraftably hypoplastic contralateral branch pulmonary artery to the first shunt. Since excess pulmonary blood flow leading to congenital heart failure and/or ventricular diastolic dysfunction is unlikely, all patients who preclude definitive repair due to decreased pulmonary blood flow even after the first shunt should be shunted again.     

Creation of a Systemic-to-Pulmonary Artery Shunt By Use of an Internal Mammary Artery

A bstract The creation of a systemic-to-pulmonary artery shunt by use of the internal mammary artery (IMA) in a 2-year-old boy with complex congenital heart defects and diminutive pulmonary arteries is described. This procedure may offer advantages over standard methods in selected patients with extremely small pulmonary arteries. The pliability, favorable anatomical position, and growth capability of the IMA reduce chances for complications, such as stenosing, kinking, and stretching of the pulmonary artery, which can cause it to become distorted, making further definitive repair difficult.     

Interim mortality in infants with systemic-to-pulmonary artery shunts

BACKGROUND: This retrospective review examines the risks and causes of death in infants with aortopulmonary shunts between the time of hospital discharge and planned reintervention. METHODS: From January 1991 through December 2000, a total of 146 infants aged 60 days or less underwent placement of systemic-to-pulmonary artery shunts and were discharged from the hospital alive. Inpatient, outpatient, and autopsy records were reviewed. RESULTS: Indications for surgery were single-ventricle anatomy in 90 cases and complex double-ventricle anatomy in 56. Of the patients, 21 (14%) died after discharge and before further planned surgery. Of these 21 infants, 17 (81%) were clinically doing well before sudden death. Autopsies were obtained in 15 cases and attributed the cause of death to shunt thrombosis in 5 infants (33%), myocardial infarction in 2 (13%), and pneumonia or lung disease in 3. Five autopsies were nondiagnostic. The mortality of patients discharged on aspirin (11.1%) was almost identical to that of patients discharged on no anticoagulation (12.3%). Four infants with sudden death had been notably irritable for 24 to 48 hours before death. CONCLUSIONS: There is a significant incidence of sudden death among infants who have undergone shunting. Death may be preceded by unexplained irritability, and such symptoms should therefore be carefully evaluated. Autopsy-proven shunt thrombosis is one of the leading causes of interim sudden death, and aspirin therapy may not be helpful. Options to reduce interim mortality include alternative regimens of anticoagulation (such as low-molecular weight heparin), alternative conduit material, and earlier reoperation.     

Ascending Aorta to Right Pulmonary Artery Interposition Shunt in Critically III Infants

In spite of a trend toward earlier complete repair, some neonates and infants with complex cyanotic heart disease continue to require interim palliation with systemic-to-pulmonary artery shunts. A variety of shunt procedures have been proposed, each with inherent advantages and disadvantages. We have found a prosthetic interposition shunt between the ascending aorta and right pulmonary artery (AA-RPA) to be effective in very young infants with small vessels. Over a 15-year period, 51 patients, mean weight 3.33 kg and mean age 59 days, underwent this procedure with a 13% perioperative mortality and a 78% 2-year overall shunt patency rate. We conclude that the AA-RPA interposition shunt is a safe, effective procedure in these infants. (J Card Surg 1994;9:37–42)     

Norwood procedure without circulatory arrest.

In the Norwood procedure for hypoplastic left heart yndrome, the distal descending thoracic aorta was cannulated just superior to the diaphragm through median sternotomy. In combination with cerebral perfusion through the graft anastomosed to the innominate artery, which was used as a systemic-to-pulmonary shunt later, this technique enabled us to completely avoid circulatory arrest and deep hypothermia throughout the operation.     

Operative technique of systemic to pulmonary artery shunt and aortoplasty utilizing in aberrant subclavian artery

This paper presents a review of our experience of 3 cases of systemic to pulmonary artery shunt operation and 2 cases of subclavian flap aortoplasty using aberrant subclavian artery. The aberrant subclavian arteries were divided to release the vascular rings and were used as shunt grafts or reversed subclavian flaps. In case of shunt operation, this graft would be superior to the EPTFE graft of modified Blalock-Taussig shunt, from the point of view of the grafts' growth ability. In case of aortoplasty, if arch hypoplasia exists, this flap can repair it beyond the coarctation segment. When the aberrant subclavian artery is dissected, it should be avoided to use the contralateral subclavian artery, as it may cause cerebral damage because of the impairment of bilateral vertebral arterial circulation.     

Method of closure of the aorta-to-right pulmonary artery (Waterston) anastomosis during corrective operation

The integrity of the right pulmonary artery following a Waterston shunt procedure must be checked by catheterization and angiography prior to complete correction of tetralogy of Fallot. If the lumen of the right pulmonary artery is adequate, the shunt may be closed by direct suture. If there is relative kinking of the right pulmonary artery at the site of the anastomosis, the vessel should be inspected carefully at the time of shunt closure. The use of a small Dacron patch to close the shunt may avoid further kinking.     

Results of systemic-to-pulmonary artery anastomosis for tricuspid atresia with reduced pulmonary blood flow.

Fifty-six patients with tricuspid atresia and decreased pulmonary blood flow received a systemic-to-pulmonary artery anastomosis as a preliminary operation. Thirty-five had a Waterston shunt, 12 a Blalock-Taussig anastomosis, and nine various other procedures. The age at operation ranged from 2 days to 10 years (median 4.5 months). Pulmonary atresia was present in eight newborn infants. Four patients (7%), all less than 3 months old, died in hospital from persistent servere hypoxia despite the palliative procedure. By actuarial methods, 93% of the survivors were alive at and after one year (longest follow-up:9.8 years), while 83% had not required further palliation at and after four years from the first operation. None of the patients subsequently restudied had raised pulmonary pressure or resistances, and in most of them the pulmonary arterial tree was deemed adequate for the Fontan operation.     

Exsanguinating hemoptysis revealing the absence of left pulmonary artery in an adult.

Isolated absence of a pulmonary artery is an exceptional cause of massive hemoptysis. We report a 35-year-old woman with agenesis of the left pulmonary artery who presented with exsanguinating hemoptysis that prompted angiography with the aim to embolize the bleeding vessels selectively. The procedure could not be completed because of the presence of an anterior spinal artery branching from the aberrant systemic-to-pulmonary circulation. The patient successfully underwent an emergent pneumonectomy.     

Pulmonary artery configuration after palliative operations for hypoplastic left heart syndrome

Pulmonary artery architecture and symmetry after palliative operations for hypoplastic left heart syndrome may affect subsequent suitability for a modified Fontan operation. Two-dimensional echocardiography was used to measure pulmonary artery diameter and assess symmetry after two types of systemic-pulmonary artery shunts: modified right Blalock-Taussig shunt (14 patients) and central shunt (from underside of aortic arch gusset to pulmonary artery confluence) (14 patients). Age, weight, preoperative diameter of right and left pulmonary arteries (proximal, middle, and distal segments), and mean interval between preoperative and postoperative echocardiographic studies (20.2 +/- 4.4 days in the Blalock shunt group; 19.1 +/- 6.8 days in the central shunt group) were similar. Early postoperatively, patients with a Blalock shunt showed a significant decrease in the diameter of all pulmonary artery segments except the distal right pulmonary artery. The diameters tapered from distal right to distal left pulmonary artery in this group. Patients with the central shunt had a significant decrease in the diameter of all pulmonary artery segments. There were no significant differences when cross comparisons were made of the various pulmonary arterial segments in patients after a central shunt. Similar findings persisted in 19 patients from both groups who had a late postoperative echocardiogram (mean interval between studies = 271 days in the group of 10 patients with Blalock shunt and 167 days in the group of nine patients with a central shunt). In conclusion, the central shunt preserves pulmonary artery symmetry, which may be important in candidates for the Fontan operation in infancy.     

The infant with single ventricle and excessive pulmonary blood flow: results of a strategy of pulmonary artery division and shunt

BACKGROUND: The infant with a single ventricle and excessive pulmonary blood flow requires early protection of the pulmonary vascular bed to insure suitability for a subsequent Fontan procedure. The traditional approach, pulmonary artery banding, has had disappointing results. We have pursued an alternate strategy: division of the pulmonary artery, and placement of a systemic-to-pulmonary artery shunt. Potential sites of systemic outflow tract obstruction are simultaneously bypassed, by either a Damus-Kaye-Stansel, or modified Norwood procedure. METHODS: From January 1996 to June 2001, 22 infants were treated by this strategy. Patients with hypoplastic left heart syndrome were excluded. Median age was 18 days (range 2 days to 6 months). In addition to pulmonary artery division and shunt, 3 of 22 patients underwent a Damus-Kaye-Stansel procedure, and 13 of 22 patients underwent a modified Norwood procedure. RESULTS: There were no operative deaths, and one late death. Actuarial survival beyond 30 months was 90%. At follow-up catheterization in 22 patients, median transpulmonary gradient was 7 mmHg (range 4 to 18), and median pulmonary vascular resistance 1.9 Wood units (range 0.9 to 3.3). Twenty-one patients have undergone a subsequent bidirectional superior cavopulmonary connection, and 6 a Fontan procedure, with no deaths. No patient developed subaortic stenosis, or aortic arch obstruction. Neoaortic insufficiency was none or trivial in 12 patients, mild in 3, and moderate in 1. CONCLUSIONS: In patients with a functional single ventricle and excessive pulmonary flow, a strategy of pulmonary artery division and shunt, along with prophylactic bypass of systemic outflow obstruction, carries low operative and midterm mortality. It provides consistent protection of the pulmonary vascular bed, avoids subaortic stenosis and aortic arch obstruction, minimizes neoaortic insufficiency, and ensures suitability for progression along a Fontan pathway. These results provide a comparison for alternate strategies, including pulmonary artery banding.     

Pleuropulmonary actinomycosis associated with a systemic-to-pulmonary artery fistula and contralateral metastatic back mass

We report a case of systemic-to-pulmonary artery fistula associated with thoracic actinomycosis and with metastatic hematogenous dissemination to the soft tissues of the back. The difficulty in diagnosing thoracic actinomycosis may predispose to the increased incidence of hematogenous spread of this disease. Although resection of pulmonary tissue including the infectious mass was required in previous cases, resection of the pleural mass alone was curative in this patient when combined with penicillin therapy.     

Bovine mesenteric venous graft as an alternative conduit in patients with cyanotic heart disease with e-polytetrafluoroethylene graft failure caused by thrombophilic factor positivity after modified Blalock-Taussig shunt

Modified Blalock-Taussig shunt (MBTS) is a palliative operation for cyanotic congenital heart disease (CCHD) in patients for whom total correction is not appropriate. Many synthetic or biologic grafts have been proposed as alternative shunt materials. The use of a bovine mesenteric venous graft (BMVG) as a systemic-to-pulmonary artery shunt conduit without the administration of antiaggregant and anticoagulant has been proposed as a treatment for neonates with CCHD, but few reports address the importance of thrombophilic risk factors in MBTS and bovine venous graft as a shunt material. We used BMVG as a shunt material without any antiaggregant or antiplatelet regimen in 13 patients with CCHD, all of whom were candidates for MBTS and had thrombophilic risk factors assessed in our initial study. Early shunt failure occurred in the first 3 patients and was attributed to less surgical experience with this graft. No complications were attributable to graft material or surgery itself. In all cases functioning MBTSs were observed on follow-up. Our study results show that thrombophilic factors should be evaluated before the MBTS procedure. BMVG could be the choice of graft for use without the administration of antiaggregant and anticoagulants in patients with thrombophilic risk factors.