An experience of the modified Norwood's operation for hypoplastic left heart syndrome with aberrant origin of right subclavian artery and persistent left superior vena cava--the procedure without total circulatory arrest and cardiac arrest

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.     

Double outlet right ventricle with L-position of the aorta, D-loop, subaortic VSD, and pulmonary stenosis

We describe a case of double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis treated successfully with cardiopulmonary bypass. We consider the clinical history and angiocardiographic and surgical findings of this rare anomaly. We stress the difficulties of reconstruction of the outflow tract of the right ventricle, because of the anomalous pathway of the right coronary artery, the posterior situation of the pulmonary artery, and the abnormal anatomy present in the outflow tract of the right ventricle.     

Late re-interventions following arterial switch operations in transposition of the great arteries. Incidence and surgical treatment of postoperative pulmonary stenosis

Seventy-six patients were studied after arterial switch operation (ASO) between May 1977 and February 1992. Pulmonary artery reconstruction was initially performed by: conduit interposition in 5 patients, direct main pulmonary artery anastomosis and button patches in 60 patients, and pantaloon-like patch repair in 11 patients. Pulmonary stenosis developed in 17 patients (22%), requiring a total of 26 late re-interventions. Re-intervention was required in four out of five patients operated with pulmonary artery conduits, 11 out of 60 with a button patch repair and 2 out of 11 following pantaloon-type repair. In this series pulmonary artery stenosis (PS) involving the pulmonary valve occurred in 9/17 patients. Involvement of the pulmonary valve was related to the technique of pulmonary artery reconstruction. In these patients surgery is necessary. Balloon angioplasty can be a valuable tool when the stenosis is more distal. The incidence of PS was not influenced by the type of reconstruction or the use of Lecompte's maneuver.     

Role of neutrophil elastase in stress-induced gastric mucosal injury in rats

To evaluate the growth of a pulmonary trunk reconstructed without an extracardiac conduit, the hemodynamics and diameter of a new pulmonary trunk were measured in 5 patients from the right ventriculogram and MRI at postoperative follow-up periods. There were tetralogy of Fallot with pulmonary atresia in two patients, tetralogy of Fallot with single coronary in one, truncus arteriosus type I in one and transposition of the great arteries with ventricular septal defect and pulmonary stenosis in one. The age at operation ranged from 26 days to 4.5 years. The posterior wall continuity of the right ventricle and pulmonary artery was established by the direct pulmonary-right ventricular anastomosis in three patients and by the interposition of the left atrial appendage in two. Postoperative follow-up periods ranged from 2 years and 6 months to 3 years and 10 months (median: 2 years and 11 months). In four of them, the postoperative right ventricular to aortic or left ventricular systolic pressure ratios were less than 0.4 without any significant systolic pressure gradients between pulmonary artery and right ventricle. In these four patients, the diameters of the reconstructed pulmonary trunks grew from 10-18 mm to 18-21 mm postoperatively. These diameters were more than 100% of normal values. In the remaining patient with tetralogy of Fallot and single coronary artery, the obstruction of the new pulmonary trunk by a bulged left atrial appendage, which was used as the posterior wall, was observed on the right ventricular outflow tract reconstruction without an extracardiac conduit has growth potential in the future.     

Slide tracheoplasty: a case report of successful concomitant reconstruction of extensive congenital tracheal stenosis and pulmonary artery sling

An 8-month-old infant presented with an extensive congenital tracheal stenosis with an aberrant left pulmonary artery. The patient was treated successfully by relocation of the left pulmonary artery and tracheal reconstruction with slide tracheoplasty. This patient is the first survivor cited in the literature after concomitant repair using slide tracheoplasty.     

Comparison of FISH PRINS, and conventional and fluorescent PCR for single-cell sexing: suitability for preimplantation genetic diagnosis

Valved homograft conduits play an important role in the right ventricular outflow tract (RVOT) reconstruction for the surgical treatment of complex congenital heart disease. An excellent immediate rather than long-term outcome could be obtained. The hemodynamics for late failure, however, remained unclear. In vitro pulsatile flow visualization was not conducted before. A simplified right heart duplicator system was set up and driven under physiologic conditions. Polystyrene of 0.18 mm in diameter was applied as the tracing particle. Flow characteristics of models of normal pulmonary circulation as well as pulmonary artery atresia with the RVOT reconstructed utilizing valved and non-valved extracardiac conduits were observed. Flow patterns in the normal pulmonary circulatory model were mainly of axial flow associated with small scope of flow disturbances. A single vortex in the right ventricle was noted in diastole. In the pulmonary artery atresia model, a couple of vortexes were found in the right ventricle, a secondary flow in the main pulmonary artery, and a stronger secondary flow than in the normal pulmonary circulatory model in the two branches in both systole and diastole. A secondary flow was found in the proximal, an axial flow was observed in the distal portion of the extracardiac conduit with normal bioprosthetic valves and a secondary flow was observed in the entire conduit with stenotic bioprosthetic valves. The secondary flow intensity became stronger with the development of the stenosis. Severe insufficiency occurred in the bileaflet ceramic tilting-disc prosthesis during the entire cardiac circle, i.e., the prosthesis was in a maximum open position. Severe reverse flow could be found in the extracardiac conduit in the deceleration phase. Concavity of the crank shaft was found by examination to be filled with tracing particles and the prosthesis became stuck. Model of RVOT reconstruction with non-valved conduit yielded reverse flow inside the extracardiac conduit as well. Secondary flow may occur in normal or diseased extracardiac conduit for RVOT reconstruction. If micro-thrombus of over 0.18 mm in diameter attached in the concave of the crank shaft of a bileaflet tilting-disc prosthesis under a condition of resistance as occurred in the present study, the prosthesis may become stuck. Model of RVOT reconstruction with non-valved extracardiac conduit yielded reverse flow inside the conduit, of which the flow pattern was of greater energy consumption. Thus, a non-valved conduit should be avoided in clinical practice as far as possible.     

Systemic collateral and pulmonary artery stenosis in patients with congenital pulmonary valve atresia and ventricular septal defect

Angiograms of 30 patients with congenital pulmonary valve atresia, ventricular septal defect, and large systemic-pulmonary collateral arteries (SPCAs) were evaluated. All had aortography, 28 had SPCA arteriography, and 26 had right ventriculography. Seventeen (65%) of 26 patients had a right ventricular infundibulum, 23 (77%) had a pulmonary artery confluence, and five of the nine patients without a right ventricular infundibulum had a confluence. Sixty-six SPCAs of aortic origin were seen; 28 (42%) had narrowing and 21 patients (70%) had one or more narrowed SPCAs. Five patients had collaterals from internal mammary, subclavian or innominate arteries. Fourteen (47%) had hilar pulmonary artery stenosis. Of these 14 patients mild peripheral stenosis was demonstrated in five. Right aortic arch was present in 15 patients (50%). Complete angiographic delineation of pulmonary vasculature is an essential procedure for preoperative detection of pulmonary and SPCA stenoses in these patients.     

Pulmonary arterial reconstruction with autologous pulmonary arterial tissue for pulmonary artery sling

One-year and two-month-old boy with pulmonary artery sling underwent pulmonary artery reconstruction with autologous pulmonary arterial tissue. Surgical repair consisted of the transection of the left pulmonary artery at its origin with trimming a button hole and the creation of an oval hole with the same size to the left pulmonary button. An autologous pulmonary patch and the left pulmonary button were exchanged and sutured to the former partner's place. He recovered uneventfully in post-operative course. Postoperative CT revealed a well developed left pulmonary artery from 5 mm to 10 mm in diameter without any distortion or stenosis. This exchange method could allow the natural growth of the anastomotic places without narrowing or distortion.     

Clinical implications and possible association of malposition of the branch pulmonary arteries with DiGeorge syndrome and microdeletion of chromosomal region 22q11

We describe a series of 10 patients with malposition of the branch pulmonary arteries (4 patients with crossing [crossed pulmonary arteries] and 6 patients without crossing), 2 of whom had a short main pulmonary artery segment that resulted in iatrogenic right pulmonary artery stenosis after pulmonary artery band placement. DiGeorge syndrome was seen in 5 patients and 4 had microscopic deletion of chromosomal region 22q11.     

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.     

Asymmetric distribution of the pulmonary blood flow between the right and left lungs in d-transposition of the great arteries

Pulmonary angiograms, radionuclide lung images and chest roentgenograms were evaluated regarding the incidence, magnitude and natural evolution of maldistribution of the pulmonary blood flow between the lungs in 63 patients with dextrotransposition of the great arteries. Approximately half of these patients had some degree of greater perfusion of the right relative to the left lung. A significant correlation was demonstrated between the incidence of this maldistribution of blood flow and the angulation between the main and the right pulmonary arteries. For any given angulation between these vessels, additional pulmonary stenosis increased the incidence of disparity in perfusion. Our observations suggest the following developmental mechanisms: The maldistribution in flow results from the abnormal rightward inclination of the main pulmonary artery in the transposition malformation which straightens the flow axis from the main to the right pulmonary artery. Under these circumstances the momentum of the blood in the main pulmonary artery carries the blood preferentially into the right pulmonary artery. This momentum is increased when there is stenosis of the left ventricular outflow tract. Consequent differences in the mechanical properties of the two pulmonary vascular beds can increase this maldistribution. The disparity in perfusion between the lungs is not present in newborns with d-transposition, appears to be progressive in severity and in time may result in almost complete cessation of effective perfusion of the left lung. The effect of the Mustard operation on this abnormality of flow is discussed.     

Homografts in congenital heart disease: current applications and future directions

The first clinical use of homograft tissue in cardiovascular surgery was in 1948, when Gross used cadaveric arterial grafts to construct systemic to pulmonary artery shunts in patients with tetralogy of Fallot, and to repair coarctation of the aorta. Eighteen years later, a valved homograft was used for the first time in the treatment of congenital heart disease for reconstruction of the right ventricular outflow tract in a child with pulmonary atresia. Since these pioneering advances, valve and vascular homografts have become central to the management of congenital anomalies of the heart and great vessels. The primary use for homografts in congenital heart surgery today is establishment of a valved connection between the right ventricle and pulmonary arteries in children with tetralogy of Fallot with pulmonary atresia or other complicating factors, truncus arteriosus, transposition complexes, and double-outlet right ventricle, and in patients undergoing the Ross procedure. Homograft reconstruction of the left ventricular outflow tract has also been performed for many years in children with aortic insufficiency or recurrent aortic stenosis, but early homograft degeneration in young children has been a significant problem. Many surgeons are turning away from homografts in the systemic circulation in favor of the pulmonary autograft. Homograft is also widely used as a vascular patch material. In the present report, we discuss the various uses of homografts in congenital heart surgery, the benefits and drawbacks of homografts in young patients, and some of the future possibilities for homograft development and application in patients with congenital heart disease.     

Stenosis at the origin of an anomalous left main coronary artery arising from the pulmonary artery in a symptom-free adolescent girl: transesophageal echocardiographic findings

We report an unusual finding of ostial stenosis of an anomalous left main coronary artery originating from the pulmonary artery in a symptom-free adolescent girl. Transesophageal echocardiography with Doppler color imaging correctly identified all of the salient features of this anomaly including proximal stenoses of the right and left coronary arteries. These findings were subsequently confirmed at cardiac catheterization.     

Extrinsic compression of the pulmonary artery by non-microcytic lung carcinoma

A patient with a remitting non-microcytic pulmonary carcinoma was subjected to Doppler-echocardiography. The detection of a pericardial effusion with compression of the pulmonary artery due to an extrinsic mass indicated that the disease was progressing. The Doppler technique was used to evaluate the degree of arterial stenosis by determining the pressure gradient caused by it (49 mmHg) and to calculate the right ventricle pressure (75 mmHg). Tumor infiltration of mediastinal structures was posteriorly confirmed by computed tomography.     

Pulsus alternans in diastolic left ventricular dysfunction--a case report

Pulsus alternans is usually found in patients with reduced systolic ventricular function. We describe a patient with recurrent pulmonary edema, hypertension, bilateral renal artery stenosis, but with normal systolic function. Pulsus alternans was demonstrated in both pulmonary artery, right ventricle, and left ventricle pressures. After successful renal artery revascularization, the pulsus alternans disappeared. This case illustrates that pulsus alternans can be present with diastolic dysfunction of the left ventricle in the absence of systolic dysfunction.     

A patch technique for reconstruction of stenotic pulmonary bifurcation in a Rastelli-type operation after palliations for cyanotic congenital heart disease. A report of two cases

A Rastelli-type operation was successfully performed on two patients with cyanotic congenital heart disease with pulmonary artesia (PA) and severe stenosis or obstruction of the pulmonary bifurcation, following the improvement of a pulmonary artery index (PA index) after palliative systemic pulmonary shunts. It is a point that stenotic or obstructive pulmonary bifurcation was reconstructed by a patch technique. An extracardiac conduit was used a combination of a bicuspid valved pericardial roll and a prosthetic vessel graft. In a 5-year-old male with an extreme type of tetralogy of Fallot associated with PA and patent ductus arteriosus (PDA), the enlargement of stenotic pulmonary bifurcation was attempted using a brimshaped equine pericardial patch in order to get sufficient suture margin and be safely anastomosed with a conduit. In a 6-year-old female with complete transposition of the great arteries, PA, PDA and right aortic arch (RAA), an obstructive pulmonary bifurcation behind the RAA was enlarged using a brimshaped equine pericardial patch. The attachment of the conduit was shifted to the left pulmonary artery to avoid compression of an extracardiac conduit and pulmonary artery behind RAA. Reconstruction of the pulmonary bifurcation using an equine pericardial patch is useful to anastomose an extracardiac conduit and the stenotic or obstructive pulmonary bifurcation.     

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.     

Enhanced detection of proximal right coronary artery stenosis with the additional analysis of right ventricular thallium-201 uptake in stress scintigrams

The value of right ventricular thallium-201 analysis in detecting proximal right coronary artery stenosis in exercise myocardial scintigraphy was analyzed in 52 patients, 27 with and 25 without proximal right coronary artery stenosis. For the detection of proximal right coronary artery stenosis, the sensitivity and specificity of thallium scintigraphic analysis were 59 and 88% for a right ventricular abnormality, 67 and 68% for a left ventricular inferior wall abnormality, and 93 and 56% for an abnormality of either. When both right and left ventricular thallium images were abnormal, all 9 patients had proximal right coronary artery stenoses, and when both were normal, 26 of 28 patients had a normal proximal right coronary artery. The sensitivity and specificity of blood pool scintigraphic variables during exercise (right ventricular ejection fraction and left ventricular inferior wall motion) were not significantly different for detection of proximal right coronary artery stenosis. Thus, the additional analysis of the right ventricle on thallium-201 stress scintigrams can improve the detection of proximal right coronary artery stenosis. When both right ventricular and left ventricular thallium scintigrams are abnormal (or normal), the ability to predict the presence (or absence) of proximal right coronary artery stenosis is very high.     

Monocusp valve and transannular patch reconstruction of the right ventricular outflow tract: an experimental study

Repair of congenital right ventricular outflow tract obstruction often requires reconstruction with a transannular patch to alleviate pulmonary stenosis. Post repair pulmonary insufficiency with right ventricular dilatation and volume overload may result and lead to acute or progressive right heart failure. The use of a monocusp valve has been proposed as a means to prevent this problem. Fresh pericardium is well known to fail clinically, leading to pulmonary insufficiency limiting mid- and long-term results. In a chronic dog model (147 +/- 34 days), three valve types were evaluated: 1) polytetrafluoroethylene (PTFE; n = 9), 2) fresh pericardium (PERI; n = 6), and glutaraldehyde fixed pericardium (GLU; n = 6). Hemodynamics, angiography, and echocardiography were performed at implantation and sacrifice. Gross and microscopic pathology were evaluated. No significant differences were found among the three groups with regard to stenosis as evaluated by echocardiography, measured right ventricular wall thickness, and hemodynamic pressure gradients across the valve. By echocardiography, both PTFE (1 of 9) and GLU (0 of 6) showed less regurgitation than PERI (5 of 6) (p < 0.05). This was confirmed by angiography. PTFE showed less neo-intimal hyperplasia, less thrombus formation, and less calcification than GLU or PERI (p = NS). The PTFE monocusp developed no prohibitive gradients, no early pathologic deterioration, and maintained competence compared with the GLU and PERI groups. Although continued investigation of long-term durability and competence of the PTFE monocusp valve is warranted, both PTFE and GLU values seem to demonstrate less regurgitation than the PERI monocusp valve in an adult dog model of right ventricular outflow tract reconstruction.     

Tooth enamel thickness in the mature dentition of domestic dogs and cats--preliminary study

Eleven patients underwent late repeated correction of tetralogy of Fallot in 1991-1993. The previous operation was repair of simple Fallot's tetralogy in seven cases, repair plus transannular patch in one case and repair of tetralogy and pulmonic atresia in three cases. The indications for reoperation were residual ventricular septal defect, right ventricular outflow tract (R.V.O.T.) obstraction, residual branch pulmonary artery stenosis, aneurysmal dilatation of R.V.O.T. Patch or combination of any of the above. At reoperation these defects were corrected. The post operative course was uneventful in eight patients. Two required mechanical ventilation for 2-3 days, and one underwent another operation for residual branch pulmonary artery stenosis. The functional and haemodynamic results were good in ten patients, and one had residual distal pulmonary artery stenosis. There were no death during 2 years of follow-up. Repeated correction of tetralogy of Fallot thus had low postoperative morbidity and good haemodynamic results. For the relatively few patients initially found to have tetralogy of Fallot and pulmonic atresia, the outcome may be less favorable.