Advances in pediatric cardiovascular surgery: anatomic reconstruction of the left ventricular outflow tract in transposition of the great arteries with pulmonic valve abnormalities.

In the past years, advances in pediatric cardiovascular surgery have occurred in many areas with some of the greatest strides being made in complex repairs in younger age groups. Aggressive early corrections while higher risk, may in the long run provide a child with a normal anatomic heart, and corresponding myocardial growth and physiology.

Urgency of operation in infracardiac total anomalous pulmonary venous connection.

BACKGROUND: Because the tendency for pulmonary venous obstruction in the infracardiac type of total anomalous pulmonary venous connection may be partially dependent on the connection of the descending vein to the portal vein, the inferior vena cava, or one of their tributary vessels, we reviewed our surgical experience with various subtypes of infracardiac total anomalous pulmonary venous connection. METHODS: The urgency of operation in 4 neonates with infracardiac total anomalous pulmonary venous connection was reviewed. RESULTS: Two patients with pulmonary venous obstruction in whom the descending vein connected to the portal vein were operated on immediately with successful outcome. One patient who had become critically ill after the ductus venosus had closed died before operation could be undertaken. One patient in whom the descending vein connected to the left hepatic vein was operated on electively with successful outcome. CONCLUSIONS: In hemodynamically stable patients with no clinical or echocardiographic signs of pulmonary venous obstruction, some form of differentiation with regard to urgency of operation may be appropriate. When the descending vein connects to the inferior vena cava or a hepatic vein, the operation may be performed on a semi-elective basis. In contrast, when the descending vein connects to the portal vein or the ductus venosus, operation should generally not be delayed because of the high likelihood of obstruction.

Extracardiac conduit fontan procedure: early and intermediate results.

OBJECTIVE: The extracardiac Fontan procedure, as compared with classic atriopulmonary connections, may have the potential for optimizing ventricular and pulmonary vascular function by maximizing the laminar flow principle, by the avoidance of intra-atrial suture lines and cardiac manipulation, and by minimizing cardiopulmonary bypass time. In this study the clinical results of this procedure are assessed. METHODS: From January 1990 until January 1997, 45 patients (33 males and 12 females) with a median age of 4.0 years (range 2.7-38 years) underwent an extracardiac Fontan procedure for univentricular physiology. The underlying diagnoses included tricuspid atresia (n=19), double-inlet left ventricle (n=11), and complex anomalies (n=15). Forty patients (89%) were in sinus rhythm. The median ventricular ejection fraction was 60%. In 37 patients (82%) the procedure was staged. RESULTS: Median cardiopulmonary bypass time was 72 min, with a decrease to a median time of 24 min in the last ten patients. Aortic cross-clamping was avoided in 33 patients (73%). The intraoperative Fontan pressure and transpulmonary gradient were low: 13.6+/-3.2 and 8.5+/-3.9 mmHg, respectively. Transient supraventricular tachyarrhythmias were observed in six patients (13%). There was no early or late mortality. At a median follow-up of 64 months (range 26-105 months), 39 patients (87%) were in NYHA class I, four (9%) were in NYHA class II, and two (4%) were in class III. Forty patients (89%) remained in sinus rhythm. The median ventricular ejection fraction was 59%. The median arterial oxygen saturation raised from 82% preoperatively to 97%. Functional class (P=0.02), maintenance of sinus rhythm (P=0.04), and preservation of ventricular function (P=0.05) was superior in patients who were appropriately staged. None of the patients had atrial thrombus, chronic pleural effusions, or protein losing enteropathy. CONCLUSIONS: In the majority of patients, the extracardiac Fontan procedure, when performed as a staged procedure, provides excellent early and midterm results in terms of quality of life, maintenance of sinus rhythm, and preservation of ventricular function.

Pulmonary artery sling: reimplantation versus antetracheal translocation.

BACKGROUND: We compared two repair techniques for pulmonary artery sling. The first comprised detachment of the aberrant left pulmonary artery from the right pulmonary artery and its implantation into the main pulmonary artery, and the second, translocation of the left pulmonary artery anterior to the trachea (without implanting it into the main pulmonary artery), resection of tracheal stenosis, and end-to-end reconstruction of the trachea. METHODS: Five symptomatic infants (3 boys and 2 girls; median age 5 months; range, 3 weeks to 11 months) with pulmonary artery sling were operated on through a median sternotomy with aid of cardiopulmonary bypass. In 3 patients, the left pulmonary artery was transected from the right pulmonary artery and implanted into the main pulmonary artery. In addition, the anterior trachea was augmented with a pericardial patch (n = 2). In the remaining 2 patients, associated tracheal stenosis was resected, the left pulmonary artery was translocated anterior to the trachea, and the trachea was reconstructed. RESULTS: All 5 infants survived the operation. The 3 patients in whom the left pulmonary artery was implanted into the main pulmonary artery had an uncomplicated postoperative course. All 3 patients, at a follow-up of 10 months to 7.9 years, were free of symptoms; the left pulmonary artery was documented to be widely patent. The remaining 2 patients in whom the left pulmonary artery was translocated anterior to the trachea could not be extubated. In both patients the distal trachea was compressed anteriorly by the left pulmonary artery. One of these patients died at 1 week postoperatively secondary to tracheal dehiscence. In the other patient, the left pulmonary artery was implanted into the main pulmonary artery with good result; at a follow-up of 3.9 years, mild residual stridor has persisted. CONCLUSIONS: In pulmonary artery sling, implantation of the aberrant left pulmonary artery into the main pulmonary artery, if necessary combined with anterior tracheoplasty, reliably eliminates tracheal and esophageal compression and maintains antegrade flow into the left pulmonary artery. Translocation of the left pulmonary artery anterior to the trachea without implanting it into the main pulmonary artery is not favored because that might result in anterior compression of the trachea. In addition, we are concerned about growth of the circumferential tracheal anastomosis in neonates and infants.

Early repair of coarctation of the aorta.

BACKGROUND: Repair of coarctation of the aorta with hypoplasia and elongation of the proximal aortic arch is a technically demanding procedure with a substantial rate of recurrent stenosis at the coarctation repair site. In addition, a high incidence of hypertension has been reported in patients who underwent repair beyond infancy. PATIENTS AND METHODS: Between January 1991 and June 1997, 52 patients (34 neonates and 18 infants with a median age of 37 days; range 2 days to 8 months) with a mean peak systolic upper to lower extremity resting gradient of 33.5 +/- 18.9 mmHg underwent repair of aortic coarctation. The echocardiographically measured median diameter of the aortic arch immediately distal to the innominate artery was 5.4 mm (range 4.0 to 8.1 mm). Eight patients (15%) were considered hypertensive. In 41 patients, through a left thoracotomy, an end-to-side anastomosis was constructed between the descending aorta and the undersurface of the proximal aortic arch. In 12 of these patients (who all had a hypoplastic and elongated aortic arch) this procedure was preceded by the construction of an extended side-to-side left carotid-subclavian arterioplasty. The remaining 11 patients, all with hypoplasia of the aortic arch, had concomitant complete repair of intracardiac anomalies through a median sternotomy. In 8 of these patients, in addition to anastomosis of the descending aorta to the undersurface of the proximal aortic arch, the ascending aorta and aortic arch were augmented with a pulmonary homograft patch. RESULTS: One neonate with associated Shone's syndrome died (2%) on the first postoperative day. There was no late mortality. Early postoperative complications included recurrent laryngeal nerve injury in 1 patient and prolonged chest tube drainage in 4 patients. At a median follow-up of 55 months (range 15 to 92 months), only 3 patients (5. 7%) developed a recurrent stenosis at the coarctation repair site. The remaining 48 patients are free of recurrent stenosis by echocardiography and clinical examination. None of the patients had systemic hypertension. CONCLUSIONS: 1. Coarctation repair consisting of resection of all ductal tissue with end-to-side anastomosis of the descending aorta to the undersurface of the (proximal) aortic arch, if necessary combined with a side-to side left carotid-subclavian arterioplasty, may lead to excellent results. 2. This technique can be applied with low mortality and morbidity via a left thoracotomy if the proximal aortic arch is at least 5 mm in diameter. 3. In view of the low mortality, the low incidence of restenosis at the coarctation repair site, and the absence of substantial morbidity including the development of hypertension, we advocate repair of aortic coarctation at neonatal age or in early infancy to avoid the detrimental sequelae of delayed repair of coarctation, in particular hypertension.

Conversion of atriopulmonary or lateral atrial tunnel cavopulmonary anastomosis to extracardiac conduit Fontan modification.

OBJECTIVE: Obstruction of the atriopulmonary anastomosis or the lateral atrial tunnel cavopulmonary anastomosis in the Fontan circulation for univentricular physiology may result in dilation of the right atrium or the right atrial free wall that is incorporated in the lateral atrial tunnel, respectively. Secondary detrimental sequelae may consist of supraventricular dysrhythmias, thromboembolism, right pulmonary vein compression, pleural effusions, and protein-losing enteropathy. Conversion of these Fontan connections to an extracardiac conduit cavopulmonary anastomosis may improve central systemic venous flow patterns and provide clinical improvement in these patients. METHODS: Eighteen patients (7-40 years old) with atriopulmonary anastomosis (n = 15) or obstructed lateral atrial tunnel cavopulmonary anastomosis (n = 3) presented at 5.7 +/- 3.9 years with moderate to severe right atrial dilation (n = 15), Fontan pathway obstruction (n = 12), atrial dysrhythmia (n = 13), pleural effusion (n = 8), right atrial thrombus (n = 3), right pulmonary vein compression (n = 3), and protein-losing enteropathy (n = 3). All patients underwent conversion to an extracardiac conduit cavopulmonary anastomosis. RESULTS: Two of the three patients with protein-losing enteropathy died (2/18; 11%) on the 30th and 52nd postoperative days. At a mean follow-up of 19 months, the remaining 16 patients had marked (n = 11) or moderate (n = 5) clinical improvement. The SaO2 improved from 90.7 +/- 5.3% to 96.0 +/- 4.1%. None of the patients had obstruction in the systemic venous pathway. In the 13 surviving patients with previous atriopulmonary anastomosis there was a drastic reduction in right atrial size. Four of 13 patients with atrial dysrhythmias converted to sinus rhythm. The right pulmonary vein compression as present in three patients resolved after conversion. Pleural effusions disappeared in four patients. CONCLUSIONS: Conversion to an extracardiac cavopulmonary connection may lead to clinical improvement in patients with atriopulmonary or lateral atrial tunnel Fontan connection associated with specific target conditions such as obstruction, pulmonary vein compression, right atrial enlargement, atrial dysrhythmia, or atrial thrombus. The conversion operation should not be unduly delayed to prevent irreversible deterioration of clinical status with chronic rhythm disturbances or protein-losing enteropathy. The benefit of the conversion operation is questionable in patients with poor clinical condition and protein-losing enteropathy.

Excision of coronary artery fistula and coronary artery reconstruction without cardiopulmonary bypass.

In three patients, coronary artery fistulas originating from a conal branch of the mid-segment of the left anterior descending coronary artery (n = 2) and right coronary artery (n = 1) with drainage into the right atrium (n = 2) and right ventricle (n = 1) were successfully closed without the use of cardiopulmonary bypass. The use of a coronary artery stabilizer greatly facilitated the operation by immobilization of the fistula, its supplying coronary artery, and the regional myocardium. In selected patients, this technique allows secure closure of the fistula and meticulous reconstruction of the coronary artery without the use of cardiopulmonary bypass.

Conversion of complex neonatal Ebstein's anomaly into functional tricuspid or pulmonary atresia.

BACKGROUND: Ebstein's anomaly, due to failure of delamination of one or more leaflets of the tricuspid valve (TV), is associated with varying degrees of tricuspid regurgitation (TR) and dysplasia of the right ventricle (RV). Although refinement of tricuspid valvuloplasty and plication techniques have opened the way to a satisfactory outlook for the majority of older children and adults, Ebstein's anomaly presenting at neonatal age, secondary to ineffective forward flow into the pulmonary and systemic circulation, has a reported mortality rate of as high as 75%. In order to improve the dismal outcome in neonatal Ebstein's anomaly, we have strived for early univentricular palliation. PATIENTS AND METHODS: Univentricular repair was performed in five neonates (median age 5 days; range 2-14 days) with Ebstein's anomaly, ductal dependent pulmonary blood flow, severe TR, absence of forward flow across the pulmonary valve, and small left ventricular (LV) area due to right-to-left bowing of the ventricular septum and ineffective LV loading (median indexed LV area 10.5 cm2/m2). In addition, two neonates had moderate pulmonary regurgitation (PR), one with additional pulmonary stenosis. In all patients, the indexed area of the combined right atrium and atrialized RV was greater than that of the combined functional RV, left atrium, and left ventricle (median 22.0 and 20.8 cm2/m2, respectively). The median preoperative systemic oxygen tension was 35 mmHg and the median pH 7.28. Repair consisted of TV closure with a pericardial patch (with the coronary sinus draining into the RV) (n = 3) or, in the presence of PR, resection of the dysplastic TV and division and oversewing of the main pulmonary artery (n = 2), as well as excision of the atrial septum, resection of redundant right atrial wall, and construction of an aortopulmonary shunt (n = 5). RESULTS: The median indexed LV area increased from 10.5 to 18.8 cm2/m2 as a result of more effective loading of the left ventricle. There was no intraoperative or late mortality. The patients were extubated at a median of 7 days postoperatively. At discharge, the median systemic oxygen tension was 46 mmHg. In all five patients, at 6, 7, 10, 12 and 16 weeks of age, a bidirectional cavopulmonary anastomosis has been constructed. CONCLUSIONS: In neonates with Ebstein's anomaly and ductal dependent pulmonary blood flow, rational palliation consists of the surgical creation of tricuspid atresia or, in the additional presence of PR or pulmonary stenosis, the creation of pulmonary atresia. These procedures may result in effective LV decompression and more effective volume loading of the left ventricle with increase of systemic output and improved clinical outcome.

Extracardiac repair versus intracardiac baffle repair of complex unroofed coronary sinus.

Complex unroofed coronary sinus with a persistent left superior vena cava has as its commonest major associated intracardiac anomaly a partial or complete atrioventricular canal defect. In this clinical setting, biventricular repair with construction of a complex intra-atrial baffle from the pulmonary veins to the mitral valve has a reported mortality rate of as high as 50%. Looking for an improvement, we have carried out an extracardiac repair of the anomalous systemic venous component with atrial septation. In 2 infants (aged 7 and 12 weeks) with unroofed coronary sinus, bilateral superior venae cavae, right isomerism, and complete atrioventricular canal, in addition to patch closure of the ventricular component of the atrioventricular septal defect, a baffle was constructed between the pulmonary veins and the mitral valve. In four subsequent infants (aged 7,10,16, and 20 weeks) with unroofed coronary sinus, bilateral superior venae cavae, complete atrioventricular canal, right isomerism (n = 2), and mild infundibular stenosis (n = 1), repair consisted of end-to-side anastomosis of the left superior vena cava to the right superior vena cava and complete repair of the atrioventricular canal and associated conditions. There was no mortality. The early postoperative course in the two patients with intra-atrial baffle was characterized by increased left-atrial pressure (18 and 20 mm Hg), with varying degrees of pulmonary venous congestion, supraventricular tachycardias, and systemic hypotension. The pulmonary venous congestion increased, so that one patient was successfully converted 10 weeks postoperatively to an extracardiac repair with septation of the atria and the other will probably follow. In the 4 patients with a primary extracardiac repair, the hemodynamic result was excellent, with a median left-atrial pressure of 11 mm Hg on the first postoperative day. At a median follow-up of 12 months, all 5 patients with an extracardiac repair are clinically well with widely patent anastomoses between the left and right superior venae cavae. The extracardiac repair technique for complex unroofed coronary sinus, as opposed to the intra-atrial baffle repair, avoids creation of a small and low-compliance left-atrial compartment with the potential for development of pulmonary venous congestion.

Aberrant left coronary artery arising from the right sinus of Valsalva with a right coronary arteriovenous malformation.

An 11-year-old boy presented with myocardial ischemia and was found to have an aberrant left main coronary artery from the right sinus of Valsalva coursing between the aorta and pulmonary artery, as well as a small arteriovenous malformation from a right atrial branch of the right coronary artery to the right atrium. Distinctive echocardiographic findings were supported by angiographic and magnetic resonance imaging studies. Treadmill and scintigraphic stress testing were normal. Corrective surgery was accomplished by a modified technique to unroof the intramural proximal course of the left coronary artery, without postoperative complications. The anatomy and pathophysiology of this rare coronary lesion are reviewed.

Predicting feasibility of biventricular repair of right-dominant unbalanced atrioventricular canal.

BACKGROUND: In right-dominant unbalanced atrioventricular (AV) canal, there are no criteria to judge adequacy of the left ventricle for biventricular repair. The purpose of this study was to test the hypothesis that right ventricular volume overload in this condition results in right-to-left septal bowing and contributes to the appearance of a small left ventricle. METHODS: Five consecutive neonates and young infants (age range, 23 days to 5 months; median age, 3 months) with right-dominant unbalanced complete AV canal underwent biventricular repair. Preoperative and postoperative echocardiographic measurements of left (LV) and right ventricular size and AV valve component size were made. Potential LV volume was assessed preoperatively using a theoretic model that assumed a normalization of septal bowing. RESULTS: There was no perioperative mortality; 1 patient died 71 days postoperatively of problems related to the left AV valve. Preoperatively, all patients had severe LV hypoplasia, with a mean end-diastolic indexed true LV volume of 14.8 +/- 9.1 mL/m2, indexed potential LV volume of 32.0 +/- 18.8 mL/m2, left AV valve to total AV valve ratio of 0.30 +/- 0.06, and LV to right ventricular long-dimension ratio of 0.65 +/- 0.1. Postoperatively, all patients had indexed true LV volumes greater than 30 mL/m2 (mean volume, 35.6 +/- 3.9 mL/m2), and the left AV valve to total AV valve ratio and the LV to right ventricular long-dimension ratio increased to 0.42 +/- 0.03 and 0.88 +/- 0.11, respectively. Both preoperative potential and true LV volumes correlated well with postoperative true LV volumes: r = 0.90 (p = 0.040) and r = 0.93 (p = 0.023), respectively. Increases in LV length and left AV annulus size indicated contributions of volume loading and surgical patching to the right of the ventricular crest to the increase in LV size. CONCLUSIONS: In our small series, preoperative indexed potential LV volume of 15 mL/m2 or greater (present in all patients) allowed biventricular repair of right-dominant unbalanced AV canal. Any previous criteria for LV hypoplasia in this condition need to be reconsidered. This study also has implications for other right-sided volume-loaded lesions in which the left ventricle initially is judged to be hypoplastic but in which biventricular repair may be feasible.

Outcomes after bidirectional cavopulmonary shunt in infants less than 6 months old.

OBJECTIVES: We sought to assess the results after bidirectional cavopulmonary shunt (BCPS) in infants < 6 months old and to identify risk factors for poor outcome. BACKGROUND: Although BCPS is a well established procedure for the palliation of patients with a single-ventricle heart, there have been very few reports of outcomes after BCPS in young infants. METHODS: Since 1990, 42 infants between 0.8 and 6.0 months of age (mean [+/-SD] 3.7 +/- 1.4) have undergone BCPS for primary (n = 16) or secondary (n = 26) palliation of tricuspid atresia (n = 13), hypoplastic left heart syndrome (n = 10) or other forms of functional single-ventricle heart (n = 19). Accessory pulmonary blood flow was included in 18 patients. Preoperative and perioperative data were gathered on retrospective review of patient records, and follow-up was conducted by means of direct physician contact or record review. RESULTS: The overall hospital mortality rate, including that associated with reoperations, was 4.8% (2 of 42 patients). Seven patients (17%) required reoperation related to the BCPS or pulmonary blood flow in the early postoperative period: Procedures included take-down of the BCPS in four patients, with one early death, and procedures to decrease pulmonary blood flow in three patients. Age < 1 month correlated significantly with early death and with early failure of the BCPS (death or take-down). Follow-up of the 37 patients discharged with intact BCPS was obtained at a mean +/-SD of 14.3 +/- 11.3 months postoperatively, during which time three patients died (at 6.5 +/- 2.5 months). The 2-year actuarial survival rate for patients undergoing BCPS at < 6 months of age was 86%. Overall freedom from death or take-down (including early and late events) was significantly lower in patients < 2 months old than in those > 2 months old. Four patients have undergone successful Fontan completion (18.3 +/- 2.9 months postoperatively), and one patient whose BCPS was taken down subsequently underwent successful restoration of a BCPS. CONCLUSIONS: Outcomes after BCPS in young infants are comparable to those in older infants and children. However, our current preference is to defer this procedure until after 2 months of age.

Instantaneous subaortic outflow obstruction after volume reduction in hearts with univentricular atrioventricular connection and discordant ventriculoarterial connection.

OBJECTIVE: To study the phenomenon of potential subaortic outflow obstruction after surgical volume unloading of the heart in patients with univentricular atrioventricular connection, discordant ventriculoarterial connection, and bulboventricular foramen (BVF)-dependent systemic flow. MATERIAL AND METHODS: Intraoperative transesophageal echocardiography was used in five patients with tricuspid atresia (N = 3) or double-inlet left ventricle (N = 2) with rudimentary right ventricle and BVF who were scheduled to undergo a bidirectional cavopulmonary anastomosis (N = 3) or completion of the Fontan procedure after previous banding of the pulmonary artery (N = 2). The BVF diameter was measured in two orthogonal views, and the area was calculated by using the formula for an ellipse. Left ventricular posterior wall thickness and left ventricular internal diameter were also measured. Intraoperative prerepair and postrepair gradients across the BVF were measured by echocardiography. RESULTS: Volume unloading of the left ventricle resulted in instantaneous contraction of left ventricular size (decrease of median left ventricular internal diameter from 38 to 34 mm and increase of median left ventricular posterior wall thickness from 5 to 7 mm), decrease of median BVF area index (from 1.82 to 1.55 cm2/m2), and development of a median gradient of 60 mm Hg across the BVF. At a mean follow-up of 19.6 months, all patients were clinically well and had no echocardiographic evidence of BVF obstruction. CONCLUSION: In hearts with univentricular atrioventricular connection, discordant ventriculoarterial connection, and BVF-dependent systemic flow, a decrease in ventricular volume is associated with an instantaneous alteration in ventricular geometry, diminution in BVF size, and potential for subaortic outflow obstruction. Intraoperative transesophageal echocardiography is of paramount importance in excluding development of subaortic outflow obstruction in this setting.

Morphologic determinants favoring surgical aortic valvuloplasty versus pulmonary autograft aortic valve replacement in children.

UNLABELLED: The pulmonary autograft is being used with increasing frequency to replace the diseased aortic valve in the pediatric population. Attempted surgical aortic valvuloplasty with an unacceptable result and return to cardiopulmonary bypass for aortic valve replacement with a pulmonary autograft results in prolonged bypass time and increased potential for morbidity. Therefore, the ability to predict an unsuccessful outcome for valvuloplasty would be of significant clinical benefit. This issue is addressed in the present study. METHODS: Twenty-two patients (median age 5.7 years, range 3 weeks to 14 years) with bicuspid (n = 11), tricuspid (n = 9), or quadricuspid (n = 2) aortic valves underwent valvuloplasty for aortic stenosis (n = 9), aortic regurgitation (n = 7), or a combination (n = 6). Previous related procedures included balloon aortic valvuloplasty (n = 3) and open surgical valvotomy (n = 1). Median pressure gradient across the aortic valve was 80 mm Hg. Surgical valvuloplasty techniques included thinning of leaflets (n = 18), commissurotomy (n = 15), suspension of reconstructed leaflet to the aortic wall (n = 10), closure of leaflet fenestration (n = 5), shortening of free edge of prolapsed cusp (n = 4), repair of torn leaflets (n = 3), and augmentation of scarred leaflets with autologous pericardium (n = 3). Concomitant subvalvular and supravalvular stenosis were repaired in nine and four patients, respectively. In five patients, during the same hospital stay, a failed valvuloplasty was converted into a valve replacement with a pulmonary autograft because of residual or resultant stenosis (n = 3) or regurgitation (n = 2). RESULTS: No early or late deaths occurred. At a median follow-up of 16.3 months the median pressure gradient across the aortic valve in the 15 patients with preoperative stenosis or combined stenosis and regurgitation was 16 mm Hg (p < 0.01 versus preoperative gradient). Of the 22 patients, the aortic valve functioned normally (defined as < or = mild stenosis or regurgitation, or both) in 14 patients (including five patients with valve replacement); four patients had stenosis (gradients 40, 45, 60, and 60 mm Hg), two patients had regurgitation, and two patients had combined stenosis (gradients 40 and 50 mm Hg) and regurgitation. Three of the patients with recurrent stenosis underwent secondary surgical valvuloplasty without improvement. Outcome after valvuloplasty was examined according to valve structure: six of nine tricuspid valves functioned normally, whereas only three of 13 nontricuspid valves functioned normally (P = 0.07). Patients with a nontricuspid aortic valve and regurgitation had a high probability of requiring immediate valve replacement (P = 0.009). The actuarial freedom from significant native valve stenosis or regurgitation at 24 months was 82% for tricuspid valves and 36% for nontricuspid valves (P = 0.007). CONCLUSIONS: (1) Surgical aortic valvuloplasty should be the preferred approach when the aortic valve is tricuspid. (2) In contrast, aortic valve replacement with a pulmonary autograft should be the preferred strategy in the presence of a nontricuspid aortic valve (especially when the aortic valve is regurgitant) and after failed surgical valvuloplasty.

Acute aortic outflow obstruction by diminution of bulboventricular foramen size after volume unloading in univentricular physiology.

A young child with [S, L, L] segmental anatomy, double-inlet left ventricle, transposition of the great arteries, rudimentary right ventricle, and mildly restrictive bulboventricular foramen is reported, in whom intraoperative temporary snaring of the modified Blalock-Taussig shunt resulted in instantaneous and dramatic volume contraction of the left ventricle, decrease in bulboventricular foramen size, and increase of the gradient across the latter from 10 mm Hg preoperatively to 50 mm Hg. A modified Damus-Stansel-Kaye procedure using autogenous aortic tissue resulted in unobstructed aortic outflow; in addition, a bidirectional cavopulmonary shunt was performed. The importance of early relief of actual or potential aortic outflow obstruction in hearts with restrictive bulboventricular foramen is emphasized.

Anomalous origin of left main coronary artery from right sinus of Valsalva: modified surgical treatment to avoid neo-coronary ostial stenosis.

A young symptomatic patient is reported with anomalous origin of the left main coronary artery from the right sinus of Valsalva and two stenoses in the intramurally coursing segment of the vessel, namely at the site of the intercoronary commissure and at its exit site in the left sinus of Valsalva. Unroofing of the intramural segment and a modified angioplasty technique resulted in a widely patent neo-coronary ostium.

Aortoventriculoplasty with the pulmonary autograft: the "Ross-Konno" procedure.

BACKGROUND: For patients with complex left ventricular outflow tract obstruction, including hypoplastic aortic anulus with or without severe diffuse subaortic stenosis, various aortoventriculoplasty procedures (e.g., Konno procedure and its modifications; extended aortic allograft root replacement) are important management options. In younger patients, however, reoperation for valve replacement is inevitably required, and anticoagulation issues pose additional problems. The pulmonary autograft provides a promising option for aortic valve replacement as part of the aortoventriculoplasty procedure in children. Long-term follow up shows that the pulmonary autograft functions well as the systemic arterial (neoaortic) valve and that valve growth occurs. METHODS: Between July 1993 and May 1995, 11 patients 4 days to 17 years old (median 12 months) underwent aortoventriculoplasty with pulmonary autograft (Ross-Konno procedure). The diagnoses were aortic stenosis with or without subaortic stenosis (n = 8), Shone complex (n = 2), and interrupted aortic arch with subaortic stenosis (n = 1). On average, 1.9 previous interventions had been performed per patient, including a previous Konno procedure in one patient. The aortic root was replaced with a pulmonary autograft valve. The left ventricular outflow tract was enlarged with a Dacron polyester fabric patch in two patients, with an allograft aortic patch in two patients and a right ventricular infundibular free wall muscular extension harvested in continuity with the autograft in seven patients. RESULTS: Intraoperative transesophageal echocardiographic assessment revealed mild aortic insufficiency in one patient. One patient had a residual left ventricular outflow tract gradient of 15 mm Hg. Significant complications were cardiac tamponade from bleeding (n = 1) and complete heart block necessitating a permanent pacemaker (n = 1). Follow-up ranged from 2 weeks to 16 months. To date, there have been no late deaths or reoperations. Follow-up echocardiography revealed mild autograft insufficiency in one patient and a 16 mm Hg residual left ventricular outflow tract gradient in one patient. CONCLUSIONS: Initial experience suggests that aortoventriculoplasty with the pulmonary autograft is an excellent alternative for young patients with complex left ventricular outflow tract obstruction. Because the pulmonary autograft has been shown to grow after implantation, reoperation on the left ventricular outflow tract is likely to be avoided.

Modified surgical techniques for relief of aortic obstruction in [S,L,L] hearts with rudimentary right ventricle and restrictive bulboventricular foramen.

Modified techniques of aortopulmonary anastomosis were performed in six neonates with atrioventricular and ventriculoarterial discordance [S,L,L], double-inlet left ventricle, and restrictive bulboventricular foramen area (mean index 1.10 cm2/m2) with unobstructed aortic arch (n = 3) or with hypoplasia (n = 2) or interruption (n = 1) of the aortic arch. In cases of unobstructed aortic arch, a flap of autogenous aortic tissue was used to augment the posterior aspect of the anastomosis of the main pulmonary artery to the ascending aorta, thus creating the potential for anastomotic growth; this technique is applicable regardless of the position of the ascending aorta relative to the main pulmonary artery. In case of levo-transposition of the aorta with hypoplasia or interruption of the aortic arch, a modified Norwood procedure was performed, in that the proximal ascending aorta was divided at the same level as the main pulmonary artery with subsequent homograft patch augmentation from the main pulmonary artery-ascending aorta anastomosis to the level of the proximal descending aorta; this technique avoids a spiraling incision of the aorta and therefore reduces the risk of torsion of the aortic root with its inherent risks of obstruction of the coronary circulation and aortic or pulmonary valve regurgitation. There was no early or late mortality. At a mean follow-up of 16 months, in all patients, there was unobstructed aortic outflow, as evidenced by echocardiographic absence of a significant ventricular-aortic systolic gradient (mean 4.5 +/- 4 mm Hg) and absence of distal aortic arch obstruction. There was no evidence of aortic or pulmonary valve regurgitation. The reported modified techniques provide effective relief of restrictive bulboventricular foramen and aortic obstruction in [S,L,L] hearts.

Extracardiac Fontan operation with adjustable communication.

A modified technique for the Fontan operation is described in which an adjustable communication between the extracardiac conduit (systemic venous chamber) and the right atrium (pulmonary venous chamber) is constructed. Potential advantages of this technique consist of minimization of surgical manipulation of atrial tissue, reduction or elimination of myocardial ischemia, creation of a uniform and stable inferior vena cava-to-pulmonary artery conduit, and increased flexibility and safety in certain high-risk patients such as those with increased pulmonary vascular resistance, pulmonary hypertension, and impaired ventricular function.

Organized thrombus in left main coronary artery in hypoplastic left heart syndrome.

A neonate is reported in whom, during Norwood stage I correction for hypoplastic left heart syndrome with mitral stenosis, an organized thrombus was found to protrude from the left coronary ostium into the aortic root. With ventricular assist device support the patient survived despite severe left ventricular ischemia. The presumed origin of the thrombus is from left ventriculocoronary arterial connections that serve to decompress the blind left ventricular cavity.