Biomedical subjects

F L Hanley

Publications and source records attributed to F L Hanley.

At least 55 records · Page 3Linked to original sources

PubMed1999

Role of the endothelium in placental dysfunction after fetal cardiac bypass.

BACKGROUND: Fetal cardiac bypass causes placental dysfunction, characterized by increased placental vascular resistance, decreased placental blood flow, hypoxia, and acidosis. Vasoactive factors produced by the vascular endothelium, such as nitric oxide and endothelin 1, are important regulators of placental vascular tone and may contribute to this placental dysfunction. METHODS: To investigate the role of the vascular endothelium in placental dysfunction related to fetal cardiac bypass, we studied 3 groups of fetal sheep. In the first group (n = 7) we determined placental hemodynamic responses before and after bypass to an endothelium-dependent vasodilator (acetylcholine), an endothelium-independent vasodilator (nitroprusside), and endothelin 1. In the second group (n = 8) a nonspecific endothelin receptor blocker (PD 145065) was administered and placental hemodynamic values were measured before and after bypass. In the third group (n = 5) endothelin 1 levels were measured before and after bypass. RESULTS: Before fetal cardiac bypass exogenous endothelin 1 decreased placental blood flow by 9% and increased placental resistance by 9%. After bypass endothelin 1 decreased placental flow by 47% and increased resistance by 106%. There was also a significant attenuation of the placental vascular relaxation response to acetylcholine after bypass, whereas the response to nitroprusside was not significantly altered. In fetuses that received the PD 145065, placental vascular resistance increased significantly less than in control fetuses (28% versus 62%). Similarly, placental blood flow decreased significantly more (from 6. 3 +/- 3.1 to 28.3 +/- 10.4 pg/mL; P =.01) in control fetuses than in fetuses receiving PD 145065 (33% versus 20%). Umbilical venous endothelin 1 levels increased significantly in fetuses exposed to fetal bypass but did not change in control fetuses. CONCLUSIONS: The basal endothelial regulatory mechanisms of placental vascular tone were deranged after fetal cardiac bypass. Endothelin receptor blockade, which substantially reduced postbypass placental dysfunction, and other interventions aimed at preserving endothelial function may be effective means of optimizing fetal outcome after cardiac bypass.

Acetylcholine↗

PubMed1999

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

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

Child, Preschool↗

PubMed1999

Echocardiographic diagnosis alone for the complete repair of major congenital heart defects.

OBJECTIVES: The study was done to determine the diagnostic accuracy of echocardiography alone in the preoperative diagnosis of children with major congenital heart defects undergoing primary complete repair. BACKGROUND: Although echocardiography is well established as the first-line imaging technique for the diagnosis of all forms of congenital heart disease, most institutions continue to perform cardiac catheterization prior to complete repair of more complex defects. METHODS: To determine the diagnostic accuracy of echocardiography alone and echocardiography plus catheterization, we reviewed the records of 503 children with major congenital heart defects who underwent primary complete repair at our institution between July 1992 and June 1997. We included children with transposition of the great arteries, tetralogy of Fallot, double-chamber right ventricle, interrupted aortic arch, aortic coarctation, atrioventricular septal defect, truncus arteriosus, aortopulmonary septal defect, and totally anomalous pulmonary venous return. We excluded children with less complex defects such as isolated shunt lesions, as well as those with the most complex defects that would require surgical palliation (e.g., functional univentricular heart). We defined major errors as those that increased the surgical risk and minor errors as those that did not. Errors in diagnosis were determined at surgery. RESULTS: Eighty-two percent of children (412 of 503) underwent surgery after preoperative diagnosis by echocardiography alone. There were 9 major (2%) and 10 minor errors in the echocardiography alone group and 7 major and 5 minor errors in the echocardiography plus catheterization group. The most common type of error was misidentification of coronary artery anatomy in patients with transposition of the great arteries. No error in either group resulted in surgical morbidity or mortality. CONCLUSIONS: This study suggests that echocardiography alone is an accurate tool for the preoperative diagnosis of major congenital heart defects in most children undergoing primary complete repair, and may obviate the need for routine diagnostic catheterization.

Adolescent↗

PubMed1999

Is it necessary to routinely fenestrate an extracardiac fontan?

OBJECTIVES: This study was conducted to assess the need for, and use of, fenestration of an extracardiac conduit Fontan. BACKGROUND: Fenestration of a Fontan connection has been proposed as a means of improving outcomes of single ventricle palliation. The benefit of fenestration is likely to be greatest in the early postoperative period when patients may experience increased pulmonary vascular resistance and decreased ventricular function due to the effects of cardiopulmonary bypass, aortic cross-clamping and positive pressure ventilation. However, there are potential drawbacks to fenestration. The utility of fenestration with extracardiac Fontan operation has not been determined. METHODS: Since 1992, 81 patients have undergone a modification of the Fontan procedure in which an extracardiac inferior cavopulmonary conduit is used in combination with a previously staged bidirectional Glenn anastomosis. We conducted a retrospective review of these patients. RESULTS: Fenestration was performed selectively in 32 patients (39%), including only 2 of the last 38 (5%). In seven patients, a fenestration was placed or clipped in the early postoperative period without cardiopulmonary bypass. There were two operative deaths. Prolonged (>2 weeks) pleural drainage occurred in 13 patients, 8 with fenestration and 5 without. In addition to undergoing earlier Fontan in our experience, patients who had a fenestration placed had significantly higher preoperative pulmonary vascular resistance, significantly higher common atrial pressure after Fontan and significantly lower post-Fontan systemic arterial oxygen saturation. Fontan pressure did not differ between nonfenestrated and fenestrated patients. At follow-up ranging to five years, there were two late deaths and no patients developed protein losing enteropathy. CONCLUSIONS: Fenestration is not necessary in most Fontan patients when an extracardiac conduit technique is performed as described in this article, and therefore, should not be performed routinely with the extracardiac conduit Fontan. The need for fenestration should be assessed after cardiopulmonary bypass when hemodynamics can be evaluated accurately. Fenestration can be placed and revised easily without bypass and with minimal intervention in patients with an extracardiac conduit Fontan.

Blood Vessel Prosthesis Implantation↗

PubMed1999

The use of 'pericardial hoods' for maintaining exact coronary artery geometry in the arterial switch operation with complex coronary anatomy.

OBJECTIVE: Complex coronary artery anatomy is the major risk factor for the arterial switch operation. Of the many approaches described the 'trap door' technique for coronary reimplantation is most flexible and allows safer transfer in complex arterial configurations. However, we have occasionally been concerned regarding torsion of the vessels with this approach. We therefore explored the role of trap-door augmentation with pericardial hoods to maintain exact coronary geometry during coronary transfer. METHODS: Between February 1992 and December 1997, 80 patients underwent an arterial switch procedure at our institution. Sixty-seven patients underwent direct coronary reimplantation. In ten, coronary/great vessel anatomy was considered unfavourable and the trap-door approach was adopted primarily. In two an augmented trap-door was performed as the primary procedure and in the last patient Aubert's approach was used. In five patients during rewarming, ischaemic changes were noted on the electrocardiogram and/or regional wall motion abnormalities on transoesophageal echocardiography. This prompted revision of the appropriate coronary anastomosis. In three it was considered the anastomosis was kinked due to angulation of the button; in two the coronary was overstretched. In four, revision of the anastomosis was by pericardial hood augmentation. RESULTS: In all patients there was normalization of the electrocardiogram and immediate improvement in cardiac function documented by transoesophageal echocardiography. No early or late death occurred in the pericardial hood group nor were there any readmissions for any reason. CONCLUSIONS: Pericardial augmentation of trap-door aortic anastomoses allows for the maintenance of exact coronary artery geometry during the arterial switch procedure and minimizes the risk of myocardial ischaemia. We believe it broadens the application of the arterial switch procedure to even the most complex coronary anatomy and is a useful adjunct to the other techniques of coronary transfer.

Adolescent↗

PubMed1999

Resection of subaortic stenosis; can a more aggressive approach be justified?

OBJECTIVES: Discrete subaortic stenosis causes left ventricular outflow tract (LVOT) obstruction and often produces aortic regurgitation (AR) which alone may precipitate surgical intervention. Conventional resection relieves the obstruction, but the recurrence rate is high, and the AR is little changed as the thick fibrous membrane which extends onto the valve leaflets remains. We studied whether an aggressive surgical approach could reduce both the severity of AR and rate of recurrence of obstruction associated with discrete subaortic stenosis, and whether this aggressive approach could be justified. METHODS: Between June 1992 and April 1996, 37 patients aged 0.5-35 years (median 7.5) underwent resection of a discrete subaortic membrane. Ten underwent re-operation for recurrent obstruction and eight followed previous ventricular septal defect closure. LVOT gradient was measured using the modified Bernoulli equation and AR was graded on a scale of 0-4 (0 = none, 4 = severe). Postoperative assessment was performed early (<7 days) and at mid-term (27.0 months; range 2-59 months). RESULTS: There was significant improvement in AR from mild/moderate to none/trivial (P = 0.019) immediately postoperatively and LVOT gradient from 66.9+/-30.4 to 15.1+/-12.2 mmHg (P < 0.0001). By stepwise logistic regression preoperative gradient correlated significantly with postoperative mild/moderate AR (P = 0.015) and LVOT gradient (P = 0.0036). Preoperative mild/moderate AR also correlated with postoperative mild/moderate AR (P = 0.034). Five patients developed complete heart block, four undergoing reoperation for recurrent obstruction, and one preoperatively had right bundle branch block from previous ventricular septal defect repair. At mid-term follow-up there was no increase in AR or LVOT gradient (14.8+/-12.8 mmHg). Early post-operative AR was the strongest predictor of late mild/moderate AR (P = 0.02). Early post-operative gradient was a weaker predictor (P = 0.04). Pre-operative and early post-operative gradient were significant predictors of late gradient (P = 0.0038; <0.0001, respectively). No patient required reoperation for recurrent obstruction; one underwent late aortic valve replacement for severe AR. CONCLUSIONS: An aggressive surgical approach to discrete subaortic stenosis produces excellent relief of obstruction and frees the valve leaflets, significantly reducing associated AR at early and mid-term follow-up with low morbidity for primary operation. Long-term follow-up is required to confirm whether this early benefit is maintained.

Adolescent↗

PubMed1999

Asymmetrically short tendinous cords causing congenital tricuspid regurgitation: improved understanding of tricuspid valvar dysplasia in the era of color flow echocardiography.

BACKGROUND: Tricuspid regurgitation as a manifestation of an isolated congenital anomaly of the tricuspid valve is rare. Cross-sectional and color Doppler echocardiography allow improved evaluation of tricuspid valvar function. As a result, the heterogeneous category of congenital tricuspid valvar dysplasia may be better understood from a functional point of view. We are reporting a distinct entity in which tricuspid valvar regurgitation results from failure of coaptation due to short tendinous cords tethering the septal leaflet. PATIENTS AND RESULTS: Three children with significant primary tricuspid regurgitation were evaluated, treated, and followed. On echocardiographic evaluation, a central regurgitant jet of moderate or severe degree was directed toward the atrial septum through poorly coapting tricuspid valvar leaflets, which did not approximate due to tethering of the septal leaflet by abnormally short cords. In one patient, the tricuspid valve was otherwise normal; in the other two the leaflets and cords were also thickened. Two patients underwent surgery at 9 and 11 years of age. The cords tethering the septal leaflet were augmented by interposing appropriate lengths of expanded polytetrafluoroethylene suture and performing commissural annuloplasty. Both patients are asymptomatic 33 and 42 months postoperatively, with mild residual tricuspid regurgitation that has not changed since surgery. The other patient, an 8 month-old infant, has not yet undergone surgery. CONCLUSIONS: Asymmetric tendinous cords of the tricuspid valve causing tethering of the septal leaflet is a distinct cause of tricuspid regurgitation that can be recognized with echocardiography. Although rare, the importance of recognizing this lesion lies in its being readily amenable to surgical repair.

Child↗

PubMed1999

A 5-year experience with surgical repair of atrial septal defect employing limited exposure.

BACKGROUND: There has been a trend in recent years towards less invasive therapy for many congenital cardiac malformations. For the past 5 years, we have employed a technique of limited surgical exposure when repairing atrial defects within the oval fossa. METHODS: Over the 5-year period from July 1992 to August 1997, 115 consecutive patients underwent surgical repair of an isolated atrial septal defect in the region of the oval fossa by a single surgeon. The patients had a limited midline skin incision starting at the line of the nipples and extending inferiorly across 2 to 3 intercostal spaces. A partial sternotomy was performed, sparing the manubrium. Standard instruments and cannulation techniques were used for cardiopulmonary bypass and fibrillatory arrest. RESULTS: There were no deaths and no major complications. The median time to extubation after leaving the operating room was 3 hours (30 minutes to 8 days). Mediastinal drains were removed the morning after surgery. The median stay in the intensive care unit was 7 hours (3 hours to 10 days), and patients were discharged from the hospital a median of 4 days postoperatively (2 to 23 days). CONCLUSIONS: This approach using limited exposure can be applied safely without any new instruments and without peripheral incisions or sites of vascular access, while providing a comfortable exposure for the surgeon and achieving a cosmetically superior result for the patient.

Adolescent↗

PubMed1998

Accessory and anomalous atrioventricular valvar tissue causing outflow tract obstruction: surgical implications of a heterogeneous and complex problem.

OBJECTIVES: The purpose of this study was to determine the effect of accessory or anomalous atrioventricular valvar apparatus on relief of outflow tract obstruction. BACKGROUND: Outflow tract obstruction due to accessory tissue or anomalous attachments of the atrioventricular valvar apparatus is an unusual but well-recognized problem. In addition to obstruction, anomalous attachments of the atrioventricular valvar apparatus may interfere with procedures to relieve outflow tract obstruction or perform outflow tract reconstruction. METHODS: Since 1992, we have operated on 21 patients (median age 4 years) with systemic (n=13), pulmonary (n=5) or bilateral (n=3) outflow tract obstruction due to accessory atrioventricular valvar tissue and/or anomalous attachments of the subvalvar apparatus. Primary diagnoses were isolated obstruction of the systemic outflow tract or aortic arch (n=7), transposition complexes (n=6), previously repaired atrioventricular septal defect (n=3), functionally single ventricle (n=3) and ventricular septal defect with pulmonary outflow obstruction (n=2). Outflow tract gradients ranged from 20-110 mm Hg (median 58 mm Hg). RESULTS: Complete relief of obstruction due to atrioventricular valvar anomalies was possible in 14 patients. In six patients, the planned procedure either had to be modified or only partial relief of the obstruction was achieved. In the remaining patient, who had borderline functionally single ventricle heart disease (unbalanced atrioventricular septal defect) and systemic outflow obstruction due to accessory and functional valvar apparatus, support was withdrawn because the parents refused univentricular palliation and the valvar anomalies precluded a Ross-Konno procedure. There were two early deaths. At follow-up ranging from 1 to 66 months (median 27 months), there was one death, and there has been no recurrence of outflow tract obstruction or residual atrioventricular valvar tissue. CONCLUSIONS: Outflow tract obstruction caused by accessory or anomalous atrioventricular valvar structures is an uncommon and heterogeneous group of conditions that can have significant surgical implications. In the majority of cases, tailoring of surgical techniques will permit complete relief of obstruction. However, such anomalies may limit standard surgical options and necessitate an innovative approach in some patients.

Adolescent↗

PubMed1998

Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants <6 months of age.

The Richardson classification system for aortopulmonary septal defect (APSD) includes simple defects between the ascending aorta and pulmonary trunk (type I), defects extending distally to include the origin of the right main pulmonary artery (type II), and anomalous origin of the right main pulmonary artery from the ascending aorta with no other aortopulmonary communication (type III). These are rare lesions that must be repaired in early infancy to avoid development of pulmonary vascular disease. Few reports have focused on patients with complex, associated lesions who underwent repair in early infancy. Between 1972 and 1995, 24 patients with Richardson type I (n = 11), II (n = 7), or III (n = 6) defects underwent repair at ages ranging from 2 to 172 days (median 34). Twelve patients had complex, associated anomalies, including interrupted or hypoplastic arch (n = 9), tetralogy of Fallot with (n = 1) or without (n = 1) pulmonary atresia, and transposition of the great arteries (n = 1). The most recent 7 patients were diagnosed by echocardiography without cardiac catheterization. There were no early or late deaths among the 12 patients with simple APSD. Four patients with complex, associated lesions died in the early postoperative period and another died 4 months after surgery. All 6 surviving patients with interrupted arch have had recurrent obstruction at the arch repair site, although reintervention for this reason has been performed in only 2 patients. Altogether, 6 early survivors have required reintervention, and all survivors are in New York Heart Association class I at follow-up ranging from 2 to 25 years. Thus, long-term survival after repair of APSD in early infancy is excellent. Late sequelae are likely to be related either to associated lesions or to obstruction at the APSD repair site. Almost all cases of APSD in young infants can be diagnosed and evaluated by echocardiography without catheterization.

Aorta, Thoracic↗

PubMed1998

Trends in the management of truncal valve insufficiency.

BACKGROUND: The single semilunar valve in patients with truncus arteriosus frequently is dysplastic and dysfunctional. Truncal valve insufficiency has been associated with poor outcome. Although the management of truncal valve insufficiency has evolved over the years, approaches to this problem vary considerably and remain a serious dilemma in many cases. METHODS: We reviewed the records of 89 patients with unrepaired truncus arteriosus and mild (n = 37), moderate (n = 33), or severe (n = 19) truncal valve insufficiency who were admitted to our institution between 1975 and 1995. Eight patients (7 neonates) with moderate or severe insufficiency died before surgical intervention, and 4 patients underwent palliative pulmonary artery banding. The remaining 77 patients underwent repair. The median age at repair was 3.2 months (range, 2 days to 15 years; 83% infants), and it decreased from 4 months between 1975 and 1985 to 1 month between 1986 and 1995. Truncal valve replacement (mechanical = 6, allograft = 4) was performed in 10 patients, and 5 patients underwent valve repair. RESULTS: All 4 patients who underwent pulmonary artery banding died either early or late. The hospital (or 30-day) mortality rate after repair was 34% (26/77). At a median follow-up of 10 years, 11 hospital survivors had died, with overall 1- and 10-year actuarial survival rates of 56% and 48%, respectively, and poorer survival among patients with severe truncal valve insufficiency (p = 0.02). Late truncal valve replacement (n = 24) had been performed in 21 patients. Freedom from truncal valve replacement was better in patients with mild truncal insufficiency than in those with moderate or severe preoperative insufficiency (p < 0.001). Four late deaths were related directly to reoperation for truncal valve replacement or to prosthetic valve dysfunction. Three of the 4 neonates who received allograft root replacements died within 7 months of repair, and severe allograft valve insufficiency requiring replacement 1 year after operation developed in the fourth. CONCLUSIONS: The prospects for patients with truncal valve insufficiency have been improving over time. Nevertheless, the results in patients with severe insufficiency continue to be poor.

Follow-Up Studies↗

PubMed1998

Esophageal compression by the aorta after arterial switch.

Extrinsic compression of the esophagus in children most often occurs in the presence of a congenital vascular ring. We recently operated on a patient in whom esophageal compression had developed that was severe enough to require feeding via a gastrostomy tube several years after the arterial switch operation. Aortopexy and extensive mediastinal mobilization were performed twice with transient relief and gradual return of symptoms. Almost 3 years after the first aortopexy, lasting relief was achieved by transposing the esophagus into the right side of the chest.

Aorta, Thoracic↗

PubMed1998

Left pulmonary artery kinking caused by outflow tract dilatation after transannular patch repair of tetralogy of Fallot.

BACKGROUND: Stenosis of the branch pulmonary arteries after tetralogy of Fallot repair can result from several mechanisms. In patients with free pulmonary regurgitation and right ventricular dilatation after transannular patch repair, we have observed that the pulmonary outflow tract can dilate and elongate craniad and rotate to the left, resulting in kinking and obstruction of the previously normal left pulmonary artery. METHODS: Ten patients referred for reoperation after tetralogy repair with severe pulmonary regurgitation and right ventricular outflow tract dilatation were found to have left pulmonary artery kinking. In 8 of these patients kinking was the sole or partial cause of left pulmonary artery obstruction, whereas there was no documented obstruction in the other 2. All patients underwent right ventricular outflow tract reconstruction and left pulmonary artery repair with removal of the redundancy at the kink point. Patching alone is not an effective method of repair in this condition, because the mechanism of obstruction is not corrected and the patch material can become redundant and lead to recurrent obstruction. RESULTS: All patients underwent successful pulmonary outflow tract reconstruction. Left pulmonary artery kinking was corrected in all patients, and relief of obstruction was attained in the 8 patients with stenosis. At follow-up ranging from 9 to 58 months, no patient has evidence of recurrent left pulmonary artery stenosis. CONCLUSIONS: Left pulmonary artery kinking should be suspected at long-term follow-up after tetralogy repair in patients with significant pulmonary regurgitation and right-sided dilatation, even if previous evaluations showed no evidence of left pulmonary arterial abnormality. Because unilateral obstruction caused by kinking may lead to asymmetric pulmonary flow, it can exacerbate pulmonary regurgitation and right ventricular dilatation, in effect accelerating the processes that led to kinking in the first place. Kinking can be relieved successfully with the techniques described in this report.

Adolescent↗

PubMed1998

Repair of congenital tricuspid valve abnormalities with artificial chordae tendineae.

BACKGROUND: Congenital abnormalities of the tricuspid valve, including Ebstein's malformation, dysplasia, straddling, and those found in pulmonary atresia with intact septum and congenitally corrected transposition, are an uncommon cause of tricuspid regurgitation. Congenital tricuspid valve anomalies are found as a spectrum of disease in which both the leaflets and the subvalvar apparatus are often involved. Tricuspid valve repair is complicated in such patients because the chordae tendineae are often abnormally short and thick. Replacement or augmentation of chordae tendineae has proved to be a useful component of mitral valve repair. In the present report, we describe the techniques and results of chordal augmentation in the repair of congenital tricuspid valve abnormalities. METHODS: Since July 1992, tricuspid valve repair has been performed in 5 children with severe tricuspid regurgitation secondary to congenital abnormalities of the tricuspid valve with significant chordal pathology. As a component of the repair, chordal replacement or augmentation was performed using expanded polytetrafluoroethylene suture. RESULTS: Intraoperative and postoperative echocardiographic assessment showed good mobility of the tricuspid valve leaflets and trivial to mild tricuspid regurgitation. There were no complications and no early or late mortality. At follow-up of 34 to 60 months (median, 49 months), tricuspid valve function has remained excellent in 4 of the 5 patients. In the remaining patient, progressive regurgitation of the right ventricle to pulmonary artery allograft conduit has led to right ventricular dilatation, with a secondary increase in tricuspid regurgitation from trivial to moderate. CONCLUSIONS: Chordal replacement or augmentation with expanded polytetrafluoroethylene suture is a useful technique in the repair of congenitally dysplastic tricuspid valves with abnormal chordal structures.

Adolescent↗

PubMed1998

Repair of secundum atrial septal defect: limiting the incision without sacrificing exposure.

A simple and effective technique for repair of secundum atrial septal defect is described. The heart is exposed through a limited midline skin incision and partial sternotomy, and the atrial septal defect is closed through a right atriotomy with ascending aortic and dual venous cannulation. This approach achieves a cosmetically superior result with standard instrumentation and cardiopulmonary bypass techniques, without compromising exposure or using peripheral incisions.

Cardiac Surgical Procedures↗

PubMed1998

Alternative approach to the repair of Ebstein's malformation: intracardiac repair with ventricular unloading.

BACKGROUND: Moderate to severe Ebstein's malformation remains a surgical challenge. Although the various approaches that have been used are appropriate and successful in many patients, there are many for which these approaches are suboptimal. To improve the prognosis for patients across the full spectrum of Ebstein's malformation, alternative surgical approaches are necessary. METHODS: From December 1995 to October 1997, 10 patients (median age, 9 years) with moderate or severe Ebstein's malformation and mild to severe tricuspid regurgitation had partial biventricular repair with reduction of right ventricular volume load. All patients were symptomatic in New York Heart Association functional class II (n = 9) or III (n = 1). In addition to bidirectional cavopulmonary anastomosis and closure of intracardiac defects in all patients, 6 underwent tricuspid valve repair using a variety of procedures, most often simple horizontal annuloplasty. RESULTS: There were no deaths. Early reoperation was required in 1 patient (atrial septostomy on the day after operation for right ventricular failure) and another required revision of the tricuspid valve repair 10 months postoperatively for recurrent regurgitation. At follow-up ranging from 2 to 24 months, all patients are in New York Heart Association class I and have trivial tricuspid regurgitation, including the 4 who had no tricuspid valvuloplasty performed. CONCLUSIONS: We have presented an alternative approach to the management of severe Ebstein's malformation that focuses on both the tricuspid valve and the right ventricle. Just as tricuspid valve repair and reduction of regurgitation will likely improve right ventricular performance, reducing the volume load on the ventricle may improve both ventricular (right and left) and tricuspid valve function. All patients have demonstrated improved exercise tolerance and right heart function at follow-up ranging to 24 months. Additional experience will be necessary to evaluate this strategy more completely.

Adult↗

PubMed1998

Extracardiac conduit Fontan procedure without cardiopulmonary bypass.

There are a number of potential advantages of extracardiac conduit cavopulmonary anastomosis for palliation of functional single ventricle heart disease, including the ability to perform the operation with no aortic cross-clamping and with minimal duration of extracorporeal circulation. In many patients, it may be possible to perform the procedure without cardiopulmonary bypass altogether. In this report, we present our technique for performing the extracardiac conduit Fontan operation without cardiopulmonary bypass.

Anastomosis, Surgical↗

PubMed1998

Geometric mismatch of pulmonary and aortic anuli in children undergoing the Ross procedure: implications for surgical management and autograft valve function.

BACKGROUND: There is often substantial mismatch between the diameters of the pulmonary and aortic anuli in young patients with systemic outflow tract disease. To implant the autologous pulmonary valve in the aortic position under such circumstances, it is necessary to adapt the geometry of the systemic outflow tract. The effects of such adaptations on autograft function in children are not well known. METHODS: To determine factors predictive of autograft regurgitation, we analyzed 41 cases of children who have undergone the Ross procedure. The diameter of the pulmonary valve was greater (by at least 3 mm) than that of the aortic valve in 20 cases, equal (within 2 mm) in 12 cases, and less (by at least 3 mm) in nine cases, with differences ranging from +10 to -12 mm. In 12 patients with a larger pulmonary anulus, aortoventriculoplasty was used to correct the mismatch. In patients with a larger aortic anulus, the mismatch was corrected by gradual adjustment along the circumference of the autograft, rather than by tailoring of the native aortic anulus. RESULTS: At follow-up (median 31 months), two patients had undergone reoperation on the neoaortic valve for moderate regurgitation. In the remaining 38 cases, autograft regurgitation was as follows: none or trivial in 30, mild in seven, and moderate in one. There was no correlation between regurgitation and age, geometric mismatch, or previous or concurrent procedures. CONCLUSIONS: Subtle technical factors that may result in distortion of the valve complex are probably more important determinants of autograft regurgitation than are indication for repair, geometric mismatch, or previous or concomitant outflow tract procedures. Significant mismatch of the semilunar anuli is not a contraindication to the Ross procedure in children.

Adolescent↗