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Mark W Turrentine

Publications and source records attributed to Mark W Turrentine.

At least 19 recordsLinked to original sources
PubMed2006

Outcomes in patients with interrupted aortic arch and associated anomalies: a 20-year experience.

OBJECTIVE: The surgical results for the repair of interrupted aortic arch (IAA) have evolved in recent years. We report our results for staged repair of this complex congenital malformation. METHODS: Sixty-five patients (mean age, 16.9+/-41.7 days) were diagnosed with IAA and referred for surgical therapy. The surgical management strategy at our institution between 1982 and 2005 has been one-stage complete repair (n=13) or staged repair (n=52) in selected patients. Non-complex patients (group I, n=51) had a ventricular septal defect (87%), aortopulmonary window (8%), and left ventricular outflow tract obstruction (27%). Group II (n=14) were patients with Taussig-Bing double outlet right ventricle (n=6) or truncus arteriosus (n=8). Method of staged repair of IAA was to transect and turn down the left carotid artery and anastomosis it to the descending aorta (n=41) or graft interposition (n=2) combined with a pulmonary artery (PA) banding followed in a few months by delayed ventricular septal defect (VSD) closure and PA de-banding. RESULTS: There were 5 early and 10 late deaths. The actuarial survival including early mortality was 92% at 1 year, 81% at 5 years, and 76% at 10 and 15 years. There was an 81% 15-year survival for children in group I compared with a 54% for children in group II (p<0.001). Risk factors for increased mortality by univariate analysis were as follows: (1) primary aortic anastomosis (p=0.03), (2) presence of complex anomalies (p=0.05), and (3) initial IAA repair performed before 1994 (p=0.05). Actuarial freedom from any type of aortic reoperation or intervention was 86% at 1 year, 69% at 5 years, and 60% at 10 and 15 years. Univariate and multivariate analyses identified no tested variables as risk factors for reoperation. The majority (86%) was in New York Heart Association (NYHA) class I, and 14% remained in NYHA class II. During the postoperative course there were no neurologic deficits, seizures, and growth disturbances in any patient. CONCLUSION: Staged repair of IAA using a left carotid artery turn down can be safely applied in IAA patients with and without other intracardiac anomalies with good results. Use of the left carotid artery for arch reconstruction did not result in any detectable neurological events or growth disturbances later in life. Associated anomalies played an important role in outcomes. The long-term probability for reoperation and/or reintervention remains high regardless of operative technique.

Aorta, Thoracic↗
PubMed2006

Evolution of aortic valve replacement in children: a single center experience.

BACKGROUND: The availability of an ideal prosthesis for aortic valve replacement (AVR) in children remains controversial due to an early degeneration of xenografts and the potential risks related to anticoagulation with mechanical prostheses. This has led many surgeons to the Ross procedure. This study outlines the evolution of our 30-year experience with AVR in children. METHODS: One hundred and forty-seven children, aged 10 days to 18 years (mean 11.9+/-5.7 years), underwent AVR between 1974 and June 2005. Preoperative diagnosis included aortic insufficiency (n=39), aortic stenosis (n=14), combined aortic stenosis with insufficiency (n=78), and complex left ventricular outflow tract obstruction (n=16). Xenografts (n=11) and mechanical prostheses (n=47) were used in 58 patients. The remaining 89 patients had placement of homografts (n=8) or underwent a Ross procedure (n=81). Of the 147 patients, 87 (59%) had previous procedures. RESULTS: Overall early and late mortality was 7.5% (11/147 pts). Overall survival estimated by the Kaplan-Meier method, including early mortality, was 94% at 1 year, and 93% at 5, 10, and 20 years. Univariate and multivariate analysis identified date of operation (before 1980) as a risk factor for death (p=0.002). Follow-up was complete in 136 patients (5 lost to follow-up), with a total follow-up of 2433.72 patient-years. The overall study group aortic valve-related reoperation rate was 20% (20/138 pts), the reoperation rate was highest in xenograft group (60%); followed by mechanical valves group (16%), homograft group (14%), and the Ross procedure group (9%). Ross patients showed significant increase of the annulus diameter (p=0.002) and the aortic sinus diameter (p=0.01) at the last follow-up. The actuarial rate for freedom from aortic valve-related reoperation was 99% at 1 year, 94% at 5 years, 88% at 10 years, and 85% at 20 years. Univariate and multivariate analysis identified the presence of a xenograft as a risk factor for aortic valve-related reoperation (p=0.001). CONCLUSION: AVR in children can be performed with acceptable mortality and minimal mid-term morbidity. The Ross procedure, although more complicated, has the advantage of not requiring anticoagulation. Pulmonary autograft, in our series, has demonstrated growth with no structural degeneration. The potential for development of significant autograft insufficiency and ascending aortic aneurysmal dilatation is small but warrants annual follow-up. Our data supports that the Ross procedure is the AVR of choice in children.

Adolescent↗
PubMed2006

Differential effects of phosphodiesterase-5 inhibitors on hypoxic pulmonary vasoconstriction and pulmonary artery cytokine expression.

BACKGROUND: Perioperative pulmonary hypertension is a challenging clinical problem with numerous etiologies including hypoxia, adrenergic stimulation, and local inflammation. New oral phosphodiesterase-5 (PDE-5) inhibitors used for the treatment of erectile dysfunction may have beneficial effects on the pulmonary vasculature owing to the abundance of PDE-5 receptors in the lung. The purpose of this study was to compare the efficacy of sildenafil, vardenafil, and tadalafil in preventing acute hypoxic pulmonary vasoconstriction and hypoxia-induced pulmonary artery tumor necrosis factor-alpha (TNF-alpha) and interleukin-1-beta (IL-1beta) expression. METHODS: Isolated rat pulmonary arteries suspended in physiologic organ baths for measurement of isometric force transduction were treated with vehicle (dimethyl sulfoxide), sildenafil, vardenafil, or tadalafil to assess (1) pulmonary artery relaxation; (2) inhibition of phenylephrine-induced pulmonary artery contraction; (3) inhibition of hypoxic pulmonary vasoconstriction (pO2 = 30-35 mm Hg); and (4) hypoxia-induced pulmonary artery TNF-alpha and IL-1beta expression (reverse transcriptase-polymerase chain reaction). RESULTS: Sildenafil, vardenafil, and tadalafil resulted in dose-dependent pulmonary artery relaxation and inhibited phenylephrine-induced pulmonary artery contraction, but only tadalafil significantly inhibited hypoxic pulmonary vasoconstriction (52.08% +/- 7.65% tadalafil versus 88.63% +/- 8.96% vehicle; 98.61% +/- 10.04% sildenafil; 68.46% +/- 15.84% vardenafil). Hypoxia-induced upregulation of TNF-alpha and IL-1beta mRNA in pulmonary artery was significantly decreased by tadalafil, but not sildenafil or vardenafil pretreatment. CONCLUSIONS: We conclude that sildenafil, vardenafil, and tadalafil were equally efficacious in causing pulmonary artery relaxation, but only tadalafil inhibited hypoxic pulmonary vasoconstriction and attenuated hypoxia-induced pulmonary artery TNF-alpha and IL-1beta expression.

3',5'-Cyclic-GMP Phosphodiesterases↗
PubMed2006

Closed transventricular aortic valvotomy for critical aortic stenosis in neonates: outcomes, risk factors, and reoperations.

BACKGROUND: Critical aortic stenosis (AS) in neonates necessitates urgent intervention for patient survival. The optimal treatment, however, continues to be controversial and still has high morbidity and mortality in many centers. This study examined our late outcome, risks, and reoperations after the treatment of critical AS in neonates. METHODS: Sixty-six neonates (47 boys and 19 girls) underwent closed transventricular aortic valvotomy for critical AS between 1978 and 2000. The mean age at the first intervention was 15.1 +/- 19.6 days (range, 1 to 78). Their weight ranged from 1.4 to 6.2 kg (mean, 3.5 +/- 0.9 kg). Sixteen patients (23%) had isolated critical AS and normal or dilated left ventricles, and 50 neonates (77%) had associated cardiovascular anomalies (ie, aortic annular hypoplasia, hypoplasia of the left ventricle, mitral valve abnormalities, and endocardial fibroelastosis, coarctation, or interruption of aorta). RESULTS: The hospital mortality was 29% (19 of 66). Kaplan-Meier 5- and 15-year survival was 61% and 58%, respectively. The operative mortality rates were 6% in neonates with isolated AS and 36% in patients with complex AS. The five risk factors significant for mortality on univariate analysis were (1) presence of endocardial fibroelastosis (p = 0.05), (2) presence of hypoplastic left ventricle (p = 0.003), (3) presence of associated cardiovascular anomalies (p = 0.04), (4) aortic valve annulus of less than 5.0 mm (p = 0.01), and (5) surgery before 1985 (p = 0.003). Of these five factors, only the presence of hypoplastic left ventricle (p = 0.001) and surgery before 1985 (p = 0.001) remain significant for mortality by multivariate analysis. At last follow-up (mean, 8.2 +/- 6.2 years), 36 of 47 of the long-term survivors were in New York Heart Association functional class I and II. Kaplan-Meier analysis showed 5- and 15-year freedom from aortic valve reoperation to be 83% and 60%, respectively. Univariate and multivariate analysis showed the presence of multilevel stenosis (p = 0.04) as the best preoperative predictor for the need for late reoperation in survivors. CONCLUSIONS: We conclude that critical AS in neonates continues to be associated with significant mortality. Reintervention such as surgical and balloon valvotomy are usually required within 10 years of initial surgery.

Alprostadil↗
PubMed2006

Caval division technique for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection.

BACKGROUND: Repair of sinus venosus atrial septal defect (ASD) with high partial anomalous pulmonary venous connection (PAPVC) using an internal patch may be complicated by obstruction of the superior vena cava (SVC) or pulmonary veins, or both, and sinus node dysfunction. In cases in which the anomalous veins insert more than 2 cm above the cavoatrial junction, we have adopted the technique of caval division in which the SVC is divided and the proximal end is anastomosed to the right atrial appendage, and the distal SVC serves as a conduit for pulmonary venous drainage to the left atrium through the ASD. We retrospectively compare the results of the internal patch repair versus the Warden technique. METHODS: Between 1991 and 2004, 54 patients diagnosed with sinus venosus ASD and PAPVC have undergone repair at our institution. Mean age was 13.4 years (range, 1.5 to 58). Thirteen patients (24%) had high insertion of anomalous veins and underwent the Warden technique. Follow-up averages 4.3 years (range, 1 to 13). RESULTS: There were no early or late deaths. All patients remain in normal sinus rhythm. Twelve of the 13 patients with Warden procedure have had postoperative echocardiograms, and 11 of these patients showed no evidence of SVC or pulmonary venous obstruction. In 1 patient, symptomatic pulmonary venous obstruction developed and required revision of a contracted intra-atrial pericardial baffle. CONCLUSIONS: Caval division for treatment of high PAPVC appears to be safe and is associated with low morbidity and mortality. The Warden procedure is an effective surgical option for patients undergoing correction of high PAPVC.

Abnormalities, Multiple↗
PubMed2006

Mitral valve replacement with Ross II technique: initial experience.

BACKGROUND: Pulmonary autograft mitral valve replacement (PA-MVR) was introduced clinically by Ross in 1967, but has been rarely utilized in North America and Europe. The aim of this study is to review our early experience with PA-MVR. METHODS: Since June 2002, 8 patients (7 female and 1 male) between 12 to 46 years of age with outgrown mechanical valves (n = 3) failed MV repair for rheumatic or congenital MV disease (n = 4) and irreparable bileaflet myxoid prolapse (n = 1), underwent PA-MVR. The pulmonary autograft was harvested and replaced using a pulmonary homograft. The autografts were mounted within a woven Dacron graft 6-8 mm greater in diameter than the autograft annulus diameter on a preoperative echocardiogram. The graft's external surface was covered with fresh autologous pericardium. RESULTS: There were no deaths. Intraoperative echocardiography confirmed a mean MV gradient of 4 mm Hg with trivial (n = 7) or mild (n = 1) regurgitation. Follow-up (range, 13 to 36 months) echocardiography in 4 patients showed no increase in MV gradient or regurgitation. One patient with severe myxoid degeneration and one patient with rheumatic disease, both with systemic hypertension, developed progressive regurgitation due to stretching of a single autograft leaflet producing prolapse. One patient developed a moderate gradient due to retention of excessive native mitral leaflet and subannular chordal tissue. Three of 4 patients have required PA-MVR replacement with mechanical valves 6 to 14 months post-PA-MVR. One asymptomatic patient with mild to moderate mitral regurgitation is being followed after treatment of her systemic hypertension. CONCLUSIONS: Pulmonary autograft mitral valve replacement offers selected patients a potentially lifelong autologous valve without the need for long-term anticoagulation. The PA-MVR technique deserves careful consideration in younger patients in sinus rhythm. Postoperative systemic hypertension should be treated aggressively to prevent excessive stress on the pulmonary autograft particularly in the early postoperative months.

Adolescent↗
PubMed2006

The Ross-Konno procedure in children: outcomes, autograft and allograft function, and reoperations.

BACKGROUND: The Ross aortic valve replacement with a modified Konno-type enlargement Ross-Konno procedure of the aortic annulus and subannular region allows an autograft aortic valve replacement for children with significant annular and subannular hypoplasia. The potential for growth and the proven durability of the autograft make the Ross-Konno procedure an ideal aortic valve replacement for this subgroup with multilevel left ventricular outflow tract obstruction. We reviewed our institutional midterm experience to assess autograft and homograft hemodynamics, and management after a Ross-Konno procedure. METHODS: Between 1995 and 2005, 14 consecutive children (mean age, 6.4 +/- 5.9 years; range, 1 month to 17 years) underwent the Ross-Konno procedure. All children had severe to critical aortic stenosis or multilevel left ventricular outflow tract obstruction. RESULTS: There was 1 early and 1 late death with a mean follow-up of 5.7 +/- 3.6 years. Actuarial survival at 10 years was 86%. Three patients underwent right ventricular outflow tract reoperation for conduit replacement for homograft dysfunction and one patient required redo aortic root replacement with a mechanical valves for progressive aortic insufficiency. Freedom from right ventricular outflow tract and autograft reoperation at 10 years is 77% and 92%, respectively. Aortic annular dilation was not observed in all patients. Univariate and multivariate analysis identified no risk factors for autograft or homograft valve-related reoperation. CONCLUSIONS: The Ross-Konno procedure is an excellent technique to treat complex multilevel left ventricular outflow tract obstruction in children with significant annular and subannular hypoplasia. The autograft demonstrated durability without development of aortic stenosis or progressive dilation and a low incidence of developing progressive aortic insufficiency. Enlargement of the aortic annulus appear to parallel somatic growth in most instances.

Adolescent↗
PubMed2006

Surgical treatment of absent pulmonary valve syndrome associated with bronchial obstruction.

BACKGROUND: Absent pulmonary valve syndrome (APVS) is a relatively rare anomaly that is usually associated with a ventricular septal defect and a restrictive pulmonary annulus with severe pulmonary regurgitation. The to-and-fro flow across the right ventricular outflow tract produces enormously dilated and pulsatile pulmonary arteries (PAs) that cause severe respiratory distress and tracheomalacia by compression of the trachea and primary bronchi. This retrospective study summarizes our 20-year experience of surgical treatment in patients with APVS. METHODS: Between 1984 and 2005, 20 patients underwent repair of APVS using a valved conduit (n = 9), transannular patch (TAP) insertion alone (n = 5), or TAP with monocusp valve (n = 6) with PA reduction arterioplasty. Median age was 7 months (range, 6 days to 9 years). RESULTS: There were one perioperative and two late deaths. All deaths were related to airway complications. Survival was 90% at 1 year and 85% at 10 and 15 years. In a multivariate analysis only preoperative ventilator dependency was associated with a worse outcome (p = 0.02). Follow-up was available in 18 operative survivors (mean follow-up, 7.3 +/- 5.2 years). Six patients (33%) underwent reoperation for pulmonary valve incompetence and right ventricular dysfunction. Three patients (17%) had episodic bronchospasm of mild to moderate severity that were responsive to sympathomimetic bronchodilators. CONCLUSIONS: Morbidity associated with perioperative respiratory complications and ventilator dependency due to underlying tracheobronchomalacia is an important problem in patients with APVS. These infants may require multiple hospitalizations for recurrent respiratory infections secondary to their tracheobronchomalacia. Complete repair with a valved conduit and reduction pulmonary arterioplasty at the onset of symptoms and a definitive diagnosis is our procedure of choice for infants with APVS. With this approach, the airway can be optimized to give the best patient outcome.

Airway Obstruction↗
PubMed2006

Lepirudin anticoagulation for heparin-induced thrombocytopenia after cardiac surgery in a pediatric patient.

A 21-month-old boy received anticoagulation with lepirudin for heparin-induced thrombocytopenia (HIT) after undergoing cardiac surgery and receiving extracorporeal membrane oxygenation (ECMO). This report illustrates the significance of HIT in pediatric patients after cardiac surgery and the successful administration of lepirudin in this setting. To our knowledge, this is the first published report of lepirudin administered to treat HIT in a child after cardiac surgery and ECMO. Although guidelines exist that suggest the potential administration of lepirudin as treatment for children with HIT, further studies are needed to determine the safest yet most effective dosage for this population.

Anticoagulants↗
PubMed2006

Fate of the autograft and homograft following Ross aortic valve replacement: reoperative frequency, outcome, and management.

BACKGROUND AND AIM OF THE STUDY: The optimal hemodynamic performance and potential for growth of the pulmonary autograft has led to expanded indications for the Ross aortic valve replacement (AVR) procedure in some centers. The authors' institutional mid-term experience was reviewed to assess autograft and homograft hemodynamics, growth profile of the autograft, and reoperative frequency following Ross AVR. METHODS: Between June 1993 and June 2005, 167 consecutive patients (mean age 24.9 +/- 15.5 years; range: 1 month to 61 years) underwent Ross AVR: 48% of patients were aged < 19 years. Additional procedures (n = 78) were performed in 55 patients (33%) at the time of the Ross procedure. In total, 151 patients had isolated aortic valve disease and 16 pediatric patients had more complex, multi-level left ventricular outflow tract obstruction. RESULTS: There were two early deaths (1.2%) and one late death (0.6%) over a mean follow up of 5.1 +/- 3.0 years (range: 1 month to 11 years). Actuarial survival at 10 years was 98%. In pediatric patients with Konno procedure (n = 16), the pulmonary autograft mean annulus diameter increased from 10.2 to 19.9 mm. Twelve patients underwent 12 reoperations without mortality for autograft insufficiency or an ascending aortic aneurysm at a median interval of 5 years (range: 2 to 8 years): aortic annuloplasty and ascending aorta replacement (n = 4), composite aortic root replacement (n = 7), and repair of left ventricular pseudoaneurysm (n = 1). Freedom from replacement of the pulmonary autograft was 96% at 10 years. Five of the 164 surviving patients (3%) developed significant obstruction of the pulmonary homograft and required conduit replacement at a median of four years. CONCLUSION: The Ross AVR can be performed with good mid-term results, including the pediatric age group. The potential for development of significant autograft insufficiency and homograft stenosis warrants annual follow up through the intermediate and late terms.

Adolescent↗
PubMed2005

Acute hemodynamic effects of pacing in patients with Fontan physiology: a prospective study.

OBJECTIVES: The purpose of this research was to assess the hemodynamic response to atrial, ventricular, and dual-chamber pacing in patients with Fontan physiology. BACKGROUND: Bradycardia, due to sinus node dysfunction or atrioventricular (AV) block, with need for pacing, is common after the Fontan operation. The optimal pacing mode for Fontan patients is unknown, but is critical, as hemodynamic aberrancies may cause severe clinical deterioration. We hypothesized that AV synchrony is vital for maximizing Fontan hemodynamics. METHODS: A cross-over trial was conducted with 21 patients (age 2 to 18 years, median 4 years; male patients = 13) in the intensive care unit after a Fontan operation. Hemodynamic parameters, including mean left atrial pressure (LAP, in mm Hg), mean pulmonary artery pressure (PAP, in mm Hg), mean arterial blood pressure (MAP, in mm Hg), and indexed cardiac output via Fick (Qs, in l/min/m2) were measured with atrial, ventricular, and dual-chamber pacing. Measurements were made after pacing for 10 min in each mode, and a 10-min rest was given between each pacing maneuver. RESULTS: Asynchronous ventricular (VOO) pacing resulted in significantly worse hemodynamics when compared to dual-chamber (DOO) and atrial (AOO) pacing with a higher LAP (9.4 VOO; 6.8 DOO; 5.4 AOO) and PAP (15.2 VOO; 13.5 DOO; 12.7 AOO) and lower Qs (3.0 VOO; 3.5 DOO; 3.9 AOO) and MAP (60.1 VOO; 66.5 DOO; 67.2 AOO). CONCLUSIONS: Asynchronous ventricular pacing, after the Fontan procedure, has acute, adverse hemodynamic consequences (elevated LAP and PAP and decreased Qs and MAP).

Adolescent↗
PubMed2005

Preconditioning: gender effects.

Preconditioning is injury induced protection from subsequent injury. During preconditioning protective cellular responses to injury are up regulated resulting in acute and delayed defense against further damage. Several studies indicate that females experience a protective advantage after acute insult compared to males. Despite evidence of gender differences in acute injury, relatively few studies have evaluated whether there are sex differences in preconditioning. Variations in patients' pre-morbid preconditioning status may explain outcome variations that are not apparent in small animal studies. This review discusses the differences in response to acute injury experienced by males and females, the basic mechanisms of preconditioning, and the sex differences in the mechanisms of preconditioning.

Animals↗
PubMed2005

Staged surgical repair of functional single ventricle in infants with unobstructed pulmonary blood flow.

OBJECTIVE: The infant with a functional single ventricle (SV) and unobstructed pulmonary blood flow (UPBF) requires early protection of the pulmonary vascular bed to ensure suitability for a subsequent Fontan procedure. Systemic obstruction by aortic arch obstruction, subaortic stenosis, or combination of both, has been widely recognized as an important risk factor for poor outcome in children with SV-UPBF who are palliated with pulmonary artery banding (PAB). We reviewed our experience with primary PAB in the subset of patients with SV-UPBF to identify risk factors for subsequent palliative procedures and Fontan completion. METHODS: Between January 1990 and May 2004, 80 patients (median age, 14 days) with functional SV and UPBF underwent PAB as their primary palliative procedure. Thirty-five neonates had concomitant aortic coarctation or interrupted aortic arch repair (44%). A Damus-Kaye-Stansel procedure was subsequently performed in 19 patients, and subaortic resection or ventricular septal defect or bulboventricular foramen enlargement was performed in five. RESULTS: There were 4 operative deaths, and 15 late deaths. The actuarial overall survival is 84% at 1 year, 76% at 5 and 15 years. Follow-up is complete in all but six children at a mean interval of 4.9+/-3.7 years (range, 2 months-15 years). Thirty-seven patients (49%; 37 of 76) have undergone the hemi-Fontan procedure (with three hospital deaths) and 40 patients (53%; 40 of 76; 12 children without previous hemi-Fontan) have undergone the completion Fontan procedure without mortality or Fontan takedown. CONCLUSION: In infants with single ventricle physiology with or without systemic outflow obstruction and unobstructed pulmonary blood flow, a strategy of pulmonary artery banding carries acceptable operative and mid-term mortality in a high-risk group of patients. Pulmonary artery banding does not compromise performance of subsequent Damus-Kaye-Stansel procedure or completion Fontan palliation.

Female↗
PubMed2005

Surgical treatment of pulmonary artery sling and tracheal stenosis.

BACKGROUND: Pulmonary artery sling is a rare vascular ring and is commonly associated with tracheal stenosis. Symptomatic newborns and infants with these complex lesions have a high mortality rate without surgical intervention. The ideal operation remains controversial, with debate focusing on the need for left pulmonary artery for reimplantation and the technique of tracheal reconstruction. METHODS: From 1983 to 2003, 14 patients with pulmonary artery sling (mean age, 7 months; range, 6 days to 27 months) underwent repair of pulmonary artery sling alone (6 patients), tracheoplasty alone (1 patient), and pulmonary artery sling repair with tracheoplasty (7 patients). Preoperatively, 7 patients were intubated, 2 had VATER (vertebral, anal, tracheal, esophageal, and radial anomalies) syndrome, and 2 patients had agenesis of the right lung. The left pulmonary artery was reimplanted at the ductal insertion site in 13 patients. One patient had left pulmonary artery translocation. Tracheoplasty employing extracorporeal circulation consisted of autologous pericardial patch (6 patients) or slide tracheoplasty (2 patients). Six patients with pulmonary artery sling and mild tracheal stenosis required only left pulmonary artery reimplantation. Concomitant procedures included closure of atrial septal defect (4 patients), ventricular septal defect (4 patients), and shunt for Fallot's tetralogy (2 patients). RESULTS: There were 2 hospital deaths (2 of 14;14%) from abdominal sepsis (1) and renal failure (1). Reoperations included diaphragm plication (2), tracheostomy (1), and bronchoscopy with laser resection of granulation tissue (5 patients: 2 slide, 3 pericardium). Follow-up was complete in all patients (mean, 42 months) with 1 late death from fungal sepsis. At follow-up, all left pulmonary artery anastomoses were patent by echocardiography, and no patient has required reoperation for trachea reconstruction. CONCLUSIONS: These data demonstrate that tracheal repair is not always necessary in the presence of pulmonary artery sling; that agenesis of the right lung is not a contraindication to successful complete repair; and that simultaneous correction of intracardiac defects can be safely performed in selected patients. This study suggests that in newborns and infants, pericardial patch and slide tracheoplasty are effective methods for trachea reconstruction.

Abnormalities, Multiple↗
PubMed2005

Operative results and outcomes in children with Shone's anomaly.

BACKGROUND: The outcome of children with multilevel left heart obstructions with mitral valve involvement (Shone's anomaly) is generally poor. This study reviews our results and outcomes in patients with staged repair for left ventricular outflow tract obstruction (LVOTO) and mitral valve reconstruction. METHODS: A total of 27 children underwent staged repair for Shone's anomaly between 1978 and 2003. The mean age at the first operation was 9.0 months (range, 2 days to 3 years). Mitral stenosis was present in all; with supravalvular mitral ring (n = 11), mitral valve abnormalities including parachute mitral valve, fused chordae, single papillary muscle (n = 10), and "typical" (Ruckman & Van Praagh) congenital mitral stenosis (n = 14). The LVOT obstruction features included subaortic stenosis (n = 16), valvar aortic stenosis (n = 7), bicuspid aortic valve (n = 24), and coarctation (n = 18). All 27 patients underwent 94 surgical procedures, including 22 mitral operations and 48 LVOT operations. RESULTS: There were no operative deaths at the first operation. Mean follow-up was 4.7 +/- 4.4 years (range, 6 months to 15 years). There were three late deaths (11%). All late deaths were secondary to severe mitral valve disease. Overall 15-year actuarial survival was 89%. All surviving patients are in New York Heart Association functional class I or II. CONCLUSIONS: Patients with Shone's anomaly are seen with a wide spectrum of major anatomic and hemodynamic abnormalities. A favorable outcome is possible for most. Operative mortality is adversely affected by the severity of mitral valve disease, the degree of left ventricular hypoplasia, and the need for multiple operative procedures.

Adolescent↗
PubMed2005

Right ventricular outflow tract reconstruction with an allograft conduit in non-ross patients: risk factors for allograft dysfunction and failure.

BACKGROUND: Allograft conduits (AC) are used for reconstruction of the right ventricular outflow tract (RVOT) in children with RVOT hypoplasia or atresia and for pulmonary valve replacement in children undergoing Ross aortic valve replacement (AVR). Children undergoing the Ross AVR are generally older and have their AC inserted in the orthotopic position as opposed to the heterotopic position used for most cases of complex RVOT obstruction. The orthotopic position of the AC combined with the fact that the AC in the Ross patients are larger and can be oversized are the three factors that increase the right ventricular-pulmonary artery (RV-PA) conduit durability in the Ross AVR group. A retrospective evaluation of our experience with use of AC in non-Ross patients for reconstruction of the RVOT was conducted to determine the risk factors for failure. METHODS: Between January 1985 and December 2003, 117 non-Ross patients received AC (94 pulmonary and 23 aortic) for RVOT reconstruction. Median age at implantation was 8 months (mean 4.3 +/- 7.1 years; range, 6 days to 43 years). There were 57 children (49%) less than 12 months of age. Endpoints were AC failure (valve explant, balloon dilatation), and AC dysfunction (AC stenosis >40 mm Hg and AC insufficiency more than 2+). There were no device-related deaths. RESULTS: Overall patient survival was 80% at 15 years. Freedom from AC failure was 60% at 5 years and 43% at 15 years. Freedom from failure was worse in infants (42% and 34% at 5 and 15 years, respectively). Freedom from AC dysfunction was 40% at 5 years and 23% at 15 years. Freedom from dysfunction was worse in infants (21% and 16% at 5 and 15 years, respectively). Univariate analysis identified younger patient age, smaller AC size, diagnosis of truncus arteriosus, and the presence of aortic AC as risk factors for AC dysfunction and failure. Multivariate analysis identified smaller AC size and the presence truncus arteriosus as risk factors for AC dysfunction and failure. CONCLUSIONS: Right ventricular outflow tract reconstruction with an AC in non-Ross patients has poor performance at midterm follow-up with AC dysfunction and failure of, respectively, 60% and 40% for the entire group and 79% and 58% in the infant group at 5 years. An alternate conduit for this application must be considered.

Adolescent↗
PubMed2005

Pulmonary arteriovenous malformations in children after the Kawashima operation.

BACKGROUND: Pulmonary arteriovenous malformations are a major cause of progressive late cyanosis in patients treated with cavopulmonary anastomoses. Previous experience suggests that exclusion of the hepatic venous effluent from the pulmonary circulation may cause the development of pulmonary arteriovenous malformations after the Kawashima operation in children with interrupted inferior vena cava with azygous continuation. METHODS: From January 1990 to November 2004, 21 children (median age, 2 years) with heterotaxy syndrome and interrupted inferior vena cava with azygous continuation underwent Kawashima operation. The average preoperative arterial oxygen saturation was 76% +/- 7% (range, 64% to 90%). RESULTS: Follow-up was complete in all survivors except 1 at a median duration of 4.5 years. Pulmonary arteriovenous malformations were diagnosed at a median of 5 years after Kawashima operation in 11 patients (58%). Completion Fontan operation has been performed in 15 (79%). Five children who underwent a completion Fontan procedure 1 to 1.5 years after Kawashima operation did not have pulmonary arteriovenous malformations. There were 2 late deaths due to chronic congestive heart failure 4 months and 7 years after Kawashima operation. Overall survival at 10 years is 90%. Univariate and multivariate analysis demonstrated presence of bilateral superior vena cavae (p = 0.002) and interval longer than 2 years between Kawashima operation and completion Fontan operation (p = 0.04) as predictors of developing pulmonary arteriovenous malformations. CONCLUSIONS: In most patients with heterotaxy and interrupted inferior vena cava with azygous continuation, clinical evidence of pulmonary arteriovenous malformations will develop after Kawashima operation if they are followed up long enough. Early redirection of the hepatic venous effluent to the pulmonary arterial circulation may prevent or lead to regression of pulmonary arteriovenous malformations, with low mortality and morbidity.

Arteriovenous Malformations↗