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Biomedical subjects

W G Williams

Publications and source records attributed to W G Williams.

At least 19 recordsLinked to original sources
PubMed1998

Elevated insulin-like growth factor-I and transforming growth factor-beta 1 and their receptors in patients with idiopathic hypertrophic obstructive cardiomyopathy. A possible mechanism.

BACKGROUND: Idiopathic hypertrophic obstructive cardiomyopathy (HOCM) is characterized by regional myocardial hypertrophy. In our previous study, we demonstrated that mRNA levels for insulin-like growth factor-I (IGF-I) and transforming growth factor-beta 1 (TGF-beta 1) were elevated in HOCM tissue. In this study, we investigated IGF-I and TGF-beta 1 protein levels and their respective receptor levels and localization. METHODS AND RESULTS: Myocardial growth factor protein levels were quantified with the use of chemiluminescent slot blot analysis with monoclonal antibodies against IGF-I and TGF-beta. The growth factor receptor binding sites were evaluated with 125I-labeled IGF-I and TGF-beta 1. The receptors were localized with immunohistochemistry. Data were expressed as mean +/- SEM. IGF-I and TGF-beta protein levels in HOCM myocardium (351.8 +/- 46.5 and 17.4 +/- 2.0 ng/g tissue, respectively; n = 6) were significantly higher (P < 0.01 for all groups) than in non-HOCM myocardium obtained from patients with aortic stenosis (AS, 182.1 +/- 22.7 and 8.0 +/- 1.2 ng/g tissue, respectively; n = 5), stable angina (SA, 117.4 +/- 20.9 and 7.5 +/- 2.7 ng/g tissue, respectively; n = 5), and transplanted hearts (TM, 166.3 +/- 30.1 and 6.4 +/- 1.2 ng/g tissue, respectively; n = 5). Maximal and high-affinity binding sites for IGF-I receptor in the HOCM were greater (P < 0.01 and P < 0.05) than the levels in AS, SA, and TM. The maximal receptor binding sites for TGF-beta 1 in HOCM were greater (P < 0.05) than those for SA and TM. Immunohistochemistry demonstrated that IGF-I and TGF-beta 1 receptors were located on the cardiomyocytes and TGF-beta 1 receptors were located on the fibroblasts. CONCLUSIONS: Increased IGF-I and TGF-beta 1 gene expression previously observed in HOCM myocardium results in elevated protein levels. IGF-I and TGF-beta 1 signals may be further amplified by increased receptor numbers on cardiomyocytes and fibroblasts. The data suggest a possible autocrine mechanism of IGF-I-stimulated cardiomyocyte hypertrophy and a paracrine mechanism of TGF-beta 1-stimulated extracellular matrix overproduction in HOCM.

Angina Pectoris↗
PubMed1998

Total cavopulmonary anastomosis (Fontan) in children with Down's syndrome.

BACKGROUND: There is a paucity of information to guide the management of the child with Down's syndrome and congenital heart disease in whom biventricular repair is precluded. METHODS: Through the cardiology and cardiovascular surgery databases of The Hospital for Sick Children and Toronto Congenital Cardiac Centre for Adults, we identified patients with trisomy 21 and ventricular hypoplasia who had undergone a Fontan procedure (or modification). RESULTS: Of 533 patients who had undergone a Fontan operation between 1976 and 1997, 4 had trisomy 21. All 4 patients had unbalanced complete atrioventricular septal defect with right ventricular hypoplasia in 3 and left ventricular hypoplasia in 1. Three patients survived, and 1 died of endocarditis. The 3 survivors have done well in the short term and medium term without complications related to the pulmonary vasculature. CONCLUSIONS: We suggest that in appropriately selected patients with trisomy 21 and ventricular hypoplasia who are unsuitable for two or one and a half ventricle repair, the Fontan procedure is not contraindicated and provides short-term and medium-term benefit.

Down Syndrome↗
PubMed1998

A practical approach to 1 1/2 ventricle repairs.

BACKGROUND: Perioperative and long-term problems associated with the Fontan circulation are substantial. There has been an exploration of extending the limits of a biventricular ventricular repair by using a superior vena cava-to-pulmonary artery anastomosis. This type of repair is known as a 1 1/2 ventricle repair. METHODS: Patients having defects of the pulmonary ventricle in size or function have undergone 1 1/2 ventricle repairs with or without creation of an atrial septal defect. Repairs with tricuspid z values as small as -10 and predicted pulmonary ventricular volumes as low as 30% have been reported. The 1 1/2 ventricle repair technique has also been used in special situations associated with an arterial switch or double switch procedure. RESULTS: Mortality has ranged from 0% to 12%. Complications have included persistent elevation of superior vena cava pressure, intermittent periorbital edema, and 1 superior vena caval aneurysm requiring takedown. There appears to be an increased risk of perioperative pleural effusions and chylothorax. Protein-losing enteropathy and chronic atrial arrhythmias have not been present. CONCLUSIONS: Successful 1 1/2 ventricle repairs have been reported for morphologically small or poorly functioning pulmonary ventricles and special situations. Intermediate-term follow-up is favorable when compared with reported outcomes for the Fontan circulation.

Anastomosis, Surgical↗
PubMed1998

Primum atrial septal defect in children: early results, risk factors, and freedom from reoperation.

BACKGROUND: Repair of primum atrial septal defect in children usually is associated with a low operative mortality, except for a subgroup of children with congestive heart failure. To determine the early mortality and incidence of reoperation in children with primum atrial septal defect, we analyzed retrospectively the results of patients who underwent repair of this defect. METHODS: Between July 1982 and December 1996, 180 children underwent repair of primum atrial septal defect. The mean age at repair was 4.6 years (median, 3.6 years; range, 1 month to 16.4 years); of the 180 children, 23 were infants less than 1 year of age. Absent or mild symptoms were present in 145 (80%), whereas 34 (20%) of children presented with severe symptoms or congestive heart failure. RESULTS: Early mortality occurred in 3 (1.6%); 2 were less than 1 year of age. Follow-up ranged from 2 months to 14.5 years (mean, 6 +/- 4.2 years). Actuarial survival is 98% at 10 years with no late deaths. Age less than 1 year is a predictor of death. During follow-up, 17 (9%) of the 180 patients underwent reoperation, 5 of whom were in the infant group. Five underwent reoperation for subaortic obstruction, and 12 for left atrioventricular valve regurgitation of whom 11 were repaired; and 1 required valve replacement. Age and preoperative moderate-to-severe left atrioventricular valve regurgitation were predictors of reoperation. CONCLUSIONS: Results of the repair of primum atrial septal defect during childhood are favorable. Infants have a higher risk for death and reoperation. Left atrioventricular valve insufficiency and subaortic stenosis are important late complications and can be repaired safely at reoperation.

Adolescent↗
PubMed1998

Surgical management of total anomalous pulmonary venous drainage: impact of coexisting cardiac anomalies.

BACKGROUND: Recent reports have cited improving results for surgical management of isolated total anomalous pulmonary venous drainage. Complex cases (with other cardiac anomalies) are less frequently reported and are associated with higher mortality. METHODS: Retrospective review identified 170 consecutive patients treated for total anomalous pulmonary venous drainage from 1982 to 1996: 44 cases were "complex" (with significant associated cardiac lesions) and 126 cases were "simple." RESULTS: Operative mortality for simple cases decreased from 26% to 8%, and mortality for complex cases remained constant at 52%. Age, size, and the presence of atrial isomerism were univariate predictors of mortality. Multivariable analysis identified only univentricular hearts and associated cardiac lesions as predictors of operative mortality. Pulmonary artery (n = 16) and arteriopulmonary (n = 7) shunting strategies for complex cases resulted in less than 30% long-term survival. CONCLUSIONS: Despite improvement in survival for simple cases, management of total anomalous pulmonary venous drainage with single-ventricle hearts or other associated cardiac lesions remains problematic.

Adolescent↗
PubMed1998

Relentless pulmonary vein stenosis after repair of total anomalous pulmonary venous drainage.

BACKGROUND: Progressive stenosis of the pulmonary veins after repair of total anomalous pulmonary venous drainage is frequently refractory to surgical therapy. METHODS: Retrospective review of 170 consecutive patients treated for total anomalous pulmonary venous drainage identified 13 patients with postrepair pulmonary vein stenosis. Preoperative and operative data were analyzed to define the patterns of progression and efficacy of surgical techniques. RESULTS: Seventeen reoperations were performed in 13 patients. Postrepair pulmonary vein stenosis was most common in the infracardiac and mixed subtypes (p < 0.02). All 4 patients with unilateral stenosis, 2 of whom had progression of stenosis resulting in nearly complete unilateral pulmonary vein occlusion, survived. Six of 9 patients with bilateral disease died (p < 0.05 versus unilateral); 2 of the 3 survivors were repaired with a novel technique creating a sutureless neoatrium without evidence of restenosis at 1.8 years' follow-up. Stenting was uniformly unsuccessful. CONCLUSIONS: In unilateral stenosis, progression of disease may be survivable with loss of single-lung perfusion. Although bilateral disease is lethal in most cases, creation of a sutureless neoatrium has demonstrated short-term freedom from disease progression.

Constriction, Pathologic↗
PubMed1998

Primary repair is superior to initial palliation in children with atrioventricular septal defect and tetralogy of Fallot.

OBJECTIVE: The objective was to explore the best management algorithm for atrioventricular septal defect in conjunction with tetralogy of Fallot. METHODS: We reviewed the cases of 38 children referred to our division (March 1981-August 1997) who had atrioventricular septal defect associated with tetralogy of Fallot; 32 (84%) had Down syndrome. Twenty-one received initial palliation with a systemic-to-pulmonary artery shunt; of these, 2 (9.5%) died before complete repair. Thirty-one children underwent complete repair; 14 of these (45%) had undergone initial palliation (mean age at shunt 20 +/- 24 months). Right ventricular outflow obstruction was relieved by a transannular patch in 22 (71%); 14 (64% of 22) had a monocuspid valve inserted. Four required an infundibular patch. RESULTS: Two children (6.4%) died early after repair; 1 had undergone previous palliation. Patients with palliation underwent repair at an older age (78 vs 36 months), required longer ventilatory support (8 vs 4 days) and inotropic support (8 vs 4 days), and had longer intensive care stays (11 vs 6 days) and hospital stays (24 vs 15 days). Eleven children (35%) underwent reoperation, 7 (58%) for right ventricular outflow reconstruction and pulmonary arterioplasty. Reoperation was more frequent in the palliation group than in the primary operation group (64% vs 12%). The single late death was related to a reoperation in the palliation group. CONCLUSIONS: Atrioventricular septal defect with tetralogy of Fallot can be repaired with a low mortality rate. Initial palliation with a shunt resulted in a more complex postoperative course and a higher reoperative rate. Primary repair is superior to initial palliation with later repair.

Cardiac Surgical Procedures↗
PubMed1998

Fate of the neopulmonary valve after the arterial switch operation in neonates.

OBJECTIVES: The purpose of this study was to determine the incidence, risk factors, and outcomes of acquired stenosis of the neopulmonary valve after the neonatal arterial switch operation. METHODS: Reviewed were the preoperative and follow-up echocardiograms from 136 of 288 patients undergoing the arterial switch operation for whom adequate studies were available. Pulmonary stenosis was defined as a thickened and doming pulmonary valve and a pressure gradient of 20 mm Hg or more. Transposition of the great arteries was present with intact ventricular septum in 91 patients, with a ventricular septal detect in 39, with an aortic coarctation in 5, and with double-outlet right ventricle in 1 patient. No patient had preoperative valvular abnormalities (i.e., a bicuspid valve). RESULTS: During a median follow-up of 18 months (range <1 to 90 months), 32 patients (24%) had the development of supravalvular pulmonary stenosis, 15 (11%) with associated pulmonary valve stenosis (group I). Kaplan-Meier estimates of freedom from any intervention were 94% (95% confidence interval, 90% to 99%) at 1 year and 79% (95% confidence interval, 64% to 94%) at 5 years. The valve anulus before the arterial switch operation was significantly larger (p < 0.03) in those in whom neopulmonary valve stenosis did not develop (group II) than it was in those in whom it did (group I). At follow-up, the pulmonary valve anulus had decreased significantly in diameter in group I (p < 0.0005) and had remained larger in group II (p = 0.06) compared with normal diameter. Group I patients had the development of significant pulmonary valve hypoplasia (p < 0.03) whereas group II patients continued to have significantly larger valves compared with normal size (p < 0.0001). CONCLUSIONS: Neopulmonary valve stenosis after the arterial switch operation is not uncommon and is associated with growth failure of the valve anulus often associated with supravalvular pulmonary stenosis.

Female↗
PubMed1998

Management and outcomes of right atrial isomerism: a 26-year experience.

OBJECTIVES: We sought to determine, in a large series of patients with right atrial isomerism, factors associated with mortality. BACKGROUND: Right atrial isomerism is associated with complex congenital heart disease and high morbidity and mortality. METHOD: All data from patients diagnosed with right atrial isomerism between January 1970 and March 1996 were reviewed. RESULTS: A total of 91 consecutive patients (54 male) were identified. Most patients (89%) presented within the first month of life, 62% at birth. Cardiac abnormalities included common atrioventricular (AV) valve (81%), ventricular hypoplasia or single ventricle (73%), abnormal ventriculoarterial connections (96%), pulmonary outflow tract obstruction (84%), anomalous pulmonary venous drainage (87%) and pulmonary vein obstruction (30%). The overall mortality rate was 69%. No interventions were planned or performed in 24%, 95% of whom died. The mortality rate for patients requiring their first cardiovascular operation in the neonatal period was 75% versus 51% for those with later first operations (p < 0.05). The surgical mortality rate for patients undergoing pulmonary vein repair was 95%. Overall survival estimates were 71% at 1 month, 49% at 1 year and 35% at 5 years. Independent risk factors for decreased time to death included the absence of pulmonary outflow obstruction (relative risk [RR] 2.23, p < 0.03), presence of major AV valve anomaly (RR 5.23, p < 0.03) and obstructed pulmonary veins (RR 5.43, p < 0.0001). CONCLUSIONS: Right atrial isomerism continues to have an associated high mortality despite surgical innovations. Management of pulmonary vein obstruction remains a serious problem and is associated with high mortality.

Female↗
PubMed1998

Systemic venous collateral development after the bidirectional cavopulmonary anastomosis. Prevalence and predictors.

OBJECTIVES: To determine the prevalence of systemic venous collaterals after the bidirectional cavopulmonary anastomosis and the factors associated with their development. BACKGROUND: Systemic venous collaterals have been found after cavopulmonary anastomosis. Methods. Cardiac catheterization was performed in 103 patients before and after a bidirectional cavopulmonary anastomosis. RESULTS: After surgery, 51 venous collaterals were identified in 32 patients (31%). Collateral development was associated with an abnormal superior vena caval connection (56% incidence vs. 26% with a single right superior vena cava, p = 0.01) and postoperative factors including pulmonary artery distortion (53% incidence vs. 22% without distortion, p = 0.002); increased superior vena caval mean pressure (14 +/- 5 mm Hg versus 11 +/- 4 mm Hg with no collaterals, p = 0.0002); increased pulmonary artery mean pressure (13 +/- 4 mm Hg vs. 11 +/- 4 mm Hg with no collaterals, p = 0.02); lower right atrial mean pressure (5 +/- 2 mm Hg vs. 6 +/- 3 mm Hg with no collaterals, p = 0.04); and increased mean gradient between superior vena cava and right atrium (8 +/- 3 mm Hg vs. 5 +/- 4 mm Hg with no collaterals, p = 0.0002). Using multiple logistic regression, only this last factor was independently associated with collateral development with an odds ratio per 1 mm Hg of 1.33 (95% CI 1.12-1.58, p = 0.001) for their presence. CONCLUSIONS: Systemic venous collaterals occur frequently after a bidirectional cavopulmonary anastomosis and are found postoperatively when a significant pressure gradient occurs between cava and right atrium.

Adolescent↗
PubMed1998

Mortality in potential arterial switch candidates with transposition of the great arteries.

OBJECTIVES: We reviewed the factors contributing to or causing death before surgery in neonates with d-transposition of the great arteries (TGA) despite anatomy suitable for the arterial switch operation (ASO) to develop strategies to minimize preoperative attrition. BACKGROUND: Currently the ASO for neonates with TGA carries a low operative mortality. However, there is a paucity of information regarding the patients who die before the ASO. Strategies to ensure survival to operation are of importance to improve overall outcome. METHODS: We reviewed all neonates with TGA and patent forearm ovale (PFO) < or = 2 mm, a birthweight <2 kg, or who died before surgery, between 1988 and 1996. RESULTS: We identified 12 out of 295 neonates with TGA (4.1%) with anatomy suitable for the ASO who died prior to surgery. All had TGA/intact ventricular septum (IVS) and presented with a severely restrictive PFO. In 11 of 12 cases the cause of death was attributed to the sequelae of profound hypoxemia from inadequate mixing. Contributing factors were prematurity, 41.7%; severe respiratory distress syndrome, 25%; and persistent pulmonary hypertension of the newborn (PPHN), 16.7%. All patients received prostaglandin E1 (PGE1) infusion. Urgent balloon atrial sepstostomy (BAS) was performed in 66.7% with improved oxygenation. No cases were diagnosed prenatally. In contrast, all patients with a PFO < or = 2 mm who survived to ASO had a significantly better response to PGE1 infusion (p=0.03) than nonsurvivors. The ASO was accomplished without mortality in four of nine with a weight <2 kg. CONCLUSIONS: Of those neonates admitted with TGA, 4.1% died before surgery. Eleven of 12 (3.7%) died due to consequences of inadequate interatrial mixing despite PGE1 infusion. Earlier diagnosis and BAS are critically important in determining survival. Early ASO may improve survival in patients weighing <2 kg. Prenatal diagnosis with delivery in a high-risk obstetrical unit with facilities for immediate BAS and supportive therapy for pulmonary hypertension and ventricular failure may be necessary to salvage this group of patients.

Cause of Death↗
PubMed1998

Anomalous origin of one pulmonary artery from the ascending aorta: 36 years' experience from one centre.

UNLABELLED: Anomalous origin of one pulmonary artery from the ascending aorta is an uncommon lesion with an uncertain outcome. We reviewed 16 consecutive children (9 males) presenting with this lesion over a 36 year period at a single institution. Median age at presentation was 2 days (range, birth to 3.2 years). The anomalous pulmonary was the right in 12 and the left in 4, all originating from the proximal ascending aorta, with no patient having stenosis at the origin of the anomalous pulmonary artery. Associated cardiac anomalies were noted in 9 patients. No intervention was attempted in 2 patients: one was deemed inoperable due to complex associated lesions and pulmonary vascular obstructive disease, while the other one died before repair. Surgical intervention was attempted in 14 patients, with 3 intraoperative deaths (21%). Of 11 operative survivors, 8 developed pulmonary arterial stenosis graded severe in 2, moderate in 1 and mild in 5. Patients with severe stenosis required surgical angioplasty, 1 after unsuccessful dilation combined with placement of an endovascular stent. One patient with moderate, and one with mild, stenosis underwent successful transcatheter balloon dilation. The remaining 4 patients with mild stenosis remain unchanged during follow-up. One patient had biopsy evidence of pulmonary vascular obstructive disease at 3.3 years of age. There were no late deaths, giving a total mortality of 25% (4/16). CONCLUSION: While early diagnosis and repair of anomalous origin of one pulmonary artery from the ascending aorta is necessary, restenosis of the site of repair is common.

Aorta↗
PubMed1998

Transzygomatic coronoidectomy through an extended coronal incision for treatment of trismus due to an osteochondroma of the coronoid process of the mandible.

Osteochondroma is the most common benign tumor of the skeletal system. In the craniofacial skeleton, however, osteochondroma is uncommon. Even more rare is osteochondroma of the coronoid process. A review of the literature reveals only 31 reported cases of osteochondroma of the coronoid process of the mandible. The preponderance of patients were young men (67.7%) and most presented with a facial deformity. As a benign tumor, the problems with surgical treatment have dealt with the inaccessibility of the lesion and hence the surgical approach of choice. The previous surgical approaches have either been intraoral, external, or a combination of both. We present a transzygomatic approach via a coronal incision that gives excellent access and a good cosmetic result, and protects the facial nerve from damage.

Bone Plates↗
PubMed1998

The Le Fort I-palatal split approach for skull base tumors: efficacy, complications, and outcome.

The Le Fort I, split-palate approach provides intraoral surgical access to a region of the midline skull base ranging from the upper clivus to the second cervical vertebra. Although this approach provides perhaps the largest exposure of all the intraoral techniques, there is little concerning it in the literature. Furthermore, there are no detailed descriptions of case histories, complications, and outcome. The purpose of this study was to evaluate this procedure's effectiveness and identify associated complications as well as outcome. Seven cases of patients who underwent eight skull base surgeries using the Le Fort I, split-palate approach were evaluated retrospectively. Particular attention was paid to postoperative occlusion, speech, mouth opening, infection, tumor recurrence, postoperative recovery period, and viability of maxillary bone and teeth. Follow-up ranged from 4 months to 7 years with a mean of 3.9 years. Pathologic diagnoses included three chordomas (two recurrent), one recurrent meningioma, one liposarcoma, one chondrosarcoma, and one inflammatory mass. One patient with chordoma underwent a second operation using the same approach. No deaths or major neurologic problems related to the procedure occurred. One patient who had known local metastases at the time of operation died several months after surgery. All other patients are still living. Duration of hospital stay ranged from 5 to 53 days with a mean of 25.4 days. Postoperative complications included one case of meningitis with an associated cerebrospinal fluid leak, three cases of malocclusion, one case of velopharyngeal insufficiency, and one extracranial soft-tissue infection. The case of meningitis was successfully treated by antibiotics. The malocclusions were corrected by conservative treatment. No problems with mouth opening or bone or tooth viability occurred. Tumor recurred in both cases in which malignancy was involved, whereas only one recurrence was noted among the benign cases. It is concluded that the Le Fort I-palatal split technique is a relatively safe and effective means for approaching midline skull base tumors. Several modifications to the surgical protocol and surgical technique are detailed herein.

Adult↗
PubMed1998

Myocardial aerobic metabolism is impaired in a cell culture model of cyanotic heart disease.

A human pediatric cardiomyocyte cell culture model of chronic cyanosis was used to assess the effects of low oxygen tension on mitochondrial enzyme activity to address the postoperative increase in lactate and decreased ATP in the myocardium and the high incidence of low-output failure with restoration of normal oxygen tension, after technically successful corrective cardiac surgery. Chronically hypoxic cells (PO2 = 40 mmHg for 7 days) exhibited significantly reduced activities for pyruvate dehydrogenase, cytochrome-c oxidase, succinate cytochrome c reductase, succinate dehydrogenase, and citrate synthase. The activity of NADH-cytochrome c reductase was unaffected. Lactate production and the lactate-to-pyruvate ratio were significantly greater in hypoxic cardiomyocytes. Western and Northern analysis demonstrated a decrease in the levels of various mRNA and corresponding polypeptides in hypoxic cells. Thus hypoxia influences mitochondrial metabolism through acute and chronic adaptive mechanisms, reflecting allosteric (posttranscriptional) and transcriptional modulation. Transcriptional downregulation of key mitochondrial enzyme systems can explain the insufficient myocardial aerobic metabolism and low-output failure in children with cyanotic heart disease after cardiac surgery.

Adenosine Triphosphate↗
PubMed1997

Complete atrioventricular septal defects: results of repair, risk factors, and freedom from reoperation.

BACKGROUND: Several perioperative risk factors influence outcomes after repair of complete atrioventricular septal defects. This study was conducted to determine their association with perioperative mortality and need for reoperation. METHOD AND RESULTS: Between July 1982 and February 1995, 363 children underwent defect repair (median age, 8.3 months; mean, 17.4+/-1.3). Tetralogy of Fallot was present in 21 patients, double-outlet right ventricle in 4, subaortic stenosis in 8, ventricular hypoplasia in 8, coarctation in 5, atrial isomerism in 2, and other congenital anomalies in 9. Down's syndrome was present in 235 (65%). One-patch technique was applied in 99, two-patch in 243. During repair, the anterior bridging leaflet was divided in 12, the posterior bridging leaflet in 31, both leaflets in 71, and neither in 249. Left atrioventricular valve (LAVV) cleft was closed partially in 181 and completely in 112. Early mortality was 10.5%; 10-year survival, 83% (95% confidence interval, 0.79 to 0.87). At 10 years, freedom from reoperation for LAVV repair was 86%; LAVV replacement, 90%; subaortic stenosis, 95%; residual ventricular septal defect, 97%; permanent pacemaker insertion, 98%; and other types of reoperation, 95%. At the time of operation, greater age, shorter ischemic time, absence of a double-orifice LAVV, and cleft closure were found to be significant independent predictors of survival. CONCLUSIONS: Repair of atrioventricular septal defects has acceptable early and late mortality and a low incidence of reoperation. Double-orifice LAVV remains a risk factor. Repairs that include complete cleft closure may confer better survival.

Follow-Up Studies↗