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Anthony Azakie

Publications and source records attributed to Anthony Azakie.

At least 19 recordsLinked to original sources
PubMed2007

Fibroblast growth factor-2 expression is altered in lambs with increased pulmonary blood flow and pulmonary hypertension.

A lamb model of pulmonary hypertension, developed by inserting an aortopulmonary vascular graft (shunt), displays vascular remodeling and increased pulmonary blood flow characteristic of children with congenital heart disease. The purpose of this study was to determine whether expression of fibroblast growth factor-2 (FGF-2), a smooth muscle cell mitogen, is altered in shunt lambs. FGF-2 mRNA and protein levels were increased in lung tissue extracts from shunt lambs at 4 wk of age relative to age-matched controls (p < 0.05). FGF-2 protein levels were also increased in the pulmonary arteries and serum of shunt lambs (p < 0.05). Pulmonary arterial smooth muscle cells (PASMC) and endothelial cells (PAEC) were isolated from 4 wk-old lambs and subjected to cyclic stretch and laminar shear stress to mimic increased pulmonary blood flow. Stretch and shear increased FGF-2 promoter activity, and intracellular and extracellular FGF-2 protein levels in both cell types (p < 0.05). Exogenous FGF-2 stimulated PASMC proliferation at levels detected in the extracellular medium of sheared cells (p < 0.05). Elevated FGF-2 signaling by PASMC and PAEC exposed to increased pulmonary blood flow may play a role in the pulmonary vascular remodeling associated with the shunt model of pulmonary hypertension secondary to congenital heart disease.

Animals↗
PubMed2006

Myocardial transcription factors are modulated during pathologic cardiac hypertrophy in vivo.

OBJECTIVES: In the current study we describe and characterize a novel ovine model of biventricular hypertrophy and heart failure and evaluate the role of selected cardiac transcription factors in the regulation of cardiac gene expression during pathologic hypertrophy in vivo. The cardiac troponin T promoter is used as a model gene. METHODS AND RESULTS: Transient transfections of ovine cardiomyocytes in culture show that Sp1, transcriptional enhancer factor-1, and myocyte enhancer factor-2 activate cardiac troponin T promoter constructs. Cotransfection of Sp3 inhibits cardiac troponin T promoter activity and represses Sp1-mediated activation of the cardiac troponin T promoter. By chromatin immunoprecipitation, transcriptional enhancer factor-1, myocyte enhancer factor-2, NKX2.5, GATA-4, and Sp factors bind the cardiac troponin T promoter in vivo. To assess the role of cardiac transcription during pathologic hypertrophy, in vivo, we created surgical aorta-pulmonary shunts in utero in fetal lambs. Two weeks after spontaneous delivery, shunted lambs showed failure to thrive, significant biventricular hypertrophy, and heart failure. Shunted hearts had significant increases in myosin and cardiac troponin T protein expression. There was a shift in expression to the high-molecular-weight fetal isoforms. Transcriptional enhancer factor-1, myocyte enhancer factor-2, GATA-4, NKX2.5, and Sp1 transcription factor levels were increased in all heart chambers of shunted animals. Sp3 expression was decreased in shunted ventricles. Immunoprecipitated Sp3 was associated with significant increases in histone acetyl transferase activity and decreases in histone-deacetylase activity. CONCLUSION: The shunted neonatal lamb is a valid, novel model of pathologic biventricular hypertrophy. During pathologic hypertrophy myocardial transactivators are upregulated while repressors are downregulated.

Animals↗
PubMed2006

Undiagnosed coronary fistula causing low cardiac output syndrome after pediatric heart surgery.

A patient with preoperative pulmonary hypertension and a large left to right intracardiac shunt underwent surgical correction of a complex cardiac anomaly and required extracorporeal life support for unexplained ventricular dysfunction following the procedure. Following recovery, a fistulous connection between the right coronary artery and main pulmonary artery was demonstrated. Implications and management strategies of unsuspected coronary fistula in pediatric heart surgery are discussed.

Arterio-Arterial Fistula↗
PubMed2006

Sp3 inhibits Sp1-mediated activation of the cardiac troponin T promoter and is downregulated during pathological cardiac hypertrophy in vivo.

Combinatorial interactions between cis elements and trans-acting factors are required for regulation of cardiac gene expression during normal cardiac development and pathological cardiac hypertrophy. Sp factors bind GC boxes and are implicated in recruitment and assembly of the basal transcriptional complex. In this study, we show that the cardiac troponin T (cTnT) promoter contains a GC box that is necessary for basal and cAMP-mediated activity of cTnT promoter constructs transfected in embryonic cardiomyocytes. Cardiac nuclear proteins bind the cTnT GC box in a sequence-specific fashion and consist of Sp1, Sp2, and Sp3 protein factors. By chromatin immunoprecipitation, Sp1 binds the cTnT promoter "in vivo." Cotransfected Sp1 trans-activates the cTnT promoter in cardiomyocytes in culture. Sp3 represses Sp1-mediated transcriptional activation of the cTnT gene in embryonic cardiomyocytes. Sp3 repression of Sp1-mediated cTnT promoter activation is dose dependent, inferring a mechanism of competitive binding/inhibition. To evaluate the role of Sp factors in cardiac gene expression in vivo, we have established a clinically relevant animal model of pathological cardiac hypertrophy where the fetal cardiac program is activated. In this animal model, cardiac hypertrophy results from increased left-right shunting, volume loading of the left ventricle, and pressure loading of the right ventricle. Sp1 expression is increased in all four hypertrophied cardiac chambers, whereas Sp3 expression is diminished. This observation is consistent with the in vitro activating function of Sp1 and inhibitory effects of Sp3 on activity of cTnT promoter constructs. Sp factor levels are modulated during the hypertrophic cardiac program in vivo.

Animals↗
PubMed2006

The Lecompte maneuver for relief of airway compression in absent pulmonary valve syndrome.

BACKGROUND: Congenital absence of the pulmonary valve syndrome (APVS) is a rare cardiac defect characterized by aneurysmal pulmonary arteries, hypoplastic pulmonary valve cusps, and tracheobronchial abnormalities. Absence of the pulmonary valve syndrome usually occurs in conjunction with ventricular septal defect (VSD) and right ventricular outflow tract obstruction (RVOTO). Surgical mortality rates as high as 16% to 56% have been reported. Here, we describe the surgical results using the Lecompte maneuver, reduction pulmonary arterioplasty, and a valved right ventricle to pulmonary artery (RV-PA) conduit. METHODS: Medical records and operative and echocardiography reports for all surgical APVS cases were retrospectively examined for pertinent clinical variables. A patient with left bronchial compression due to enlarged pulmonary arteries associated with totally anomalous pulmonary venous drainage (TAPVD) is included to illustrate the value of the Lecompte maneuver. RESULTS: From January 2002 to December 2004, 4 children with APVS had surgery at a median age of 5 months (range, 3 months to 3.5 years). Three had malalignment VSD and RVOTO. Four had respiratory signs (cough, wheeze, tachypnea, oxygen dependence, ventilator dependence), and all 5 had evidence of tracheobronchial compression by computed tomography or magnetic resonance imaging. Repair included a Lecompte maneuver, a valved conduit with reduction pulmonary arterioplasty, and VSD closure as necessary. The TAPVD patient had repair of the anomalous veins and a Lecompte maneuver. There have been no deaths, and all patients were discharged in good condition. Follow-up is complete at a median of 24 months (range, 9 to 35). Echocardiography has shown no progressive enlargement of the pulmonary arteries. CONCLUSIONS: Use of the Lecompte maneuver, reduction arterioplasty, and a valved conduit for repair of APVS provides favorable early and midterm results.

Anastomosis, Surgical↗
PubMed2006

Invited commentary.

Explore the source record for details and available documents.

Child, Preschool↗
PubMed2006

Alterations in plasma B-type natriuretic peptide levels after repair of congenital heart defects: a potential perioperative marker.

OBJECTIVES: B-type natriuretic peptide, a cardiac hormone with diuretic, natriuretic, and vasoactive properties, is used in the diagnosis, risk stratification, and management of adult cardiac patients. However, no study has yet determined the prognostic value of B-type natriuretic peptide after surgical intervention for congenital heart disease. The objectives of this study were (1) to determine alterations in B-type natriuretic peptide levels after repair of congenital heart disease with cardiopulmonary bypass and (2) to investigate potential associations between B-type natriuretic peptide levels and outcomes in this patient population. METHODS: Fifty-one infants and children undergoing repair of congenital heart disease were studied. B-type natriuretic peptide levels were measured before and after surgical intervention, and the ability of the postoperative 12-hour B-type natriuretic peptide level to predict postoperative outcomes was evaluated. RESULTS: B-type natriuretic peptide levels increased after separation from cardiopulmonary bypass, with an 8-fold peak increase at 12 hours (P < .005). Postoperative 12-hour B-type natriuretic peptide levels were associated with the duration of mechanical ventilation and the presence of a low cardiac output state after surgical intervention. On multivariate analysis, the 12-hour B-type natriuretic peptide level was an independent predictor of the duration of mechanical ventilation. In fact, B-type natriuretic peptide levels of greater than 540 pg/mL predicted mechanical ventilation beyond 48 hours, with a sensitivity of 88.9% and a specificity of 82.5%. In addition, B-type natriuretic peptide levels of greater than 815 pg/mL predicted the presence of a low cardiac output state within 48 hours after surgical intervention, with a sensitivity of 87.5% and a specificity of 90.2%. CONCLUSIONS: B-type natriuretic peptide determinations might be a useful tool for clinicians caring for infants and children after surgical intervention for congenital heart disease.

Adolescent↗
PubMed2006

Increased oxidative stress in lambs with increased pulmonary blood flow and pulmonary hypertension: role of NADPH oxidase and endothelial NO synthase.

Although oxidative stress is known to contribute to endothelial dysfunction-associated systemic vascular disorders, its role in pulmonary vascular disorders is less clear. Our previous studies, using isolated pulmonary arteries taken from lambs with surgically created heart defect and increased pulmonary blood flow (Shunt), have suggested a role for reactive oxygen species (ROS) in the endothelial dysfunction of pulmonary hypertension, but in vivo data are lacking. Thus the initial objective of this study was to determine whether Shunt lambs had elevated levels of ROS generation and whether this was associated with alterations in antioxidant capacity. Our results indicate that superoxide, but not hydrogen peroxide, levels were significantly elevated in Shunt lambs. In addition, we found that the increase in superoxide generation was not associated with alterations in antioxidant enzyme expression or activity. These data suggested that there is an increase in superoxide generation rather than a decrease in scavenging capacity in the lung. Thus we next examined the expression of various subunits of the NADPH oxidase complex as a potential source of the superoxide production. Results indicated that the expression of Rac1 and p47(phox) is increased in Shunt lambs. We also found that the NADPH oxidase inhibitor diphenyliodonium (DPI) significantly reduced dihydroethidium (DHE) oxidation in lung sections prepared from Shunt but not Control lambs. As DPI can also inhibit endothelial nitric oxide synthase (eNOS) superoxide generation, we repeated this experiment using a more specific NADPH oxidase inhibitor (apocynin) and an inhibitor of NOS (3-ethylisothiourea). Our results indicated that both inhibitors significantly reduced DHE oxidation in lung sections prepared from Shunt but not Control lambs. To further investigate the mechanism by which eNOS becomes uncoupled in Shunt lambs, we evaluated the levels of dihydrobiopterin (BH(2)) and tetrahydrobiopterin (BH(4)) in lung tissues of Shunt and Control lambs. Our data indicated that although BH(4) levels were unchanged, BH(2) levels were significantly increased. Finally, we demonstrated that the addition of BH(2) produced an increase in superoxide generation from purified, recombinant eNOS. In conclusion our data demonstrate that the development of pulmonary hypertension in Shunt lambs is associated with increases in oxidative stress that are not explained by decreases in antioxidant expression or activity. Rather, the observed increase in oxidative stress is due, at least in part, to increased expression and activity of the NADPH oxidase complex and uncoupled eNOS due to elevated levels of BH(2).

Animals↗
PubMed2005

Nitric oxide-endothelin-1 interactions after surgically induced acute increases in pulmonary blood flow in intact lambs.

Several congenital heart defects require surgery that acutely increases pulmonary blood flow (PBF). This can lead to dynamic alterations in postoperative pulmonary vascular resistance (PVR) and can contribute to morbidity and mortality. Thus the objective of this study was to determine the role of nitric oxide (NO), endothelin (ET)-1, and their interactions in the alterations of PVR after surgically induced increases in PBF. Twenty lambs underwent placement of an aortopulmonary vascular graft. Lambs were instrumented to measure vascular pressures and PBF and studied for 4 h. Before and after shunt opening, lambs received an infusion of saline (n = 9), tezosentan, an ETA- and ETB -receptor antagonist (n = 6), or Nomega-nitro-L-arginine (L-NNA), a NO synthase (NOS) inhibitor (n = 5). In control lambs, shunt opening increased PBF by 117.8% and decreased PVR by 40.7% (P < 0.05) by 15 min, without further changes thereafter. Plasma ET-1 levels increased 17.6% (P < 0.05), and total NOS activity decreased 61.1% (P < 0.05) at 4 h. ET-receptor blockade (tezosentan) prevented the plateau of PBF and PVR, such that PBF was increased and PVR was decreased compared with controls at 3 and 4 h (P < 0.05). These changes were associated with an increase in total NOS activity (+61.4%; P < 0.05) at 4 h. NOS inhibition (L-NNA) after shunt placement prevented the sustained decrease in PVR seen in control lambs. In these lambs, PVR decreased by 15 min (P < 0.05) but returned to baseline by 2 h. Together, these data suggest that surgically induced increases in PBF are limited by vasoconstriction, at least in part by an ET-receptor-mediated decrease in lung NOS activity. Thus NO appears to be important in maintaining a reduction in PVR after acutely increased PBF.

Animals↗
PubMed2005

Divergent transcriptional enhancer factor-1 regulates the cardiac troponin T promoter.

MCAT elements are essential for cardiac gene expression during development. Avian transcriptional enhancer factor-1 (TEF-1) proteins are muscle-enriched and contribute to MCAT binding activities. However, direct activation of MCAT-driven promoters by TEF-1-related proteins has not been uniformly achieved. Divergent TEF (DTEF)-1 is a unique member of the TEF-1 multigene family with abundant transcripts in the heart but not in skeletal muscle. Herein we show that DTEF-1 proteins are highly expressed in the heart. Protein expression is activated at very early stages of chick embryogenesis (Hamburger-Hamilton stage 4, 16-18 h), after which DTEF-1 becomes abundant in the sinus venosus and is expressed in the trabeculated ventricular myocardium and ventricular outflow tracts. By chromatin immunoprecipitation, DTEF-1 interacts with the cardiac troponin T (cTnT) promoter in vivo. DTEF-1 also interacts with MEF- 2 by coimmunoprecipitation and independently or cooperatively (with MEF-2) trans-activates the cTnT promoter. DTEF-1 isoforms do not activate the cTnT promoter in fibroblasts or skeletal muscle. DTEF-1 expression occurs very early in chick embryogenesis (16-18 h), preceding sarcomeric protein expression, and it activates cardiac promoters. As such, DTEF-1 may be an early marker of the myocardial phenotype. DTEF-1 trans-activates the cTnT promoter in a tissue-specific fashion independent of AT-rich, MEF-2, or GATA sites. The observed spatial pattern suggests decreasing levels of expression from the cardiac inlet to the ventricular outflow tracts, which may mark a cardiogenic or differentiation pathway that parallels the direction of flow through the developing chick heart.

Amino Acid Motifs↗
PubMed2005

Cerebral oxygen balance is impaired during repair of aortic coarctation in infants and children.

OBJECTIVE: During repair of aortic coarctation through a left thoracotomy without cardiopulmonary bypass, clamping the proximal transverse aortic arch occludes antegrade flow to the left carotid and vertebral arteries. It is assumed that flow through the right carotid and vertebral arteries is adequate for cerebral perfusion. The study objective is to determine whether aortic occlusion impairs left hemispheric cerebral oxygen balance measured by near-infrared spectroscopy. METHODS: In 18 children having repair of aortic coarctation, we measured the maximum change and integral for hemoglobin D (difference of oxyhemoglobin and deoxyhemoglobin), total oxygenation index, and the redox state of cytochrome aa3. Thirteen subjects had recordings from the left hemisphere to test the hypothesis that aortic occlusion impairs left hemispheric oxygen balance. Five subjects had recordings from the right hemisphere for comparison. RESULTS: After aortic clamping, a significant decrease in hemoglobin D was observed in recordings from the left hemisphere compared with those from the right hemisphere (P = .03, maximum change in hemoglobin D). Total oxygenation index and cytochrome aa3 were generally preserved. There was an inverse linear relationship for the change in hemoglobin D during clamp application and after removal (Spearman rho = -0.74), with increased hemoglobin D after clamp removal in those subjects with the greatest decrease of hemoglobin D during arch occlusion. Linear regression analysis identified nitroprusside administration as significantly associated with a decrease in hemoglobin D (P < .001). CONCLUSIONS: Significant impairment in left hemispheric cerebral oxygen balance was identified during arch clamping. The neurodevelopmental significance of impaired cerebral oxygen balance detected by near-infrared spectroscopy during aortic coarctation repair remains to be elucidated.

Aorta, Thoracic↗
PubMed2005

Late pulmonary valve implantation after repair of tetralogy of Fallot.

Ten cases of elective late pulmonary valve implantation after repair of tetralogy of Fallot were reviewed. The interval after initial repair ranged from 1.5 to 43 years (mean, 20.0 +/- 12.3 years). There was no hospital mortality or late death during a mean follow-up of 12.5 months. Preoperatively, 9 patients were in New York Heart Association functional class III-IV; after pulmonary valve implantation, all 10 patients were in class I-II (average improvement, 1.7 classes). Left ventricular ejection fraction improved significantly (from 62.1% +/- 4.7% to 70.2% +/- 4.9%), as did fractional shortening (from 34.0% +/- 5.0% to 40.0% +/- 4.2%). Right ventricular diameter decreased significantly (from 32.3 +/- 7.5 to 24.4 +/- 5.4 mm). QRS duration decreased significantly (155.2 +/- 27.1 vs. 140.0 +/- 21.2 msec), but there was no significant difference in QT interval (460.9 +/- 29.6 vs. 451.9 +/- 50.6 msec). Hospital stay was 4-7 days. One patient had preoperative ventricular fibrillation requiring resuscitation and an implantable cardiac defibrillator; another needed a defibrillator at the time of pulmonary valve implantation, because of ventricular arrhythmias. It was concluded that late pulmonary valve implantation after tetralogy of Fallot repair had significant benefits and carried low operative risk.

Adolescent↗
PubMed2004

Endothelial alterations during inhaled NO in lambs with pulmonary hypertension: implications for rebound hypertension.

Clinically significant increases in pulmonary vascular resistance (PVR) have been noted upon acute withdrawal of inhaled nitric oxide (iNO). Previous studies in the normal pulmonary circulation demonstrate that iNO increases endothelin-1 (ET-1) levels and decreases endogenous nitric oxide synthase (NOS) activity, implicating an endothelial etiology for the increase in resistance upon iNO withdrawal. However, the effect of iNO on endogenous endothelial function in the clinically relevant pulmonary hypertensive circulation is unknown. The objective of this study was to determine the effects of iNO on endogenous NO-cGMP and ET-1 signaling in lambs with preexisting pulmonary hypertension secondary to increased pulmonary blood flow. Eight fetal lambs underwent in utero placement of an aortopulmonary vascular graft (shunt lambs). After delivery (4 wk), the shunt lambs were mechanically ventilated with iNO (40 ppm) for 24 h. After 24 h of inhaled NO, plasma ET-1 levels increased by 34.8% independently of changes in protein levels (P < 0.05). Contrary to findings in normal lambs, total NOS activity did not decrease during iNO. In fact, Western blot analysis demonstrated that tissue endothelial NOS protein levels decreased by 43% such that NOS activity relative to protein levels actually increased during iNO (P < 0.05). In addition, the beta-subunit of soluble guanylate cyclase decreased by 70%, whereas phosphodiesterase 5 levels were unchanged (P < 0.05). Withdrawal of iNO was associated with an acute increase in PVR, which exceeded baseline PVR by 45%, and a decrease in cGMP concentrations to levels that were below baseline. These data suggest that the endothelial response to iNO and the potential mechanisms of rebound pulmonary hypertension are dependent upon the underlying pulmonary vasculature.

Administration, Inhalation↗
PubMed2004

Chronic endothelin A receptor blockade in lambs with increased pulmonary blood flow and pressure.

Endothelin receptor blockade is an emerging therapy for pulmonary hypertension. However, hemodynamic and structural effects and potential changes in endogenous nitric oxide (NO)-cGMP and endothelin-1 signaling of chronic endothelin A receptor blockade in pulmonary hypertension secondary to congenital heart disease are unknown. Therefore, the objectives of this study were to determine hemodynamic and structural effects and potential changes in endogenous NO-cGMP and endothelin-1 signaling of chronic endothelin A receptor blockade in a lamb model of increased pulmonary blood flow following in utero placement of an aortopulmonary shunt. Immediately after spontaneous birth, shunt lambs were treated lifelong with either an endothelin A receptor antagonist (PD-156707) or placebo. At 4 wk of age, PD-156707-treated shunt lambs (n = 6) had lower pulmonary vascular resistance and right atrial pressure than placebo-treated shunt lambs (n = 8, P < 0.05). Smooth muscle thickness or arterial number per unit area was not different between the two groups. However, the number of alveolar profiles per unit area was increased in the PD-156707-treated shunt lambs (190.7 +/- 5.6 vs. 132.9 +/- 10.0, P < 0.05). Plasma endothelin-1 and cGMP levels and lung NOS activity, cGMP, eNOS, preproendothelin-1, endothelin-converting enzyme-1, endothelin A, and endothelin B receptor protein levels were similar in both groups. We conclude that chronic endothelin A receptor blockade attenuates the progression of pulmonary hypertension and augments alveolar growth in lambs with increased pulmonary blood flow.

Animals↗
PubMed2004

Increased pulmonary blood flow does not alter surfactant protein gene expression in lambs within the first week of life.

Neonates and infants with congenital heart disease with increased pulmonary blood flow suffer morbidity from poor oxygenation and decreased lung compliance. In a previous experiment involving 4-wk-old lambs with pulmonary hypertension secondary to increased pulmonary blood flow following an in utero placement of an aortopulmonary vascular graft, we found a decrease in surfactant protein (SP)-A gene expression as well as a decrease in SP-A and SP-B protein contents. To determine the timing of these changes, the objective of the present study was to characterize the effect of increased pulmonary blood flow and pulmonary hypertension on SP-A, -B, and -C gene expressions and protein contents within the first week of life. Of eight fetal lambs that underwent the in utero placement of the shunt, there was no difference in the expression of SP-A, -B, and -C mRNA levels or SP-A and -B protein contents compared with age-matched controls. The results showed that, in this model of congenital heart disease with pulmonary hypertension and increased pulmonary blood flow, the effect of the shunt on SP gene expression and protein content was not apparent within the first week of life.

Age Factors↗
PubMed2004

The Triiodothyronine for Infants and Children Undergoing Cardiopulmonary Bypass (TRICC) study: design and rationale.

BACKGROUND: Cardiopulmonary bypass induces marked and persistent depression of circulating thyroid hormones in infants and children, possibly contributing to postoperative morbidity. Clinical studies have evaluated parenteral triiodothyronine supplementation after cardiopulmonary bypass in children. However, these investigations had relatively small subject numbers as well as age and diagnosis heterogeneity, thereby limiting ability to determine clinical effect. A double-blind, randomized, placebo-controlled trial is needed to define clinical safety and efficacy of triiodothyronine supplementation in infants. METHODS AND RESULTS: The Triiodothyronine for Infants and Children Undergoing Cardiopulmonary Bypass (TRICC) study is a multicenter, randomized, clinical trial designed to determine safety and efficacy of triiodothyronine supplementation in children <2 years of age undergoing surgical procedures for congenital heart disease. Duration of mechanical ventilation after completion of cardiopulmonary bypass is the primary clinical outcome parameter with multiple secondary clinical and hemodynamic parameters. Nearly 200 patients will be randomly assigned to receive either triiodothyronine or placebo. Patient assignment will be performed using a stratified block randomization according to specific preoperative diagnosis. CONCLUSIONS: The TRICC study will provide important data regarding the efficacy and safety of triiodothyronine in this age-specific population undergoing surgery for congenital heart disease.

Cardiopulmonary Bypass↗
PubMed2004

Outcomes of tricuspid atresia in the Fontan era.

BACKGROUND: Whereas indications expand and results improve with increasing refinements to the Fontan procedure the overall impact on outcomes related to tricuspid atresia remains suboptimally defined. METHODS: We reviewed 225 consecutive patients presenting between 1971 and 1999. All patients had classic tricuspid atresia with absent right atrioventricular connection and with D-transposition of the great arteries in 21%, pulmonary outflow obstruction in 75%, and aortic outflow obstruction in 11%. RESULTS: Ten patients died before any intervention and 3 patients were lost to follow-up. Palliative procedures (includes 151 with systemic shunt, 27 pulmonary artery banding, 60 venous shunt) were performed in 203 patients, with 44 deaths, 8 patients awaiting Fontan, 12 patients Fontan contraindicated, and 11 patients lost-to-follow-up. A total of 137 patients had the Fontan procedure (9 patients without previous procedures) with 7 early deaths, 11 late deaths, and 3 patients progressing to heart transplantation. Total survival for the cohort was 90% at the age of 1 month, 81% at 1 year, 70% at 10 years, and 60% at 20 years with no significant change over the time period. Independent factors associated with ineligibility or death without Fontan (n = 68, 30%) included earlier birth date, lower birth weight, presence of aortic arch anomaly and greater right ventricular hypoplasia, and no palliative procedure. There were no significant changes in mortality with Fontan over the study time period with survival of 95% at 1 month, 93% at 1year, and 82% at 10 years. CONCLUSIONS: Improvements in outcomes with tricuspid atresia will require attention to management and risk factors before Fontan.

Adolescent↗