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ABSTRACT: BACKGROUND: Aortic valve disease in association with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals (PA/VSD/MAPCAs) is an extremely rare combination of congenital heart defects. The presence of aortic stenosis or insufficiency or both imposes an additional physiologic burden that complicates the management of PA/VSD/MAPCAs. This report summarizes our experience with 7 patients who underwent surgical repair of this rare combination of defects. METHODS: This was a retrospective review of patients who had both aortic valve disease and PA/VSD/MAPCAs. Four patients had predominantly aortic stenosis, 2 patients had aortic insufficiency, and 1 had mixed stenosis and insufficiency. Anatomically, this corresponded to a dysplastic trileaflet valve in 4, bicuspid valve in 2, and quadricuspid valve in 1. RESULTS: Four of the 7 patients underwent aortic valve surgery at the time of their first operation for PA/VSD/MAPCAs. The remaining 3 patients had aortic valve surgery at subsequent operations. All 7 patients are alive, with a median duration of follow-up of 3 years. One patient has required aortic valve replacement 2 years after repair of an insufficient bicuspid valve. CONCLUSIONS: This manuscript summarizes our experience with 7 patients who had the rare combination of aortic valve disease and PA/VSD/MAPCAs. Although aortic valve disease has been reported for other conotruncal defects, we believe this is the first report of its presence in association with PA/VSD/MAPCAs.
The Annals of thoracic surgery 05/2013; · 3.74 Impact Factor
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ABSTRACT: BACKGROUND: Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is a complex form of congenital heart defect. There are limited data regarding late hemodynamics of patients after repair of PA/VSD/MAPCAs. This study evaluated the hemodynamics of patients who underwent complete repair of PA/VSD/MAPCSs and subsequently returned for a conduit change. METHODS: This was a retrospective review of 80 children undergoing a right ventricle (RV)-to-pulmonary artery conduit replacement after complete repair of PA/VSD/MAPCAs. All patients underwent preoperative cardiac catheterization to define the cardiac physiology. Patients were an average age of 6.5 ± 1.2 years, and the average interval between complete repair and conduit change was 4.5 ± 1.1 years. RESULTS: The preoperative cardiac catheterization demonstrated an average RV right peak systolic pressure of 70 ± 22 mm Hg and pulmonary artery pressure of 38 ± 14 mm Hg. This pressure gradient of 32 mm Hg reflects the presence of conduit obstruction. After conduit change, the intraoperative RV systolic pressure was 34 ± 8 mm Hg, similar to 36 ± 9 mm Hg at the conclusion of the previous complete repair. The corresponding RV/aortic pressure ratios were 0.36 ± 0.07 and 0.39 ± 0.09, respectively. CONCLUSIONS: The data demonstrate that patients who underwent complete repair of PA/VSD/MAPCAs had nearly identical pulmonary artery pressures when they returned for conduit change some 4.5 years later. This finding indicates that the growth and development of the unifocalized pulmonary vascular bed is commensurate with visceral growth. We would hypothesize that complete repair, along with low RV pressures, will confer a long-term survival advantage.
The Annals of thoracic surgery 02/2013; · 3.74 Impact Factor
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ABSTRACT: BACKGROUND: Pulmonary stenosis with ventricular septal defect and major aortopulmonary collaterals (PS/VSD/MAPCAs) is an extremely rare form of congenital heart defect. Although it has been assumed that PS/VSD/MAPCAs would be similar to pulmonary atresia (PA) with VSD/MAPCA, there are currently no data to support this conjecture. This study reviewed our surgical experience with reconstruction of PS/VSD/MAPCA. METHODS: This was a retrospective review of 25 patients (14 boys, 11 girls) who were born with PS/VSD/MAPCA and underwent surgical reconstruction. Preoperative pulmonary angiography was used to define the central branch pulmonary arteries and MAPCA. Patients were a median age of 4 months at the first operation. RESULTS: There was one operative death (4%) in this cohort of 25 patients, and complete repair was achieved in the 24 survivors (96%). There were two distinct subgroups of patients: 11 demonstrated cyanosis in the neonatal timeframe and underwent an initial procedure to augment pulmonary blood flow (+PBF). The remaining 14 patients formed the second group (-PBF). The median age at the first operation was 0.8 months in the +PBF group and 5.2 months in the -PBF group (p < 0.005). Complete repair was achieved in 91% of patients in the +PBF group and in 100% in the -PBF group; however, the average number of procedures to achieve complete repair was 2.8 in the +PBF group vs 1.0 in the -PBF group (p < 0.005). CONCLUSIONS: Outcomes for PS/VSD/MAPCAs as a whole were excellent, with a low surgical mortality and high rate of complete repair. There were two identifiable subgroups with distinctive differences required in their surgical management. These results provide a prognostic outlook for patients with PS/VSD/MAPCAs that can be compared and contrasted with PA/VSD/MAPCAs.
The Annals of thoracic surgery 02/2013; · 3.74 Impact Factor
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ABSTRACT: OBJECTIVES: Peripheral pulmonary artery stenosis is a rare congenital heart defect frequently found in association with Williams and Alagille syndromes. Controversy exists regarding the optimal treatment of peripheral pulmonary artery stenosis, with most centers favoring catheter-based interventions. In contrast, we have preferentially used surgical reconstruction of peripheral pulmonary artery stenosis. The purpose of the present study was to review our experience with surgical reconstruction of peripheral pulmonary artery stenosis. METHODS: We performed a retrospective review of patients who underwent surgical reconstruction of peripheral pulmonary artery stenosis. A total of 16 patients were identified: 7 had Williams syndrome, 6 had Alagille syndrome, and 3 had no identifiable syndrome. Detailed pulmonary angiography was performed in all patients to define stenoses at the main, branch, lobar, and segmental arterial levels. The mean preoperative right ventricular/left ventricular pressure ratio was 0.88 ± 0.07. The surgical approach was a median sternotomy with cardiopulmonary bypass. All peripheral stenoses were augmented with pulmonary artery homograft tissue. The median age at surgery was 14 months, and concomitant procedures were performed in 9 of the 16 patients. RESULTS: There was 1 operative mortality (6%). The mean right ventricular/left ventricular pressure ratio decreased to 0.40 ± 0.04 postoperatively (P < .005), representing a 55% reduction compared with the preoperative values. The patients were followed up for a median of 5 years. No late mortality occurred and reoperation was not required. CONCLUSIONS: The data have demonstrate that this comprehensive surgical approach to the treatment of peripheral pulmonary artery stenosis was associated with low early and no late mortality. Surgical reconstruction of the peripheral pulmonary artery stenosis resulted in a significant decrease in right ventricular pressure. We hypothesize that this reduction in right ventricular pressures will confer a long-term survival advantage for this cohort of patients.
The Journal of thoracic and cardiovascular surgery 12/2012; · 3.41 Impact Factor
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ABSTRACT: Pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCAs) is a complex form of congenital heart defect. One identifiable subset has small (<2.5 mm) intrapericardial branch pulmonary arteries that are (1) confluent, (2) have normal arborization, and (3) have dual-supplied collateral vessels. When this anatomy is associated with limited pulmonary blood flow, the patients are candidates for creation of an aortopulmonary window to stimulate growth of the pulmonary arteries. The purpose of this study was to review our experience with creation of an aortopulmonary window as the initial palliative procedure.
This was a retrospective review of our surgical experience with 35 children undergoing aortopulmonary window creation from 2002 to 2011. Patients were identified by preoperative cardiac catheterization to define the cardiac and pulmonary artery anatomy.
There was no mortality in 35 patients undergoing aortopulmonary window creation. These patients have subsequently undergone 78 cardiac procedures (with 2 operative mortalities). Eighteen of these patients have achieved complete repair, 4 patients in a second procedure, 6 patients in a third procedure, 5 patients in a fourth procedure, and 3 patients in a fifth procedure.
The data demonstrate that patients can undergo creation of an aortopulmonary window with excellent early results. Few patients were amenable to complete repair at the second operation, and most required multiple reoperations to recruit sufficient arborization. We interpret these counterintuitive results to suggest that hypoplastic central pulmonary arteries and diminished pulmonary blood flow are markers for a less well developed pulmonary vascular bed.
The Annals of thoracic surgery 07/2012; 94(3):842-8. · 3.74 Impact Factor
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ABSTRACT: Pulmonary atresia with major aortopulmonary collateral arteries (PA/MAPCAs) is a complex congenital heart defect that has undergone significant advances in treatment over the past 15 years. A small subset of patients with PA/MAPCAs have associated Alagille syndrome, which can have an adverse impact on many other organ systems. The purpose of this study was to review our institutional outcomes for the surgical patients with PA/MAPCAs and Alagille syndrome.
This was a retrospective review of patients with PA/MAPCA's and Alagille who underwent surgical reconstruction from November 2001 to August 2011. Fifteen patients were identified in our data base. Thirteen had pulmonary atresia with ventricular septal defect (PA/VSD) and two had pulmonary atresia with intact ventricular septum (PA-IVS).
There has been no early or late mortality in this cohort of 15 patients with PA/MAPCA' and Alagille syndrome. The patients have undergone a total of 38 cardiac surgical procedures. Ten of the 13 patients with PA/VSD have achieved complete repair, including unifocalization, a right ventricle to pulmonary artery conduit and closure of all intra-cardiac shunts. The three unrepaired patients with PA/VSD remain potential candidates for eventual complete repair, while the two patients with PA-IVS remain viable candidates for a single ventricle pathway. The patients in this series have also undergone 12 major non-cardiac procedures.
The data demonstrate that surgical reconstruction of PA/MAPCAs can be successfully achieved in patients with Alagille syndrome. The longer-term prognosis remains guarded on the basis of the multi-organ system involvement of Alagille syndrome.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 03/2012; 42(2):235-40; discussion 240-1. · 2.40 Impact Factor
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ABSTRACT: Surgical reconstruction is the primary method of treating airway obstruction in children. Tracheal stenosis is frequently associated with congenital heart defects, which may further complicate the overall management strategy. The purpose of this study was to review our experience with surgical reconstruction of airway obstruction in conjunction with congenital heart defects.
This was a retrospective review of our surgical experience with tracheal stenosis from February 2003 to August 2011. Twenty-seven patients were identified in our database. Six patients had isolated, congenital tracheal stenosis, and 21 had tracheal stenosis in association with congenital heart defects. There were two identifiable subgroups. Thirteen patients had airway stenoses identified concurrently with congenital heart defects and underwent combined repair. The second group comprised 8 patients who had previous correction of their congenital heart defects and experienced delayed presentation of tracheal (n = 6) or bronchial (n = 2) obstruction.
The median age at surgery was 9 months. There were 2 postoperative deaths, both in children with single ventricle. The median duration of follow-up for the entire cohort of 25 surviving patients was 4 years. None of the patients have required reoperations on the trachea; 5 have had minor reinterventions.
The data demonstrate that tracheal obstruction is frequently found in conjunction with congenital heart defects. Nearly one third of our patients had delayed presentation of airway obstruction that was identified subsequent to previous congenital heart defect repair. Tracheal reconstructive techniques were effective regardless of the cause of the airway obstruction.
The Annals of thoracic surgery 02/2012; 93(4):1266-72; discussion 1272-3. · 3.74 Impact Factor
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ABSTRACT: Pulmonary regurgitation developing late after tetralogy of Fallot repair is now recognized as a serious threat to the long-term welfare of these patients. This article summarizes our experience with 5 patients who underwent reoperations for treatment of severe pulmonary regurgitation after transannular patch repair of tetralogy of Fallot. In each case, the intraoperative findings revealed anatomy favorable for valve repair and enabled preservation of the native pulmonary valves.
The Annals of thoracic surgery 02/2012; 93(2):677-9. · 3.74 Impact Factor
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ABSTRACT: Pulmonary atresia and major aortopulmonary collaterals (PA/MAPCAs) is a complex form of congenital heart disease. One to two percent of patients with PA/MAPCAs will also have total anomalous pulmonary venous connection (TAPVC). This study summarizes our surgical experience with this rare combination of life-threatening congenital heart defects.
A retrospective review was performed to identify patients who had surgery for PA/MAPCAs in association with TAPVC. From November 2001 to March 2011, 9 patients presented with this combination of defects. Eight of the 9 patients had heterotaxy with an unbalanced atrioventricular canal and functional single ventricle. The ninth patient had double outlet right ventricle (two ventricles). Timing of surgical intervention was typically predicated on the degree of pulmonary venous obstruction. The median age at surgery was 14 days. All nine patients had surgical correction of TAPVC, unifocalization of MAPCA's into a central confluence, and placement of a shunt.
There was one early mortality (< 30 days) and two late mortalities. For the 6 survivors, 5 have subsequently undergone a bidirectional Glenn procedure, and 3 had completion of their Fontan. Two patients are currently at the bidirectional Glenn stage; one is a good candidate for Fontan completion while the other is not suitable. The sixth patient is awaiting further assessment.
The PA/MAPCAs, in association with TAPVC, is a challenging combination of defects. The data suggest that the combination of PA/MAPCAs and TAPVC can be undertaken with a reasonable midterm prognosis.
The Annals of thoracic surgery 09/2011; 92(5):1756-60. · 3.74 Impact Factor
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ABSTRACT: Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart defect that has been associated with myocardial ischemia and sudden death. Controversies exist regarding the diagnosis, treatment, and long-term recommendations for patients with AAOCA. The purpose of this study is to evaluate the medium-term results of surgical repair for AAOCA.
From January 1999 through August 2010, 50 patients underwent surgical repair of AAOCA. The median age at surgery was 14 years (range, 5 days to 47 years). Thirty-one patients had the right coronary originate from the left sinus of Valsalva, 17 had the left coronary originate from the right sinus, and 2 had an eccentric single coronary ostium. Twenty six of the 50 patients had symptoms of myocardial ischemia preoperatively, and 14 patients had associated congenital heart defects. Repair was accomplished by unroofing in 35, reimplantation in 6, and pulmonary artery translocation in 9.
There was no operative mortality. The median time of follow-up has been 5.7 years. Two patients were lost to follow-up, and 1 patient required heart transplantation 1 year after AAOCA repair. In the remaining 47 postoperative patients, all have remained free of cardiac symptoms and no one has experienced a sudden death event.
The surgical treatment of AAOCA is safe and appears to be highly effective in eliminating ischemic symptoms. These medium-term results are encouraging and suggest that many patients may be able to resume normal activities.
The Annals of thoracic surgery 06/2011; 92(2):691-7. · 3.74 Impact Factor
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ABSTRACT: Vascular rings are a relatively rare entity caused by abnormal development of the fetal aortic arches. Most patients with vascular rings present in infancy or early childhood with respiratory (inspiratory) symptoms. The treatment of patients with symptomatic vascular rings is generally straight-forward, focusing on surgical division of the ring. The majority of patients are "cured" by this simple procedure. However, a small percentage of patients do not follow this typical course. The purpose of this manuscript is to review and discuss three patients who demonstrated unusual manifestations of vascular rings. Two patients required reoperation after double aortic arch repair and one had successful treatment of an aberrant right subclavian artery.
Journal of Cardiac Surgery 10/2009; 25(1):79-83. · 0.87 Impact Factor
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Richard D. Mainwaring M.D,
V. Mohan Reddy M.D,
Olaf Reinhartz M.D,
John J. Lamberti M.D,
John G. Jacobson M.D,
Dinice L. Jimenez P.A,
Frank L. Hanley M.D, Richard D. Mainwaring,
V. Mohan Reddy,
Olaf Reinhartz,
John J. Lamberti,
John G. Jacobson,
Dinice L. Jimenez,
Frank L. Hanley
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ABSTRACT: Abstract There will be a continuing need to start new congenital heart surgery programs to serve communities experiencing significant population growth. However, small congenital heart programs frequently underperform their larger counterparts. This study summarizes the clinical outcome data for the first 42 months of a small, start-up congenital heart surgery program. Clinical outcomes were summarized from the start of the program in September 2003 through March 2007. Risk adjustment analysis was performed using the risk adjustment in congenital heart surgery (Rachs-1) risk adjustment model and Society of Thoracic Surgeons (STS) congenital database. Three hundred eighty-six operations have been performed during this time period, including 47 newborns, 96 infants, 217 children/adolescents, and 26 adults (greater than 18 years). There have been two operative mortalities (0.5%). Assigning these cases to the Rachs-1 categories, there were 64 level I, 188 level II, 90 level III, 12 level IV cases, with 32 “others.” The predicted mortality for the 354 categorized cases calculates to be 17.4 (Rachs-1) and 10.2 (STS). The data demonstrate that a start-up program with a relatively modest surgical volume can achieve satisfactory clinical results. This model has relied upon careful case selection and direct, senior-level surgeon involvement through an affiliation with a university-based program. These results suggest that a small congenital heart surgery program can be successfully started if the circumstances are carefully controlled.
Journal of Cardiac Surgery 10/2008; 23(6):622 - 626. · 0.87 Impact Factor
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JAAPA: official journal of the American Academy of Physician Assistants 12/2007; 20(11):26-7.
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ABSTRACT: Brain natriuretic peptide is a relatively recently discovered circulating mediator that has been correlated with the degree of heart failure in adults. This study evaluated the preoperative and postoperative brain natriuretic peptide levels in infants and children undergoing ventricular septal defect repair.
The study enrolled 18 infants and children (ages 2 months to 15.6 years) scheduled for surgical repair of their ventricular septal defects. Brain natriuretic peptide levels were drawn preoperatively and then postoperatively at 1, 24, 48, and 72 hours. The amount of shunt (the ratio of pulmonary blood flow [Q(p)]/systemic blood flow [Q(s)]) through the ventricular septal defect was determined by saturation levels performed in the catheterization laboratory or intraoperatively.
The preoperative brain natriuretic peptide levels (pg/mL) averaged 78 +/- 57, and the postoperative levels were 168 +/- 241 at 1 hour, 418 +/- 330 at 24 hours, 405 +/- 364 at 48 hours, and 391 +/- 397 at 72 hours. These differences were significant for each postoperative time point compared with preoperative values. Preoperative brain natriuretic peptide and the Q(p)/Q(s) were significantly correlated (age-adjusted R(2) = 0.33, p < 0.001).
Brain natriuretic peptide levels have a close correlation with the physiologic volume load caused by ventricular septal defects. The preoperative brain natriuretic peptide levels were also found to be predictive for the postoperative time course of brain natriuretic peptide level changes. These results suggest that brain natriuretic peptide levels may be a useful clinical marker in infants and children with ventricular septal defects.
The Annals of thoracic surgery 12/2007; 84(6):2066-9. · 3.74 Impact Factor
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Ed Petrossian,
V Mohan Reddy,
Kathryn K Collins,
Casey B Culbertson,
Malcolm J MacDonald,
John J Lamberti,
Olaf Reinhartz, Richard D Mainwaring,
Paul D Francis,
Sunil P Malhotra,
David B Gremmels,
Sam Suleman,
Frank L Hanley
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ABSTRACT: Our approach to the extracardiac conduit Fontan operation has evolved over time from full-pump, to partial-pump, to completely off-pump. This study is designed to report our overall experience with the extracardiac conduit Fontan operation and to evaluate the evolution in bypass technique on postoperative outcomes.
From September 1992 to April 2005, 285 patients, median age 4.5 years (1.4-44 years), median weight 16 kg (9.4-94 kg), underwent a primary extracardiac conduit Fontan procedure. Early and late outcomes were analyzed for the entire cohort and for 2 patient groups depending on whether an oxygenator was used in the bypass circuit (166 patients; 58%) or not (119 patients; 42%).
Early failure (including death and takedown) occurred in 7 patients (2.5%). Prevalence of new early postoperative sinus node dysfunction necessitating a permanent pacemaker was 0.4%, and that of new tachyarrhythmias necessitating discharge home on a regimen of antiarrhythmia medications was 2.5%. Ten-year actuarial freedom from Fontan failure, new sinus node dysfunction necessitating a permanent pacemaker, and reoperation for conduit thrombosis or stenosis was 90%, 96%, and 98%, respectively. Fenestration rate was lower (P = .001) in the no-oxygenator group (8%) than in the oxygenator group (25%). Patients in the no-oxygenator group had lower intraoperative Fontan pressure (12.0 +/- 2.3 vs 13.5 +/- 2.4 mm Hg, P < .001), common atrial pressure (4.6 +/- 1.8 vs 5.3 +/- 1.8 mm Hg, P = .003), and transpulmonary gradient (7.5 +/- 2.1 vs 8.3 +/- 2.2 mm Hg, P = .013) than did the oxygenator group.
The extracardiac conduit Fontan operation coupled with minimal use of extracorporeal circulation is associated with favorable intraoperative hemodynamics, low fenestration rate, minimal risk of thrombosis or stenosis, and minimal early and late rhythm disturbance.
The Journal of thoracic and cardiovascular surgery 11/2006; 132(5):1054-63. · 3.41 Impact Factor
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ABSTRACT: Unifocalization of major aortopulmonary collateral arteries (MAPCAs) in pulmonary atresia with ventricular septal defect and intracardiac repair has become the standard of care. However, there are no reports addressing unifocalization of MAPCAs in single-ventricle patients. It is unknown whether their pulmonary vascular bed can be reconstructed and low enough pulmonary vascular resistance achieved to allow for superior or total cavopulmonary connections.
We reviewed data on all patients with functional single ventricles and unifocalization procedures of MAPCAs. From 1997 to 2005, 14 consecutive children with various single-ventricle anatomies were operated on.
Patients had a median of three surgical procedures (range, 1 to 5). Two patients had absent, all others diminutive central pulmonary arteries, with an average of 3.5 +/- 1.2 MAPCAs. Seven patients (50%) had bidirectional Glenn procedures, and 3 of these had Fontan procedures. Median postoperative pulmonary artery pressures measured 12.5 mm Hg (Glenn) and 14 mm Hg (Fontan), respectively. Six patients are alive today (46%), with 1 patient lost to follow-up. Three patients died early and 3 late after initial unifocalization to shunts. One other patient survived unifocalization, but was not considered a candidate for a Glenn procedure and died after high-risk two-ventricle repair. Another patient with right-ventricle-dependent coronary circulation died of sepsis late after Glenn.
In selected patients with functional single ventricles and MAPCAs, the pulmonary vascular bed can be reconstructed sufficiently to allow for cavopulmonary connections. Venous flow to the pulmonary vasculature decreases cardiac volume load and is likely to increase life expectancy and quality of life for these patients.
The Annals of thoracic surgery 10/2006; 82(3):934-8; discussion 938-9. · 3.74 Impact Factor
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ABSTRACT: General Robert E. Lee has been interpreted by history as one of the most intriguing personalities of the American Civil War era. In more recent years, there has been much speculation regarding General Lee's health during the war and the possible influence this had on his decision making and performance. Lee's personal letters during and after the war provide some documentation about his health condition, albeit through the eyes of a lay person. The history that was recorded by his personal physicians in Lexington, Virginia provides invaluable insights into his medical history. This manuscript focuses on the lives of these two physicians, Drs. Howard Barton and Robert Madison, and their interactions with their famous patient.
Southern Medical Journal 09/2005; 98(8):800-4. · 0.83 Impact Factor
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The Annals of Thoracic Surgery 01/2004; 76(6):2167; author reply 2167. · 3.74 Impact Factor
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ABSTRACT: Patients undergoing the modified Fontan procedure may develop low cardiac output postoperatively. Since thyroid hormone has important effects on cardiovascular function, the present study was undertaken to evaluate the influence of triiodothyronine supplementation. Ten consecutive patients under-going the Fontan procedure were administered intravenous triiodothyronine (0.4 mcg per kg) following surgery. Clinical outcome and thyroid hormone profiles were assessed and then compared to a previous series of patients undergoing the Fontan procedure who had not received triiodothyronine supplementation. Both groups initially demonstrated marked decreases in serum free triiodothyronine levels. The group which received triiodothyronine supplementation demonstrated a more rapid return of serum triiodothyronine levels to baseline [259±17 vs 121±15 pg/dl (p<0.05) on the fifth postoperative day and 336±18 vs 178±12 pg/dl (p<0.05) on the eighth day]. In addition, patients receiving supplemental triiodothyronine demonstrated more rapid recovery of total triiodothyronine, free thyroxine, total thyroxine and thyroglobulin levels. The group which received triiodothyronine supplementation had a shorter length of hospital stay [9±2 vs 14±3 (p<0.05)] as compared to patients who did not receive exogenous triiodothyronine. The results of this study demonstrate that triiodothyronine supplementation aids in the recovery of thyroid hormone levels following Fontan procedure. This endocrinologic finding correlated with improved clinical outcome.
Cardiology in the Young 03/1997; 7(02):194 - 200. · 0.76 Impact Factor
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ABSTRACT: The Fontan procedure provides a means of separating the systemic and pulmonary circulations in patients with congenital heart defects characterized by a functionally single ventricle. The procedure has undergone numerous changes since its introduction 25 years ago. Technical modifications have now increased its applicability to virtually all forms of univentricular atrioventricular connection, as well as many hearts with biventricular connections. More recently, philosophical changes have been introduced to address increased rates of morbidity and mortality observed in certain subsets of patients. These changes appear to have improved short- and midterm results. The purpose of this review is to offer our strategy for management of such patients. We will discuss the interrelationship between the bidirectional Glenn and Fontan procedures. We will also focus on a number of observations concerning the possible role of accessory pulmonary blood flow, aortopulmonary collateral arteries, and endocrinologic changes.
Cardiology in the Young 06/1996; 6(03):198 - 207. · 0.76 Impact Factor
