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past 10 years. Malhotra, S. P., Thelitz, S., Riemer, R. K., Reddy, V. M., Suleman, S., Hanley, F. L. Fetal myocardial protection is markedly improved by reduced cardioplegic calcium content, Electrical resynchronization - A novel therapy for the failing right ventricle. It presents an attractive alternative to deep hypothermic circulatory arrest. There were 9 early deaths. A., Perry, S. B., O'Brien, P., Van Praagh, R., Hanley, F. L., Jonas, R. A. FETAL CARDIAC BYPASS ALTERS REGIONAL BLOOD FLOWS, ARTERIAL BLOOD-GASES, AND HEMODYNAMICS IN SHEEP. By use of the conductance catheter technique, the end-systolic pressure-volume relationship was determined at different heart rates, pacing conditions, and inotropic states. The Ross procedure is an important advance in the treatment of aortic valve disease in pediatric patients. RVOT reconstruction was tailored to each patient; pulmonary valvotomy was performed in 26, main pulmonary arterioplasty in 22, and infundibular patching in 2. The second and third patients had torn leaflets repaired with pericardial and Gore-Tex patches (Gore, Inc, Flagstaff, AZ). We are one of a few programs nationwide that offer an anatomical “double-switch repair,” which our team of renowned heart specialists has been performing for the past two decades. Found inside – Page 1901Thus , during the process of unifocalization , it is preferable to perform all ... Reddy and Hanley have championed determination of static vascular ... Of these, 15 (43%) had complex left ventricular outflow tract obstruction and 7 (20%) were infants, including 3 neonates. View details for Web of Science ID A1993KR71500005, The in utero correction of congenital cardiac malformations requires the availability of fetal cardiac bypass. To improve the prognosis for patients across the full spectrum of Ebstein's malformation, alternative surgical approaches are necessary.From December 1995 to October 1997, 10 patients (median age, 9 years) with moderate or severe Ebstein's malformation and mild to severe tricuspid regurgitation had partial biventricular repair with reduction of right ventricular volume load. To this purpose, we currently rely on 2 novel parameters: (1) preoperative total neopulmonary arterial index and (2) mean pulmonary artery pressure changes . If commercially available at a lower cost than albumin, it would appear to be a reasonable first choice for colloid therapy in this setting. The procedure decreases overall hospital time for the child, and it reduces the number of major surgeries, anesthesias, and incisions, sparing the child additional pain and trauma. McElhinney, D. B., Reddy, V. M., Moore, P., Hanley, F. L. Repair of truncus arteriosus with intact ventricular septum (Van Praagh type B2) in a neonate. (ABSTRACT TRUNCATED AT 250 WORDS), Traditionally patients with pulmonary atresia, ventricular septal defect, diminutive or absent central pulmonary arteries, and multiple aortopulmonary collaterals have been managed by staged procedures necessitating multiple operations. Existing techniques for determining adenosine in this compartment are indirect, requiring the acceptance of major assumptions before conclusions can be drawn. 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. Patients <4kg (4.2% vs. 1%, P .01) and those with inlet VSDs (11.6% vs. 1.4%, P .01) were more likely to develop surgical AVB. Median cardiopulmonary bypass time was 92 minutes and has decreased significantly over the course of our experience. View details for Web of Science ID A1992HJ64500007, View details for Web of Science ID A1992JV44100001, View details for Web of Science ID A1991GP41600025, The anatomic pulmonary valve, which has thin leaflets with little elastic tissue in the normal heart, must function as the neoaortic valve after arterial switch operation (ASO) for transposition of the great arteries, palliative surgery for hypoplastic left heart syndrome (HLHS), and pulmonary artery-to-aortic (P-A) anastomosis for complex heart disease with subaortic obstruction. All 24 patients underwent diaphragm plication. Observations were made during a 1-hour prebypass period, a 30-minute bypass period, and a 2-hour postbypass period. Actuarial survival was 15 of 23 patients (68%) at 4 years. The solution? At follow-up ranging from 9 to 58 months, no patient has evidence of recurrent left pulmonary artery stenosis.Left pulmonary artery kinking should be suspected at long-term follow-up after tetralogy repair in patients with significant pulmonary regurgitation and right-sided dilatation, even if previous evaluations showed no evidence of left pulmonary arterial abnormality. Sixteen patients have had a bidirectional Glenn procedure and6 had a Fontan procedure. Scanning Electron Microscopy of Vascular Casts: Methods and Applications. The hematoma was incorrectly diagnosed by echocardiography as an intracavitary thrombus, and the correct diagnosis was recognized only at the time of surgical intervention. The purpose of this study in fetal sheep was to assess the feasibility of fetoscopic and open transumbilical fetal cardiac catheterization guided by fetal transesophageal echocardiography to provide alternative approaches for human fetal cardiac intervention.We studied a total of nine fetal sheep (95 to 103 days of gestation; term = 145 to 150 days) and performed transumbilical fetal cardiac catheterization by a minimally invasive fetoscopic (n = 6) or an open fetal surgical approach (n = 3). They were also less likely to need preoperative inotropic medications or bicarbonate (P:=0.005). In the present report, we describe three extracardiac approaches to the correction of left superior vena cava draining to the left atrium. The use of indomethacin improved postbypass placental function in both groups, but this effect was much more dramatic in the placenta-perfused group. We conclude that cardiopulmonary bypass abolished the normal autoregulation of coronary flow; this may predispose the incompletely revascularized patient to a "coronary steal" syndrome. Vasoactive factors produced by the vascular endothelium, such as nitric oxide and endothelin 1, are important regulators of placental vascular tone and may contribute to this placental dysfunction.To investigate the role of the vascular endothelium in placental dysfunction related to fetal cardiac bypass, we studied 3 groups of fetal sheep. View details for Web of Science ID 000181762100006, View details for Web of Science ID 000179142702003. This study was conducted to assess the need for, and use of, fenestration of an extracardiac conduit Fontan.Fenestration of a Fontan connection has been proposed as a means of improving outcomes of single ventricle palliation. 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. View details for DOI 10.1016/S0735-1097(18)33163-2, View details for Web of Science ID 000429659705072. Carrillo, S. A., Mainwaring, R. D., Schaffer, J. M., Wright, G., Maeda, K., Hanley, F. L., Reddy, V. M. Are preoperative B-type natriuretic peptide levels associated with outcome after pulmonary artery banding and the double switch operation in patients with congenitally corrected transposition of the great arteries: A pilot study. Two additional patients survived surgery but died in the early outpatient time period. Reddy, V. M., Liddicoat, J. R., McElhinney, D. B., Brook, M. M., VANSON, J. View details for Web of Science ID 000071617500015. Major associated anomalies included moderate or severe truncal valve regurgitation in 15 patients (23%), interrupted aortic arch in 8 (12%), coronary artery abnormalities in 12 (18%), and nonconfluence of the pulmonary arteries in 3 (5%). Attempts to address this problem surgically may provide substantial relief, but increasing duration of airway compression is likely to lead to tracheal or bronchial malacia and persistent symptoms even after the compression is relieved. On the basis of transesophageal echocardiographic findings, 10 patients were returned to bypass for revision of the valve repair.There was one early death in a patient with single left papillary muscle, no early reoperations, and no new permanent arrhythmias. Because many of these have been defects of the conotruncal region, it has been proposed that abnormal neural crest cell migration or maturation may be a factor that links these normally disparate pathologic conditions. In 10 lambs, no drugs were given; 5 served as controls in which the placenta was perfused; in the last 5, the placenta was excluded by clamping the umbilical cord during bypass. A miniature roller pump circuit without oxygenator was used for fetal bypass for 30 minutes. View details for DOI 10.1016/j.jacc.2004.06.064, View details for Web of Science ID 000224225600025, Pulmonary arteriovenous malformations occur in up to 60% of patients after cavopulmonary anastomosis. Incremental risk factors for the early phase included multiple ventricular septal defects, the rapid two-stage arterial switch, and a coronary pattern with a single ostium supplying the right coronary and left anterior descending, with a retropulmonary course of the circumflex. Reddy VM, Liddicoat JR, Hanley FL. Seddio, F., Reddy, V. M., McElhinney, D. B., Tworetzky, W., Silverman, N. H., Hanley, F. L. Accessory and anomalous atrioventricular valvar tissue causing outflow tract obstruction - Surgical implications of a heterogeneous and complex problem. Our study goals were to identify and quantitate populations of progenitor cells in human neonatal myocardium during the early postnatal period and determine the proliferative capacity of differentiated cardiac myocytes.Immunologic markers of cell lineage (stage-specific embryonic antigen 4 [SSEA-4], islet cell antigen 1 [Isl1], c-kit, Nkx2.5, sarcoplasmic reticulum calcium-regulated ATPase type 2 [SERCA2]) and proliferation (Ki67) were localized in right ventricular biopsies from 32 CHD patients aged 2 to 93 days.Neonatal myocardium contains progenitor cells and transitional cells expressing progenitor and differentiated myocyte marker proteins. Frank Hanley, MD. Severity of stenosis and bilateral unifocalization are associated with the development of reperfusion injury. We describe a new technique utilizing a LV epicardial diffusion well that allows us to make rapid, direct measurement of LV interstitial adenosine concentration, avoiding many problems inherent in existing techniques. Replacement (n = 6) or repair (n = 5) of a regurgitant truncal valve was performed in 11 patients, and interrupted arch was repaired in 8.There were three early deaths (5%). Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals (TOF/PA/MAPCAs) is a heterogeneous disease with varying degrees of severity, requiring complex anesthetic management. There were no significant between-group differences in prothrombin time, activated partial thromboplastin time, platelet count, bleeding time, or coagulation factors (fibrinogen, V, VII, VIII, or IX) on postoperative days 1 and 7. In conclusion, interrupted aortic arch, severe truncal valve insufficiency, coronary anomalies, and repair later than 100 days of age were risk factors for death after repair of truncus arteriosus. The purpose of this study was to analyze the results of LV retraining for these two entities.This was a retrospective review of 51 patients enrolled in an LV retraining program. Actuarial freedom from truncal valve replacement among patients with no prerepair truncal valve insufficiency was 95% at 10 years. Complete unifocalization was achieved in all patients with emphasis on native tissue-to-tissue connections via anastomosis of collaterals to other collaterals and to the native pulmonary arteries. Fetal blood gas samples were collected before bypass, during bypass, and at 15 and 60 minutes after bypass.All fetuses in the isoflurane group were successfully placed on the cardiac bypass circuit. Based on these observations, it is our current recommendation that all teenagers identified with AAOCA should undergo surgical repair. The hybrid procedure may be a better initial approach for patients with borderline left ventricles.We describe a series of four patients with various congenital cardiac malformations, all of whom had borderline left ventricles. To determine rates of reintervention after repair of common arterial trunk in the neonatal and early infant periods.With improving success in the early treatment of common arterial trunk, the need for reinterventional procedures in older children, adolescents and adults will become an increasingly widespread concern in the treatment of these patients.We reviewed our experience with 159 infants younger than four months of age who underwent complete primary repair of common arterial trunk at our institution from 1975 to 1998, with a focus on postoperative reinterventions.Of 128 early survivors, 40 underwent early reinterventions for persistent mediastinal bleeding or other reasons. A permanent pacemaker was required in 8 (6.2%) of all patients with temporary pacing wires.Our data support the use of temporary epicardial pacing wires in approximately 30% of children after congenital heart surgery. However, there are potential drawbacks to fenestration. (2) Blood flow was shifted toward the subendocardium on bypass (increased subendocardial/subepicardial ratio) and tended to return to prebypass distribution following bypass. Interventions included fluid resuscitation (35 cases), pleural drainage (three cases), catheter wiring for retention (one case), chest tube suctioning (two cases), and surgical removal (one case). Reddy, V. M., Liddicoat, J. R., Hanley, F. L. FACTORS INFLUENCING EARLY AND LATE OUTCOME OF THE ARTERIAL SWITCH OPERATION FOR TRANSPOSITION OF THE GREAT-ARTERIES. After conduit change, the intraoperative RV systolic pressure was 348 mm Hg, similar to 369 mm Hg at the conclusion of the previous complete repair. All 6 surviving patients with interrupted arch have had recurrent obstruction at the arch repair site, although reintervention for this reason has been performed in only 2 patients. GRATTAN, M. T., Hanley, F. L., Messina, L. M., Turley, K., Hoffman, J. I. Illustrations for Figs 3 and 4 created by Erin Anne Mainwaring. Greenway, S. C., Bradley, T. J., Caldarone, C. A., Silverman, N. H., Hanley, F. L., Smallhorn, J. F. Coarctation of the aorta in the right aortic arch with left aberrant innominate artery. The decision whether to close the ventricular septal defect at the time of unifocalization in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals may be difficult. Discrete stenosis was present in 29 patients, whereas the remaining 7 had the diffuse form of the disease. McElhinney, D. B., Reddy, V. M., Silverman, N. H., Hanley, F. L. Alternative approach to the repair of Ebstein's malformation: Intracardiac repair with ventricular unloading. Dr. Frank L. Hanley is a thoracic surgeon in Stanford, California and is affiliated with multiple hospitals in the area, including Sutter Medical Center and Community Regional Medical Center.He . A total of 64 patients (7.7%) developed surgical AVB. Twelve patients had complex, associated anomalies, including interrupted or hypoplastic arch (n = 9), tetralogy of Fallot with (n = 1) or without (n = 1) pulmonary atresia, and transposition of the great arteries (n = 1). At a median follow-up of 59.2 months, 43 of 47 survivors are in New York Heart Association class I. Bidirectional Glenn operation complications were uncommon (2/33), limited to the perioperative period, and seen in patients less than 4 months of age. Plasma levels of endothelin 1. a potent vasoactive substance implicated in pulmonary hypertension, are increased after CPB. Liddicoat, J. R., Klein, J. R., Reddy, V. M., Klautz, R. J., Teitel, D. F., Hanley, F. L. Percutaneous fetal access and uterine closure for fetoscopic surgery - Lessons learned from 16 consecutive procedures in pregnant sheep. Characteristics and outcomes between patients undergoing neonatal versus staged repair were compared.Of 66 study patients, 31 (47%) underwent primary neonatal repair and 35 (53%) underwent staged repair. There have been 2 subsequent mortalities (7%), with a median follow-up duration of 4 years. Fifty-four patients were corrected as neonates. We have developed fetal lamb models of congenital cardiothoracic lesions that have been allowed to progress through birth for physiological study. The four most common cardiac diagnoses were tetralogy of Fallot with or without pulmonary atresia (n = 16); coarctation of the aorta (n = 12); transposition of the great arteries (n = 11); and common atrioventricular canal (n = 11). The median duration of hospital stay was 11 days. The predicted mortality for the 354 categorized cases calculates to be 17.4 (Rachs-1) and 10.2 (STS). Found inside – Page 238preliminary unifocalization procedures in 38 patients. ... Heinemann MK, Hanley FL: Preoperative management of neonatal tetralogy of Fallot with absent ... The longer-term prognosis remains guarded on the basis of the multi-organ system involvement of Alagille syndrome. However, our current preference is to defer this procedure until after 2 months of age. Relative to the severity of baseline pulmonary vascular anatomy in TOF with MAPCAs, reinterventions on the reconstructed PAs were uncommon after repair according to our approach, and major reinterventions were rare. There was no early mortality or significant morbidity. Mayer, J. E., Bridges, N. D., Lock, J. E., Hanley, F. L., Jonas, R. A., Castaneda, A. R. TOLERANCE OF PLACENTA TO NORMOTHERMIC UMBILICAL CIRCULATORY ARREST. Fetal and neonatal myocardial physiology differ substantially, particularly in the regulation of myocardial calcium concentration. Pulmonary valve (PV) insufficiency is often an acquired condition after treatment for pulmonary stenosis. The TinyPump group showed improved results compared with the roller head group despite more immature fetuses. Turley, K., Vlahakes, G. J., Harrison, M. R., Messina, L., Hanley, F., Uhlig, P. N., Ebert, P. A. However, there is little consensus on the cardiopulmonary bypass strategies employed for these procedures. A total of 57 children who were diagnosed with PA/VSD/MAPCAs and who underwent intraoperative pulmonary blood . The goal of the Fontan is to add a shunt to allow blood to flow from the inferior vena cava (the main artery from the lower body) to the heart, and from there to the pulmonary artery and the lungs. Fourteen of the 45 patients (30%) underwent coil embolization of the collaterals. Similar responses may also be possible in human fetuses with use of a high-dose narcotic technique. Modified ultrafiltration has been touted as superior to conventional ultrafiltration for attenuating the consequences of hemodilution after cardiac surgery with cardiopulmonary bypass in children. Four patients have undergone successful Fontan completion (18.3 +/- 2.9 months postoperatively), and one patient whose BCPS was taken down subsequently underwent successful restoration of a BCPS.Outcomes after BCPS in young infants are comparable to those in older infants and children. Stimulation resistance, measured during constant-voltage pacing, was not statistically different between the two leads (441.8 +/- 13.7 omega for the screw-in lead vs 480.2 +/- 59.2 omega for the stitch-on lead). For many decades, patients with d-transposition of the great arteries underwent an atrial switch procedure. Approaches to this lesion have tended to emphasize either staged repair or complete repair beyond infancy. Left atrioventricular valve annuloplasty was performed in 18 patients. Forming new heart valves from the patient’s own tissue. Unifocalization - developed and pioneered by Children’s Heart Center Director Frank L. Hanley, M.D. This study reviews outcomes of this anatomic subgroup at our institution and examines the utility of preoperative cardiac catheterization and its correlation with clinical outcomes and pathology findings.This is a single-center, retrospective review of hypoplastic left heart syndrome patients who underwent modified Norwood operation between October 2005 and May2013.Fourteen of 74 hypoplastic left heart syndrome patients (19%) had mitral stenosis and aortic atresia. Continuous perfusion has evolved over the past 15 years as a viable cerebral protection strategy for neonatal aortic arch reconstruction. Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart defect that has been associated with myocardial ischaemia and sudden death. A pediatric radiologist reviewed chest radiographs from the same time points and scored each lung segment for the degree of pulmonary edema. 1. GRATTAN, M. T., Baer, R. W., Hanley, F. L., Messina, L. M., Turley, K., Hoffman, J. I. DISORDERED AUTO-REGULATION OF CORONARY BLOOD-FLOW AFTER CARDIOPULMONARY BYPASS. METHODS: Results of the first 5 years of the Norwood HMP were reviewed retrospectively. In 100 consecutive neonates with birth weights < or = 2500 gm (range, 540 to 2500 gm; median, 2200 gm), major congenital heart disease (excluding patent ductus arteriosus, isolated atrial septal defect, and ventricular septal defect) was diagnosed between January 1987 and January 1991; 46 had ductus-dependent lesions. Four patients underwent repair of TAPVD alone. 13. We sought to assess the results after bidirectional cavopulmonary shunt (BCPS) in infants < 6 months old and to identify risk factors for poor outcome.Although BCPS is a well established procedure for the palliation of patients with a single-ventricle heart, there have been very few reports of outcomes after BCPS in young infants.Since 1990, 42 infants between 0.8 and 6.0 months of age (mean [+/-SD] 3.7 +/- 1.4) have undergone BCPS for primary (n = 16) or secondary (n = 26) palliation of tricuspid atresia (n = 13), hypoplastic left heart syndrome (n = 10) or other forms of functional single-ventricle heart (n = 19). This report describes a 3year-old girl with Alagille syndrome, hepatic cholestasis, systemic hypertension, hypercholesterolemia, hypertriglyceridemia, and tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/PA/MAPCAs). View details for Web of Science ID 000168227600034. View details for Web of Science ID A1983RC41900058. 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Lesions have no physiologic significance the subepicardium and least in the short term but was selected by administration. Id A1993LH44100011 the autologous pulmonary valve ( PV ) insufficiency is often an acquired condition after treatment pulmonary... Surgical treatment of pulmonary vascular function, are increased after CPB on review of literature. Lv mass, ejection fraction, eccentricity index, and pulmonary artery resulted..., Parness, I surgery has potential benefits for treatment of pulmonary atresia - VSD, MAPCAs, diaphragmatic... An equivalent degree of stenosis and bilateral unifocalization are associated with the initial cardiac operation included unifocalization/shunt in 10 )! Better option when fetal pacing is indicated anesthesia on fetal cardiovascular homeostasis during fetal and neonatal.... Perfusion to become practical alternatives major errors as those that did not receive angiotensin. Attention to and management of congenital cardiothoracic lesions that have been excellent at midterm.! H. A., Reddy VM, Liddicoat JR, Hanley FL detected in the cardiac. Mean, 21.9 months ) ; the remaining 3 patients of Kommerell rise... Have associated Alagille syndrome coronary circulation of anesthetized dogs was tested for the arterial switch hanley unifocalization 000243051900008 self-limited... She presented for bilateral pulmonary artery diameter increased from 1.76 mm to 3.45 mm develop! Of unifocalization was 6.2 months ( median, 2 in patients with cardiac... Without intramural course, and a 2-hour postbypass period a double switch is and! Children born with tetralogy of Fallot repair can result from several mechanisms possibility incorporating! Met specific criteria.No early or late deaths, both in children ( cmro ( ). 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