D J Driscoll

Mayo Clinic - Rochester, Rochester, Minnesota, United States

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Publications (169)1070.86 Total impact

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    ABSTRACT: To describe and analyze the cardiopulmonary responses to exercise for patients with repaired tetralogy of Fallot (TOF) before and after pulmonary valve replacement (PVR) and compare our results with those in the literature.
    Congenital Heart Disease 08/2014; · 1.01 Impact Factor
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    ABSTRACT: Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis.
    Journal of the American College of Cardiology 07/2014; 64(1):54-62. · 14.09 Impact Factor
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    ABSTRACT: Background Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis. Objectives The aim of this study was to review outcomes in patients with PLE following the Fontan operation. Methods From 1992 to 2010, 42 patients (55% male) with PLE following the Fontan operation were identified from clinical databases at the Mayo Clinic. Data were collected retrospectively. Results Mean age at PLE diagnosis was 18.9 ± 11.0 years. Initial Fontan operation was performed at 10.1 ± 10.8 years of age. Mean time from Fontan operation to PLE diagnosis was 8.4 ± 14.2 years. Survival was 88% at 5 years. Decreased survival was seen in patients with high Fontan pressure (mean >15 mm Hg; p = 0.04), decreased ventricular function (ejection fraction <55%; p = 0.03), and New York Heart Association functional class >2 at diagnosis (p = 0.04). Patients who died had higher pulmonary vascular resistance (3.8 ± 1.6 Wood units [WU] vs. 2.1 ± 1.1 WU; p = 0.017), lower cardiac index (1.6 ± 0.4 l/min/m2 vs. 2.7 ± 0.7 l/min/m2; p < 0.0001), and lower mixed venous saturation (53% vs. 66%; p = 0.01), compared with survivors. Factors were assessed at the time of PLE diagnosis. Treatments used more frequently in survivors with PLE included spironolactone (21 [68%]), octreotide (7 [21%]), sildenafil (6 [19%]), fenestration creation (15 [48%]), and relief of Fontan obstruction (7 [23%]). Conclusions PLE remains difficult to treat; however, in the current era, survival has improved with advances in treatment. Further study is needed to better understand the mechanism of disease and ideal treatment strategy.
    Journal of the American College of Cardiology 07/2014; 64(1):54–62. · 14.09 Impact Factor
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    ABSTRACT: Patients with atriopulmonary Fontan tend to undergo conversion to total cavopulmonary connections secondary to arrhythmias or poor flow dynamics. However, the ideal candidate is unknown. Between December 1994 and May 2011, 70 patients (40 males [57%]) underwent Fontan conversion. Median age was 23 years (range, 4 to 46 years). Excluded were 1.5 ventricle conversions. The most common diagnoses included tricuspid atresia in 34patients (49%) and double-inlet left ventricle in 16 (23%). Atrial tachyarrhythmia was present in 62 patients (89%), 41 (59%) had atrioventricular valve (AVV) regurgitation, and 32 (46%) were in New York Heart Association class III or IV. Atriopulmonary Fontan was the original connection in 58 patients (83%), whereas the Björk modification was performed in 8 (11%). Fontan was performed with an intraatrial conduit in 41 patients, an extracardiac conduit in 18, and a lateral tunnel in 11. Forty-nine patients (70%) underwent concomitant arrhythmia operations. Early death occurred in 10 patients (14%). Multivariate analysis revealed age older than 27 years (p = 0.009), AVV regurgitation (p = 0.016), lack of arrhythmia operation (p = 0.04), and male sex (p = 0.02) were predictors of perioperative death. Mean follow-up was 5 years (maximum, 17 years). Overall survival at 1, 5, and 10 years was 81%, 70%, and 67%, respectively, and 84% of patients were in New York Heart Association class I or II. Proper selection of Fontan conversion candidates is critical. Concomitant arrhythmia operations may be associated with improved survival. Older age and AVV regurgitation increase the risk of poor outcome, and cardiac transplantation may be a better option.
    The Annals of thoracic surgery 04/2014; · 3.45 Impact Factor
  • Mayo Clinic Proceedings 03/2014; 89(3):e23-5. · 5.79 Impact Factor
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    ABSTRACT: Objective To report our single-center experience with patients who had cardiac and multiorgan transplantation for end-stage congenital heart disease (CHD). Patients and Methods We reviewed records for all patients with CHD who had undergone heart transplantation at Mayo Clinic, Rochester, Minnesota, from November 1, 1990, through June 30, 2012. Patients with cardiomyopathy were excluded, unless CHD was present. Results Overall, 45 patients had cardiac transplantation for end-stage CHD (mean age, 26.1±18.4 years; range, 1 month to 65 years). Two patients (4%) had combined heart/liver transplantation; 1 (2%) had heart/kidney transplantation. Six patients (13%) had no previous cardiac operation; the remaining 39 patients had a mean of 3 (range, 1-8) previous cardiac operations. Patient survival (95% CI) at 1, 5, and 10 years was 89% (80%-98%), 89% (80%-98%), and 72% (56%-87%), respectively, while graft survival at 1, 5, and 10 years was 89% (80%-98%), 89% (80%-98%), and 61% (44%-78%), respectively. During the same era, the International Society for Heart & Lung Transplantation reported that survival in patients undergoing transplant for non-congenital diagnoses was 85%, 72%, and 56%, respectively. Over a mean follow-up of 8.7±6.2 years, rejection requiring treatment was documented in 35 patients (78%). Eleven patients (24%) have been diagnosed with neoplasia (8 skin, 1 blood, 1 lymph, and 1 other), and 3 patients (7%) have required retransplantation. Four patients (9%) have developed significant coronary vasculopathy; 1 successfully underwent retransplantation, and 3 died 6, 8, and 14 years after transplantation. Conclusion With appropriate patient selection and posttransplant monitoring, survival has improved for patients with complex end-stage CHD. Multiorgan transplantation is an option for selected patients with CHD.
    Mayo Clinic Proceedings 01/2014; 89(4):478–483. · 5.79 Impact Factor
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    ABSTRACT: Sirolimus is used in heart transplant patients with CAV and CNI-induced nephropathy. However, little is known regarding the tolerability, rejection rate, and effect on renal function when used empirically in children. We describe our experience with the empiric use of a sirolimus-based immunosuppressive regimen in pediatric heart transplantation recipients. We reviewed records of patients in whom conversion was attempted to a CNI-free sirolimus-based regimen. Rejection episodes and measures of renal function were recorded. We attempted to convert 20 patients, of which 16 were successful. In total, six of 20 patients (30%) experienced adverse effects. Of the 16 converted, four patients converted to sirolimus due to CNI-induced disease (three nephropathy, one CAV), while 12 patients (mean age 5.5 yr, range 0.1-21 yr; 33% female; 33% with a history of congenital heart disease) were empirically switched to sirolimus at a mean of 2.3 yr after transplant. Follow-up was available for a mean of 2.5 yr after conversion (range 0.5-8.3 yr). The rate of rejection while taking CNIs was 0.18 rejection episodes per patient-year (total of five episodes), compared with 0.03 rejection episodes per patient-year (total of one episode) while on sirolimus. Renal function, in terms of GFR, significantly improved after sirolimus conversion at latest follow-up (from 86 ± 37 mL/min to 130 ± 49 mL/min, p = 0.02). Here, we demonstrate the potential benefit of empiric use of sirolimus in pediatric heart transplant patients in a CNI-free regimen. Larger and longer studies are needed to further clarify risks of rejection and adverse effect profiles.
    Pediatric Transplantation 12/2013; 17(8):794-9. · 1.50 Impact Factor
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    ABSTRACT: Vascular malformations result from an arrest of development of a normal vascular structure or from abnormal growth of a vascular structure. Treatment continues to be a challenge. We sought to study the outcomes of sclerotherapy and embolization for vascular malformations. We reviewed clinical data of all patients treated with sclerotherapy or embolization for arteriovenous or venous malformations between 2006 and 2010 at Mayo Clinic. Follow-up information was obtained from clinical charts and a questionnaire sent to all patients. Overall, 60 patients (24 male and 36 female; mean age 31.7 years; range, 5.6 to 72.4 years) had 163 unique sclerotherapy or embolization procedures for lesions involving the lower extremity (55%), upper extremity (18.3%), pelvis (11.7%), abdomen (5%), chest (5%), back (3.3%), and multiple locations (1.7%). Thirty-one patients had low-flow venous malformations and 29 patients had high-flow arteriovenous malformations. Twenty-four patients required more than three sessions. The most common indication for intervention was pain (57 of 60 [95%]). Sixteen patients (27%) had documented or patient-reported complications. There was no significant difference in complication rates or lesion size between patients with low-flow or high-flow lesions. There were no procedural deaths. Mean available follow-up was 2.0 ± 1.3 years (range, 0.5 to 5.0 years). Median pain scores at most recent follow-up decreased significantly (P<.001). Eighty-three percent of the responders (24 of 29) would recommend treatment to others. With appropriate patient selection, sclerotherapy and embolization can decrease the pain of patients with arteriovenous and venous malformations. Multiple interventions might be necessary. Practitioners should be aware of the potential complications and counsel their patients about these risks.
    Seminars in Vascular Surgery 03/2013; 26(1):48-54. · 1.02 Impact Factor
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    ABSTRACT: Posterior reversible encephalopathy syndrome (PRES) is a disorder characterized by gray and white matter abnormalities in the temporal, parietal, and occipital lobes of the brain. Its etiology has been attributed to renal failure, immunosuppressive drugs such as cyclosporine and tacrolimus, and other potential entities leading to acute hypertension. Clinical findings include headaches, altered mental status, seizures, visual changes, and focal neurologic deficits. We report the case of a child who developed PRES with intracerebral and subarachnoid hemorrhages associated with tacrolimus exposure 10 days after heart transplantation for restrictive cardiomyopathy. The patient initially presented with complex partial seizures, headache, agitation, and hypertension. Head MRI was suggestive of PRES along with intracerebral and subarachnoid hemorrhages. Tacrolimus was discontinued and blood pressure was controlled. The patient's encephalopathy resolved, but he has had ongoing neurologic symptoms secondary to hemorrhage. Generally, PRES is less common in children than in the adult population and is a rare complication of calcineurin inhibitors (CNI). Presentation with secondary hemorrhage also can occur. In children receiving CNIs presenting with new neurologic symptoms, PRES should be considered as prompt discontinuation of the offending agent can induce resolution of symptoms. Children can develop hemorrhage in the context of PRES, leading to increased morbidity.
    Pediatric Transplantation 01/2013; · 1.50 Impact Factor
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    ABSTRACT: OBJECTIVE: We characterized the histologic liver changes that occur after the Fontan operation and identified the factors associated with worsening hepatic disease. METHODS: From 1979 to 2009, 33 patients who died after the Fontan operation had a postmortem analysis at our institution. The liver histologic findings were graded for sinusoidal and portal fibrosis. RESULTS: The median interval from the Fontan operation to death was 14 days (range, 0 days to 25.6 years). The median age at death was 7.7 years (range, 1.9-52.9 years). Hepatic sinusoidal fibrosis occurred in 25 of 33 patients (76%), with significant fibrosis (grade 2 or greater) in 15 of 25 patients (60%). Portal fibrosis occurred in 17 of 33 patients (52%). The degree of sinusoidal (P < .003) and portal fibrosis (P < .002) correlated with an increased period from the Fontan operation to death. Sinusoidal fibrosis was also associated with increased age at the initial Fontan operation (P < .03). Of the patients who died early (< 35 days postoperatively) after the Fontan operation, sinusoidal fibrosis was present in 15 of the 23 patients (65%) and portal fibrosis in 7 of 23 patients (30%). Significant (grade 2 or greater) sinusoidal fibrosis was seen in 7 patients, and 4 of the 7 had heterotaxy syndrome. Significant portal fibrosis was observed in only 2 patients; both had heterotaxy syndrome. CONCLUSIONS: The interval from the initial Fontan operation to death is associated with increased sinusoidal and portal fibrosis. In this small cohort, older age at Fontan operation was associated with sinusoidal fibrosis. Significant sinusoidal fibrosis was observed in patients who died early after the Fontan operation, with more severe disease present in patients with heterotaxy syndrome.
    The Journal of thoracic and cardiovascular surgery 10/2012; · 3.41 Impact Factor
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    ABSTRACT: Protein-losing enteropathy (PLE) is a complex disorder characterized by enteric protein loss and often is associated with cardiovascular abnormalities, particularly those with elevated central venous pressure. The Fontan operation is a surgical procedure used to palliate patients with a functional single ventricle. Although the Fontan operation eliminates cyanosis and decreases the workload of the functionally single ventricle, it also elevates central venous pressure. This can result in hepatic and enteric congestion as well as PLE. Despite the universal elevation in central venous pressure, only a fraction of patients who have had a Fontan operation develop PLE. However, PLE is associated with significant morbidity and mortality. Presenting signs and symptoms of PLE include abdominal bloating, diarrhea, edema, pleural effusions, ascites, and failure to thrive. In this review, the authors discuss the diagnosis and prevalence of PLE after the Fontan operation and review currently available therapeutic strategies.
    Nutrition in Clinical Practice 04/2012; 27(3):375-84. · 1.58 Impact Factor
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    ABSTRACT: Introduction.  Diffuse cutaneous systemic sclerosis (SSc) is rare in children, but has a poor prognosis when cardiomyopathy is present. Methods.  We reviewed the case of a 14-year-old female with progressive skin thickening/tightness and dyspnea on exertion who was diagnosed with SSc. Results.  Our patient was found to have severe restrictive cardiomyopathy with poor left ventricular systolic function (ejection fraction = 20%), unresponsive to the immunosuppression used to treat her SSc. There was no evidence of pulmonary fibrosis. The patient underwent orthotopic cardiac transplantation, with improvement in systemic symptoms. Two years after transplantation, she had elevated filling pressures during a surveillance catheterization, with no evidence of cellular rejection or coronary artery vasculopathy. Four months later, she developed severe ventricular dysfunction and dyspnea, despite a negative biopsy and negative C4d immunofluorescence staining. Her immunosuppression was intensified with improvement of cardiac function. Despite this, 1 month later she had sudden pulseless cardiac arrest from which she could not be revived. The family declined an autopsy. Discussion.  We report a 14-year-old female with SSc who had cardiac transplantation. The etiology behind her recurrent restrictive physiology, cardiac dysfunction, and subsequent cardiac arrest remains unclear.
    Congenital Heart Disease 07/2011; · 1.01 Impact Factor
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    ABSTRACT: Approximately 5% to 15% of patients develop protein-losing enteropathy (PLE) after the Fontan operation. Oral controlled release (CR) budesonide has been used as a treatment strategy, but its use in the older Fontan population has not been described. Seven patients with refractory PLE after the Fontan operation were started on oral CR-budesonide at 9 mg. After 3 to 9 months, the dose was weaned to 3 mg. Response to treatment was assessed by clinical evaluation, serum albumin levels, and fecal α-1 antitrypsin clearance when available. Median age at last evaluation was 20 years (range, 16 to 32 years). Six patients had increases in serum albumin levels but only 4 patients had symptomatic improvement. Systemic side effects included: cushingoid features (5), adrenal insufficiency (4), and new-onset type 2 diabetes mellitus (2). One patient had improvement in cushingoid features after weaning CR-budesonide to 3 mg. Older patients (ages 27 to 32 years) had the worst side effect profiles and were the most refractory to treatment. These patients had sonographic evidence of hepatic cirrhosis but normal serum liver function tests. Two deaths occurred: 1 from sepsis 1 month after CR-budesonide initiation and 1 from respiratory arrest 5 months after CR-budesonide discontinuation. CR-budesonide can be used to treat PLE in certain patients, but careful assessment of hepatic function should be performed before initiation of therapy as systemic side effects can limit treatment. Normal serum liver function tests do not preclude hepatic dysfunction in the Fontan patient, and it is important to perform radiographic assessments as well.
    The Annals of thoracic surgery 07/2011; 92(4):1451-6. · 3.45 Impact Factor
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    ABSTRACT: Octreotide has had limited use for the treatment of protein-losing enteropathy following the Fontan operation. We describe three cases where subcutaneous octreotide was successfully used to treat refractory protein-losing enteropathy following the Fontan operation. Patients received octreotide therapy for a period of 14-28 months. Octreotide was discontinued in one patient due to symptomatic cholelithiasis; this patient died 7 months after treatment was discontinued. Octreotide may be a useful treatment in these patients, but further study on its efficacy and mechanism of action is needed.
    Congenital Heart Disease 05/2011; 6(6):653-6. · 1.01 Impact Factor
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    ABSTRACT: Chronic thromboembolic pulmonary hypertension (CTEPH) occurs in patients with recurrent or chronic pulmonary embolism, and is a rare but potentially devastating disease in adolescents and young adults. Pulmonary thromboendarterectomy (PTE) is an important curative therapy for patients with CTEPH. The importance of this treatment may be under-appreciated and under-utilized in adolescents and young adults. We performed a chart review of all patients <24 years of age who had PTE performed for CTEPH at our institution from 2003-2009 (seven patients, average age 20.2 +/- 2.5 years, range 17-23 years, average BMI 34 +/- 12 kg/m(2)). All patients had their operations performed by a single surgeon. There were no deaths. Three of the seven patients were incorrectly diagnosed prior to the diagnosis of CTEPH. Right ventricular systolic pressure decreased from 61.3 +/- 19 mmHg pre-operatively to 31.8 +/- 5 mmHg post-operatively (P = 0.008). All patients reported symptomatic relief, and no patient has had recurrence of CTEPH. Although rare in the young patient, recurrent pulmonary emboli can have devastating consequences. The potentially lethal implications of CTEPH may be under-appreciated in adolescents and young adults. When indicated, PTE is a definitive, curative procedure with low morbidity and mortality.
    Pediatric Pulmonology 06/2010; 45(6):614-8. · 2.38 Impact Factor
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    The Journal of thoracic and cardiovascular surgery 04/2010; 140(3):e41-3. · 3.41 Impact Factor
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    David J Driscoll
    Clinical Cardiology 10/2009; 32(11):E78-9. · 1.83 Impact Factor
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    ABSTRACT: Vascular malformations occur as a result of an arrest in the development of the vascular system. The modified Hamburg classification distinguishes arterial, venous, arteriovenous, capillary, lymphatic, and mixed vascular malformations. Each malformation is further subdivided based on anatomy and on the time when arrest in development of the embryogenesis occurred; malformations can be truncular or extratruncular. Progress in the last decade in management has been significant because of improvements in open surgical procedures and perfection of percutaneous and hybrid endovascular interventions and devices, such as balloons, stents, and stent-grafts. There has been increasing use of embolization for the treatment of malformations with coils, other particles, glue, or with endovascular placement of occlusive plugs. Absolute alcohol, detergent liquids, or foam have been used for sclerotherapy with improved efficacy. The agents are delivered percutaneously or through a catheter placed either into the feeding arteries or the draining veins. This review aims to aid vascular and endovascular specialists in staying familiar with vascular malformations. These specialists need to be able to evaluate the patients, perform treatment if appropriate, or refer complex cases to multidisciplinary vascular malformation clinics and vascular centers.
    Perspectives in Vascular Surgery and Endovascular Therapy 09/2009; 21(2):133-48.
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    ABSTRACT: To describe our experience with surgical intervention for symptomatic intraarticular vascular malformations of the knee in patients with peripheral vascular malformations including Klippel-Trénaunay syndrome (KTS). Eleven patients underwent surgical intervention for symptomatic intraarticular vascular malformations of the knee between 1987 and 2008. Seven patients had KTS, and 4 patients had venous malformations. Surgical indications, imaging studies, clinical course, surgical procedures, complications, and follow-up visits were reviewed and recorded. A total of 11 patients (8 males; 3 females; mean age, 11.7 years; range, 2.5-23 years) underwent 12 surgical procedures. Five patients had an amputation, and 6 patients had knee synovectomies. One patient had bilateral knee synovectomies. Surgical indications included pain, swelling, limited mobility, and/or loss of knee motion. The average time of follow-up was 54 months (range, 7-109 months). Patient-reported pain scores decreased significantly from a mean of 2.9 +/- 1.4 preoperatively to 1.3 +/- 0.9 postoperatively (P = 0.01). When necessary, surgical intervention for intraarticular vascular malformations of the knee (amputation or synovectomy) may be effective in decreasing pain and improving mobility in patients with peripheral vascular malformations. It is possible that early synovectomy may slow or prevent the rapid destructive arthritis that occurs in these knees. Surgeons and patients should anticipate complications related to bleeding from vascular malformations. We recommend a multidisciplinary approach to the patient with KTS, particularly when surgical intervention is indicated.
    Journal of pediatric orthopedics 07/2009; 29(4):380-4. · 1.23 Impact Factor
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    ABSTRACT: AGGF1 is an angiogenic factor, and its deregulation is associated with a vascular malformation consistent with Klippel-Trenaunay syndrome (KTS). This study defines the molecular mechanism for transcriptional regulation of AGGF1 expression. Transcription of AGGF1 starts at two nearby sites, -367 and -364 bp upstream of the translation start site. Analyses of 5'- and 3'-serial promoter deletions defined the core promoter/regulatory elements, including two repressor sites (from -1971 to -3990 and from -7521 to -8391, respectively) and two activator sites (a GATA1 consensus binding site from -295 to -300 and a second activator site from -129 to -159). Both the GATA1 site and the second activator site are essential for AGGF1 expression. A similar expression profile was found for GATA1 and AGGF1 in cells (including various endothelial cells) and tissues. Electrophoretic mobility shift assay and chromatin immunoprecipitation assays demonstrated that GATA1 was able to bind to the AGGF1 DNA in vitro and in vivo. Overexpression of GATA1 increased expression of AGGF1. We identified one rare polymorphism -294C>T in a sporadic KTS patient, which is located in the GATA1 site, disrupts binding of GATA1 to DNA, and abolishes the GATA1 stimulatory effect on transcription of AGGF1. Knockdown of GATA1 expression by siRNA reduced expression of AGGF1, and resulted in endothelial cell apoptosis and inhibition of endothelial capillary vessel formation and cell migration, which was rescued by purified recombinant human AGGF1 protein. These results demonstrate that GATA1 regulates expression of AGGF1 and reveal a novel role for GATA1 in endothelial cell biology and angiogenesis.
    Journal of Biological Chemistry 06/2009; 284(35):23331-43. · 4.65 Impact Factor

Publication Stats

4k Citations
1,070.86 Total Impact Points


  • 1984–2014
    • Mayo Clinic - Rochester
      • • Department of Pediatric and Adolescent Medicine
      • • Department of Cardiovascular Diseases
      • • Department of Laboratory Medicine & Pathology
      • • Department of Hospital Internal Medicine
      Rochester, Minnesota, United States
  • 2011
    • George Washington University
      • Division of Cardiology
      Washington, D. C., DC, United States
  • 1990–2011
    • Mayo Foundation for Medical Education and Research
      • • Department of Pediatrics
      • • Division of Vascular Surgery
      • • Division of Cardiovascular Diseases
      • • Department of Pediatric and Adolescent Medicine
      • • Department of Pathology
      Scottsdale, AZ, United States
  • 1984–2005
    • University of Minnesota Rochester
      Rochester, Minnesota, United States
  • 2001
    • Lerner Research Institute
      Cleveland, Ohio, United States
  • 1993
    • Johns Hopkins University
      Baltimore, Maryland, United States
    • New York Presbyterian Hospital
      New York City, New York, United States