Joseph W. Turek

Detecting pheochromocytoma: defining the most sensitive test.

Objective:To define the most sensitive biochemical test to establish the diagnosis of pheochromocytoma and also to assess the potential role of iodine 131-labeled metaiodobenzylguanidine scintigraphy (131I-MIBG) in the diagnosis of this tumor. Summary Background Data:Pheochromocytoma is a rare, catecholamine-producing tumor with preferential localization in the adrenal gland. Despite its importance, the most sensitive test to establish the diagnosis remains to be defined. Methods:Prospective data collection was done on patients with pheochromocytoma treated at the Duke University Medical Center and the Durham Veterans Affairs Medical Center, Durham, NC. All urinary, plasma, and platelet analyses were highly standardized and supervised by one investigator (J.M.F.). 131I-MIBG scans were independently reviewed by 2 nuclear medicine physicians. Results:A total of 152 patients (55.3% female) were enrolled in the present analysis. Patients were predominantly white (73.7%). Spells (defined as profuse sweating, tachycardia, and headache) and hypertension at diagnosis were present in 51.4% and 66.6%, respectively. Bilateral disease was found in 12.5%, malignant pheochromocytoma in 29.6%, and hereditary forms in 23.0%. The most sensitive tests were total urinary normetanephrine (96.9%), platelet norepinephrine (93.8%), and 131I-MIBG scintigraphy (83.7%). In combination with 131I-MIBG scintigraphy, platelet norepinephrine had a sensitivity of 100%, plasma norepinephrine/MIBG of 97.1%, total urine normetanephrine/MIBG of 96.6%, and urine norepinephrine/MIBG of 95.3%. Conclusions:The tests of choice to establish the diagnosis of pheochromocytoma are urinary normetanephrine and platelet norepinephrine. A combination of 131I-MIBG scintigraphy and diagnostic tests in urine, blood, or platelets does further improve the sensitivity. We thus advocate performing an MIBG scan if the diagnosis of pheochromocytoma is clinically suspected and catecholamine measurements are within the normal range.

Report of the 2010 society of thoracic surgeons congenital heart surgery practice and manpower survey.

BACKGROUND The Society of Thoracic Surgeons (STS) Workforce on Congenital Heart Surgery undertook a second subspecialty Practice and Manpower Survey (2005, 2010) to obtain contemporary data. METHODS Preliminary research suggested a potential target group of 273 congenital heart surgeons, including 258 at 125 US centers and 15 at 8 Canadian centers. The web-based survey was sent to these surgeons, plus all individuals listing pediatric cardiac surgery on CTSNet. RESULTS Two hundred forty-six responders included 213 active congenital heart surgeons, 16 retired congenital heart surgeons, and 17 surgeons in training. Retirement age was 63.5±7.5 years. Two hundred thirteen active congenital heart surgeon responders represent 78% of the original estimate. Their responses generated the following data: The mean age was 49.2±8.5 (range, 35 to 75 years). American medical school graduates included 159 of 201 respondents (79%). Years of postgraduate training was 9.7±1.7. One hundred ninety-seven (92%) respondents were certified in thoracic surgery by the American Board of Thoracic Surgery (ABTS). Twenty-eight of 200 (14%) received their congenital heart surgery training outside the United States or Canada. One hundred forty-three of 190 respondents (75%) perform exclusively congenital heart operations; 31 (16%) perform exclusively pediatric heart operations. Of 186 respondents, 54 (29%) perform fewer than 100 major congenital heart operations per year, 78 (42%) perform 100 to 199 procedures, and 54 (29%) perform 200 procedures or more. Active congenital heart surgeons have been in their current positions for 9.3±8.6 years. Eight respondents are in their first year of practice. For 203 respondents, mean anticipated years to retirement is 16.1±7.6. Twenty-eight anticipate retirement within 5 years; 31 in 6 to 10 years. CONCLUSIONS These data should help facilitate rational plans to meet workforce needs for an expanding patient population.

Anomalous Aortic Origin of a Coronary Artery: Preoperative Diagnosis and Surgical Planning

Background: Anomalous aortic origin of a coronary artery (AAOCA), the anomalous coronary artery arises from an inappropriate coronary sinus and travels between the aorta and pulmonary artery. Proper surgical management depends upon correct diagnosis and accurate characterization of the origin and course of the coronary artery. Transthoracic echocardiography (TTE) has been the mainstay for diagnosis, but magnetic resonance imaging (MRI) and computed tomographic angiography (CTA) have been increasingly utilized. In this study, we report the largest series of surgically repaired AAOCA and accuracy of preoperative diagnostic studies. Methods: A review of 53 consecutive patients (mean age 13.9 years, range 4-65 years) undergoing repair of an AAOCA from 1995 to 2009 was performed. In all, 40 patients were identified with an anomalous right coronary artery (ARCA) from the left sinus of Valsalva, 13 patients had an anomalous left coronary artery (ALCA) arising from the opposite sinus. Symptoms of angina or syncope were present in 58% and 46% of cases with ARCA and ALCA, respectively. Results of preoperative diagnostic testing were compared to actual surgical findings to determine the accuracy of the tests. Results: Lack of an intramural course was observed intraoperatively in 7 cases (5 ARCA and 2 ALCA). Preoperative TTE accurately predicted whether the AAOCA was intramural or extramural in 49 (92.5%) of 53 cases. Magnetic resonance imaging was predictive in 5 (83.3%) of 6 patients and CTA in 11 (64.7%) of 17. Survival was 100%. Complications occurred in 4 (7.5%) of 53 patients (mean follow-up 29 months). Patency was confirmed in 97.7% with TTE, and 23 (95.8%) of 24 patients had a negative postoperative functional study. Conclusions: Transthoracic echocardiography was found to be very accurate at defining the presence or absence of an intramural course in AAOCA. Both MRI and CTA can provide additional information but may not be as accurate as TTE.

The Correction Index: Setting the Standard for Recommending Operative Repair of Pectus Excavatum

BACKGROUND The Haller index, derived from a chest computed tomography scan, remains the standard for determining candidacy for pectus excavatum repair (Haller index≥3.25). However, it has been suggested that this index may not accurately reflect pectus severity in patients with abnormal chest wall morphologies. This study explores a new, more appropriate criteria for recommending repair based on a correction index, while still incorporating the standard set by the Haller index. METHODS A database of 75 patients with pectus excavatum who received computed tomography scans was compiled. For each patient, a staff radiologist calculated the Haller index, a correction index, and an ideal chest index from the computed tomography image with the greatest sternal depression. A correlation was assessed between all Haller and correction indices and separately for those with standard and nonstandard chest dimensions as assessed by an ideal chest index. RESULTS There was a modest correlation between the Haller and correction indices measured in this cohort (r=0.79; p<0.0001). When patients with aberrant ideal chest dimensions were removed from analysis, Haller and correction indices showed a stronger correlation (r=0.86; p<0.0001). CONCLUSIONS The correction index provides an accurate assessment of pectus severity, and by the nature of the measurement, reflects the potential degree of operative repair. The Haller index correlates well with the correction index in pectus patients with standard chest wall dimensions, but is quite discrepant in the nonstandard chest. We recommend operative repair for pectus excavatum with a correction index of 28% or more, because this value correlates with the long-accepted standard (Haller index≥3.25) and this index remains accurate even in nonstandard chest morphologies.

Outcomes Before and After Implementation of a Pediatric Rapid-Response Extracorporeal Membrane Oxygenation Program

BACKGROUND Rapid-response extracorporeal membrane oxygenation (RR-ECMO) has been implemented at select centers to expedite cannulation for patients placed on ECMO during extracorporeal cardiopulmonary resuscitation (ECPR). In 2008, we established such a program and used it for all pediatric venoarterial ECMO initiations. This study was designed to compare outcomes before and after program implementation. METHODS Between 2003 and 2011, 144 pediatric patients were placed on venoarterial ECMO. Records of patients placed on ECMO before (17 ECPR and 62 non-ECPR) or after (14 ECPR and 51 non-ECPR) RR-ECMO program implementation were retrospectively compared. RESULTS The peak performance of the ECMO team was assessed by measuring ECMO initiation times for the ECPR patient subgroup (n = 31). There was a shift toward more ECPR initiations achieved in less than 40 minutes (24% pre-RR-ECMO versus 43% RR-ECMO; p = 0.25) and fewer requiring more than 60 minutes (47% pre-RR-ECMO versus 21% RR-ECMO; p = 0.14) after program implementation, although these changes did not reach statistical significance. After multivariable risk adjustment, RR-ECMO was associated with a 52% reduction in neurologic complications for all patients (adjusted odds ratio, 0.48; 95% confidence interval, 0.23 to 0.98; p = 0.04), but the risk of in-hospital death remained unchanged (adjusted odds ratio, 0.99; 95% confidence interval, 0.50 to 1.99; p = 0.99). CONCLUSIONS Implementation of a pediatric RR-ECMO program for venoarterial ECMO initiation was associated with reduced neurologic complications but not improved survival during the first 3 years of program implementation. These data suggest that development of a coordinated system for rapid ECMO deployment may benefit both ECPR and non-ECPR patients, but further efforts are required to improve survival.

Norwood Reconstruction Using Continuous Coronary Perfusion: A Safe and Translatable Technique

BACKGROUND Continuous coronary perfusion during Norwood reconstruction offers the theoretic advantage of less postoperative cardiac dysfunction. The avoidance of a cardiac and circulatory arrest period allows time for a more deliberate aortic reconstruction while the heart remains beating. This single-center study was designed to compare patient results using this method vs standard cardiac arrest for Norwood reconstruction. METHODS A retrospective review was done of 32 patients undergoing Norwood reconstruction from November 2004 to July 2011. The operations in the most recent 16 consecutive patients were performed under deep hypothermia with constant coronary and cerebral perfusion. Continuous coronary perfusion was provided by a cannula inserted into the proximal aorta. The operations in the prior 16 consecutive patients were performed using deep hypothermia, selective cerebral perfusion, and cardioplegic arrest during aortic reconstruction. RESULTS Survival in the beating-heart group was 87.5% (14 of 16) vs 62.5% (10 of 16) in the standard group (p = 0.22). No patients in the beating-heart group required extracorporeal membrane oxygenation vs 3 in the standard group. Postoperative cardiac function was similar for both groups. The beating-heart cohort had lower peak lactate levels (8.2 mEq/L) than the standard group (10.7 mEq/L, p = 0.022). CONCLUSIONS This study presents the largest series of Norwood operations in which the entire aorta is augmented while delivering continuous coronary perfusion. The technique is applicable to any size aorta and represents a safe alternative because outcomes for survival, freedom from extracorporeal membrane oxygenation, postoperative cardiac function, and lactate levels were all noninferior compared with the standard technique.

Impact of Oropharyngeal Dysphagia on Long-Term Outcomes of Lung Transplantation

BACKGROUND Lung transplantation, definitive therapy for end-stage lung disease, is limited long-term by allograft dysfunction including bronchiolitis obliterans syndrome (BOS). Few modifiable risk factors for pulmonary transplant-related mortality are recognized. However, oropharyngeal dysphagia frequently occurs after thoracic surgical procedures, including lung transplantation, and increases morbidity. We evaluated the impact of oropharyngeal dysphagia on survival and BOS after lung transplantation. METHODS A total of 263 consecutive lung transplant patients were reviewed. Each underwent clinical swallowing evaluation early after surgery; 149 patients underwent additional fiberoptic or videofluoroscopic swallowing evaluation (SE). Results of SE were correlated with BOS, defined by accepted criteria, and mortality using Kaplan-Meier survival curves. Cox proportional hazard modeling assessed preoperative and postoperative variables associated with development of BOS and mortality. RESULTS Mean follow-up was 920 ± 560 days. The SE identified tracheal aspiration and (or) laryngeal penetration in 70.5%. Preoperative tobacco abuse, gastroesophageal reflux, and cardiopulmonary bypass independently predicted oropharyngeal dysphagia. Peak FEV(1) (forced expiratory volume in the first second of expiration) alone independently predicted BOS (hazard ratio 0.98; confidence interval 0.975 to 0.992, p < 0.0001); oropharyngeal dysphagia was not associated with BOS. Independent predictors of mortality by multivariable analysis were ventilator dependence (p = 0.038) and peak FEV(1) (p < 0.0001); normal SE was associated with improved survival (hazard ratio 0.13; confidence interval 0.03 to 0.54, p = 0.03). CONCLUSIONS Oropharyngeal dysphagia, often overlooked on clinical examination, is common after lung transplantation. Normal deglutition may improve survival after lung transplantation, but oropharyngeal dysphagia does not independently affect BOS. Institution of protocols aimed at identifying previously unrecognized dysphagia may improve results of pulmonary transplantation.

The case for using the correction index obtained from chest radiography for evaluation of pectus excavatum

BACKGROUND We previously reported the use of a computed tomography (CT)-based Correction Index (CI) as a more accurate assessment of pectus excavatum (PE) severity than the historically used Haller Index (HI). This study examines the diagnostic capabilities of the CI as assessed by lateral chest radiography (CXR). METHODS A database of PE patients receiving preoperative CXR and CT was created. For each patient, a radiologist calculated a CT-based CI, while two pediatric surgeons independently calculated CXR CIs. RESULTS The database was composed of 69 patients. Significant correlations were found between CXR CI estimates of the two observers and between the CXR and CT CI for each observer. Per our previous work, CT CIs were used in this study for identifying patients meeting surgical criteria (CT CI≥28%). Observed CXR CIs demonstrated good interrater reliability. The sensitivity (0.83) and specificity (0.77) of CXR in diagnosing severe PE (CT CI≥28%) was high. However, sensitivity (0.89) markedly improved when only considering measured CXR CIs≤26%, and combined specificity rose to 0.86 when only considering measured CXR CIs≥30%. CONCLUSIONS We recommend the CI as measured by lateral CXR for the preoperative evaluation of PE, with CT used as a confirmatory test in patients measured to have a CXR CI between 26% and 30%.

Endovascular Treatment of a Traumatic Thoracic Aortic Injury in an Eight-Year Old Patient: Case Report and Review of Literature

Traumatic aortic injuries in children and adolescents are rare. Although endovascular repair has become the preferred approach for such injuries in adults, open repair has endured as the gold standard in children owing mainly to the smaller aortic and access vessel diameter and the scarcity of long-term follow-up data. We report a successful endovascular repair of a traumatic thoracic aortic injury in an 8-year-old girl using a Zenith Alpha thoracic endograft (Cook Medical, Bloomington, IN). We also review the literature on endovascular treatment of traumatic aortic injuries in the pediatric population.

Pentalogy of Cantrell with Ectopia Cordis Totalis, Total Anomalous Pulmonary Venous Connection, and Tetralogy of Fallot: A Case Report and Review of the Literature

Pentalogy of Cantrell is a rare condition with a varied expression and a high mortality. We present a patient with the classic pentad (type 1), but with a previously undescribed constellation of cardiac manifestations including ectopia cordis totalis, total anomalous pulmonary venous return, and tetralogy of Fallot. This case reminds us of the challenges associated with the management of various forms of this condition. We discuss the prenatal diagnosis, genetic basis, postnatal evaluation, and management of this entity.