Dartmouth General Hospital

In the area of patient safety, recent attention has focused on diagnostic error. The reduction of diagnostic error is an important goal because of its associated morbidity and potential preventability. A critical subset of diagnostic errors arises through cognitive errors, especially those associated with failures in perception, failed heuristics, and biases; collectively, these have been referred to as cognitive dispositions to respond (CDRs). Historically, models of decision-making have given insufficient attention to the contribution of such biases, and there has been a prevailing pessimism against improving cognitive performance through debiasing techniques. Recent work has catalogued the major cognitive biases in medicine; the author lists these and describes a number of strategies for reducing them ("cognitive debiasing"). Principle among them is metacognition, a reflective approach to problem solving that involves stepping back from the immediate problem to examine and reflect on the thinking process. Further research effort should be directed at a full and complete description and analysis of CDRs in the context of medicine and the development of techniques for avoiding their associated adverse outcomes. Considerable potential exists for reducing cognitive diagnostic errors with this approach. The author provides an extensive list of CDRs and a list of strategies to reduce diagnostic errors.

BACKGROUND: Capsular contracture is a common complication associated with the use of breast implants. Numerous randomized controlled trials addressing the efficacy of textured surface breast implants in reducing capsular contracture have yielded nonuniform results. This meta-analysis addresses the use of textured breast implants in the prevention of capsular contracture. METHODS: MEDLINE, EMBASE, and the Cochrane Central Register of Controlled Trials databases were searched to identify all randomized controlled trials involving the use of textured versus smooth breast implants. The results of these trials were meta-analyzed to obtain a pooled odds ratio of the effect of textured surfacing on capsular contracture rates. In addition, subgroup analyses were performed based on implant type (saline or silicone gel), type of surface texturing (Siltex or Biocell), placement (subglandular or submuscular), and length of follow-up. RESULTS: Eleven trials were reviewed. Four were excluded because they failed to meet a priori inclusion criteria. The remaining seven trials were meta-analyzed. Only three of these studies found significantly lower rates of capsular contracture with the use of textured implants. However, when all seven studies were pooled, the odds ratio was found to be 0.19 (95 percent confidence interval, 0.07 to 0.52), indicating a protective effect for surface texturing on the rate of capsular contracture. Submuscular placement was the only subgroup in which significance was not achieved. However, this subgroup consisted of a single study, which was dramatically underpowered. CONCLUSION: The results of this meta-analysis demonstrate the superiority of textured over smooth breast implants in decreasing the rate of capsular contracture.

Forty-five cases of cardiac angiosarcomas were reviewed, and the data were compared with those of a 1968 review of 41 other cases which revealed these tumors to be typically located within the right atrium as large symptomatic masses and to be rapidly fatal, with the diagnoses usually determined only at autopsy. The relationship of these tumors to Kaposi's sarcoma was also examined. The findings paralleled those of the previous review. Additionally, the following points emerged: With the aid of newer imaging techniques, localization, biopsy diagnosis and resection of the atrial tumors are now being achieved more often, with some improvement in survival. Like angiosarcomas of other organs, atrial angiosarcomas exhibit highly variable histologic patterns, which often overlap those of Kaposi's sarcoma, and may also present metastatic patterns simulating widespread Kaposi's sarcoma or malignant melanoma. In reported cases of classical, endemic, or epidemic Kaposi's sarcoma, cardiac lesions are uncommon and typically are small, asymptomatic, restricted to the epicardium/or pericardium and, thus, are clearly different, both clinically and pathologically, from the atrial tumor group. The justification for designating cases of the latter group as "primary cardiac Kaposi's sarcoma" is open to debate. A case report illustrates many of the above points.

The 2015 update of the Canadian Stroke Best Practice Recommendations Hyperacute Stroke Care guideline highlights key elements involved in the initial assessment, stabilization, and treatment of patients with transient ischemic attack (TIA), ischemic stroke, intracerebral hemorrhage, subarachnoid hemorrhage, and acute venous sinus thrombosis. The most notable change in this 5th edition is the addition of new recommendations for the use of endovascular therapy for patients with acute ischemic stroke and proximal intracranial arterial occlusion. This includes an overview of the infrastructure and resources required for stroke centers that will provide endovascular therapy as well as regional structures needed to ensure that all patients with acute ischemic stroke that are eligible for endovascular therapy will be able to access this newly approved therapy; recommendations for hyperacute brain and enhanced vascular imaging using computed tomography angiography and computed tomography perfusion; patient selection criteria based on the five trials of endovascular therapy published in early 2015, and performance metric targets for important time-points involved in endovascular therapy, including computed tomography-to-groin puncture and computed tomography-to-reperfusion times. Other updates in this guideline include recommendations for improved time efficiencies for all aspects of hyperacute stroke care with a movement toward a new median target door-to-needle time of 30 min, with the 90th percentile being 60 min. A stronger emphasis is placed on increasing public awareness of stroke with the recent launch of the Heart and Stroke Foundation of Canada FAST signs of stroke campaign; reinforcing the public need to seek immediate medical attention by calling 911; further engagement of paramedics in the prehospital phase with prehospital notification to the receiving emergency department, as well as the stroke team, including neuroradiology; updates to the triage and same-day assessment of patients with transient ischemic attack; updates to blood pressure recommendations for the hyperacute phase of care for ischemic stroke, intracerebral hemorrhage, and subarachnoid hemorrhage. The goal of these recommendations and supporting materials is to improve efficiencies and minimize the absolute time lapse between stroke symptom onset and reperfusion therapy, which in turn leads to better outcomes and potentially shorter recovery times.

Clinical decision making (CDM) describes a form of qualitative inquiry that examines the thought processes involved in making medical decisions. A significant body of literature exists on the orderly "hypothetico-deductive" model of clinical decision making. However, very little has been written on how CDM differs in the acute setting. This paper reviews the common methods of CDM and their relevance to emergency medicine (EM). The concept of diagnostic uncertainty and the utility of the diagnosis of unknown etiology in the disposition phase of the emergency patient visit are discussed. Finally, a unique EM perspective on clinical decision-making errors is presented.

BACKGROUND: Joint arthrodesis employing autogenous bone graft (autograft) remains a mainstay in the treatment of many foot and ankle problems. However, graft harvest can lead to perioperative morbidity and increased cost. We tested the hypothesis that purified recombinant human platelet-derived growth factor-BB (rhPDGF-BB) homodimer combined with an osteoconductive matrix (beta-tricalcium phosphate [β-TCP]) would be a safe and effective alternative to autograft. METHODS: A total of 434 patients were enrolled in thirty-seven clinical sites across North America in a prospective, randomized (2:1), controlled, non-inferiority clinical trial to compare the safety and efficacy of the combination rhPDGF-BB and β-TCP with those of autograft in patients requiring hindfoot or ankle arthrodesis. Radiographic, clinical, functional, and quality-of-life end points were assessed through fifty-two weeks postoperatively. RESULTS: Two hundred and sixty patients (394 joints) underwent arthrodesis with use of rhPDGF-BB/β-TCP. One hundred and thirty-seven patients (203 joints) underwent arthrodesis with use of autograft. With regard to the primary end point, 159 patients (61.2% [262 joints (66.5%)]) in the rhPDGF-BB/β-TCP group and eighty-five patients (62.0% [127 joints (62.6%)]) in the autograft group were fused as determined by computed tomography at six months (p < 0.05). Clinically, 224 patients (86.2%) [348 joints (88.3%)]) in the rhPDGF-BB/β-TCP group were considered healed at fifty-two weeks, compared with 120 patients (87.6% [177 joints (87.2%)] in the autograft group (p = 0.008). Overall, fourteen of sixteen secondary end points at twenty-four weeks and fifteen of sixteen secondary end points at fifty-two weeks demonstrated statistical non-inferiority between the groups, and patients in the rhPDGF-BB/β-TCP group were found to have less pain and an improved safety profile. CONCLUSIONS: In patients requiring hindfoot or ankle arthrodesis, treatment with rhPDGF-BB/β-TCP resulted in comparable fusion rates, less pain, and fewer side effects as compared with treatment with autograft.

BACKGROUND: Treatment of fractures is sometimes performed after normal daytime operating hours and in such instances may be performed under less than ideal conditions. The consequence of performing operations under such conditions is largely unknown and was therefore studied in the context of intramedullary nail fixation of tibial and femoral shaft fractures. METHODS: Two hundred and three consecutive patients with either a femoral or tibial shaft fracture (Orthopaedic Trauma Association classification 32 or 42) treated with intramedullary nail fixation were included in a prospective, multicenter, nonrandomized study. Patients were divided into an after-hours group defined as an operation beginning from 4:00 P.M. to 6:00 A.M. or a daytime group defined as an operation beginning from 6:00 A.M. to 4:00 P.M. These groups were further divided on the basis of the injured bone into the following subgroups: after-hours femoral fracture (fifty-five patients), daytime femoral fracture (forty-four patients), after-hours tibial fracture (forty-eight patients), and daytime tibial fracture (fifty-six patients). The demographic and fracture characteristics were similar among the subgroups. All patients were treated with the same type of femoral antegrade, femoral retrograde, or tibial nail fixation with reaming. Data for fracture-healing, complications, operative time, and fluoroscopy time were collected prospectively. RESULTS: The healing rates were similar between daytime and after-hours surgery groups for both the tibial and femoral nailing. On the basis of univariate analysis, operative times were shorter in the after-hours group compared with the daytime group for both the tibial and femoral nail fixation groups (p < 0.02), but regression analysis failed to identify time of surgery as an independent variable associated with operative time. Radiation exposure was similar for the after-hours group and the daytime group for both tibial and femoral nail fixation (p > 0.05). The after-hours group had more unplanned reoperations than the daytime group (p < 0.02). Removal of painful hardware was more frequent in the after-hours femoral fracture group (27%) than in the daytime femoral fracture group (3%) (p < 0.02), and after-hours surgery was an independent variable associated with the need for removal of painful femoral fracture hardware (p < 0.05). CONCLUSIONS: Rates of nonunion, infectious complications, and radiation exposure are similar for after-hours and daytime surgery for intramedullary nail fixation of both femoral and tibial fractures. After-hours femoral nail fixation was associated with an increased frequency for removal of painful hardware, which may be related to technical errors associated with nonideal conditions and shorter operative times. An increase in the allocated amount of daytime operative time for orthopaedic trauma surgery has the potential to reduce minor complication rates for intramedullary nail fixation.

The emergency department (ED) is a complex environment. Its equilibrium, or homeostasis, is critically dependent on the continuous action of feedback processes. For any system to function efficiently, it needs to know the outcomes of specific actions in a consistent, reliable, and expeditious way. Historical attitudes and the unique operating characteristics of the ED have combined to impose sanctions on the proper provision of feedback. The following features have been identified as obstructive to optimal feedback operation: incomplete awareness of the significance of the problem, excessive time and work pressures, case infrequency, deficiencies in specialty follow-up, communication failures, deficient reporting systems for near-misses, error, and adverse events, biases in case review processes, shift changeover times, and shiftwork. The result is that clinicians, nurses, and trainees are working in conditions that are suboptimal for the provision of safe care, as well as for learning and job fulfillment. Good feedback is a necessary condition for well-calibrated performance by individuals, and is integral to effective team function. More needs to be known about outcomes for feedback to work efficiently. The critical role of feedback in other aspects of ED function, such as education and human factors engineering, should be emphasized. The current interest in medical error and evolving attitudes toward a new culture of patient safety provide a unique opportunity to examine feedback and the critical role it plays in ED function.

Although previous studies have suggested a depression of myofibrillar ATPase in the presence of heart failure, their interpretation has been limited by failure to exclude mitochondrial contamination and lack of information regarding the contractility of the tissue studied. To explore this fundamental question, myofibrils were prepared from the right and left ventricles of 15 normal cats, 10 cats with right ventricular hypertrophy and 19 with right ventricular hypertrophy and right ventricular failure, the latter groups following graded pulmonary artery stenosis. The contractility of these hearts was assessed using their right ventricular papillary muscles isolated in a myograph. Myofibrillar ATPase in all groups was maximally activated by 5 mM ATP, 5 mM MgCl 2 at pH 7.0 and 37°C, while mitochondrial contamination was eliminated by sodium azide. In right ventricular failure, right ventricular myofibrillar ATPase was depressed by 39% from an average of 0.18±0.01 in normal cats to 0.11±0.01 µmole inorganic phosphate/mg protein per min (P&lt;.001). In right ventricular hypertrophy, right ventricular myofibrillar ATPase was not significantly depressed (0.16±0.01 µmole/mg per min). Of note, left ventricular myofibrillar ATPase was also significantly (P&lt;.05) depressed in right ventricular failure from 0.16±0.01 to 0.13±0.01 µmole/mg per min. Contractility of the associated right ventricular papillary muscles, expressed as maximum rate of force development at the apex of the length-active tension curve, was correlated with myofibrillar ATPase activity.

A 59-year-old man presented to the emergency department (ED) with the chief complaint of “panic attacks.” In total, he was evaluated by 14 faculty physicians, 2 fellows, and 16 residents from emergency medicine, cardiology, neurology, psychiatry, and internal medicine. These multiple transitions were responsible, in part, for the perpetuation of a failure to accurately diagnose the patient's underlying medical illness. The case illustrates the discontinuity of care that occurs at transitions, which may threaten the safety and quality of patient care. Considerable effort must be directed at making transitions effective and safe. Recommendations to improve transitions include a heightened awareness of cognitive biases operating at these vulnerable times, improving team situational awareness and communication, and exploring systems to facilitate effective transfer of relevant data.

BACKGROUND: Fistulas occur in about 25% of patients with Crohn's disease (CD) and can be difficult to treat. The aim of this consensus was to provide guidance for the management of patients with perianal fistulizing CD. METHODS: A systematic literature search identified studies on the management of fistulizing CD. The quality of evidence and strength of recommendations were rated according to the Grading of Recommendation Assessment, Development and Evaluation (GRADE) approach. Statements were developed through an iterative online platform using a modified Delphi process, then finalized, and voted on by a group of specialists. RESULTS: The quality of evidence for treatment of fistulizing CD was generally of very low quality, and because of the scarcity of good randomized controlled trials (RCTs), these consensus statements generally provide conditional suggestions (5 of 7 statements). Imaging and surgical consultations were recommended in the initial assessment of patients with active fistulizing CD, particularly those with complicated disease. Antibiotic therapy is useful for initial symptom control. Antitumor necrosis factor (anti-TNF) therapy was recommended to induce symptomatic response, and continued use was suggested to achieve and maintain complete remission. The use of concomitant immunosuppressant therapies may be useful to optimize pharmacokinetic parameters when initiating anti-TNF therapy. When there has been an inadequate symptomatic response to medical management strategies, surgical therapy may provide effective fistula healing for some patients. CONCLUSIONS: Optimal management of perianal fistulizing CD requires a collaborative effort between gastroenterologists and surgeons and may include the evidence-based use of existing therapies, as well as surgical assessments and interventions when needed.

OBJECTIVES: Antegrade femoral nailing through a piriformis fossa starting point in patients who are obese has been demonstrated to be problematic. Retrograde femoral nailing therefore has been advocated in this patient population, but little data exist to support such a recommendation. The purpose of this study was to evaluate and compare antegrade and retrograde femoral nailing technique in both patients who are and are not obese. DESIGN: Prospective, multicenter, nonrandomized, internal review board (IRB)-approved study. SETTING: Four Level 1 trauma centers. PATIENTS: Patients (151) with a femoral shaft fracture (OTA 32) treated with intramedullary nailing were studied. Thirty-two with a body mass index (BMI) of >or=30 comprised the obese group (OG), and 119 with a BMI of <30 comprised the nonobese group (NOG). Antegrade nailing was performed in 15 patients from the OG and 84 from the NOG. Retrograde nailing was performed in 17 patients from the OG and 35 from the NOG. INTERVENTION: Reamed intramedullary nailing of a femoral shaft fracture. MAIN OUTCOME MEASURES: Patient and fracture characteristics, operative time, fluoroscopy time, healing, complications, and functional outcome based on the lower extremity measure (LEM) were evaluated. RESULTS: Antegrade technique in the OG was associated with a 52% greater average operative time (94 minutes) compared with antegrade nailing in the NOG (62 minutes; P < 0.003). For retrograde nailing technique, there was no difference in the average operative time between the OG (67 minutes) and NOG (62 minutes; P = 0.51). Antegrade technique in the OG was associated with a 79% greater average radiation exposure time (247 seconds) compared with antegrade nailing in the NOG (135 seconds; P < 0.03). For retrograde nailing technique, average fluoroscopy time was similar between the OG (76 seconds) and the NOG (63 seconds; P = 0.44). Within the OG, antegrade nailing required 40% greater average operative time (94 minutes versus 67 minutes, P < 0.02) and more than 3 times more average fluoroscopy time (242 seconds versus 76 seconds, P < 0.002) than retrograde nailing. Thirty-eight patients from the original cohort were not available for follow-up. Of the 113 patients followed (average 9 months, range: 4 to 25 months), healing complications occurred similarly between the 2 groups, with 1 nonunion and 2 delayed unions in the OG (12%), and 3 nonunions and 9 delayed unions in the NOG (14%). CONCLUSIONS: This study provides evidence, in the form of decreased operative and radiation exposure times, to support the use of retrograde nailing technique for the treatment of femoral shaft fractures in patients who are obese. Also, antegrade nailing was found to require significantly more operative and radiation exposure time in the patient who is obese as opposed to the patients who is not obese. Although having similar baseline functional scores, patients who are obese recovered at a slower rate and more incompletely than patients who are not obese.

The acute blockade of spinal glycinergic inhibition with intrathecal strychnine (i.t. STR; a glycine antagonist) in rats induces a change in somatosensory processing which is very similar to the sensory dysesthesia of clinical neural injury pain. In the present study, the effects of i.t. STR were examined in urethane-anesthetized rats. Noxious paw pinch (PP) or tail immersion (TI) in 55 degree C water evoked a pronounced pressor response, increased heart rate (HR) and desynchronized the electroencephalogram; a non-noxious, hair deflection (HD) elicited only minor cardiovascular responses. After i.t. STR (40 micrograms), an identical HD stimulus evoked markedly enhanced cardiovascular responses, resembling those evoked by noxious stimuli, and a HD-evoked motor withdrawal was observed. Consistent STR-dependent responses were only observed if a light plane of anesthesia was maintained for the duration of the experiment. The effects of i.t. STR were dose-dependent and reversible, lasting 15-30 min. Spinal morphine (50 micrograms) completely abolished the cardiovascular responses to PP and TI, but the HD-evoked, STR-dependent cardiovascular and motor withdrawal responses remained unchanged. In contrast, the non-selective excitatory amino acid antagonist, gamma-D-glutamylglycine (DGG; 50 micrograms) was effective in suppressing both the STR-dependent cardiovascular and motor withdrawal responses. These data suggest that STR-dependent responses evoked by non-noxious stimuli are mediated by mechanisms distinct from those of conventional noxious stimuli and that i.t. STR may be useful for investigating the spinal pharmacology of somatosensory processing following the loss of spinal glycinergic inhibition.

This is a re-appraisal of the supposedly rare Mallory-Weiss syndrome in which 11 patients with mucosal tears at the oesophagogastric junction are described. The fact that these cases were collected from general hospitals within a short period suggests that the condition is more common than supposed and may account for a considerable proportion of the 20 to 25% of patients with upper gastrointestinal bleeding in whom no radiological abnormality can subsequently be found. Of the 11 patients, eight presented with gastrointestinal bleeding, two with mediastinitis, and one without relevant symptoms. The classical history of antecedent vomiting before the bleeding was obtained in only four patients, its absence not excluding the diagnosis. The presence of a small hiatal hernia in four patients appeared to predispose to mucosal tears as did mucosal atrophy occurring with advancing age. Some experimental findings pertaining to the mechanism of the tears are presented.

Medication errors are frequent in the emergency department (ED). The unique operating characteristics of the ED may exacerbate their rate and severity. They are associated with variable clinical outcomes that range from inconsequential to death. Fifteen adult and pediatric cases are described here to illustrate a variety of errors. They may occur at any of the previously described five stages, from ordering a medication to its delivery. A sixth stage has been added to emphasize the final part of the medication administration process in the ED, drawing attention to considerations that should be made for patients being discharged home. The capability for dispensing medication, without surveillance by a pharmacist, provides an error-producing condition to which physicians and nurses should be especially vigilant. Except in very limited and defined situations, physicians should not administer medications. Adherence to defined roles would reduce the team communication errors that are a common theme in the cases described here.

Chronic obstructive pulmonary disease (COPD) will be the third leading cause of death worldwide by 2020. The burdens of this increasingly prevalent illness borne by patients, their family caregivers and the healthcare system are substantial. Dyspnoea as the predominant symptom becomes increasingly difficult to palliate as COPD progresses through advanced stages and, for 50% of patients, can become refractory to conventional treatment. This narrative review focuses on the potential role for carefully initiated and titrated opioids in the management of dyspnoea for patients with advanced COPD who are not yet in a terminal stage, yet struggle with symptoms that reflect underlying mechanisms of dyspnoea that lend themselves to this approach. The many barriers that currently exist to the provision of opioids in this setting are addressed, and recommendations are provided for an approach that should engender confidence among patients, their caregivers and the physicians who treat them.

INTRODUCTION: Provoked vestibulodynia (PVD) is a prevalent vulvovaginal pain condition that is triggered primarily during sexual intercourse. PVD adversely impacts women's and their partners' sexual relationship and psychological well-being. Over 80% of women with PVD continue to have intercourse, possibly because of sexual goals that include wanting to pursue desirable outcomes (i.e., approach goals; such as a desire to maintain intimacy) and avoid negative outcomes (i.e., avoidance goals; such as avoiding a partner's disappointment). AIM: The aim of this study was to investigate associations between approach and avoidance sexual goals and women's pain, as well as the sexual, relational, and psychological well-being of affected couples. METHODS: Women with PVD (N = 107) and their partners completed measures of sexual goals, sexual satisfaction, relationship satisfaction, and depression. Women also completed measures of pain during intercourse and sexual functioning. MAIN OUTCOME MEASURES: (1) Global Measure of Sexual Satisfaction Scale, (2) Dyadic Adjustment Scale-Revised or the Couple Satisfaction Index, (3) Beck Depression Inventory-II, (4) numerical rating scale of pain during intercourse, and (5) Female Sexual Function Index. RESULTS: When women reported higher avoidance sexual goals, they reported lower sexual and relationship satisfaction, and higher levels of depressive symptoms. In addition, when partners of women reported higher avoidance sexual goals, they reported lower relationship satisfaction. When women reported higher approach sexual goals, they also reported higher sexual and relationship satisfaction. CONCLUSIONS: Targeting approach and avoidance sexual goals could enhance the quality and efficacy of psychological couple interventions for women with PVD and their partners.

Titanium and titanium alloys remain the gold standard for dental and orthopedic implants. These materials are heavily used because of their bioinert nature, robust mechanical properties, and seamless integration with bone. However, implant-associated infections (IAIs) remain one of the leading causes of implant failure. Eradicating an IAI can be difficult since bacteria can form biofilms on the medical implant, protecting the bacterial cells against systemic antibiotics and the host's immune system. If the infection is not treated promptly and aggressively, device failure is inevitable, leading to costly multi-step revision surgeries. To circumvent this dire situation, scientists and engineers continue to develop novel strategies to protect the surface of medical implants from bacteria. In this review, details on emerging strategies to prevent infection in titanium implants are reported. These strategies include anti-adhesion properties provided by polymers, superhydrophobic, superhydrophilic, and liquid-infused surface coatings, as well as strategies and coatings employed to lyse the bacteria. Additionally, commercially available technologies and those under preclinical trials are examined while discussing current and future trends.

Considerable efforts have been placed on the development of degradable microspheres for use in transarterial embolization indications. Using the guidance of the U.S. Food and Drug Administration (FDA) special controls document for the preclinical evaluation of vascular embolization devices, this review consolidates all relevant data pertaining to novel degradable microsphere technologies for bland embolization into a single reference. This review emphasizes intended use, chemical composition, degradative mechanisms, and pre-clinical safety, efficacy, and performance, while summarizing the key advantages and disadvantages for each degradable technology that is currently under development for transarterial embolization. This review is intended to provide an inclusive reference for clinicians that may facilitate an understanding of clinical and technical concepts related to this field of interventional radiology. For materials scientists, this review highlights innovative devices and current evaluation methodologies (i.e., preclinical models), and is designed to be instructive in the development of innovative/new technologies and evaluation methodologies.

Echocardiographic abnormalities in patients with sickle cell disease (SCD) were determined, and pulmonary arterial systolic pressure (PASP) was estimated. Clinical data and echocardiograms of 38 adult hospitalized patients with SCD at two tertiary care hospitals were reviewed. Fisher's exact test was performed to determine correlation between pulmonary hypertension and various clinical variables. Pulmonary hypertension was the most common abnormality identified in 22 (58%) patients. The estimated mean PASP was 37.5 +/- 10.9 mmHg. Older age and prior history of acute chest syndrome were significantly correlated with an increased prevalence of pulmonary hypertension (P < 0.05). Patients with hemoglobin levels <8 g/dL had PASP 43.2 +/- 0.5 compared to a mean PASP of 33.3 +/- 6.0 in patients with hemoglobin > or =8 g/dL (P = 0.01). Eight (21%) patients had evidence of a hyperdynamic left ventricle. Left heart abnormalities included dilated atrium in 14 (37%), dilated ventricle in 5 (13%), ventricle hypertrophy in 5 (13%), and ventricle dysfunction in 3 (9%) patients. Right heart abnormalities included dilated atrium in 9 (24%), dilated ventricle in 6 (16%), and ventricle dysfunction in 3 (9%) patients. Despite an increased incidence of abnormal flow across the valves on Doppler analysis, no patient had structurally abnormal valves. A majority of patients with SCD had evidence of pulmonary hypertension, which correlated with older age and history of acute chest syndrome. Other structural and functional echocardiographic abnormalities were less common.