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Objective: To explore the use of an episode grouper to more accurately identify the complete set of surgical services typically provided in a surgical episode of care and the corresponding range of prices, using colectomy for cancer as the example., Background: Price transparency is an important policy issue that will require surgeons to better understand the components and cost of care., Methods: This study uses the Episode Grouper for Medicare business logic to construct colectomy surgical episodes of care for cancer using Medicare claims data for the Boston Hospital Referral Region from 2012 to 2015. Descriptive statistics show the mean reimbursement based on patient severity and stage of surgery, along with the number of unique clinicians billing for care and the mix of services provided., Results: The Episode Grouper for Medicare episode grouper identified 3182 colectomies in Boston between 2012 and 2015, with 1607 done for cancer. The mean Medicare allowed amount per case is $29,954 and varies from $26,605 to $36,850 as you move from low to high-severity cases. The intrafacility stage is the most expensive ($23,175 on average) compared with the pre ($780) and post ($6,479) facility stages. There is tremendous heterogeneity in the service mix., Conclusions: Episode groupers are a potentially valuable tool for identifying variations in service mix and teaming patterns that correlate with a total price. By looking at patient care holistically, stakeholders can identify opportunities for price transparency and care redesign that have heretofore been hidden., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: Dystrophic epidermolysis bullosa (DEB) is a devastating condition that causes painful corneal abrasions and vision loss. Epidermolysis Bullosa Eye Disease Index (EB-EDI) for the first time captures and quantifies EB-specific assessment of ocular symptoms and activities of daily living scales. This survey will become critical in developing new interventions on patients' quality of life., Methods: Three-part set of the EB-EDI baseline, EB-EDI interval, and Ocular Surface Disease Index (OSDI) survey was distributed to 92 patients with DEB who previously reported eye symptoms on previous surveys. It was then posted online through several EB patient organizations. We compared the EB-EDI with the gold standard OSDI and examined the repeatability of the EB-EDI over a 7- to 15-day interval., Results: Of the 45 individuals who initially responded, 30 of 45 (67%) completed the surveys sent 7 to 15 days later. The age of participants ranged from 6 to 51 years (mean 21 ± 15 years), and 60% (18 of 30) of participants were younger than 18 years. The overall Cronbach alpha values for the subscales of EB-EDI baseline and interval tools presented a good internal consistency (≥0.7). From 2 visits, the domain scores of EB-EDI baseline (0.94) and interval tools (0.83) were shown to have excellent test-retest reliability (intraclass correlation coefficient >0.8). By comparison, OSDI had the intraclass correlation coefficient score of 0.72 ± 0.11. The convergent validation analysis showed that correlations between the domain scores of EB-EDI baseline and interval tools and the subscales of the OSDI reached the hypothesized strength., Conclusions: Based on a 30-person repeated-measures study, we found that the EB-EDI has excellent reliability and validity specifically in patients with DEB., Competing Interests: V. M. Chen is a consultant for Phoenix Tissue Repair, Inc. The remaining authors have no conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Nurse practitioners (NPs) play a critical role in delivering primary care, particularly to chronically ill elderly. Yet, many NPs practice in poor work environments which may affect patient outcomes., Objective: We investigated the relationship between NP work environments in primary care practices and hospitalizations and emergency department (ED) use among chronically ill elderly., Research Design: We used a cross-sectional design to collect survey data from NPs about their practices. The survey data were merged with Medicare claims data., Subjects: In total, 979 primary care practices employing NPs and delivering care to chronically ill Medicare beneficiaries (n=452,931) from 6 US states were included., Measures: NPs completed the Nurse Practitioner-Primary Care Organizational Climate Questionnaire-a valid and reliable measure for work environment. Data on hospitalizations and ED use was obtained from Medicare claims. We used Cox regression models to estimate risk ratios., Results: After controlling for covariates, we found statistically significant associations between practice-level NP work environment and 3 outcomes: Ambulatory Care Sensitive (ACS) ED visits, all-cause ED visits, and all-cause hospitalizations. With a 1-unit increase in the work environment score, the risk of an ACS-ED visit decreased by 4.4% [risk ratio (RR)=0.956; 99% confidence interval (CI): 0.918-0.995; P=0.004], an ED visit by 3.5% (RR=0.965; 99% CI: 0.933-0.997; P=0.005), and a hospitalization by 4.0% (RR=0.960;99% CI: 0.928-0.993; P=0.002). There was no relationship between NP work environment and ACS hospitalizations., Conclusion: Favorable NP work environments are associated with lower hospital and ED utilization. Practice managers should focus on NP work environments in quality improvement strategies., Competing Interests: The authors declare no conflict of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: While optimal utilization of the nurse practitioner (NP) workforce is an increasingly popular proposal to alleviate the growing primary care shortage, federal, state, and organizational scope of practice policies inhibit NPs from practicing to the full extent of their license and training. In March of 2020, NP state-specific supervisory requirements were temporarily waived to meet the demands of the coronavirus disease 2019 (COVID-19) pandemic in Massachusetts., Objective: The objective of this study was to examine the impact of temporarily waived state practice restrictions on NP perception of care delivery during the initial surge of the COVID-19 pandemic in Massachusetts., Research Design: Mixed methods descriptive analysis of a web-based survey of Massachusetts NPs (N=391), conducted in May and June 2020., Results: The vast majority (75%) of NPs believed the temporary removal of practice restriction did not perceptibly improve clinical work. Psychiatric mental health NPs were significantly more likely than other NP specialties to believe the waiver improved clinical work (odds ratio=6.68, P=0.001). NPs that experienced an increase in working hours during the pandemic surge were also more likely to report a positive effect of the waiver (odds ratio=2.56, P=0.000)., Conclusions: Temporary removal of state-level practice barriers alone is not sufficient to achieve immediate full scope of practice for NPs. The successful implementation of modernized scope of practice laws may require a collective effort to revise organizational and payer policies accordingly., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Although recent research suggests that primary care provided by nurse practitioners costs less than primary care provided by physicians, little is known about underlying drivers of these cost differences., Research Objective: Identify the drivers of cost differences between Medicare beneficiaries attributed to primary care nurse practitioners (PCNPs) and primary care physicians (PCMDs)., Study Design: Cross-sectional cost decomposition analysis using 2009-2010 Medicare administrative claims for beneficiaries attributed to PCNPs and PCMDs with risk stratification to control for beneficiary severity. Cost differences between PCNPs and PCMDs were decomposed into payment, service volume, and service mix within low-risk, moderate-risk and high-risk strata., Results: Overall, the average PCMD cost of care is 34% higher than PCNP care in the low-risk stratum, and 28% and 21% higher in the medium-risk and high-risk stratum. In the low-risk stratum, the difference is comprised of 24% service volume, 6% payment, and 4% service mix. In the high-risk stratum, the difference is composed of 7% service volume, 9% payment, and 4% service mix. The cost difference between PCNP and PCMD attributed beneficiaries is persistent and significant, but narrows as risk increases. Across the strata, PCNPs use fewer and less expensive services than PCMDs. In the low-risk stratum, PCNPs use markedly fewer services than PCMDs., Conclusions: There are differences in the costs of primary care of Medicare beneficiaries provided by nurse practitioners and MDs. Especially in low-risk populations, the lower cost of PCNP provided care is primarily driven by lower service volume., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc.)

: Quality measurement is at the heart of efforts to achieve high-quality surgical and medical care at a lower cost. Without accurate quality measures, it is not possible to appropriately align incentives with quality. The aim of these National Quality Forum (NQF) guidelines is to provide measure developers and other stakeholders with guidance on the standards used by the NQF to evaluate the scientific acceptability of performance measures. Using a methodologically rigorous and transparent process for evaluating health care quality measures is the best insurance that alternative payment plans will truly reward and promote higher quality care. Performance measures need to be credible in order for physicians and hospitals to willingly partner with payers in efforts to improve population outcomes. Our goal in creating this position paper is to promote the transparency of NQF evaluations, improve the quality of performance measurements, and engage surgeons and all other stakeholders to work together to advance the science of performance measurement.

Background: Primary care providers are at the center of the opioid epidemic. Whether nurse practitioners (NPs) have different opioid-prescribing outcomes from physicians is not known., Objective: To examine opioid-prescribing outcomes of Medicare beneficiaries receiving care from NPs and physicians in primary care., Research Design: We used Medicare data from 2009 to 2013 and a propensity score-weighted analysis., Subjects: Beneficiaries residing in states in which NPs are able to prescribe controlled substances without physician oversight and who did not have a cancer diagnosis, hospice care, or end-stage renal disease., Measures: First, we measured whether beneficiaries received any opioid prescription. Second, for beneficiaries who received opioids, we measured acute (<90 d supply) and chronic (≥90 d supply) use at baseline (2009-2010) and follow-up (2012-2013). Third, we measured potential misuse of opioid prescribing using a daily morphine milligram equivalent dose of >100 mg, overlapping prescriptions of opioids >7 days, and overlapping prescriptions of opioids with benzodiazepines >7 days., Results: Beneficiaries managed by NPs were less likely to receive an opioid [odds ratio (OR), 0.87; P<0.001], were less likely to be acute users at baseline (OR, 0.84; P<0.001), and were more likely to receive a high daily opioid dose of morphine milligram equivalent >100 mg compared with physician-managed beneficiaries (OR, 1.11; P=0.048)., Conclusions: Findings suggest educational programs and clinical guidelines may require approaches tailored to different providers. Future research should examine the contributing factors of these patterns to ensure high-quality pain management and guide policy makers on NP-controlled substance-prescribing regulations.

Objective: To examine differences in the quality of care provided by primary care nurse practitioners (PCNPs), primary care physicians (PCMDs), or both clinicians., Data Sources: Medicare part A and part B claims during 2012-2013., Study Design: Retrospective cohort design using standard risk-adjustment methodologies and propensity score weighting assessing 16 claims-based quality measures grouped into 4 domains of primary care: chronic disease management, preventable hospitalizations, adverse outcomes, and cancer screening., Extraction Methods: Continuously enrolled aged, disabled, and dual eligible beneficiaries who received at least 25% of their primary care services from a random sample of PCMDs, PCNPs, or both clinicians., Principal Findings: Beneficiaries attributed to PCNPs had lower hospital admissions, readmissions, inappropriate emergency department use, and low-value imaging for low back pain. Beneficiaries attributed to PCMDs were more likely than those attributed to PCNPs to receive chronic disease management and cancer screenings. Quality of care for beneficiaries jointly attributed to both clinicians generally scored in the middle of the PCNP and PCMD attributed beneficiaries with the exception of cancer screening., Conclusions: The quality of primary care varies by clinician type, with different strengths for PCNPs and PCMDs. These comparative advantages should be considered when determining how to organize primary care to Medicare beneficiaries.

Background: Little is known about the geographic distribution of the overall primary care workforce that includes both physician and nonphysician clinicians--particularly in areas with restrictive nurse practitioner scope-of-practice laws and where there are relatively large numbers of uninsured., Objective: We investigated whether geographic accessibility to primary care clinicians (PCCs) differed across urban and rural areas and across states with more or less restrictive scope-of-practice laws., Research Design: An observational study., Subjects: 2013 Area Health Resource File (AHRF) and US Census Bureau county travel data., Measures: The measures included percentage of the population in low-accessibility, medium-accessibility, and high-accessibility areas; number of geographically accessible primary care physicians (PCMDs), nurse practitioners (PCNPs), and physician assistants (PCPAs) per 100,000 population; and number of uninsured per PCC., Results: We found divergent patterns in the geographic accessibility of PCCs. PCMDs constituted the largest share of the workforce across all settings, but were relatively more concentrated within urban areas. Accessibility to nonphysicians was highest in rural areas: there were more accessible PCNPs per 100,000 population in rural areas of restricted scope-of-practice states (21.4) than in urban areas of full practice states (13.9). Despite having more accessible nonphysician clinicians, rural areas had the largest number of uninsured per PCC in 2012. While less restrictive scope-of-practice states had up to 40% more PCNPs in some areas, we found little evidence of differences in the share of the overall population in low-accessibility areas across scope-of-practice categorizations., Conclusions: Removing restrictive scope-of-practice laws may expand the overall capacity of the primary care workforce, but only modestly in the short run. Additional efforts are needed that recognize the locational tendencies of physicians and nonphysicains.

Objective: This study examined access to and use of neurologists among a broad, national sample of people with multiple sclerosis (MS); identified demographic, economic, and clinical factors associated with access and use; and examined differences in treatment and management of MS., Methods: We used computer-assisted telephone interviews to collect data from 2,156 people with MS on demographics, disease characteristics, and use of neurologists, other specialists, and disease-modifying agents (DMAs). We used chi(2) tests and logistic regressions to compare patients of neurologists and other providers and identify predictor variables and treatment factors associated with seeing neurologists., Results: For their usual MS care, 72.2% of participants saw a neurologist. The probability of seeing a neurologist was significantly lower for people who lacked health insurance, were poor, lived in rural areas, or were African American; had been ill for more than 15 years; had difficulty walking but did not use an assistive device; or required a wheelchair/scooter or were confined to bed. People who reported one to two relapses in the preceding year and women were significantly more likely to see neurologists. Patients of neurologists were significantly more likely to take a DMA, attend an outpatient rehabilitation program, or see an occupational therapist, urologist, or physical therapist., Conclusions: People with multiple sclerosis who see neurologists are more likely than people who see other providers to receive treatment with disease-modifying agents and see rehabilitation specialists and urologists. While some people may choose other providers, economic, insurance, racial, and geographic factors appear to limit access to neurologists.

Background: Multistage surgery culminating in completed hemodynamic repair is now performed for pulmonary atresia with ventricular septal defect (PA-VSD). Justification for operation in patients with an adequate collateral pulmonary circulation is controversial. Data on natural adult survival are scant but are necessary to provide the rationale for multistage reconstructive procedures., Methods and Results: All cyanotic adults with PA-VSD in the UCLA Adult Congenital Heart Disease Center Registry from 1978 through 1992 formed the basis for this study. Registry data and echocardiographic, hemodynamic, and angiographic information were used to determine longevity, clinical course, and operative feasibility. Of 26 patients, 16 were unoperated when referred (group A), and 10 had been palliated before age 18 years (group B). Two thirds were 18 to 29 years old. Only 2 patients survived beyond age 40 years. Six died during follow-up at a mean age of 31 years (+/- 12.1 SD). Eight group A patients were in New York Heart Association class II, and 8 were in class III. Of the 26 group A and B patients, 20 had aortic regurgitation, which was moderate or severe in 10. Eight had cardiac failure. Of 11 group A patients who remained unoperated, 5 died. Twelve patients were considered eligible for surgery at > or = 18 years of age. Ten underwent completed hemodynamic repair with a mean post-operative right ventricular-to-left ventricular systolic pressure ratio of 0.45 (+/- 0.16 SD), and there were no early or late deaths., Conclusions: Even when collateral blood flow permits adult survival, all such patients are symptomatic. Mean life expectancy without operation did not exceed three decades. Aortic regurgitation and cardiac failure are significant negative variables. Nearly half of unoperated adults died during follow-up. Staged hemodynamic repair can be performed with a low surgical risk in properly selected adults with PA/VSD.

Background: Adults with cyanotic congenital heart disease and elevated hematocrit levels are often phlebotomized because of an assumed risk of cerebral arterial thrombotic stroke. Whether a relation exists between hematocrit level, symptomatic erythrocytosis (hyperviscosity), and stroke remains to be established in this patient population., Methods and Results: Accordingly, 112 cyanotic patients 19-74 years old (mean, 36 +/- 11.7 years) in the UCLA Adult Congenital Heart Disease Center Registry were selected for study by virtue of continuous observation for 1-12 years (total, 748 patient-years). Patients with independent risk factors for embolic or vasospastic stroke were excluded. The study patients were then divided into two groups: 1) compensated erythrocytosis (stable hematocrit levels of 46.0-72.7% [mean, 57.5 +/- 7.2%], iron replete, absent or mild hyperviscosity symptoms), and 2) decompensated erythrocytosis (unstable rising hematocrit levels of 61.5-75.0% [mean, 69.5 +/- 10.6%], iron deficiency, marked-to-severe hyperviscosity symptoms). No patient with either compensated or decompensated erythrocytosis, irrespective of hematocrit level, iron stores, or the presence, degree, or recurrence of cerebral hyperviscosity symptoms, progressed to clinical evidence of a complete stroke (cerebral arterial thrombosis with brain infarction)., Conclusions: Because a risk of stroke caused by cerebral arterial thrombosis was not demonstrated, because the circulatory effects of phlebotomy are transient, and because of the untoward sequelae of phlebotomy-induced iron deficiency, we recommend phlebotomy for the temporary relief of significant, intrusive hyperviscosity symptoms but not for the hematocrit level per se. According to our data, phlebotomy is not warranted to reduce an assumed risk of stroke because that risk did not materialize.

In adults with congenital heart disease who are confronted with noncardiac surgery, perioperative risks can be reduced, often appreciably, when problems inherent to this patient population are anticipated. The first necessity is to clarify the diagnosis and to be certain that appropriate information is obtained from a cardiologist with adequate knowledge of congenital heart disease in adults. Physiology and anatomy can vary significantly among patients who superficially carry identical diagnoses and would seem to fit under the same rubric. Elective noncardiac surgery should be preceded by clinical cardiovascular assessment, including reviewing clinical and laboratory data and securing necessary diagnostic studies. Preoperative assessment should be performed far enough in advance of the anticipated surgery to allow for critical assessment of the data. Appropriate cardiovascular laboratory studies to be obtained or reviewed include electrocardiograms, chest radiographs, echocardiograms, and cardiac catheterization data (which may include specialized intracardiac electrophysiologic testing). Congenital heart disease in adults is a new and evolving area of special interest and expertise in cardiovascular medicine. Multidisciplinary centers for the care of these patients are being developed. The 22nd Bethesda Conference recommended that these centers include anesthesiologists with special expertise in managing patients with congenital heart disease among their consultants. These anesthesiologists can function either as attending anesthesiologists when patients require noncardiac surgery, or as consultants and resource individuals to other anesthesiologists. Adults with congenital heart disease may present with age-related acquired cardiovascular and noncardiovascular disorders in addition to postoperative cardiac residua and sequelae, all of which require meticulous preoperative planning and consultation before noncardiac surgery is performed. We recommend that, particularly in larger departments of anesthesiology, select members of the department serve as consultants to anesthesiologists and to other members of the medical staff for these cases, especially when large numbers of adults with congenital heart disease are to be treated.

Isolated noncompaction of left ventricular myocardium is a rare disorder of endomyocardial morphogenesis characterized by numerous, excessively prominent ventricular trabeculations and deep intertrabecular recesses. This study comprised eight cases, including three at necropsy. Ages ranged from 11 months to 22.5 years, with follow-up as long as 5 years. Gross morphological severity ranged from moderately abnormal ventricular trabeculations to profoundly abnormal, loosely compacted trabeculations. Echocardiographic images were diagnostic and corresponded to the morphological appearances at necropsy. The depths of the intertrabecular recesses were assessed by a quantitative echocardiographic X-to-Y ratio and were significantly greater than in normal control subjects (p less than 0.001). Clinical manifestations of the disorder included depressed left ventricular systolic function in five patients, ventricular arrhythmias in five, systemic embolization in three, distinctive facial dysmorphism in three, and familial recurrence in four patients. We conclude that isolated noncompaction of left ventricular myocardium is a rare if not unique disorder with characteristic morphological features that can be identified by two-dimensional echocardiography. The incidence of cardiovascular complications is high. The disorder may be associated with facial dysmorphism and familial recurrence.

This report deals with the ramifications of the concept of left axis deviation. In early life, the leftward shift of the frontal plane QRS axis is determined chiefly, if not solely, by the relative weights of the ventricles. Once adult ventricular weight ratios are reached, there is a long period of axis stability, then a gradual leftward drift of the QRS, governed principally by left anterior fascicular conduction. Thus, the normal QRS axis is age-dependent, and left axis deviation must be considered accordingly.

Electrophysiologic characteristics of five patients with Ebstein's anomaly of the tricuspid valve were defined with studies using luminal intracardiac electrode catheters. The diagnosis was made in each case from clinical data and confirmed at cardiac catheterization by the presence of an atrialized right ventricular chamber with atrial mechanical activity and ventricular electrical activity. In three cases intra-right atrial conduction was prolonged (P-A intervals of 50, 50, and 65 msec), a finding which reflected the presence of a characteristically large right atrium. The bundle of His electrogram was recorded in its usual anatomical location. Atrioventricular nodal conduction was prolonged in only one case. Intra-His delay was observed in two cases (bundle of His duration of 30 and 30 msec). Infranodal conduction was prolonged in four cases with H-V intervals of 60, 65, 65, and 80 msec. The anatomical abnormalities were least severe in the only patient with a normal H-V interval (50 msec). The prolonged H-V interval was thought to result from stretching of the conduction system over the atrialized right ventricle (ARV). The late depolarization during the splintered R' of the electrocardiogram found during intracardiac mapping of the ARV in three patients confirms the theory that the ARV produces the "second QRS" typically seen in this anomaly. The ARV was particularly irritable, and ventricular fibrillation was produced in two patients during catheter manipulation in this area. In one case the ARV had a shorter refractory period than the body of the right ventricle. Re-entrant supraventricular tachycardia was induced in the only patient with Wolff-Parkinson-White syndrome. In addition to the previously recognized electrophysiologic features reconfirmed here, patients with Ebstein's anomaly of the tricuspid valve usually have: normal position of the bundle of His, prolonged intra- right atrial conduction, prolonged infranodal conduction, and irritable ARV with delayed activation.

We postulated that the abnormal shape, size, and function of the right heart and adjoining ventricular septum in adults with Ebstein's anomaly of the tricuspid valve might in turn alter the shape and function of the left ventricle. Seven adult patients with uncomplicated Ebstein's anomaly were studied. Left ventricular geometry was determined by two-dimensional echocardiography. Left ventricular function was assessed by treadmill exercise and radionuclide angiography at rest and with exercise. Paradoxic ventricular septal motion was consistently present. Left ventricular eccentricity (ratio of two minor axes in the short-axis view) was uniformly abnormal, averaging 1.35 +/- 0.23 (normal = 1.02 +/- 0.05). The ratio of right to left ventricular cavity size averaged 1.70 +/- 0.44 (normal 0.65 +/- 0.30), and tricuspid valve displacement into the right ventricular cavity averaged 52% (normal 8%). Functional right atrial size averaged 27.6 +/- 5.2 cm2 (normal right atrial area = 13.1 +/- 2.2 cm2). Resting left ventricular ejection fractions were below 50% in all but two patients. In response to Bruce protocol exercise stress, there were consistently appropriate increments in heart rate, blood pressure, and peak double product and, with one exception, radionuclide left ventricular ejection fraction. There were significant correlations between tricuspid valve displacement and functional right atrial size versus resting left ventricular ejection fraction and left ventricular eccentricity. These data support the hypothesis that derangements in right heart morphology and function in Ebstein's anomaly contribute to significant alterations in left ventricular geometry, but the geometric alterations are associated with tangible but less significant changes in left ventricular systolic function.

Diminished glomerular filtration rate, proteinuria, and large hypercellular congested glomeruli with segmental sclerosis are found in late survivors with cyanotic congenital heart disease (CCHD). Hyperuricemia is common, acute gouty arthritis is less common than uric acid levels would predict, and overt tophaceous deposits of uric acid are exceptional. The role of the kidney in causing the basic biochemical disturbances, and the relative importance of impaired urate excretion vs urate overproduction have not been established. Accordingly, we reviewed the courses of two index patients and prospectively studied eight additional CCHD patients from 28 years to 46 years old with mean hematocrits of (62 +/- 10%). Plasma creatinine concentration was normal (0.9 +/- 0.1 mg/dl) yet glomerular filtration rate was mildly reduced to 93 +/- 14 ml/min as measured by creatinine clearance and to 81 +/- 6 ml/min as measured by 111In DTPA. Three patients had significant proteinuria and one was nephrotic. Plasma uric acid concentration was high in all but one (8.2 +/- 2.1 mg/dl), mean 24 hr uric acid excretion was normal (564 +/- 221 mg), and fractional uric acid excretion was relatively low (6.3 +/- 2.6%). The two patients with highest plasma uric acid levels (12.0 and 10.2 mg/dl) had the lowest fractional excretions (2.8% and 4.0%). Both of these patients had diminished capacity to excrete a water load (38% and 27%/4 hr) and to maximally concentrate urine (520 and 635 mOsm/kg after water deprivation and vasopressin). In conclusion, high plasma uric acid levels in late survivors with CCHD are secondary to inappropriately low fractional uric acid excretion, not to urate overproduction.(ABSTRACT TRUNCATED AT 250 WORDS)

Despite increasing use of Fontan or modified Fontan repairs, the comparative hemodynamic efficacy of different types of connections are unresolved. Accordingly, we undertook a prospective study designed to determine postoperative flow patterns after Fontan's operation. Seven subjects had tricuspid atresia and eight had single ventricle. Ages ranged from 5 to 38 years (mean 16.4). Ten subjects had nonvalved right atrial-to-pulmonary arterial connection, and four had nonvalved right atrial-to-right ventricular communication. A valved conduit established continuity between the right atrium and right ventricle in one subject. Doppler flow profiles were recorded in the pulmonary artery and in the superior and inferior venae cavae of each. A reference electrocardiogram was used for timing purposes. In 14 patients, forward flow in the pulmonary artery was biphasic. Flow began at the end of the T wave (early ventricular diastole), peaked at or before the P wave (atrial systole), and returned to baseline by the peak of the R wave. Forward flow recommenced at the peak of the R wave (ventricular systole) and returned to baseline at the end of the T wave. Flow in the superior vena cava varied, and could not be recorded in three subjects. Between the end of the P wave and peak of the R wave (atrial systole) flow was reversed in eight, absent in three, and forward in one patient. Forward flow occurred between the peak of the R wave and the end of the T wave and was either continuous or biphasic. Fourteen patients had adequate studies of inferior vena cava flow; reversed flow during atrial systole occurred in 10 subjects.(ABSTRACT TRUNCATED AT 250 WORDS)

Studies at necropsy have shown that the cardiomyopathy of Duchenne muscular dystrophy selects the posterobasal and contiguous lateral left ventricular (LV) walls as initial and primary sites of myocardial dystrophy in the absence of small-vessel coronary artery disease in these areas. The present investigation was designed chiefly to determine whether a myocardial metabolic abnormality could be identified in these same areas during a patient's life. Positron emission computed tomography was used to study regional LV metabolism with 18F 2-fluorodeoxyglucose, and metabolism and/or perfusion was studied with 13NH3. In addition, all subjects had the following performed: thallium-201 scans, technetium-99m multiple-gated equilibrium blood pool imaging, electrocardiograms, vectorcardiograms, and M mode and two-dimensional echocardiograms. 18F 2-fluorodeoxyglucose activity was selectively increased in the posterobasal and posterolateral walls of the left ventricle in 11 of 12 patients with technically adequate images, indicating accelerated regional exogenous glucose utilization. 13NH3 activity was selectively decreased in the same areas in 13 of 15 patients, indicating either a regional metabolic alteration in uptake and trapping, a reduction in regional blood flow, or both. These data identify a myocardial metabolic abnormality concentrated in specific segments of the LV free wall in living patients with Duchenne dystrophy.

For half a century the systolic click and late systolic murmur lay dormant as innocent auscultatory curiosities. The thirteen years since Barlow related these phenomena to mitral leaflet prolapse have witnessed an astonishing information explosion. We have sought to bring together the accumulated data in this review. An Historical Perspective traces the evolution from the now abandoned "pericardial" or "extracardiac" phases, through the leafletchordal phase (redundancy), the myocardial phase (segmental left ventricular contraction abnormalities), to the anular phase (dilatation and faulty systolic contraction). Functional Anatomy is dealt with in terms of pathology, pathophysiology, hemodynamics, angiocardiography, echocardiography, and physical and pharmacological interventions. Clinical Manifestations are concerned with prevalence, natural history, symptoms, physical signs, electrocardiographic abnormalities and roentgen fingings. The four Major Complications- sudden death, infective endocarditis, spontaneous rupture of chordae tendineae, and progressive mitral regurgitation- are examined. Associated Cardiac Diseases, i.e., Marfan's syndrome, ostium secundum atrial septal defect and atherosclerotic coronary artery disease, are discussed, and a section on Treatment deals chiefly with prophylaxis for infective endocarditis and the management of arrhythmias and chest pain. A final section on Evolving Information considers etiologic concepts, the nature of left ventricular contration abnormalities, the cause of chest pain, the relationship to Marfan's syndrome and ostium secundum atrial septal defect, and the effect of aging and sex differences on leaflet chordal redundancy.

The dynamic increase in oxygen uptake (VO2) at the start of exercise reflects the circulatory adjustments to metabolic changes induced by the exercise. Because VO2 measured at the lungs is the product of pulmonary blood flow and arteriovenous oxygen difference, pathologic conditions affecting the capacity of these factors to change would be expected to alter VO2 kinetics. To determine whether measurement of VO2 kinetics can detect conditions in which the pulmonary blood flow response to exercise is abnormal, VO2 was measured, breath-by-breath, during the transition from rest to exercise in 13 adults with cyanotic congenital heart disease (central venoarterial shunting) and in nine normal subjects. The increase in VO2 above baseline during the first 20 sec of exercise (phase I), reflecting the immediate increase in pulmonary blood flow, was diminished in the patients compared with that in normal subjects (14.8 +/- 10.9 vs. 49.8 +/- 19.2 ml of oxygen) (p less than .001). The patients' phase I responses correlated with their reported physical activity tolerance (p less than .01). In addition, the second phase of the VO2 response kinetics was prolonged in patients compared with normal subjects (half-time = 63 +/- 13 vs 15 +/- 13 sec) (p less than .001). We conclude that striking disturbances in VO2 kinetics occur in patients with cyanotic congenital heart disease and that these measurements provide a useful noninvasive means of evaluating the degree to which the increase in pulmonary blood flow is constrained in response to exercise.