A binational registry of adults with pulmonary arterial hypertension complicating congenital heart disease.

BACKGROUND: The management of children with congenital heart disease (CHD) has improved over recent decades and several patients surviving with CHD into adulthood are increasing. In developed countries, there are now as many adults as there are children living with CHD. Pulmonary arterial hypertension (PAH) occurs in ∼ 5% of patients with CHD. AIM: We aimed to understand the characteristics and outcomes of this emerging population. METHODS: We collected data retrospectively and prospectively from 12 contributing centres across Australia and New Zealand (2010-2013). Patients were included if they had been diagnosed with PAH and CHD and had been seen once in an adult centre after 1 January 2000. RESULTS: Of 360 patients with CHD-PAH, 60% were female and 90% were New York Heart Association functional class II or III at the time of adult diagnosis of PAH. Mean age at diagnosis of PAH in adulthood was 31.2 ± 14 years, and on average, patients were diagnosed with PAH 6 years after symptom onset. All-cause mortality was 12% at 5 years, 21% at 10 years and 31% at 15 years. One hundred and six patients (30%) experienced 247 hospitalisations during 2936 patient years of follow up. Eighty-nine per cent of patients were prescribed PAH specific therapy (mean exposure of 4.0 years). CONCLUSIONS: Adults with PAH and CHD often have this diagnosis made after significant delay, and have substantial medium-term morbidity and mortality. This suggests a need for children transitioning to adult care with CHD to be closely monitored for this complication.

Deepening the quality of clinical reasoning and decision-making in rural hospital nursing practice.

INTRODUCTION: Rural acute care nursing requires an extensive breadth and depth of knowledge as well as the ability to quickly reason through problems in order to make sound clinical decisions. This reasoning often occurs within an environment that has minimal medical or ancillary support. Registered nurses (RN) new to rural nursing, and employers, have raised concerns about patient safety while new nurses make the transition into rural practice. In addition, feeling unprepared for the rigors of rural hospital nursing practice is a central issue influencing RN recruitment and retention. Understanding how rural RNs reason is a key element for identifying professional development needs and may support recruitment and retention of skilled rural nurses. The purpose of this study was to explore how rural RNs reason through clinical problems as well as to assess the quality of such reasoning. METHODS: This study used a non-traditional approach for data collection. Fifteen rural acute care nurses with varying years of experience working in southern Alberta, Canada, were observed while they provided care to patients of varying acuity within a simulated rural setting. Following the simulation, semi-structured interviews were conducted using a substantive approach to critical thinking. RESULTS: Findings revealed that the ability to engage in deep clinical reasoning varied considerably among participants despite being given the same information under the same circumstances. Furthermore, the number of years of experience did not seem to be directly linked to the ability to engage in sound clinical reasoning. Novice nurses, however, did rely heavily on others in their decision making in order to ensure they were making the right decision. Hence, their relationships with other staff members influenced their ability to engage in clinical reasoning and decision making. In situations where the patient's condition was deteriorating quickly, regardless of years of experience, all of the participants depended on their colleagues when making decisions and reasoning throughout the simulation. CONCLUSIONS: Deep clinical reasoning and decision making is a function of reflection and self-correction that requires a critical self-awareness and is more about how nurses think than what they think. The degree of sophistication in reasoning of experts and novices is at times equivalent in that the reasoning of experts and novices can be somewhat limited and focused primarily on human physicality and less on conceptual knowledge. To become proficient in clinical reasoning, practice is necessary. The study supports the accumulating evidence that using clinical simulation and reflective interviewing that emphasize how clinical decisions are made enhances reasoning skills and confidence.

Congenital heart disease-associated pulmonary arterial hypertension: preliminary results from a novel registry.

BACKGROUND/AIMS: Pulmonary arterial hypertension (PAH) frequently accompanies childhood congenital heart disease (CHD) and may persist into adult life. The advent of specific PAH therapies for PAH prompted formation of a national Australian and New Zealand registry in 2010 to document the incidence, demographics, presentation and outcomes for these patients. METHODS: This multicentre, prospective, web-based registry enrols patients with CHD-associated PAH being followed in a tertiary centre. The inclusion criteria stipulated patient age ≥16 years, a measured mean pulmonary arterial pressure >25 mmHg at rest or echocardiographical evidence of PAH or a diagnosis of Eisenmenger syndrome, and followed since 1 January 2000. A single observer collected standardised data during a series of site visits. RESULTS: Of the first 50 patients enrolled, 30 (60%) were female. The mean age (standard deviation (SD)) at the time of PAH diagnosis or confirmation in an adult centre was 27.23 (10.07) years, and 32 (64%) patients are currently aged >30 years. Fourteen (28%) patients were in World Health Organization Functional Class II and 36 (72%) in Class III at the time of diagnosis. Forty-seven of 50 (94%) had congenital systemic-pulmonary shunts, and 36 (72%) never underwent intervention. Thirteen (26%) had Down syndrome. Confirmation of PAH by recent cardiac catheterisation was available in 30 (60%) subjects. During follow up, a total of 32 (64%) patients received a PAH-specific therapy. CONCLUSIONS: CHD associated with PAH in adult life has resulted in a new population with unique needs. This registry will allow documentation of clinical course and long-term outcomes for these patients.

Aorto-left atrial fistula post-percutaneous device ASD closure.

Use of percutaneous devices for closure of atrial septal defects (ASD) continues to increase owing to relative safety and ease of implementation compared with traditional surgical repair. Complications such as perforation and displacement requiring surgical intervention have been reported. We describe a case of perforation with intracardiac fistula formation, with an underlying mechanism likely to be similar to the few cases previously described, occurring during medium term follow up after ASD device closure. Appropriate case selection can reduce the incidence of this complication with caution taken in ASD cases with deficient aortic and superior rims.

Cardiovascular testing in Fabry disease: exercise capacity reduction, chronotropic incompetence and improved anaerobic threshold after enzyme replacement.

AIM: The aim of this study was to document exercise capacity and serial electrocardiogram and echocardiograph findings in a cohort of Australian patients with Fabry disease, in relation to their history of enzyme replacement therapy (ERT). BACKGROUND: Fabry disease has multifactorial effects on the cardiovascular system. Most previous studies have focused on electrocardiographic and echocardiographic parameters. Exercise capacity can be used as an integrated measure of cardiovascular function and allows the effects of treatment to be monitored. METHODS: A total of 38 patients (30 men and 8 women) with Fabry disease were monitored by 12-lead electrocardiograms every 6-12 months, and by annual standardized-protocol echocardiograms. Bicycle stress tests with VO(2) max measurement and once-only 6 minutes' walk tests were also carried out in subsets of patients whose general health status allowed testing. RESULTS: Seventy per cent of patients met electrocardiogram criteria for left ventricular hypertrophy. Left ventricular hypertrophy on echocardiograph was present in 64% of patients (80% of men). Exercise capacity was reduced in patients with Fabry disease compared with that predicted from normative population data. Mild improvement in anaerobic threshold was seen in the first year of ERT (14.1 +/- 3.0 to 15.8 +/- 3.0, P = 0.02), but no consistent further increase was seen beyond the first year. Most patients had resting bradycardia, with impaired ability to increase heart rate during exercise. Serial testing on ERT showed an improvement in anaerobic threshold but no significant change in VO(2) max. CONCLUSIONS: Male patients with Fabry disease were unable to attain predicted maximal heart rate on exercise or to achieve normal exercise levels. ERT was associated with a small improvement in anaerobic threshold over the first year.

Improvement in door-to-balloon times in management of acute ST-segment elevation myocardial infarction STEMI through the initiation of 'Code AMI'.

BACKGROUND: ST-segment elevation myocardial infarction (STEMI) mandates rapid percutaneous coronary intervention (PCI) for optimal outcomes. The aim of this study was to assess our hospital practice for managing acute STEMI, identify processes associated with time delays, instrument changes to our acute STEMI management protocol and assess their effectiveness for improving our door-to-balloon time. METHODS: We aimed to achieve this through the establishment of a quality improvement programme involving the cardiology and emergency departments. We analysed consecutive patients presenting with STEMI (April to September 2005 (group A) and the corresponding period in 2006 (group B), and compared patients presenting 'in hours' (0700 hours to 1800 hours (Monday to Friday)) versus 'out of hours' (all other times including public holidays). RESULTS: In group A, 38 patients presented with a STEMI. Assessing time-to-treatment analysis, the median door to balloon time for primary PCI was significantly greater for 'out of hours' than 'in hours' (120 vs 67 min). The greatest time delay to PCI was from the PCI decision time to catheter laboratory arrival. Local changes were implemented to improve 'out of hours' times, including initiation of 'Code AMI'. There were 59 patients in group B. We found that our changes led to a 29% improvement in 'out of hours' door-to-balloon time (median time 82 min, P = 0.005) with 69% being managed < or =90 min (P = 0.049) (group B). CONCLUSION: We have shown that ongoing review through a quality improvement programme improves door-to-balloon times, which is integral in the optimal management of patients with acute STEMI treated with primary PCI.

Analysis of clinical outcomes following in-hospital adult cardiac arrest.

AIMS: The outcome of in-hospital resuscitation following cardiac arrest depends on many factors related to the patient, the environment and the extent of resuscitation efforts. The aim of the present study was to determine predictors of successful resuscitation and survival to -hospital discharge following in-hospital cardiac arrest and to assess functional outcomes of survivors (cerebral performance scores). METHODS: Medical records of adult patients sustaining in-hospital cardiac arrest between June 2001 and January 2003 were reviewed. Successful resuscitation was defined as the return of spontaneous circulation at the completion of resuscitative efforts, irrespective of degree of inotropic/vasopressor support. Thirty demographic and clinical variables were analysed to determine predictors of successful resuscitation and in-hospital survival. RESULTS: In 105 patients with cardiac arrest, 46 patients (44%) were successfully resuscitated and 22 (21%) survived to hospital discharge. Predictors of successful resuscitation included a primary cardiac admission diagnosis, monitoring at the time of the arrest, a longer duration of resuscitation and the absence of the need for endotracheal intubation. Patients with ventricular tachycardia/fibrillation were more likely to survive to hospital discharge than those with asystolic or pulseless electrical activity (45 vs 12 vs 20%, P = 0.01). The sole independent predictor of survival to hospital discharge was the absence of the need for endotracheal intubation (odds ratio 0.14, 95% confidence interval 0.02-0.88, P < 0.01). The majority of survivors (73%) had normal cerebral performance scores. CONCLUSIONS: Identification of predictors of successful resuscitation following cardiac arrest is important for risk stratification. Ongoing appraisal of in-hospital cardiac arrests through a multicentre registry could improve clinical outcomes.

Clinical features of Fabry's disease in Australian patients.

BACKGROUND: Anticipating the prospect of specific treatment, we studied a large group of Australians with Fabry's disease. AIMS: We aimed to: (i) document the clinical features of Fabry's disease in Australian patients, (ii) test the hypothesis that clinical features vary with specific mutation and blood group and (iii) assess small-fibre peripheral nerve function. METHODS: A questionnaire was forwarded to all Australian patients known to us. Patients were invited to attend for clinical, renal cardiac, ophthalmological and neurological assessment. RESULTS: Sixty-seven patients (29 men and 38 women) from 18 families participated. Diagnosis in index cases was delayed by > or = 10 years in nearly all families. Common clinical features are: (i) episodic acroparaesthesia (100% of hemizygotes; 53% of heterozygotes), (ii) anhydrosis (93%; 1%), (iii) characteristic rash (93%; 13%), (iv) renal disease (69%; 21%), (v) ischaemic heart disease (28%; 26%), (vi) palpitations (62%; 29%), (vii) mitral valve murmurs (37%; 23%) and (viii) premature cerebrovascular disease (31%; 5%). Ophthalmic findings of cornea verticillata (96%; 76%) and anterior cataract (48%; 14%) were common. Findings were variable within and between families. In women, anhydrosis reliably predicts the presence of significant Fabry's renal disease. Small nerve fibre testing using quantitative sensory testing was clearly abnormal in 95% of male patients, and in those female patients with paraesthesiae. CONCLUSIONS: Symptoms of anhydrosis, acroparaesthesiae, rash and renal disease suggest diagnosis of Fabry's. Women are commonly symptomatic, and the advent of therapy highlights the practical advantage of earlier diagnosis.

Impaired cardiac functional reserve and left ventricular hypertrophy in adult sheep after prenatal dexamethasone exposure.

We have shown that exposure of pregnant ewes to dexamethasone (11.5 mg/d for 2 days) at 27 days of gestation (term, 150 days) led to increased blood pressure and cardiac output in adult offspring. In this study, we hypothesized that dexamethasone-induced hypertension is associated with left ventricular hypertrophy and a reduced cardiac functional reserve (CO(max-0)). Six control animals (group C) and five dexamethasone-exposed animals (group D) were volume-loaded with Hemaccel until the wedge pressure was 13 mm Hg (baseline). The wedge pressure was held constant during an infusion of dobutamine at incremental doses (0.4 to 12 microgram/kg/min) while blood pressure and cardiac output were measured. The same protocol was repeated in each animal 5 days later under mild general anesthesia (1.5% isoflurane), when transthoracic echocardiography (M-mode) was obtained. Group D showed a reduced CO(max-0) in response to dobutamine during both conscious (89+/-22 versus 150+/-25 mL/kg/min in control; P<0.01) and anesthetized states (91+/-38 versus 156+/-56 mL/kg/min in control; P<0.05). Reduced CO(max-0) in group D was associated with higher left ventricular mass index compared with group C (2.6+/-0.67 versus 1.8+/-0.51 g/kg; P<0.05). In addition, group D showed a reduced cardiac contractility reserve (FS(max-0)) in response to dobutamine (21+/-22% versus 54+/-34% in group C; P<0.05). An impaired cardiac functional reserve in group D was associated with increased left ventricular type I collagen content. In conclusion, brief prenatal exposure to dexamethasone led to the development of hypertension, left ventricular hypertrophy, and reduced cardiac functional reserve in adult life.

Physiological relationships between central vascular haemodynamics and left ventricular structure.

Left ventricular hypertrophy is an independent cardiovascular risk factor. In hypertensives, the pattern of hypertrophy is influenced by central haemodynamic characteristics. Central haemodynamics may also determine physiological differences in left ventricular structure and predispose to particular responses of the left ventricle to pathological increases in load. M-mode echocardiography was used to measure left ventricular diastolic dimension and to estimate left ventricular mass index, relative wall thickness and stroke volume in 159 healthy volunteers aged between 19 and 74 years. Tonometric sphygmography was used to estimate augmentation index, central end-systolic and mean arterial blood pressure. Effective arterial elastance was calculated as the ratio of end-systolic pressure to stroke volume. Left ventricular mass index and relative wall thickness were adjusted for variation in age, sex and blood pressure before analyses. Left ventricular diastolic dimension exhibited significant inverse correlations with both effective arterial elastance (r=-0.72, P<0.0001) and augmentation index (r=-0.23, P=0.004). Adjusted left ventricular mass index was inversely correlated with effective arterial elastance (r=-0.35, P<0.0001), but no correlation was observed between left ventricular mass index and augmentation index (r=0.04). Adjusted relative wall thickness correlated with increasing effective arterial elastance (r=0.32, P<0.0001) and augmentation index (r=0.18, P=0.02). Relative wall thickness (r=0.34, P<0.0001), but not left ventricular mass index, correlated with age. Higher elastance and augmentation correlates with relatively smaller left ventricular cavity size but larger relative wall thickness. Age-related changes in left ventricular afterload may affect relative wall thickness more significantly than left ventricular mass index and may contribute to a particular change in left ventricular geometry with age.

Effects of haemoglobin normalization on quality of life and cardiovascular parameters in end-stage renal failure.

BACKGROUND: The optimal haemoglobin concentration ([Hb]) for patients with end-stage renal failure is uncertain. In particular, it is unclear whether Hb normalization may be an advantage to such patients who are otherwise well. METHODS: A prospective, randomized, double-blind cross-over study was completed in 14 haemodialysis patients (12 male) aged between 23 and 65 years over a period of 18 months, using a variety of measures to examine the effect of epoetin at target [Hb] of 10 g/dl ([Hb](10)) and 14 g/dl ([Hb](14)). Patients were randomized to maintain one or other of the target levels for 6 weeks before being crossed over to the alternative [Hb]. Baseline data (mean [Hb]: 8.5+/-0.2 g/dl) were also included selectively. Six patients were known to be hypertensive. Comparisons were made between 24-h ambulatory blood pressure levels (ABP), echocardiographic findings and estimates of blood volume (BV), plasma volume (PV) and Hb mass. Quality of life estimates were obtained using the Sickness Impact Profile (SIP), and epoetin dosage requirements at target [Hb] were assessed. RESULTS: Daytime and nocturnal ABP (systolic and diastolic) were not different at the respective target [Hb], although nocturnal diastolic levels were higher compared with baseline (73+/-4 mmHg) at both [Hb](10) (83+/-3, P:<0.01) and [Hb](14) (81+/-6, P:<0.05). Significant reductions in cardiac output (5.2+/-0.3 vs 6.6+/-0.5 l/min, P:<0.01) and left ventricular end-diastolic diameter (4.8+/-0.2 vs 5.2+/-0.2 cm, P:<0. 001) were found at [Hb](14) compared with [Hb](10). Left ventricular mass index was correlated with both PV (P:<0.001) and BV (P:<0.01), but not with Hb mass. The PV decreased as the [Hb] rose (P:<0.001) but BV remained unchanged. Quality of life was significantly improved at [Hb](14) compared with [Hb](10) for both total score (6. 5+/-1.7 vs 13.4+/-3.0, P:=0.01) and psychosocial dimension score (5. 4+/-1.9 vs 15.4+/-4.0, P:<0.01). The maintenance weekly dose of epoetin required was 80% higher at [Hb](14) compared with [Hb](10) (P:<0.001). CONCLUSION: These data suggest there may be a significant haemodynamic and symptomatic advantage in maintaining a physiological [Hb] in haemodialysis patients. Although untoward effects were not identified in this study at [Hb](14), a substantially higher dose of epoetin is required to maintain this level.

Reduced neuropsychological dysfunction using epiaortic echocardiography and the exclusive Y graft.

BACKGROUND: To examine the effect of screening the aorta for atheroma before aortic manipulation and use of exclusive Y graft revascularization on the incidence of neuropsychological dysfunction after coronary artery bypass. METHODS: Aortic atheroma was detected using epiaortic and transesophageal echocardiography. Atheroma avoidance was facilitated by use of the exclusive Y graft technique, which has no aortic coronary anastomoses. In the control group aortic atheroma was assessed by manual palpation, and we attempted to avoid any atheroma detected. In this group we also used aorta-coronary grafts. Transcranial Doppler imaging of the right middle cerebral artery was used to detect cerebral microemboli. Neuropsychological dysfunction was defined as a 20% or more decline in score for at least 20% of a neuropsychometric battery of ten tests for each patient. RESULTS: Late dysfunction at 57 +/- 2 days postoperatively in the control group was 38.1% and in the echo/Y group was 3.8% (p' = 0.012). Microemboli detected by transcranial Doppler imaging during periods of aortic manipulation was greater for those with late dysfunction (5.2 +/- 3.0 compared with 0.5 +/- 0.2) (p' = 0.018). No clinical strokes occurred in either group. CONCLUSIONS: The combined techniques of epiaortic screening and exclusive Y graft for coronary artery bypass operations resulted in a low incidence of late neuropsychological dysfunction.

Instantaneous end diastolic stiffness (IEDS): a simple, load independent measurement of left ventricular diastolic function in patients undergoing cardiac surgery.

The end-diastolic pressure-volume relation is a load-independent measurement of diastolic function. However, its clinical utility is limited because of its complexity. Instantaneous end-diastolic stiffness (IEDS) is a simple to perform, single-point, measurement of ventricular stiffness. We have validated it against the end-diastolic pressure-area relation (EDPAR) in patients undergoing cardiac surgery. EDPARs were analyzed before and after cardiopulmonary bypass in 29 patients and compared with IEDS. Data was collected in an additional 69 patients in order to estimate the range of values of IEDS. End-diastolic area (EDA) measured by transesophageal echocardiography (TEE) was substituted for end-diastolic volume, and pulmonary capillary wedge pressure (PWCP) was substituted for end-diastolic pressure. IEDS = 100 x (log10 PWCP)/EDA. Comparison of the methods was done by ordinary least products regression analysis. Agreement between EDPAR and IEDS was identified by the absence of fixed and proportional bias. The maximal range of corresponding values identified by 95% confidence intervals was within +/- 16% of the mean indicating satisfactory agreement. The geometric mean and 95% confidence intervals (CI) for IEDS were 8. 7 mmHg/dm2 (8.1 to 9.4) and for IEDS indexed to body surface area were 17.2 mmHg/dm(2)/m2 (16.0 to 18.6). IEDS is a load independent index of left ventricular stiffness.

Increased left ventricular mass is not associated with impaired left ventricular diastolic filling in normal individuals.

BACKGROUND: Hypertensive left ventricular (LV) hypertrophy has been associated with diastolic dysfunction. However, the underlying physiological relationship between LV size and diastolic function remains to be clarified. The aim of this study was to evaluate the relationship between several measures of diastolic filling and LV mass in a population sample. METHODS: We used M-mode and Doppler echocardiography to compare left ventricular mass index (LVMI) and wall thickness with five measures of ventricular diastolic filling (ratio of the peak early mitral inflow velocity to the peak atrial mitral inflow velocity, deceleration time of early mitral inflow, isovolumetric relaxation time, ratio of the peak pulmonary venous systolic to diastolic flow and difference between the durations of the pulmonary venous and mitral inflow atrial waves) in 159 healthy volunteers. RESULTS: LVMI was significantly (P< 0.0001) greater in men (81.3 g/m2, interquartile range: 67-94) than women (59.7 g/m2, interquartile range: 49-74), but no gender differences were observed in diastolic filling. Higher age, blood pressure and heart rate showed significant correlation with diminished diastolic filling. However, no measure of diastolic filling correlated with LVMI or wall thickness in either univariate or multiple regression analyses that adjusted for relevant covariates. CONCLUSIONS: LVMI does not explain physiological differences in diastolic filling. The significant decline in diastolic filling with age reflects changes in the quality rather than the quantity of myocardial tissue.

Postoperative radial artery angiography for coronary artery bypass surgery.

OBJECTIVE: To compare radial artery (RA) patency with internal mammary artery (IMA) patency for coronary artery bypass surgery in our early experience. METHODS: Symptomatic as well as asymptomatic patients with > or =1 RA coronary graft underwent postoperative angiography. Each anastomosis was considered separately. A string sign referred to a diffusely narrowed conduit, which did not fill the grafted coronary artery, as well as all occluded conduits. The raw value of P was adjusted for the testing of multiple hypotheses (P'). The patency data for each conduit was divided into two parts. 'Cut-off' stenosis for a conduit was the lowest dividing coronary stenosis at which a difference in patency rate with P< or =0.05 occurred. RESULTS: One-hundred-and-twenty-nine patients had 137 radial arteries and 157 angiograms. Only the most recent angiogram was analyzed for each patient at 13+/-0.7 months (n=129). Overall patency for arterial conduit 91% (n=404) was not different from venous conduit 91% (n=42) and patency for RA 90% (n=226) was not different from IMA 92% (n=178), (P'=0.999). Cut-off stenosis for RA was 70% and IMA was 40%. Patent arterial conduit had a mean coronary stenosis of 85% and non-patent conduit 64%, (P'<0.001). Right coronary territory patency was 82 vs. 94% for other territories (P'=0.022). No overall differences in patency were noted for patients with sequential anastomoses, symptoms or coronary disease at the anastomosis at the time of surgery. Reversible ischaemia was detected in the distribution of only two of 14 string signs in patients undergoing sestamibi exercise protocol following angiogram. CONCLUSION: There were no differences in patency between radial artery and internal mammary artery at 13 months post-operative. Lower coronary stenosis and right coronary territory predicted lower patency. The clinical importance of a string sign remains to be determined.

Exercise hemodynamic performance of the pulmonary autograft following the Ross procedure.

BACKGROUND AND AIMS OF THE STUDY: The Ross procedure, in which the aortic valve is replaced with the patient's own pulmonary valve (pulmonary autograft), is considered an excellent alternative for younger patients requiring elective aortic valve replacement. Although resting pulmonary autograft hemodynamics are excellent, exercise hemodynamic data are lacking. The study aim was to measure the hemodynamic performance of the pulmonary autograft with exercise Doppler echocardiography (DE). METHODS: Twenty-four Ross procedure patients (20 males, four females; mean age 46 +/- 11 years) were studied at 25 +/- 14 months after aortic valve replacement with a pulmonary autograft. Patients had baseline supine DE to measure the maximum velocity (Vmax), and the peak and mean pressure gradient across the pulmonary autograft. Effective orifice area was calculated from the continuity equation and indexed to body surface area (EOAi). Patients then underwent symptom-limited upright bicycle exercise with supine DE repeated immediately on stopping exercise. For comparison, 10 normal controls (age 41 +/-10 years) and five mechanical aortic valve patients (mean age 55 +/- 10 years) were studied. RESULTS: At rest: Ross procedure patients had similar Vmax (1.2 +/- 0.2 m/s), peak gradient (6 +/- 2 mmHg), mean gradient (4 +/- 1 mmHg) and EOAi (1.7 +/- 0.4 cm2/m2) to those of normal controls. Mechanical-valve patients had significantly higher Vmax (2.5 +/- 0.2 m/s, p <0.001), peak gradient (25 +/- 4 mmHg, p <0.001) and mean gradient (14 +/- 3 mmHg, p <0.001) than Ross patients and normal controls. At exercise: Ross procedure patients had similar Vmax (1.8 +/- 0.4 m/s versus 2.1 +/- 0.2, p = NS), peak gradient (14 +/- 6 mmHg versus 17 +/- 4, p = NS) and mean gradient (8 +/- 4 mmHg versus 10 +/- 2, p = NS) to normal controls, with no significant change in EOAi. Mechanical-valve patients had significantly higher Vmax (3.4 +/- 0.3, p <0.001), peak gradient (48 +/- 7 mmHg, p <0.001) and mean gradient (30 +/- 5 mmHg, p <0.001) than Ross patients and normal controls. CONCLUSIONS: Aortic valve replacement using the Ross procedure provides excellent hemodynamic results at rest and on exercise, with DE parameters indistinguishable from those of normal controls. This study provides further support for the use of the Ross procedure as a preferred method of aortic valve replacement in younger patients.

Mechanical remodeling of the left atrium after loss of atrioventricular synchrony. A long-term study in humans.

BACKGROUND: Tachycardia-mediated mechanical remodeling of the atrium is considered central to the pathogenesis of thromboembolism associated with chronic atrial fibrillation. Whether atrial mechanical remodeling also occurs in response to atrial stretch induced by chronic asynchronous ventricular pacing in patients with permanent pacemakers is unknown. METHODS AND RESULTS: The study design was a prospective randomized comparison between 21 patients paced chronically in the VVI mode and 11 patients paced chronically in the DDD mode for 3 months. Left atrial appendage (LAA) function and the presence of spontaneous echo contrast (SEC) were determined with transesophageal echocardiography (TEE) within 24 hours of pacemaker implantation and after 3 months. The VVI patients were then programmed to DDD and underwent a third TEE after DDD pacing for an additional 3 months. After chronic VVI pacing, LAA velocity decreased from 82.4+/-29.0 to 42.1+/-25.4 cm/s (P<0.01), LAA fractional area change decreased from 74.9+/-17.2% to 49.8+/-22.0% (P<0.01), and 4 patients (19%) developed left atrial SEC (P<0.05). With the reestablishment of chronic AV synchrony, LAA velocity increased to 61.6+/-18.5 cm/s (P<0.01), LAA fractional area change increased to 76.4+/-18.1% (P<0.01), and SEC resolved. In the 11 patients undergoing chronic DDD pacing, no significant changes in LAA velocity (baseline, 86.0+/-28.8 cm/s versus 3 months, 79.6+/-14. 9 cm/s) or LAA fractional area change (baseline, 76.2+/-19.4% versus 72.5+/-15.7%) were demonstrated, and SEC did not develop. CONCLUSIONS: Chronic loss of AV synchrony induced by VVI pacing is associated with mechanical remodeling of the left atrium, which may reverse after the reestablishment of AV synchrony with DDD pacing. This process may be partly responsible for the higher incidence of thromboembolism observed in patients undergoing VVI pacing compared with AV sequential pacing.

Discrepancies between echocardiographic measurements of left ventricular mass in a healthy adult population.

Increased left ventricular (LV) mass is associated with increased cardiovascular morbidity and mortality. LV mass is commonly estimated from echocardiography according to the Penn or ASE (American Society of Echocardiography) conventions. No formal statistical test of agreement between these methods has been published. Therefore we compared M-mode echocardiographic LV mass estimates by the Penn and ASE methods in a normal adult population. M-mode echocardiographic tracings were obtained in 169 healthy volunteers and used to calculate LV mass using the Penn and ASE methods. Median values of the estimates were similar [Penn, 126 g (interquartile range 96-170 g); ASE, 129 g (105-164 g); P=0.08] and were highly intercorrelated (r=0.98, P<0.0001). However, the Bland-Altman analysis of agreement revealed significant inconsistencies between Penn and ASE LV mass values. The difference between Penn and ASE values was correlated significantly with heart size (P<0.0001), such that, for small hearts, the Penn LV mass was lower than the ASE LV mass; in contrast, for large hearts, Penn estimates were greater than ASE values. In the upper 5% of the LV mass distribution, the median value for the Penn LV mass index was 132.4 g/m(2), compared with 116.5 g/m(2) for ASE values (2P=0.017). Thus the two most common methods of echocardiographic estimation of LV mass differ significantly at the upper and lower ends of the heart size distribution. These results have important implications for both cardiac research and clinical evaluation.

Aortic valve area: measurement by transesophageal echocardiography and prediction by left ventricular outflow tract area.

We compared three techniques of aortic valve area (AVA) measurement using transesophageal echocardiography (TEE) and determined if AVA can be predicted from simple patient or echocardiographic measurements. AVA was simultaneously measured with direct planimetry, the continuity equation and with a novel technique combining stroke volume using thermodilution and continuous wave Doppler. Using planimetry as the reference in patients with normal valves, left ventricular outflow tract area (LVOTA), lean body mass (LBM), body surface area (BSA) and height were assessed as predictors of AVA. All three methods of AVA measurement showed close agreement and can be used interchangeably. Both LVOTA and LBM were predictors of AVA, but LVOTA was better. BSA and height were not acceptable as predictors of AVA. TEE can be used to measure AVA either with planimetry, the continuity equation, or in combination with thermodilution. LVOTA was the best predictor of AVA.