Subclinical Aortic Inflammation in Patients with Polymyalgia Rheumatica.

OBJECTIVES: To examine the clinicopathologic features of patients with polymyalgia rheumatica (PMR) who had thoracic aorta repair surgery. Findings were compared with those of a cohort of patients with giant cell arteritis (GCA) requiring thoracic aorta repair. METHODS: All patients evaluated at Mayo Clinic in Rochester, MN, with Current Procedural Terminology (CPT) codes for thoracic aorta repair surgery between 2000- 2021 were identified. All patients were screened for prior PMR diagnosis. Patients with PMR and no signs of GCA were categorized as clinically isolated PMR. The medical records of all patients were manually reviewed, and pathologists re-examined all the aortic tissues. RESULTS: Of the 4621 patients with at least one CPT code for thoracic aorta repair surgery, 43 patients were diagnosed with clinically isolated PMR before the surgery. Detailed histopathological examination of the aortic tissues revealed active inflammation in 30/43 (70%) patients after a median (IQR) of 10.0 (4.7- 13.3) years from the PMR diagnosis. When compared with aortic tissue from patients with a prior diagnosis of GCA, the aorta of patients with PMR had more severe inflammation (Grade 3: 15/30 [50%] vs 5/34 [15%], p= 0.002). Patients with PMR and thoracic aorta repair may experience a 40% increased risk of mortality compared with the general population, but this did not reach statistical significance (standardized mortality ratio: 1.40; 95% CI: 0.91- 2.07). CONCLUSIONS: Some patients with PMR have subclinical aortic inflammation that is detectable many years after initial diagnosis and may contribute to the development of aortic aneurysm.

Single-Center Retrospective Comparison of Opioid-Based and Multimodal Analgesic Regimens in Adult Cardiac Surgery.

OBJECTIVES: To compare the outcomes of 2 multimodal analgesic regimens with an opioid-based one. DESIGN: A 2-stage, retrospective study. SETTING: A large tertiary-care facility. PARTICIPANTS: Adult cardiac surgical patients. INTERVENTIONS: Patients received one of three regimens: opioid-only or 2 multimodal regimens. The opioid regimen included intraoperative fentanyl and patient-controlled analgesia pumps. Multimodal regimen 1 included preoperative extended-release oxycodone, intraoperative ketamine infusion, and postoperative morphine suppository. Multimodal regimen 2 included intraoperative methadone and dexmedetomidine infusion. MEASUREMENTS AND MAIN RESULTS: Outcomes measured included opioid use, pain scores, time to tracheal extubation, postoperative antiemetic use as a surrogate marker for postoperative nausea and vomiting (PONV), age, sex, surgical procedure(s), body mass index, time to first bowel movement, intensive care unit length of stay (LOS), and hospital LOS. Intraoperative median oral morphine equivalents (OMEs) declined from 425 mg (314, 518) to 150 mg (75, 150) and 230 mg (160, 240), p < 0.001, in multimodal regimens 1 and 2, respectively, compared with the opioid-only regimen. Predischarge opioid use was reduced from a median OME of 7.5 mg (0, 22.5) to 5 mg (0, 22.5) and 0 mg (0, 15.0), p < 0.001, in multimodal regimens 1 and 2, respectively. Pain scores were reduced in the multimodal regimen 2 for hours 0 to 6 (estimated difference = -1.5, 95% CI -1.8 to -1.2, p < 0.001) compared with the opioid-only regimen. The PONV treatment was reduced in multimodal regimen 1 versus the opioid-based or multimodal regimen 2 (53% v 64% and 62%), and time to tracheal extubation was clinically equivalent across all regimens: 4.2 (2.8, 6.0), 3.6 (2.3, 5.7), and (3.0, 6.2) hours for the opioid and multimodal regimens 1 and 2, respectively. CONCLUSIONS: Multimodal analgesic regimens, particularly when incorporating methadone and dexmedetomidine, significantly reduced total and predischarge opioid use in cardiac surgical patients.

Hypertrophic Obstructive Cardiomyopathy: Discrepancy Between Hemodynamic Measurements in the Cardiac Laboratory and Operating Room Is to Be Expected.

OBJECTIVES: It is not uncommon to observe some discrepancy in hemodynamic values characterizing left ventricular outflow tract (LVOT) obstruction preoperatively and in the operating room in patients with hypertrophic obstructive cardiomyopathy. Interpretation of this discrepancy can be challenging. To clarify the extent of the discrepancy, the authors compared hemodynamic variables in patients undergoing septal myectomy at the time of preoperative and intraoperative evaluation. DESIGN: Retrospective study. SETTING: Single academic medical center. INTERVENTIONS: Medical records review, study group-173 patients. MEASUREMENTS AND MAIN RESULTS: While there was no statistically significant difference in resting peak LVOT gradients by preoperative transthoracic echocardiography (TTE) compared to intraoperative transesophageal echocardiography (46 mmHg [19-87 mmHg] v 36 mmHg [16-71 mmHg], p = 0.231), the former were higher compared to direct needle-resting LVOT gradient measurements before myectomy (49 mmHg [19-88 mmHg] v 32 mmHg [14-67 mmHg], p = 0.0022). The prevalence of systolic anterior motion was high (94.6% v 91.6%, P = 1.000) both on pre- and intraoperative evaluation. The incidence of moderate/severe mitral was higher intraoperatively (p < 0.0001). Pulmonary artery systolic pressures measured by pulmonary artery catheter provided higher values compared to preoperative TTE estimate (39 mmHg [34-45 mmHg] v 34 mmHg [28-41 mmHg], p < 0.0001). CONCLUSIONS: Discrepancy between hemodynamic measurements in the cardiac laboratory and operating room is common and generally should not affect planned patients' care. These changes in hemodynamics might be explained by preoperative fasting, anesthetic agents, volume shifts while supine, and positive-pressure ventilation, as well as the difference in measurement techniques.

Mitral Valve Cleft-like Indentations in Hypertrophic Obstructive Cardiomyopathy: Insights From Intraoperative Three-Dimensional Transesophageal Echocardiography.

OBJECTIVES: Cleft-like indentations (CLIs) of the mitral valve (MV) are best assessed with three-dimensional (3D) transesophageal echocardiography (TEE). The present study examined the prevalence, characteristics, and surgical effect of MV CLIs in patients with hypertrophic cardiomyopathy (HCM). DESIGN: Prospective, observational, case-control study. SETTING: Tertiary medical center. PARTICIPANTS: The study comprised 90 patients with HCM undergoing myectomy and 59 patients undergoing cardiac surgery for non-MV related indications. MEASUREMENTS AND MAIN RESULTS: Intraoperative 3D TEE was used to evaluate the presence and characteristics of MV CLIs compared, with a random control group of 59 patients undergoing cardiac surgery for non-MV related indications. Ninety patients with HCM (mean age 54.8 ± 13.3 y, 67.8% male) were compared with 59 control patients (mean age 67 ± 12.7 y, 79.7% male). Three-dimensional TEE images were interpreted by consensus of two experienced echocardiographers. At least one MV CLI was present in 84 patients with HCM (93.3%), compared with 23 control patients (39%; p < 0.01). Compared with control patients, patients with HCM were more likely to have deep MV CLIs (85.6% v 25.4%; p < 0.01) and ≥2 CLIs (52.2% v 26.1%; p = 0.02). Six HCM patients (7%) appeared to have true congenital posterior leaflet clefts versus 0% in control patients (p = 0.08). Preoperative mitral regurgitation severity and jet direction were not associated with the presence of deep or multiple MV CLIs (all p > 0.2). None of the MV CLIs in the HCM group required MV surgical intervention or second pump runs for MV regurgitation correction after myectomy. CONCLUSION: Deep and multiple MV CLIs are common in patients with HCM undergoing septal myectomy, including possible true posterior clefts, but they are not associated with the premyectomy severity of mitral regurgitation or jet direction, and do not result in surgical MV intervention.

Outcomes of cardiac surgery in nonagenarians.

BACKGROUND: While advanced age can be considered by some a contraindication to open-heart surgery, there is a paucity of data regarding outcomes of cardiac surgery in nonagenarians. We, therefore, sought to investigate the outcomes of nonagenarians undergoing cardiac surgery. METHODS: A retrospective review of our institutional Society of Thoracic Surgeons database between 1993 and 2019 was performed. Among a total of 32,421 patients who underwent open-heart surgery, 134 patients (0.4%) were nonagenarians (50.7% females, median age 91.6 [interquartile range: 90.7-92.9]). A comparison was performed between nonagenarians and patients aged 80-89 years. A regression analysis was performed to evaluate factors associated with midterm mortality in nonagenarians. RESULTS: The incidence of cardiac surgery in nonagenarians has been stable over time, from 0.4% in (1993-2000), 0.5% in (2001-2010) to 0.4% in (2011-2019). Valve surgery and CABG+valve were higher in nonagenarians compared to octogenarians (44.8% vs. 25.6%, 39.6% vs. 30.7%, respectively), but CABG was lower (15.7% vs. 33.8%); p < .01. Urgent/emergent surgery status was similar between groups (p = .7). Operative mortality was similar in the two groups (6% vs. 4.6%, p = .5). Hospital complications were comparable between groups. CONCLUSION: Cardiac surgery in nonagenarians can be achieved with acceptable morbidity and mortality. This study can be a benchmark for risk stratification for cardiac surgery in this high-risk population.

2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.

Aim This executive summary of the hypertrophic cardiomyopathy clinical practice guideline provides recommendations and algorithms for clinicians to diagnose and manage hypertrophic cardiomyopathy in adult and pediatric patients as well as supporting documentation to encourage their use. Methods A comprehensive literature search was conducted from January 1, 2010, to April 30, 2020, encompassing studies, reviews, and other evidence conducted on human subjects that were published in English from PubMed, EMBASE, the Cochrane Collaboration, Agency for Healthcare Research and Quality reports, and other relevant databases. Structure Many recommendations from the earlier hypertrophic cardiomyopathy guidelines have been updated with new evidence or a better understanding of earlier evidence. This summary operationalizes the recommendations from the full guideline and presents a combination of diagnostic work-up, genetic and family screening, risk stratification approaches, lifestyle modifications, surgical and catheter interventions, and medications that constitute components of guideline directed medical therapy. For both guideline-directed medical therapy and other recommended drug treatment regimens, the reader is advised to follow dosing, contraindications and drug-drug interactions based on product insert materials.

An unusual presentation of dyspnea following septal ablation for obstructive hypertrophic cardiomyopathy.

Residual or recurrent symptoms after septal reduction therapy are most often related to inadequate relief of left ventricular outflow gradients. We recently encountered a 71-year-old woman with hypertrophic cardiomyopathy and prior alcohol septal ablation who had a unique constellation of findings causing her symptoms. She was found to have four potential causes for her symptoms, residual midventricular obstruction, apical distribution of hypertrophy reducing end-diastolic volume, constrictive pericarditis, and marked arterial stiffness, as reflected by aortic atherosclerosis. She underwent complete pericardiectomy, transaortic septal myectomy, transapical myectomy, and replacement of a heavily calcified ascending aorta.

Comparison of expected and observed outcomes for septal myectomy in hypertrophic obstructive cardiomyopathy.

BACKGROUND: Septal myectomy remains the criterion standard for treatment of symptomatic, medically refractory hypertrophic cardiomyopathy (HCM). There is no specific surgical risk calculator for septal myectomy. METHODS: This study compares the outcomes of septal myectomy at a tertiary referral center with predicted outcomes of mitral valve (MV) repair and aortic valve replacement (AVR) using the Society of Thoracic Surgeons Adult Cardiac Surgery Risk Calculator (STS Calculator). A total of 298 consecutive patients with HCM underwent isolated septal myectomy from 2011 to 2014. Observed outcomes of septal myectomy were compared with the STS Calculator predicted risk of isolated MV repair and AVR predicted within this population using 1-sample tests of proportions. RESULTS: Thirty-day mortality for myectomy in this cohort was zero. STS Calculator predicted risk of mortality for MV repair was 0.7% (P = .14) and for AVR = 1.1% (P = .06). Follow-up for vital status was 6.0 ±â€¯0.7 years, at which 294 (98.7%) patients were alive. Hospital stay length was 4.9 ±â€¯1.9 days. One (0.3%) patient experienced a postoperative deep sternal wound infection, and 1 (0.3%) patient experienced a prolonged ventilated state. Postoperative atrial fibrillation occurred in 64 (21.5%) patients. During 30 days of follow-up, no patients experienced stroke, renal failure, or needed dialysis. CONCLUSIONS: Septal myectomy, performed in a tertiary referral center, had a 30-day mortality rate of 0% and low morbidity rate. There was no difference between observed myectomy mortality and STS Calculator predicted risk for AVR and MV repair. It is possible that a larger sample could reveal lower mortality than STS prediction.

Management of Coronary Artery Aneurysms at the Time of Surgical Revascularization.

BACKGROUND: Coronary artery aneurysms (CAAs) represent a rare pathology occurring in 1.5%-5% of routine coronary angiograms. Limited data exist on the management of CAA at the time of cardiac surgery. MATERIALS AND METHODS: A single-institution retrospective review was performed on 53 patients who underwent cardiac surgery in the setting of atherosclerotic CAA between 1993 and 2015. Patients were stratified based on treatment strategy: exclusion and distal bypass (n = 26) versus revascularization alone (n = 27). Comparisons were made with respect to mortality, need for further/concomitant interventions, and long-term cardiac function including myocardial infarctions and congestive heart failure. RESULTS: A total of 53 patients underwent cardiac surgery in the setting of CAA disease. Management strategies included ligation and bypass in 26 patients and distal bypass only in 27 patients (with four of the patients in this group undergoing coronary stenting across the aneurysm). There were no significant differences in patient demographics between the two groups. No significant difference was found in either 30-d (P = 0.74) or long-term mortality when exclusion of the CAA was performed compared with revascularization alone (P = 0.20). More exclusion procedures were performed earlier in the experience (median surgical date 2000), whereas revascularization alone predominated later in the experience (median surgical date 2007; P ≤ 0.001). CONCLUSIONS: The practice of CAA exclusion, while still performed in selected cases, has largely been supplanted in patients undergoing revascularization. Exclusion does not appear to offer any advantage over isolated revascularization, supporting the current trends in managing this rare condition.

Dissecting myocardial mechanics in patients with severe aortic stenosis: 2-dimensional vs 3-dimensional-speckle tracking echocardiography.

BACKGROUND: Aortic stenosis (AS) causes left ventricular (LV) pressure overload, leading to adverse LV remodeling and dysfunction. Identifying early subclinical markers of LV dysfunction in patients with significant AS is critical as this could provide support for earlier intervention, which may result in improved long-term outcomes. We therefore examined the impact of severe AS and its consequent increase in LV afterload on myocardial deformation and rotational mechanics by 2-dimensional (2D) and 3-dimensional (3D) speckle-tracking echocardiography. METHODS: We prospectively measured various strain parameters in 168 patients (42% female, mean age 72 ± 12 years) with severe AS and LV ejection fraction (EF) ≥50%, and compared them to normal values found in literature. 2D and 3D images were analyzed for global longitudinal strain (GLS), global circumferential strain (GCS), global radial strain (GRS), basal rotation, apical rotation, and peak systolic twist. We further assessed the degree of concordance between 2D and 3D strain, and examined their association with measures of LV preload and afterload. RESULTS: Patients with severe AS exhibited significantly lower GLS and GRS but higher GCS, apical rotation, and twist by 2D and 3D echocardiography compared with published normal values (P = 0.003 for 3D twist, P < 0.001 for all others). Agreement between 2D- and 3D-GLS by concordance correlation coefficient was 0.49 (95% confidence interval: 0.39-0.57). GLS was correlated with valvulo-arterial impedance, a measure of LV afterload (r = 0.34, p < 0.001 and r = 0.23, p = 0.003, respectively). CONCLUSION: Patients with severe AS demonstrated lower-than-normal GLS and GRS but appear to compensate with higher-than-normal GCS, apical rotation, and twist in order to maintain a preserved LVEF. GLS showed a modest correlation with valvulo-arterial impedance.

Thrombin Generation and Antithrombin Activity in Infants Undergoing Cardiopulmonary Bypass-An Exploratory Study.

OBJECTIVES: Coagulopathic bleeding is a major complication of pediatric cardiac surgery. Investigating perioperative dynamics of thrombin generation and antithrombin (AT) activity might provide more insight into the underlying mechanisms of coagulopathy. This can help develop a targeted hemostatic approach in the future. The authors hypothesized that there is a decline in both thrombin generation and AT activity in infants undergoing cardiopulmonary bypass (CPB). DESIGN: Prospective observational study. SETTING: Single academic medical center. PARTICIPANTS: Infants <10 kg of weight undergoing cardiac surgery with CPB. INTERVENTIONS: Blood specimen collection and testing. MEASUREMENTS AND RESULTS: The authors performed assays of thrombin generation and AT activity on the samples of platelet-poor plasma of 25 infants, repeating them at 3 points: before CPB and heparinization, after separation from CPB and protamine administration, and after chest closure. The authors observed a statistically significant decline in thrombin generation shortly after separation from CPB compared with baseline. The geometric mean for lag time was prolonged (4.0 v 5.5 minutes, p = 0.013), and peak thrombin and the net amount of generated thrombin declined almost 3-fold (80.7 v 25.1 nmol, p < 0.001; 1264 v 476 nmol, p < 0.001, respectively). This was accompanied by a decline in AT activity (59.8 v 50.1, p = 0.001). After platelet and cryoprecipitate transfusion, at the case conclusion AT activity had recovered marginally (59.8 v 55.4, p = 0.042), but thrombin generation remained reduced. CONCLUSIONS: In pediatric patients <10 kg undergoing cardiac surgery with CPB, thrombin generation and AT activity decline and do not recover completely after transfusion of platelets and cryoprecipitate.

Hypertrophic Obstructive Cardiomyopathy: Surgical Myectomy and Septal Ablation.

Hypertrophic cardiomyopathy is a genetic disorder characterized by marked hypertrophy of the myocardium. It is frequently accompanied by dynamic left ventricular outflow tract obstruction and symptoms of dyspnea, angina, and syncope. The initial therapy for symptomatic patients with obstruction is medical therapy with ß-blockers and calcium antagonists. However, there remain a subset of patients who have continued severe symptoms, which are unresponsive to medical therapy. These patients can be treated with septal reduction therapy, either surgical septal myectomy or alcohol septal ablation. When performed by experienced operators working in high-volume centers, septal myectomy is highly effective with a >90% relief of obstruction and improvement in symptoms. The perioperative mortality rate for isolated septal myectomy in most centers is <1%. Alcohol septal ablation is a less invasive treatment. In many patients, the hemodynamic and clinical results are comparable to that of septal myectomy. However, the results of alcohol septal ablation are dependent on the septal perforator artery supplying the area of the contact between the hypertrophied septum and the anterior leaflet of the mitral valve. There are some patients, particularly younger patients with severe hypertrophy, who do not uniformly experience complete relief of obstruction and symptoms. Both techniques of septal reduction therapy are highly operator dependent. The final decision as to which approach should be selected in any given patient is dependent up patient preference and the availability and experience of the operator and institution at which the patient is being treated.

Impact of Left Atrial Appendage Closure During Cardiac Surgery on the Occurrence of Early Postoperative Atrial Fibrillation, Stroke, and Mortality: A Propensity Score-Matched Analysis of 10 633 Patients.

BACKGROUND: Prophylactic exclusion of the left atrial appendage (LAA) is often performed during cardiac surgery ostensibly to reduce the risk of stroke. However, the clinical impact of LAA closure in humans remains inconclusive. METHODS: Of 10 633 adults who underwent coronary artery bypass grafting and valve surgery between January 2000 and December 2005, 9792 patients with complete baseline characteristics, surgery procedure, and follow-up data were included in this analysis. A propensity score-matching analysis based on 28 pretreatment covariates was performed and 461 matching pairs were derived and analyzed to estimate the association of LAA closure with early postoperative atrial fibrillation (POAF) (atrial fibrillation ≤30 days of surgery), ischemic stroke, and mortality. RESULTS: In the propensity-matched cohort, the overall incidence of POAF was 53.9%. In this group, the rate of early POAF among the patients who underwent LAA closure was 68.6% versus 31.9% for those who did not undergo the procedure (P<0.001). LAA closure was independently associated with an increased risk of early POAF (adjusted odds ratio, 3.88; 95% confidence interval, 2.89-5.20), but did not significantly influence the risk of stroke (adjusted hazard ratio, 1.07; 95% confidence interval, 0.72-1.58) or mortality (adjusted hazard ratio, 0.92; 95% confidence interval, 0.75-1.13). CONCLUSIONS: After adjustment for treatment allocation bias, LAA closure during routine cardiac surgery was significantly associated with an increased risk of early POAF, but it did not influence the risk of stroke or mortality. It remains uncertain whether prophylactic exclusion of the LAA is warranted for stroke prevention during non-atrial fibrillation-related cardiac surgery.

Pathophysiology of Aortic Valve Stenosis: Is It Both Fibrocalcific and Sex Specific?

Our understanding of the fundamental biology and identification of efficacious therapeutic targets in aortic valve stenosis has lagged far behind the fields of atherosclerosis and heart failure. In this review, we highlight the most clinically relevant problems facing men and women with fibrocalcific aortic valve stenosis, discuss the fundamental biology underlying valve calcification and fibrosis, and identify key molecular points of intersection with sex hormone signaling.

Women with hypertrophic cardiomyopathy have worse survival.

AIMS: Sex differences in hypertrophic cardiomyopathy (HCM) remain unclear. We sought to characterize sex differences in a large HCM referral centre population. METHODS AND RESULTS: Three thousand six hundred and seventy-three adult patients with HCM underwent evaluation between January 1975 and September 2012 with 1661 (45.2%) female. Kaplan-Meier survival curves were assessed via log-rank test. Cox proportional hazard regression analyses evaluated the relation of sex with survival. At index visit, women were older (59 ± 16 vs. 52 ± 15 years, P < 0.0001) had more symptoms [New York Heart Association (NYHA) Class III-IV 45.0% vs. 35.3%, P < 0.0001], more obstructive physiology (77.4% vs. 71.8%, P = 0.0001), more mitral regurgitation (moderate or greater in 56.1% vs. 43.9%, P < 0.0001), higher E/e' ratio (n = 1649, 20.6 vs. 15.6, P < 0.0001), higher estimated pulmonary artery systolic pressure (n = 1783, 40.8 ± 15.4 vs. 34.8 ± 10.8 mmHg, P < 0.0001), worse cardiopulmonary exercise performance (n = 1267; percent VO2 predicted 62.8 ± 20% vs. 65.8 ± 19.2%, P = 0.007), and underwent more frequent alcohol septal ablation (4.9% vs. 3.0%, P = 0.004) but similar frequency of myectomy (28% vs. 30%, P = 0.24). Median follow-up was 10.9 (IQR 7.4-16.2) years. Kaplan-Meier analysis demonstrated lower survival in women compared with men (P < 0.0001). In multivariable modelling, female sex remained independently associated with mortality (HR 1.13 [1.03-1.22], P = 0.01) when adjusted for age, NYHA Class III-IV symptoms, and cardiovascular comorbidities. CONCLUSION: Women with HCM present at more advanced age, with more symptoms, worse cardiopulmonary exercise tolerance, and different haemodynamics than men. Sex is an important determinant in HCM management as women with HCM have worse survival. Women may require more aggressive diagnostic and therapeutic approaches.

Aortic Root Surgery in Marfan Syndrome: Medium-Term Outcome in a Single-Center Experience.

BACKGROUND: The study aim was to analyze the authors' experience with aortic root surgery in Marfan syndrome (MFS), and to expand the surgical outcome data of patients meeting the Ghent criteria (Marfan registry). METHODS: Analyses were performed of data acquired from MFS patients (who met the Ghent criteria), including an aortic root surgery and Kaplan-Meier survival. RESULTS: Between April 2004 and February 2012, a total of 59 MFS patients (mean age at surgery 36 ± 13 years) underwent 67 operations for aortic root aneurysm (n = 52), aortic valve (AV) regurgitation (n = 15), acute aortic dissection (n = 2), and/or mitral valve (MV) regurgitation resulting from MV prolapse (n = 7). Of 59 initial operations, 21 (36%) involved AV-replacing root surgery, 38 (64%) AV-sparing root surgery, seven (12%) aortic arch or hemi-arch repair, and five (8%) simultaneous MV surgery. There were no early mortalities. The mean follow up was 6.8 ± 1.2 years, with five deaths (8%) and a relatively low reoperation rate (10 reoperations in nine patients; 14%). Seven reoperations involved AV or aortic root surgery (including four for AV regurgitation following failed AV-sparing surgery), two MV repair/replacements, and one coronary artery bypass graft. Eight patients (21%) with AV-sparing surgery had moderate/severe AV regurgitation at the last follow up before re-intervention. The mean five-year freedom from postoperative death was 91.2 ± 8.8%, from cardiac reoperation 86.3 ± 4.5%, and more-than-moderate AV regurgitation 90.3 ± 4.8%. CONCLUSIONS: Prophylactic aortic surgery in MFS patients with AV-replacing root or AV-sparing root surgery carries a low risk of operative morbidity and death when performed at an experienced center. AV-sparing root surgery increases the risk of AV regurgitation and, possibly, of re-intervention. Regular clinical follow up is important after any aortic root surgery in MFS patients, with a delineation of risk factors for AV regurgitation after AV rootsparing surgery.

Hemodynamic changes in systolic and diastolic function during isoproterenol challenge predicts symptomatic response to myectomy in hypertrophic cardiomyopathy with labile obstruction.

OBJECTIVES: We aimed to assess the utility of changes in systolic and diastolic function by isoproterenol challenge in predicting symptom resolution post-myectomy in selected patients with hypertrophic cardiomyopathy (HCM) and labile obstruction. BACKGROUND: In a subset of symptomatic HCM patients without resting/provocable obstruction on noninvasive assessment, isoproterenol challenge during hemodynamic catheterization may elicit labile left ventricular outflow tract (LVOT) obstruction, and demonstrate the effect of obstruction on diastolic function. These changes may determine whether patients achieve complete symptom resolution post-myectomy. METHODS: Between February 2003 and April 2009, 18 symptomatic HCM patients without LVOT obstruction on noninvasive testing underwent isoproterenol provocation and septal myectomy due to presence of provocable gradient and were followed for 4 (IQR 3-7) years. RESULTS: Thirteen (72.2%) had complete symptom resolution, while 5 (27.8%) had improved, but persistent symptoms. Those with provoked gradient >100 mm Hg or increase in left atrial pressure (LAP) with isoproterenol had symptom resolution. CONCLUSIONS: Symptomatic HCM patients without LVOT gradient on noninvasive testing may demonstrate labile obstruction with isoproterenol. With isoproterenol, patients with high LVOT gradient or increase in LAP concomitant with an increase in gradient achieved complete symptom resolution post-myectomy. Thus, improved diastolic filling as well as outflow gradient production in patients with HCM may predict symptom response to myectomy. © 2016 Wiley Periodicals, Inc.