Joseph A. Dearani

2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines

Writing committee me tions to which their s ply; see Appendix ACCF/AHATask Fo Surgeons Representa tative. Heart Rhythm ography and Int Echocardiography Re ciety of America Rep resentative. kkACCF/ Task Force member d This document was app Board of Trustees and ordinating Committee gery, American Soc Cardiology, Heart Fa for Cardiovascular A geons approved the d The American Associat as follows: Gersh BJ Naidu SS, Nishimura Bernard J. Gersh, MB, ChB, DPhil, FACC, FAHA, Co-Chair* Barry J. Maron, MD, FACC, CoChair* Robert O. Bonow, MD, MACC, FAHA, Joseph A. Dearani, MD, FACC,§,k Michael A. Fifer, MD, FACC, FAHA,* Mark S. Link, MD, FACC, FHRS,* Srihari S. Naidu, MD, FACC, FSCAI,* Rick A. Nishimura, MD, FACC, FAHA, Steve R. Ommen, MD, FACC, FAHA, Harry Rakowski, MD, FACC, FASE,** Christine E. Seidman, MD, FAHA, Jeffrey A. Towbin, MD, FACC, FAHA, James E. Udelson, MD, FACC, FASNC, and Clyde W. Yancy, MD, FACC, FAHAkk

2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy A Report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines Developed in Collaboration With the American Association for Thoracic Surgery, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons

Alice K. Jacobs, MD, FACC, FAHA, Chair, 2009–2011; Sidney C. Smith, Jr, MD, FACC, FAHA, Immediate Past Chair, 2006–2008[¶¶][1]; Jeffrey L. Anderson, MD, FACC, FAHA, Chair-Elect; Nancy M. Albert, PhD, CCNS, CCRN, FAHA; Christopher E. Buller, MD, FACC[¶¶][1]; Mark A. Creager, MD, FACC, FAHA;

2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy: Executive Summary

2011;58;2703-2738; originally published online Nov 8, 2011; J. Am. Coll. Cardiol. W. Yancy Rakowski, Christine E. Seidman, Jeffrey A. Towbin, James E. Udelson, and Clyde Fifer, Mark S. Link, Srihari S. Naidu, Rick A. Nishimura, Steve R. Ommen, Harry Bernard J. Gersh, Barry J. Maron, Robert O. Bonow, Joseph A. Dearani, Michael A. Thoracic Surgeons Society for Cardiovascular Angiography and Interventions, and Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, of Thoracic Surgery, American Society of Echocardiography, American Society Developed in Collaboration With the American Association for Guidelines Cardiology Foundation/American Heart Association Task Force on Practice Cardiomyopathy: Executive Summary: A Report of the American College of 2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic This information is current as of May 14, 2012 http://content.onlinejacc.org/cgi/content/full/58/25/2703 located on the World Wide Web at: The online version of this article, along with updated information and services, is

Ebstein's anomaly.

Ebstein’s anomaly is a rare congenital heart disorder occurring in 1 per 200 000 live births and accounting for 1% of all cases of congenital heart disease.2–6 This anomaly was described by Wilhelm Ebstein in 1866 in a report titled, “Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation.” 7,8 The patient was a 19-year-old cyanotic man with dyspnea, palpitations, jugular venous distension, and cardiomegaly. 7,8 At autopsy, Ebstein described an enlarged and fenestrated anterior leaflet of the tricuspid valve. The posterior and septal leaflets were hypoplastic, thickened, and adherent to the right ventricle. There was also a thinned and dilated atrialized portion of the right ventricle, an enlarged right atrium, and a patent foramen

Efficacy of a simple intraoperative transfusion algorithm for nonerythrocyte component utilization after cardiopulmonary bypass.

Background Abnormal bleeding after cardiopulmonary bypass (CPB) is a common complication of cardiac surgery, with important health and economic consequences. Coagulation test–based algorithms may reduce transfusion of non-erythrocyte allogeneic blood in patients with abnormal bleeding. Methods The authors performed a randomized prospective trial comparing allogeneic transfusion practices in 92 adult patients with abnormal bleeding after CPB. Patients with abnormal bleeding were randomized to one of two groups: a control group following individual anesthesiologist’s transfusion practices and a protocol group using a transfusion algorithm guided by coagulation tests. Results Among 836 eligible patients having all types of elective cardiac surgery requiring CPB, 92 patients developed abnormal bleeding after CPB (incidence, 11%). The transfusion algorithm group received less allogeneic fresh frozen plasma in the operating room after CPB (median, 0 units; range, 0–7 units) than the control group (median, 3 units; range, 0–10 units) (P = 0.0002). The median number of platelet units transfused in the operating room after CPB was 4 (range, 0–12) in the algorithm group compared with 6 (range, 0–18) in the control group (P = 0.0001). Intensive care unit (ICU) mediastinal blood loss was significantly less in the algorithm group. Multivariate analysis demonstrated that transfusion algorithm use resulted in reduced ICU blood loss. The control group also had a significantly greater incidence of surgical reoperation of the mediastinum for bleeding (11.8%vs. 0%;P = 0.032). Conclusions Use of a coagulation test–based transfusion algorithm in cardiac surgery patients with abnormal bleeding after CPB reduced non-erythrocyte allogeneic transfusions in the operating room and ICU blood loss.

Cox-Maze Procedure for Atrial Fibrillation: Mayo Clinic Experience

The Cox-Maze procedure corrects atrial fibrillation in 90% of patients, and successful operation restores sinus rhythm, thereby reducing risks of thromboembolism and anticoagulant-associated hemorrhage. Symptoms such as palpitation and fatigability also improve with restoration of atrioventricular synchrony. At the Mayo Clinic, 221 Cox-Maze procedures were performed from March 1993 through March 1999. Over 75% of patients had associated cardiac disease and concomitant operations. Overall, early mortality was 1.4%, and the incidence of postoperative pacemaker implantation was 3.2%. Limiting incisions to the right atrium simplifies the operation for patients who primarily have tricuspid valve disease, and in early follow-up, outcome appeared to be as good as that achieved with biatrial incisions. The Cox-Maze procedure has proved particularly useful for patients with preoperative atrial fibrillation who require valvuloplasty for acquired mitral valve regurgitation; 87 patients have had this combined procedure, and there have been no early deaths. Further, our experience indicates that ventricular dysfunction is not a contraindication for operation and that restoration of sinus rhythm after the Cox-Maze procedure improves left ventricular ejection fraction in most patients.

Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits

BACKGROUND Pulmonary ventricle (PV) to pulmonary artery (PA) conduits have made possible the correction of many complex congenital cardiac anomalies. METHODS Between April 1964 and January 2001, 1270 patients underwent operation with conduit placement from the PV to PA. The present study evaluates late outcome of 1095 patients (612 males, 483 females) having an operation before July 1992. Mean age was 9.6 +/- 8.2 years old. Diagnoses included pulmonary atresia/tetralogy of Fallot (459), transposition of the great arteries (TGA) (232), truncus arteriosus (193), double outlet right ventricle (DORV) (121), corrected TGA (49), septated univentricular heart (36), and other (5). A porcine-valved Dacron conduit was used in 730, homograft in 239, and non-valved conduit in 126. RESULTS Early mortality decreased from 23.5% prior to 1980 to 3.7% for the most recent decade. Mean follow-up was 10.9 years (maximum, 29 years). Actuarial survival for early survivors at 10 and 20 years was 77.0% +/- 1.5% and 59.5% +/- 2.6%. On univariate analysis, clinical and hemodynamic factors associated with late mortality were male gender, older age at operation, higher post-repair PV/systemic ventricle (SV) pressure ratio, higher distal PA pressure, and longer bypass time (p < or = 0.01 for all). On multivariate analysis, independent risk factors for late mortality were male gender, older age at operation, diagnosis of TGA, corrected TGA, truncus, or univentricular heart, and PV/SV pressure ratio > or = 0.72 (p < or = 0.03 for all). Freedom from reoperation for conduit failure at 10 and 20 years was 55.5% +/- 2.0% and 31.9% +/- 2.7%. On multivariate analysis, independent risk factors for conduit failure were homograft conduit, diagnosis of TGA, younger age at operation, and smaller conduit size (p < or = 0.007 for all). Reoperation for one conduit replacement was performed in 306 patients, two conduit replacements in 55 patients, three in 6 patients, and four in 3 patients. Overall early mortality for conduit replacement in this series was 4.9%; it was 1.7% for patients operated on from 1989 through 1992. At follow-up, 84% of survivors were in NYHA class I or II. CONCLUSIONS Operations that include conduit placement and replacement can be performed with low early mortality. Younger age at operation was associated with improved late survival. The diagnosis of TGA was associated with increased risk for conduit failure, and the durability of the homograft, in this series, was inferior to the porcine-valved Dacron conduit. Quality of life was excellent for most patients despite the need for reoperation.

Clinical accuracy of RIFLE and Acute Kidney Injury Network (AKIN) criteria for acute kidney injury in patients undergoing cardiac surgery

IntroductionThe RIFLE (risk, injury, failure, loss of kidney function, and end-stage renal failure) classification for acute kidney injury (AKI) was recently modified by the Acute Kidney Injury Network (AKIN). The two definition systems differ in several aspects, and it is not clearly determined which has the better clinical accuracy.MethodsIn a retrospective observational study we investigated 4,836 consecutive patients undergoing cardiac surgery with cardiopulmonary bypass from 2005 to 2007 at Mayo Clinic, Rochester, MN, USA. AKI was defined by RIFLE and AKIN criteria.ResultsSignificantly more patients were diagnosed as AKI by AKIN (26.3%) than by RIFLE (18.9%) criteria (P < 0.0001). Both definitions showed excellent association to outcome variables with worse outcome by increased severity of AKI (P < 0.001, all variables). Mortality was increased with an odds ratio (OR) of 4.5 (95% CI 3.6 to 5.6) for one class increase by RIFLE and an OR of 5.3 (95% CI 4.3 to 6.6) for one stage increase by AKIN. The multivariate model showed lower predictive ability of RIFLE for mortality. Patients classified as AKI in one but not in the other definition set were predominantly staged in the lowest AKI severity class (9.6% of patients in AKIN stage 1, 2.3% of patients in RIFLE class R). Potential misclassification of AKI is higher in AKIN, which is related to moving the 48-hour diagnostic window applied in AKIN criteria only. The greatest disagreement between both definition sets could be detected in patients with initial postoperative decrease of serum creatinine.ConclusionsModification of RIFLE by staging of all patients with acute renal replacement therapy (RRT) in the failure class F may improve predictive value. AKIN applied in patients undergoing cardiac surgery without correction of serum creatinine for fluid balance may lead to over-diagnosis of AKI (poor positive predictive value). Balancing limitations of both definition sets of AKI, we suggest application of the RIFLE criteria in patients undergoing cardiac surgery.

Surgery for aneurysms of the aortic root: A 30-year experience

Background—This study evaluated long-term results of aortic root replacement and valve-preserving aortic root reconstruction for patients with aneurysms involving the aortic root. Methods and Results—Two-hundred three patients aged 53±16 years (mean±SD; 153 male, 50 female) underwent elective or urgent aortic root surgery from 1971 to 2000 for an aortic root aneurysm: 149 patients underwent a composite valve conduit reconstruction, and 54 patients underwent valve-preserving aortic root reconstruction. Fifty patients had Marfan syndrome. In-hospital and 30-day mortality was 4.0% (8/203) overall: for a composite valve conduit procedure, the corresponding value was 4.0% (6/149) and for valve-preserving procedure, 3.7% (2/54) (P=NS). Morbidity included 3 strokes (1%), 10 perioperative myocardial infarctions (5%), and 8 reoperations for bleeding (4%). Actuarial survival at 5, 10, 15, and 20 years was 93% (95% confidence interval [CI] = 88% to 97%), 79% (95% CI = 71% to 87%), 67% (95% CI = 57% to 79%), and 52% (95% CI = 36% to 69%), respectively. Freedom from reoperation was 72% (95% CI = 54% to 86%) at 20 years. Complications with anticoagulation occurred in 29 patients; with valve thrombosis, in 2; and with hemorrhage, in 27 (4 life threatening and 23 minor). Freedom from thromboembolism was 91% (95% CI = 77% to 98%) at 20 years. Freedom from endocarditis was 99% (95% CI = 92% to 100%) at 20 years. Multivariate analysis revealed preoperative mitral valve regurgitation (+3 to 4) and older age to be significant predictors of late death (P≤0.005), and Marfan syndrome, initial valve-preserving aortic root reconstruction, and need for a concomitant procedure at initial operation to be significant predictors of the need for reoperation (P≤0.01). Conclusions—Aortic root replacement for aortic root aneurysms can be done with low morbidity and mortality. Composite valve conduit reconstruction resulted in a durable result. There were few serious complications related to the need for long-term anticoagulation or a prosthetic valve. Reoperation was most commonly required because of failure of the aortic valve when a valve-preserving aortic root reconstruction was performed or for other cardiac or aortic disease elsewhere.

Survival After Alcohol Septal Ablation for Obstructive Hypertrophic Cardiomyopathy

Background— The clinical efficacy of alcohol septal ablation for obstructive hypertrophic cardiomyopathy (HCM) has been demonstrated, but the long-term effects of the procedure remain uncertain. This study examined the survival of patients after septal ablation performed in a tertiary HCM referral center. Methods and Results— We examined 177 patients (mean age, 64 years; 68% women) who underwent septal ablation at our institution. Over a follow-up of 5.7 years, survival free of all mortality was no different than the expected survival for a comparable general population, and similar to that of age- and sex-matched patients who underwent isolated surgical myectomy (8-year survival estimate, 79% versus 79%; P=0.64). For the end point of documented sudden cardiac death or unknown cause of death, the incidence per 100 person-year follow-up was 1.31 (95% confidence interval, 0.60–2.38). Residual left ventricular outflow tract gradient after ablation was an independent predictor of long-term survival free of any death. Conclusions— In this nonrandomized study of carefully selected patients undergoing septal ablation by experienced operators in a tertiary referral HCM center, long-term survival was favorable and similar to that of an age- and sex-matched general population, and to patients undergoing surgical myectomy, as well, without an increased risk of sudden cardiac death.