Barlow disease: effect of mitral valve repair on ventricular arrhythmias in 82 patients in a retrospective long-term study.

BACKGROUND: Patients (pt) with mitral valve prolapse (MVP) due to Barlow disase (BD) have an increased incidence of ventricular arrhythmias (VA; including ventricular tachycardias VT) and sudden cardiac death (SCD). Data on the effect of MV repair on VA are scarce. METHODS: Pre- and postoperative VA in severe mitral regurgitation (MR) with MVP due to BD undergoing surgical mitral valve repair were analyzed. Patients with degenerative mitral valve disease not fulfilling BD criteria were excluded. Information was from charts, ECG/Holter ECG and/or pacemaker/ implantable cardioverter defibrillator (ICD) data. SCD, sustained VT>30 sec and/or ventricular fibrillation necessitating an ICD-shock were considered major events. Event probability was calculated using the Kaplan-Meier estimator throughout the follow-up period of 20.7 years. RESULTS: There were 82 pts (61% males), mean age at surgery 62±14 years. Bileaflet MVP was present in 54%, mitral annular dysjunction (MAD) in 37% and left ventricular ejection fraction (LVEF) <50% in 12%. MV repair included ring annuloplasty in all and artificial chords in 48%. Mean follow-up was 3.1 years (0.2 to 14.2 years). Postoperative rhythm surveillance by Holter ECG and/or pacemaker was available in 67%. A VA load of ≥10% and/or any VT was noted in 26% before and 32% after surgery (P=0.44). Postoperative VA load was not predicted by MAD, artificial chords, LVEF of <50%, age at surgery >50 years and/or residual ≥moderate MR (all P<0.05), it correlated only with bileaflet MVP (P=0.009). Major events occurred in 3 pts: SCD in 2 pts and ICD for sustained polymorphic VT in 1 pt (incidence 1.2%/year). The event probability of receiving a SCD or an ICD-shock was 4.9%. CONCLUSIONS: VA burden does not seem to change after MV repair in MVP due to BD. The occurrence of major arrhythmic events can not be predicted reliably, thus, patients with MVP due to BD may need lifelong postoperative follow-up, especially in bileaflet MVP which was an independent risk factor for increased VA burden in this retrospective long-term study in a small but well selected patient group.

Marfan syndrome and related connective tissue disorders in the current era in Switzerland in 103 patients: medical and surgical management and impact of genetic testing.

INTRODUCTION: Marfan syndrome (MFS) and related connective tissue disorders (CTDs) are increasingly recognised. Genetic testing has greatly improved the diagnostic outcome/power over the last two decades. In this study we describe a multicentre cohort of adults with MFS and related CTDs, with a particular focus on results from genetic testing. METHODS: All patients with MFS and related CTDs were identified from the databases of five centres in the canton of Zurich. Echocardiographic and clinical findings including systemic Marfan score, use of medication and genetic results were retrospectively analysed. MFS was diagnosed using the revised Ghent criteria (including FBN1 genetic testing if available); other CTDs (Loeys-Dietz syndrome) were diagnosed by genetic testing only. RESULTS: A cohort of 103 patients were identified (62 index patients, 41 relatives of family members): 96 patients with MFS and 7 patients with other CTD, 54 males (52%), median age 23 years (range 1–75). The median systemic Marfan score was 5 (range 0–18). Only 40 patients (40/103, 39%) fulfilled criteria for systemic involvement (≥7 points). A history of aortic dissection was present in 14 out of 103 patients (14%). Echocardiographic data were available for all: aortic root enlargement (Z-score ≥2 in adults, Z-score ≥3 in children) was found in 49 patients (48%) and mitral valve prolapse in 64 (62%). Genetic testing had been performed in 80 patients (78%); FBN1 mutations were present in 69 patients (86%); other pathogenic mutations could be identified in seven patients (9%); no disease-causing mutation was found in four patients, three of them fulfilling the Ghent criteria of MFS. Of the mutation-positive patients, 33 had a systemic score of ≥7 and 43 had a systemic score of ≥5. Revised Ghent criteria were fulfilled in 70 patients: in 69 patients with FBN1 mutations and 1 patient with another CTD. Recommended treatment (beta-blocker, angiotensin receptor blocker) was taken by 63% of patients. CONCLUSIONS: In this cohort a high percentage of patients fulfilling the revised Ghent criteria for MFS underwent genetic testing, often leading to or confirming the diagnosis of MFS. Other CTDs could be discriminated best by genetic testing. With respect to the diagnosis of MFS and related CTDs, the usefulness of the systemic score is limited, showing the importance of genetic testing, which enabled definitive diagnosis in 95% of tested patients. Patient education on medical treatment still has to be improved. (Trial registration no: KEK-ZH-Nr. 2013-0241).

Cerebrovascular accidents in Ebstein's anomaly.

INTRODUCTION: Mechanisms and risk factors for cerebrovascular accidents (CVAs) in Ebstein's anomaly (EA) are not well understood; hence, we aimed to clarify these in a large cohort of EA patients. METHODS: Patients with a confirmed diagnosis of EA were retrospectively reviewed. Baseline characteristics were compared between patients with and without a prior history of CVA using logistic regression modeling. Cox regression analysis was used to identify predictors of CVA following initial evaluation. CVA incidence from birth and following tricuspid valve surgery were estimated using the Kaplan-Meier method. RESULTS: Nine hundred sixty-eight patients (median age 21.1 years, 41.5% male) were included, in which, 87 patients (9.0%) had a history of CVA (54 strokes, 33 transient ischemic attacks; 5 associated with brain abscesses) prior to their initial evaluation. The odds of atrial septal defect/patent foramen ovale (odds ratio [OR] 4.91; 95% CI 2.60-21.22; p = .0002) and migraines/headaches (OR 2.38; 95% CI 1.40-4.04; p = .0013) but not atrial arrhythmias (OR 0.75; 95% CI 0.44-1.30; p = .31) were significantly higher among patients with prior CVA following multivariable adjustment. Seventeen patients experienced CVA following initial evaluation; no examined variables including atrial arrhythmias (HR 2.38; 0.91-6.19; p = .076) were predictive of CVA risk. The 10-year, 50-year, and 70-year incidences of CVA were 1.4%, 15.9%, and 23.5%, respectively, with paradoxical embolism heavily implicated. CONCLUSION: Patients with EA are at substantive risk for CVA. Histories of migraines/headaches and interatrial shunts should prompt concern for paradoxical embolic CVAs. This has significant implications for all patients with atrial-level shunting.

Sudden death in patients with Ebstein anomaly.

Aims: Ventricular dysfunction or structural alteration of either ventricle is a well-established risk factor for sudden death (SD). Ebstein anomaly (EA) can present with both right and left heart abnormalities; however, predictors of SD have not been described. We therefore sought to characterize the incidence and risk factors of SD among a large cohort of patients with EA. Methods and results: All EA patients who underwent evaluation at a high-volume institution over a 4-decade period were retrospectively reviewed. Clinical variables, cardiovascular surgical procedure(s), and cause of death were recorded. Sudden death incidence from birth and following tricuspid valve (TV) surgery were estimated using the Kaplan-Meier method. Cox regression analysis was used to identify clinical and surgical predictors of SD. The cohort comprised of 968 patients [mean age 25.3 years, 41.5% male; 79.8% severe EA, 18.6% accessory pathway, 0.74% implantable cardioverter-defibrillator (ICD) placement]. The 10-, 50-, and 70-year cumulative incidences of SD from birth were 0.8%, 8.3%, and 14.6%, respectively. Prior ventricular tachycardia [hazard ratio (HR) 6.37, P < 0.001)], heart failure (HR 5.64, P < 0.001), TV surgery (HR 5.94, P < 0.001), syncope (HR 2.03, P = 0.019), pulmonic stenosis (HR 3.42, P = 0.001), and haemoglobin > 15 g/dL (HR 2.05, P = 0.026) were multivariable predictors of SD. In a similar subgroup analysis of patients who underwent TV surgery, all of the above factors except syncope were significantly associated with post-operative SD on multivariable analysis. Conclusion: Patients with EA are at significant risk for SD. Key clinical SD predictors identified can aid in risk stratification and potentially guide primary prevention ICD implantation.

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.

Dilatation and Dysfunction of the Neo-aortic Root and in 76 Patients After the Ross Procedure.

Pulmonary autograft replacement (Ross procedure) is used as an alternative to prosthetic aortic valve replacement patients with aortic valve disease. There are limited data on incidence and risk factors for dilatation and dysfunction of the neo-aortic after the Ross procedure. Ross procedure was performed in 100 patients at our institution between 1993 and 2011. In 76 patients, complete follow-up data were available. Their median age at surgery was 16 (0.4-58) years (76 % males; 95 % with congenital aortic valve disease). Median follow-up duration was 5.2 years (0.3-16.0 years). We analyzed their clinical and echocardiographic follow-up to identify possible risk factors for neo-aortic root dilatation and dysfunction. Ross procedure included reduction plasty of the native ascending aorta in 25 % of patients. During follow-up, 21 patients (28 %) developed neo-aortic root dilatation, 38 patients (50 %) dilatation oft the native ascending aorta and 7 patients (9 %) at least moderate neo-aortic regurgitation. Univariate risk factors for neo-aortic root dilatation were preoperative aortic regurgitation (p = 0.04), concomitant reduction plasty of the ascending aorta (p = 0.009) and a longer duration of follow-up (p = 0.005). Younger age at surgery was associated with dilatation of the ascending aorta (p = 0.03). Reoperation on the neo-aortic root because of severe dilatation was necessary in 6 patients (8 %), where 2 patients had at least moderate neo-aortic root regurgitation. Neo-aortic root and aortic dilatation are common after the Ross procedure. This is often combined with neo-aortic valve dysfunction. Close follow-up of these patients is mandatory.

Safety and efficacy of the nMARQ catheter for paroxysmal and persistent atrial fibrillation.

AIMS: Circular irrigated radiofrequency ablation using the nMARQ catheter has recently been introduced for the treatment of atrial fibrillation (AF). The aim of this study is to report the safety and efficacy of catheter ablation using this technology in patients with paroxysmal and persistent AF. METHODS AND RESULTS: The data of a prospective registry describing the experience of a single operator using this technology on 327 consecutive patients were analysed. The mean procedure time was 69 ± 22 min for paroxysmal AF (n = 228) and 75 ± 23 min for persistent AF (n = 97). Follow-up was available for 206 (63%) patients for 6 ± 5 months (range 1-23, median 3.3). Single procedure success off antiarrhythmic drugs was 75% in paroxysmal AF and 52% in persistent AF. Including the 5% redo cases and those on antiarrhythmic medication, freedom from AF was documented in 90 and 83% of paroxysmal and persistent AF patients, respectively. There were no serious complications in the first 325 patients, but the last two consecutive patients (0.6%) developed atrio-oesophageal fistulas and had a fatal outcome. The catheter has been recalled from market. CONCLUSION: The nMARQ catheter is a highly effective tool for treatment of paroxysmal and persistent AF. Nevertheless, the occurrence of life-threatening oesophageal fistulas is of major concern and requires further investigation.

Left Ventricular Noncompaction Diagnosis and Management Relevant to Pre-participation Screening of Athletes.

Left ventricular noncompaction (LVNC) has been extensively studied over the last years, and an increasing number of cases have been reported worldwide, with a large proportion comprising young and asymptomatic subjects, including athletes. The current epidemic of LVNC is likely the consequence of several causes, that is, the increased awareness of the disease and the refined cardiovascular imaging techniques. The current diagnostic methods, based uniquely on definition of morphologic findings, do not always resolve the overlap of a physiological myocardial architecture comprising a prominent trabecular pattern from a mild phenotypic expression of the real disease. Appropriate criteria for identification and management of LVNC in athletes have, therefore, become a novel challenge for cardiologists and sport physicians, who are required to solve the question of diagnosis and appropriate management in the setting of pre-participation cardiovascular screening. Indeed, although it is important to timely identify a true myocardial disease, to reduce the burden of adverse cardiac event in a young athlete, in contrast, a misdiagnosis of LVNC may lead to unwarranted restriction of the athlete lifestyle, with detrimental psychological, social, and economic consequences. This review report has been planned, therefore, to help physicians in diagnosing and managing athletes presenting with a morphologic pattern suggestive of LVNC with specific focus on criteria for advising sport participation.

Medical treatment of aortic aneurysms in Marfan syndrome and other heritable conditions.

Thoracic aortic aneurysms can be triggered by genetic disorders such as Marfan syndrome (MFS) and related aortic diseases as well as by inflammatory disorders such as giant cell arteritis or atherosclerosis. In all these conditions, cardiovascular risk factors, such as systemic arterial hypertension, may contribute to faster rate of aneurysm progression. Optimal medical management to prevent progressive aortic dilatation and aortic dissection is unknown. ß-blockers have been the mainstay of medical treatment for many years despite limited evidence of beneficial effects. Recently, losartan, an angiotensin II type I receptor antagonist (ARB), has shown promising results in a mouse model of MFS and subsequently in humans with MFS and hence is increasingly used. Several ongoing trials comparing losartan to ß-blockers and/or placebo will better define the role of ARBs in the near future. In addition, other medications, such as statins and tetracyclines have demonstrated potential benefit in experimental aortic aneurysm studies. Given the advances in our understanding of molecular mechanisms triggering aortic dilatation and dissection, individualized management tailored to the underlying genetic defect may be on the horizon of individualized medicine. We anticipate that ongoing research will address the question whether such genotype/pathogenesis-driven treatments can replace current phenotype/syndrome-driven strategies and whether other forms of aortopathies should be treated similarly. In this work, we review currently used and promising medical treatment options for patients with heritable aortic aneurysmal disorders.

Increased risk of possible paradoxical embolic events in adults with ebstein anomaly and severe tricuspid regurgitation.

OBJECTIVE: Determine incidence and risk factors for possible paradoxical embolic events in patients who have Ebstein anomaly with severe tricuspid regurgitation. DESIGN: Retrospective study of clinical and imaging data. SETTING: Tertiary care center. PATIENTS: Patients undergoing clinical evaluation and echocardiography prior to cardiac surgery for Ebstein anomaly (1975-2010) performed at age ≥ 40 years. RESULTS: Mean age of 128 patients (81 female) was 53 ± 9 years. All had severe tricuspid regurgitation. Twenty-four (19%) had previous cardiac surgery (at <40 years), including 17 for interatrial shunt closure. Most (112 [88%]) had New York Heart Association functional class III/IV heart failure; 84 (66%) had interatrial shunting (58 had an atrial septal defect and 29 had a patent foramen ovale [3 had both]). During their lifetime, 29 patients (23%) had a history of ≥1 possible paradoxical embolic events (stroke or transient ischemic attack, brain abscess, or myocardial infarction). The best predictors of preoperative possible paradoxical embolic events were an atrial septal defect (P = .002) and older age at surgery (P = .007). There was no association of possible paradoxical embolic events with cardiovascular risk factors (hypertension, dyslipidemia, smoking, or family history of coronary artery disease) (all P ≥ .3) or atrial fibrillation (P = .69). Median age at occurrence of paradoxical embolism was 49 (range, 1.5-74 years). CONCLUSIONS: Possible paradoxical embolic events are common in adults with Ebstein anomaly and severe tricuspid regurgitation and are strongly associated with atrial septal defect. In patients with atrial septal defect or patent foramen ovale, shunt closure should be considered to reduce risk of possible paradoxical embolic events.

Heart transplantation in congenital heart disease: in whom to consider and when?

Due to impressive improvements in surgical repair options, even patients with complex congenital heart disease (CHD) may survive into adulthood and have a high risk of end-stage heart failure. Thus, the number of patients with CHD needing heart transplantation (HTx) has been increasing in the last decades. This paper summarizes the changing etiology of causes of death in heart failure in CHD. The main reasons, contraindications, and risks of heart transplantation in CHD are discussed and underlined with three case vignettes. Compared to HTx in acquired heart disease, HTx in CHD has an increased risk of perioperative death and rejection. However, outcome of HTx for complex CHD has improved over the past 20 years. Additionally, mechanical support options might decrease the waiting list mortality in the future. The number of patients needing heart-lung transplantation (especially for Eisenmenger's syndrome) has decreased in the last years. Lung transplantation with intracardiac repair of a cardiac defect is another possibility especially for patients with interatrial shunts. Overall, HTx will remain an important treatment option for CHD in the near future.

Risk of death in long-term follow-up of patients with apical hypertrophic cardiomyopathy.

Apical hypertrophic cardiomyopathy (HC) has been considered a "benign" form of HC, with limited data on long-term outcome. We compared apical HC patients with a non-HC, age- and gender-matched Minnesota white population to identify outcomes and prognostic factors. Between 1976 and 2006, 193 patients (62% men) with apical HC were seen at our clinic. Their most recent echocardiographic examinations were reviewed. Mean ± SD age at first presentation was 58 ± 17 years. A family history of HC or sudden cardiac death (SCD) was reported by 43 patients (22%); coronary artery disease was known in 22 (11%). An apical pouch was present in 29 patients, including an apical aneurysm in 6 and apical dilatation with hypokinesis in 23. Median follow-up (187 patients [97%]) was 78 months (range, 1-350). Death from all causes occurred in 55 patients (29%; 33 women) at a mean age of 72 years (range, 20-92). During follow-up, more women had heart failure (p = 0.001), atrial fibrillation (p = 0.009), or died (p <0.001) than men. Survival was worse than expected (p = 0.001); the observed versus expected 20-year survival was 47% versus 60%. SCD, resuscitated cardiac arrest, and/or defibrillator discharge was observed in 11 patients (6%) during follow-up. Multivariate predictors of decreased survival were higher age at baseline (p <0.001), female gender (p <0.001), and atrial fibrillation at baseline (p = 0.06). In conclusion, apical HC in this population was associated with increased mortality, especially in women. Because apical HC is less benign than previously suspected, careful longitudinal care is warranted.

Management and outcome of Ebstein's anomaly in children.

OBJECTIVES: To assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly. BACKGROUND: Data on long-term outcome of children with Ebstein's anomaly are scarce. METHODS: Retrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention. RESULTS: A total of 42 patients were diagnosed with Ebstein's anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1-16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%. CONCLUSION: In children, Ebstein's anomaly is usually diagnosed in the first year of age. Even though children with Ebstein's anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcome.

Left ventricular non-compaction: prevalence in congenital heart disease.

INTRODUCTION: Left ventricular non-compaction cardiomyopathy (LVNC) is a rare cardiomyopathy, originally described as an isolated disease without other structural cardiac abnormalities. The aim of this study was to explore the prevalence of LVNC among adults with different types of congenital heart disease. METHODS: From our databases we identified adults with congenital heart disease who fulfilled diagnostic criteria for LVNC. We report frequencies of associated congenital cardiac defects and the prevalence of LVNC among patients with different congenital heart defects. RESULTS: From a total of 202 patients with LVNC, 24 patients (12%; mean age 32 ± 11 years, 19 males) had additional congenital cardiac defects. Associated defects were left ventricular outflow tract abnormalities in 11 patients (46%), including 7 uni- or bicuspid aortic valves; two aortic coarctations; one diffuse aortic hypoplasia and one subaortic stenosis, Ebstein anomaly in 6 patients (25%), tetralogy of Fallot in two (8%), and double outlet right ventricle in one patient (4%). In our cohort, the prevalence of LVNC was highest among patients with Ebstein anomaly (6/40, 15%), followed by aortic coarctation (2/60, 3%), tetralogy of Fallot (3/129, 2%) and uni- or bicuspid aortic valves (7/963, 1%). CONCLUSION: In adults, various forms of congenital heart disease are associated with LVNC, particularly stenotic lesions of the left ventricular outflow tract, Ebstein anomaly, and tetralogy of Fallot. In the future, studying these patients in more depth may provide a better understanding of the interplay between genetic and hemodynamic factors that lead to the phenotype of LVNC.

Outcome of cardiac surgery in patients 50 years of age or older with Ebstein anomaly: survival and functional improvement.

OBJECTIVES: This study sought to analyze the presentation, surgical procedures, and outcomes in patients ≥ 50 years of age with Ebstein anomaly (EA). BACKGROUND: Data on management and surgical outcomes in older patients with EA are limited. METHODS: Operative and clinical data from patients with EA ≥ 50 years of age undergoing cardiac surgery at our center between October 1980 and January 2010 were analyzed. RESULTS: During the study period, 89 procedures were performed in 81 patients with EA (63% women; mean [range] age 59 [50 to 79] years). Pre-operative symptoms included palpitations (n = 69), edema (n = 30), and previous stroke/transient ischemic attack (n = 21). Seventy-six patients (85%) had functional class III or IV symptoms, and 13 (16%) had previous cardiac surgery. Tricuspid valve surgery was necessary in 87 of the 89 procedures (98%): replacement in 65 (73%) and repair in 22 (25%). Three early deaths occurred (4%). On long-term follow-up (available in 73 of 78 early survivors), 63 patients (89%) had improved functional class and 13 patients died (19%). The 20-year survival was 65% versus 74% for age- and sex-matched controls (p = 0.001). The best predictors of late death were lack of post-operative improvement and older age at surgery. CONCLUSIONS: Although cardiac surgery in patients with EA ≥ 50 years of age was often complex, early mortality was low (4%) when surgery was performed at an experienced center. Long-term survival was good, although less than expected. These data suggested that surgery in older patients with EA may have to be performed earlier.

Prospective comparison of echocardiography versus cardiac magnetic resonance imaging in patients with Ebstein's anomaly.

Ebstein's anomaly (EA) is primarily diagnosed by echocardiography. The purpose of this study was to compare echocardiography and magnetic resonance imaging (MRI) in EA. Data from cardiac MRI and echocardiography were prospectively collected from 16 patients with EA. Imaging data also were compared with intraoperative findings. Information provided by MRI and echocardiography were comparable for left ventricular size and function, tricuspid valve repairability, qualitative assessment of right-sided cavities, and visibility of septal and anterior tricuspid valve leaflets. The posterior tricuspid valve leaflet and tricuspid valve fenestrations were better visualized with MRI; associated heart defects were equally recognized, apart from small shunts that tended to be more readily diagnosed with echocardiography. Quantification of right-cavity size and right ventricular ejection fraction was possible only with cardiac MRI. The degree of tricuspid valve regurgitation was underestimated by echocardiography (2 patients) and by MRI (4 patients) when compared with intraoperative assessment. When evaluating EA, echocardiography and MRI provide complementary data. For visualization of the posterior tricuspid valve leaflet and quantitative assessment of right ventricular size and function, MRI is preferable. For appropriate risk stratification in EA, both MRI and echocardiography should be performed before cardiac surgery.

Predictors of adverse outcome in adolescents and adults with isolated left ventricular noncompaction.

Isolated left ventricular noncompaction is a rare form of primary cardiomyopathy. Although increasingly diagnosed, data on the outcomes are limited. To define the predictors of adverse outcomes, we performed a retrospective analysis of a prospectively defined cohort of consecutive patients (age >14 years) diagnosed with left ventricular noncompaction at a single center. The baseline characteristics included presentation with a cardiovascular complication (i.e., decompensated heart failure, systemic embolic event, or sustained ventricular arrhythmia). The primary end point was survival free from cardiovascular death or transplantation. The predictors of survival were evaluated using the Kaplan-Meier method and Cox proportional hazards analysis. A total of 115 patients were included, 77% of whom were symptomatic at diagnosis. Compared to the asymptomatic patients, the symptomatic patients were significantly older and had larger left ventricular cavities and worse left ventricular ejection fraction. Of the 115 patients, 49 (43%) presented with a cardiovascular complication. During a median follow-up of 2.7 years (range 0.1 to 19.4), none of the asymptomatic patients died or underwent transplantation compared to 31% (27 of 88) of the symptomatic patients (p = 0.001). The major determinants of cardiovascular death or transplantation were presentation with a cardiovascular complication (hazard ratio 20.6, 95% confidence interval 4.9 to 87.5, p <0.0001) or New York Heart Association class III or greater (hazard ratio 8.8, 95% confidence interval 3.2 to 24.0, p <0.0001). Left ventricular dilation and systolic dysfunction were less strong predictors. In conclusion, in patients with left ventricular noncompaction, New York Heart Association class III or greater and cardiovascular complications at presentation are strong predictors for adverse outcome.