Christine H. Attenhofer Jost

Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis

OBJECTIVES We sought to describe characteristics and outcome in adults with isolated ventricular noncompaction (IVNC). BACKGROUND Isolated ventricular noncompaction is an unclassified cardiomyopathy due to intrauterine arrest of compaction of the loose interwoven meshwork. Knowledge regarding diagnosis, morbidity and prognosis is limited. METHODS Echocardiographic criteria for IVNC include-in the absence of significant heart lesions-segmental thickening of the left ventricular myocardial wall consisting of two layers: a thin, compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep recesses. Thirty-four adults (age >16 years, 25 men) fulfilled the diagnostic criteria and were followed prospectively. RESULTS At diagnosis, mean age was 42 + 17 years, and 12 patients (35%) were in New York Heart Association class III/IV. Left ventricular end-diastolic diameter was 65 + 12 mm and ejection fraction 33 + 13%. Apex and/or midventricular segments of both the inferior and lateral wall were involved in >80% of patients. Follow-up was 44 + 40 months. Major complications were heart failure in 18 patients (53%), thromboembolic events in 8 patients (24%) and ventricular tachycardias in 14 patients (41%). There were 12 deaths: sudden in six, end-stage heart failure in four and other causes in two patients. Four patients underwent heart transplantation. Automated cardioverter/defibrillators were implanted in four patients. CONCLUSIONS Diagnosis of IVNC by echocardiography using strict criteria is feasible. Its mortality and morbidity are high, including heart failure, thrombo-embolic events and ventricular arrhythmias. Risk stratification includes heart failure therapy, oral anticoagulation, heart transplantation and implantation of an automated defibrillator/cardioverter. As IVNC is a distinct entity, its classification as a specific cardiomyopathy seems to be more appropriate.

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

Left Heart Lesions in Patients With Ebstein Anomaly

OBJECTIVE To identify the incidence of left heart abnormalities in patients with Ebstein anomaly, recognizing that left-sided lesions in this patient group have been overlooked. PATIENTS AND METHODS According to the echocardiography database at the Mayo Clinic in Rochester, Minn, 106 consecutive patients with Ebstein anomaly underwent echocardiography between July 1, 2001, and February 28, 2003. Clinical data as well as electrocardiographic and echocardiographic reports and images were reviewed. RESULTS Ebstein anomaly was severe in 76 patients (72%). Previous tricuspid valve surgery was reported in 46 patients (43%), and previous closure of an atrial septal defect or patent foramen ovale was reported in 34 patients (32%). Left ventricular (LV) myocardial changes resembling noncompaction occurred in 19 patients (17.9%), LV systolic dysfunction in 7 patients (7%), LV diastolic dysfunction in 34 (36%) of 95 patients, and LV dilatation in 4 patients (4%). Additional left-sided cardiac lesions included mitral valve prolapse in 16 patients (15%), bicuspid aortic valve in 8 (8%), mitral valve dysplasia in 4 (4%), and ventricular septal defect in 8 (8%). Wolff-Parkinson-White syndrome occurred in 22 patients (21%). The QRS axis tended to be different in LV noncompaction with a mean +/- SD axis of 12 degrees +/- 74 degrees vs 36 degrees +/- 66 degrees overall (P=.08). Otherwise, there were no differences in clinical or surgical data between the groups with normal and abnormal LV myocardium. CONCLUSIONS In patients with Ebstein anomaly, left heart abnormalities involving the myocardium or valves were observed in 39% of patients. Ebstein anomaly should not be regarded as a disease confined to the right side of the heart.

Sinus Venosus Atrial Septal Defect Long-Term Postoperative Outcome for 115 Patients

Background—Sinus venosus atrial septal defect (SVASD) differs from secundum atrial septal defect by its atrial septal location and its association with anomalous pulmonary venous connection (APVC). Data on long-term outcome after surgical repair are limited. Methods and Results—We reviewed outcomes of 115 patients (mean age±SD 34±23 years) with SVASD who had repair from 1972 through 1996. APVC was present in 112 patients (97%). Early mortality was 0.9%. Complete follow-up was obtained for 108 patients (95%) at 144±99 months. Symptomatic improvement was noted in 83 patients (77%), and deterioration was noted in 17 patients (16%). At follow-up, 7 (6%) of 108 patients had sinus node dysfunction, a permanent pacemaker, or both, and 15 (14%) of 108 patients had atrial fibrillation. Older age at repair was predictive of postoperative atrial fibrillation (P=0.033). No reoperations were required during follow-up. Survival was not different from expected for an age- and sex-matched population. Clinical improvement was more common with older age at surgery (P=0.014). Older age at repair (P=0.008) and preoperative New York Heart Association class III or IV (P=0.038) were independent predictors of late mortality. Conclusions—Operation for SVASD is associated with low morbidity and mortality, and postoperative subjective clinical improvement occurs irrespective of age at surgery. Postoperative atrial fibrillation appears to be related to older age at operation. SVASD repair achieves survival similar to that of a matched population and should be considered whenever repair may impact survival or symptoms.

Echocardiography in the evaluation of systolic murmurs of unknown cause.

PURPOSE Systolic murmurs are common, and it is important to know whether physical examination can reliably determine their cause. Therefore, we prospectively assessed the diagnostic accuracy of a cardiac examination in patients without previous echocardiography who were referred for evaluation of a systolic murmur. SUBJECTS AND METHODS In 100 consecutive adults (mean [+/- SD] age of 58 +/- 22 years) who were referred for a systolic murmur of unknown cause, the diagnostic accuracy of the cardiac examination by cardiologists (without provision of clinical history, electrocardiogram, or chest radiograph) was compared with the results of echocardiography. RESULTS The echocardiographic findings included a normal examination (functional murmur) in 21 patients, aortic stenosis in 29 patients, mitral regurgitation in 30 patients, left or right intraventricular pressure gradient in 11 patients, mitral valve prolapse in 11 patients, ventricular septal defect in 4 patients, hypertrophic obstructive cardiomyopathy in 3 patients, and associated aortic regurgitation in 28 patients. In 28 (35%) of the 79 patients with organic heart disease, more than one abnormality was found; combined aortic and mitral valve disease was the most frequent combination (n = 22). The sensitivity of the cardiac examination was acceptable for detecting ventricular septal defect (100% [4 of 4]), isolated mitral regurgitation (88% [26 of 36]), aortic stenosis (71% [21 of 29]), and a functional murmur (67% [14 of 21]), but not for intraventricular pressure gradients (18% [2 of 11]), aortic regurgitation (21% [6 of 28]), combined aortic and mitral valve disease (55% [6 of 11]), and mitral valve prolapse (55% [12 of 22]). In 6 patients, the degree of aortic stenosis was misjudged on the clinical examination, mainly because of a severely diminished left ventricular ejection fraction. Significant heart disease was missed completely in only 2 patients. CONCLUSION In adults with a systolic murmur of unknown cause, a functional murmur can usually be distinguished from an organic murmur. However, the ability of the cardiac examination to assess the exact cause of the murmur is limited, especially if more than one lesion is present. Thus, echocardiography should be performed in patients with systolic murmurs of unknown cause who are suspected of having significant heart disease.

Long term follow up of prosthetic valve endocarditis: what characteristics identify patients who were treated successfully with antibiotics alone?

OBJECTIVE To identify predictors for the safe use of antibiotic treatment without reoperation in patients with prosthetic valve endocarditis. SETTING Retrospective study in a tertiary care centre. SUBJECTS AND DESIGN All 49 episodes of definite prosthetic valve endocarditis (Duke criteria) diagnosed at one institution between 1980 to 1997 were analysed. Ten episodes (20%) were treated with antibiotics only (antibiotic group) and 39 episodes (80%) with combined antibiotic and surgical treatment (surgery group). The analysis included detailed study of hospital records and data on long term follow up which were obtained in all patients by a questionnaire or telephone contact with physician or patient. The length of follow up (mean (SD)) was 41 (32) months in the antibiotic group and 45 (24) months in the surgery group (NS). Long term survival was estimated by the Kaplan–Meier method and compared by the log-rank test. RESULTS There was no significant difference in age, history of previous endocarditis, number of previous heart operations, vegetations, emboli, type of prosthesis, or percentage of early prosthetic valve endocarditis and positive blood cultures between the two groups. In the antibiotic group, there were more enterococcal (50%; p = 0.005) and in the surgery group more staphylococcal infections (55%; p = 0.048). Annular abscesses (p < 0.0001) and aortoventricular dehiscence (p = 0.02) were more common in the surgery group. No patient in the antibiotic group had heart failure. Long term follow up showed no significant difference between the surgery and antibiotic groups regarding late mortality (14% v 18%) and five year rates of recurrent endocarditis (14%v 16%), event related mortality (14% v 3%, log-rank test), and the need for reoperation (14% v 19%; log-rank test). The only patient with conservatively treated staphylococcal prosthetic valve endocarditis died after reoperation for recurrence. CONCLUSIONS Haemodynamically stable patients with non-staphylococcal prosthetic valve endocarditis who are carefully supervised can be treated with antibiotics alone without an increased rate of reinfection, reoperation, or death.

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.

Spectrum of Reoperations After Repair of Aortic Coarctation: Importance of an Individualized Approach Because of Coexistent Cardiovascular Disease

OBJECTIVE To determine the indications for and spectrum of late reoperations in adults who had previously undergone coarctation repair. PATIENTS AND METHODS We reviewed clinical, cardiac catheterization, and echocardiographic data and criteria for reoperation, surgical procedures, and outcome in 43 patients who underwent 54 reoperations between 1972 and 1996. RESULTS Of the reoperations for recoarctation or associated cardiovascular disease (or both), 20% were performed in asymptomatic patients and 80% in symptomatic patients. Associated cardiovascular disease included bicuspid aortic valve in 36 patients (84%), aortic arch hypoplasia in 12 (28%), true or false aortic aneurysm in 6 (14%), mitral valve disease in 6 (14%), and subvalvular aortic stenosis in 5 (12%). Surgical procedures included 22 recoarctation repairs and 32 other cardiovascular interventions. Simultaneous repair of recoarctation and associated cardiovascular disease was performed as a single-stage repair in 5 reoperations through a median sternotomy using an extra-anatomic, ascending-to-descending aortic bypass, with no complications. One patient died (surgical mortality, 1.9%) of preexisting severe pulmonary vascular obstructive disease. CONCLUSIONS After coarctation repair, associated cardiovascular diseases are the most common cause for reoperation. An individualized surgical approach is important and may range from valve replacement or recoarctation surgery to extra-anatomic bypass combined with other cardiovascular procedures, enabling simultaneous repair of recoarctation and associated lesions. Despite complex surgical techniques and multiple reoperations, morbidity and mortality were low in our series.

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.

Mechanical Complications after Myocardial Infarction Reliably Predicted Using C-Reactive Protein Levels and Lymphocytopenia

We assessed the accuracy of C-reactive protein (CRP) levels and lymphocyte counts to predict a mechanical complication (MC) after myocardial infarction (MI). Within 10 years, we identified 36 patients with 39 echocardiographically confirmed MC within 30 days of MI: ventricular septal defect (17 cases), papillary muscle rupture (10 cases), and left ventricular free wall rupture (12 cases). They were compared to 41 controls with an uncomplicated hospital course after MI. Peak CRP levels and minimum relative lymphocyte counts obtained within 96 h of the acute MI (AMI) and before diagnosis of the complication were compared with clinical parameters. Prior to the MC, peak CRP levels were significantly higher (p < 0.001) and relative lymphocyte counts lower (p < 0.001) than in controls while creatine kinase levels did not differ (p = nonsignificant). Using multivariate logistic regression, the following score was identified to have excellent prognostic significance for MC: CRP (mg/l) – 10 × Lyc (%). The area under the receiver-operating characteristic curve was 0.90 ± 0.05 (p < 0.001). Combined use of CRP levels and relative lymphocyte counts may be helpful in accurately predicting an MC after AMI and should therefore be routinely assessed.