Beatriz Bouzas

Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication.

Background—Although some patients with adult congenital heart disease (ACHD) report limitations in exercise capacity, we hypothesized that depressed exercise capacity may be more widespread than superficially evident during clinical consultation and could be a means of assessing risk. Methods and Results—Cardiopulmonary exercise testing was performed in 335 consecutive ACHD patients (age, 33±13 years), 40 non–congenital heart failure patients (age, 58±15 years), and 11 young (age, 29±5 years) and 12 older (age, 59±9 years) healthy subjects. Peak oxygen consumption (peak &OV0312;o2) was reduced in ACHD patients compared with healthy subjects of similar age (21.7±8.5 versus 45.1±8.6; P<0.001). No significant difference in peak &OV0312;o2 was found between ACHD and heart failure patients of corresponding NYHA class (P=NS for each NYHA class). Within ACHD subgroups, peak &OV0312;o2 gradually declined from aortic coarctation (28.7±10.4) to Eisenmenger (11.5±3.6) patients (P<0.001). Multivariable correlates of peak &OV0312;o2 were peak heart rate (r=0.33), forced expiratory volume (r=0.33), pulmonary hypertension (r=−0.26), gender (r=−0.23), and body mass index (r=−0.19). After a median follow-up of 10 months, 62 patients (18.5%) were hospitalized or had died. On multivariable Cox analysis, peak &OV0312;o2 predicted hospitalization or death (hazard ratio, 0.937; P=0.01) and was related to the frequency and duration of hospitalization (P=0.01 for each). Conclusions—Exercise capacity is depressed in ACHD patients (even in allegedly asymptomatic patients) on a par with chronic heart failure subjects. Lack of heart rate response to exercise, pulmonary arterial hypertension, and impaired pulmonary function are important correlates of exercise capacity, as is underlying cardiac anatomy. Poor exercise capacity identifies ACHD patients at risk for hospitalization or death.

Significado clínico del realce tardío de gadolinio con resonancia magnética en pacientes con miocardiopatía hipertrófica

Introduccion y objetivos La fibrosis miocardica puede ser detectada en la miocardiopatia hipertrofica (MCH) mediante resonancia magnetica cardiaca (RM) con realce tardio de gadolinio (RT). Analizamos la relacion entre la extension del RT y la morfologia y funcion del ventriculo izquierdo (VI) y los datos clinicos. Metodos Estudiamos con RM a 104 pacientes diagnosticados de MCH. Se obtuvieron secuencias de cine-RM y secuencias de realce tardio. Resultados Cincuenta pacientes presentaron RT (48%; rango: 1-11 segmentos). La extension del RT se correlaciono positivamente con el grosor maximo (r = 0,53; p Conclusiones La extension del RT refleja una mayor expresion de esta enfermedad. Se asocia con un dano miocardico mas severo (menor fraccion de eyeccion y mayor numero de segmentos hipocineticos) y con parametros clinicos adversos (edad mas joven en el momento del diagnostico, hipertrofia severa, TVNS y respuesta isquemica al ejercicio), lo que indica que podria vincularse al pronostico.

Clinical significance of late gadolinium enhancement on cardiovascular magnetic resonance in patients with hypertrophic cardiomyopathy

INTRODUCTION AND OBJECTIVES In patients with hypertrophic cardiomyopathy, myocardial fibrosis can be detected by late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging. We investigated the relationships between the extent of LGE, left ventricular morphology and function, and clinical characteristics. METHODS Both cine and gadolinium-enhanced magnetic resonance imaging were performed in 104 patients with hypertrophic cardiomyopathy. RESULTS Fifty patients (48%) showed LGE (range: 1-11 segments). The extent of LGE was positively correlated with maximum left ventricular wall thickness (r=0.53, P< .001), left ventricular mass (r=0.41, P< .001), and the number of hypokinetic segments (r=0.51, P< .001), and inversely correlated with ejection fraction (r=-0.32, P=.001), the magnitude of the subaortic gradient increase during exercise echocardiography (r=-0.26, P=.023), and age at diagnosis (r=-0.20, P=.04). Four of the five patients with an ischemic response on exercise echocardiography had > or =3 segments showing LGE (P=.003). Severe hypertrophy (i.e., > or =30 mm) and nonsustained ventricular tachycardia occurred more frequently as the number of LGE segments increased (P< .001 and P=.04, respectively). CONCLUSIONS Extensive LGE reflects greater disease expression. It is associated with more severe myocardial damage (i.e., a lower ejection fraction and a larger number of hypokinetic segments) and with adverse clinical characteristics (e.g., young age at diagnosis, severe hypertrophy, nonsustained ventricular tachycardia, and an ischemic response on exercise), suggesting that it may be closely linked to prognosis.

Prognostic value of exercise echocardiography in patients with left bundle branch block.

OBJECTIVES Our aim was to evaluate the role of exercise echocardiography for predicting outcome in a cohort of patients with left bundle branch block (LBBB). BACKGROUND Although the prognostic value of exercise echocardiography has been well established in several subgroups of patients, it has not been specifically assessed in patients with LBBB. METHODS Of the 8,050 patients who underwent treadmill exercise echocardiography, 618 demonstrated complete LBBB. Nine patients were lost to follow-up and 609 patients were included in this study. Wall motion score index (WMSI) was evaluated at rest and at peak exercise, and the difference (DeltaWMSI) was calculated. Ischemia was defined as the development of new or worsening wall motion abnormalities with exercise. End points were all-cause mortality and major cardiac events (including cardiac death, myocardial infarction, or cardiac transplantation). Mean follow-up was 4.6 +/- 3.4 years. RESULTS Mean age was 66 +/- 10 years, and 331 patients (54%) were men. A total of 177 patients (29%) developed ischemia with exercise. During follow-up, 124 deaths occurred, and 74 patients had a major cardiac event before any revascularization procedure. Patients with ischemia had a greater 5-year mortality rate (24.6% vs. 12.6%, p < 0.001) and 5-year major cardiac events rate (18.1% vs. 9.7%, p = 0.003). In multivariate analysis, DeltaWMSI remained an independent predictor of mortality (hazard ratio: 2.42, 95% confidence interval: 1.21 to 4.82, p = 0.012) and major cardiac events (hazard ratio: 3.38, 95% confidence interval: 1.30 to 8.82, p = 0.013). Exercise echocardiographic results also provided incremental value over clinical, resting echocardiographic, and treadmill exercise data for the prediction of mortality (p = 0.014) and major cardiac events (p = 0.017). CONCLUSIONS Exercise echocardiography provides significant prognostic information for predicting outcome in patients with LBBB. As compared to patients with normal exercise echocardiograms, patients with abnormal results are at increased risk of mortality and major cardiac events.

The shape and function of the right ventricle in Ebstein's anomaly

BACKGROUND Ebsteins anomaly involves both the right ventricle (RV) and tricuspid valve. METHODS The functional RV and tricuspid orifice were traced from magnetic resonance images in 29 adult Ebstein patients and 9 normal subjects and reconstructed for visualization and measurement of regional RV size, function, and shape at 20 cross sections, and inlet and outflow tract ejection fractions (EFs). RESULTS The RV in Ebsteins had RV dilation (end diastolic volume index 179 ± 69 vs. 84 ± 22 ml/m(2) in normals, p<0.001) and global dysfunction (EF 45 ± 8 vs. 55 ± 5% in normals, p<0.001). Longitudinal contraction was preserved (26 ± 13 vs. 26 ± 4 mm in normals) and correlated more weakly with EF than short axis fractional shortening (r=0.44 vs. r=0.71, p<0.05 for both). The apical region in Ebsteins RV was enlarged, rounded and contributed more than normal to the global stroke volume. However this contribution correlated inversely with global EF. In contrast slices in the basal region had normal cross sectional area and their function correlated directly with global EF. Inlet EF was depressed (46 ± 8% vs. 55 ± 6 in normals, p=.002); outflow tract EF was even more depressed (39 ± 14, p=0.019). CONCLUSION The three dimensional shape of the RV in adult Ebstein patients was demonstrated. The Ebsteins RV remodels in diverse regional patterns rather than following a shape continuum. Changes at the apex and base had opposing effects on function. Global EF was supported more by short axis than longitudinal contraction.

Pulmonary Arterial Hypertension in Adults With Congenital Heart Disease

Pulmonary arterial hypertension is a chronic, persistent elevation in pulmonary artery pressure without evidence of left heart failure. Pulmonary hypertension is common in patients with adult congenital heart disease and is usually the result of an increase of pulmonary blood flow through a large left to right shunt. This condition is progressive and patients are symptomatic and usually die between the third and fifth decades of life. To date, there is no standardized treatment for this condition and a general policy of non-intervention to avoid destabilization of the balanced physiology is recommended. Intravenous prostanoids have been shown to have an effect but they are invasive and associated with major side effects. Lung and combined heart and lung transplantation might be a therapeutic option for selected patients. However, donor shortage is a major issue. Oral advanced therapies have been recently shown to improve haemodynamics and survival in idiopathic pulmonary hypertension or in pulmonary hypertension related to scleroderma and may have a role in patients with pulmonary hypertension secondary to congenital heart disease.

Miocardiopatía dilatada familiar en pacientes trasplantados por miocardiopatía dilatada idiopática

Objective. To evaluate the prevalence, clinical features, and pattern of inheritance of familial dilated cardiomyopathy (DCM) in heart transplant patients. Patients and method. Patients with idiopathic DCM who had undergone heart transplantation were invited to participate. Patients with alcohol abuse were not excluded. A clinical evaluation, 12-lead ECG, echocardiogram, blood tests, and DNA extraction were performed in patients and relatives. Familial DCM was defined as the presence of at least one relative with idiopathic DCM. Possible familial DCM was considered when at least one relative had left ventricular enlargement (LVE) (> 112% predicted LVEDD). Results. One hundred and ninety-nine relatives of 43 families were studied. DCM was familial in 11 probands (25.6%) and possibly familial in 11 (25.6%). Fifteen relatives had DCM (7.5%), 26 (13.1%) LVE, and 5 (2.5%) hypertrophic cardiomyopathy. The pattern of inheritance was autosomal dominant in most families. Five probands (3 with familial DCM) had antecedents of consanguinity and possible recessive inheritance. Six probands (14%, 1 with familial DCM) had relatives with conduction system defects. Creatine kinase was moderately increased in 9 relatives (4.5%), 3 of them with LVE. Fifteen patients had at least moderate alcohol intake. Three of them had familial DCM (relatives without alcohol abuse) and 6 had possible familial DCM. Conclusions. The prevalence of familial DCM is high in patients who undergo heart transplant. Left ventricular enlargement, conduction system abnormalities, and elevated creatine kinase may be early markers of familial disease. Hypertrophic cardiomyopathy is present in some relatives of patients with idiopathic DCM. Familial DCM is present in patients with a previous diagnosis of alcoholic DCM.

Major adverse events and atrial tachycardia in Ebstein’s anomaly predicted by cardiovascular magnetic resonance

Objectives Patients with Ebstein’s anomaly of the tricuspid valve (EA) are at risk of tachyarrhythmia, congestive heart failure and sudden cardiac death. We sought to determine the value of cardiovascular magnetic resonance (CMR) for predicting these outcomes. Methods Seventy-nine consecutive adult patients (aged 37±15 years) with unrepaired EA underwent CMR and were followed prospectively for a median 3.4 (range 0.4–10.9) years for clinical outcomes, namely major adverse cardiovascular events (MACEs: sustained ventricular tachycardia/heart failure hospital admission/cardiac transplantation/death) and first-onset atrial tachyarrhythmia (AT). Results CMR-derived variables associated with MACE (n=6) were right ventricular (RV) or left ventricular (LV) ejection fraction (EF) (HR 2.06, 95% CI 1.168 to 3.623, p=0.012 and HR 2.35, 95% CI 1.348 to 4.082, p=0.003, respectively), LV stroke volume index (HR 2.82, 95% CI 1.212 to 7.092, p=0.028) and cardiac index (HR 1.71, 95% CI 1.002 to 1.366, p=0.037); all remained significant when tested solely for mortality. History of AT (HR 11.16, 95% CI 1.30 to 95.81, p=0.028) and New York Heart Association class >2 (HR 7.66, 95% CI 1.54 to 38.20, p=0.013) were also associated with MACE; AT preceded all but one MACE, suggesting its potential role as an early marker of adverse outcome (p=0.011). CMR variables associated with first-onset AT (n=17; 21.5%) included RVEF (HR 1.55, 95% CI 1.103 to 2.160, p=0.011), total R/L volume index (HR 1.18, 95% CI 1.06 to 1.32, p=0.002), RV/LV end diastolic volume ratio (HR 1.55, 95% CI 1.14 to 2.10, p=0.005) and apical septal leaflet displacement/total LV septal length (HR 1.03, 95% CI 1.00 to 1.07, p=0.041); the latter two combined enhanced risk prediction (HR 6.12, 95% CI 1.67 to 22.56, p=0.007). Conclusion CMR-derived indices carry prognostic information regarding MACE and first-onset AT among adults with unrepaired EA. CMR may be included in the periodic surveillance of these patients.

Spontaneous Left Atrial Hematoma Mimicking an Acute Aortic Syndrome The Utility of High-Resolution Computed Tomography

A 69-year-old man with no history of chest trauma was admitted to our institution complaining of blunt and severe central chest pain of cataclysmic onset radiating to the back. His medical history was remarkable only for a chronic atrial fibrillation treated with warfarin and amiodarone. On arrival, his ECG showed atrial fibrillation at 120 bpm with no signs of myocardial ischemia. Laboratory examination was uneventful except for an international normalized ratio of 7. With the presumptive diagnosis of acute aortic syndrome, a thoracoabdominal high-resolution contrast-enhanced computed tomography scan was performed that showed a large mass at the level of the left atrium (LA) and a moderate pericardial effusion (Figure 1 and Movie I in the online-only Data Supplement). Linear attenuation coefficients of the LA mass and pericardial fluid were suggestive of acute clot and blood (60 and 50 Hounsfield units, respectively). No signs of malignancy, pulmonary embolism, or aortic disease were found. During evaluation, our patients clinical condition …

Combined Aortic and Pulmonic Stenosis in an Octogenarian Findings From Echocardiography, Catheterization, and Pathology

Combined aortic and pulmonic stenosis is a very rare condition, with only a few patients reported in the literature.1 Moreover, most of those cases are children and young adults, and thus, the pathogenesis has been often considered congenital.2,3 We report an 81-year-old man with a diagnosis of severe aortic and pulmonic stenosis who underwent successful aortic and pulmonic valve replacement, and we comment on the echocardiographic and pathological findings and speculate on the most likely cause. To the best of our knowledge, this is the first report of this condition in an octogenarian patient. An 81-year-old man with a medical history of smoking, arterial hypertension, dyslipidemia, mild renal …