José M. Oliver

Discrete subaortic stenosis in adults: increased prevalence and slow rate of progression of the obstruction and aortic regurgitation☆

OBJECTIVES We sought to determine the prevalence and rate of progression of left ventricular outflow tract obstruction (LVOTO) and aortic regurgitation (AR) in adults with discrete subaortic stenosis (DSS). BACKGROUND Discrete subaortic stenosis is an uncommon form of LVOTO, with rapid hemodynamic progression in children, but the prevalence and rate of progression in adults have not been studied so far. METHODS The prevalence of DSS was determined in 2,057 consecutive adults diagnosed with congenital heart disease (CHD). The relationship between LVOTO on Doppler echocardiography and patient age was analyzed. Sequential changes in LVOTO and AR were determined for patients with two or more Doppler echocardiograms obtained with at least a two-year interval. RESULTS A total of 134 adults (mean age 31 +/- 17 years) were diagnosed with DSS. The prevalence was 6.5% for all adults with CHD. Sixty patients (44%) had other associated CHD. The mean age of 29 patients who had undergone an operation for DSS during their adult life (56 +/- 15 years) was significantly higher than that of 64 patients (27 +/- 13 years) who had not required a surgical intervention (p < 0.0001). A significant relationship between LVOTO and patient age (r = 0.61, p < 0.0001) was found: 21 +/- 16 mm Hg in patients <25 years old, 51 +/- 47 mm Hg for those between 25 and 50 years old, and 78 +/- 36 mm Hg for those >50 years old. The LVOTO increased from 39.2 +/- 28 to 46.8 +/- 34 mm Hg (p = 0.01) during a mean follow-up of 4.8 +/- 1.8 years in 25 patients. The slope of the change in LVOTO was 2.25 +/- 4.7 mm Hg per year of follow-up. Aortic regurgitation was detected by color Doppler imaging in 109 patients (81%), but it was hemodynamically significant in <20%. An increase in the mean degree of AR over time was not significant (baseline: 1.3 +/- 0.8; follow-up: 1.5 +/- 0.9; p = 0.096). CONCLUSIONS The prevalence of DSS is increasing in adults due to the greater number of repaired CHDs that develop into evolutive DSS. In contrast to infants and children, adults with DSS show a slow rate of LVOTO progression. Aortic regurgitation is a common but usually mild and nonprogressive consequence. The current indications for surgical intervention should be revised.

Pheochromocytoma-Related Cardiomyopathy Inverted Takotsubo Contractile Pattern

A 41-year-old woman with no history of cardiac disease or hypertension was admitted to the intensive care unit with acute headache, psychomotor agitation, diaphoresis, nausea, and vomiting. A cerebral computed tomography scan ruled out subarachnoid hemorrhage. The ECG showed sinus tachycardia with ST-segment depression in leads V3–V6, II, III, and aVF. The troponin I level was elevated. The patient continued to have progressive respiratory deterioration, which required mechanical ventilation 24 hours after admission. She also developed 6 episodes of electromechanical dissociation, with circulatory recovery after successful cardiopulmonary resuscitation. Transthoracic echocardiography revealed severe left ventricular dysfunction and a contractile abnormality, …A 41-year-old woman with no history of cardiac disease or hypertension was admitted to the intensive care unit with acute headache, psychomotor agitation, diaphoresis, nausea, and vomiting. A cerebral computed tomography scan ruled out subarachnoid hemorrhage. The ECG showed sinus tachycardia with ST-segment depression in leads V3–V6, II, III, and aVF. The troponin I level was elevated. The patient continued to have progressive respiratory deterioration, which required mechanical ventilation 24 hours after admission. She also developed 6 episodes of electromechanical dissociation, with circulatory recovery after successful cardiopulmonary resuscitation. Transthoracic echocardiography revealed severe left ventricular dysfunction and a contractile abnormality, …

Images in cardiovascular medicine. Pheochromocytoma-related cardiomyopathy: inverted Takotsubo contractile pattern.

A 41-year-old woman with no history of cardiac disease or hypertension was admitted to the intensive care unit with acute headache, psychomotor agitation, diaphoresis, nausea, and vomiting. A cerebral computed tomography scan ruled out subarachnoid hemorrhage. The ECG showed sinus tachycardia with ST-segment depression in leads V3–V6, II, III, and aVF. The troponin I level was elevated. The patient continued to have progressive respiratory deterioration, which required mechanical ventilation 24 hours after admission. She also developed 6 episodes of electromechanical dissociation, with circulatory recovery after successful cardiopulmonary resuscitation. Transthoracic echocardiography revealed severe left ventricular dysfunction and a contractile abnormality, …A 41-year-old woman with no history of cardiac disease or hypertension was admitted to the intensive care unit with acute headache, psychomotor agitation, diaphoresis, nausea, and vomiting. A cerebral computed tomography scan ruled out subarachnoid hemorrhage. The ECG showed sinus tachycardia with ST-segment depression in leads V3–V6, II, III, and aVF. The troponin I level was elevated. The patient continued to have progressive respiratory deterioration, which required mechanical ventilation 24 hours after admission. She also developed 6 episodes of electromechanical dissociation, with circulatory recovery after successful cardiopulmonary resuscitation. Transthoracic echocardiography revealed severe left ventricular dysfunction and a contractile abnormality, …

Predisposing conditions for atrial fibrillation in atrial septal defect with and without operative closure

The aims of this study were to determine the prevalence and predisposing conditions for atrial fibrillation (AF) in adults with atrial septal defect (ASD) and to evaluate the influence of age at surgical repair. The study population consisted of 286 adults with ASD (mean age 39.5 +/- 19 years). All patients had >or = 1 follow-up visit and a Doppler echocardiographic study. One hundred ninety-two of the patients underwent surgical closure 1 to 34 years before the study. Analyzed variables were entered into univariate (Mann-Whitney U) and multivariate (stepwise logistic regression) models to assess independent predictors for AF. The prevalence of AF was similar in surgically treated patients (15.6%) and in the nonsurgical group (13.8%) (p = 0.69). Multivariate analysis showed that current age (RR 1.9 per each decade of age, 95% confidence interval [CI] 1.3 to 2.7, p = 0.001), mitral regurgitation (RR 3.0 per each degree of regurgitation, 95% CI 1.6 to 5.8, p = 0.001), left atrial enlargement (RR 2.8 per each 10 mm increase in size, 95% CI 1.5 to 5.2, p = 0.001), and tricuspid regurgitation (RR 1.9 per each degree of regurgitation, 95% CI 1.0 to 3.7, p = 0.04) were independent predictors of AF; however, gender, anatomic type, defect size, Qp:Qs, pulmonary artery pressure, right ventricular dimension, left ventricular shortening fraction, and prior surgical repair were not related to late AF development. In the surgical group, age >25 years at the time of surgery was the only predictor for AF independent of age at the time of the study (p = 0.02).

Incidence and Predictors of Sudden Cardiac Arrest in Adults With Congenital Heart Defects Repaired Before Adult Life

Many adult survivors of repaired congenital heart disease (CHD) are at premature risk of death. Sudden cardiac arrest (SCA) is 1 of the leading causes of death but little is known about determinants for SCA in adults with repaired lesions. We sought to determine incidence and risk factors for SCA in a study population of 936 adults with previously repaired CHD who had completed follow-up at a single tertiary center during a mean period of 9 ± 7 years. Mean age at first examination in our institution was 21 ± 7 years. Diagnostic categories included tetralogy of Fallot (216), coarctation of the aorta (157), transposition complexes (99), single ventricle (55), and other CHD (409). During a total follow-up of 8,387 person-years, 22 patients (2.6 per 1,000 person-years) presented with SCA. Incidence of SCA varied widely between specific lesions; the highest incidence was observed in transposition complexes (10 per 1,000 person-years). Independent predictors of SCA were retrospectively identified using multivariate Cox proportional hazard modeling. Age at initial examination and severely impaired subaortic ventricular systolic function were independent risk factors for SCA (severe subaortic ventricular systolic dysfunction, adjusted hazard ratio 29, 95% confidence interval 11 to 72, p <0.001). SCA occurred in 23% of patients with severe subaortic ventricular systolic dysfunction versus 0.7% of patients with nonsevere decreased subaortic ventricular function (p <0.001). In conclusion, severe subaortic ventricular systolic dysfunction is a dominant multivariate predictor of SCA in an unselected population of adult survivors after surgery for CHD. Our data support the consideration of primary prevention strategies in these patients.

Risk of Aortic Root or Ascending Aorta Complications in Patients With Bicuspid Aortic Valve With and Without Coarctation of the Aorta

The actual incidence of ascending aorta complications (AACs) in adults with bicuspid aortic valve (BAV) and the role of associated coarctation of the aorta (COA) as an independent risk factor for AACs remain unknown. From the Adult Congenital Heart Disease database at La Paz Hospital, 631 patients in whom a BAV was diagnosed by echocardiography or surgical inspection since December 1989 were identified. These patients were then further subdivided into 2 groups according to the presence of an associated COA. AACs included aortic aneurysms (ascending aorta > or =55 mm) and aortic dissection, rupture, or perforation. Patients with a BAV and COA had a greater prevalence of AACs (8.0%) than those with an isolated BAV (3.7%; p = 0.037). The coexistence of COA was the only significant predictor of AACs (odds ratio 4.7, 95% confidence interval 1.5 to 15; p = 0.01). From the total patient group with a BAV, the clinical and echocardiographic data were reviewed for 341 patients without an AAC at baseline (97 with and 244 without COA) who had undergone serial examinations >1 year apart. The median follow-up was 7 years (interquartile range 3.5 to 10.2; total 2,436 patient-years). A new AAC occurred in 13 patients (0.5/100 patient-years). The incidence of AACs was 1.3/100 patient-years in the COA group versus 0.2/100 patient-years in the non-COA group (hazard ratio 7.5, 95% confidence interval 2.0 to 28, p = 0.002). All acute aortic events (dissection or rupture) at follow-up occurred in patients with a BAV and COA. In conclusion, the long-term incidence of AACs in patients with isolated BAV is low, but patients with BAV and associated COA are at increased risk.

Rapid progression of midventricular obstruction in adults with double-chambered right ventricle.

OBJECTIVE The purpose of this study was to determine the rate of progression of midventricular obstruction in adolescents and adults with double-chambered right ventricle. METHODS Clinical and echocardiographic findings in 45 patients (mean age 26 +/- 6 years, range 15-44) diagnosed with double-chambered right ventricle were retrospectively analyzed. Twenty patients underwent surgical repair before the age of 15 years. The relationship between Doppler midventricular pressure gradient and patient age was analyzed in 25 patients without previous repair. Sequential change in midventricular obstruction was determined for patients with 2 or more Doppler echocardiographic examinations performed within at least a 2-year interval. RESULTS Right midventricular pressure gradient in nonrepaired patients was 70 +/- 38 mm Hg (range 25-150). A significant relationship between midventricular obstruction and patient age (r = 0.64, P <.001) was found. Midventricular pressure gradient at initial evaluation was 32 +/- 27 mm Hg in 16 patients < 25 years and 73 +/- 45 mm Hg in 9 patients >/= 25 years (P <.03). After the initial study, 5 patients underwent surgical repair and 13 patients without repair were followed up for a period of 6.1 +/- 2.7 years (range 2-9), in which midventricular pressure gradient increased from 32 +/- 26 mm Hg to 67 +/- 35 mm Hg (P <.001). The slope of the change in midventricular pressure gradient was 6.2 +/- 3 mm Hg per year of follow-up. Seven more patients underwent surgical repair during follow-up due to progression of the obstruction. There was no mortality nor residual midventricular obstruction in surgically repaired patients. CONCLUSIONS Mild right midventricular obstruction shows a fast rate of progression in adolescents and young adults. Thus, close clinical and echocardiographic follow-up is advised, and surgical repair should be considered if significant progression of obstruction is detected.

Bioprosthetic mitral valve thrombosis: Clinical profile, transesophageal echocardiographic features, and follow-up after anticoagulant therapy

Cardiac bioprosthetic valve thrombosis is frequently found on pathologic examination, but preoperative diagnosis is rarely performed. Four hundred six patients with mitral porcine xenograft bioprostheses were examined by transthoracic echocardiography. Transesophageal echocardiography (TEE) was performed in 161 of the patients, with clinical or echocardiographic criteria of prosthetic malfunction. Fairly homogeneous and echodense masses, attached to the ventricular surface of the mitral bioprosthetic cusps, were detected by TEE in 15 patients. Only 10 patients, in whom diagnosis of bioprosthetic thrombosis was confirmed, are included in this study. After TEE, two patients underwent prosthetic replacement and eight patients received anticoagulants. A new TEE was performed 85.6 +/- 29.8 days after anticoagulation in these eight patients. Clinical follow-up was continued for 13.6 +/- 8.6 months, and one additional patient underwent surgery during the follow-up. Pathologic examination of removed grafts (three cases) identified these masses as being thrombotic tissue. TEE examination after therapeutic anticoagulation demonstrated complete disappearance of the echogenic masses on bioprosthetic cusps and normal mobility of all leaflets in six cases. In the other two cases, cusp masses were notably reduced, but partially restrictive mobility of affected leaflets persisted, suggesting incomplete resolution of thrombi. Mitral valve prosthetic mean gradient decreased from 11.8 +/- 4.5 to 7.6 +/- 3.7 mm Hg (p < 0.001), and mitral valve area increased from 1.13 +/- 0.3 to 1.72 +/- 0.6 cm2 (p < 0.001). Long-term symptomatic improvement after anticoagulation was obtained in seven patients. Thus this study shows that mitral bioprosthetic thrombosis is a relatively frequent cause of valve dysfunction, TEE is useful for detecting thrombus in relation to mitral bioprosthetic valves, and oral anticoagulation is effective in resolving thrombosis on bioprostheses.

Ventrículo derecho y cardiopatías congénitas en el adulto

La disfuncion ventricular derecha no es infrecuente en adultos con cardiopatias congenitas. A diferencia de las cardiopatias adquiridas, en estos pacientes el ventriculo derecho no es siempre el ventriculo subpulmonar y puede soportar la circulacion sistemica, como ocurre en la transposicion de grandes arterias, lo que lleva a una sobrecarga de presion cronica del ventriculo derecho. Por el contrario, la insuficiencia pulmonar –un problema frecuente despues de la reparacion quirurgica de la tetralogia de Fallot– implica una sobrecarga de volumen del ventriculo derecho. Con el tiempo, ambas situaciones pueden generar disfuncion ventricular derecha, lo que a menudo supone un problema clinico importante. Por ello es crucial que todo aquel que trate a pacientes con cardiopatias congenitas conozca exhaustivamente tanto la anatomia como la fisiologia ventricular derecha. Este articulo revisa la anatomia del ventriculo derecho y los efectos adversos de su disfuncion en pacientes adultos con cardiopatias congenitas.

Guía de práctica clínica de la ESC para el manejo de cardiopatías congénitas en el adulto (nueva versión 2010)

Responsabilidad: Las Guias de Practica Clinica recogen la opinion de la ESC y se han elaborado tras una consideracion minuciosa de las evidencias disponibles en el momento en que fueron escritas. Se anima a los profesionales de la sanidad a que las tengan en plena consideracion cuando ejerzan su juicio clinico. No obstante, las Guias de Practica Clinica no deben invalidar la responsabilidad individual de los profesionales de la salud a la hora de tomar decisiones adecuadas a las circunstancias individuales de cada paciente, consultando con el propio paciente y, cuando sea necesario y pertinente, con su tutor o representante legal. Tambien es responsabilidad del profesional de la salud verificar las normas y los reglamentos que se aplican a los farmacos o dispositivos en el momento de la prescripcion. El contenido de las Guias de Practica Clinica de la Sociedad Europea de Cardiologia (ESC) ha sido publicado para uso exclusivamente personal y educacional. No esta autorizado su uso comercial. No se autoriza la traduccion o reproduccion en ningun formato de las Guias de la ESC ni de ninguna de sus partes sin un permiso escrito de la ESC. El permiso puede obtenerse enviando una solicitud por escrito a Oxford University Press, la empresa editorial de European Heart Journal y representante autorizada de la ESC para gestionar estos permisos.