Corazón univentricular sin cirugía de Fontan: el tipo de paliación importa / Univentricular hearts not undergoing Fontan: the type of palliation matters

Introducción y objetivos: La información sobre pacientes con fisiología univentricular (FU) y flujo pulmonar restrictivo no sometidos a cirugía de Fontan es escasa. El objetivo de este estudio es comparar la supervivencia y los eventos cardiovasculares en estos pacientes según el tipo de paliación.MétodosLos datos de pacientes con FU se obtuvieron de las bases de datos de 7 centros con unidades de cardiopatías congénitas del adulto. Se excluyó a los pacientes que completaron la circulación de Fontan o desarrollaron un síndrome de Eisenmenger. Se crearon 3 grupos según la fuente de flujo pulmonar: G1, flujo anterógrado pulmonar restrictivo; G2, shunt cavopulmonar, y G3, shunt aortopulmonar±shunt cavopulmonar. El objetivo principal fue la muerte.ResultadosSe identificó a 120 pacientes. La media de edad en la primera visita fue 32,2 años. El seguimiento medio fue de 7,1 años. Se asignó a 55 pacientes (45,8%) al G1, 30 (25%) al G2 y 35 (29,2%) al G3. Los pacientes del G3 tenían peores función renal, clase funcional y fracción de eyección en la primera visita y mostraron una disminución más marcada de la fracción de eyección durante el seguimiento, especialmente en comparación con el G1. Veinticuatro pacientes (20%) fallecieron, 38 (31,7%) ingresaron por insuficiencia cardiaca y 21 (17,5%) presentaron aleteo/fibrilación auricular durante el seguimiento. Estos eventos fueron más frecuentes en el G3 y al compararlos con los del G1 se encontraron diferencias significativas en muerte (HR=2,9; IC95%, 1,14-7,37; p=0,026) y aleteo/fibrilación auricular (HR=2,9; IC95%, 1,11-7,68; p=0,037).ConclusionesEl tipo de paliación de los pacientes con FU y flujo pulmonar restrictivo no sometidos a cirugía de Fontan identifica distintos perfiles. Los pacientes paliados con derivaciones aortopulmonares presentan un peor pronóstico, con más morbilidad y mortalidad. (AU)

Introduction and objectives: There is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation.MethodsSVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts±cavopulmonary shunt). The primary endpoint was death.ResultsWe identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P=.026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P=.037).ConclusionsThe type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality. (AU)

Univentricular hearts not undergoing Fontan: the type of palliation matters.

INTRODUCTION AND OBJECTIVES: There is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation. METHODS: SVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts±cavopulmonary shunt). The primary endpoint was death. RESULTS: We identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P=.026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P=.037). CONCLUSIONS: The type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality.

Increased Chamber Resting Tone Is a Key Determinant of Left Ventricular Diastolic Dysfunction.

BACKGROUND: Twitch-independent tension has been demonstrated in cardiomyocytes, but its role in heart failure (HF) is unclear. We aimed to address twitch-independent tension as a source of diastolic dysfunction by isolating the effects of chamber resting tone (RT) from impaired relaxation and stiffness. METHODS: We invasively monitored pressure-volume data during cardiopulmonary exercise in 20 patients with hypertrophic cardiomyopathy, 17 control subjects, and 35 patients with HF with preserved ejection fraction. To measure RT, we developed a new method to fit continuous pressure-volume measurements, and first validated it in a computational model of loss of cMyBP-C (myosin binding protein-C). RESULTS: In hypertrophic cardiomyopathy, RT (estimated marginal mean [95% CI]) was 3.4 (0.4-6.4) mm Hg, increasing to 18.5 (15.5-21.5) mm Hg with exercise (P<0.001). At peak exercise, RT was responsible for 64% (53%-76%) of end-diastolic pressure, whereas incomplete relaxation and stiffness accounted for the rest. RT correlated with the levels of NT-proBNP (N-terminal pro-B-type natriuretic peptide; R=0.57; P=0.02) and with pulmonary wedge pressure but following different slopes at rest and during exercise (R2=0.49; P<0.001). In controls, RT was 0.0 mm Hg and 1.2 (0.3-2.8) mm Hg in HF with preserved ejection fraction patients and was also exacerbated by exercise. In silico, RT increased in parallel to the loss of cMyBP-C function and correlated with twitch-independent myofilament tension (R=0.997). CONCLUSIONS: Augmented RT is the major cause of LV diastolic chamber dysfunction in hypertrophic cardiomyopathy and HF with preserved ejection fraction. RT transients determine diastolic pressures, pulmonary pressures, and functional capacity to a greater extent than relaxation and stiffness abnormalities. These findings support antimyosin agents for treating HF.

Transvalvular jet velocity, aortic valve area, mortality, and cardiovascular outcomes.

AIMS: The interplay between aortic stenosis (AS), cardiovascular events, and mortality is poorly understood. In addition, how echocardiographic indices compare for predicting outcomes remains unexplored for the full range of AS severity. METHODS AND RESULTS: We prospectively calculated peak jet velocity (Vmax) and aortic valve area (AVA) in 5994 adult subjects with and without AS. We linked ultrasound data to 5-year mortality and clinical events obtained from electronic medical records. Proportional-hazard and negative binomial regression models were adjusted for relevant covariables such as age, sex, comorbidities, stroke-volume, LV ejection fraction, left valve regurgitation, aortic valve sclerosis or calcification, and valve replacement. We observed a strong linear relationship between Vmax and all-cause mortality (hazard ratio: 1.26, 95% confidence interval: 1.19-1.33 per 100 cm/s), cardiovascular events, as well as incidental and recurrent heart failure (HF). Adjusted risks were highly significant even at Vmax values in the range of 150-200 cm/s, risk curves separating very early after the index exam. Vmax was not associated with coronary, arrhythmic, cerebrovascular, or non-cardiovascular events. Although risks were confirmed when AVA was entered in place of Vmax, the risks estimated for categories based on the two indices were mismatched, even in patients with normal flow. An external cohort comprising 112 690 patients confirmed augmented risks of all-cause and cardiovascular mortality starting at values of Vmax and AVA in the range of mild AS. CONCLUSIONS: Aortic stenosis is strongly associated to all-cause mortality, cardiovascular mortality, and cardiac events, specifically HF. Risks increase in parallel to the degree of outflow obstruction but are apparent very early in patients with mild disease. Criteria for grading AS based on Vmax and AVA are mismatched in terms of outcomes.

A comparison of the clinical efficacy of echocardiography and magnetic resonance for chronic aortic regurgitation.

AIMS: Timing surgery in chronic aortic regurgitation (AR) relies mostly on echocardiography. However, cardiac magnetic resonance (CMR) may be more accurate for quantifying regurgitation and left ventricular (LV) remodelling. We aimed to compare the technical and clinical efficacies of echocardiography and CMR to account for the severity of the disease, the degree of LV remodelling, and predict AR-related outcomes. METHODS AND RESULTS: We studied 263 consecutive patients with isolated AR undergoing echocardiography and CMR. After a median follow-up of 33 months, 76 out of 197 initially asymptomatic patients reached the primary endpoint of AR-related events: 6 patients (3%) were admitted for heart failure, and 70 (36%) underwent surgery. Adjusted survival models based on CMR improved the predictions of the primary endpoint based on echocardiography: R2 = 0.37 vs. 0.22, χ2 = 97 vs. 49 (P < 0.0001), and C-index = 0.80 vs. 0.70 (P < 0.001). This resulted in a net classification index of 0.23 (0.00-0.46, P = 0.046) and an integrated discrimination improvement of 0.12 (95% confidence interval 0.08-0.58, P = 0.02). CMR-derived regurgitant fraction (<28, 28-37, or >37%) and LV end-diastolic volume (<83, 183-236, or >236 mL) adequately stratified patients with normal EF. The agreement between techniques for grading AR severity and assessing LV dilatation was poor, and CMR showed better reproducibility. CONCLUSIONS: CMR improves the clinical efficacy of ultrasound for predicting outcomes of patients with AR. This is due to its better reproducibility and accuracy for grading the severity of the disease and its impact on the LV. Regurgitant fraction, LV ejection fraction, and end-diastolic volume obtained by CMR most adequately predict AR-related events.

Long-term Outcomes of Adults With Single Ventricle Physiology Not Undergoing Fontan Repair: A Multicentre Experience.

BACKGROUND: To describe long-term survival and cardiovascular events in adult patients with single ventricle physiology (SVP) without Fontan palliation, focusing on predictors of mortality and comparing groups according to their cardiovascular physiology. METHODS: Multicentre observational and retrospective study including adult patients with SVP without Fontan palliation since their first adult clinic visit. The cohort was subdivided into 3 groups: Eisenmenger, restricted pulmonary flow, and aortopulmonary shunt. Death was considered as the main end point. Other clinical outcomes occurring during follow-up were considered as secondary end points. RESULTS: A total of 146 patients, mean age 32.5 ± 11.1 years, were analysed. Over a mean follow-up of 7.3 ± 4.1 years, 33 patients (22.6%) died. Survival was 86% and 74% at 5 and 10 years, respectively. Right ventricular morphology was not associated with higher mortality. Four variables at baseline were related to a higher mortality: at least moderate atrioventricular valve regurgitation, platelet count < 150 × 103/mm3, GFR < 60 mL/min/1.73 m2, and QRS > 120 ms). A total of 34.2% of patients were admitted to the hospital due to heart failure, and 7.5% received a heart transplant. Other cardiovascular outcomes were also frequent: atrial arrhythmias in 19.2%, stroke in 15.1%, and pacemaker/implantable cardioverter-defibrillator in 6.2%/2.7%. CONCLUSIONS: Adult patients with SVP who had not undergone Fontan exhibit a high mortality rate and frequent major cardiovascular events. At least moderate atrioventricular valve regurgitation, thrombocytopenia, renal dysfunction, and QRS duration > 120 ms at baseline visit allow identification of a cohort of patients at higher risk of mortality.