Surgical management of tricuspid regurgitation: a new algorithm to minimise recurrent tricuspid regurgitation.

INTRODUCTION: Recurrent tricuspid regurgitation (TR) is frequently observed after cardiac surgery; however, the correct approach remains controversial. We developed an algorithm for action on the tricuspid valve (TV) and conducted a 1-year follow-up study. The aim was to assess the efficacy of the algorithm to minimise residual TR after TV surgery. The hypothesis was that the TR rate at 1 year would be reduced by selecting the surgical approach in accordance with a set of preoperative clinical and echocardiographic variables. METHODS: A prospective, observational, single-centre study was performed in 76 consecutive patients with TV involvement. A protocol was designed for their inclusion, and data on their clinical and echocardiographic characteristics were gathered at 3 months and 1-year postsurgery. The treatment of patients depended on the degree of TR. Surgery was performed in all patients with severe or moderate-to-severe TR and in those with mild or moderate TR alongside the presence of certain clinical or echocardiographic factors. They underwent annuloplasty or extended valve repair when the TV was distorted. If repair techniques were not feasible, a prosthesis was implanted. Residual TR rates were compared with published reports, and predictors of early/late mortality and residual TR were evaluated. RESULTS: TR was functional in 69.9% of patients. Rigid ring annuloplasty was performed in 35.7% of patients, De Vega annuloplasty in 27.1%, extended repair in 11.4% and prosthetic replacement in 25.7%. TR was moderate or worse in 8.19% of patients (severe in 3.27%) at 1 year postintervention. No clinical, surgical or epidemiological variables were significantly associated with residual TR persistence, although annulus diameter showed a close-to-significant association. Total mortality was 12.85% for all causes and 10% for cardiovascular causes. In multivariate analysis, left ventricular ejection fraction was related to both early and late mortality. CONCLUSIONS: Severe residual TR was significantly less frequent than reported in other series, being observed in less than 4% of patients at 1-year postsurgery.

Pulmonary hypertension in adults with congenital heart disease. Clinical phenotypes and outcomes in the advanced pulmonary vasodilator era.

BACKGROUND: Mortality of pulmonary hypertension associated with congenital heart disease (PAH-CHD) in adults remains high. OBJECTIVES: To identify predictors of death and to assess the impact of treatment on outcome. METHODS: Retrospective, multicenter cohort study of 103 adults with PAH-CHD followed-up for 8.6 ± 4.6 years. Patients were grouped according to underlying shunt type into pre-tricuspid, post-tricuspid and complex. Survival rates were analyzed and predictors of death were investigated with Cox regression models. RESULTS: In the post-tricuspid and complex groups (38 and 37 patients, respectively), the most common clinical PAH-CHD subgroup was Eisenmenger syndrome (76.3% and 59.5%, respectively) whereas, in the pre-tricuspid group (28 patients), 46.5% of patients had small or corrected defects. Overall, 88 patients received vasodilators; 39% required combination-therapy. Overall survival at 10 years was 65%. Mortality was highest in the pre-tricuspid group, FC-III-IV and amongst patients receiving monotherapy (p < 0.050). On multivariate analysis, predictors of poor outcome were pericardial effusion (HR: 4,520 [1,470-13,890]; p = 0,008), oxygen saturation(HR: 0.940 [0,900 - 0,990]; p = 0,018) and genetic syndromes(HR: 3,280 [1,098-9,780]; p = 0,033). CONCLUSIONS: Patients in advanced stages at initiation of treatment were at high risk of death and strong consideration should be given for more aggressive therapy.

Layer-Specific Global Longitudinal Strain Predicts Arrhythmic Risk in Arrhythmogenic Cardiomyopathy.

Background: Arrhythmogenic cardiomyopathy (AC) is a life-threatening disease which predispose to malignant arrhythmias and sudden cardiac death (SCD) in the early stages of the disease. Risk stratification relies on the electrical, genetic, and imaging data. Our study aimed to investigate how myocardial deformation parameters may identify the subjects at risk of known predictors of major ventricular arrhythmias. Methods: A cohort of 45 subjects with definite or borderline diagnosis of AC was characterized using the advanced transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) and divided into the groups according to the potential arrhythmic risk markers, such as non-sustained ventricular tachycardia (NSVT), late gadolinium enhancement (LGE), and genetic status. Layer-specific global longitudinal strain (GLS) by TTE 2D speckle tracking was compared in patients with and without these arrhythmic risk markers. Results: In this study, 23 (51.1%) patients were men with mean age of 43 ± 16 years. Next-generation sequencing identified a potential pathogenic mutation in 39 (86.7%) patients. Thirty-nine patients presented LGE (73.3%), mostly located at the subepicardial-to-mesocardial layers. A layer-specific-GLS analysis showed worse GLS values at the epicardial and mesocardial layers in the subjects with NSVT and LGE. The epicardial GLS values of -15.4 and -16.1% were the best cut-off values for identifying the individuals with NSVT and LGE, respectively, regardless of left ventricular ejection fraction (LVEF). Conclusions: The layer-specific GLS assessment identified the subjects with high-risk arrhythmic features in AC, such as NSVT and LGE. An epicardial GLS may emerge as a potential instrument for detecting the subjects at risk of SCD in AC.

Clinical outcome of COVID-19 in patients with adult congenital heart disease.

AIMS: Patients with adult congenital heart disease (ACHD) are a potentially vulnerable patient cohort in case of COVID-19. Some cardiac defects may be associated with a poor COVID-19 outcome. Risk estimation in ACHD is currently based on expert opinion. The aim of this study was to collect clinical outcome data and to identify risk factors for a complicated course of COVID-19 in patients with ACHD. METHODS: Twenty-five ACHD centres in nine European countries participated in the study. Consecutive patients with ACHD diagnosed with COVID-19 presenting to one of the participating centres between 27 March and 6 June 2020 were included. A complicated disease course was defined as hospitalisation for COVID-19 requiring non-invasive or invasive ventilation and/or inotropic support, or a fatal outcome. RESULTS: Of 105 patients with a mean age of 38±13 years (58% women), 13 had a complicated disease course, of whom 5 died. In univariable analysis, age (OR 1.3, 95% CI 1.1 to 1.7, per 5 years), ≥2 comorbidities (OR 7.1, 95% CI 2.1 to 24.5), body mass index of >25 kg/m2 (OR 7.2, 95% CI 1.9 to 28.3) and cyanotic heart disease (OR 13.2, 95% CI 2.5 to 68.4) were associated with a complicated disease course. In a multivariable logistic regression model, cyanotic heart disease was the most important predictor (OR 60.0, 95% CI 7.6 to 474.0). CONCLUSIONS: Among patients with ACHD, general risk factors (age, obesity and multiple comorbidities) are associated with an increased risk of complicated COVID-19 course. Congenital cardiac defects at particularly high risk were cyanotic lesions, including unrepaired cyanotic defects or Eisenmenger syndrome.

Persistent Pulmonary Hypertension in Corrected Valvular Heart Disease: Hemodynamic Insights and Long-Term Survival.

Background The determinants and consequences of pulmonary hypertension after successfully corrected valvular heart disease remain poorly understood. We aim to clarify the hemodynamic bases and risk factors for mortality in patients with this condition. Methods and Results We analyzed long-term follow-up data of 222 patients with pulmonary hypertension and valvular heart disease successfully corrected at least 1 year before enrollment who had undergone comprehensive hemodynamic and imaging characterization as per the SIOVAC (Sildenafil for Improving Outcomes After Valvular Correction) clinical trial. Median (interquartile range) mean pulmonary pressure was 37 mm Hg (32-44 mm Hg) and pulmonary artery wedge pressure was 23 mm Hg (18-26 mm Hg). Most patients were classified either as having combined precapillary and postcapillary or isolated postcapillary pulmonary hypertension. After a median follow-up of 4.5 years, 91 deaths accounted for 4.21 higher-than-expected mortality in the age-matched population. Risk factors for mortality were male sex, older age, diabetes mellitus, World Health Organization functional class III and higher pulmonary vascular resistance-either measured by catheterization or approximated from ultrasound data. Higher pulmonary vascular resistance was related to diabetes mellitus and smaller residual aortic and mitral valve areas. In turn, the latter correlated with prosthetic nominal size. Six-month changes in the composite clinical score and in the 6-minute walk test distance were related to survival. Conclusions Persistent valvular heart disease-pulmonary hypertension is an ominous disease that is almost universally associated with elevated pulmonary artery wedge pressure. Pulmonary vascular resistance is a major determinant of mortality in this condition and is related to diabetes mellitus and the residual effective area of the corrected valve. These findings have important implications for individualizing valve correction procedures. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT00862043.

Predicting sudden cardiac death in adults with congenital heart disease.

OBJECTIVES: To develop, calibrate, test and validate a logistic regression model for accurate risk prediction of sudden cardiac death (SCD) and non-fatal sudden cardiac arrest (SCA) in adults with congenital heart disease (ACHD), based on baseline lesion-specific risk stratification and individual's characteristics, to guide primary prevention strategies. METHODS: We combined data from a single-centre cohort of 3311 consecutive ACHD patients (50% male) at 25-year follow-up with 71 events (53 SCD and 18 non-fatal SCA) and a multicentre case-control group with 207 cases (110 SCD and 97 non-fatal SCA) and 2287 consecutive controls (50% males). Cumulative incidences of events up to 20 years for specific lesions were determined in the prospective cohort. Risk model and its 5-year risk predictions were derived by logistic regression modelling, using separate development (18 centres: 144 cases and 1501 controls) and validation (two centres: 63 cases and 786 controls) datasets. RESULTS: According to the combined SCD/SCA cumulative 20 years incidence, a lesion-specific stratification into four clusters-very-low (<1%), low (1%-4%), moderate (4%-12%) and high (>12%)-was built. Multivariable predictors were lesion-specific cluster, young age, male sex, unexplained syncope, ischaemic heart disease, non-life threatening ventricular arrhythmias, QRS duration and ventricular systolic dysfunction or hypertrophy. The model very accurately discriminated (C-index 0.91; 95% CI 0.88 to 0.94) and calibrated (p=0.3 for observed vs expected proportions) in the validation dataset. Compared with current guidelines approach, sensitivity increases 29% with less than 1% change in specificity. CONCLUSIONS: Predicting the risk of SCD/SCA in ACHD can be significantly improved using a baseline lesion-specific stratification and simple clinical variables.

Recanalisation of subclavian-pulmonary artery shunt in adult with tetralogy of Fallot.

We report the case of a patient diagnosed with extreme tetralogy of Fallot who received subclavian-pulmonary artery shunt at the age of 3 years and underwent corrective surgery at the age of 16 years. The patient developed progressive effort dyspnea and left ventricular dysfunction from the age of 40 years. The exploratory finding of a continuous jugular and left subclavian murmur prompted an echocardiographic study that evidenced a continuous flow in the suprasternal view. CT study confirmed the patency of the surgical subclavian-pulmonary artery shunt, which was percutaneously embolised with the implantation of an Amplatzer plug, with a good final outcome and no residual flow. The patient showed a satisfactory development with clinical improvement, decreased left ventricular volume and enhanced left ventricular function.

Sildenafil for improving outcomes in patients with corrected valvular heart disease and persistent pulmonary hypertension: a multicenter, double-blind, randomized clinical trial.

Aims: We aimed to determine whether treatment with sildenafil improves outcomes of patients with persistent pulmonary hypertension (PH) after correction of valvular heart disease (VHD). Methods and results: The sildenafil for improving outcomes after valvular correction (SIOVAC) study was a multricentric, randomized, parallel, and placebo-controlled trial that enrolled stable adults with mean pulmonary artery pressure ≥ 30 mmHg who had undergone a successful valve replacement or repair procedure at least 1 year before inclusion. We assigned 200 patients to receive sildenafil (40 mg three times daily, n = 104) or placebo (n = 96) for 6 months. The primary endpoint was the composite clinical score combining death, hospital admission for heart failure (HF), change in functional class, and patient global self-assessment. Only 27 patients receiving sildenafil improved their composite clinical score, as compared with 44 patients receiving placebo; in contrast 33 patients in the sildenafil group worsened their composite score, as compared with 14 in the placebo group [odds ratio 0.39; 95% confidence interval (CI) 0.22-0.67; P < 0.001]. The Kaplan-Meier estimates for survival without admission due to HF were 0.76 and 0.86 in the sildenafil and placebo groups, respectively (hazard ratio 2.0, 95% CI = 1.0-4.0; log-rank P = 0.044). Changes in 6-min walk test distance, natriuretic peptides, and Doppler-derived systolic pulmonary pressure were similar in both groups. Conclusion: Treatment with sildenafil in patients with persistent PH after successfully corrected VHD is associated to worse clinical outcomes than placebo. Off-label usage of sildenafil for treating this source of left heart disease PH should be avoided. The trial is registered with ClinicalTrials.gov, number NCT00862043.

Una variante rara del síndrome de la cimitarra / Uncommon Variant of Scimitar Syndrome

No disponible

Diagnóstico clínico y genético de la muerte súbita cardiaca de origen no isquémico / Clinical and genetic diagnosis of nonischemic sudden cardiac death

Introducción y objetivos: La muerte súbita cardiaca (MSC) de origen no isquémico está causada predominantemente por miocardiopatías y canalopatías. La batería de test diagnósticos es amplia e incluye pruebas complejas. El objetivo de nuestro estudio es analizar la rentabilidad diagnóstica del estudio etiológico sistematizado de la MSC. Métodos: Se estudió a 56 familias con al menos 1 caso índice con MSC (reanimada o no). En los supervivientes se exploró con electrocardiograma, imagen cardiaca avanzada, ergometría, estudio familiar, estudio genético y, puntualmente, test farmacológicos. En los fallecidos se examinó la necropsia, así como la autopsia molecular con next generation sequencing (NGS), junto con estudio clínico familiar. Resultados: El diagnóstico se alcanzó en el 80,4% de los casos, sin diferencias entre supervivientes y fallecidos (p = 0,53). Entre los supervivientes, el diagnóstico de canalopatía fue más frecuente que entre los fallecidos (el 66,6 frente al 40%; p = 0,03). De los 30 sujetos fallecidos, en 7 la autopsia aportó un hallazgo concluyente. El diagnóstico de miocardiopatía tendía a asociarse con mayor tasa de eventos en la familia. El test genético con NGS se realizó en 42 de los casos; se obtuvo resultado positivo en 28 (66,6%), sin diferencias entre supervivientes y fallecidos (p = 0,21). Conclusiones: La probabilidad de alcanzar el diagnóstico en la MSC tras un protocolo exhaustivo es alta, con mayor prevalencia de canalopatías en los supervivientes y un aparente peor pronóstico en las miocardiopatías. El test genético mediante NGS muestra utilidad en casos de MSC e incrementa la rentabilidad respecto al estudio con Sanger (AU)

Introduction and objectives: Nonischemic sudden cardiac death (SCD) is predominantly caused by cardiomyopathies and channelopathies. There are many diagnostic tests, including some complex techniques. Our aim was to analyze the diagnostic yield of a systematic diagnostic protocol in a specialized unit. Methods: The study included 56 families with at least 1 index case of SCD (resuscitated or not). Survivors were studied with electrocardiogram, advanced cardiac imaging, exercise testing, familial study, genetic testing and, in some cases, pharmacological testing. Families with deceased probands were studied using the postmortem findings, familial evaluation, and molecular autopsy with next-generation sequencing (NGS). Results: A positive diagnosis was obtained in 80.4% of the cases, with no differences between survivors and nonsurvivors (P = .53). Cardiac channelopathies were more prevalent among survivors than nonsurvivors (66.6% vs 40%, P = .03). Among the 30 deceased probands, the definitive diagnosis was given by autopsy in 7. A diagnosis of cardiomyopathy tended to be associated with a higher event rate in the family. Genetic testing with NGS was performed in 42 index cases, with a positive result in 28 (66.6%), with no differences between survivors and nonsurvivors (P = .21). Conclusions: There is a strong likelihood of reaching a diagnosis in SCD after a rigorous protocol, with a more prevalent diagnosis of channelopathy among survivors and a worse familial prognosis in cardiomyopathies. Genetic testing with NGS is useful and its value is increasing with respect to the Sanger method (AU)

Clinical and Genetic Diagnosis of Nonischemic Sudden Cardiac Death.

INTRODUCTION AND OBJECTIVES: Nonischemic sudden cardiac death (SCD) is predominantly caused by cardiomyopathies and channelopathies. There are many diagnostic tests, including some complex techniques. Our aim was to analyze the diagnostic yield of a systematic diagnostic protocol in a specialized unit. METHODS: The study included 56 families with at least 1 index case of SCD (resuscitated or not). Survivors were studied with electrocardiogram, advanced cardiac imaging, exercise testing, familial study, genetic testing and, in some cases, pharmacological testing. Families with deceased probands were studied using the postmortem findings, familial evaluation, and molecular autopsy with next-generation sequencing (NGS). RESULTS: A positive diagnosis was obtained in 80.4% of the cases, with no differences between survivors and nonsurvivors (P=.53). Cardiac channelopathies were more prevalent among survivors than nonsurvivors (66.6% vs 40%, P=.03). Among the 30 deceased probands, the definitive diagnosis was given by autopsy in 7. A diagnosis of cardiomyopathy tended to be associated with a higher event rate in the family. Genetic testing with NGS was performed in 42 index cases, with a positive result in 28 (66.6%), with no differences between survivors and nonsurvivors (P=.21). CONCLUSIONS: There is a strong likelihood of reaching a diagnosis in SCD after a rigorous protocol, with a more prevalent diagnosis of channelopathy among survivors and a worse familial prognosis in cardiomyopathies. Genetic testing with NGS is useful and its value is increasing with respect to the Sanger method.

Novedades en imagen cardiaca 2014 / Update on cardiac imaging techniques 2014

En este artículo se realiza una revisión de las aportaciones de las técnicas de imagen más relevantes a la cardiología que se han publicado durante este año. El ecocardiograma sigue siendo la piedra angular en el diagnóstico y el seguimiento de las valvulopatías, con un esfuerzo continuo para mejorar su cuantificación y obtener parámetros pronósticos de seguimiento. El estudio de la función miocárdica regional se afianza en el diagnóstico de la disfunción ventricular subclínica, y el ecocardiograma transesofágico tridimensional se ha convertido en el perfecto aliado del intervencionismo en las cardiopatías estructurales. La cardiorresonancia y la tomografía computarizada cardiaca acaparan la mayoría de las publicaciones en imagen cardiaca relativas a la cardiopatía isquémica, reflejo de unas técnicas más que consolidadas en la práctica clínica. La medicina nuclear destaca en el estudio de la viabilidad miocárdica tras el intervencionismo en el síndrome coronario agudo y refuerza su rendimiento en el diagnóstico de la cardiopatía isquémica (AU)

In this article, we review the contributions of the most important imaging techniques used in cardiology, reported in 2014. Echocardiography remains the cornerstone for diagnosing and monitoring valvular heart disease, and there has been a continuing effort to improve quantification of this condition and obtain prognostic parameters for follow-up. The study of regional myocardial function is anchored in the diagnosis of subclinical ventricular dysfunction, and 3-dimensional transesophageal echocardiography has become the perfect ally in interventional procedures for structural heart disease. Cardiac magnetic resonance imaging and cardiac computed tomography are the focus of most publications on cardiac imaging in ischemic heart disease, reflecting their consolidated use in clinical practice. Nuclear medicine excels in the study of myocardial viability after interventional treatment of acute coronary syndromes and its performance is validated in the diagnosis of ischemic heart disease (AU)

Update on cardiac imaging techniques 2014.

In this article, we review the contributions of the most important imaging techniques used in cardiology, reported in 2014. Echocardiography remains the cornerstone for diagnosing and monitoring valvular heart disease, and there has been a continuing effort to improve quantification of this condition and obtain prognostic parameters for follow-up. The study of regional myocardial function is anchored in the diagnosis of subclinical ventricular dysfunction, and 3-dimensional transesophageal echocardiography has become the perfect ally in interventional procedures for structural heart disease. Cardiac magnetic resonance imaging and cardiac computed tomography are the focus of most publications on cardiac imaging in ischemic heart disease, reflecting their consolidated use in clinical practice. Nuclear medicine excels in the study of myocardial viability after interventional treatment of acute coronary syndromes and its performance is validated in the diagnosis of ischemic heart disease.

Update on cardiac imaging techniques 2013.

Cardiac imaging is a cornerstone of diagnosis in heart conditions, and an essential tool for assessing prognosis and establishing treatment decisions. This year, echocardiography stands out as a guide in interventional procedures and in choosing the size of the prosthesis. It is also proving to be a valuable technique in low-flow, low-gradient aortic stenosis. Three-dimensional echocardiography is advancing our knowledge of cardiac anatomy and valvular measurements. The parameters indicating tissue deformation have predictive power in valve disease and in the follow-up of drug-induced cardiotoxicity. Single-photon emission computed tomography and positron emission tomography are proving useful in ischemic heart disease and in the diagnosis of cardiac inflammation and infections. The role of computed tomography has been strengthened in noninvasive coronary angiography, the emergency room management of chest pain, assessment of chronic occlusions, and morphologic study of coronary plaque. Cardiac magnetic resonance imaging remains the gold standard for tissue characterization in ischemic heart disease and cardiomyopathies, and is assuming a greater role in stress studies and in the assessment of myocardial viability.

Novedades en imagen cardiaca 2013 / Update on cardiac imaging techniques 2013

La imagen cardiaca es la piedra angular del diagnóstico en cardiología, así como un instrumento esencial en la valoración pronóstica y las decisiones terapéuticas. Este año, la ecocardiografía destaca como guía en el intervencionismo y en la elección del tamaño de las prótesis. Se consolida la entidad de la estenosis aórtica de bajo flujo y bajo gradiente. El método tridimensional avanza en el conocimiento anatómico y la cuantificación valvular. Los parámetros de deformación muestran su poder predictor en valvulopatías y en el seguimiento en la cardiotoxicidad inducida por fármacos. La tomografía computarizada por emisión monofotónica y la tomografía por emisión positrónica consolidan su utilidad en cardiopatía isquémica y destacan en el diagnóstico de inflamaciones e infecciones cardiacas. La tomografía computarizada cardiaca afianza su papel en la coronariografía no invasiva, el manejo del dolor torácico en urgencias, la valoración de las oclusiones crónicas y el estudio morfológico de la placa. La resonancia magnética cardiaca permanece como técnica de referencia en la caracterización tisular tanto en cardiopatía isquémica como en las miocardiopatías y afianza su papel en el estudio de estrés y la valoración de la viabilidad (AU)

Cardiac imaging is a cornerstone of diagnosis in heart conditions, and an essential tool for assessing prognosis and establishing treatment decisions. This year, echocardiography stands out as a guide in interventional procedures and in choosing the size of the prosthesis. It is also proving to be a valuable technique in low-flow, low-gradient aortic stenosis. Three-dimensional echocardiography is advancing our knowledge of cardiac anatomy and valvular measurements. The parameters indicating tissue deformation have predictive power in valve disease and in the follow-up of drug-induced cardiotoxicity. Single-photon emission computed tomography and positron emission tomography are proving useful in ischemic heart disease and in the diagnosis of cardiac inflammation and infections. The role of computed tomography has been strengthened in noninvasive coronary angiography, the emergency room management of chest pain, assessment of chronic occlusions, and morphologic study of coronary plaque. Cardiac magnetic resonance imaging remains the gold standard for tissue characterization in ischemic heart disease and cardiomyopathies, and is assuming a greater role in stress studies and in the assessment of myocardial viability (AU)

Novedades en imagen cardiaca 2012 / Update on cardiac imaging techniques 2012

La imagen cardiaca es uno de los pilares de la cardiología actual. La cantidad de situaciones en que las técnicas de imagen aportan información importante en clínica o investigación es sencillamente interminable, por lo que es imposible recoger en el formato reducido de un artículo todas las novedades importantes sobre imagen cardiaca que han aparecido en la literatura médica en el año 2012. Resumimos las publicaciones más importantes del año, entre las que destacan el desarrollo progresivo de las técnicas de deformación miocárdica y la ecocardiografía tridimensional y la incorporación creciente al trabajo clínico diario de la resonancia magnética y la tomografía computarizada (AU)

Cardiac imaging is one of the basic pillars of modern cardiology. The potential list of scenarios where cardiac imaging techniques can provide relevant information is simply endless so it is impossible to include all relevant new features of cardiac imaging published in the literature in 2012 in the limited format of a single article. We summarize the year's most relevant news on cardiac imaging, highlighting the ongoing development of myocardial deformation and 3-dimensional echocardiography techniques and the increasing use of magnetic resonance imaging and computed tomography in daily clinical practice (AU)