Cardiopatías congénitas del adulto en España: estructura, actividad y características clínicas / Adult congenital heart disease in Spain: health care structure and activity, and clinical characteristics

INTRODUCCIÓN Y OBJETIVOS: Analizar la estructura asistencial y las características clínicas de las cardiopatías congénitas del adulto en España. MÉTODOS: En 2014 se realizó una encuesta entre 32 centros que se clasificaron como nivel 1 o 2 en función de su estructura asistencial. En 2017 se realizó un registro clínico de todos los pacientes asistidos consecutivamente en cada centro durante un periodo de 2 meses. RESULTADOS: Un total de 31 centros (97%) respondieron la encuesta. Se excluyó a 7 por no disponer de consulta especializada. Hasta el año 2005 solo había 5 centros con dedicación específica, pero en 2014 había 10 centros de nivel 1 y 14 de nivel 2 con un total de 19.373 pacientes en seguimiento. La estructura institucional era completa en la mayoría de los centros, pero solo el 33% disponía de enfermería propia y el 29%, de unidad de transición estructurada. La actividad terapéutica específica supuso el 99 y el 91% de la publicada en los registros nacionales de cirugía y cateterismo terapéutico. Del total, el 44% de los pacientes tenían cardiopatía de complejidad moderada y el 24%, de gran complejidad. Aunque el 46% de los pacientes atendidos en centros de nivel 2 tenían cardiopatías simples, el 17% eran cardiopatías de gran complejidad. CONCLUSIONES: La estructura y la actividad de los centros españoles cumplen las recomendaciones internacionales y son comparables a las de otros países desarrollados. El espectro de cardiopatías en seguimiento muestra una concentración de lesiones de complejidad moderada y gran complejidad incluso en centros de nivel 2. Sería aconsejable reordenar el seguimiento de los pacientes en función de las recomendaciones internacionales

INTRODUCTION AND OBJECTIVES: To assess the structure of health care delivery and the clinical characteristics of adults with congenital heart disease (ACHD) attending specialized centers in Spain. METHODS: A survey was conducted among 32 Spanish centers in 2014. The centers were classified into 2 levels based on their resources. In 2017, a clinical dataset was collected of all consecutive patients attended for a 2-month period at these centers. RESULTS: A total of 31 centers (97%) completed the survey. Seven centers without specialized ACHD clinics were excluded from the analysis. In 2005, only 5 centers met the requirements for specific care. In 2014, there were 10 level 1 and 14 level 2 centers, with a total of 19 373 patients under follow-up. Health care structure was complete in most centers but only 33% had ACHD nurse specialists on staff and 29% had structured transition programs. Therapeutic procedures accounted for 99% and 91% of those reported by National Registries of Cardiac Surgery and Cardiac Catheterization, respectively. Among attended patients, 48% had moderately complex lesions and 24% had highly complex lesions. Although 46% of patients attending level 2 centers had simple lesions, 17% had complex lesions. CONCLUSIONS: The structure for ACHD health care delivery in Spain complies with international recommendations and is similar to that of other developed countries. Congenital heart diseases under specialized care consist mostly of moderately and highly complex lesions, even in level 2 centers. It would be desirable to reorganize patient follow-up according to international recommendations in clinical practice

Adult congenital heart disease in Spain: health care structure and activity, and clinical characteristics.

INTRODUCTION AND OBJECTIVES: To assess the structure of health care delivery and the clinical characteristics of adults with congenital heart disease (ACHD) attending specialized centers in Spain. METHODS: A survey was conducted among 32 Spanish centers in 2014. The centers were classified into 2 levels based on their resources. In 2017, a clinical dataset was collected of all consecutive patients attended for a 2-month period at these centers. RESULTS: A total of 31 centers (97%) completed the survey. Seven centers without specialized ACHD clinics were excluded from the analysis. In 2005, only 5 centers met the requirements for specific care. In 2014, there were 10 level 1 and 14 level 2 centers, with a total of 19 373 patients under follow-up. Health care structure was complete in most centers but only 33% had ACHD nurse specialists on staff and 29% had structured transition programs. Therapeutic procedures accounted for 99% and 91% of those reported by National Registries of Cardiac Surgery and Cardiac Catheterization, respectively. Among attended patients, 48% had moderately complex lesions and 24% had highly complex lesions. Although 46% of patients attending level 2 centers had simple lesions, 17% had complex lesions. CONCLUSIONS: The structure for ACHD health care delivery in Spain complies with international recommendations and is similar to that of other developed countries. Congenital heart diseases under specialized care consist mostly of moderately and highly complex lesions, even in level 2 centers. It would be desirable to reorganize patient follow-up according to international recommendations in clinical practice.

Successful cryoablation of dual atrioventricular nodal nonreentrant tachycardia: First reported cases.

We describe two consecutive adult males with incessant dual atrioventricular nodal nonreentrant tachycardia and associated severe cardiomyopathy. After invasive diagnosis, this is the first published report showing the successful outcome of this rare tachycardia with effective cryoablation. Cryothermal lesions in the roof of the proximal coronary sinus and right midseptal area lead to complete resolution of the tachycardia-mediated cardiomyopathy in the mid-term follow-up of our patients.

Shone Complex: An Under-recognized Congenital Heart Disease With Substantial Morbidity in Adulthood.

BACKGROUND: Shone complex consists of a constellation of left-sided, usually obstructive, cardiac lesions, including supravalvar mitral ring, parachute mitral valve, subaortic stenosis, and aortic coarctation. Incomplete Shone complex consists of a mitral valve anomaly associated with lesions involving the subaortic region, aortic valve, or thoracic aorta. There is a paucity of data regarding long-term outcomes in adults with Shone complex. METHODS: We reviewed records of adults with complete or incomplete Shone complex followed at the Montreal Heart Institute between 1982 and 2014. RESULTS: Among 4189 adults with congenital heart disease, 28 (0.67%) patients (mean age, 35 ± 11 years; 50% women) had complete or incomplete Shone complex and were followed for a median of 8 years. Only 39% were previously diagnosed as having Shone complex. The most common defects were congenital mitral stenosis (93%), aortic coarctation (75%), and bicuspid aortic valve (71%). Heart transplantation was required in 2 patients (7.1%) at age 22 and 28 years, respectively. Overall, 48% had cardiovascular hospitalizations during adulthood, predominantly for arrhythmias or heart failure. Freedom from cardiovascular intervention was 55%, 18%, and 8% at 10, 20, and 30 years of age, respectively. Although aortic coarctation was the most common indication for initial intervention (61%), adult interventions occurred predominantly for aortic valve/left ventricular outflow tract (60%) and mitral valve (33%) lesions. CONCLUSIONS: Shone complex is an under-recognized entity associated with relatively low mortality in adulthood but substantial morbidity related to arrhythmias, heart failure, and interventions. Increased awareness of this condition and associated complications may allow for more tailored follow-up.

Wide QRS Complex Arrhythmia with Alternating QRS Morphology: What Is the Mechanism?

A 75-year-old man was admitted due to an electrical storm with appropriate recurrent implantable cardioverter defibrillator (ICD) discharges. The patient had had an extensive anterolateral myocardial infarction with associated severe left ventricular dysfunction 10 years earlier (left ventricular ejection fraction, 25%), and an ICD was placed 9 years before admission for primary prevention of sudden cardiac death. A first invasive study induced up to five ventricular tachycardias and an extensive endocardial substrate ablation was performed. Despite intravenous ß-blockers, general anesthesia and procainamide infusion, the patient continued to have recurrent episodes of very slow sustained ventricular tachycardia with a right bundle branch block pattern. In a subsequent invasive study, no mid-diastolic activity was found despite careful mapping during the induced clinical ventricular tachycardia and ablation attempts inside the apical endocardial scar were unsuccessful. A percutaneous epicardial approach with navigation system support (EnSite PrecisionTM Cardiac Mapping System v. 2.0, St. Jude Medical, St. Paul, MN, USA) without antiarrhythmic infusion was planned. A wide QRS complex rhythm with alternating QRS morphology was readily induced by epicardial ventricular pacing trains (Fig. 1, top) that elicited both arrhythmia QRS patterns with very long stimulus QRS intervals (Fig. 1, bottom). What is the possible mechanism of this arrhythmia? Do we need further pacing maneuvers during the arrhythmia to localize critical sites at which ablation pulses can predictably be successful?

Seguridad del implante ambulatorio de desfibrilador automático implantable / Safety of outpatient implantation of the implantable cardioverter-defibrillator

Introducción y objetivos. Es importante desarrollar estrategias que reduzcan los costes sanitarios y mejoren la atención de los pacientes. El objetivo de nuestro estudio es analizar la seguridad del implante ambulatorio de desfibriladores automáticos implantables. Métodos. Se estudió retrospectivamente a 401 pacientes consecutivos a los que se implantó un desfibrilador automático implantable entre 2007 y 2012. Se comparó la tasa de complicaciones relacionadas con la intervención de los 232 (58%) pacientes cuyo implante se programó como ambulatorio frente a los 169 (42%) intervenidos durante ingreso hospitalario. Resultados. La media de edad era 62 ± 14 años; 336 (84%) pacientes eran varones. Los pacientes ambulatorios tuvieron, en comparación con los hospitalizados, menor fracción de eyección del ventrículo izquierdo y mayor porcentaje de indicación por prevención primaria de muerte súbita. Sólo 21 pacientes (9%) del grupo ambulatorio requirieron ingreso hospitalario. La tasa de complicaciones hasta el tercer mes tras el implante fue similar en los dos grupos (el 6,0% en ambulatorios frente al 5,3% en ingresados; p = 0,763). En el análisis multivariable, solo el tratamiento anticoagulante previo se relacionó con la presencia de complicaciones (odds ratio = 3,2; intervalo de confianza del 95%, 1,4-7,4; p < 0,01), principalmente por un incremento en la tasa de hematomas de la bolsa del dispositivo. Cada implante ambulatorio supuso un ahorro de 735 euros. Conclusiones. El implante ambulatorio de desfibrilador automático implantable es seguro y reduce los costes. En pacientes con tratamiento anticoagulante crónico, se incrementa el riesgo de complicaciones, por lo que debería recomendarse un control específico (AU)

Introduction and objectives. Strategies are needed to reduce health care costs and improve patient care. The objective of our study was to analyze the safety of outpatient implantation of cardioverter-defibrillators. Methods. A retrospective study was conducted in 401 consecutive patients who received an implantable cardioverter-defibrillator between 2007 and 2012. The rate of intervention-related complications was compared between 232 patients (58%) whose implantation was performed in the outpatient setting and 169 patients (42%) whose intervention was performed in the inpatient setting. Results. The mean age (standard deviation) of the patients was 62 (14) years; 336 (84%) were male. Outpatients had lower left ventricular ejection fraction and a higher percentage had an indication for primary prevention of sudden death, compared to inpatients. Only 21 outpatients (9%) required subsequent hospitalization. The rate of complications until the third month postimplantation was similar for outpatients (6.0%) and inpatients (5.3%); P = .763. In multivariate analysis, only previous anticoagulant therapy was related to the presence of complications (odds ratio = 3.2; 95% confidence interval, 1.4-7.4; P < .01), mainly due to an increased rate of pocket hematomas. Each outpatient implantation saved approximately €735. Conclusions. Outpatient implantation of implantable cardioverter-defibrillators is safe and reduces costs. Close observation is recommended for patients receiving chronic anticoagulation therapy due to an increased risk of complications (AU)

Safety of Outpatient Implantation of the Implantable Cardioverter-defibrillator.

INTRODUCTION AND OBJECTIVES: Strategies are needed to reduce health care costs and improve patient care. The objective of our study was to analyze the safety of outpatient implantation of cardioverter-defibrillators. METHODS: A retrospective study was conducted in 401 consecutive patients who received an implantable cardioverter-defibrillator between 2007 and 2012. The rate of intervention-related complications was compared between 232 patients (58%) whose implantation was performed in the outpatient setting and 169 patients (42%) whose intervention was performed in the inpatient setting. RESULTS: The mean age (standard deviation) of the patients was 62 (14) years; 336 (84%) were male. Outpatients had lower left ventricular ejection fraction and a higher percentage had an indication for primary prevention of sudden death, compared to inpatients. Only 21 outpatients (9%) required subsequent hospitalization. The rate of complications until the third month postimplantation was similar for outpatients (6.0%) and inpatients (5.3%); P = .763. In multivariate analysis, only previous anticoagulant therapy was related to the presence of complications (odds ratio = 3.2; 95% confidence interval, 1.4-7.4; P < .01), mainly due to an increased rate of pocket hematomas. Each outpatient implantation saved approximately €735. CONCLUSIONS: Outpatient implantation of implantable cardioverter-defibrillators is safe and reduces costs. Close observation is recommended for patients receiving chronic anticoagulation therapy due to an increased risk of complications.