Diferencias clínicas entre la amiloidosis cardiaca por transtirretina y la cardiopatía hipertensiva / Clinical differences between transthyretin cardiac amyloidosis and hypertensive heart disease

Introducción: Un porcentaje importante de pacientes finalmente diagnosticados de amiloidosis cardIaca por transtirretina (ATTR) fueron previamente diagnosticados de cardiopatía hipertensiva (CHTA), ya que ambas enfermedades suelen cursar con insuficiencia cardíaca (IC) con fracción de eyección preservada (ICFEp) e hipertrofia ventricular. Nuestros objetivos fueron evaluar las diferencias clínicas, electrocardiográficas y ecocardiográficas, y analizar si existe un pronóstico diferencial entre ambas entidades nosológicas. Material y métodos: Se incluyeron retrospectivamente todos los pacientes con CHTA a los que se solicitó una gammagrafía cardíaca con 99mTc-Difosfonatos (GDPD) y estudio de cadenas ligeras en sangre y orina para despistaje de ATTR en nuestro centro, en el periodo 2016-2021. Para el análisis, se excluyeron aquellos diagnosticados de otros tipos de amiloidosis. Resultados: Se analizaron un total de 72 pacientes: 33 fueron diagnosticados de ATTR y 39 de CHTA, finalmente. Los pacientes con ATTR presentaron mayores niveles de troponina I ultrasensible (TnI-US) y propéptido natriurético cerebral N-terminal (NT-ProBNP); en electrocardiograma (ECG) presentaron más frecuentemente patrón de seudoinfarto y alteraciones de la conducción; en ecocardiograma transtorácico (ETT) presentaron mayor grado de hipertrofia ventricular, disfunción ventricular izquierda y parámetros de peor función diastólica, con presiones de llenado más elevadas. En el seguimiento a 4 años, el grupo de ATTR mostró mayor necesidad de marcapasos (MCP), sin evidenciarse evidencias en cuanto a mortalidad, desarrollo de fibrilación auricular o más ingresos por IC. Conclusiones: En nuestra serie, los pacientes con ATTR presentaron diferencias clínicas, electrocardiográficas y ecocardiográficas respecto a aquellos con CHTA, con mayor riesgo necesidad de MCP en el seguimiento.(AU)

Introduction: A significant percentage of patients eventually diagnosed with cardiac transthyretin amyloidosis (TTRA) was previously diagnosed with hypertensive heart disease (HHD), since both conditions usually present with heart failure (HF) with preserved ejection fraction (HFpEF) and ventricular hypertrophy. Our objectives were to evaluate the clinical, electrocardiographic and echocardiographic differences, and to analyse whether there exists a differential prognosis between these two nosological entities. Materials and methods: We retrospectively included all patients with HHD for whom a cardiac scintigraphy with 99mTc-diphosphonate (GDPD) and a free light chains test in blood and urine were ordered for ATTR screening in our centre, in the period between 2016 and 2021. Those diagnosed with other types of amyloidosis were excluded from the analysis. Results: A total of 72 patients were analyzed: 33 were finally diagnosed with TTRA and 39 with CHTA. Patients with TTRA had higher levels of ultrasensitive troponin I (TnI-US) and N-terminal brain natriuretic propeptide (NT-ProBNP); in electrocardiography (ECG) they presented a pseudo-infarction pattern more frequently as well as conduction disturbances; in echocardiography (TTE) they presented a higher degree of ventricular hypertrophy, left ventricular dysfunction and worse diastolic function parameters, with elevated filling pressures. In the 4-year follow-up, the ATTR group showed greater need for pacemaker (PCM), with no evidence regarding mortality, development of atrial fibrillation (AF), or more admissions for heart failure (HF). Conclusions: In our series, patients with TTRA showed clinical, electrocardiographic and echocardiographic differences compared to patients with HHD, with increased risk of need for PCM.(AU)

Clinical differences between transthyretin cardiac amyloidosis and hypertensive heart disease. / Diferencias clínicas entre la amiloidosis cardiaca por transtirretina y la cardiopatía hipertensiva.

INTRODUCTION: A significant percentage of patients eventually diagnosed with cardiac transthyretin amyloidosis (TTRA) was previously diagnosed with hypertensive heart disease (HHD), since both conditions usually present with heart failure (HF) with preserved ejection fraction (HFpEF) and ventricular hypertrophy. Our objectives were to evaluate the clinical, electrocardiographic and echocardiographic differences, and to analyse whether there exists a differential prognosis between these two nosological entities. MATERIALS AND METHODS: We retrospectively included all patients with HHD for whom a cardiac scintigraphy with 99mTc-diphosphonate (GDPD) and a free light chains test in blood and urine were ordered for ATTR screening in our centre, in the period between 2016 and 2021. Those diagnosed with other types of amyloidosis were excluded from the analysis. RESULTS: A total of 72 patients were analyzed: 33 were finally diagnosed with TTRA and 39 with CHTA. Patients with TTRA had higher levels of ultrasensitive troponin I (TnI-US) and N-terminal brain natriuretic propeptide (NT-ProBNP); in electrocardiography (ECG) they presented a pseudo-infarction pattern more frequently as well as conduction disturbances; in echocardiography (TTE) they presented a higher degree of ventricular hypertrophy, left ventricular dysfunction and worse diastolic function parameters, with elevated filling pressures. In the 4-year follow-up, the ATTR group showed greater need for pacemaker (PCM), with no evidence regarding mortality, development of atrial fibrillation (AF), or more admissions for heart failure (HF). CONCLUSIONS: In our series, patients with TTRA showed clinical, electrocardiographic and echocardiographic differences compared to patients with HHD, with increased risk of need for PCM.

Electrocardiographic features and need for pacemaker in cardiac amyloidosis: Analysis of 58 cases. / Alteraciones electrocardiográficas y necesidad de marcapasos en amiloidosis cardiaca: análisis de 58 casos.

AIM: Amyloidosis is a disease in which amyloid fibrils can be deposited in different cardiac structures, and several electrocardiographic abnormalities can be produced by this phenomenon. The objective of this study was to describe the most common basal electrocardiographic alterations in patients diagnosed with cardiac amyloidosis (CA) and to determine if these abnormalities have an impact on the need of pacemaker. METHODS: This retrospective study included patients who had an established diagnosis of CA [light-chain cardiac amyloidosis (LA-CA) or transthyretin cardiac amyloidosis (TTR-CA)] between January 2013 and March 2021. The baseline heart rate, the percentage of patients with a pseudo-infarct pattern, low-voltage pattern or cardiac conductions disturbances, and the impact of these factors on the need of pacemaker were analysed. RESULTS: Fifty-eight patients with CA (20 with LA-CA and 38 with TTR-CA) were included, and the majority were male (69.0%). Twenty-one patients had atrial fibrillation (AF) at diagnosis. Thirty-five patients had a pseudo-infarct pattern, 35% had a low-voltage pattern, and 22% had criteria for ventricular hypertrophy. Two hirds had a conduction disorder: 18 patients with first degree atrioventricular block, 12 right bundle branch block, 3 left bundle branch block and 25 with a branch hemiblock. There were no differences between LA-CA and TTR-CA. Patients with TTR-CA had a greater need for pacemakers in the folow-up (39±40 meses). Bundle branch block was a predictor of the need for a permanent pacemaker (HR: 23.43; CI 95%: 4.09.134.09; P=.01). CONCLUSIONS: Electrocardiographic abnormalities in patients diagnosed wich CA are heterogeneus. Most frecuent is the presence of conduction disorders, the pseudoinfarction pattern, followed by the low voltage pattern. Patients with any bundle branch block at the baseline electrocardiogram need more frecuent to require a pacemaker during follow-up, especially in TTR-CA.