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Published on: June 9, 2018
Cardiac amyloidosis: a review of a series of cases
Marina Povar-Echeverría1, Pablo E Auquilla-Clavijo2, Bienvenido J Povar-Marco3
1Servicio de Medicina Interna. Hospital Universitario Miguel Servet, Zaragoza, España.
Insights
Cardiac amyloidosis is often underdiagnosed. Echocardiographic findings like biatrial dilatation and diastolic dysfunction, alongside clinical signs, should prompt further imaging for early diagnosis and improved outcomes.
Area of Science:
- Cardiology
- Medical Imaging
- Diagnostic Medicine
Background:
- Cardiac amyloidosis remains underdiagnosed despite diagnostic and therapeutic advancements.
- Early identification is crucial for improving patient prognosis and treatment efficacy.
Purpose of the Study:
- To review a series of cardiac amyloidosis cases.
- To describe key clinical data and imaging findings in diagnosed patients.
Main Methods:
- Retrospective analysis of 30 patients diagnosed with cardiac amyloidosis (2006-2016).
- Review of clinical data and imaging tests including ECG, echocardiogram, cardiac scintigraphy, and cardiac MRI.
- Patient follow-up until January 2018.
Main Results:
- The study included 30 patients (average age 65 years; 20 male).
- 97% showed biatrial dilatation, diastolic dysfunction, and left ventricular hypertrophy (LVH) on echocardiogram; only 6.7% met ECG criteria for LVH.
- 33% had systolic dysfunction; 33% underwent scintigraphy and cardiac MRI. 12-month survival was 61%.
Conclusions:
- Clinical suspicion for cardiac amyloidosis should be raised with heart failure, atrial fibrillation, or conduction disorders, especially with suggestive echocardiographic findings.
- Systolic dysfunction or female sex should not lower suspicion.
- Completion of diagnosis with scintigraphy and cardiac MRI is vital due to prognostic and therapeutic implications of early detection.
Antecedentes Y Objetivo:
La amiloidosis cardíaca es una entidad que permanece infradiagnosticada, a pesar de los avances recientes en su diagnóstico y tratamiento. El objetivo de este estudio es revisar una serie de casos de amiloidosis cardíaca para describir los principales datos clínicos y los hallazgos en las pruebas de imagen.
Materiales Y MéTodos:
Estudio retrospectivo de pacientes con diagnóstico principal o secundario de amiloidosis cardíaca en los informes de alta de pacientes hospitalizados en este centro desde 2006 hasta 2016. Se revisaron los datos clínicos de los pacientes, así como las pruebas de imagen (ECG, ecocardiograma, gammagrafía cardíaca, resonancia magnética cardíaca). Se realizó seguimiento de los pacientes hasta enero de 2018.
Resultados:
Se analiza a 30 pacientes (20 varones) con media de 65 años. Los principales datos ecocardiográficos fueron dilatación biauricular, disfunción diastólica e hipertrofia ventricular izquierda (HVI) en un 97%. Sólo el 6.7% cumplía criterios de HVI en el electrocardiograma. Hasta un 33% tenía disfunción sistólica. Se realizó gammagrafía y resonancia magnética cardíaca en un 33%. La supervivencia a los 12 meses fue de 61%.
ConclusióN:
La presencia de insuficiencia cardíaca, fibrilación auricular o trastornos de conducción junto a datos ecocardiográficos indicativos debe alertar al clínico. Otros datos como disfunción sistólica o sexo femenino no deben disminuir la sospecha. El estudio debe completarse con gammagrafía y resonancia magnética cardíaca, ya que el diagnóstico temprano tiene implicaciones pronósticas y terapéuticas.
Background And Objective:
Cardiac amyloidosis is an entity that remains underdiagnostic, despite recent advances in its diagnosis and treatment. The aim of this study is to review a series of diagnosed cases of cardiac amyloidosis to describe the main clinical data and the findings in the imaging tests.
Materials And Methods:
Retrospective study of patients with primary or secondary diagnosis of cardiac amyloidosis in discharge reports of patients hospitalized in our center from 2006 to 2016. The clinical data of the patients were reviewed, as well as the imaging tests (ECG, echocardiogram, cardiac scintigraphy, cardiac magnetic resonance). Patients were followed until January 2018.
Results:
We analyze 30 patients (20 men) with an average of 65 years. The main echocardiographic data were biatrial dilatation, diastolic dysfunction and left ventricular hypertrophy (LVH) in 97%. Only 6.7% met criteria for LVH in the electrocardiogram. Up to 33% had systolic dysfunction. Scintigraphy and cardiac magnetic resonance were performed in 33%. Survival at 12 months was 61%.
Conclusion:
The presence of heart failure, atrial fibrillation or conduction disorders with suggestive echocardiographic data should alert the clinician. Other data such as systolic dysfunction or female sex should not decrease the suspicion. The study should be completed with gammagraphy and cardiac magnetic resonance since early diagnosis has prognostic and therapeutic implications.
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