27064109 Visualization

A 53 Age - year Age - old Age man Sex came Clinical_event to our hospital Nonbiological_location with signs and symptoms of acute Detailed_description heart Disease_disorder failure Disease_disorder after a 2 Duration - week Duration history Duration of progressive Detailed_description breathlessness Sign_symptom .
He had a history History of History recurrent Frequency skin Biological_structure abscesses History and atopic Detailed_description dermatitis Disease_disorder and regularly Frequency visited Clinical_event a dermatologist Nonbiological_location in our hospital Nonbiological_location .
On arriving Clinical_event at our hospital Nonbiological_location , his extremities Biological_structure were warm Sign_symptom and dry Sign_symptom .
According to the New Diagnostic_procedure York Diagnostic_procedure Heart Diagnostic_procedure Association Diagnostic_procedure criteria Diagnostic_procedure , he had class Lab_value III Lab_value congestive Disease_disorder heart Disease_disorder failure Disease_disorder ( CHF Disease_disorder ).
An electrocardiogram Diagnostic_procedure revealed diffuse Detailed_description nonspecific Detailed_description T Sign_symptom - wave Sign_symptom changes Sign_symptom , low Lab_value voltage Diagnostic_procedure ( <5 Lab_value mm Lab_value ) in the extremity Diagnostic_procedure leads Diagnostic_procedure and poor Lab_value R Diagnostic_procedure - wave Diagnostic_procedure progression Diagnostic_procedure in the anterior Diagnostic_procedure chest Diagnostic_procedure leads Diagnostic_procedure .
Multiple Quantitative_concept sporadic Frequency ventricular Biological_structure premature Sign_symptom beats Sign_symptom were seen (Figure 1).
Chest Biological_structure x Diagnostic_procedure - ray Diagnostic_procedure film confirmed right Detailed_description pleural Disease_disorder effusion Disease_disorder , and mild Severity cardiomegaly Sign_symptom but no pulmonary Disease_disorder congestion Disease_disorder (Figure 2).
Blood Diagnostic_procedure tests Diagnostic_procedure showed severely Lab_value elevated Lab_value B Diagnostic_procedure - type Diagnostic_procedure natriuretic Diagnostic_procedure peptide Diagnostic_procedure ( 901 Lab_value pg/mL Lab_value ) and markedly raised Lab_value IgE Diagnostic_procedure ( 12 Lab_value 000 Lab_value IU/mL Lab_value ) without eosinophilia Sign_symptom ( eosinophil Diagnostic_procedure count Diagnostic_procedure of 1.62×108/L Lab_value ).
Biochemical Diagnostic_procedure analysis Diagnostic_procedure revealed no significant findings: blood Diagnostic_procedure urea Diagnostic_procedure nitrogen Diagnostic_procedure of 14.3 Lab_value mg/dL Lab_value [reference value (RV): 7.00–22.00], creatinine Diagnostic_procedure of 0.90 Lab_value mg/dL Lab_value (RV: 0.60–1.00), C Diagnostic_procedure - reactive Diagnostic_procedure protein Diagnostic_procedure ( CRP Diagnostic_procedure ) of 0.2 Lab_value mg/dL Lab_value (RV: 0.00–0.50), serum Diagnostic_procedure amyloid Diagnostic_procedure A Diagnostic_procedure ( SAA Diagnostic_procedure ) of 7.0 Lab_value μg/mL Lab_value (RV: 0–8.0), and troponin Diagnostic_procedure T Diagnostic_procedure of 0.07 Lab_value ng/mL Lab_value (RV: 0–0.1).
Immunology Diagnostic_procedure testing Diagnostic_procedure revealed negative Lab_value perinuclear Diagnostic_procedure anti Diagnostic_procedure - neutrophil Diagnostic_procedure cytoplasmic Diagnostic_procedure antibodies Diagnostic_procedure and no Lab_value elevation Lab_value of myeloperoxidase Diagnostic_procedure antibodies Diagnostic_procedure .
The distribution of albumin Diagnostic_procedure and globulin Diagnostic_procedure in the serum Detailed_description was normal Lab_value .
Serum Detailed_description protein Diagnostic_procedure immuno Diagnostic_procedure - electrophoresis Diagnostic_procedure did Lab_value not Lab_value reveal Lab_value M Diagnostic_procedure - protein Diagnostic_procedure , and urinalysis Diagnostic_procedure revealed no Lab_value Bence Diagnostic_procedure - Jones Diagnostic_procedure protein Diagnostic_procedure .
Transthoracic Biological_structure echocardiography Diagnostic_procedure (Figure 3) showed concentric Detailed_description mild Severity left Biological_structure ventricular Biological_structure ( LV Biological_structure ) hypertrophy Sign_symptom ( 12 Distance mm Distance ) without the characteristic granular Sign_symptom sparkling Sign_symptom appearance Sign_symptom and pericardial Disease_disorder effusion Disease_disorder , preserved Lab_value ejection Diagnostic_procedure fraction Diagnostic_procedure ( 60% Lab_value ), and bi Biological_structure - atrial Biological_structure enlargement Sign_symptom with normal Lab_value ventricular Diagnostic_procedure chambers Diagnostic_procedure .
Doppler Detailed_description - derived Detailed_description LV Biological_structure diastolic Diagnostic_procedure filling Diagnostic_procedure demonstrated a restrictive Lab_value pattern Lab_value with a trans Biological_structure - mitral Biological_structure early Diagnostic_procedure filling Diagnostic_procedure wave Diagnostic_procedure deceleration Diagnostic_procedure time Diagnostic_procedure of 160 Lab_value ms Lab_value and an elevated Lab_value E/A Diagnostic_procedure ratio Diagnostic_procedure of 2.8 Lab_value .
A markedly elevation Lab_value of E/e’ Diagnostic_procedure ratio Diagnostic_procedure of 27.3 Lab_value indicated elevated Lab_value LV Diagnostic_procedure filling Diagnostic_procedure pressure Diagnostic_procedure .
On day Date 1 Date of hospitalization Clinical_event , we prescribed an angiotensin Medication - converting Medication enzyme Medication (ACE) Medication inhibitor Medication and low Dosage - dose Dosage diuretics Medication .
On day Date 3 Date of hospitalization Clinical_event , after initiating ACE Medication inhibitor Medication and diuretic Medication therapy, the patient’s symptoms Sign_symptom resolved.
The dermatologists Nonbiological_location performed a biopsy Diagnostic_procedure of a blue Color macula Sign_symptom of the forehead Biological_structure skin Biological_structure .
On day Date 4 Date , we introduced a low Dosage - dose Dosage β Medication - blocker Medication and performed an endomyocardial Diagnostic_procedure biopsy Diagnostic_procedure ( EMB Diagnostic_procedure ), obtaining 3 Quantitative_concept fragments Quantitative_concept of the right Biological_structure ventricular Biological_structure septum Biological_structure because diagnostic confirmation of cardiac Biological_structure amyloidosis Disease_disorder requires the demonstration of amyloid Sign_symptom deposits Sign_symptom .
In addition, we performed right Diagnostic_procedure heart Diagnostic_procedure catheterization Diagnostic_procedure ( RHC Diagnostic_procedure ), and coronary Diagnostic_procedure angiography Diagnostic_procedure to exclude obstructive Disease_disorder coronary Disease_disorder artery Disease_disorder disease Disease_disorder .
The data obtained from the RHC Diagnostic_procedure indicated subset Lab_value type Lab_value IV Lab_value according to the Forrester Diagnostic_procedure classification Diagnostic_procedure .
The right Biological_structure ventricular Biological_structure pressure Diagnostic_procedure curve Diagnostic_procedure did not show a dip Sign_symptom - and Sign_symptom - plateau Sign_symptom configuration Sign_symptom .
Skin Biological_structure biopsy Diagnostic_procedure revealed hyperkeratosis Sign_symptom in the epidermis Biological_structure and mild Severity inflammatory Sign_symptom changes Sign_symptom throughout the dermis Biological_structure with an infiltration Sign_symptom of lymphocytes Biological_structure .
The specimen exhibited apple Color - green Color birefringence Lab_value with polarized Detailed_description light Detailed_description after Congo Diagnostic_procedure red Diagnostic_procedure staining Diagnostic_procedure (Figure 4).
Histological Diagnostic_procedure examination of the myocardial Biological_structure specimen Biological_structure showed no signs suggesting myocarditis Disease_disorder , eosinophilic Detailed_description granulomas Disease_disorder , or cardiomyopathy Disease_disorder with iron Detailed_description deposition Detailed_description , but Congo Diagnostic_procedure red Diagnostic_procedure staining Diagnostic_procedure revealed amyloid Sign_symptom deposits Sign_symptom (Figure 5).
In addition, a strongly Lab_value positive Lab_value immunohistochemical Diagnostic_procedure reaction Diagnostic_procedure to immunoglobulin Diagnostic_procedure λ Diagnostic_procedure - chain Diagnostic_procedure in the myocardial Biological_structure interstitium Biological_structure led us to diagnose this patient’s systemic Detailed_description amyloidosis Disease_disorder as AL Disease_disorder amyloidosis Disease_disorder .
The patient generally tolerated the low-dose β Medication - blocker Medication well and was discharged Clinical_event on day Date 10 Date with no complications Disease_disorder .
The patient is currently being followed Clinical_event - up Clinical_event at 6 Frequency - month Frequency intervals Frequency as an out-patient, with no exacerbation of the CHF Disease_disorder thus far.