Echocardiographic diagnosis of cardiac amyloidosis: Does the masquerader require only a "cherry on top"?

Kiotsekoglou, Anatoli; Saha, Samir K.; Nanda, Navin C.; Lindqvist, Per

doi:10.1111/echo.14952

Cited by 7 publications

(7 citation statements)

References 15 publications

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“…Aside from pleural effusion, cardiomyopathy is also one of the many complications suffered by our patients. Cardiac amyloidosis leads to restrictive infiltrative cardiomyopathy and heart failure, which may cause arrhythmias, embolic events, and sudden death [ 6 ]. On echocardiography, the appearance of "cherry‐like" apical sparing strain on two‐dimensional speckle‐tracking echocardiography based on Kumamoto criteria is associated with the increased left and right ventricular (LV, RV) thickness, atrial enlargement, restrictive LV filling pattern, and pericardial effusion raises suspicion for cardiac amyloidosis [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Primary Systemic Amyloidosis With Cardiac and Renal Involvement

Wong¹,

Wong²,

Nazri³

et al. 2022

Cureus

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Systemic amyloidosis is a life-threatening disorder with a poor prognosis. Accurate and early diagnosis of the condition is of paramount importance as early initiation of therapy improves the prognosis and survival rate. A 49-year-old gentleman presented with recurrent right exudative pleural effusion. Thoracocentesis revealed unexplained exudative pleural effusion. Pleuroscopy and pleural biopsy showed chronic inflammatory changes with no atypical cells. Echocardiography revealed global dilated cardiomyopathy with an ejection fraction (EF) of 35%. He also had nephrotic range proteinuria of 2.83g/dL. A cystoscopy examination was performed due to macroscopic haematuria, and the bladder biopsy revealed focal acellular eosinophilic material within the stroma. Salmon red staining and apple-green birefringence were noticed under polarizing microscopy, suggestive of amyloidosis. Serum protein electrophoresis revealed raised alpha 1 globulin and alpha 2 globulins which support the diagnosis of primary systemic amyloidosis. Unfortunately, the patient passed away before the initiation of treatment due to cardiogenic shock. Early and less invasive tests for diagnosing systemic amyloidosis, such as abdominal fat pad aspiration and salivary gland biopsy, can be done. Given its systemic nature, early complications screening may benefit patients whereby targeted treatment can be given.

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Section: Discussionmentioning

confidence: 99%

Primary Systemic Amyloidosis With Cardiac and Renal Involvement

Wong¹,

Wong²,

Nazri³

et al. 2022

Cureus

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show abstract

“…However, diastolic dys-function is a central aspect of CA, and this case was no exception, since grade III diastolic dysfunction was found with increased LV filling pressures and restrictive trans-mitral flow velocity (E/A 2.9, E wave 1.18 m/s, A 0.40 m/s, acceleration 111 ms), and the estimation of spectral Doppler velocities showed E/e’ 29.5 relation. In addition, there was biatrial enlargement (left atrial volume index: 74 ml/m2 and right volume index: 50 ml/m 2 ), which aided the diagnosis [ 9 – 11 ]. Unfortunately, the acoustic window for this patient did not allow the determination of the global longitudinal strain where the “apical sparing” phenomenon is characteristic of CA.…”

Section: Discussionmentioning

confidence: 99%

Acute Mitral Regurgitation Due to Chordae Tendineae Rupture: A Rare Presentation of Cardiac Amyloidosis

et al. 2022

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Patient: Male, 22-year-old Final Diagnosis: Cardiac amyloidosis Symptoms: Functional class deterioration (I-IV) • ascending pelvic extremity edema • chest pain Medication: — Clinical Procedure: — Specialty: Cardiology Objective: Unusual clinical course Background: In cardiac amyloidosis (CA), misfolded proteins deposit in the extracellular space of cardiac tissue. These deposits classically cause restrictive cardiomyopathy with diastolic dysfunction. Although there are at least 30 proteins known to cause amyloid aggregates, 2 main types make up most diagnosed cases: light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). Since CA is considered a rare condition, it is often underdiagnosed or recognized in the advanced stages. Once amyloid deposits involve the heart tissue, they are associated with a worse outcome and higher mortality rates, especially in patients presenting symptoms of heart failure. Case Report: We report a case of a 22-year-old man presenting with acute severe mitral regurgitation, secondary to posterior mitral leaflet chordae tendineae rupture (CTR). Surgical mitral valve replacement with a mechanical pros-thesis was performed, and cardiac tissue biopsy samples were obtained. After surgery, the patient improved significantly but suddenly presented with hemodynamic deterioration, until he died due to severe hemodynamic compromise and multiorgan failure. Although the etiology of the CTR was not established before surgical intervention, the histopathological analysis suggested CA. Conclusions: CA diagnosis can be complex, especially in a 22-year-old-man with atypical clinical and imaging manifestations. In this patient, other differential diagnoses were considered, since CA presenting in a young patient is a rare phenomenon and acute mitral regurgitation secondary to CTR presents more frequently in other heart conditions. Furthermore, rapid postoperative deterioration resulted in the patient’s death before biopsy samples were available because suspicion of amyloidosis had not been raised until that point.

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“…Echocardiographic diagnosis of cardiac amyloidosis is challenging because of diverse forms of clinical presentation and imaging. Besides some dimensional and functional left ventricular (LV) echocardiographic parameters like LV posterior wall thickness >13.6 mm, LV septal wall thickness >16 mm, E / E ′ ratio (or diastolic index) >14 and at least grade 2 diastolic dysfunction, there are a few speckle tracking echocardiographic (STE) parameters that could raise the suspicion and refine cardiac amyloidosis diagnosis: apex‐to‐base LV strain ratio >2.5; right ventricle apical sparing with right ventricle apical free wall strain >27%; right atrial reservoir strain by 3D STE strain >24%; left atrial reservoir strain <29% 1 . Due to the multiple echo parameters identified in this complex disease, it was imagine multiparametric echocardiographic scores to facilitate the diagnosis of these patients.…”

Section: Figurementioning

confidence: 99%

“…STE has brought a new index in the evaluation of cardiac amyloidosis namely relative apical sparing longitudinal index or the classic Bull's eye pattern of strain distribution or “cherry‐shaped” apical strain pattern or “cherry‐like” strain preservation pattern in the apex segment, comparing with other LV segments 1 . Relative apical sparing of myocardial longitudinal strain seems to be due to the regional differences in total amyloid mass instead of the proportion of amyloid deposits.…”

Section: Figurementioning

confidence: 99%