scholarly journals Coexistence of aortic valve stenosis and cardiac amyloidosis: echocardiographic and clinical significance

2019 ◽  
Vol 17 (1) ◽  
Author(s):  
Gergely Peskó ◽  
Zsigmond Jenei ◽  
Gergely Varga ◽  
Astrid Apor ◽  
Hajnalka Vágó ◽  
...  

Abstract Background Left ventricular hypertrophy and diastolic dysfunction are common echocardiographic features of both aortic valve stenosis (AS) and cardiac amyloidosis (CA). These two different entities therefore may mask each other. From recent years, there is a growing body of evidence about the relatively high incidence of wild-type transthyretin (wtTTR) amyloidosis in AS, but there are scarce data on the prevalence of AS in CA, particularly in AL-type amyloidosis. The echocardiographic approach to these patients is not obvious, and not evidence based. We aimed to study the prevalence, severity, and type of AS in patients with CA and also to evaluate the potential of echocardiography in the diagnostic process. Methods Between January 2009 and January 2019, we retrospectively analyzed the clinical and echocardiographic data, and the echocardiographic work up of 55 consecutive CA patients. Results 80% of our CA patients had AL amyloidosis. We identified 5 patients (9%) with moderate to severe AS: two with moderate AS and three with low-flow, low-grade AS (LFLG AS). Further analysis of the latter three patients with dobutamine stress echocardiography revealed pseudo-severe LFLG AS in two, and true-severe AS in one patient. Conclusion The prevalence of moderate to severe AS is 9% in our population of CA patients, the majority of whom have AL amyloidosis. Dobutamine echocardiography seems to be appropriate for the further characterization of patients with LFLG AS, even with normal ejection fraction.

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Stoebe ◽  
J Kandels ◽  
B Tayal ◽  
U Laufs ◽  
P Sogaard ◽  
...  

Abstract Background The prognostic and therapeutic consequences of aortic valve stenosis (AS) are incompletely described by the effective orifice area (EOA) and transvalvular flow velocities (AV-Vmax) in echocardiography. The aim of the study was to evaluate parameters of left ventricular hypertrophy (LVH), diastolic dysfunction and pulmonary artery hypertension (PAH) in severe AS. Methods Patients (n = 320) with severe AS (mean age 78 ± 9.5 years) defined by EOA < 1cm2 and/or a AV-Vmax > 4m/s were included. Left ventricular (LV) volumes and ejection fraction (EF), relative wall thickness (RWT), LV mass index (LVmassi), several parameters of diastolic function, e.g. E/A-ratio, e/e", indexed left atrial (LA) volumes (LAVI min. and max.) and systolic pulmonary artery pressure were assessed. Patients were grouped in four subgroups according to their mean pressure gradient (mPGAV) and indexed stroke volume (Low-Flow/Low-Gradient (LFLG), Normal-Flow/Low-Gradient (NFLG), Low-Flow/High-Gradient (LFHG) and Normal-Flow/High-Gradient (NFHG)). Results LVH was documented in 83%, diastolic dysfunction in 75% and PAH in 79% of all patients, whereas > 50% exhibited pathological values for all three and 82% for at least two of these cardiac alterations. Further, in no patient normal ranges of all three cardiac alterations were observed. EOA (0.74 ± 0.18 vs 0.78 ± 0.17, p = 0.047) was lower and mPGAV (34.0 ± 16.6 vs 29.3 ± 14.1, p = 0.009) was higher in patients presenting all three cardiac alterations vs. patients presenting less than three cardiac alterations. Prevalences of diastolic dysfunction and LVH did not differ among AS subgroups (Fig.1/Tab. 1, p > 0.05). In contrast, higher prevalence of PAH was observed in HG-AS (89%) compared to LG-AS (76%) (p = 0.02). Conclusion Severe AS is highly associated with LVH, diastolic dysfunction and PAH. This analysis sets the stage to determine the prognostic importance of the analyzed cardiac alterations in patients with severe AS. Tab.1-Prevalence of cardiac alterations Parameters LFLG-AS (n = 135) NFLG-AS (n = 97) LFHG-AS (n = 21) NFHG-AS (n = 67) Relative wall thickness > 0.42 121 (89%) 90 (92%) 19 (90%) 67 (100%) LVmass index > 115 g/m2 (men), > 95 g/m2 (women) 120 (88%) 84 (86%) 17 (81%) 62 (93%) e/e" > 14 94 (70%) 56 (59%) 15 (71%) 41 (61%) Tricuspid regurgitation > 2.8 m/s 95 (70%) 71 (75%) 16 (76%) 47 (70%) Increased LAP and diastolic dysfunction grade 2 or 3 103 (76%) 69 (73%) 17 (81%) 50 (75%) Abstract P1367 Figure. Fig.1-Prevalence of cardiac alterations


2021 ◽  
Vol 46 (5) ◽  
pp. 100801
Author(s):  
João Abecasis ◽  
Daniel Gomes Pinto ◽  
Sância Ramos ◽  
Pier Giorgio Masci ◽  
Nuno Cardim ◽  
...  

2021 ◽  
Vol 5 (2) ◽  
Author(s):  
Nicholas Sunderland ◽  
Ahmed El-Medany ◽  
Justin Temporal ◽  
Laura Pannell ◽  
Gemina Doolub ◽  
...  

Abstract Background  The Gerbode defect is a rare abnormal communication between the left ventricle (LV) and right atrium (RA). The lesion is either congenital or acquired. Acquired defects are largely iatrogenic or infective in origin. We present two cases of acquired Gerbode defects with similar clinical presentations but very different outcomes. Case summaries Patient 1 A 64-year-old male presented with features of decompensated cardiac failure and a low-grade temperature. Dehiscence of a recently implanted bioprosthetic aortic valve and high-velocity LV to RA jet (Gerbode defect) was found on echocardiography. Blood cultures grew Staphylococcus warneri and the diagnosis of infective endocarditis was established. The patient was treated with intravenous antibiotics and the aortic valve and Gerbode defect were successfully surgically repaired. Patient 2 An 81-year-old male presented after being found on the floor at home. On admission, he was clinically septic with evidence of decompensated heart failure. No clear infective focus was initially found. Transthoracic echocardiography revealed severe left ventricular impairment, with a normal bioprosthetic aortic valve. He was treated with intravenous antibiotics, but later deteriorated with evidence of embolic phenomena. Repeat echocardiography revealed a complex infective aortic root lesion with bioprosthetic valve dehiscence and flow demonstrated from the LV to RA. Unfortunately, the patient succumbed to the infection and cardiac complications. Discussion  The Gerbode defect is a rare but important complication of infective endocarditis and valve surgery. Care needs to be taken to assess for Gerbode defect shunts on echocardiogram, especially in the context of previous cardiac surgery.


Author(s):  
Said Alsidawi ◽  
Sana Khan ◽  
Sorin V. Pislaru ◽  
Jeremy J. Thaden ◽  
Edward A. El-Am ◽  
...  

Background: Atrial fibrillation (AF) is a low-flow state and may underestimate aortic stenosis (AS) severity. Single-high Doppler signals (HS) consistent with severe AS (peak velocity ≥4 m/s or mean gradient ≥40 mm Hg) are averaged down in current practice. The objective for the study was to determine the significance of HS in AF low-gradient AS (LGAS). Methods: One thousand five hundred forty-one patients with aortic valve area ≤1 cm 2 and left ventricular ejection fraction ≥50% were identified and classified as high-gradient AS (HGAS) (≥40 mm Hg) and LGAS (<40 mm Hg), and AF versus sinus rhythm (SR). Available computed tomography aortic valve calcium scores (AVCS) were retrieved from the medical record. Outcomes were assessed. Results: Mean age was 76±11 years, female 47%. Mean gradient was 51±12 in SR-HGAS, 48±10 in AF-HGAS, 31±5 in SR-LGAS, and 29±7 mm Hg in AF-LGAS, all P ≤0.001 versus SR-HGAS; HS were present in 33% of AF-LGAS. AVCS were available in 34%. Compared with SR-HGAS (2409 arbitrary units; interquartile range, 1581–3462) AVCS were higher in AF-HGAS (2991 arbitrary units; IQR1978–4229, P =0.001), not different in AF-LGAS (2399 arbitrary units; IQR1817–2810, P =0.47), and lower in SR-LGAS (1593 arbitrary units; IQR945–1832, P <0.001); AVCS in AF-LGAS were higher when HS were present ( P =0.048). Compared with SR-HGAS, the age-, sex-, comorbidity index-, and time-dependent aortic valve replacement-adjusted mortality risk was higher in AF-HGAS (hazard ratio=1.82 [1.40–2.36], P <0.001) and AF-LGAS with HS (hazard ratio=1.54 [1.04–2.26], P =0.03) but not different in AF-LGAS without HS or SR-LGAS (both P =not significant). Conclusions: Severe AS was common in AF-LGAS. AVCS in AF-LGAS were not different from SR-HGAS. AVCS were higher and mortality worse in AF-LGAS when HS were present.


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