Cardiac “hypertrophy” phenotyping: differentiating aetiologies with increased left ventricular wall thickness on echocardiography

Ferkh, A.; Tjahjadi, Catherina; Stefani, L.; Geenty, P.; Silva, Kasun De; Boyd, Anita; Richards, David; Mollee, Peter; Korczyk, Dariusz; Taylor, Mark; Kwok, Fiona; Kizana, Eddy; Ng, Arnold C.T.; Thomas, Liza

doi:10.3389/fcvm.2023.1183485

Cited by 5 publications

(1 citation statement)

References 42 publications

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“…While BHCM can be easily detected through echocardiography, it’s crucial to differentiate it from other hypertrophy-causing diseases or phenotypes, such as storage diseases and RASopathies [ 20 ]. It requires a complete diagnostic workup, including family history, clinical presentation, laboratory tests, detailed imaging examination, and genetic testing [ 21 ].…”

Section: Discussionmentioning

confidence: 99%

Clinical Effect of the Modified Morrow Septal Myectomy Procedure for Biventricular Hypertrophic Cardiomyopathy

Tan,

Zhu,

et al. 2024

Rev. Cardiovasc. Med.

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Background: Right ventricular involvement in hypertrophic cardiomyopathy is uncommon. This study aimed to evaluate clinical outcomes of the modified septal myectomy in patients diagnosed with biventricular hypertrophic cardiomyopathy (BHCM), a subject seldom explored in the literature. Methods: We conducted a retrospective cohort study from January 2019 to January 2023, enrolling 12 patients with BHCM. Each patient underwent a modified septal myectomy and was followed postoperatively. Clinical data and echocardiographic parameters, including the ventricular outflow tract peak pressure gradient and maximum interventricular septum thickness, were collected and analyzed. Results: The study cohort had a median age of 43.0 (interquartile range 14.5–63.0) years at surgery, with four patients (33.3%) being children. Two patients (16.7%) previously underwent percutaneous transluminal septal myocardial ablation. Surgical relief of biventricular outflow tract obstruction (BVOTO) was achieved in five patients (41.7%), aside from those managed solely for left ventricular outflow tract obstruction. In five instances, three-dimensional (3D) printing technology assisted in surgical planning. The postoperative interventricular septum thickness was significantly reduced (21.0 mm preoperative vs. 14.5 mm postoperative, p 0.001), effectively eliminating residual ventricular outflow tract obstruction. There were no severe complications, such as septal perforation or third-degree atrioventricular block. During a mean follow up of 21.2 15.3 months, no sudden deaths, residual outflow tract obstruction, permanent pacemaker implantation, recurrent systolic anterior motion, or reoperations were reported. Conclusions: Our findings affirm that the modified septal myectomy remains the gold standard treatment for BHCM, improving patient symptoms and quality of life. BVOTO relief can be safely and effectively achieved through septal myectomy via transaortic and pulmonary valve approaches in selected patients. For intricate cases, the application of 3D printing technology as a preoperative planning tool is advised to optimize surgical precision and safety.

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Section: Discussionmentioning

confidence: 99%

Clinical Effect of the Modified Morrow Septal Myectomy Procedure for Biventricular Hypertrophic Cardiomyopathy

Tan,

Zhu,

et al. 2024

Rev. Cardiovasc. Med.

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Diagnostic and prognostic value of the left atrial myopathy evaluation in cardiac amyloidosis using echocardiography

Ferkh,

Geenty,

Stefani

et al. 2024

ESC Heart Failure

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BackgroundCardiac amyloidosis (CA) is an under‐recognized cause of heart failure. Left atrial (LA) myopathy contributes to a worse prognosis in heart failure and is a feature of transthyretin (ATTR) and light‐chain (AL) CA. LA mechanical dispersion (LA‐MD) is a novel marker of intra‐atrial dyssynchrony implicated in LA myopathy and the future development of atrial fibrillation (AF).AimsThis study aimed to determine the characteristics and prognostic value of LA myopathy in ATTR and AL cardiomyopathy through a comprehensive LA echocardiographic evaluation.MethodsATTR (n = 86) and AL (n = 86) CA patients were compared with hypertensive heart disease (HHT) patients (n = 58). Transthoracic echocardiographic measurements including LA strain and LA‐MD were obtained with patient follow‐up for mortality.ResultsATTR and AL patients had a median follow‐up of 66 months, with 26 mortality events. Left ventricular (LV) mass, diastolic function (average‐e′ and E/e′), LV global longitudinal strain, and LA volume and function (LA function index and strain) were more impaired in ATTR versus AL; these echocardiographic parameters were more impaired in both amyloid groups compared to HHT patients (P < 0.05). LA‐MD was increased in ATTR versus AL [median 72.2 (inter‐quartile range 55–88.9) vs. 54 (43.5–64.2), respectively, P < 0.001]. Multivariable logistic regression adjusted for age, presence of AF, LV mass, global and basal strain, and E/e′ demonstrated that LA‐MD was an independent determinant of ATTR CA (P = 0.014). On multivariable analysis, LA reservoir strain was independently associated with the presence of heart failure in the CA group (P < 0.001). LA minimum volume (cut‐off ≥18 mL/m2) was a determinant of mortality in AL CA [Cox proportional hazard ratio (HR) 1.042 (1.003–1.082), P = 0.034 and Kaplan–Meier analysis, P = 0.016].ConclusionCharacterizing LA myopathy has significant diagnostic and prognostic utility in CA. ATTR patients have increased atrial dyssynchrony, which may have implications for AF development. LA reservoir strain was associated with heart failure in CA, whilst LA minimum volume was a predictor of mortality in AL CA.

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Limitations of apical sparing pattern in cardiac amyloidosis: a multicentre echocardiographic study

Cotella,

Randazzo,

Maurer

et al. 2024

European Heart Journal - Cardiovascular Imaging

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Background Although impaired left ventricular (LV) global longitudinal strain (GLS) with apical sparing is a feature of cardiac amyloidosis (CA), its diagnostic accuracy has varied across studies. We aimed to determine the ability of apical sparing ratio (ASR) and most common echocardiographic parameters to differentiate patients with confirmed CA from those with clinical and/or echocardiographic suspicion of CA, but with this diagnosis ruled out. Methods We identified 544 patients with confirmed CA and 200 controls as defined above (CTRL Patients). Measurements from transthoracic echocardiograms (TTE) were performed using artificial intelligence software (Us2.AI, Singapore) and audited by an experienced echocardiographer. Receiver-operating characteristic curve analysis was used to evaluate the diagnostic performance and optimal cutoffs for the differentiation of CA patients from CTRL Patients. Additionally, a group of 174 healthy subjects (Healthy CTRL) was included to provide insight on how Patients and Healthy controls differed echocardiographically. Results LV GLS was more impaired (-13.9 ± 4.6% vs -15.9 ± 2.7%, p < 0.0005) and ASR was higher (2.4 ± 1.2 vs 1.7 ± 0.9, p < 0.0005) in the CA group vs. CTRL Patients. Relative wall thickness and ASR were the most accurate parameters for differentiating CA from CTRL Patients (AUC: 0.77 and 0.74, respectively). However, even with the optimal cutoff of 1.67, ASR was only 72% sensitive and 66% specific for CA, indicating presence of apical sparing in 32% of CTRL Patients and even in 6% Healthy CTRLs. Conclusions Apical sparing did not prove to be a CA-specific biomarker for accurate identification of CA, when compared to clinically similar controls with no CA.

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Cardiac “hypertrophy” phenotyping: differentiating aetiologies with increased left ventricular wall thickness on echocardiography

Cited by 5 publications

References 42 publications

Clinical Effect of the Modified Morrow Septal Myectomy Procedure for Biventricular Hypertrophic Cardiomyopathy

Clinical Effect of the Modified Morrow Septal Myectomy Procedure for Biventricular Hypertrophic Cardiomyopathy

Diagnostic and prognostic value of the left atrial myopathy evaluation in cardiac amyloidosis using echocardiography

Limitations of apical sparing pattern in cardiac amyloidosis: a multicentre echocardiographic study

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