Echocardiographic Features Defining Right Dominant Unbalanced Atrioventricular Septal Defect

Cohen, Meryl S.; Jegatheeswaran, Anusha; Baffa, Jeanne M.; Gremmels, David B.; Overman, David M.; Caldarone, Christopher A.; McCrindle, Brian W.; Mertens, Luc

doi:10.1161/circimaging.112.000189

Cited by 64 publications

(43 citation statements)

References 12 publications

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“…Right ventricular (RV) performance is known to be a major determinant of clinical status and long-term outcomes in patients with pulmonary hypertension, cardiomyopathies, and congenital heart disease. [1][2][3][4][5][6][7][8][9][10][11][12][13][14] Cardiovascular magnetic resonance (CMR) imaging is considered the reference for RV volume and ejection fraction (EF). 15 Because of the complicated structure and geometry of the right ventricle, conventional two-dimensional echocardiography is unable to correctly estimate RV volume using reasonable geometric assumptions.…”

mentioning

confidence: 99%

Novel Approach to Three-Dimensional Echocardiographic Quantification of Right Ventricular Volumes and Function from Focused Views

Medvedofsky

Addetia

Patel

et al. 2015

Journal of the American Society of Echocardiography

106

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mentioning

confidence: 99%

Novel Approach to Three-Dimensional Echocardiographic Quantification of Right Ventricular Volumes and Function from Focused Views

Medvedofsky

Addetia

Patel

et al. 2015

Journal of the American Society of Echocardiography

106

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“…17 Cluster analysis has been used successfully to classify patients with congenital abnormalities into clinically relevant subgroups based on their phenotype, 18 including patient classification based on cardiac imaging. [19][20][21][22] Adequate sample size for cluster analysis was determined considering the number of variables, which may exponentially increase data dimensionality. For this purpose, an approach has been proposed in which meaningful classifications can be obtained using a sample size of 2 × , where x is the number of variables.…”

Section: Discussionmentioning

confidence: 99%

Identification of clinically relevant phenotypes in patients with Ebstein anomaly

Cabrera

Miranda-Fernández

Huertas-Quiñones

et al. 2018

Clinical Cardiology

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BackgroundEbstein anomaly (EA) is a heterogeneous congenital heart defect (CHD), frequently accompanied by diverse cardiac and extracardiac comorbidities, resulting in a wide range of clinical outcomes.HypothesisPhenotypic characterization of EA patients has the potential to identify variables that influence prognosis and subgroups with distinct contributing factors.MethodsA comprehensive cross‐sectional phenotypic characterization of 147 EA patients from one of the main referral institutions for CHD in Colombia was carried out. The most prevalent comorbidities and distinct subgroups within the patient cohort were identified through cluster analysis.ResultsThe most prevalent cardiac comorbidities identified were atrial septal defect (61%), Wolff‐Parkinson‐White syndrome (WPW; 27%), and right ventricular outflow tract obstruction (25%). Cluster analysis showed that patients can be classified into 2 distinct subgroups with defined phenotypes that determine disease severity and survival. Patients in cluster 1 represented a particularly homogeneous subgroup with a milder spectrum of disease, including only patients with WPW and/or supraventricular tachycardia (SVT). Cluster 2 included patients with more diverse cardiovascular comorbidities.ConclusionsThis study represents one of the largest phenotypic characterizations of EA patients reported. The data show that EA is a heterogeneous disease, very frequently associated with cardiovascular and noncardiovascular comorbidities. Patients with WPW and SVT represent a homogeneous subgroup that presents with a less severe spectrum of disease and better survival when adequately managed. This should be considered when searching for genetic causes of EA and in the clinical setting.

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“…RV/LV inflow angle was measured from the apical 4 chamber view. This angle was obtained by placing a line from the AV valve insertion point on the right and left ventricular free wall to the crest of the septum 16 ( Figure 1C). The aortoseptal angle was measured from the parasternal long axis images.…”

Section: Methodsmentioning

confidence: 99%

Echocardiographic Predictors of Left Ventricular Outflow Tract Obstruction following Repair of Atrioventricular Septal Defect

Abarbanell

Morrow

Kelleman

et al. 2016

Congenital Heart Disease

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Echocardiographic Features Defining Right Dominant Unbalanced Atrioventricular Septal Defect

Cited by 64 publications

References 12 publications

Novel Approach to Three-Dimensional Echocardiographic Quantification of Right Ventricular Volumes and Function from Focused Views

Novel Approach to Three-Dimensional Echocardiographic Quantification of Right Ventricular Volumes and Function from Focused Views

Identification of clinically relevant phenotypes in patients with Ebstein anomaly

Echocardiographic Predictors of Left Ventricular Outflow Tract Obstruction following Repair of Atrioventricular Septal Defect

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