Valve Disease
Mary N. Sheppard in Practical Cardiovascular Pathology, 2022
Ebstein's anomaly is characterized by apical displacement of the posterior and septal leaflets of the tricuspid valve into the right ventricle, and occurs in approximately 1/200,000 live births. The septal and posterior leaflets are greatly enlarged and billow up into the right ventricular outflow tract resulting in obstruction. As a result of the displacement, there is poor coaptation of the valve leaflets leading to tricuspid regurgitation which, in turn, causes right atrial enlargement. Patients can survive into adulthood and present with right-sided heart failure, requiring tricuspid valve surgery. Supraventricular arrhythmia occurs frequently and ablation therapy is often indicated. These patients can present in adulthood with sudden death.14
Congenital mitral stenosis
Neeraj Parakh, Ravi S. Math, Vivek Chaturvedi in Mitral Stenosis, 2018
Chest radiograph has limited utility in the diagnosis of mild or moderate MS and, therefore, should not be routinely performed. Even in children with severe MS, chest x-ray is not a sensitive tool. Hemodynamically significant MS may present with radiologic signs of left-atrial enlargement and pulmonary venous hypertension. Cardiomegaly and right-atrial enlargement indicate pulmonary hypertension and right-ventricular dysfunction.
Cardiology
Stephan Strobel, Lewis Spitz, Stephen D. Marks in Great Ormond Street Handbook of Paediatrics, 2019
ECG may show right atrial enlargement, left ventricular hypertrophy and left axis deviation. Left ventricular hypertrophy may be visualised on CXR and pulmonary markings depend on the haemodynamics caused by the associated lesions.
Tricuspid Valve Dysplasia at Fetal Autopsy
Published in Fetal and Pediatric Pathology, 2022
Sumathi Shanmugam, Usha Nandhini Sennaiyan, Mani Ram Krishna
Ebstein anomaly and tricuspid valve dysplasia are the common right A-V valve anomalies described. The differentiation hinges on the displacement of the valve leaflets from the A-V groove. The valve leaflets are displaced apically and anteriorly in Ebstein’s anomaly but there is no displacement in tricuspid valve dysplasia. Lang and colleagues noticed that the right atrial enlargement resulted in the lungs being pushed to the side (a feature apparent in our autopsy). However the lung parenchyma and vessel histology was normal [1]. The characteristic morphological features of tricuspid valve dysplasia include thickening and nodularity of the leaflets and rolling of the leaflets. Such changes have been known to affect all 3 leaflets almost universally. The leaflets have been described as “bumpy” because of the nodularity [3].
COVID-19 in a Patient with β-Thalassemia Major and Severe Pulmonary Arterial Hypertension
Published in Hemoglobin, 2020
Valeria M. Pinto, Giorgio E. Derchi, Lorenzo Bacigalupo, Emanuele Pontali, Gian Luca Forni
From a clinical point of view, the patient was stable until hospitalization. His last 2D-Doppler echocardiogram showed right ventricle enlargement (basal diameter 63 mm) with mild reduced global systolic function [tricuspid annular plane systolic excursion (TAPSE) 15 mm]; right atrial enlargement (area 43 cm2); severe tricuspid regurgitation (vena contracta 7 mm) with regurgitant jet velocity 3.9 m/s (as index of severe pulmonary hypertension); inferior vena cava was enlarged 28 mm; aneurismatic dilatation of the pulmonary artery annulus (55 mm); normal left ventricular dimension and mild systolic dysfunction [ejection fraction (EF) 55.0%] with paradoxical movement of the interventricular septum (D-shape); and left atrial enlargement (area 31 cm2).
Current and emerging imaging techniques in the diagnosis and assessment of pulmonary hypertension
Published in Expert Review of Respiratory Medicine, 2018
Christopher S. Johns, Jim M. Wild, Smitha Rajaram, Andy J. Swift, David G. Kiely
Group 2: Patients with PH due to left heart disease often have biatrial dilation and there is a varying amount of left ventricular right ventricular and right atrial enlargement in this patient group (Figure 8). Features of PH seen in IPAH, such as septal flattening may be masked due to high left ventricular filling pressures, and the presence of a flattened septum in addition to features of left heart disease suggests a pre-capillary component to the PH. There may also be features of previous cardiac surgery or valvular replacement. Patients with left heart disease may also have features of cardiac decompensation such as pleural effusions, septal lines, ascites and reflux of contrast into the IVC/hepatic veins.
Related Knowledge Centers
- Cardiomegaly
- Jugular Venous Pressure
- Pulmonary Hypertension
- Tetralogy of Fallot
- Atrial Septal Defect
- Heart Failure
- Tricuspid Valve Stenosis
- Pulmonic Stenosis
- Tetralogy of Fallot
- Pulmonary Heart Disease
- Tricuspid Regurgitation