Isolated right ventricular noncompaction in an adult woman with severe pulmonary hypertension following at the intensive care unit

2021 ◽  
Author(s):  
Mustafa Yildiz ◽  
Yasemin Ozsahin ◽  
Hulya Yilmaz Ak ◽  
Dogac Oksen
Keyword(s):  
Magnetic Resonance Imaging ◽  
Pulmonary Hypertension ◽  
Magnetic Resonance ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Heart Catheterization ◽  
Severe Pulmonary Hypertension ◽  
Resonance Imaging ◽  
Atypical Chest Pain ◽  
Ventricular Noncompaction

Pulmonary hypertension is defined by a mean pulmonary artery pressure ≥25 mmHg at rest, measured during right heart catheterization. Ventricular noncompaction is a genetic cardiomyopathy which mostly effects left ventricle. It is related with deterioration of myocardial embryogenesis and commonly together with other cardiac diseases (1). Isolated ventricular non-compaction is characterized by modified morphology of myocardial wall, increased trabeculation in ventricular cavity and deep intertrabecular recesses. A 43-year-old woman presented exercise induced dyspnea and atypical chest pain. She has not any medical history prior. On admission, her 12 lead ECG showed complete righ bundle branch block, her blood pressure was 120/80 and pulse rate 80 per minutes. Transthoracic 2D echocardiogram and magnetic resonance imaging showed dilated and hypertrophied right ventricle with non-compaction of the right ventricular apex. The systolic pulmonary arterial pressure was 80 mmHg on the Doppler echocardiography. The coronary angiography revealed normal coronary arteries. The catheterization was showed pulmonary hypertension, right ventricle non-compaction and negative pulmonary vasoreactivity testing. Ventricular noncompaction, especially right ventricular noncompaction, complicated by severe pulmonary hypertension is exceptional. Only a few isolated right ventricular noncompaction has been reported but inclusion of pulmonary hypertesion cases are rare subsets . Diagnosis of pulmonary hypertension may be a consequence of increased pulmonary venous pressures caused by systolic and diastolic ventricular dysfunction secondary to right ventricular noncompaction. Widespread usage of cardiac magnetic resonance imaging, may enhance visual quality and evaluation of ventricular morphology, probably this will provide prevalance increment and clinical outcome improvements. Early diagnosis would bring better results.

scholarly journals Metabolic pathways associated with right ventricular adaptation to pulmonary hypertension: 3D analysis of cardiac magnetic resonance imaging

2018 ◽  
Vol 20 (6) ◽  
pp. 668-676 ◽  
Author(s):  
Mark I Attard ◽  
Timothy J W Dawes ◽  
Antonio de Marvao ◽  
Carlo Biffi ◽  
Wenzhe Shi ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Pulmonary Hypertension ◽  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Right Ventricular ◽  
Cardiac Magnetic Resonance Imaging ◽  
Metabolic Pathways ◽  
Wall Stress ◽  
3D Analysis ◽  
Resonance Imaging

Abstract Aims We sought to identify metabolic pathways associated with right ventricular (RV) adaptation to pulmonary hypertension (PH). We evaluated candidate metabolites, previously associated with survival in pulmonary arterial hypertension, and used automated image segmentation and parametric mapping to model their relationship to adverse patterns of remodelling and wall stress. Methods and results In 312 PH subjects (47.1% female, mean age 60.8 ± 15.9 years), of which 182 (50.5% female, mean age 58.6 ± 16.8 years) had metabolomics, we modelled the relationship between the RV phenotype, haemodynamic state, and metabolite levels. Atlas-based segmentation and co-registration of cardiac magnetic resonance imaging was used to create a quantitative 3D model of RV geometry and function—including maps of regional wall stress. Increasing mean pulmonary artery pressure was associated with hypertrophy of the basal free wall (β = 0.29) and reduced relative wall thickness (β = −0.38), indicative of eccentric remodelling. Wall stress was an independent predictor of all-cause mortality (hazard ratio = 1.27, P = 0.04). Six metabolites were significantly associated with elevated wall stress (β = 0.28–0.34) including increased levels of tRNA-specific modified nucleosides and fatty acid acylcarnitines, and decreased levels (β = −0.40) of sulfated androgen. Conclusion Using computational image phenotyping, we identify metabolic profiles, reporting on energy metabolism and cellular stress-response, which are associated with adaptive RV mechanisms to PH.

scholarly journals Interventional Cardiovascular Magnetic Resonance Imaging (iCMR) in an Adolescent with Pulmonary Hypertension

Medicina ◽  
2020 ◽  
Vol 56 (12) ◽  
pp. 636
Author(s):  
Surendranath R. Veeram Reddy ◽  
Yousef Arar ◽  
Tarique Hussain ◽  
Gerald Greil ◽  
Luis Zabala ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Pulmonary Hypertension ◽  
Magnetic Resonance ◽  
Septal Defect ◽  
Heart Defects ◽  
Ductus Arteriosus ◽  
Severe Pulmonary Hypertension ◽  
Resonance Imaging ◽  
Catheterization Procedure

The interventional cardiac magnetic resonance imaging (iCMR) catheterization procedure is feasible and safe for children and adults with pulmonary hypertension and congenital heart defects (CHD). With iCMR, the calculation of pulmonary vascular resistance (PVR) in children with complex CHD with multilevel shunt lesions is accurate. In this paper, we describe the role of the MRI-guided right-sided cardiac catheterization procedure to accurately estimate PVR in the setting of multiple shunt lesions (ventricular septal defect and patent ductus arteriosus) and to address the clinical question of operability in an adolescent with trisomy 21 and severe pulmonary hypertension.

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