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Missed Tetralogy of Fallot in an Elderly Woman With a Known Ventricular Septal Defect.

ABSTRACT: Tetralogy of Fallot is a congenital heart disease comprised of a tetrad of ventricular septal defect, pulmonary stenosis, overriding aorta, and right ventricular hypertrophy. In developed countries, most cases are diagnosed in babies; mortality is high if not surgically corrected in a timely manner. We describe herein a woman who was diagnosed at age 73 years. Several factors accounted for her unusual longevity. We highlight the importance of multimodal imaging to look for other associated anomalies of tetralogy of Fallot in cases of apparent simple ventricular septal defect when the echocardiographic images are either suggestive or suboptimal.

SUBMITTER: Kalra D

PROVIDER: S-EPMC7710949 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature

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Missed Tetralogy of Fallot in an Elderly Woman With a Known Ventricular Septal Defect.

Kalra Dinesh D Sohal Sumit S

CJC open 20200621 6

Tetralogy of Fallot is a congenital heart disease comprised of a tetrad of ventricular septal defect, pulmonary stenosis, overriding aorta, and right ventricular hypertrophy. In developed countries, most cases are diagnosed in babies; mortality is high if not surgically corrected in a timely manner. We describe herein a woman who was diagnosed at age 73 years. Several factors accounted for her unusual longevity. We highlight the importance of multimodal imaging to look for other associated anoma ...[more]

PMID: 33305230

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Project description:Background and purposeElectromechanical dyssynchrony, manifested by right bundle branch block and regional wall mechanical dysfunction, contributes to inefficient RV function in repaired Tetralogy of Fallot (ToF). This study aims to evaluate the synchronicity of multiple RV walls using two-dimensional multi-plane echocardiography (2D-MPE) in order to augment current understanding of the mechanisms behind RV dyssynchrony.MethodsSixty-nine adult ToF patients [aged 33 (23-45) years; 61% male] and twenty-five matched healthy controls underwent deformational analysis of the RV lateral, anterior, inferior and septal walls following 2D-MPE acquisitions. RV synchronicity was assessed by the intra-RV deformation delay between each basal RV wall and mid-septal segment in addition to mechanical dispersion calculated across four, six and eight segments (MD).ResultsAll RV wall-septum delays plus MD-4 and MD-6 indices were significantly greater in ToF patients compared to healthy controls (p < 0.001-0.03). In ToF patients, the lateral and anterior RV walls were last to reach peak deformation and anterior wall longitudinal strain was lower (p = 0.001). Post systolic shortening of at least one RV wall segment was identified in 19 (28%) ToF patients. Despite similar ECG characteristics, lateral and anterior wall-septum delays were significantly longer in patients with greater degrees of dyssynchrony (73 [37-108]ms vs. 37 [0-63]ms, p = 0.006; 91 [52-116]ms vs. 41 [1-69]ms, p = 0.013), although RV ejection fraction (RVEF) was not significantly lower. MD-4 and MD-8 indices displayed moderate negative associations with RVEF, strengthened by inclusion of lateral wall longitudinal strain (r = 0.64/0.65; p ≤0.01).ConclusionRV dyssynchrony in ToF is characterised by electromechanical delays between the lateral, anterior and septal walls, with anterior wall dysfunction likely associated with surgical repair of the RV outflow tract. Prospectively, 2D-MPE may have an emerging role evaluating RV mechanical response to electrical resynchronisation therapy.

Dataset Information

Missed Tetralogy of Fallot in an Elderly Woman With a Known Ventricular Septal Defect.

Publications

Missed Tetralogy of Fallot in an Elderly Woman With a Known Ventricular Septal Defect.

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