case study of valvular heart disease

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Article Contents

142 a sub-aortic valve mass in a rheumatoid arthritis patient: an unconventional mechanism of aortic regurgitation, 143 symptomatic severe aortic regurgitation with coronary obstruction due to chronic type a dissection, 144 mitral valve prolapse and ventricular tachycardia: a long-lasting love story.

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Clinical Cases: Valvular heart disease

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Clinical Cases: Valvular heart disease, European Heart Journal - Cardiovascular Imaging , Volume 17, Issue suppl_2, December 2016, Pages ii12–ii13, https://doi.org/10.1093/ehjci/jew234

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Rheumatoid arthritis (RA) is a chronic inflammatory disease of unknown aetiology, clinically characterized by a symmetric peripheral polyarthritis. RA can affect cardiovascular (CV) system, leading to a wide range of manifestations that can remain clinically silent or lead to substantial morbidity and mortality. Indeed, CV disease is the most common cause of death in RA patients.

A 46-year-old woman with a history of RA on disease–modifying antirheumatic drug, was referred to our hospital due to palpitations with 1-year onset and ventricular tachycardia episodes on 24-hour Holter monitoring. On CV examination, there were signs of aortic regurgitation (AR), without congestive heart failure; on articular examination, there were no signs of acute or chronic joint damage. Basal electrocardiogram (ECG) showed sinus rhythm, with 63 bpm, and right bundle branch block; an ECG recorded during tachycardia relapse showed wide QRS complexes, with left bundle branch block morphology and inferior axis. Transthoracic echocardiogram (echo) showed a tricuspid aortic valve, with a non-mobile sub-aortic valve mass, adherent to the right coronary cusp (RCC) and to membranous interventricular septum, causing severe AR and left ventricular outflow tract obstruction, with a non-dilated and normal functioning left ventricle. There was no clinical or analytical data supporting an inflammatory or infectious process, nor a previous echo showing heart valve disease (HVD). A transoesophageal echo supported the presence of a sub-aortic valve mass, adherent to the RCC, which lead to leaflet retraction on diastole and causing an eccentric AR jet, impinging on the anterior mitral valve leaflet. On cardiac magnetic resonance, a T1w isointense structure was observed, with hyperintensity on T1 fat suppression and T2w sequences; no gadolium uptake was detected on first passage, but a homogenous late enhancement was clear; these findings were consistent with a fibrous non-vascularized mass. At surgery, the RCC was found to be thickened and immobilized by the mass; both were excised as a single structure; the non coronary cusp was also found to be involved by the adjacent fibrous process, and an aortic valve replacement with a mechanical valve was performed. On histopathology, the resected valve and fibrous mass showed an intense inflammatory infiltrate, characterized by lymphocytes and plasma cells, with a focal granulomatous lesion being found. There were no signs of neoplasm and no microorganisms were identified. The overall appearance was of an inflammatory reaction in the aortic valve, with characteristic features of RA. The postoperative recovery was complicated by complete atrioventricular block, which required a permanent pacemaker implantation. No relapse of CV manifestations was observed on follow-up.

This case underscores the need to recognize RA as aetiology of HVD. In these patients, valve-sparing procedures may be undesirable as an autoimmune process underlies the pathological mechanism of disease.

Introduction: Aortic valve plasty has become an accepted alternative to surgical intervention in patients with aortic valve regurgitation (AR) who are candidates to surgery. It is important to identify the etiology of AR for successfull aortic valve plasty. We report a case of chronic type A dissection with severe AR and left main coronary artery (LMCA) obstruction.

Case report: A 48-year-old man with a history of hypertension was admitted because of worsening dyspnea over 5 months. An electrocardiogram revealed a sinus tachycardia at 102 beats per minutes with left ventricular hypertrophy. A transthoracic echocardiogram demonstrated left ventricular dilation and systolic dysfunction with regional hypokinesis of anterior and lateral wall. Small pericardial effusion was also noticed. The aortic valve was tricuspid. The non-coronary cusp was prolapsed with eccentric regurgitation. Quantitative analysis showed regurgitant volume was 69 ml, regurgitant fraction 64%, effective orifice area 0.38 cm2 and pressure half time 210 msec, indicating severe AR. Three-dimensional transesophageal echocardiography (TEE) revealed type A aortic dissection with avulsion of aortic valve commissures between non- and right-coronary cusp and non- and left-coronary cusp, resulting in the prolapse of non coronary cusp. Furthermore, the dissection extended to the LMCA. Contrast-enhanced computed tomography ascertained communicating type A dissection. Valve-sparing aortic root replacement and coronary artery bypass graft surgery were performed. The postoperative course was uneventful, residual regurgitation was absent and left ventricular function was restored to normal.

Discussion: The differential diagnosis of severe AR and reduced left ventricular systolic function with regional hypokinesis is presented as follows: long-standing aortic regurgitation, ischemic heart disease or some kind of myopathy. In our case, these observations indicated chronic type A aortic dissection with severe AR due to the avulsion of aortic valve commissures and LMCA dissection concurrently.

Abstract 143 Figure.

Introduction and case report description: A significant link has recently been reported between a quite frequent valvular disease, mitral valve prolapse (MVP) and sudden cardiac death (SCD). A 46-year-old man was admitted in our intensive care unit after an inaugural episode of sustained ventricular tachycardia (VT). VT spontaneously aborted 10 minutes later. The patient had a familial history of SCD and had undergone a thorough cardiological workup 3 years before. A high rate of premature ventricular beats led to an electrophysiological study but only induced polymorphic non-sustained VT. Cardiac MRI found a late gadolinium enhancement in the mid septal wall and in papillary muscles.

Description of the problem, procedures, techniques and/or equipment used: Echocardiography revealed a bileaflet MVP with an exaggerated traction motion of the basal infero-lateral LV wall with a trivial regurgitation. LVEF was measured at 55%, speckle tracking echocardiography (STE) revealed a preserved global peak longitudinal strain value (-18.4%) with significant abnormal regional post systolic deformation in basal segments and an early stretch in septal basal and infero-lateral median segments. Cardiac MRI identified LGE predominantly in the infero lateral and inferior basal walls and an uptake in papillary muscles.

Questions, problems or possible differential diagnosis: Numerous studies reaffirmed an undoubted connection between MVP and the occurrence of ventricular arrhythmias raising the issue of a need for a comprehensive evaluation of the MV morphology and motion. The presence of MVP in such context raises an important differential diagnosis concern in young patients with apparent normal heart, non-obvious underlying coronary artery disease or suspicion of channelopathy. The presence of fibrosis in MVP patients presenting a VT has been identified in previous studies predominantly in papillary muscles and the infero-basal wall. Histological and cardiac MRI confrontations have demonstrated a concordance between areas of myocardial fibrosis and LGE distribution. MVP responsible of SCD or otherwise aborted VT/VF has been called "malignant MVP" and may show a higher fibrotic burden that is a potential arrhythmogenic triger.

Answers and discussion: One of the many explications is the impact of MVP on regional and global ventricular function. The hypothesis at the origin of fibrosis is that the protruding leaflet creates a tension on the sub-valvular apparatus, transmitted to the LV through the papillary muscles. Repeated stretch on the myocardial wall by elongated chordae or leaflet, along with adaptative ventricular remodeling due to volume overload, may lead to interstitial inflammation and ultimately induce replacement fibrosis. The role of CMR in the detection of fibrosis seems difficult to generalize. Using STE, an abnormal early pre-systolic shortening and post-systolic deformation was identified despite preserved LVEF and peak longitudinal strain values. Post-systolic contractions and pre-stretch were noted in the surrounding segments of the insertions of papillary muscles. Premature ventricular beats may be generated in these segments due to the heterogeneity of ventricular electrical depolarization and repolarization.

Conclusions and implications for clinical practice: This case highlights the need?to find an innovative tool to detect, evaluate and quantify the MVP-myocardial?interaction and the subsequent connections with myocardial stretch and fibrosis. The precise relation between myocardial stretch, induced by a prolapsing leaflet, and myocardial fibrosis needs to be further evaluated in order to estimate their eventual role in the identification of a sub-group of MVP patients at risk of ventricular arrhythmia.

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