Mankad, Sunil

This present case pertains to a 48-year-old woman with a history of antiphospholipid syndrome, who presented with progressive fatigue, generalized weakness, and orthopnea acutely. She had a prior diagnosis of antiphospholipid syndrome with recurrent deep vein thromboses (DVTs) and repeated demonstration of lupus anticoagulants. She presented in cardiogenic shock with markedly elevated troponin and global myocardial dysfunction on echocardiography, and cardiac catheterization revealed minimal disease. Cardiac magnetic resonance imaging was performed, which revealed findings of perfusion defects and microvascular obstruction, consistent with the pathophysiology of catastrophic antiphospholipid syndrome (CAPS). Diagnosis was made based on supportive imaging, including head magnetic resonance imaging (MRI) revealing multifocal, acute strokes; microvascular thrombosis in the dermis; and subacute renal infarctions. The patient was anticoagulated with intravenous unfractionated heparin and received high-dose methylprednisolone, plasmapheresis, intravenous immunoglobulin, and one dose each of rituximab and cyclophosphamide. She convalesced with eventual myocardial recovery after a complicated course. The diagnosis of CAPS relies on the presence of (1) antiphospholipid antibodies and (2) involvement of multiple organs in a microangiopathic thrombotic process with a close temporal association. The myocardium is frequently affected, and heart failure, either as the presenting symptom or cause of death, is common. Despite echocardiographic evidence of myocardial dysfunction in such patients, MRIs of CAPS have not previously been reported. This case highlights the utility in assessing the involvement of the myocardium by the microangiopathic process with MRI. Because the diagnosis of CAPS requires involvement in multiple organ systems, cardiac MRI is likely an underused tool that not only reaffirms the pathophysiology of CAPS, but could also clue clinicians in to the possibility of a diffuse thrombotic process.

Background— Fibro-elastic deficiency (FED) and diffuse myxomatous degeneration (DMD) are phenotypes of degenerative mitral valve disease defined morphologically. Whether physiological differences in annular and valvular dynamics exist between these phenotypes remains unknown. Methods and Results— We performed triple quantitation of cardiac remodeling and of mitral regurgitation severity and of annular and valvular dimensions by real-time 3-dimensional-transesophageal-echocardiography. Forty-nine patients with degenerative mitral valve disease classified as FED (n=31) and DMD (n=18) by surgical observation showed no difference in age (65±10 versus 59±13; P =0.5), body surface area (2.0±0.2 versus 2.0±0.2 m 2 ; P =0.5), left ventricular and atrial dimensions (all P >0.55), and mitral regurgitation regurgitant orifice ( P =0.62). On average, annular dimensions were larger in DMD versus FED, but height was similar resulting in lower saddle shape. Dynamically, annular DMD versus FED display poorer contraction and saddle-shape accentuation in early systole and abnormal enlargement, particularly intercommissural, in late-systole (all P <0.05). Valvular dynamics showed stable valvular area in systole in FED versus considerable systolic increased area in DMD ( P <0.001). Prolapse height and volume increased little throughout systole in FED versus marked increase in DMD ( P <0.001). Conclusions— Our novel observations show that FED and DMD, although both labeled myxomatous, display considerable physiological phenotypic differences. In DMD, the annular increased size and profoundly abnormal dynamics demonstrate DMD-specific annular degeneration compared with the enlarged but relatively normal FED annulus. DMD does not incur more severe mitral regurgitation, despite larger prolapse and valve redundancy, underscoring potential compensatory role of tissue redundancy of DMD (or aggravating role of tissue paucity of FED) on mitral regurgitation severity.

Objectives Cleft-like indentations (CLI) are deep separations between scallops of the mitral posterior leaflet observed in myxomatous mitral valve disease (MMVD), but their diagnosis, mechanisms and implications are unknown. Using 3D transoesophageal echocardiography (3DTOC), we aimed at assessing diagnostic accuracy and defining mechanisms of CLI in patients undergoing surgery for MMVD. Methods 3DTOC of mitral valve was acquired in 49 patients with MMVD and severe regurgitation prior to valve repair. Qualitative review compared 3DTOC diagnosis of CLI with surgical inspection. Mitral, annular and leaflet dimensions were quantified with dedicated software and compared between those with and without CLI. Results Diagnosis of CLI was made by 3DTOC in 17 (35%) while none was identified by 2D and was confirmed in 15 (88%) by surgical inspection. Mechanistically, LV diameters and mitral regurgitant volume (RVol) were similar with and without CLI (p>0.49). Conversely, mitral annulus was smaller with CLI (anteroposterior diameter 42.2±7.1 vs 47.0±7.5 mm, p=0.04; circumference 133±16 vs 148±19 mm, p=0.009; area 1289±326 vs 1619±427 mm2, p=0.008). Prolapse volume tended to be smaller with CLI (1.9±1.2 vs 4.0±4.3 mL, p=0.06) involving single posterior scallop at surgery (82% vs 44%, p=0.007) with smaller 3DTOC leaflet area (1574±409 vs 2019±652 mm2, p=0.01). During valve repair, surgical closure of all surgically diagnosed CLI was required. Conclusions Posterior leaflet CLI are frequent in MMVD, are identified by 3DTOC with high accuracy and require closure during valve repair. CLI are mechanistically not related to excess annular enlargement or excess prolapse. Conversely, CLI occur in the context of single scallop prolapse with tissue paucity causing excess separation of scallops. These 3DTOC data enhance diagnostic and mechanistic comprehension of the diversity of MMVD phenotypical presentation.

Background Three‐dimensional transthoracic echocardiography (3D‐ TTE ) with dedicated software permits quantification of mitral annulus dynamics and papillary muscle motion throughout the cardiac cycle. Methods and Results Mitral apparatus 3D‐ TTE was acquired in controls (n=42), patients with left ventricle dysfunction and functional mitral regurgitation ( LVD ‐ FMR ; n=43) or without FMR ( LVD ‐no MR , n=35). Annulus in both normal and LVD ‐no MR subjects displayed saddle shape accentuation in early‐systole (ratio of height to intercommissural diameter, 10.6±3.7 to 13.5±4.0 in normal and 9.1±4.3 to 12.6±3.6 in LVD ‐no MR ; P <0.001 for diastole to early‐systole motion, P = NS between those groups). In contrast, saddle shape was unchanged from diastole in FMR patients (10.0±6.4 to 8.0±5.2; P = NS , P <0.05 compared to both other groups). Papillary tips moved symmetrically towards to the midanterior annulus in control and LVD ‐no MR subjects, maintaining constant ratio of the distances between both tips to midannulus (Pt AR ) throughout systole. In LVD ‐ FMR patients midsystolic posterior papillary tip to anterior annulus distance was increased, resulting in higher Pt AR ( P =0.05 compared to both other groups). Mechanisms of early‐ and midsystolic FMR differed between different etiologies of LV dysfunction. In patients with anterior MI and global dysfunction annular function and dilatation were the dominant parameters, while papillary muscle motion was the predominant determinant of FMR in patients with inferior MI . Conclusions Inadequate early‐systolic annular contraction and saddle‐shape accentuation in patients with impaired LV contribute to early–mitral incompetency. Asymmetric papillary tip movement towards the midanterior annulus is a major determinant of mid‐ and late‐systolic functional mitral regurgitation.

Although renal failure is one of the known comorbidities associated with rapid progression of aortic stenosis, it is unclear whether hemodialysis alters the progression of prosthetic aortic valve stenosis. We describe a 79-year-old female who underwent bioprosthetic aortic valve replacement 8 years ago with stable prosthetic valve area for the initial 6 years. In the last two years, coinciding with the initiation of maintenance hemodialysis, she developed progressive prosthetic valve stenosis to the point of clinical decompensation. She underwent a second prosthetic aortic valve replacement with symptom resolution. This case suggests that circulating milieu in end-stage renal failure and dialysis can accelerate the progression of prosthetic aortic valve stenosis. More frequent clinical followup and surveillance echocardiogram for dialysis patients with bioprosthetic aortic valve may facilitate timely management of valvular stenosis.

Background— Mitral annulus is a complex structure of poorly understood physiology. Full-volume real-time 3-dimensional transesophageal echocardiography offers a unique opportunity to completely image and quantify mitral annulus size and motion. Methods and Results— Real-time 3-dimensional transesophageal echocardiography of the mitral valve was acquired in 32 patients with myxomatous valve disease (MVD) and moderate to severe regurgitation, 15 normal control subjects, and 10 patients with ischemic mitral regurgitation of identical body surface area. Mitral annular dimensions (circumference, area, anteroposterior and intercommissural diameters, height, and ratio of height to intercommissural diameter ratio, which appraises annular saddle-shape depth) were measured throughout the cardiac cycle with dedicated quantification software. Compared with direct surgical measurement, 3-dimensional anterior annular dimension provided reliable measurements (mean difference, 0.1±0.1 mm; P =0.73; 95% confidence interval, ±4.4 mm). Annular dimensions were larger in MVD patients compared with control subjects in diastole (all P <0.05). Normal annulus displayed early-systolic anteroposterior ( P <0.001) and area ( P =0.04) contraction, increased height ( P <0.001), and deeper saddle shape (ratio of height to intercommissural diameter, 15±1% to 21±1%; P <0.001), whereas intercommissural diameter was unchanged ( P =0.30). In contrast, MVD showed early-systolic intercommissural dilatation ( P =0.02) and no area contraction ( P =0.99), height increase ( P =0.11), or saddle-shape deepening ( P =0.35). Late-systolic MVD annular saddle shape deepened but annular area excessively enlarged ( P <0.04) as a result of persistent intercommissural widening ( P <0.02). MVD annulus also contrasts with ischemic mitral regurgitation annulus, which, despite similar anteroposterior enlargement, is narrower and essentially adynamic. After MVD repair, the annulus remained dynamic without systolic saddle-shape accentuation ( P =0.30). Conclusions— Real-time 3-dimensional transesophageal echocardiography provides insights into normal, dynamic mitral annulus function with early-systolic area contraction and saddle-shape deepening contributing to mitral competency. MVD annulus is also dynamic but considerably different with loss of early-systolic area contraction and saddle-shape deepening despite similar magnitude of ventricular contraction, suggestive of ventricular-annular decoupling. Subsequent area enlargement may contribute to mitral incompetence. After mitral repair, MVD annulus remains dynamic without systolic saddle-shape accentuation. Thus, real-time 3-dimensional transesophageal echocardiography provides new insights that allow the refining of mitral pathophysiology concepts and repair strategies.