William A. Zoghbi, MD, FASE (Chair), David Adams, RCS, RDCS, FASE, Robert O. Bonow, MD, Maurice Enriquez-Sarano, MD, Elyse Foster, MD, FASE, Paul A. Grayburn, MD, FASE, Rebecca T. Hahn, MD, FASE, Yuchi Han, MD, MMSc,* Judy Hung, MD, FASE, Roberto M. Lang, MD, FASE, Stephen H. Little, MD, FASE, Dipan J. Shah, MD, MMSc,* Stanton Shernan, MD, FASE, Paaladinesh Thavendiranathan, MD, MSc, FASE,* James D. Thomas, MD, FASE, and Neil J. Weissman, MD, FASE, Houston and Dallas, Texas; Durham, North Carolina; Chicago, Illinois; Rochester, Minnesota; San Francisco, California; New York, New York; Philadelphia, Pennsylvania; Boston, Massachusetts; Toronto, Ontario, Canada; and Washington, DC

Recommendations for Noninvasive Evaluation of Native Valvular Regurgitation: A Report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance

Background Sudden death from cardiac arrest in a young person may occur during sports play after a blunt blow to the chest in the absence of structural cardiovascular disease or traumatic injury (cardiac concussion or commotio cordis). We studied the clinical features of this apparently uncommon but important phenomenon. Methods We identified cases from the registries of relevant agencies and organizations, as well as news-media accounts, and developed a clinical profile of 25 children and young adults, 3 to 19 years of age. Results Each victim collapsed with cardiac arrest immediately after an unexpected blow to the chest, which was usually inflicted by a projectile (such as a baseball or hockey puck). Incidents took place during organized competitive sports in 16 cases and in recreational settings at home, at school, or on the playground in 9. In each instance, the impact to the chest was not judged to be extraordinary for the sport involved and did not appear to have sufficient force to cause death. Tw...

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Blunt impact to the chest leading to sudden death from cardiac arrest during sports activities.

The gross surgical pathologic features of the mitral valve were reviewed in 712 patients who had undergone mitral valve replacement at our institution during 1965, 1970, 1975, 1980, and 1985. Among the 452 cases of mitral stenosis, either with or without mitral insufficiency, 99% were attributable to postinflammatory disease and 1% were related to congenital mitral stenosis. Among the 260 cases of pure mitral regurgitation, the two most common causes were a floppy valve (38%) and postinflammatory disease (31%). Moreover, a floppy valve was observed in 73% of the 59 examples of chordal rupture and in 38% of the 16 cases of infective endocarditis. Women accounted for 73% of the 452 cases of mitral stenosis and for 72% of the 530 cases of postinflammatory disease. In contrast, men accounted for 58% of the 260 cases of pure mitral regurgitation, including 76% of the floppy valves and 69% of the infected valves. During the 21 years spanned by the study, the relative frequency of postinflammatory mitral insufficiency progressively decreased, whereas that of floppy mitral valves increased. It is unclear whether aging, heredity, environmental factors, changes in the frequency of acute rheumatic fever, or changes in patient referral practices may account for this observation.

https://www.mayoclinicproceedings.org/article/S0025-6196(12)61522-5/pdf

Surgical pathology of the mitral valve: a study of 712 cases spanning 21 years

To determine whether the left ventricular end systolic pressure-dimension and end-systolic stress-dimension relations in human beings are linear and sensitive to altered contractility, we studied 13 normal subjects during methoxamine infusion and with postextrasystolic potentiation induced by an external mechanical cardiac stimulator. End-systolic diameter was obtained with echocardiography and end-systolic pressure was estimated in six subjects from the dicrotic notch of a simultaneously recorded carotid pulse tracing, standardized by cuff pressure, whereas in seven subjects intraarterial pressure was recorded. For each subject, the end-systolic pressure-dimension relation was linear ( r = 0.83–0.99) over a range of 76 mm Hg (84 to 160) for end-systolic pressure. The mean slope of the end-systolic pressure-dimension line was 62 ± 22 mm Hg/cm. Peak systolic pressure was also linearly related to end-systolic diameter ( r = 0.82–0.99) over a range of 100 mm Hg (104 to 204). End-systolic stress was a linear function of end-systolic dimension as well ( r = 0.93–0.99) over an end-systolic stress range of 181 g/cm 2 . With postextrasystolic potentiation the potentiated beat had a smaller end-systolic dimension for any given end-systolic pressure and thus shifted the end-systolic pressure-dimension relation to the left. Thus, end-systolic pressure-dimension and stress-dimension relations in human subjects appear to be linear and are sensitive to the inotropic state.

Left ventricular end-systolic pressure-dimension and stress-length relations in normal human subjects.

More than half of all patients evaluated in our clinic for the possible diagnosis of a heritable disorder of connective tissue could not be classified in the current nosology, yet they had considerable clinical evidence of a systemic defect of the extracellular matrix. As a group, these patients share many manifestations of the Marfan syndrome including long limbs, deformity of the thoracic cage, striae atrophicae, mitral valve prolapse, and mild dilatation of the aortic root. Clinical clustering did not emerge when patients were stratified by mitral valve prolapse or aortic dilatation. The clinical phenotype of patients with mitral valve prolapse constitutes a continuum, from Marfan syndrome at one extreme to isolated mitral valve prolapse due to myxomatous proliferation of the valve leaflets. In the absence of biochemical or DNA markers, discerning whether a patient with mitral valve prolapse and mild aortic root dilatation (in the absence of ectopia lentis or a family history) has Marfan syndrome, or another heritable disorder of connective tissue, will continue to be a clinical challenge. Until subclassification based on refined clinical, genetic, and laboratory investigations is possible, the patients we describe are best seen as having an "overlap" heritable connective-tissue disorder. We suggest the acronym "MASS phenotype" to emphasize involvement of the mitral valve, aorta, skeleton, and skin. (JAMA. 1989;262:523-528)

https://jamanetwork.com/HttpHandlers/ArticlePdfHandler.ashx?journal=JAMA&articleId=378111&pdfFileName=jama_262_4_032.pdf

Association of mitral valve prolapse and systemic abnormalities of connective tissue. A phenotypic continuum.

https://www.amjmed.com/article/0002-9343(79)91122-7/pdf

Multiple floppy valves: an echocardiographic syndrome.

Left ventricular function was evaluated in 34 patients with the echocardiogram, and an external mechanical cardiac stimulator was used to induce a ventricular premature contraction (VPC) noninvasively. Extent of post-extrasystolic potentiation (PESP) was determined by comparing systolic dimensional shortening and ejection fraction of the sinus beat preceding the VPC to that of the potentiated beat which followed it. Using this technique, a VPC could be introduced into the cardiac cycle of 30 of the 34 patients, six of whom were free of obvious cardiac disease and 24 of whom had valvular, coronary or myopathic heart disease. The only complication observed was mild breast ecchymosis in a female patient. Systolic dimensional shortening and ejection fraction increased from control values by an average of 21% and 17% respectively, with a range of 0-100%. The degree of PESP was very reproducible in repeat studies and when the same patients were subsequently evaluated during a spontaneously occurring or catheter-induced VPC. The technique can safely and reliably induce post-extrasystolic potentiation during echocardiography and is a potentially important adjunct to the noninvasive evaluation of left ventricular function.

A new, noninvasive technique for inducing post-extrasystolic potentiation during echocardiography.

A case of mitral regurgitation simulating aortic stenosis is discussed. Echocardiographic and angiographie evidence is provided demonstrating the mechanism whereby the murmur of mitral regurgitation mimics aortic stenosis. This case offers direct confirmation of the hypothesis that acoustic energy derived from a mitral regurgitant jet may be transmitted to the aorta by impingement of the jet upon the portion of the left atrial wall adjacent to the aortic root.

Demonstration of the mechanism by which mitral regurgitation mimics aortic stenosis

Mitral valve prolapse in adults with congenital heart disease

Fifteen patients with idiopathic hypertrophic subaortic stenosis had a ventricular extrasystole induced with a new external mechanical cardiac stimulator during noninvasive evaluation of left ventricular outflow tract obstruction. Ten patients were monitored with simultaneous echocardiogram, phonocardiogram and indirect carotid pulse tracing; five were monitored with the phonocardiogram and indirect carotid pulse tracing alone. Nine of the 15 patients showed obstruction in the beat after the ventricular extrasystole, as defined by appearance of the characteristic bifid carotid pulse contour and, where recorded, an increase in systolic anterior motion of the mitral valve on echocardiography. Six patients did not show obstruction. All nine patients with obstruction had greater than 20 msec prolongation of uncorrected systolic ejection time in the post-extrasystolic beat of the carotid pulse tracing. Change in the uncorrected ejection time was + 0.038 +/- 0.15 second (mean +/- standard deviation) in these nine patients compared with -0.003 +/- 0.005 second in the six not showing obstruction (P less than 0.01). Six patients underwent cardiac catheterization: Three patients without obstruction after a noninvasively induced ventricular extrasystole had no obstruction at catheterization and three patients with obstruction after noninvasively induced ventricular premature beats demonstrated obstruction at rest or after provocative maneuvers during catheterization. These results indicate that the noninvasive induction of a ventricular extrasystole is a useful and easily performed procedure for both diagnosing and evaluating the dynamic left ventricular outflow tract obstruction of idiopathic hypertrophic subaortic stenosis.

Value of a noninvasively induced ventricular extrasystole during echocardiographic and phonocardiographic assessment of patients with idiopathic hypertrophic subaortic stenosis

Gerald H. Angoff

Papers

Multiple floppy valves: an echocardiographic syndrome.

A new, noninvasive technique for inducing post-extrasystolic potentiation during echocardiography.

Demonstration of the mechanism by which mitral regurgitation mimics aortic stenosis

Mitral valve prolapse in adults with congenital heart disease

Value of a noninvasively induced ventricular extrasystole during echocardiographic and phonocardiographic assessment of patients with idiopathic hypertrophic subaortic stenosis