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Systolic Ejection Murmur Presenting with Dyspnea on Exertion

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Systolic Ejection Murmur Presenting with Dyspnea on Exertion CARDIOVASCULAR BOARD REVIEW C. RIMMERMAN, B.P. GRIFFIN, EDITORS A SELF-TEST ON A TOBIAS PEIKERT, MD CRAIG R. ASHER, MD BRIAN P. GRIFFIN CLINICAL Department of Internal Medicine, Department of Cardiovascular Department of Cardiovascular Cleveland Clinic Medicine, Cleveland Clinic Medicine, Cleveland Clinic CASE Systolic ejection murmur presenting with dyspnea on exertion 43-YEAR-OLD WOMAN was referred to ■ DIFFERENTIAL DIAGNOSIS A the cardiology outpatient clinic for OF SYSTOLIC EJECTION MURMUR evaluation of a systolic ejection murmur. Her symptoms at presentation consisted of dysp- Which of the following conditions should nea on exertion, decreased exercise tolerance, 1 not be included in the differential diagno- generalized fatigue, and intermittent palpita- sis of a systolic ejection murmur? tions. Her functional impairment was consis- tent with New York Heart Association ❑ Aortic valve stenosis (NYHA) class 2 to class 3. She had a known ❑ Mitral valve regurgitation cardiac murmur since childhood. In the past ❑ Aortic valve sclerosis she experienced presyncopal episodes and, on ❑ Hypertrophic cardiomyopathy one occasion, syncope. ❑ Subaortic membrane She admits to the social use of cigarettes and alcohol. Her father had coronary artery Mitral valve regurgitation is the only one of disease and diabetes mellitus, and an uncle the above conditions that does not present had died suddenly of an unknown cause. with a systolic ejection murmur. Keep an open Systolic murmurs are characterized as mind when Physical examination “ejection” (TABLE 1) or “regurgitant”. Systolic Her vital signs were within normal limits. She ejection murmurs are audible only during part evaluating had no carotid bruits or jugular venous disten- of systole, that is, they begin after S1 and end patients with tion. The carotid upstroke was slightly before S2. However, regurgitant murmurs, delayed, with normal volume and without such those caused by mitral valve prolapse, are preexisting pulsus bisferiens (a midsystolic dip). The holosystolic, ie, they are audible throughout diagnoses point of maximum impulse was nondisplaced all of systole: they generally start with S1 and and sustained. Cardiac auscultation revealed a end with S2. normal S1 and S2 (P2) and no S3 or S4. A 3/6 The proper evaluation of any systolic systolic ejection murmur with early onset and murmur requires consideration of such fac- harsh crescendo was noted throughout the tors as the location, intensity, timing, con- precordium, with no radiation to the axilla or figuration, character, radiation, change of the neck. The murmur did not change with characteristics with certain maneuvers, and standing, hand-gripping, and the Valsalva associated findings and symptoms (TABLE 2). maneuver. Her pulses were symmetric, and To differentiate aortic valve stenosis from she had no peripheral edema. aortic valve sclerosis it is important to know that patients with aortic valve sclerosis usu- Initial studies ally have no symptoms and that there is no Chest radiography revealed slight car- radiation of the murmur, no change in pulse diomegaly. Baseline electrocardiography character, and no delay or decreased inten- showed sinus rhythm and left ventricular sity of the aortic component of S2. The hypertrophy with repolarization abnormali- murmur is usually brief and soft. ties. To distinguish a fixed stenosis (as in aor- CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 68 • NUMBER 9 SEPTEMBER 2001 809 Downloaded from www.ccjm.org on September 25, 2021. For personal use only. All other uses require permission. CARDIOVASCULAR BOARD REVIEW PEIKERT AND COLLEAGUES T ABLE 1 CASE CONTINUED Prior evaluation and treatment Differential diagnosis at other institutions of systolic ejection murmurs The patient had been evaluated in different Aortic systolic ejection murmurs hospitals before presenting to our institution. Left ventricular outflow tract (LVOT) obstruction Echocardiography and cardiac catheterization Valvular aortic stenosis had detected a pressure gradient of 100 mm Rheumatic fever Hg within the LVOT (the normal pressure Degenerative (tricuspid valve) gradient is zero). The aortic valve was deemed Degenerative (bicuspid valve) morphologically normal with regular excur- Subvalvular aortic stenosis sion, and no evidence of coronary artery dis- Hypertrophic obstructive cardiomyopathy ease had been seen. Fixed (discrete) subvalvular stenosis The patient had been given a diagnosis Supravalvular aortic stenosis of hypertrophic cardiomyopathy, but treat- Aortic dilation Hypertension ment with beta-blockers, calcium channel Aneurysms blockers, and disopyramide and the implan- Coarctation of the aorta tation of a DDDR (dual-chamber, adaptive- Aortic valve sclerosis rate) pacemaker had failed. Holter monitor- Increased aortic flow ing prompted by the syncopal episode had Aortic regurgitation shown frequent premature ventricular con- Anemia tractions and runs of nonsustained ventricu- Thyrotoxicosis lar tachycardia. Fever Pregnancy ■ LEFT VENTRICULAR Exercise OUTFLOW TRACT OBSTRUCTION Bradycardia Pulmonic systolic ejection murmurs On the basis of the patient’s clinical presenta- Right ventricular outflow tract (RVOT) obstruction tion and the prior echocardiographic and arte- Pulmonic valvular stenosis riographic findings, especially the presence of Infundibular stenosis a pressure gradient between the left ventricu- Supravalvular pulmonic stenosis lar cavity and the LVOT, a diagnosis of LVOT Pulmonary artery dilation Idiopathic obstruction can be made. Pulmonary hypertension What would be the most likely cause of 2 LVOT obstruction in this patient? tic valve stenosis) from a dynamic left ven- ❑ Hypertrophic cardiomyopathy tricular outflow tract (LVOT) obstruction (as ❑ Valvular aortic stenosis in hypertrophic cardiomyopathy), it is ❑ Supravalvular (ie, supra-aortic valve) important to evaluate changes of the murmur stenosis during certain functional maneuvers that ❑ Fixed subvalvular stenosis produce changes in cardiac preload, after- ❑ Aortic coarctation load, and contractility (TABLE 2).1 For exam- ple, standing and the Valsalva maneuver For reasons discussed below, a subvalvular decrease the intensity of the murmur in cause is the most likely in this patient, based patients with aortic valve stenosis, whereas on the history, symptoms, and physical find- they accentuate the murmur in patients with ings. hypertrophic cardiomyopathy. Patients with The differential diagnosis of LVOT a fixed subvalvular stenosis have a preserved obstruction can be divided into three major S2 and carotid upstroke, and the murmur may categories on the basis of the location of the decrease in intensity with the Valsalva obstructing lesion (TABLE 2): supravalvular, maneuver and standing. valvular, and subvalvular. 810 CLEVELAND CLINIC JOURNAL OF MEDICINE VOLUME 68 • NUMBER 9 SEPTEMBER 2001 Downloaded from www.ccjm.org on September 25, 2021. For personal use only. All other uses require permission. T ABLE 2 Features of the physical examination that help to differentiate the causes of left ventricular outflow tract obstruction FEATURE VALVULAR SUPRAVALVULAR SUBVALVULAR DISCRETE HYPERTROPHIC SUBVALVULAR STENOSIS CARDIOMYOPATHY Pulse pressure after Increased Increased Increased Decreased ventricular premature beat Effect of Valsalva maneuver Decreases Decreases Decreases Increases on systolic murmur Murmur of aortic regurgitation Common Rare Sometimes Rare Fourth heart sound If severe Uncommon Uncommon Common Paradoxic splitting Sometimes Absent Absent Common Ejection click Most, except in Absent Absent Absent cases of calcified valve Maximal thrill and murmur Second right First right Second right Fourth left intercostal space intercostal space intercostal space intercostal space Carotid pulse Reduced Unequal Normal to reduced Brisk, jerky upstroke upstroke upstroke, systolic rebound ADAPTED FROM MARRIOTT HJL. BEDSIDE CARDIAC DIAGNOSIS. PHILADELPHIA: J.B. LIPPINCOTT, 1993:116. Supravalvular causes of LVOT obstruction require surgical intervention early in child- Supravalvular causes of LVOT obstruction are hood, which makes them an unlikely diagno- aortic coarctation, fixed supravalvular stenosis, sis in our patient.2,3 Occasionally, severe fibrous membranes, and fibromuscular ridges. familial hyperlipidemia leads to fatty deposi- Aortic coarctation and fixed supravalvu- tion above the aortic valve and to stenosis at lar stenosis are congenital conditions that usu- that point. ally become symptomatic and are diagnosed earlier in life. Aortic coarctation is caused by Valvular causes of LVOT obstruction a fibromuscular ridge in the location of the The most frequent valvular abnormality former ductus arteriosus distal to the origin of resulting in aortic stenosis is degenerative dis- the left subclavian artery. Symptoms at pre- ease. Depending on the underlying anatomy, sentation usually include hypertension aortic stenosis becomes hemodynamically sig- involving the upper extremities and delayed nificant in different age groups. The most and decreased pulses in the lower extremities. common cause of aortic stenosis in people Aortic coarctation is a cause of secondary under age 55 is a congenitally abnormal aortic hypertension. In this particular case, aortic valve. Often, the valve is still pliable at the coarctation is very unlikely since hyperten- time of presentation, resulting in an ejection sion is absent and the peripheral pulses are click preceding the systolic ejection murmur. normal on physical examination. The most common congenital
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