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Transcript
HISTORY 30-year-old woman. CHIEF COMPLAINT: Palpitations of three years duration. PRESENT ILLNESS: The patient has had brief, fleeting “flip-flop”sensations in her chest. At other times she occasionally has sharp chest pains at rest lasting 1 to 3 seconds. There is no past history of rheumatic fever, chest trauma or heart murmur. FAMILY HISTORY: Her 25-year-old sister has a murmur. Question: Is a specific diagnosis suggested by this history? 9-1 Answer: No. Both her palpitations, which suggest premature contractions, and her chest pain, are nonspecific and may be entirely innocent. PHYSICAL SIGNS a. GENERAL APPEARANCE – Normal slender young woman with mild scoliosis. b. VENOUS PULSE - The CVP is estimated to be 3 cm H2O. UPPER RIGHT STERNAL EDGE JUGULAR VENOUS PULSE Question: How do you interpret the venous pulse? 9-2 Answer: The venous pulse is normal. c. ARTERIAL PULSE - (BP = 120/70 mm Hg) S1 S2 UPPER RIGHT STERNAL EDGE CAROTID ECG Question: How do you interpret the carotid arterial pulse? 9-3 Answer: The arterial pulse is normal. d. PRECORDIAL MOVEMENT PHONO UPPER RIGHT STERNAL EDGE S1 S2 APEXCARDIOGRAM Question: How do you interpret the apical pulsation? 9-4 Answer: The apical impulse is normal. e. CARDIAC AUSCULTATION 4L APEX S1 S2 Questions: 1. How do you interpret the acoustic events at the left sternal edge and apex? CAROTID ECG 2. What additional bedside maneuvers will help to assess this murmur? 9-5 Answers: 1. There are multiple systolic clicks and a late systolic murmur. These findings indicate mitral valve prolapse. The clicks are likely due to acute tensing of the valve as it everts into the left atrium, or less likely may emanate from the chordae tendineae. 2. Examine the patient standing and squatting as shown below. 2L APEX STANDING Question: SQUATTING What is your explanation for these postural changes? 9-6 Answer: Standing decreases venous return, reducing ventricular size, resulting in earlier and more marked prolapse of the redundant mitral leaflet, and hence earlier and more often louder clicks and murmur. Squatting, by increasing peripheral resistance and hence afterload, increases ventricular size, and the murmur is later and often fainter. Through similar mechanisms amyl nitrite makes the ventricle smaller (like standing), allows more mitral valve prolapse, and the murmur begins earlier. Vasopressors or isometric handgrip (like squatting) increase ventricular size, allow less prolapse, and make the murmur begin later in systole. These changes are in contrast to patients with rheumatic mitral regurgitation. For example, in such patients, a reduction in afterload enhances forward flow, reducing the degree of regurgitation and the murmur. Proceed 9-7 e. CARDIAC AUSCULTATION (continued) ECG 2 1 1 1 2L A2 P2 EXPIRATION Question: 0.6 sec A2 P2 INSPIRATION How do you interpret the acoustic events at the upper left sternal edge? 9-8 Answer: There is normal splitting of the second heart sound in inspiration. f. PULMONARY AUSCULTATION Question: How do you interpret the acoustic events in the pulmonary lung fields? Proceed 9-9 Answer: In all lung fields, there are normal vesicular breath sounds. ELECTROCARDOGRAM I II III aVR aVL aVF V1 V2 V3 V4 V5 V6 NORMAL STANDARD RHYTHM STRIP Question: How do you interpret this ECG? 9-10 Answer: There are nonspecific ST-T wave changes and an isolated ventricular premature contraction (VPC) in the rhythm strip. Such ST-T changes are common in patients with mitral valve prolapse, especially in the inferolateral leads. In many patients, especially those with the isolated clicks and no murmur, the ECG is normal. Premature atrial and ventricular contractions are also common and are the usual cause of “palpitations.” Atrial tachycardia and atrial fibrillation are sometimes present. Exercise stress testing results in a high percentage of “false positives.” Proceed 9-11 CHEST X RAYS Questions: 1. How do you interpret these chest X rays? 2. Based on the history, physical examination, ECG and X rays, what is your diagnostic impression and plan to further evaluate this patient? 9-12 Answers: 1. The heart and lungs are normal. There is a mild scoliosis of the thoracic spine. On the lateral view, the spine is quite “straight,” rather than mildly kyphotic as it usually is in normal adults. Thoracic skeletal abnormalities are quite frequent in this syndrome of mitral valve prolapse, particularly “straight back,” pectus excavatum, and mild thoracic scoliosis. 2. The history, physical examination, ECG and chest X rays are essentially diagnostic of the systolic click - systolic murmur (Barlow’s) syndrome. Echocardiography is a non-invasive procedure likely to show the mitral prolapse and confirm the clinical diagnosis. The patient’s study follows. 9-13 LABORATORY - ECHOCARDIOGRAM TWO-DIMENSIONAL ECHOCARDIOGRAM (SYSTOLE) RV AoV LV LA AL PL LV RV RA AL PL LA RV = Right Ventricle LV = Left Ventricle LA = Left Atrium RA = Right Atrium AL = Anterior Mitral Leaflet PL = Posterior Mitral Leaflet AoV = Aortic Valve PARASTERNAL LONG AXIS APICAL FOUR CHAMBER Question: What are the diagnostic features of this echocardiogram? 9-14 Answer: There is a marked systolic displacement of the posterior leaflet into the left atrium that is diagnostic of mitral valve prolapse. The size of the chambers and function of the left ventricle are normal. This, together with absence of leaflet thickening, implies a good prognosis. Proceed 9-15 The Doppler ultrasound examination can help confirm the presence and determine the severity of mitral regurgitation. The patient’s color Doppler flow map below shows mild MR. PARASTERNAL LONG AXIS VIEW (Systole) RV RV = right ventricle LV = left ventricle AoV = aortic valve AoV LV MV = mitral valve LA = left atrium MV LA Question: Blue (large arrow) represents the mild regurgitant jet. Red represents LV outflow. Is cardiac catheterization necessary? 9-16 Answer: No. The precise diagnosis has been established by non-invasive techniques, the most valuable of which is auscultation. However, if cardiac catheterization were performed, the result would be similar to the following case. LABORATORY (continued) LEFT VENTRICULAR ANGIOGRAM - Right Anterior Oblique (Systolic Frame) Left Atrium (LA) Ao LA = 5mm Hg MEAN Left Ventricle (LV) = 120/4mm Hg Aorta (AO) = 120/70 mm Hg Cardiac Index = 3.5L/Min/M2 LV POST ANT Question: How do you interpret the hemodynamics and angiographic data? 9-17 Answer: Intracardiac pressures and cardiac output are all normal, as is usually the case, since the mitral regurgitation is minimal. The angiogram shows scalloping (arrows) of the prolapsing posterior leaflet as well as some prolapse of the anterior leaflet (broken arrow) as they balloon into the left atrium in late systole. Question: The patient has received some conflicting advice about her cardiac status and about antibiotics. Some clinicians have told her to take penicillin daily, while others have said to take it only one hour prior to dental work. One healthcare provider advised both. What is your advice? 9-18 Answer: The patient should be reassured, as the natural history of the great majority of patients with mitral prolapse is benign. She requires prophylaxis for infective endocarditis, not rheumatic fever. Therefore, she should take antibiotics whenever she is exposed to bacteremia, e.g., dental procedures likely to cause gingival bleeding, cystoscopies, etc. Rheumatic fever prophylaxis (Penicillin) should be reserved for patients who previously had rheumatic fever. It is inadequate for the prevention of infectious endocarditis. The patient was advised that her younger sister should be checked, as she also has a murmur, and this syndrome may occasionally be familial. Her sister’s evaluation follows. 9-19 The patient’s sister’s evaluation in the supine position, was entirely normal. Auscultation in the standing position revealed a click and systolic murmur, as shown below. The examination must include listening in multiple positions (and occasionally even at different times), as these vasoactive maneuvers may vary ventricular size and hence vary the murmur. 2L APEX CAROTID SUPINE STANDING This patient was also instructed to undergo infective endocarditis prophylaxis for any surgical or dental procedures likely to induce bacteremia. Proceed for Summary 9-20 SUMMARY Mitral valve prolapse is an extremely common disorder and is sometimes familial. Young women are most commonly affected. It is usually a very mild disorder, and in some cases may also involve the tricuspid valve. In most instances its cause is unknown. However, irregular elongation of the posterior or both leaflets and myxomatous changes microscopically have been found in the valve in many of the cases studied at autopsy or surgery. These changes are similar to those seen in Marfan’s and the “floppy valve” syndrome which, however, are more severe and progressive disorders. The gross pathology of a patient who developed infective endocarditis follows. 9-21 While the natural history of the systolic click-murmur syndrome is generally benign, this specimen is from a patient who died of embolic complications of infective endocarditis. SCALLOPING (BALLOONING) OF MITRAL LEAFLET JET LESION LEFT ATRIUM FROM REGURGITAITON BACTERIAL VEGETATIONS Proceed 9-22 SUMMARY(continued) The mid to late systolic click(s) with or without a late systolic murmur is the acoustic hallmark of the prolapsed valve. The click presumably originates from sudden tensing of the valvular structures and/or deceleration of blood flow as the mitral leaflets reach there maximally prolapsed position. An isolated late systolic murmur may also be seen with prolapse, but in the absence of a click it is not diagnostic, as such murmurs may also be seen with papillary muscle dysfunction and flail chordae tendineae in the absence of prolapse. 9-23 To Review This Case of Mitral Valve Prolapse: The HISTORY is typical, including palpitations reflecting VPC’s and atypical chest pain of the musculoskeletal type. Occasionally there is chest pain which sounds “ischemic” in nature. The exact cause of this symptom is unknown. The syndrome may occasionally be familial, as in this case. PHYSICAL SIGNS: a. The GENERAL APPEARANCE reveals her slender build with slight scoliosis and a “straight back,” common in this syndrome. b. The JUGULAR VENOUS PULSE is normal in mean pressure and wave form. c. The ARTERIAL PULSE is normal. Proceed 9-24 d. PRECORDIAL MOVEMENT is normal. e. CARDIAC AUSCULTATION reveals normal splitting of the second sound at the upper left sternal edge. Midsystolic clicks and a late systolic murmur are best heard at the apex and are less intense at the left sternal edge. The latter may reflect some degree of tricuspid valve prolapse. The acoustic events are notoriously variable, and maneuvers which decrease ventricular size augment the findings, with the clicks and murmur appearing earlier in systole. No examination for mitral valve prolapse is complete unless the patient is examined in the standing position. On occasion the murmur associated with prolapse of the mitral valve has a quality which has been described as a “honk.” f. PULMONARY AUSCULTATION reveals normal vesicular breath sounds in all lung fields. 9-25 The ELECTROCARDIOGRAM shows nonspecific ST-T changes and occasional VPCs. Serious arrhythmias occur rarely. The CHEST X RAY shows no abnormality of the heart or lungs, reflecting the fact that the lesion is hemodynamically insignificant. Associated skeletal abnormalities are seen, including slight scoliosis and a “straight back.” LABORATORY STUDIES include the echocardiogram which shows late systolic posterior movement of the posterior leaflet. While invasive study is unnecessary, a typical angiogram shows the late systolic posterior leaflet prolapse and normal intracardiac pressures and cardiac output. 9-26 TREATMENT is mainly reassurance and infective endocarditis prophylaxis. Arrhythmias are usually untreated because they are brief, infrequent and of little consequence. Considering the common occurrence of this disorder, the rare instances of serious ventricular arryhthmias that have been reported should not set the standard for therapy. Arrhythmias that are frequent, prolonged and symptomatic should be evaluated and treated. Patients with atypical chest pain should also be reassured. Beta-blockers may help alleviate this symptom. 9-27 To Review a patient with Mitral Valve Prolapse with an isolated click and murmur, change to disease #10 on the keypad. You will note the following findings: a. The JUGULAR VENOUS PULSE mean venous pressure and wave form are normal. b. The CAROTID PULSE is normal in upstroke, peak, and downstroke. c. PRECORDIAL MOVEMENT reveals a normal brief apical impulse in the fifth intercostal space at the midclavicular line, occurring at the time of the first heart sound. d. CARDIAC AUSCULTATION at the apex reveals the high frequency, grade 2, late systolic crescendo murmur. The murmur is due to mitral regurgitation associated with late systolic prolapse of the redundant mitral leaflets. At the tricuspid area, there is an isolated high frequency mid systolic click. This may reflect some degree of tricuspid valve prolapse or be transmitted from the mitral area. e. PULMONARY AUSCULTATION reveals normal vesicular breath sounds in all lung fields. 9-28