Status of cardiac transplantation with a report of the first year's experience at Henry Ford Hospital.
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Biomedical subjects
Publications and source records attributed to J B Lakier.
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We recently observed the unusual origin of the posterior descending from the left anterior descending coronary artery in three patients undergoing coronary angiography. These three cases are presented and the clinical implications of this variation are discussed.
To establish the etiology of isolated aortic valvular regurgitation, histologic examination was carried out on 27 consecutive surgically removed aortic valves from patients with aortic regurgitation. In 12 patients, the regurgitation was due to rheumatic or syphilitic valvular disease or a congenital bicuspid aortic valve. In the remaining 15, no etiology was apparent. In the latter group, seven aortic valves were identified by the surgeon as redundant and eight as thickened and retracted. Despite these gross differences, the histologic features of the 15 valves were similar and consisted of increased and disorganized elastic and collagen fibers, with variable quantities of acid mucopolysaccharide and calcium. Although small foci of myxomatous stroma were present, they did not differ substantially from those observed in age-matched competent aortic valves removed at necropsy, nor were they as extensive as described in reports of floppy aortic valves. Idiopathic degeneration was the most common cause of aortic regurgitation, occurring in more than half of the surgically treated patients. An underlying defect in the synthesis of collagen or elastic fibers, similar to that described in mitral valve prolapse, may be an important feature in aortic valve degeneration.
To determine the usefulness of the frequency of heart sounds in the assessment of porcine bioprosthetic valve degeneration, frequency spectra of phonocardiograms of the first heart sound and the aortic component of the second sound were analyzed in 31 patients with degenerated porcine bioprosthetic valves. Comparisons were made with 35 control patients whose valves were inserted 1 month or less. Among 23 patients with degenerated porcine bioprosthetic valves in the mitral position, the dominant frequency of the first heart sound was 95 +/- 11 Hz, which exceeded the first sound in 18 controls (51 +/- 3 Hz) (p less than 0.01). The degenerated mitral porcine bioprosthetic valves of 14 patients showed calcification or fibrosis and the first heart sound in these patients was 115 +/- 16 Hz, which exceeded that of control subjects (p less than 0.001). The degenerated mitral porcine bioprosthetic valves of 9 patients showed torn leaflets only, and the first heart sound in these patients was 64 +/- 9 Hz, which did not differ from that of control subjects. In the aortic position, 8 valves were degenerated and the aortic component of the second sound was 109 +/- 12 Hz, which was higher than that in 17 control subjects (63 +/- 4 Hz) (p less than 0.001). Only 2 of these degenerated valves showed tears unaccompanied by calcific deposits or fibrosis, and the frequencies were comparable to that of control subjects. These observations indicate that the frequency of heart sounds in patients with degenerated porcine bioprosthetic valves becomes abnormally elevated when degeneration is accompanied by calcification or fibrosis, which causes the cusps to stiffen.
Radionuclide angiographic studies were performed in 26 patients with a first myocardial infarction, 11 with anterior and 15 with an inferior location. Right ventricular (RV) and left ventricular (LV) ejection fractions (EF) were determined. Mean LVEF in anterior infarctions (33.2 +/- 7.3) was lower than in inferior myocardial infarctions (59.9 +/- 10.2) (p less than 0.001). Mean RVEF in the anterior infarct group was 41.3 +/- 15.1 and in the inferior myocardial infarction group 33.7 +/- 12.8 (p = NS). RVEF was less than 40% in 11 of 15 (73%) with inferior and 7 of 11 (66%) with anterior myocardial infarction. The ratio of LVEF:RVEF was 0.84 +/- 0.19 in the anterior and 2.13 +/- 1.28 in the inferior myocardial infarction group (p less than 0.001). LVEF correlated with RVEF in patients with anterior infarcts (r = 0.77; p less than 0.05) but not in those with inferior myocardial infarction (r = 0.14). RV regional wall motion abnormalities were observed in the inferolateral zones in 10 of 15 (66%) with inferior and in none of the 11 patients with anterior myocardial infarctions (p less than 0.001). Inferoseptal wall motion abnormalities were observed in 3 of 15 (20%) with inferior and 6 of 11 (56%) with anterior myocardial infarctions (p = NS). RV dysfunction in the inferior infarctions is probably an expression of primary RV dysfunction rather than secondary to LV dysfunction. RV dysfunction was proportional to LV dysfunction in the anterior infarction group and was often accompanied by RV septal contraction abnormalities.
Sequential changes in radionuclide angiographic measurement of left and right ventricular performance and quantitative thallium-201 scintigraphy were studied in 20 patients sustaining their first acute myocardial infarction (AMI). The studies were performed on the average 29.4 hours and 9.4 days after hospital admission. Anterior infarction had greater impairment of left ventricular (LV) performance in terms of ejection fraction and percentage of abnormal contraction area in addition to larger thallium-201 perfusion defects. At the time of the late study evidence of thallium-201 perfusion of infarcted area was seen in 14 of 20 patients, five of whom demonstrated improvement of regional wall motion. The remaining patients in the reperfused group and all of the patients in the nonperfused group failed to show evidence of LV functional improvement. This study indicates that reperfusion as measured by thallium-201 scintigraphy does occur spontaneously in 70% of patients with AMI and only in those patients with established reperfusion is there any potential for improvement in LV performance.
Acquired intracardiac shunt after prosthetic valve replacement is a rare complication, and reoperation for correction of this disorder carries a high mortality rate. We report such a case of left ventricular-right atrial fistula after mitral valve replacement in which the patient died of multiple complications. Awareness, prevention, and early diagnosis are, therefore, extremely important.
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To determine the clinical value of echocardiographic evaluation of porcine bioprosthetic valves, the findings in all patients who had porcine bioprosthetic valve replacement and adequate quality echocardiographic studies from 1978 to 1982 were analyzed. The study includes 309 normal and 59 dysfunctioning valves. Valve dysfunction resulted from spontaneous cusp degeneration in 39 (34 valve regurgitations, 5 stenoses), infective endocarditis in 12, paravalvular regurgitation in 5, regurgitation of redundant cusps, mitral valve thrombi, and aortic stent stenosis in 3 others. Echocardiographic findings were correlated with gross surgical pathologic or autopsy findings in 45 of the 59 dysfunctioning valves. Echocardiographic abnormalities were demonstrated in 41 of 59 (69%) dysfunctioning valves. A systolic mitral or diastolic aortic valve flutter was diagnostic of a regurgitant valve caused by a torn or unsupported cusp margin and was observed in 28 of 34 (82%) regurgitant valves with no false-positive studies. Echocardiographic cusp thickness of greater than or equal to 3 mm correctly identified all regurgitant and stenotic valves with gross anatomic evidence of localized or generalized cusp thickening or calcific deposits. Echocardiographic valve abnormalities were observed in only 4 of 12 patients with infective endocarditis and in 1 of 5 with paravalvular regurgitation. Thus, echocardiography provides important information regarding the function of porcine bioprosthetic valves and is of value in the decision to replace these valves, especially when dysfunction is due to spontaneous cuspal degeneration. Echocardiography is neither sensitive nor specific in patients with infective endocarditis and paravalvular regurgitation.
Although our observations are limited to studies performed on a degenerated bioprosthetic valve that produced a musical murmur, we believe that they can be applied to musical murmurs caused by abnormal natural valves. Several points regarding the characteristics of musical murmurs have been clarified. A musical murmur results from a uniform periodic vibration of a cardiac structure. A non-musical murmur results from turbulent blood flow which initiates random vibrations of adjacent structures. The broad spectrum of frequency of a non-musical murmur reflects the broad range of random fluctuations of blood velocity that characterizes turbulent blood flow. The frequency, amplitude, and time of occurrence during systole or diastole of a musical murmur are dependent upon the hemodynamics in the vicinity of the vibrating structure. Variability of all of these characteristics of the murmur, therefore, may be expected. Musical murmurs may have a purer tone at a site distal to the source than close to the source. This may reflect a superimposition of a broad spectrum of noise due to turbulence close to the valve. With distance from the valve, turbulence attenuates more than the sound-pressure fluctuations which are due to the uniform vibrations of the valve. A pure tone, uncontaminated by this broad spectrum of noise due to turbulence, therefore, is heard at some distance from the valve.
A comparison was made of the estimated size of the myocardial infarction occurring in 26 patients with a first infarction using creatine kinase (CK) enzyme release between radionuclide gated blood pool measurement of total and regional ventricular function and thallium-201 scintigraphic measurement of myocardial perfusion defects. Creatine kinase estimates of infarct size (enzymatic infarct size) correlated closely with the percent of abnormal contracting regions, left ventricular ejection fraction and thallium-201 estimates of percent of abnormal perfusion area (r = 0.78, 0.69 and 0.74, respectively, p less than 0.01). A close correlation also existed between percent abnormal perfusion area and percent of abnormal contracting regions (r = 0.81, p less than 0.01) and left ventricular ejection fraction (r = 0.69, p less than 0.01). Enzymatic infarct size was larger in anterior (116 +/- 37 CK-g-Eq) than inferior (52 +/- 29 CK-g-Eq) myocardial infarction (p less than 0.01) and was associated with significantly more left ventricular functional impairment as determined by left ventricular ejection fraction (33 +/- 7 versus 60 +/- 10%) (p less than 0.01) and percent abnormal perfusion area (58 +/- 14 versus 13 +/- 12) (p less than 0.01). No significant correlation was observed between enzymatic infarct size and right ventricular ejection fraction. These different methods of estimating infarct size correlated closely with each other in these patients with a first uncomplicated myocardial infarction.
We studied two sisters 29 and 31 years old who had skin and tendon xanthomas, corneal clouding, and severe coronary atherosclerosis. Histologic examination showed collections of lipid-laden histiocytes in the skin. The patients' plasma cholesterol concentrations were 177 and 135 mg per deciliter (4.58 and 3.49 mmol per liter). Levels of high-density-lipoprotein cholesterol were 4 and 7 mg per deciliter (0.1 and 0.2 mmol per liter). Only traces of apolipoprotein A-I were detected in whole plasma. The plasma density fraction from 1.06 to 1.21 g per milliliter contained no high-density lipoprotein on high-pressure liquid chromatography, no apolipoprotein A-I on sodium dodecyl sulfate electrophoresis, and only traces of apolipoprotein A-I on radioimmunoassay. Apolipoprotein C-III was also not detectable. The activity of lecithin-cholesterol acyltransferase was 40 per cent of normal. The half-life of infused normal high-density lipoprotein was three days (normal, 5.8 days). The parents and children of these two patients had low levels of high-density-lipoprotein cholesterol and apolipoprotein A-I. These cases support the hypothesis that low concentrations of high-density lipoprotein promote atherosclerosis.
The clinical, electrocardiographic and radiological features and cardiac catheterization findings in 3 young infants with large intracranial arteriovenous fistulas are presented. In addition to the congestive cardiac failure, the haemodynamic disturbances which give rise to central cyanosis, pulmonary hypertension and, in some instances, poor peripheral pulses, are discussed. The cyanosis in these children may have been the result of a combination of right-to-left shunting at atrial or ductal level, as well as intrapulmonary shunting in association with pulmonary engorgement. Left ventricular dysfunction, with elevated end-diastolic pressures, appeared to be most marked in those cases in which poor peripheral pulses were present. Increased pulmonary arterial pressures (considerably greater than systemic arterial pressure in 2 of the cases) are considered to be due to a combination of the obligatory left-to-right shunt which results from the fistula, and to the super-added effect of altitude (1765 metres) with resultant pulmonary vasoconstriction.
The purpose of this study was to assess the hemodynamic determinant of the amplitude and frequency of a musical murmur produced by a regurgitant degenerated bioprosthetic valve in the mitral position. The prosthetic valve, obtained at surgery, was studied in the mitral position of an in vitro pulse duplicating system. In vitro, the valve produced a musical murmur that was caused by flutter of a flail leaflet. When the peak pressure difference between the left ventricle and left atrium was increased from 95 to 150 mm Hg, the fundamental frequency of the musical murmur increased from 91 to 187 hertz and the amplitude increased from 2,080 to 11,420 dynes/cm2. The fundamental frequency of the musical murmur was linearly related to the peak systolic pressure difference between the left ventricle and the left atrium (correlation coefficient [r] = 0.99). Similarly, the fundamental frequency of the musical murmur was linearly related to the magnitude of regurgitant flow across the valve (r = 0.99); and the regurgitant flow as expected was linearly related to the systolic pressure difference between the left ventricle and left atrium. The amplitude of the musical murmur was also related to both the peak systolic pressure difference between the left ventricle and left atrium (r = 0.99) and the magnitude of regurgitant flow (r = 0.99). These results indicate that the magnitude of regurgitant flow, determined by the systolic pressure difference between the left ventricle and left atrium, was a determinant of both the amplitude and the frequency of the musical murmur.
The cause of a musical (cooing) murmur produced by a degenerated bioprosthetic valve in the mitral position was investigated. Spectral analysis of the murmur recorded at the chest wall at the site of the maximum palpable impulse showed virtually all sound in a narrow frequency band around the dominant frequency of 158 hertz. The same valve, surgically removed and mounted in the mitral position in a pulse duplicating system, produced an audible musical murmur detected by a phonocatheter in the atrial chamber. Nearly all of the sound-pressure occurred in a narrow band of frequency around 145 hertz. High speed motion pictures (500 frames/s) showed systolic flutter of a flail leaflet. The frequency of this leaflet flutter was 142 hertz. Hot film anemometry showed minimal turbulence, all located near the margin of the regurgitant leaflet. The intensity of the murmur was unrelated to the intensity of turbulence. A second degenerated bioprosthetic valve that produced in vivo a typical blowing holosystolic mitral regurgitant murmur produced in vitro a murmur with a broad range of frequencies (20 to 500 hertz). With this valve, the intensity of the murmur was related to the intensity of the turbulence. Motion pictures showed no leaflet flutter. Flutter of an insufficient valve leaflet causing uniform and periodic high frequency fluctuating pressures therefore appeared to be the cause of the musical quality of the systolic murmur in a degenerated bioprosthetic valve.