Case Reports

Case Reports Quadrivalvular Heart Disease An Autopsied Case with Massive Pulmonary Regurgitation Tsuguya SAKAMOTO, M.D., Zen'ichiro UOZUMI, M.D., Nobuyoshi KAWAI, M.D., Yoshiyuki SAKAMOTO, M.D., Ryoko KATO, M.D., and Hideo UEDA, M.D. SUMMARY An autopsied case of quadrivalvular heart disease was described, in which pulmonary regurgitation due to possible bicuspid valve was pre- dominant and tricuspid stenosis, mitral stenosis, and aortic stenosis with insufficiency coexisted. The patient was 47, and finally 53 years old female with long-term history of cough due to bronchial compression by the enormously dilated pulmonary artery. Clinical examination revealed massive pulmonary regurgitation, which was further substantiated by right heart catheterization and cineangiocardiography. The phonocardiograms and the reference tracings suggested the co-existence of tricuspid stenosis, aortic stenosis with regurgitation and mitral stenosis. Cardiac catheterization, intracardiac phonocardiography and angio- cardiography also favored to the diagnosis of organic tricuspid stenosis. However, the ignorance of the presence of such an unusual combination misled to the precise antemortem diagnosis. Discussion was made on the rarity of quadrivalvular heart disease, and the pathogenesis of this unusual pulmonary regurgitation was analyzed based on the autopsy finding and the history as well as the clinical mani- festation. Finally, combination of the murmurs of organic and relative tricuspid stenosis was presented to explain the acoustical findings of the present case. Additional Indexing Words: Phonocardiography Mechanocardiography Bronchial compression Right-sided Austin Flint murmur UADRIVALVULAR heart disease was first described by Shattuck1) in 1891. However, the involvement of all four valves in a given patient is extremely rare.2) The present paper describes one of such case, in which From the Second Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Tokyo. Presented at the 45th Kanto-Koshinetsu-Hokuriku Regional Meeting of Japanese Circulation Society in May 27, 1967. Received for publication August 20, 1967. 303 304 SAKAMOTO, ET AL. Heart J. y, 1968 the co-existing severe pulmonary regurgitation made the other right -sided valve lesion difficult to estimate and , furthermore, almost completely masked the left-sided lesions. CASEREPORT The patient, working as a house-keeper over past 20 years, was first seen at age 47 years and finally at age 53. She was admitted to the Second Department of Internal Medicine, Tokyo University Hospital, with a half-year history of dry cough. Her history of heart disease was described at age 19, when she suffered from arthralgia of hip, knee and ankle joints without appreciable fever. At age 28, fever up t38 accompanied by polyarthritis persisted about 1 month and subsided by appropriate treatment (details unknown). At age 34, she occasionally complained of shortness of breath and palpitation after exertion. No history of gonococcal or syphilitic infection was found. On physical examination, excessive dry cough was observed paroxysmally, but no respiratory distress was noticed. There were slight edema on face, distinct jugular venous engorgement with dominant pulsation, and definite parasternal im- pulse suggesting right ventricular hypertrophy. Neither anemia nor cyanosis were noticed. Pulse was regular with the rate of 54 at rest and blood pressure was 124/80 mm.Hg with no difference in the pulse among four limbs. Liver was palpable 2 finger breadths below the costal margin. Routine blood studies disclosed 110 per cent hemoglobin, 5.21 million red blood cells and 6,800 white blood cells with a normal differential count. Serologic test for syphilis was negative. Laboratory studies for rheumatic fever or other infection were also negative. Palpation over the precordium revealed prominent parasternal heave maximally located in the 3rd intercostal space near the left sternal border. However, apex beat was hardly palpable. On auscultation, a grade 5 ejection systolic murmur with systolic thrill was present in the 3rd intercostal space at the left sternal border and widely transmitted over the precordium. The other main auscultatory findings were a loud and medium to high-pitched blowing diastolic murmur in the 4th intercostal space at the left sternal border and a loud mid-diastolic rumble with diastolic thrill in the 5th intercostal space at the left parasternal region. The second heart sound was not sufficiently audible. Phonocardiogram confirmed the 2 components of the second heart sound which showed a wide splitting. The one of the diastolic murmurs was following the pulmonary component (IIP), thus the pulmonary regurgitation was readily diagnosed as a main valve affection. Tricuspid stenosis was suspected based on the diastolic rumble, though the positive Rivero Carvallo's sign was lacking. However, the conclusion of either organic or relative (functional) stenosis was not reached, because of the massive pulmonary regurgitation. The loud systolic murmur was thought to be caused by relative pulmonary stenosis, and the further investigation was not performed. Chest roentgenogram and fluoroscopy revealed the prominent pulmonary artery with marked pulsation, small aorta just over the pulmonary artery, and the adhesion of left pleura without exudation. Electrocardiogram revealed the right ventricular hypertrophy, and the P-wave abnormality was not so seriously analyzed as the sign of atrial overloading. All of these including physical and laboratory examination were thought to be consistent Vol. 9 No. 3 QUADRIVALVULAR HEART DISEASE 305 with the clinical diagnosis of massive pulmonary regurgitation with dilated pulmonary artery. She was on sedative and cough medicine several days and discontinued to visit the hospital. She was seen as an outpatient at age 52 for the second time, because of intract- able cough even at rest. Physical examination was essentially unchanged. The neck vein was pulsated as before, and showed prominent a wave, but venous pressure was only 120mm. H2O. The blood pressure was 136/82mm.Hg. Slight cyanosis was observed and edema on face and legs was also noticed slightly. Chest roentgenogram (Fig. 1) and electrocardiogram (Fig. 2) were not changed significantly, Fig. 1. Chest roentgenogram. Fig. 2. Electrocardiogram. except occasional atrial premature beats. She was once hospitalized to examine the cause of persistent cough. Bronchography proved the mechanical compression of the left main bronchus and bronchoscopy pointed out the diffuse edematous swelling at the mouth of this bronchus. Conclusion was attained to the fact that the compression of bronchus by the enormously enlarged pulmonary artery is the primary cause of the persistent cough. Administration of codeine phosphoricum partly succeeded in the sedation of cough, and the edema disappeared subsequently during hospital course. Phonocardiographic examination including pulse studies was repeatedly done at 7 times during her course. The loud ejection systolic murmur was recorded in all areas (Fig. 3, A to G) and was best recorded at the upper base (Fig. 3, D and E). Though auscultation could not reveal, another type of ejection murmur of long dura- tion and slightly delayed peaked configuration was present at the upper part of the parasternal line (Fig. 3, F), which partly drowned the aortic component (IIA) of the second heart sound. The conclusion of these ejection murmurs was that the former is aortic and the latter is pulmonary. The aortic ejection sound was then identified along left sternal border (Fig. 3, B to E). The second heart sound was widely split (Fig, 3, B to D) and IIA-IIP interval was 0.06 to 0.07 sec. Neither IIA nor UP showed accentuation. No mitral or tricuspid opening snap was identified. Tracing from apex (Fig. 3, A), where the first heart sound was best audible, revealed 306 SAKAMOTO, ET AL. Heart J. , 1968 Fig. 3. Phonocardiograms. Paper speed: 100mm./sec. Time lines: 0.01 and 0.1 sec. Details: see text. Vol. 9 No. 3 QUADRIVALVULAR HEART DISEASE 307 Jap. Heart J.M 308 SAKAMOTO, ET AL. ay, 1968 the prolongation of Q-I interval (0.075 sec.) and the mid-diastolic and the faint but definite atrio-systolic murmurs, the latter of which terminated shortly before the first heart sound probably because of the upper normal limit of the P-R interval (0.19 to 0.20 sec.). Over the right ventricular area (Fig. 3, B), the mid-diastolic murmur was of large amplitude and the time course was slightly different from the apical one. The atrio-systolic murmur was of lesser amplitude, but this was of relatively large amplitude one intercostal space above (Fig. 3, C), and the time course was quite different from the apical murmur. The pulmonary regurgitant murmur was easily identified by the definite time relationship to IIP, which was clearly seen in Fig. 3D, and partly in Fig. 3C, in which both tricuspid and pulmonary diastolic murmurs were superimposed. This pulmonary regurgitant murmur con- tained both low- and high-pitched components and of decrescendo configuration. Fig. 4. Reference tracings. Details: see text. Vol. 9 No. 3 QUADRIVALVULAR HEART DISEASE 309 Fig. 5. Pressure curves from PA, RV and RA. Though this configuration is commonly observed in case with Graham Steell murmur, the quality was much more similar to the murmur of organic pulmonary insufficiency. In the upper part of the left parasternal line (Fig. 3, F), another type of regurgitant murmur started from IIA was recorded, and this was mainly high-pitched, suggesting the presence of aortic regurgitation. Again, one intercostal space below, both regurgitant diastolic murmurs were superimposed (Fig. 3, E). In the upper sternal border of both left and right sides the atrial sound was recorded with significant amplitude (Fig. 3, G) and this was distinctly audible in the neck (Fig. 3, H). The ejection systolic murmur was also present in both sides of the neck with lesser in- tensity. The jugular phlebogram (Fig. 4, A) taken the other day disclosed prominent a wave, decreased x and v and almost absent y with little or no subsequent ascent.

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