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Natural history and surgical indications of ventricular septal defect
Natural history and of ventricular septal
surgical defect
indications
Masayoshi Yokoyama, M.D. Atsuyoshi Takao, M.D. Shigeru Sakakibara, M.D. Tokyo, Japan
‘umerous valuable surveys on the natural history of ventricular septal defect (VSD) have been suggesting that there is a high incidence of spontaneous closure which occurs in 50 per cent or more of all cases.l At present, cardiac surgeons might be involved in repairing VSD without consideration of its natural history. If there is a good possibility for VSD to undergo spontaneous closure, then surgery should be considered for asymptomatic patients with relatively small VSD. Th.is paper includes: (1) statistics of VSD patients in our outpatient clinic; (2) statistics of autopsy records in all hospital:; in Japan; and (3) clinical findings of the patients with VSD over 40 years of age. Through these data, the authors have attempted to suggest the high possibility of spontaneous closure of VSD, and to reconsider its surgical indications. Materials
and
methods
Statistics of patients with VSD in our outpatient clinic. From January, 1967, to June, 1969, 22,444 patients visited the outpatient clink (OPC) of the Heart Institute of From the Heart Institute of Japan. Tokyo Received for publication Jan. 9, 1970. Reprint requests to: Masayoshi Yokoyama, Shinjukuku, Tokyo, Japan.
Vol. 30: No. 5,
pp.
597-605
November, 1970
Women’s M.D.,
Japan, which is one of the leading cardiac hospitals in Japan. All patients had some complaints concerned with the cardiac or vascular system. Since the medical insurance controlled by the government covers all Japanese people, they can visit the Heart Institute, regardless of their financial situations. Patients can come to the Institute without any reference letters written by other doctors. Most examinations for diagnosis, including x-ray films of the chest, electrocardiogram (ECG), phonocardiogram (PCG), blood chemistry, dye-dilution test, and so on, could be done in the outpatient clinic. Only cardiac catheterizations and angiographies were performed after admissions. Therefore, most patients were clinically diagnosed in the outpatient clinic. These clinical diagnoses in the outpatient clinic were done by cardiologists who had finished their training. Isolated VSD was found in 1,874 out of 22,444 visitors. VSD associated with other anomalies, such as coarctation of aorta, pulmonary stenosis, and mitral insufficiency, were excluded from this series, In some patients, especially infants, the diagnosis of VSD might be confused with other Medical Heart
College, Tokyo,
Institute,
Tokyo
Japan.
Women’s
American
Medical
College,
lo Kawadacho,
Heart Journal
597
598
Yokoyama, Takao, and Sakakibara
anomalies, such as aortic stenosis, pulmonary stenosis, acyanotic tetralogy of Fallot, etc. If there were any doubt about the clinical diagnosis, the patients were discarded from the series. Patients with the isolated VSD (1,874) were classified according to age groups. Statistics of autopsy records in all hospitals in Japan. The Japanese Association for Pathology publishes the annual report of autopsy cases of the year. According to these publications, 56,582 cases were autopsied throughout Japan in three years from January, 1965, through December, 1967. These figures had included 260 patients with-the isolated VSD. Complicated cardiac malformations in which a VSD was a part of the anomaly were discarded. The patients who died following surgery were also discarded. We divided these 260 patients with VSD into age groups in order to know the age distribution of natural deaths in VSD patients. Some patients might have died prior to admission. We also examined the autopsy records of the Medical Examiner Office in order to study whether cases of VSD were included in autopsy materials of sudden deaths. In Japan, all people who died before any doctor’s examinations were transferred to the Medical Examiner Office for legal autopsies. In three years (January, 1964, to December, 1966), there were 6,337 cases of sudden death in the Tokyo metropolitan area. All of the people were autopsied in the Medical Examiner Office. Clinical jifindings of eatients with VSD over 40 years of age. Out of 1,874 cases with the diagnosis of VSD, there were 12 people over the age of 40 years. Two of these 12
patients unclerwent surgical treatment. As it is infrequen-t to find aged patients with VSD, clinical findings of these 12 patients will be described and discussed later. Statistics of patients with VSD in our outpatient cZinic. The number of patients for the period, January, 1967, to June, 1969, is shown in Table I. Out of 22,444 cases, 1,874 cases were diagnosed as VSD by the clinical findings. As shown in Table I, the ratio of VSD patients to OPC visitors was about 8 per cent in each of the three years. These VSD patients were classified according to age groups in Table II. The age group 0 to 4 years included 1,217 of 1,874 children, which was 64.9 per cent of all VSD visitors. These 1,217 patients under 4 years old were further divided into 0, 1, 2, 3, and 4-year-old groups (Table III). Out of 1,217 patients, 740 were in the O-year-old group (birth to 11 months old). Thus, age distribution of VSD was concentrated in young age groups, especially in the O-year-old group. With the advance of age, the number of OPC visitors with VSD decreased markedly and patients in old age groups were very rare. This pattern of age distribution was fairly similar in each of three years (Tables 11 and III). There were only 12 cases of VSD in patients over 40 years old, which was 0.7 per cent of 1,874 patients. Statistics of autopsy records in all hospitals in Japan. Out of a total autopsy population, 260 cases of isolated VSD were revealed. Age distribution was concentrated within 12 months after birth (Table IV).
Table I. The nmnber of patients with VSD in our outpatient cl&c, January, 1967, to June, 1969 No.
of patients
in
Year 1967 1968 1969
OPC
8,307 9,608 4,529 Total
22,444
No.
of VSD patients in OPC 739 744 391 1,874
OPC
VSD patients per patients (per cent) 8.9 7.8 8.6 8.4
599
Vemtricular sepal defect
Table II. Age distribution
Age in years oto .sto 10 to 15 to 20 to 25 to 30 to 35 to 40 to 4.5 to 50 to 55 to 60 to 65 to
4 9 14 19 24 29 34 39 44 49 54 59 64 69
of patients with VSD in our outpatient clink No.of~~nl,.of~~~.)No.of~~in~ 460 146 59 29 1.5 1.5 I 6 0 0 0 1 1 0 739
Totals
Table III. Age distribution
0 1 2 3 4 Totals
Table IV. Age distributio,n
Age in years 0 to 11 (months) 1 2 3 4 5to 9 10 to 19 20 to 29 30 to 39 40 to 49 50 to 59 60 to 69 Totals
498 102 50 31 30 16 6 5 2 3 1 0 0 0 744
259 62 22 20 9 9 5 1 2 1 0 0 1 0 391
1,217 310 131 80 54 40 18 12 4 4 1 1 2 0 1,874
of patients with VSD under 4 years old A70. of patients
Age in years
y&A&
1967
in
No. of pafients 1968
in
No.
of patients 1969
257 78 50 35 40 -
319 60 40 42 37 -
164 46 18 15 16 -
460
498
259
in
Total No. of patients 740 184 108 92 93 1,217
of autofisied putients in Japan No. off$xtsin
65 7 0 2 1 0 2 3 2 0 0 1 83
1 No. offa$xtsin
70 3 3 2 1 1 1 2 1 0 0 0 84
/ No. ofj$entsin
73 6 0 1 1 1 3 4 3 0 1 0 93
1
:;;I&
208 16 3 5 3 2 6 9 6 0 1 1 260
kbkoyama,
600
Takao, and Sakakibara
Table V. Age distribution
of autopsied pabients with VSD utider 11 months oki -
Age in months 0 1 2 3 4
21 6 6 8 9 4 3
i 7 8 9 10 11
: 1 1 0 Totals
Table
VI.
23 5 11 9 8 6 2 1 3 0 0 2 70
6.5
Autopsies ifz the Tokyo Medical Ewminer No.
Year
of cases autopsied
1964
2,138
1965
2,222
1966
1,977 Total
6,337
Out of 260 patients, 208 were under one year old. Children under 5 years old numbered 235 (90.4 per cent of 260 patients). The relative rarity of death from VSD beyond 2 years old was striking. The number of autopsy cases decreased prominently in the older age groups, as observed in the statistics of our OPC visitors. Autopsy cases of VSD were very rare in patients over 40 years old. The deaths of the 208 children, one year or younger, revealed an interesting pattern of time of death (Table V). In the first month (0 months old) 69 infants died, but many of them probably died sooner because of such conditions as pulmonary bleeding, respiratory troubles, prematurity, and so on. In the second month (one month old), the number of deaths decreased to
25 4 8 11 8 2 6
69 15 25 28 2.5 12 11 2 13 5 1 2
5” 4 0 0 73
208
@ice No.
of VSD
2 (IO-month-old ( 4-month-old 4 (13.day-old (l-year-old (l-year-old @O-year-old 0 ,.5
cases girl) girl) boy) girl) boy) boy)
15, but in the third, fourth, and fifth month, autopsy cases of VSD increased again to 25 cases. This tendency was definitely observed in each of the three years studied. Autopsies of sudden death, numbering 6,337, in the Medical Examiner Office! Tokyo, included 6 cases of VSD. Of great interest is that only one patient of these 6 was 20 years old, and the other 5 patients with VSD were less than one year old (Table VI). VSD in patients over 40 years of age. Clinical findings of twelve patients with VSD over 40 years of age are shown in Table VII. There were six men and six women. They ranged in age up to 63. Patient No. 5, 63 years old, served as a president of a big industrial company.
Ventricularsefital deject
Table VII. --
Clinical jindings
601
of 12 $atients oveY 40 years of age ECG
Yr.,
sex
Functional and therapeutic classi$cation (New York Heart
Cardiothoracic
Association)
(%I
ratio
sin
Rhythm
VI
ST-T
+ Rin
Vs
segment
(mJ.)
52, XI 42, F 61, M
III, IV, III,
c E c
60 63 63
Sinus Sinus Sinus
4.0 7.0 2.5
42, 63, 42, 47,
F M NI M
II, I, I, II,
B A A B
54 51 48 64
Sinus Sinus Sinus Sinus
4.5 4.0 5.2 5.6
,44, 45, 41, 45, 46.
F M F F F
II,
B
55 60 46 60 70
Sinus Sinus Sinus Sinus Sinus
4.0 4.0 4.0 3.3 1.5
I, A I. B 111; c III, c
Patient No. 6, 42 years old, had the ability of Karate third grade. M’ost patients were diagnosed as having congenital heart disease in their school days. From their histories, symptoms seemed to have been stationary for the last couple of decades, except for Patients No. 1, 2, and 3. Patients No. 1 and 2 had surgical repair of V7SD, as they had high left-to-right shunt. In Patient No. 3, a 61-year-old man, the operation was not recommended becausle of pulmonary hypertension and the patient’s age. Functional classification by the New York Heart Association revealed that there were 4 patients with Class I, 3 with Class II, and 5 with Class III or IV VSD. Cardiac shadows on chest x-ray films had enlarged in 7 cases out of 12. Pulmonary vascularity increased markedly or moderately in all cases. All 12 patients had sinus rhythm. There were no patients with arrhythmias such as :auricular fibrillation or A-V block. Arrhythmias in aged patients with ASD are fairly common. However, in aged groups with VSD, arrhythmias are rare.2-4 The lack of atria1 overloading in VSD
Normal T flat in V4-6 Right ventricular hypertrophy Normal lSorma1 Normal Right ventricular hypertrophy Normal T flat in V-7 Normal T flat in Vd-7 Right ventricular hypertrophy
might be the cause of rarity of arrhythmias even in old age groups. As far as voltage of QRS complexes is concerned, there was right ventricular or left ventricular hypertrophy in all patients (Table VII). Flattened T waves in the left precordial leads were noticed in 3 cases (Patients 2, 10, and 11). Case reports Patients 1 to 4 are presented below because of their interesting histories. Patient No. 1: 5Z-year-old nzan, OPD No. 67,429. Although heart murmur was pointed out in his school days, the patient had been free from any subjective symptoms until one year prior to admission. Since then, the patient complained of palpitation on exertion. On admission, the blood pressure was 130/90 mm. Hg. A harsh pansystolic murmur was audible along the left third and forth intercostal sternal border. A thrill was palpable in systole. Pulmonary second tone was not accentuated. X-ray films of the chest showed increased pulmonary flow and moderate cardiomegaly. The ECG revealed left ventricular hypertrophy. The S in V1 plus R in Vs was 4.0 mv. The S-T segment was within normal limits. Cardiac catheterization demonstrated that right ventricular pressure was 45/O mm. Hg. Pulmonary systolic pressure was 25 mm. Hg. There was oxygen step-up at the ventricular level,
602
Yokoyama, Takao, and Sakakibara
The shunt ratio was 50 per cent and the flow ratio was 2.0. At surgery, VSD was found at the membranous portion of the ventricular septum, about 0.8 cm. in diameter. The defect was closed by continuous suture. The postoperative course was uneventful.
Fig.
f. Chest
x-ray
film
Fig.
2. ECG
recordings
of Patient
of Patient
No.
No.
2.
2.
Left-to-right shunt completely disappeared after the operation. Patient No. 2: Wyear-old uronzan, OPD No. 72,061. The patient had lived an almost normal life. She was delivered of one child without any cardiac troubles during her pregnancy. Six months prior to surgery, she suffered from a cold, followed by severe attacks of dyspnea and palpitation. At that time, she developed general edema and an enlarged liver. For the next 6 months, she stayed in bed and was treated for cardiac failure. After then, she was referred to our hospital for surgery. Her blood pressure was 120,&O mm. Hg. Grade 6 systolic murmur was audible over the precordium. No diastolic murmur was noted. The pulmonary second tone was remarkably increased. The heart was enlarged. Pulmonary vascularity was prominent. The pulmonary artery was markedly widened (Fig. 1). The ECG showed sinus rhythm and left ventricular hypertrophy (Fig. 2). Cardiac catheterization w-as performed, which revealed a right ventricular pressure of 78/O mm. Hg. Pulmonary pressure was 70/35 mm. Hg. Oxygen step-up was observed at the right ventricuiar cavity. The shunt ratio was 70.3 per cent, The flow ratio was 3.84. During the operation, median mediasternotomy was done. The pulmonary trunk was extremely enlarged, 5.0 cm. in external diameter. The VSD was 2.5 cm. in diameter. The Dacron patch was sutured on the defect. The membranous portion of the ventricular septum was intact. The right ventricular wall was quite fragile. After the repair of VSD, mattress sutures were done to close the right ven-
Vokmc Number
:30 5
Ventricular
sefital deject
603
triculat’ wall, added by over-and-over running sutures on those mattress sutures. Post.operative course was satisfactory. One month after surgery, cardiac catheterization was performed, indicating the complete disappearance of left-.to-right shunt. Pulmonary arterial pressure decreased to 35 mm. Hg in systole. Pahent No. 3: 61-year-old man, OPD No. 37,666. Until the age of 50, he had been quite healthy. Since then, he had developed palpitation on exertion. Since about a year ago, he had frequent attacks of coughing, associated with profuse sputum. He had also had frequent nasal bleeding. There was Grade 3 systolic murmur in the left third and fourth intercostal spaces along the left sternal border. No diastolic murmur was audible. The murmur was not pansystolic but limited only to the early systolic phase. Pulmonary second sound was remarkably increased (Fig. 3). X-ray film of the chest revealed cardiomegaly and prominence of pulmonary artery. ECG’s showed ventricular hypertrophy (Figs. 4 and 5). Cardiac catheterization showed that right ventricular pressure was lZO/ZO mm. Hg. Pulmonary arterial pressure was 107/42 mm. Hg. The shunt ratio was 38 per cent. The flow ratio was 1.6. Systemic blood pressure was 120/60 mm. Hg. Because of the pulmonary hypertension and the patient’s age, an operation was not recommended in this, case. Patient No. 4: 42-year-old woman, OPD No. 73,004:. The patient was delivered of two children when she was in her twenties. At the age of 31, she suffered from high fever and was admitted to a hospital for 4 months under the diagnosis of sepsis. She was completely cured of the infection with no apparent deterroration of cardiac function. At this time, she was admitted to our hospital for cardiac evaluations. Grade 5 systolic murmur was audible over the precordium. A systolic thrill was palpable. The pulmonary second tone increased moderately and
heart size had increased moderately too. The ECG revealed sinus rhythm. ST-T segments were normal. Right ventricular pressure was 65/O mm. Hg. Pulmonary pressure was 35/20 mm. Hg. Oxygen step-up was noticed at the right ventricular chamber. The shunt ratio was 31 per cent. The flow ratio was 1.5. The systolic blood pressure was 130/80 mm. Hg. An operation was not performed on this patient because the shunt ratio was not high, and because the patient had no special symptoms.
Fit ~. 3. Phonocardiograms
Fig.
of Patient
No.
3.
Discussion
The marked rarity of VSD in adults is noted in our outpatient clinic statistics. If patients with VSD surviving childhood were to die from this malformation in adult ages, there would be many autopsy cases in adult life. However, our studies revealed that adult patients with VSD were seldom autopsied in Japan. Considering that the present financial and economic situation in Japan is relatively good, it seems improbable that severely ill adults with VSD could die at home without any consultations with doctors. Therefore, we did suspect that a number of adult patients with VSD might have died suddenly at home. However, the study of autopsies in the Medical Examiner Office denied the possibility of sudden deaths of patients with
4. Chest
x-ray
film
of Patient
No.
3.
504
Fig.
Yokoymna, Takao, and Sakakibara
5. ECG
recordings
of Patient
No.
3.
VSD. As the population of Tokyo is over 12 million, it is considered that there are 6,000 to 12,000 VSD patients (one VSD patient per one or two thousand people) in the Tokyo area. Since only one adult with VSD suffered from sudden death in these three years, it is clear that adult patients with VSD rarely die suddenly. The disappearance of adult patients with VSD from the outpatient clinic and from the autopsy records of Japan provides a mystery in cardiology. Ventricular septal defects constitute about 20 per cent of all isolated congenital cardiac malformations in children but only 7 per cent of congenital defects in adults. This difference cannot be explained because of early death in those with VSD.516 The one reasonable possibility is spontaneous closure of VSD. In 1964, Bloomfield combined clinical, cardiac catheterization, and autopsy data and concluded that spontaneous closure might occur in as much as 2.5 per cent of all infants born with VSD and that it could also occur in adult life. In 1968, Xoffmanr reported 50 per cent of ventricular septal defects may develop spontaneous closure. If our figures in Tables II and IV correlated with the exact number of VSD pa-
tients through the ages, spontaneous closure of VSD would seem to have occurred at an extremely high rate, in more than 90 per cent of all infants born with VSD. Most pediatric cardiologists support the opinion that the natural history of VSD is fairly good if the patients can survive over 2 years. Our statistics also show that most autopsy subjects were those under 2 years old. Clinical findings of VSD in patients over 2 years old usually remain unchanged. The increase of the cardiothoracic ratio on x-ray films of the chest with the advance of age seems to be rare, unless the patients develop aortic insufficiency. The increase of pulmonary vascular resistance in the course of VSD is still in controversy.8-10 In our clinic, progressive increases of pulmonary vascular resistances in patients with VSD have not yet been experienced. Considering the high possibility of spontaneous closure as well as nonprogressive clinical findings of VSD, asymptomatic patients with small or moderate shunt should be followed up without any prophylactic surgery. Especially in Roger type VSD, natural history should be preferred to the surgical interruption. The incidence of bacterial endocarditis would appear to be relatively small,
Ventricular septal defect
approximately 1 in 1,000 patient years.” Blount’s studie9 also show that with prompt and adequate therapy the mortality rate from bacterial endocarditis is very low. Therefore, surgery should not be advised for small uncomplicated VSD merely to eliminate the risk of bacterial endocarditis. The surgical treatment is not yet necessary for patients over 40 years of age with relatively small left-to-right shunt. Surgical repair is indicated only in patients with high left-to-right shunt (shunt ratio more than 30 per cent), and in patients who show signs of the occurrence of aortic insufficiency. Operations are possible for patients at any age, even after 50 years old, and even after episodes of heart failure or of endocarditis. Our experiences of operations upon very old patients with VSD showed that the hypertrophied right ventricular wall was relatively fragile. The right ventricular wall should be closed carefully. Bleeding began easily, even from the needle holes in the right ventricular muscle. Small bleeders should not be repaired by adding other suturles, but by pressing gauzes. If the myocardial muscle were protected carefully, the operations upon VSD patients over 40 years old provided no difficulties.
should be left for natural development without any surgical interruptions. Operations are indicated for patients with a large left-to-right shunt, bacterial endocarditis, occurrence of aortic insufficiency, and pulmonary hypertension. REFERENCES 1.
2.
3.
4.
5.
6.
7.
8.
9.
Summary
Tvvo cases of VSD in patients over 40 years old were presented. Both of them were operated upon without any postoperative complications. Considering that the natural history of VSD patients is fairly good, and that most young children with VSD have spontaneous closure, we believe that asymptomatic patients with a small or moderate size VSD
605
10.
11.
12.
Hoffman,
J. I.: Natural history of congenital heart disease. Problems in its assessment with special reference to ventricular septal defects, Circulation 37:97, 1968. Fisher, J. M., Wilson, W. R., and Theilen, E. 0.: Recognition of congenital heart disease in the fifth to eighth decades of life, Circulation 25:821, 1962. Mark, H., and Young, D.: Congenital heart disease in the adult, Amer. J. Cardiol. 15:293, 1965. Wolf, P. S., Vogel, J. H., Pryor, R., and Blount, S. G.. Tr.: Atria1 seotal defect in patients over 4.5 years of age, Brit. Heart J. 3O:i15, 1968. Johnson, J. B., Lawlaw, J. W., and Hedgepath, L. E.: Congenital cardiac anomalies in adults, J.A.M.A. 165:915, 1959. Keith, J. D., Rowe, R. D., and Vlad, P.: Heart disease in infancy and childhood, New York, 1958, The Macmillan Company, p. 212. Bloomfield, D. K.: The natural history of ventricular septal defects in patients surviving infancy, Circulation 29:914, 1964. Kirklin, J. W., and DuShane, J. W.: Indications for repair of ventricular septal defects, Amer. J. Cardiol. 12:75, 1963. Griffiths, S. P., Blumenthal, S., Jameson, A. G., Ellis, K., Morgan, B. C., and Malm, J. R.: Ventricular septal defect. Survival in adult life, Amer. T. Med. 37:23. 1964. Kaplan: S., Daoud, ‘G. I., Benzing, G., III, Devine, F. J., Glass, 1. H., and MacGuire, J.: Natural history of ventricular septal defect, Amer. J. Dis. Child. 105:581, 1963. Shah, P., Singh, W. S. A., Rose, V., and Keith, J. D.: Incidence of bacterial endocarditis in ventricular septal defects, Circulation 34:127, 1966. Blount, J. G.: Bacterial endocarditis, Amer. J. Med. 38:909, 1965.
surgical defect
indications
Masayoshi Yokoyama, M.D. Atsuyoshi Takao, M.D. Shigeru Sakakibara, M.D. Tokyo, Japan
‘umerous valuable surveys on the natural history of ventricular septal defect (VSD) have been suggesting that there is a high incidence of spontaneous closure which occurs in 50 per cent or more of all cases.l At present, cardiac surgeons might be involved in repairing VSD without consideration of its natural history. If there is a good possibility for VSD to undergo spontaneous closure, then surgery should be considered for asymptomatic patients with relatively small VSD. Th.is paper includes: (1) statistics of VSD patients in our outpatient clinic; (2) statistics of autopsy records in all hospital:; in Japan; and (3) clinical findings of the patients with VSD over 40 years of age. Through these data, the authors have attempted to suggest the high possibility of spontaneous closure of VSD, and to reconsider its surgical indications. Materials
and
methods
Statistics of patients with VSD in our outpatient clinic. From January, 1967, to June, 1969, 22,444 patients visited the outpatient clink (OPC) of the Heart Institute of From the Heart Institute of Japan. Tokyo Received for publication Jan. 9, 1970. Reprint requests to: Masayoshi Yokoyama, Shinjukuku, Tokyo, Japan.
Vol. 30: No. 5,
pp.
597-605
November, 1970
Women’s M.D.,
Japan, which is one of the leading cardiac hospitals in Japan. All patients had some complaints concerned with the cardiac or vascular system. Since the medical insurance controlled by the government covers all Japanese people, they can visit the Heart Institute, regardless of their financial situations. Patients can come to the Institute without any reference letters written by other doctors. Most examinations for diagnosis, including x-ray films of the chest, electrocardiogram (ECG), phonocardiogram (PCG), blood chemistry, dye-dilution test, and so on, could be done in the outpatient clinic. Only cardiac catheterizations and angiographies were performed after admissions. Therefore, most patients were clinically diagnosed in the outpatient clinic. These clinical diagnoses in the outpatient clinic were done by cardiologists who had finished their training. Isolated VSD was found in 1,874 out of 22,444 visitors. VSD associated with other anomalies, such as coarctation of aorta, pulmonary stenosis, and mitral insufficiency, were excluded from this series, In some patients, especially infants, the diagnosis of VSD might be confused with other Medical Heart
College, Tokyo,
Institute,
Tokyo
Japan.
Women’s
American
Medical
College,
lo Kawadacho,
Heart Journal
597
598
Yokoyama, Takao, and Sakakibara
anomalies, such as aortic stenosis, pulmonary stenosis, acyanotic tetralogy of Fallot, etc. If there were any doubt about the clinical diagnosis, the patients were discarded from the series. Patients with the isolated VSD (1,874) were classified according to age groups. Statistics of autopsy records in all hospitals in Japan. The Japanese Association for Pathology publishes the annual report of autopsy cases of the year. According to these publications, 56,582 cases were autopsied throughout Japan in three years from January, 1965, through December, 1967. These figures had included 260 patients with-the isolated VSD. Complicated cardiac malformations in which a VSD was a part of the anomaly were discarded. The patients who died following surgery were also discarded. We divided these 260 patients with VSD into age groups in order to know the age distribution of natural deaths in VSD patients. Some patients might have died prior to admission. We also examined the autopsy records of the Medical Examiner Office in order to study whether cases of VSD were included in autopsy materials of sudden deaths. In Japan, all people who died before any doctor’s examinations were transferred to the Medical Examiner Office for legal autopsies. In three years (January, 1964, to December, 1966), there were 6,337 cases of sudden death in the Tokyo metropolitan area. All of the people were autopsied in the Medical Examiner Office. Clinical jifindings of eatients with VSD over 40 years of age. Out of 1,874 cases with the diagnosis of VSD, there were 12 people over the age of 40 years. Two of these 12
patients unclerwent surgical treatment. As it is infrequen-t to find aged patients with VSD, clinical findings of these 12 patients will be described and discussed later. Statistics of patients with VSD in our outpatient cZinic. The number of patients for the period, January, 1967, to June, 1969, is shown in Table I. Out of 22,444 cases, 1,874 cases were diagnosed as VSD by the clinical findings. As shown in Table I, the ratio of VSD patients to OPC visitors was about 8 per cent in each of the three years. These VSD patients were classified according to age groups in Table II. The age group 0 to 4 years included 1,217 of 1,874 children, which was 64.9 per cent of all VSD visitors. These 1,217 patients under 4 years old were further divided into 0, 1, 2, 3, and 4-year-old groups (Table III). Out of 1,217 patients, 740 were in the O-year-old group (birth to 11 months old). Thus, age distribution of VSD was concentrated in young age groups, especially in the O-year-old group. With the advance of age, the number of OPC visitors with VSD decreased markedly and patients in old age groups were very rare. This pattern of age distribution was fairly similar in each of three years (Tables 11 and III). There were only 12 cases of VSD in patients over 40 years old, which was 0.7 per cent of 1,874 patients. Statistics of autopsy records in all hospitals in Japan. Out of a total autopsy population, 260 cases of isolated VSD were revealed. Age distribution was concentrated within 12 months after birth (Table IV).
Table I. The nmnber of patients with VSD in our outpatient cl&c, January, 1967, to June, 1969 No.
of patients
in
Year 1967 1968 1969
OPC
8,307 9,608 4,529 Total
22,444
No.
of VSD patients in OPC 739 744 391 1,874
OPC
VSD patients per patients (per cent) 8.9 7.8 8.6 8.4
599
Vemtricular sepal defect
Table II. Age distribution
Age in years oto .sto 10 to 15 to 20 to 25 to 30 to 35 to 40 to 4.5 to 50 to 55 to 60 to 65 to
4 9 14 19 24 29 34 39 44 49 54 59 64 69
of patients with VSD in our outpatient clink No.of~~nl,.of~~~.)No.of~~in~ 460 146 59 29 1.5 1.5 I 6 0 0 0 1 1 0 739
Totals
Table III. Age distribution
0 1 2 3 4 Totals
Table IV. Age distributio,n
Age in years 0 to 11 (months) 1 2 3 4 5to 9 10 to 19 20 to 29 30 to 39 40 to 49 50 to 59 60 to 69 Totals
498 102 50 31 30 16 6 5 2 3 1 0 0 0 744
259 62 22 20 9 9 5 1 2 1 0 0 1 0 391
1,217 310 131 80 54 40 18 12 4 4 1 1 2 0 1,874
of patients with VSD under 4 years old A70. of patients
Age in years
y&A&
1967
in
No. of pafients 1968
in
No.
of patients 1969
257 78 50 35 40 -
319 60 40 42 37 -
164 46 18 15 16 -
460
498
259
in
Total No. of patients 740 184 108 92 93 1,217
of autofisied putients in Japan No. off$xtsin
65 7 0 2 1 0 2 3 2 0 0 1 83
1 No. offa$xtsin
70 3 3 2 1 1 1 2 1 0 0 0 84
/ No. ofj$entsin
73 6 0 1 1 1 3 4 3 0 1 0 93
1
:;;I&
208 16 3 5 3 2 6 9 6 0 1 1 260
kbkoyama,
600
Takao, and Sakakibara
Table V. Age distribution
of autopsied pabients with VSD utider 11 months oki -
Age in months 0 1 2 3 4
21 6 6 8 9 4 3
i 7 8 9 10 11
: 1 1 0 Totals
Table
VI.
23 5 11 9 8 6 2 1 3 0 0 2 70
6.5
Autopsies ifz the Tokyo Medical Ewminer No.
Year
of cases autopsied
1964
2,138
1965
2,222
1966
1,977 Total
6,337
Out of 260 patients, 208 were under one year old. Children under 5 years old numbered 235 (90.4 per cent of 260 patients). The relative rarity of death from VSD beyond 2 years old was striking. The number of autopsy cases decreased prominently in the older age groups, as observed in the statistics of our OPC visitors. Autopsy cases of VSD were very rare in patients over 40 years old. The deaths of the 208 children, one year or younger, revealed an interesting pattern of time of death (Table V). In the first month (0 months old) 69 infants died, but many of them probably died sooner because of such conditions as pulmonary bleeding, respiratory troubles, prematurity, and so on. In the second month (one month old), the number of deaths decreased to
25 4 8 11 8 2 6
69 15 25 28 2.5 12 11 2 13 5 1 2
5” 4 0 0 73
208
@ice No.
of VSD
2 (IO-month-old ( 4-month-old 4 (13.day-old (l-year-old (l-year-old @O-year-old 0 ,.5
cases girl) girl) boy) girl) boy) boy)
15, but in the third, fourth, and fifth month, autopsy cases of VSD increased again to 25 cases. This tendency was definitely observed in each of the three years studied. Autopsies of sudden death, numbering 6,337, in the Medical Examiner Office! Tokyo, included 6 cases of VSD. Of great interest is that only one patient of these 6 was 20 years old, and the other 5 patients with VSD were less than one year old (Table VI). VSD in patients over 40 years of age. Clinical findings of twelve patients with VSD over 40 years of age are shown in Table VII. There were six men and six women. They ranged in age up to 63. Patient No. 5, 63 years old, served as a president of a big industrial company.
Ventricularsefital deject
Table VII. --
Clinical jindings
601
of 12 $atients oveY 40 years of age ECG
Yr.,
sex
Functional and therapeutic classi$cation (New York Heart
Cardiothoracic
Association)
(%I
ratio
sin
Rhythm
VI
ST-T
+ Rin
Vs
segment
(mJ.)
52, XI 42, F 61, M
III, IV, III,
c E c
60 63 63
Sinus Sinus Sinus
4.0 7.0 2.5
42, 63, 42, 47,
F M NI M
II, I, I, II,
B A A B
54 51 48 64
Sinus Sinus Sinus Sinus
4.5 4.0 5.2 5.6
,44, 45, 41, 45, 46.
F M F F F
II,
B
55 60 46 60 70
Sinus Sinus Sinus Sinus Sinus
4.0 4.0 4.0 3.3 1.5
I, A I. B 111; c III, c
Patient No. 6, 42 years old, had the ability of Karate third grade. M’ost patients were diagnosed as having congenital heart disease in their school days. From their histories, symptoms seemed to have been stationary for the last couple of decades, except for Patients No. 1, 2, and 3. Patients No. 1 and 2 had surgical repair of V7SD, as they had high left-to-right shunt. In Patient No. 3, a 61-year-old man, the operation was not recommended becausle of pulmonary hypertension and the patient’s age. Functional classification by the New York Heart Association revealed that there were 4 patients with Class I, 3 with Class II, and 5 with Class III or IV VSD. Cardiac shadows on chest x-ray films had enlarged in 7 cases out of 12. Pulmonary vascularity increased markedly or moderately in all cases. All 12 patients had sinus rhythm. There were no patients with arrhythmias such as :auricular fibrillation or A-V block. Arrhythmias in aged patients with ASD are fairly common. However, in aged groups with VSD, arrhythmias are rare.2-4 The lack of atria1 overloading in VSD
Normal T flat in V4-6 Right ventricular hypertrophy Normal lSorma1 Normal Right ventricular hypertrophy Normal T flat in V-7 Normal T flat in Vd-7 Right ventricular hypertrophy
might be the cause of rarity of arrhythmias even in old age groups. As far as voltage of QRS complexes is concerned, there was right ventricular or left ventricular hypertrophy in all patients (Table VII). Flattened T waves in the left precordial leads were noticed in 3 cases (Patients 2, 10, and 11). Case reports Patients 1 to 4 are presented below because of their interesting histories. Patient No. 1: 5Z-year-old nzan, OPD No. 67,429. Although heart murmur was pointed out in his school days, the patient had been free from any subjective symptoms until one year prior to admission. Since then, the patient complained of palpitation on exertion. On admission, the blood pressure was 130/90 mm. Hg. A harsh pansystolic murmur was audible along the left third and forth intercostal sternal border. A thrill was palpable in systole. Pulmonary second tone was not accentuated. X-ray films of the chest showed increased pulmonary flow and moderate cardiomegaly. The ECG revealed left ventricular hypertrophy. The S in V1 plus R in Vs was 4.0 mv. The S-T segment was within normal limits. Cardiac catheterization demonstrated that right ventricular pressure was 45/O mm. Hg. Pulmonary systolic pressure was 25 mm. Hg. There was oxygen step-up at the ventricular level,
602
Yokoyama, Takao, and Sakakibara
The shunt ratio was 50 per cent and the flow ratio was 2.0. At surgery, VSD was found at the membranous portion of the ventricular septum, about 0.8 cm. in diameter. The defect was closed by continuous suture. The postoperative course was uneventful.
Fig.
f. Chest
x-ray
film
Fig.
2. ECG
recordings
of Patient
of Patient
No.
No.
2.
2.
Left-to-right shunt completely disappeared after the operation. Patient No. 2: Wyear-old uronzan, OPD No. 72,061. The patient had lived an almost normal life. She was delivered of one child without any cardiac troubles during her pregnancy. Six months prior to surgery, she suffered from a cold, followed by severe attacks of dyspnea and palpitation. At that time, she developed general edema and an enlarged liver. For the next 6 months, she stayed in bed and was treated for cardiac failure. After then, she was referred to our hospital for surgery. Her blood pressure was 120,&O mm. Hg. Grade 6 systolic murmur was audible over the precordium. No diastolic murmur was noted. The pulmonary second tone was remarkably increased. The heart was enlarged. Pulmonary vascularity was prominent. The pulmonary artery was markedly widened (Fig. 1). The ECG showed sinus rhythm and left ventricular hypertrophy (Fig. 2). Cardiac catheterization w-as performed, which revealed a right ventricular pressure of 78/O mm. Hg. Pulmonary pressure was 70/35 mm. Hg. Oxygen step-up was observed at the right ventricuiar cavity. The shunt ratio was 70.3 per cent, The flow ratio was 3.84. During the operation, median mediasternotomy was done. The pulmonary trunk was extremely enlarged, 5.0 cm. in external diameter. The VSD was 2.5 cm. in diameter. The Dacron patch was sutured on the defect. The membranous portion of the ventricular septum was intact. The right ventricular wall was quite fragile. After the repair of VSD, mattress sutures were done to close the right ven-
Vokmc Number
:30 5
Ventricular
sefital deject
603
triculat’ wall, added by over-and-over running sutures on those mattress sutures. Post.operative course was satisfactory. One month after surgery, cardiac catheterization was performed, indicating the complete disappearance of left-.to-right shunt. Pulmonary arterial pressure decreased to 35 mm. Hg in systole. Pahent No. 3: 61-year-old man, OPD No. 37,666. Until the age of 50, he had been quite healthy. Since then, he had developed palpitation on exertion. Since about a year ago, he had frequent attacks of coughing, associated with profuse sputum. He had also had frequent nasal bleeding. There was Grade 3 systolic murmur in the left third and fourth intercostal spaces along the left sternal border. No diastolic murmur was audible. The murmur was not pansystolic but limited only to the early systolic phase. Pulmonary second sound was remarkably increased (Fig. 3). X-ray film of the chest revealed cardiomegaly and prominence of pulmonary artery. ECG’s showed ventricular hypertrophy (Figs. 4 and 5). Cardiac catheterization showed that right ventricular pressure was lZO/ZO mm. Hg. Pulmonary arterial pressure was 107/42 mm. Hg. The shunt ratio was 38 per cent. The flow ratio was 1.6. Systemic blood pressure was 120/60 mm. Hg. Because of the pulmonary hypertension and the patient’s age, an operation was not recommended in this, case. Patient No. 4: 42-year-old woman, OPD No. 73,004:. The patient was delivered of two children when she was in her twenties. At the age of 31, she suffered from high fever and was admitted to a hospital for 4 months under the diagnosis of sepsis. She was completely cured of the infection with no apparent deterroration of cardiac function. At this time, she was admitted to our hospital for cardiac evaluations. Grade 5 systolic murmur was audible over the precordium. A systolic thrill was palpable. The pulmonary second tone increased moderately and
heart size had increased moderately too. The ECG revealed sinus rhythm. ST-T segments were normal. Right ventricular pressure was 65/O mm. Hg. Pulmonary pressure was 35/20 mm. Hg. Oxygen step-up was noticed at the right ventricular chamber. The shunt ratio was 31 per cent. The flow ratio was 1.5. The systolic blood pressure was 130/80 mm. Hg. An operation was not performed on this patient because the shunt ratio was not high, and because the patient had no special symptoms.
Fit ~. 3. Phonocardiograms
Fig.
of Patient
No.
3.
Discussion
The marked rarity of VSD in adults is noted in our outpatient clinic statistics. If patients with VSD surviving childhood were to die from this malformation in adult ages, there would be many autopsy cases in adult life. However, our studies revealed that adult patients with VSD were seldom autopsied in Japan. Considering that the present financial and economic situation in Japan is relatively good, it seems improbable that severely ill adults with VSD could die at home without any consultations with doctors. Therefore, we did suspect that a number of adult patients with VSD might have died suddenly at home. However, the study of autopsies in the Medical Examiner Office denied the possibility of sudden deaths of patients with
4. Chest
x-ray
film
of Patient
No.
3.
504
Fig.
Yokoymna, Takao, and Sakakibara
5. ECG
recordings
of Patient
No.
3.
VSD. As the population of Tokyo is over 12 million, it is considered that there are 6,000 to 12,000 VSD patients (one VSD patient per one or two thousand people) in the Tokyo area. Since only one adult with VSD suffered from sudden death in these three years, it is clear that adult patients with VSD rarely die suddenly. The disappearance of adult patients with VSD from the outpatient clinic and from the autopsy records of Japan provides a mystery in cardiology. Ventricular septal defects constitute about 20 per cent of all isolated congenital cardiac malformations in children but only 7 per cent of congenital defects in adults. This difference cannot be explained because of early death in those with VSD.516 The one reasonable possibility is spontaneous closure of VSD. In 1964, Bloomfield combined clinical, cardiac catheterization, and autopsy data and concluded that spontaneous closure might occur in as much as 2.5 per cent of all infants born with VSD and that it could also occur in adult life. In 1968, Xoffmanr reported 50 per cent of ventricular septal defects may develop spontaneous closure. If our figures in Tables II and IV correlated with the exact number of VSD pa-
tients through the ages, spontaneous closure of VSD would seem to have occurred at an extremely high rate, in more than 90 per cent of all infants born with VSD. Most pediatric cardiologists support the opinion that the natural history of VSD is fairly good if the patients can survive over 2 years. Our statistics also show that most autopsy subjects were those under 2 years old. Clinical findings of VSD in patients over 2 years old usually remain unchanged. The increase of the cardiothoracic ratio on x-ray films of the chest with the advance of age seems to be rare, unless the patients develop aortic insufficiency. The increase of pulmonary vascular resistance in the course of VSD is still in controversy.8-10 In our clinic, progressive increases of pulmonary vascular resistances in patients with VSD have not yet been experienced. Considering the high possibility of spontaneous closure as well as nonprogressive clinical findings of VSD, asymptomatic patients with small or moderate shunt should be followed up without any prophylactic surgery. Especially in Roger type VSD, natural history should be preferred to the surgical interruption. The incidence of bacterial endocarditis would appear to be relatively small,
Ventricular septal defect
approximately 1 in 1,000 patient years.” Blount’s studie9 also show that with prompt and adequate therapy the mortality rate from bacterial endocarditis is very low. Therefore, surgery should not be advised for small uncomplicated VSD merely to eliminate the risk of bacterial endocarditis. The surgical treatment is not yet necessary for patients over 40 years of age with relatively small left-to-right shunt. Surgical repair is indicated only in patients with high left-to-right shunt (shunt ratio more than 30 per cent), and in patients who show signs of the occurrence of aortic insufficiency. Operations are possible for patients at any age, even after 50 years old, and even after episodes of heart failure or of endocarditis. Our experiences of operations upon very old patients with VSD showed that the hypertrophied right ventricular wall was relatively fragile. The right ventricular wall should be closed carefully. Bleeding began easily, even from the needle holes in the right ventricular muscle. Small bleeders should not be repaired by adding other suturles, but by pressing gauzes. If the myocardial muscle were protected carefully, the operations upon VSD patients over 40 years old provided no difficulties.
should be left for natural development without any surgical interruptions. Operations are indicated for patients with a large left-to-right shunt, bacterial endocarditis, occurrence of aortic insufficiency, and pulmonary hypertension. REFERENCES 1.
2.
3.
4.
5.
6.
7.
8.
9.
Summary
Tvvo cases of VSD in patients over 40 years old were presented. Both of them were operated upon without any postoperative complications. Considering that the natural history of VSD patients is fairly good, and that most young children with VSD have spontaneous closure, we believe that asymptomatic patients with a small or moderate size VSD
605
10.
11.
12.
Hoffman,
J. I.: Natural history of congenital heart disease. Problems in its assessment with special reference to ventricular septal defects, Circulation 37:97, 1968. Fisher, J. M., Wilson, W. R., and Theilen, E. 0.: Recognition of congenital heart disease in the fifth to eighth decades of life, Circulation 25:821, 1962. Mark, H., and Young, D.: Congenital heart disease in the adult, Amer. J. Cardiol. 15:293, 1965. Wolf, P. S., Vogel, J. H., Pryor, R., and Blount, S. G.. Tr.: Atria1 seotal defect in patients over 4.5 years of age, Brit. Heart J. 3O:i15, 1968. Johnson, J. B., Lawlaw, J. W., and Hedgepath, L. E.: Congenital cardiac anomalies in adults, J.A.M.A. 165:915, 1959. Keith, J. D., Rowe, R. D., and Vlad, P.: Heart disease in infancy and childhood, New York, 1958, The Macmillan Company, p. 212. Bloomfield, D. K.: The natural history of ventricular septal defects in patients surviving infancy, Circulation 29:914, 1964. Kirklin, J. W., and DuShane, J. W.: Indications for repair of ventricular septal defects, Amer. J. Cardiol. 12:75, 1963. Griffiths, S. P., Blumenthal, S., Jameson, A. G., Ellis, K., Morgan, B. C., and Malm, J. R.: Ventricular septal defect. Survival in adult life, Amer. T. Med. 37:23. 1964. Kaplan: S., Daoud, ‘G. I., Benzing, G., III, Devine, F. J., Glass, 1. H., and MacGuire, J.: Natural history of ventricular septal defect, Amer. J. Dis. Child. 105:581, 1963. Shah, P., Singh, W. S. A., Rose, V., and Keith, J. D.: Incidence of bacterial endocarditis in ventricular septal defects, Circulation 34:127, 1966. Blount, J. G.: Bacterial endocarditis, Amer. J. Med. 38:909, 1965.