Clinical Studies on Congenital Heart Disease (1964~1975)

The clinical studies were performed on 757 patients who had been admitted to Severance Hospital from May, 1964 to June, 1975 with the diagnosis of congenital heart disease. The following results were obtained; 1. Among 757 patients, cardiac catheterization was performed on 367 patients. 2. Four hundred and thirty eight patients were male and 319 were female. Over half of them were below two years of age, but among 357 cardiac catheterized patients, 137 (37.3%) patients were ranged between 6 to 10 years. 3. V.S.D., Tetralogy of Fallot, P.D.A., A.S.D. were found in order of frequency. 4. Among 757 patients, combined extra-cardiac anomalies were found in 42 patients, and 14 patients were with Downanjx syndrome. 5. Among 367 cardiac catheterized patients, 164 (44.7%) patients had cardiac operations, and among these, 18 (11.0%) patients died including 7 patients of Tetralogy of Fallot.

Two Cases of Single Ventricle

Two cases of single ventricle were diagnosed in a 2 year and 5 months old female baby an. a 8 year old boy according to cardiac catheterization with selective cineangiogram. We report these cases with review of a little literature.

A Hemodynamic Study of Isolated Congenital Pulmonary Stenosis

Isolated pulmonary stenosis, a relatively common congenital anomaly that accounts for about 10 percent of all congenital heart disease, is characterized by stenosis of pulmonary valve itself, infundibulum or both of them. Since cardiac catherterization was applied to man by Cournand and Ranges, pulmonary stenosis had been easily diagnosed and many clinical studies had been investigated. It has a wide clinical spectrum depending on the degree of stenosis. The electrocardiogram, phonocardiogram and chest X-ray have proved useful in estimating the severity of hemodynamic facotrs in individual cases. This series comprises 47 cases in whom the clinical diagnosis of isolated pulmonary stenosis was confirmed by right heart catheterization with cardiac cineangiography at Severance Hospital, Yonsei University. An attempt was made to correlate the electrocardiographic, phonocardiographic, chest X-ray findings, and types of stenosis with the hemodynamic data in these cases. 1. Of 47 patients, 33 were male and 14 female. Their ages ranged from 2 to 42 years: the mean age was 19.1 years. 2. The incidence was 5.9 percent of all 797 catheterized congenital heart disease cases. The pulmonary valvular stenosis was 30 (68.3%), infundibular 7 (14.6%) and combined 10 (21.6%) cases. 3. The correlation between electrocardiogram and hemodynamic data were as follows. i) The regression equation between right ventricular sysytolic pressure (RVSP) and height of R wave in V1 lead (RV1) was RVSP=3.32 RV1+48.2: its correlation coefficient was 0.818 and it was very significant (p=0.000). ii) The higher the RVSP, the more the frontal axis of QRS complex shifted to the right side (r=0.55. p=0.001). iii) The RVSP of the groups with positive ECG findings such as p-pulmonale, right ventricular hypertrophy or right ventricular strain were much higher than the RVSP of the groups without such findings (p=0.032.0.000, 0.000). iv) The group with RV1 higher than 20 mm showed much more elevated mean of RVSP than the group with lower RV1 (p=0.000). v) The groups with the above mentioned positive ECG findings showed good correlation with the severity of RVSP which was arbitrarily classified as 49 or less, 50~100, and 100 mmHg or more (chi-square=8.96, 26.69, 19.06; p=0.011, 0.000, 0.000). 4. The group with late occurrence of the maximum intensity of the ejection systolic murmur showed higher mean of RVSP than the group with early peak of the murmur (p=0.014). 5. The means of RVSP of the groups with chest X-ray findings such as decreased pulmonary vascularity, were much higher than the means of RVSP of the groups without such findings (p=0.000, 0.005, 0.015). The groups with above mentioned positive chest X-ray findings showed good corelations with the severity of RVSP which was classified as above limits (chi-square=7.55, 10.94, 13.36; p=0.022, 0.004, 0.001). 6. Combined pulmonary valvular and infundibular stenosis showed more severe systolic pressure gradient and higher mean of RVSP than the isolated types (p=0.000).

Silent Aortic Regurgitation

Aortic regurgitation is a common valvular heart disease, usually the result of rheumatic fever, or syphilis, and rarely of congenital origin. It is frequently associated with other valvular heart disease, especially mitral valve disease. It can be diagnosed by the presence of pulse pressure widening, a Corrigan pulse, and an early decreascendo diastolic murmur at the left sternal border between the second and third intercostal spaces. After the clinical application of cineaortography in the diagnosis of valvular disease, Segal et al (1964) first reported rheumatic aortic regurgitation without an audible murmur in patients having mitral valve disease. The importance of discovering aortic reguritation in patients with predominent mitral disease has begun to be appreciated recently, especially as commisurotomies for the relief of mitral stenosis are performed more frequently. Nowadays eventhough the severity of aortic regurgitation is often not evident preoperatively, aortic regurgitation can become very evident when mitral stenosis is relieved. This study was comprised of seventeen patients with silent aortic regurgitation which was confirmed by cineaortography at Severance Hospital from January, 1970 to August, 1976. 1. Of the seventeen patients, 12 patients were associated with mitral stenosis, 4 with mitral steno-insufficiency, and 1 with mitral insufficiency. 2. Silent aortic regurgitation was suggested from the accompanying clinical features such as chest pain, apical heaving, and left ventficular hypertrophy pattern on both roentgenogram of the chest and electrocardiogram. 3. The severity of the aortic regurgitation was mild to moderate; 7 of the 17 patients being grade I, and 10 patients being grade II on cineaortogram.

Clinical Study on Mitral Valve Prolapse Syndrome

Recently the syndrome of mitral valve prolapse with associated auscultatory and other clinical findings has generated considerable interest. Although this syndrome was originally described as benign, more recent observations have demonstrated that the patients are subject to sudden death, life threatening arrhythmias, bacterial endocarditis or hemodynamically significant mitral regurgitation. It is therefore important to identify such patients so that appropriate antibiotic prophylaxis and antiarrhythmic therapy may be instituted. Since earlier reports of this syndrome by Barlow et al. in 1963, various names or descriptions have been applied to the condition based upon pathologic findings, on cinical investigators has stressed different aspects of the entity. Now it is well known that the syndrome is no longer a benign condition. We have recently had the opportunity to study 15 cases of mitral valve prolapse, which was confirmed by left ventricular cineangiography. Among them 9 cases had associated other cardiac anomalies, most frequently secundum type of atrial septal defect. Among 15 cases 8 were female and the ages ranged from 20 to 52. Selective cine-coronary arteriography was also performed in 2 cases who complained of severe chest pain. We discussed generally the clinical features including hemodynamic and angiocardiographic findings, and pertinent literature published until recently were reviewed.

Blood Oxygen Content in the Various Chambers of the Right Side of the Heart in Patients without Intracardiac Shunts

It would seem logical that a left to right shunt may be localized to that chamber in which an increase in oxygen content beyond the normal variation is first seen, while this is generally true, there are certain limitations to such direct diagnostic interpretation. The interpretation of an observed difference in oxygen content is influenced by the result of extremely poor mixing or of a change in respiratory, circulatory or metabolic steady states. This is one of the major challenges to proper interpretation. The purpose of this study was to analyze the blood oxygen content in various chambers of the right side of the heart in patients without shunt and to study the difference in oxygen content between the various chambers. The right heart catheterization was performed in 19 cardiac patients without shunt in Cardiac Laboratory in Hospital. 1. The blood oxygen content was 12.36+/-2.23 volume per cent (mean+/-standard error) in superior vena vava, 12.59+/-2.23 volume per cent in inferior vena cava, 12.59+/-2.23 volume per cent in mid-right atrium, 12.92+/-1.90 volume per cent in mid-right ventricle and 12.26+/-2.13 volume per cent in main pulmonary artery. 2. The mean difference of oxygen content was 0.16 volume per cent between superior vena cava and right atrium, 0.08 volume per cent between right atrium and right ventricle, and 0.06 volume per cent between right ventricle and main pulmonary artery.

Mesangial IgA/IgG Deposit Glomerulonephritis

Percutaneous renal biopsy was performed on a 34 year old male patient with mild proteinuria and microhematuria. Histopathologic examination showed a focal mesangiopathic glomerulonephritis, simulating a "minimal change" disease pattern by light microscope. Granular deposits of IgA, C3, IgG, IgM, and fibrinogen were present in the glomerular mesangial area by immunofluorescent technique. A special prevalence of IgA was found. The intensity of immunofluorescent staining was correlated with the mesangial proliferative reaction by light microscopy. Electron microscopy showed electron dense granular deposits in the mesangial areas. The glomerulonephritis in this patient was related with the IgA antibody associated mesangial immune complex deposit disease mediated by the classic complement pathway. This glomerulonephritis is known to have a good prognosis. The antigenic nature, the reason of predominant immune deposits in the mesangium, and the mechanism of a special prevalence of IgA and IgM immunoglobulin classes are discussed, and special attention to the value of immunofluorescent study of renal diseases, with a review of the literature, is given.

A Study on the Use of the Electrocardiogram for Diagnostic Evaluation of Patients with Mitral Valvular Disease

Electrocardiography has been long an important tool in cardiac diagnosis and, with advances in electrocardiography, the accuracy of the electrocardiographic diagnosis has been greatly increased. Though the most accurate methods for quantitative diagnosis of mitral valvular disease are cardiac catheterization and ventriculography, these procedures are time consuming, expensive, and not without risk, thus, it would be helpful if routine catheterization of the heart could be avoided in patients who are potential condidates for mitral valvulotomy. This could be done if reliable electrocardiographic criteria could be found for estimating the amount of obstrcution and leak at the mitral valve. As mitral valvular dysfunction progress, changes (hypertrophy and/or dilation) in the left atrium and both ventricles are inevitable. Many authors attempted to characterize the electrocardiographic findings of such changes according to the specific lesion of the mitral valve. In addition to atrial fibrillation, characteristic P wave changes and their diagnostic significance have been reported (Macruz et al., 1958; Arevalo et al., 1963: Morris et al., 1964). The diagnostic importance of QRS voltage difference in precordial leads has been stressed in the differential diagnosis of specific lesions of mitral valvular disease (Janton et al., 1954: Bateman and January, 1955: Wierum and Glenn, 1957: Bentivoglio et al., 1958: Imperial et al., 1960). Semle and Pruitt(1960) reported that a mean QRS electrical axis of +91degrees or more degrees was the most frequent positive single index of increased total pulmonary resistance in mitral stenosis, and Fowler et al. (1955) stated that precordial lead V1 was very helpful in evaluating the degree of pulmonary hypertension. In Korea there are only a few reports on the electrocardiographic changes in mitral valvular disease and the correlation of electrocardiographic findings and hemodynamics (Oh et al., 1961: Kim, 1970: Kim, 1971). It would be evident that the various electrocardiographic findings noted in western races can't be applied to Koreans. The main objectives of this study are: 1. To determine the electrocardiographic characteristics of pure mitral valvular disease and the differentiation between the specific lesions of pure mitral stenosis, pure mitral insufficiency and combined lesions of mitral stenosis and insufficiency. 2. To know whether the characteristic electrocardiographic changes of mitral stenosis are directly related to the narrowed valve area or to the hemodynamic abnormalities secondary to obstruction. SUBJECTS AND METHODS: 139 cases of isolated mitral valvular disease were reviewed: of these 93 were cases of pure mitral stenosis, 18 were pure mitral insufficiency, and 28 were combined mitral stenosis and insufficiency. Of the total patients, 68 were male and 71 were female. The ages ranged from 10 to 54 years with an average of 35.6 years. Diagnosis was based on cardiac catheterization and supplemented by cienangiocardiography. The conventional 12 lead electrocardiogram was taken at normal sensitivity and at a paper speed of 25mm/sec. The mitral valve area was estimated according to the Gorlin's formula and cardiac output was determined by the direct Fick's principle. The electrocardiograms were analyzed with respect to: 1. Rhythm (atrial fibrillation and sinus rhythm) 2. Presence or absence of P-mitrale 3. Terminal P force in lead V1 (by the method of Morris et. al., 1964) 4. Mean QRS electrical axis in frontal plane 5. QRS voltage in percordial leads(V1S, V5R, V6R & V1S+V(5-6)R) 6. R/S ratio in lead V1 7. Conduction disturbance of right bundle branch block In patients with pure mitral stenosis the electrocardiographic findings of atrial fibrillation P-mitrale, terminal P force in lead V1 were correlated with the hemodynamic data of mean pulmonary artery pressure, mean pulmonary arterial wedge pressure and mitral valve area. An attempt was made to ascertain whether or not a quantitative correlation could be found. A patient showing electrocardiographic pattern of right bundle branch block was excluded in the evaluation of QRS voltage in lead V1 and mean QRS electrical axis in frontal plane. RESULTS AND SUMMARY: 1. P wave abnormality, which was noted in most (131/139) cases, is apparently a characteristic and most frequent electrocardiographic finding in mitral valvular disease. Of the P weve abnormalities the development of atrial fibrillation and P-mitrale were thought to be related to the duration of the illness rather than to the types of lesion or hemodynamic abnormalities secondary to valvular dysfunction. However, the terminal P force in lead V1 was thought to be related to the mean pulmonary arterial wedge pressure rather than to narrowing of the valve. 2. 15 patients showed the electrocardiographic pattern of right bundle branch block. In patients with mitral stenosis this electrocardiographic pattern was noted at almost all levels of mean pulmonary artery pressure, mean pulmonary arterial wedge pressure, mitral gradient and mitral valve area. 3. Mean QRS electrical axis and QRS voltage in precordial leads; There was no case which deviated leftward more than +30degrees even among cases with a predominant or pure mitral insufficiency. Although the difference of mean value in mean QRS electrical axis and QRS voltage in precordial leads according to the types of the lesion was significant, this difference was generally not helpful in the differential diagnosis in individual patients because of much overlapping among cases. 4. There was no definite electrocardiographic criteria to differentiate clearly the types of mitral valvular disease. However, the following aspects of electrocardiogram may be useful in differential diagnosis. a. Difference of QRS voltage in precordial leads: The volage of V1S and V1S+V(5-6)R in all patients with pure mitral insufficiency was over 1mm and 11mm respectively. That of V1S+V(5-6)R in all patients with pure mitral stenosis was below 39mm. b. R/S ratio in lead V1: There was no case showing "R wave only" in lead V1 among patients with pure or predominant mitral insufficiency. c. Mean QRS electrical axis in frontal plane: The mean QRS electrical axis of all patients with pure mitral stenosis deviated rightward more than +60degrees in all except one case. None of the patients with pure mitral insufficiency deviated rightward more than +110degrees. 5. Relationship between hemodynamics and electrocardiography in paitents with mitral stenosis: Among the hemodynamic abnormalities, mean pulmonary artery pressure showed a close relationship with the following aspects of the electrocardiogram. a. R/S ratio in lead V1:The mean value of mean pulmonary artery pressure (45.9+/-3.8mmHg) in groups showing R/S>1 was significantly elevated as compared with that (34.8+/-1.5mmHg) of groups showing R/Sdegrees1. b. Mean QRS electrical axis in frontal plane: There was a weak positive correlation (r=+0.53) between mean pulmonary artery prersure and QRS electrical axis in the frontal plane. The QRS axis of all patients with a mean pulmonary artery pressure of 41mmHg or more was +91degrees or more except for one case. c. Terminal P force in lead V1: The difference of mean value in mean pulmonary artery pressure according to the size of terminal P force in lead V1 was significant in all cases.

A Study on Intraventricular Conduction Disturbances in Electrocardiogram

The electrocardiograms of a total of 12,796 patients taken in the past three years at Severance Hospital were reviewed for conduction disturbances which were found in 314 cases(2.46%). Th first degree atrioventricular block occurred in 65 cases, and second degree atrioventricular block with Wenckebach periodicity in 13. There were 9 cases of third degree atrioventricular block with nomal QRS complexes causing Adams-Stokes attacks which represented 2.9% of all conduction disturbances. It occurred equally in both sexes and predominantly after 6th decade of life. There were 99 cases of complete right bundle branch block(RBBB) and 12 cases of complete left bundle branch block(LBBB). The latter were mainly associated with significant heart diseases. As for the intraventricular conduction defects other than the simple bundle branch blocks, the findings were as follows; 1. Simple hemiblocks were found in 92 cases or 0.73% of total series and 29.3% of all conduction disturbances. Left anterior hemiblock(LAH) occurred 3 times more frequently than left posterior hemiblock(LPH). 2. Partial bilateral bundle branch block(BBBB) were found in 25 cases representing 0.2% of the total series and 8.0% of all conduction disturbances. Among the 25 cases of BBB, 11 had RBBB plus LAH, 10 RBBB plus LPH, 1 LBBB plus first degree A-V block, and 3 LBBB of diphtheritic cardic involvement. Trifascicular heart block causing Adams-Stokes attacks occurred in 9 of the 25 cases resulting in death in 7 cases. The underlying diseases were ischemic heart disease, hypertensive heart disease, congenital heart disease and diphtheritic carditis. This report represents the first electrocardiographical documentation of occurrence of trifascicular heart block progressing from BBBB in Korea.

Retrograde Conduction in Complete Heart Block

A 45 year old man with a history of syncopal attacks of 3 days' duration showed complete heart block with occasional retrograde P waves following QRS complexes in the electrocardiogram. The site of A-V block seemed to be below A-V node judging from the QRS complexes when occasional capture occured. This may be a case of complete heart block progressing from right bundle branch block plus left posterior hemiblock. There was a rapid improvement of conduction with sublingual isoproterenol in a few days, EKG's showing normal A-V conduction and QRS complexes on discharge. The mechanism of the retrograde conduction in the presence of complete heart block is discussed with a review of literature.

Two Cases of Ventricular Septal Defect with Aortic Insufficiency

The ventricular septal defect with aortic insufficiency is a relatively uncommon congenital heart disease. Once the prolapse of aortic cusp complicates the ventricular septal defect, the prolapse of the aortic cusp is a progressive, rather than static lesion. With the curent possibility of surgical correction in cases of ventricular septal defect and other abnormalities, the differential diagnosis assumes marked importance. vTwo cases of ventricular septal defect with aortic insufficiency are presented with a review of pertinent literature. These young female patients had long standing palpitation and dyspnea on exertion since childhood. The physical examination showed with pulse pressure, bounding carotid pulsation and a systolic ejection-type murmur and a decrescendo diastolic murmur at the upper left sternal border, which simulated "machinery" type murmur of patent ductus arteriosus. The ventricular septal defect with aortic insufficiency was demonstrated by cardiac catheterization and cineangiocardiography.

Estimation of Intracardiac Shunts in Congenital Heart Disease: A comparison of the dy-edilution and the Fick methods

During cardiac catheterizations in 20 cases with congenital heart disease, intracardiac shunts were measured by two methods, dye dilution method and the Fick method, and the results were compared. Arterial dilution curves were used for the estimation of both the right-to-left and left-to-right shunts-Venous dilution curves were used for measurement of left-to-right shunts. In cases with a left-to-right shunt, the amount of the shunt was expressed as per cent of total pulmonary blood flow and, in cases with a right-to-left shunt, as per cent of total systemic blood flow. The following results were obtained. 1. In 8 cases with a right-to-left shunt, the amount of the shunt was 35.6 +/-18.1% by the Fick method and 34.0 +/-19.2% by arterial dilution curves. The difference was not statistically significant(p> 0.l). In 15 cases with a left-to-right shunt, the amount of shunt was 41.3 +/-26.6% by the arterial dilution curve and 36.6 +/-14.O% by the Fick method. The difference was not statistically significant(p> 0.1). Also venous dilution curve was performed in 8 cases of left-to-right shunt and the amount of the shunt was 33.0 +/-21.0%, as compared to 35.5 +/-17.3% by the Fick methods. The difference was not statistically significant (p> 0.1). 2. Arterial dilution curves could be used to localize right-to-left shunts and venous dilution curves left-to-right shunts, whereas tile Fick method was helpful only for the localization of left-to-right shunts. 3. It was possible to detect small left-to-right (less than 10%) and right-to-left (less than 5%) shunts by dye dilution curves, which was not possible with the Fick method. 4. In detection of small intracardiac shunts as well as in shunt localization, the dye dilution method was more accurate than the Fick method. The difference of the amount of the shunt estimated by the two methods was not statistically significant.

Open Heart Surgery Under Cardiopulmonary Bypass Combined with Hypothermia and 5% Dextrose Priming

Open heart surgery under cardiopulmonary bypass which is combined with moderate hypothermia and 5% dextrose priming offers several advantages over the conventional methods; i.e., simplification of the machine, economy in operation, elimination of the need for a large amount of heparinized blood, avoidance of hemaological complications and utilization of the unit as a standby method. The results of open heart operations with this method in our initial 10 cases were comparable to other methods. None of the complications and the mortality were directly attributable to the oxygenator itself.