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From May 1980 through April 1982, 9 patients underwent the Mustard operation for simple transposition of the great arteries (simple TGA) at the Tohoku University Hospital, Sendai. There was no early postoperative death, although one patient was lost with pulmonary venous obstruction in the late postoperative period. No pulmonary venous obstruction occurred since the introduction of expanded polytetrafluoroethylene (EPTFE) intraatrial baffle. Normal sinus rhythm has been preserved in all patients since direct, high superior vena cava cannulation was adopted. Postoperative hemodynamic study at an average 12 months after surgery revealed normal right ventricular function at rest. Work-function curve which is related to the right ventricular end-diastolic pressure and minute work index revealed good response of the right ventricle in two of three patients after methoxamine infusion test. All late survivors are acyanotic and clinically well. The Mustard procedure has dramatically improved the survival rate and quality of life for those with simple TGA. Presently, we continue to utilize the Mustard procedure for simple TGA until the superiority of other operations are demonstrated.

Pulmonary vascular disease (PVD) was histologically evaluated and its severity was expressed as an index of PVD in 14 autopsied and eight biopsied cases of complete transposition of the great arteries (TGA) in patients more than 6 months of age. The index varied from 1.0 to 2.3 in six patients who had survived complete surgical repair and ranged from 2.3 to 3.3 in five patients who had died of PVD postoperatively. Consequently, an index of 2.2 could be regarded as an upper limit of PVD for complete surgical repair. The index was significantly correlated to some hemodynamic factors. From the regression equation, the value 2.2 of histologic index could be translated into the clinical factors concerning hemodynamics. We consider that patients with pulmonary vascular resistance of less than 10.6 units . m2, and pulmonary arterial mean pressure less than 51 mm Hg, are suitable candidates for complete surgical repair in TGA, if more than 6 months of age. Thus histologic assessment of PVD in a lung biopsy as an indication for total correction of TGA with pulmonary hypertension appears to be useful in patients who are in borderline hemodynamic condition or in whom the hemodynamics could not be evaluated although pulmonary hypertension was suspected.

Left subclavian artery-left coronary artery end-to-end anastomosis was successfully performed on a six-month-old female infant with anomalous origin of the left coronary artery from the pulmonary artery and severe mitral regurgitation. A piece of pulmonary arterial wall including the opening of the anomalous left coronary artery (ALCA) was isolated and was used for anastomosis instead of utilizing the left coronary arterial wall itself. Her postoperative course was uneventful and mitral regurgitation disappeared 6 months after surgery. This improved technique seems to be applicable for this anomaly, regardless of the age of patient or size of the left coronary artery.

This paper presents our recent results of the Mustard procedure (intra-artrial baffle operation) for the complete transposition of the great arteries performed in 24 infants and children during the past 6 years at the Tohoku University Hospital. All intracardiac repairs were performed using "bypass hypothermia" (surface-induced deep hypothermia, circulatory arrest, and limited cardiopulmonary bypass). The hospital mortality rate was 8% in patients with simple transposition without pulmonary hypertension (Group I, one death in 12 patients), 50% in patients with ventricular septal defect (VSD) and pulmonary hypertension (Group II, 3 deaths in 6 patients), and 20% in patients with VSD and pulmonary stenosis (Group III, one death in 5 patients). Various types of longstanding dysrhythmia were found in 6 out of 18 long-term survivors, and subsequent pace-maker implantation was necessary in one infant. Postoperative pulmonary venous obstruction occurred in 3 infants, and surgical relief of the obstruction was successfully undertaken in 2 of them. Prevention of postoperative dysrhythmia, pulmonary venous obstruction, and possible brain damage are also discussed.

Histopathologic and morphometric studies of small pulmonary arteries were performed in 16 cases of secundum atrial septal defect (ASD) and severe pulmonary hypertension. Besides typical plexogenic arteriopathies found in six cases, organized microthrombi and what we call "musculoelastosis," that is, proliferation of longitudinal smooth muscle bundles and elastic fibers, were seen in small pulmonary arteries in three and four cases, respectively. These changes were observed co-existing in the remaining three cases. Thrombi of the small pulmonary arteries and musculoelastosis were the forms of pulmonary vascular disease characteristically found in the older patients with both ASD and pulmonary hypertension. Yamaki's index of pulmonary vascular disease, though effective in describing severity of plexogenic arteriopathy, proved to be less so for the intimal lesions in old microthrombi and for musculoelastosis. Among the cases with plexogenic arteriopathy, there was a positive significant correlation between the medial thickness of small pulmonary arteries and peak arterial pressure, which, however, was not demonstrated when all the cases of ASD were included.

Various kinds of operations for coarcation of the aorta in infancy are life saving, but they still carry a high operative mortality rate and are followed frequently by residual or recurrent coarctation. Fifteen years ago we performed a modified Blalock-Park procedure on a 12 month old male infant with preductal coarctation, a large VSD and PDA. Recently a follow-up study was performed on this patient. Aortography revealed a normally developed aortic arch without any sign of stenosis. The intraaortic pressure tracings revealed no pressure gradient through the aortic arch and thoracic aorta. These findings suggest that this procedure is recommendable for the treatment of infants with severely hypoplastic aortic isthmus.

KAGAWA, Y., HORIUCHI, T., ITO, T., SUZUKI, Y., HANEDA, K., ISHIZAWA, E., ARAI, S., SEKINO, Y. and. SATO, N. Long-Term Results of Open Heart Repair of Ventricular Septal Defect. Tohoku J. exp. Med., 1987, 151(1), 1-14-Long-term follow-up results of 321 patients who underwent open hert repair of ventricular septal defect in our institute and have lived for more than five years were analyzed using questionnaires. The follow-up period was up to 24 years with an averagr of 12.9 years. The questionnaire mainly concerned the patient's state of health. Beside this, IQ was measured in 52 children, ECG was analyzed in 92 patients and heart catheterization was performed on 21 patients with preoperative severe pulmonary hypertension (PH). From this survey, it was ascertained that (1)no desths resulted from cardiac disorders; (2) physical activity and development were satisfactory in nearly all patients; (3) the scholastic acheivement score was within the normal range in nearly all school children; (4) among 30 live births from 24 adult female patients, two babies with simple cardiac anomalies were found and (5) there remained very few patients suffering from residual PH, residual shunt or conduction disturbances. IQ as tested by individual intelligence tests was within the normal range in all children. On ECG, complete right bundle branch block with left anterior hemiblock was found in 9% and complete atrioventricular block in 7%. Amelioration of PH was achived especially in those who underwent operations in their younger years.

A 58-day-old girl with hypoplastic left heart syndrome underwent bypass grafting from the pulmonary artery to the descending aorta, ligation of the ductus arteriosus, and banding of the main pulmonary artery distal to the graft. Anastomoses of the graft were performed by partial clamping of the arteries under moderate surface hypothermia. The patient tolerated the procedure well and was discharged from the intensive care unit on the eleventh postoperative day, but she died on the twentieth day of metabolic derangement. Our total experience with this anomaly includes three other infants, and a summary of these patients and applied surgical procedures are presented. Surgical and diagnostic considerations for this anomaly based on our experiences are discussed.

Among the heart diseases, there are some cases in which pulmonary arteries cannot be visualized by standard right ventriculography and aortography. We have categorized such a case as "angiographically absent pulmonary artery syndrome", which may include congenital or acquired absent pulmonary artery, and anatomically present but non-visualized pulmonary artery. It has been extremely difficult to achieve an accurate diagnosis in such cases and there has been no report of successful surgical treatment for them. Recently, we developed a method of retrograde pulmonary arteriography, with which the pulmonary artery can be successfully visualized in cases of angiographically absent pulmonary artery. Subsequently, re-establishment of the blood flow to the non-visualized pulmonary artery has been successfully performed in 7 of 8 cases of this syndrome. --angiographically absent pulmonary artery syndrome; retrograde pulmonary arteriography; surgical repair.

Morphometric study of the pulmonary vasculature was performed on lung biopsy specimens from a one-year-old girl who underwent anatomic repair of isolated aortic origin of the right pulmonary artery. Medial hypertrophy of small pulmonary arteries in the right lung was much less remarkable than that in the left lung. In contrast, intimal lesions in the right lung were much more advanced than those in the left lung. Fully oxygenated blood in the right pulmonary artery might suppress medial hypertrophy in response to high pressure and thin media fail to protect intima from high pressure, resulting in severe intimal lesions. This situation in the right lung resembles that in complete transposition of the great arteries.

In 14 of 15 patients ranging in age from 1 to 62 years (mean of 34) with secundum atrial septal defect (ASD) and pulmonary hypertension over 60 mm Hg peak pressure, operative indication was determined by morphometric diagnosis of open biopsy of lung specimens. In one patient, open lung biopsy was also performed during corrective surgery. Pulmonary arterial changes in the 15 patients were grouped into four classifications as follow: (1) plexogenic pulmonary arteriopathy (six patients); (2) thromboembolism in small pulmonary arteries (three patients); (3) "musculoelastosis," intimal proliferation of longitudinal smooth muscle bundles and elastic fibers (three patients); and (4) combinations of (1), (2) or (1), (3) (three patients). We conclude concerning the operative indication that group 2 patients are operable in all cases and group 1 patients with Yamaki's index of pulmonary vascular disease of 2.2 or less; group 3 patients with the absence of complete occlusion of the small pulmonary arterial lumen are operable, and patients with clear evidence of severe plexogenic pulmonary arteriopathy in group 4 are not operable. Comparative analysis of pulmonary pathology and hemodynamic performance revealed that open lung biopsy should be performed to determine operative candidacy in cases with a pulmonary vascular resistance greater than 8 unit X m2, which is considered to represent the borderline of operability.

The postoperative change in pulmonary vascular disease following complete surgical repair in cases of the complete transposition of the great arteries with severe pulmonary hypertension remains an extremely interesting problem. We have performed complete surgical repair on an 8-month-old boy with severe pulmonary hypertension having a pulmonarysystemic pressure ratio of 1.0 and 14 units of pulmonary vascular resistance, but who suddenly died 9 months postoperatively. The results of a comparison of the histometrical measurements of biopsy lung and autopsy lung showed postoperative hypertrophy of the media of the small pulmonary arteries and progressed pulmonary vascular disease. It is thought that this phenomenon was brought about in the following way: The specific factor in this congenital heart disease which suppresses the hypertrophy of the pulmonary arterial media is removed due to the total correction and the media over-reacts to the postoperative pulmonary arterial pressure, resulting in hypertrophy. Repeated vasoconstriction of the abnormally thick pulmonary arterial walls leads to ischemic change in the peripheral blood vessels and obstructive pulmonary vascular disease is progressively brought about.

The corrected transposition of the great arteries with pulmonary stenosis was completely repaired in a 5 1/2-year-old boy. To relieve the stenosis of the pulmonary valve ring which was located posterior to the aorta and heart chambers, the valve-bearing tube-graft made of the pericardium was implanted as a bypass graft between the right-sided ventricle and the pulmonary trunk. Postoperative hemodynamics and cineangiographic findings were satisfactory. The use of the viable autologous tissue such as the pericardium is definitely more advantageous than that of the other homografts, since the former requires neither sterilization nor long-term preservation.

Cardiac anomalies in 34 consecutive cases with malposition of the heart were classified according to Van Praagh and surgical treatment was reviewed with special reference to the possibility of total correction. Mirror image-dextrocardia and mesocardia are deemed mostly adaptable to total corrective surgery with the criteria of indication almost identical with VSD or tetralogy of Fallot. Dextrocardia and levocardia in heterotaxy, frequently too complex in malformation, are generally considered inaccessible for total corrective surgery at present. However, total correction may be applicable to some of these cases.

ISHIZAWA, E., HORIUCHI, T., TADOKORO, M., SUZUKI, Y. and YAMAKI, S. Surgical Management of Pulmonary Atresia, and Critical Pulmonary Stenosis with Intact Ventricular Septum. Tohoku J. exp. Med., 1986, 150 (2), 135-144-Our experience with the surgical management of hypoplastic right ventricle with intact ventricular septum has included 20 patients with pulmonary atresia and 3 patients with critical pulmonary senosis. Group 1 consisted of 7 infants who were treated by transarterial pulmonary valvotomy. Four of them were lost shortly after surgery. Group 2 included 11 neonates and one infant who were treated by systemic-pulmonary shunt with 2 operative death. Group 3 included 4 infants who underwent primary prosthetic enlargement of the right ventricular outflow tract. Two of the 4 were lost in the early postoperative period. Eight of 13 patients in groups 1 and 2 who survived the initial palliative procedures underwent additional operations to enlarge the right ventricular outflow tract, and 5 patients survived. However, adequate right ventricular and tricuspid valve growth was obtained in only patients in whom the prosthetic enlargement of the right ventricle was performed in early infancy. These findings strongly suggest the necessity to decompress the right ventricle completely, as early as possible. In conclusion, 1) transarterial pulmonary valvotomy seldom provided satisfactory decompression of the right ventricle, and operative risk was high. 2) systemic-pulmonary shunt to re-establish pulmonary blood flow improved neonatal survival, although this procedure may not provide a satisfactory long term palliation. 3) primary or secondary operative enlargement of the right ventricle in early infancy provided decompression of the right ventricle and accelerated substantial right ventriculargrowth. For patients without an infundibulum or for those in whom attempts to induce tricuspid valve growth had failed, a Fontan type procedure seemed to be the procedure of choice.

A 6-month-old female infant with anomalous origin of the left coronary artery underwent an end-to-end anastomosis of the left subclavian artery to the left coronary artery. A cuff of the pulmonary artery was used for the anastomosis. The child continued to have mitral regurgitation but has done well with medical treatment over the last four years. Cardiac catheterization 26 months after operation confirmed a patent graft without narrowing, improved contractility of the left ventricle, normal end-diastolic pressure of the left ventricle, and persistent mitral regurgitation. This technique is applicable in infants regardless of the size of the left coronary artery.

Forty-three infants (less than 12 months of age) underwent VSD closure without operative mortality between June 1982 and December 1987. Average age and body weight at the operation were 6.9 months and 5.3 kg, respectively. Associated cardiac anomalies were PDA (11 patients), ASD (5) and PS (1). Preoperative pulmonary to systemic peak pressure ratio (Pp/Ps), resistance ratio and flow ratio, and pulmonary vascular resistance were 0.79 +/- 0.15, 0.29 +/- 0.16, 2.52 +/- 0.60 and 3.05 +/- 1.94 unit.m2, respectively. VSD was closed under combined surface/perfusion hypothermia with total circulatory arrest in 25 patients and standard cardiopulmonary bypass in 18. All patients were discharged from the hospital in good condition, but there was one late death due to pneumonia. Postoperative Pp/Ps decreased to 0.39 +/- 0.11. Most of the patients demonstrated satisfactory body weight gain after VSD closure. These results support our current policy of aggressive surgical intervention for refractory VSD in the early stage of life.

YAMAKI, S., HORIUCHI, T., MIURA, M., SUZUKI, Y., ABE, Y., ISHIZAWA, E. and KAGAWA, Y. Musculoelastosis: A Change of Small Pulmonary Arteries Found in a Case with Atrial Septal Defect and Pulmonary Hypertension. Tohoku J. exp. Med., 1985, 145 (4), 455-463-Interesting findings were obtained in the lung biopsy of a 46year old female patient with secundum atrial septal defect and pulmonary hypertension. In the intima of small pulmonary arteries, there were a marked increase in elastic fibers and marked proliferation of longitudinal smooth muscle cells. The vascular lumen was markedly stenotic, but reconstruction of small pulmonary arteries indicated that all vascular lumina were patent. The case was accordingly diagnosed as operable and closure of atrial septal defect was undertaken. Three years postoperatively, the patient is well with much improved condition and lowered pulmonary arterial pressure was demonstrated by cardiac catheterization. This case had apparently serious vascular lesions due to severe intimal thickening which we labeled “musculoelastosis”, but it was found that such thickening alone did not cause peripheral pulmonary arterial occlusion. It is therefore thought to be a benign pulmonary vascular condition.

HANEDA, K., SATO, S., ISHIZAWA, E. and HORIUCHI, T. The Importance of Colloid Osmotic Pressure during Open Heart Surgery in Infants. Tohoku J. exp. Med., 1985, 147 (1) 65-71-Fifty-five infants with transposition of the great arteries and with total anomalous pulmonary venous return underwent intracardiac repair under combined surface/perfusion hypothermia and total circulatory arrest in 1975 to 1983. Although cardiopulmonary bypass (CPB) time is limited when hypothermic circulatory arrest is employed, fluid balance derangement is one of the major postoperative complications. Fluid balance at the end of CPB averaged +299.5ml (+63.4ml/kg) when hemodilution with lactated Ringer's was utilized (Group A). Since colloid osmotic pressure (COP) plays an important role in regulating fluid balance, colloid hemodilution prime (whole blood and plasma) was employed in the last 3 years (Group B). COP and total protein concentration during CPB with colloid prime were maintained at around 19mmHg and 5g/100ml, respectively. In Group B, fluid balance at the end of CPB averaged +81.1ml (+16.3ml/kg) and was significantly less than in Group A (p

A total of 74 patients under 24 months of age with large ventricular septal defects (VSD) and pulmonary hypertension were subjected to surgical treatment from 1969 through 1975. Emergency pulmonary artery (PA) banding was performed in 13 patients during the first year of life with 1 death from postoperative respiratory failure. Primary closure of the VSD was performed in 61 patients using simple hypothermia and short-term coronary perfusion, with an operative mortality of 1.6%. There were no late deaths or neurological disturbances. Normal hemodynamic data were obtained in all 7 patients who underwent postoperative cardiac catheterization from one month to five years after the primary correction. It is concluded that primary closure of a VSD in infancy is reasonable and that PA banding is indicated only for those patients less than 6 months old with a complicated defect or in an emergency situation.

YAMAKI, S., SUZUKI, Y., KAGAWA, Y., KAHATA, O., ONODERA, H., TOGO, T., HONGO, T., SEKINO, Y., ISHIZAWA, E. and HORIUCHI, T. Histologic Findings of Ductus Arteriosus and Pulmonary Vascular Disease in Interrupted Aortic Arch. Tohoku J. exp. Med., 1982, 136 (3), 291-298 - There are still a number of unsolved problems concerning the total correction of interruption of the aortic arch. Here we report a histopathological investigation of the patent ductus arteroous and pulmonary vascular disease using autopsy material of a patient with Celoria type B interruption of the aortic arch who died postoperatively. The so-called ductus arteriosus between the pulmonary arterial trunk and the descending aorta was lacking in elastic fibers and smooth muscle cells at the media showing discontinuous, transverse fibrous tissue. The vessel walls were weak and normal development was apparently impossible. Consequently, it was concluded that in the reconstruction of the aortic arch in such cases with PDDT, it is advisable not to use the patent ductus arteriosus. In this case, hypertrophy of the media of small pulmonary arteries was marked, but pulmonary vascular intimal lesions were mild. With regard to the pathogenesis, it is thought that there was sufficient thickening of the media of the pulmonary arterial system in response to the pulmonary hypertension present from birth, and it prevented the progression of the intimal lesions by accomodating to the intraarterial pressure.

A total of 216 cases underwent open-cardiac operations up to December 1963. The hypothermia was applied for 121 instances, while 95 individuals were operated upon by means of heart-lung machine. On the basis of the above clinical experiences, advantages and disadvantages of hypothermia and heart-lung machine have been described and our current point of view about operative indications has been presented. Furthermore, detailed account has been made on history and development of hypothermia and heart-lung machine for open-heart surgery in our department. Some improved points which may contribute to widening indications of hypothermia, in particular, have been discussed.

In normal postnatal development, physiological regression of the muscular coat is accomplished to the major part in the first 6 months. Thereafter, the medial thickness still continues slowly to diminish and practically attains the level of normal adults in 4 years. With the present histometrical method, the medial thickness at an arterial radius of 100 μ was estimated at 9.8 μ immediately after birth, was reduced to 6.8 μ in 6 months, to 6.5 μ in a year and to 5.9 μ in 4 years, while the value for normal adults was 5.4 μ. In patients with ventricular septal defect (VSD), physiological medial regression does not take place, and the muscular coat retains or increases its neonatal thickness. The medial thickness is correlated with pulmonary blood pressure level and can be expressed by an exponential function of blood pressure. Pulmonary hypertension in VSD is correlated with the dimension of septal defect. Medial hypertorphy in VSD is probably associated with increased vascular resistance of pulmonary circulation.

This is a follow-up study of the second consecutive series of 71 infants during the year of 1965 through 1970. Over-all mortality rate was 15.5 per cent. In the last two years, the result has improved markedly as exemplified by only one death occuring in 33 patients (3 per cent) operated. The striking decrease in the mortality rate during this period was mainly due to the intensive care of postoperative respiratory dysfunction and prevention of low cardiac output especially in infants under one year of age. Permanent heart block and complications of the central nervous system have not been observed in this series. In conclusion, patients with VSD and pulmonary hypertension should be advised to undergo radical operation even in early stage of infancy.