Togo Horiuchi

Quantitative analysis of pulmonary vascular disease in simple cardiac anomalies with the Down syndrome.

Intimal changes and medial thickness of small pulmonary arteries were morphometrically examined in 21 cases of simple cardiac anomalies with the Down syndrome, and their correlations with age and with pulmonary arterial peak pressure were then compared with those of 20 cases of simple cardiac anomalies without the Down syndrome and 17 cases of complete transposition of the great arteries (TGA). Results indicate that (1) intimal changes developed at an earlier age in patients with simple cardiac anomalies and the Down syndrome than in those without the Down syndrome, (2) the intimal changes were more severe than those in simple cardiac anomalies without the Down syndrome at the same level of pulmonary arterial pressure and milder than those in TGA, and (3) the media of small pulmonary arteries in simple cardiac anomalies with the Down syndrome was thinner than the media in cases without the syndrome at the same radius and the same level of pulmonary arterial pressure but thicker than the media in TGA. Retarded development of medial hypertrophy in the Down syndrome or TGA in response to pulmonary hypertension appears to make the pulmonary arteries susceptible to even moderate pressure load and appears to be responsible for early development of severe intimal changes.

Quantitative analysis of postoperative changes in the pulmonary vasculature of patients with complete transposition of the great arteries and pulmonary hypertension

Postoperative changes in the medial thickness of the small pulmonary arteries and the degree of pulmonary vascular disease were estimated histometrically and histopathologically in three cases of late death after total correction of complete transposition of the great arteries with large ventricular septal defect and pulmonary hypertension. In the postoperative course of two of the three cases extreme medial hypertrophy of the small pulmonary arteries as well as severe pulmonary vascular disease were found. In the third case, the thickening of the media was mild and pulmonary vascular disease had not progressed owing to a residual ventricular septal defect. Examination of three additional cases of late death and 15 autopsy cases of complete transposition of the great arteries revealed that hypertrophy of pulmonary arterial media after radical surgery for complete transposition of the great arteries is a common phenomenon. In cases of complete transposition of the great arteries with severe pulmonary hypertension, the deveopment of marked hypertrophy of the media accompanied by pulmonary vascular disease after total correction is usually seen and seems to be the most likely cause of death in the postoperative period.

Congenital Coronary Arteriovenous Fistulas

Abstract One hundred and twelve cases of congenital coronary arteriovenous fistula from the literature and 5 cases of our own were studied. These were classified into seven types according to origin and outlet in an effort to evaluate operative techniques for each type of fistula. Ligation or division of the fistula without the aid of cardiopulmonary bypass is generally the operative procedure of choice, and the results have been excellent (7 deaths among 81 patients operated upon in this collected series). However, serious postoperative complications such as myocardial ischemia, conduction disturbances, and aneurysm formation with sudden rupture were not infrequently reported. It is therefore concluded that operative techniques other than simple ligation or division should be considered for some patients, such as those in Type R 1 or L 1 of our classification, and cardiopulmonary bypass should be employed whenever the fistula is located in the posterior aspect of the heart.

Surgical Treatment of Annuloaortic Ectasia: Experience in 7 Consecutive Patients

Seven patients with annuloaortic ectasia were treated according to the method described by Bentall and De Bono. A Björk-Shiley valve in a composite graft was the prosthesis of choice and was used in all patients except 1, who received a Starr-Edwards valve. Profound topical cooling without selective coronary perfusion was applied in 5 patients for myocardial preservation during aortic occlusion. The was 1 operative death and 1 late death, the latter from cerebral thromboembolism. Five survivors have been followed from 1 year 5 months to 2 years 10 months with an average follow-up of 2 years. Excellent results were obtained in all survivors, their physical capabilities putting them in Class I of the New York Heart Association Functional Classification. Postoperative aortograms showed no signs of kinking or compression of vascular prostheses nor abnormalities of prosthetic calves. A modified technique to secure graft fixation is discussed.

Subclavian–Coronary Artery Anastomosis in Infancy for the Bland-White-Garland Syndrome: A Two-Year Angiographic Follow-up

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.

Surgical Treatment of Large Ventricular Septal Defect with Pulmonary Hypertension in the First 24 Months of Life

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.

Open-heart repair of ventricular septal defect in infancy by means of surface-induced hypothermia and coronary perfusion.

SummaryThis 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.

Double-Outlet Left Atrium with Intact Ventricular Septum

The clinical course and surgical repair of double-outlet left atrium with intact ventricular septum in a 13-year-old girl are presented. The only outlet of the right atrium was a secundum atrial septal defect, and the left atrium drained into both ventricles through two atrioventricular valves. To our knowledge, there has been only one other published report of repair of double-outlet left atrium.

FROZEN SECTION DIAGNOSIS OF HYPERTENSIVE PULMONARY VASCULAR CHANGES AT SURGERY FOR CONGENITAL HEART ANOMALIES

Frozen sections of lung biopsy were prepared in 30 cases of congenital heart anomalies with pulmonary hypertension, and evaluation of severity of the pulmonary vascular changes was made during the surgery. Statistically, significant difference was not found between the diagnosis made by frozen sections and those made by paraffin sections. It is concluded that rapid and accurate diagnosis of pulmonary vascular changes can be made by means of frozen section diagnosis. This method will prove to be clinically important by assisting during operation in rapid assessment of indication for total correction of congenital heart anomalies. ACTA PATHOL. JPN. 35 : 803–807, 1985.