Congenital coronary artery fistula in infancy and childhood: diagnostic and therapeutic aspects.

Clinical symptoms and age at manifestation of a congenital coronary artery fistula may vary considerably. They depend on the underlying anatomy and also on the size of the fistulous connection to the left or right side of the heart. Using colour Doppler echocardiography for direct visualization of the entire course of the fistulous vessel, including the site of termination, succeeds only in a small number of cases. Furthermore, regular coronary vessels branching off proximally and distally of the coronary artery fistula usually are not recognizable by this method. Only selective angiography provides this information and is unchallenged the most important and indispensable diagnostic technique, especially with regard to surgical treatment. This publication presents physical, echocardiographic, and angiographic data of 15 patients, who were admitted to the German Heart Center Munich between 1970 and 1993. By an invasive diagnostic approach the following arteriovenous fistulous connections were found: from right coronary artery to right atrium (3 patients) or to right ventricle (3 patients), from left coronary artery to right atrium or coronary sinus (3 patients), from left coronary artery to right ventricle (4 patients) and from right and left coronary artery to right ventricle (2 patients). In 5 patients a "proximal" form of coronary artery fistula ("side-to-side pattern") was found, in 8 patients a "distal" form ("end-artery type"), and in 2 patients a combination of both forms. In 14 patients surgical closure was performed (6 symptomatic infants, mean age at surgery = 95 days, and 8 asymptomatic children, mean age at surgery = 7.1 years): 13 patients survived surgery. On an average of 5 years after surgery all of these 13 patients are in excellent condition (NYHA functional class I). The experiences in surgical treatment verify the importance of an exact angiographic visualization of the anatomy of a coronary artery fistula and the regular coronary vessels branching off proximally and distally of the fistula. Closure of coronary artery fistulas at the time of diagnosis is recommended also in asymptomatic patients, since perioperative morbidity and mortality increases in older patients.

Assessment of congenital heart defects by dynamic three-dimensional echocardiography: methods of data acquisition and clinical potential.

To evaluate the use of three-dimensional (3D) echocardiography in the diagnosis of congenital heart defects, we studied 238 patients aged 3 days to 19 years (mean 4.3 years) with normal hearts (n=13) or a variety of congenital heart defects (n=225). Three different modalities of data acquisition suitable for 3D reconstruction were applied. For parallel scanning, the transducer is held in a 6-cm long scan frame and then moved over the thorax, or in the subcostal position, by a stepper motor using 0.5-mm steps with acquisition of perpendicular parallel images of the heart. For rotational scanning, the transducer is rotated at sectors of 2 degrees over a span of 180 degrees. For fan-like scanning, the transducer is moved in an arc 45 degrees each way from its vertical axis. Movement of the transducer is computer-controlled and performed with electrocardiographic and respiratory gating. Between 80 and 120 slices of the heart are thus obtained, which form the 3D dataset. This dataset can then be ¿sectioned¿ in any desired plane, thus permitting generation of views simulating intraoperative perspectives. Ventricular septal defects and atrioventricular valves can be displayed as viewed via the atrium. Muscular ventricular septal defects can be viewed as seen through a ventriculotomy. Obstruction in the left ventricular outflow tract can be viewed as via an aortotomy, and so on. We concluded that this new imaging modality has a vast potential and may facilitate planning of intracardiac surgery.

Left ventricular function assessed with echocardiography and myocardial perfusion assessed with scintigraphy under dipyridamole stress in pediatric patients after repair for anomalous origin of the left coronary artery from the pulmonary artery.

Twenty-three patients who underwent operation for anomalous origin of the left coronary artery from the pulmonary artery were reexamined with two-dimensional echocardiography and thallium 201 perfusion imaging. Follow-up studies were performed 0.6 to 16.2 years (median 2.9 years) after operation. In 22 of 23 patients, a two coronary artery system had been established by implantation of the left coronary artery into the aorta (n = 8) or by anastomosis of the left subclavian artery with the left coronary artery (n = 14). The left coronary artery had been ligated in only one patient. For stress testing, 0.8 mg dipyridamole per kilogram body weight was infused in a 10-minute period in 20 of the 23 patients. High-dose dipyridamole infusion increased mean heart rate (98.1 +/- 27.1 to 122.3 +/- 19.2 beats/min, p < 0.001) and mean left ventricular ejection fraction (54.8% +/- 11.8% to 61.3% +/- 12.5%, p < 0.05) and decreased left ventricular end-diastolic volume index (38.8 +/- 26.7 to 29.9 +/- 8.3 ml/m2, p < 0.005). At rest, left ventricular dimensions were abnormal in only one patient, in whom the anastomosis with the left coronary artery proved to be occluded, as seen with subsequent angiography. Left ventricular function seen with two-dimensional echocardiography was normal in 19 patients and was compromised in 3 (all of whom had major structural anomalies of the left ventricle, such as left ventricular aneurysm, occlusion of the anastomosis, or mitral valve prosthesis). Patients with R-wave loss as seen with preoperative electrocardiography tended to have larger left ventricular volumes at follow-up (69.2 +/- 56.5 ml/m2 versus 32.4 +/- 9.6 ml/m2, p < 0.07). Ten of 20 patients had normal thallium 201-perfusion scans. In 9 of 20 patients defects revealed by permanent thallium 201-perfusion were observed and determined to be myocardial scars. Transient perfusion defects under dipyridamole stress with redistribution at rest occurred in three children, two of whom also had permanent thallium 201 defects. None of the three patients had angina-like symptoms or S-T segment changes during dipyridamole stress. Left ventricular ejection fraction, however, decreased severely during dipyridamole infusion in the single patient with ligature of the left coronary artery. The two remaining patients had normal echocardiographic left ventricular function under stress, and the diagnosis of myocardial ischemia as seen with scintigraphy must be questioned.(ABSTRACT TRUNCATED AT 400 WORDS)

Risk factors for perioperative mortality in children with anomalous origin of the left coronary artery from the pulmonary artery.

The present study was conducted on 33 children (median age at initial cardiac catheterization 0.4 years [0.1 to 11.8]) with anomalous origin of the left coronary artery from the pulmonary artery, without associated hemodynamically significant cardiovascular anomalies, who were treated throughout a period of 18 years in our hospital. A two coronary artery circulation was reestablished in 31 of 33 children. One child died before the intended operation, and in one child the left coronary artery was ligated. There were six operative deaths, five intraoperative and one 12 hours after operation. The purpose of the study was to assess which preoperative clinical and angiographic features were associated with a higher perioperative mortality. The following preoperative factors were associated with a statistically significant higher perioperative mortality: young age at operation (p less than 0.03), left and balanced type of coronary circulation (p less than 0.01), and electrocardiographic signs of extensive acute myocardial infarction, namely, marked ST elevation (greater than or equal to 0.2 mV in at least two leads) (p less than 0.03). Left axis deviation on the electrocardiogram was associated with an extreme right dominant type of coronary circulation (p less than 0.005). The latter was also linked with adequate perfusion of the posterolateral left ventricular wall (p less than 0.005). At autopsy, severe increase of heart weight to two or three times the normal heart weight was established in six of seven children. Thus the perioperative mortality was determined primarily by the extent of myocardial ischemia. This in turn is decisively influenced by the dominant type of coronary circulation and the extent of inter-arterial collateralization. Young age, in addition, proved to be a risk factor for mortality at corrective surgery.

The influence of pulmonary artery banding on outcome after the Fontan operation.

Thirty-eight patients were selected from a total of 120 patients who underwent the Fontan operation between 1974 and 1988. They were classified into two groups. Group 1 consisted of 18 patients with previous pulmonary artery banding at a mean age of 7 months (2 days to 59 months), and group 2 comprised 20 patients with native pulmonary stenosis. In group 1, 10 children had tricuspid atresia (seven with normally connected and three with transposed great arteries), six had double-inlet ventricle, and two had complex heart malformations. Group 2 consisted of 12 patients with tricuspid atresia and normally connected great arteries, six with double-inlet ventricle, and two with complex malformations. The following clinical and hemodynamic parameters at cardiac catheterization and cineangiocardiography were determined in both groups before the Fontan operation: age and body surface area, hemoglobin concentration and hematocrit value, atrial and pulmonary artery pressures, end-diastolic pressure of the systemic ventricle, arterial oxygen saturation, pulmonary/systemic flow ratio, end-diastolic volume, ejection fraction and mass of the systemic ventricle, cardiac index, and Nakata index. After the Fontan operation in all patients, the presence or absence of pericardial and pleural effusions, ascites, protein-losing enteropathy, and liver and kidney dysfunction was assessed and the clinical status was classified according to New York Heart Association criteria. All preoperative and postoperative parameters were tested for differences between the two groups, and they were compared with normal values. Hematocrit value was higher in group 2 than in group 1 (57.8% versus 53.1%; p less than 0.05). Ventricular mass index was increased in group 1 when compared with group 2 (125.8 gm/m2 versus 87 gm/m2; p less than 0.05). Severe pericardial effusions in the early postoperative period were significantly more frequent in group 1 and were particularly prevalent in the subgroup with long-standing pulmonary artery banding (p less than 0.01). Subaortic stenosis was observed more frequently in group 1. The remaining parameters were not statistically different between the two groups. We conclude that the significant increment in ventricular mass after pulmonary artery banding may represent a risk for unfavorable outcome after the Fontan operation, which increases with time. Therefore, long-standing pulmonary artery banding as a palliative procedure for candidates for the Fontan operation should be avoided.

Influence of age at time of surgery on pre-operative left ventricular mass and postoperative outcome of Fontan operation in children with tricuspid atresia and native pulmonary stenosis.

Purpose of this study was to examine the influence of early (less than two and half years) versus later (greater than four years) age at time of Fontan type palliation in tricuspid atresia with native pulmonary stenosis on outcome with special reference to left ventricular mass and function. Among the 21 patients with tricuspid atresia, twelve (group A) underwent a Fontan type palliation at a median age of one (.6 to 2.5) years and nine (group B) at a median age of 7.5 (4.8 to 28) years. Left ventricular mass was assessed by cross-sectional echocardiography in the apical two and four chamber view. Mass was calculated as difference between epicardial and endocardial volume x 1.05 (specific gravity of heart muscle). Mass divided by volume at end-diastole yielded the mass/volume index. There was a weak correlation between age and left ventricular mass with an r-value of 0.74. Ejection fraction was calculated from the endocardial volume measurements at end-diastole and end-systole. Patient data were compared to normal values previously established in 95 controls, who were age-matched for the patients. Immediately before surgery left ventricular mass was significantly higher in the nine patients, who underwent surgery at a later age. While postoperative stay in hospital and duration of treatment in the intensive care unit did not differ significantly between both groups, the incidence of pleural and pericardial effusions and the duration of insertion of drainage tubes for these effusions differed significantly with the group A patients (under two and half years of age) doing better.(ABSTRACT TRUNCATED AT 250 WORDS)

[Long-term results of Mustard operation in transposition of the great arteries. Angiographic and nuclear medicine study of ventricular function]. / Langzeitergebnisse nach Mustard-Operation bei Transposition der grossen Arterien. Angiographische und nuklearmedizinische Untersuchung der Ventrikelfunktion.

The fate of the right ventricle as systemic ventricle after atrial repair of complete transposition of the great arteries has not been clearly elucidated. In order to assess the long-term results of the Mustard operation in patients with complete transposition of the great arteries we present the clinical data of 23 patients who had been operated in the years 1974 and 1975. Twenty of these patients had simple complete transposition of the great arteries with intact ventricular septum, two had an additional small ventricular septal defect and one an additional left ventricular outflow tract obstruction with a 40 mm Hg systolic pressure gradient. The Mustard operation had been performed at a mean age of 2.2 (1 to 3.7) years. Seventeen of the 23 patients underwent a postoperative hemodynamic study with angiocardiography 1.1 (1 to 1.8) years following surgery. At that time the right ventricular ejection fraction, which had been calculated from biplane angiographic right ventricular volume measurements in twelve patients was 62 (52 to 68) %. However the right ventricle was dilated and the mean enddiastolic volume was 132 (108 to 192) % of normal. In twelve of the 23 patients right ventricular function was reassessed 12.6 (11 to 15.3) years after surgery by Technetium-99m-scintigraphy at rest and in ten of those after exercise with a workload of 2 watt/kg. The mean ejection fraction was 51 (38 to 66) % at rest and 52 (40 to 80) % during exercise. Only three patients had a normal response to exercise, which was defined as an increase of ejection fraction with exercise of more than 5%.(ABSTRACT TRUNCATED AT 250 WORDS)

Left ventricular function and myocardial mass after aortic valvotomy in infancy.

Eighteen of 25 survivors of aortic valvotomy in infancy were reinvestigated by cross-sectional echocardiography a mean of 7.5 (2.3-13.4) years after surgery. They had been operated at a median age of 38 (5-330) days. At the follow-up examination the gradient across the aortic valve was 41 +/- 19 (15-85) mmHg and the ejection fraction was 0.73 +/- 0.10 (0.48-0.84). Left ventricular (LV) end-diastolic volume was 66 +/- 17 (33-191) ml/m2. LV mass was 96 +/- 36 (44-204) g/m2 and the LV mass volume index (LVMVI) (mass divided by end-diastolic volume) was 1.43 +/- 0.4 (0.9-2.28). Eleven of 18 patients had an abnormally high mass volume index compared with 95 age-matched controls with structurally normal hearts. The correlation between the residual pressure gradient across the aortic valve and mass volume index yielded an r value of 0.75 (p less than 0.0004). One patient had been reoperated and underwent resection of a subaortic stenosis 4 years after the initial operation. Four patients with a resting gradient of more than 50 mmHg and one with grade 4 aortic regurgitation are scheduled for further surgical treatment. We conclude that, although LV function was normal in most patients who underwent aortic valvotomy in infancy, LV mass remains elevated in a significant number of patients, who may remain at risk of developing subendocardial ischemia.

Brock transventricular pulmonary valvotomy in patients with pulmonary stenosis: long-term results.

As no long-term results of a blind opening up of the pulmonary valve either by balloon valvoplasty or closed Brock valvotomy have been published, we examined the outcome of 12 patients with valvar pulmonary stenosis 17 +/- 5 (11-22) years after surgery. The Brock valvotomy had been carried out at a mean age of 3 +/- 2.8 (0.2-8.5) years. The mean pressure gradient across the right ventricular outflow tract had been 116 +/- 45 (75-97) mmHg at the catheter study or 106 +/- 43 (40-160) mmHg as measured intraoperatively. The right ventricular pressure after the Brock procedure was measured in the operating room in five patients as 46 +/- 15 (30-60) mmHg. Seven patients had been recatheterized at a mean age of 9.5 +/- 2 (7-12.7) years; at that time the gradient across the pulmonary valve had been 20 +/- 14 (10-37) mmHg. At a mean age of 21.7 +/- 3 (15-26) years these and five further patients were reexamined by echo Doppler. This time the pressure gradient across the pulmonary valve was 13 +/- 6 (7-20) mmHg. Moderate pulmonary incompetence was present in four and mild incompetence in eight patients; two had mild tricuspid insufficiency. All except one patient, who had suffered a cerebrovascular accident before surgery, were in NYHA functional class 1 and pursuing a profession. From these data we conclude that the blind opening-up of the pulmonary valve achieves excellent long-term palliation.

[The accuracy of Doppler echocardiography determination of shunt volume in children with isolated atrial septal defect and left-right shunt]. / Genauigkeit dopplerechokardiographischer Shuntgrössenbestimmung bei Kindern mit isoliertem Vorhofseptumdefekt und Links-rechts-Shunt.

The accuracy of shunt calculations derived from Doppler echocardiography was assessed in 15 children (5-15 years, mean 8.5 yrs.) with secundum-type atrial septal defect and left-to-right (L-R) shunt. Qp/Qs was calculated by measuring stroke volumes over the aorta and pulmonary artery prior to and 4-15 days after corrective surgery. The measuring error of the Doppler method was defined as deviation of the postoperative shunt value from zero. A residual shunt was excluded by careful intraoperative testing and physical examination. Intraoperative inspection revealed normal pulmonary venous drainage. L-R-shunt, as calculated by Doppler echo, significantly decreased after surgery (p less than 0.001). Although all septal defects were closed, calculations by Doppler echo resulted in a median residual shunt of 16% (0-50.7%). IN 2/15 children a L-R shunt of more than 40% was derived by echo recordings. The size of the measuring error increased in children with great preoperative shunts. There was a significant correlation between the ratio of pulmonary to aortic cross-sectional area and the measuring error (p less than 0.03, r = 0.59). Poor estimation of cross-sectional areas in dilated pulmonary arteries was the most important limiting factor in shunt calculation. Doppler echocardiography provides a valuable noninvasive method for shunt calculation. Similarly to Fick's principle, in some patients the measuring error may be considerable and become relevant for clinical decisions. Thus Doppler-derived shunt calculations must be complemented by additional information of two-dimensional echo and physical examination.

Coronary arteries in the hypoplastic left heart syndrome. Histopathologic and histometrical studies and implications for surgery.

The subepicardial coronary arteries (CAs) in the hypoplastic left heart syndrome (HLHS) were studied for pathology dependent on the patency of the mitral valve and the presence of endocardial fibroelastosis (EFE) of the left ventricle (LV). Nine heart specimens with aortic and mitral atresia (group 1) were studied and compared with 19 hearts with aortic atresia and mitral stenosis (group 2) (EFE of the LV was present in all cases) and 10 normal hearts. The median age at death was 3 days. For the histological and histometrical studies, 5-mm blocks from six comparable sites of the left (L) and right (R) CAs were serially sectioned and routinely processed. The arterial size and wall thickness were measured. Macroscopically, the CAs in group 1 were normal except for the prevalence of left dominance (56%). In contrast in group 2, the CAs were thicker and tortuous. Histopathology was restricted almost exclusively to group 2 specimens (11 of 19), with the proximal LCAs being the most affected. However, eight hearts with EFE in group 2 had no structural wall changes of the CAs. Histometrically, the sizes (external diameters) of the LCAs and RCAs were similar (0.5-1.0 mm) in groups 1 and 2 and in the normal group; however, we noted an increase in percent medial thickness in group 2 specimens, especially of the LCAs. Furthermore, in some group 2 hearts, the wall thickness (intima and media) relative to the luminal size (internal radius) of proximal and middle LCAs was significantly increased, suggesting the presence of ventricle-CA communications.(ABSTRACT TRUNCATED AT 250 WORDS)

Atrioventricular septal defect complicated by right ventricular outflow tract obstruction. Analysis of risk factors regarding surgical repair.

Eighteen patients with complete atrioventricular septal defect (AVSD) were found to have either associated tetralogy (n = 15) or pulmonary atresia (n = 3). Their pre- and post-operative course was reviewed focusing attention on risk factors for complete repair. These included right ventricular (RV) hypoplasia, identified by cineangiographic volume determinations in two cases (11%) and inadequate pulmonary artery size in one patient. In the 2 cases with RV hypoplasia and in one case, in whom all chordae of the AV valve were connected to a single right ventricular papillary muscle (AVSD type Rastelli B), complete repair was not feasible. In addition, angiography revealed coronary artery variations in 5/18 (28%), which did not preclude surgical repair. Palliative surgery was undertaken in 10 patients and complete repair carried out in five. Six of 18 patients died, 2 among those after complete repair.

Competition of coronary arteries and ventriculo-coronary arterial communications in pulmonary atresia with intact ventricular septum.

Pulmonary atresia with intact ventricular septum can be complicated by the presence of large ventriculo-coronary arterial communications which disturb normal myocardial perfusion. In the selection of patients for surgery the presence of these communications provides an additional problem. On the basis of previous cineangiocardiographic study, we performed a histopathological study of 16 cases. Twelve presented with ventriculo-coronary arterial communications and four only showed myocardial sinusoids. A tripartite right ventricle was seen in the latter group but not exclusively. It is shown that subepicardial coronary arterial pathology is exclusively present in cases with ventriculo-coronary arterial communications although not solely at a connection site between a communication and an artery. The affected coronary artery is itself focally abnormal and hypoplastic and can be blocked or even absent. The interruption of such an artery can also occur after birth and not necessarily at a site of connection with a communication. This implies that infants with communications either already have a coronary circulation partly or completely dependent on ventriculo-coronary arterial communications at birth or can develop such a condition in time. This hazard to myocardial perfusion, often compromised by a large "steal" from the aorta to the right ventricle, limits the choice of surgical procedures.

[Clonidine therapy of "paradoxical hypertension" following surgery of isolated stenosis of the aortic isthmus in childhood]. / Clonidin-Therapie der "paradoxen Hypertension" nach Operation der isolierten Aortenisthmusstenose im Kindesalter.

In the period from 1974 to 1983, altogether 234 children underwent surgery of isolated coarctation of the aorta at the Heart Center Munich. In 146 of these patients, a reactive increase of blood pressure has been noted following surgery, and in about half of them the blood pressure was considerably elevated above the 95-percentile. Since the success of surgery may be endangered by a critical increase of blood pressure, in 66 patients an antihypertensive therapy with clonidine has been started immediately after operation. By this treatment the intended decrease of blood pressure has been effected within the first two days after surgery, and in 91% of the patients the therapy could be stopped within the first two postoperative weeks. In 6 out of these 66 patients, the "paradoxical hypertension" persisted so that the clonidine therapy had to be continued after discharge from hospital. The empirically determined dosage and the duration of treatment have been modified by the clinical requirements, i.e. by the actual level of the blood pressure. The average duration of drug administration has been lengthened according to the advanced age at time of surgery, while the required clonidine dosage simultaneously showed a tendency to decrease. Since no side-effects have been registered in the course of this procedure, the use of clonidine suggests a promising therapeutical approach to "paradoxical hypertension".

Non-invasive oscillometric measurement of systolic, mean and diastolic blood pressure in infants with congenital heart defects after operation. A comparison with direct blood pressure measurements.

The accuracy of indirect oscillometric blood pressure measurements has been evaluated in 32 infants up to 11 months of age undergoing heart surgery. In a number of 1029 simultaneous measurements the indirect blood pressure was compared with the direct value obtained from a radial artery catheter. Cuffs of different sizes were applied. The main results were as follows: Regarding the ratio of cuff width to arm circumference, the best correlation between oscillometric and direct blood pressure measurements was obtained with a ratio of 0.38-0.41. The value of indirect blood pressure measurements depends on the absolute height of the blood pressure. In low blood pressure there is a tendency to underestimate and in high blood pressure there is a tendency to overestimate by the oscillometric blood pressure measurement. By applying an appropriate cuff size and by using our diagrams it should be possible to derive a direct value for the blood pressure on the basis of indirect oscillometric blood pressure measurements.

[Clinical uses of digital subtraction angiography for imaging anatomy immediately following heart operations in childhood]. / Klinische Anwendungen der digitalen Subtraktionsangiographie zur Darstellung der Anatomie unmittelbar nach Herzoperationen im Kindesalter.

Within the last three years, digital subtraction angiography (DSA) was performed 60 times in 58 children with congenital heart disease (coarctation of the aorta, tetralogy of Fallot or transposition of the great vessels) in the immediate post-operative period to delineate residual defects. The DSA was carried out 3.7 +/- 1 days postoperatively with a total of 3.8 +/- 2.5 ml (0.47 +/- 0.2 ml/kg) Conray 70 or Solutrast 300 injected by hand into a catheter which generally had been positioned preoperatively at the transition between superior vena cava and right atrium. The outflow of the contrast medium was recorded on video tape and, additionally, single images of important anatomical details were obtained. In six patients with resected aortic coarctation, the patency of the entire aortic arch was documented with DSA; these patients had undergone surgery at a mean age of 3.3 +/- 4.8 years. Seven patients, with a mean age of 2.5 +/- 4.7 years, had tetralogy of Fallot, three additionally with pulmonary valve atresia; in all, postoperatively, the entire right ventricular outflow tract and the large pulmonary vessels could be demonstrated. In 45 patients with transposition of the great vessels, mean age 4.5 +/- 3.1 months, an atrial inversion with the Senning procedure was carried out; postoperatively, in all patients, the confluence of the superior and inferior vena cavae into the systemic venous portion of the atrium could be delineated. The pulmonary veins were visualized in one-third of the patients. In eight patients, DSA demonstrated obstruction at the opening of the superior vena cava into the atrium together with the collateral circulation via the azygos vein and the inferior vena cava.(ABSTRACT TRUNCATED AT 250 WORDS)

[Isolated aortic isthmus stenosis: operation indications and results]. / Isolierte Aortenisthmusstenose: Operationsindikation und Ergebnisse.

The goal of surgical correction of isolated coarctation of the aorta is normalization of the blood pressure. To assess the optimal age for the intervention with respect to operative morbidity and mortality, residual stenosis and recoarctation rate as well as the postoperative persistence of arterial hypertension without recoarctation, the pre- and postoperative findings of 237 patients (age at surgery: range one week to 17 years) were analyzed. The blood pressure, which was above the 95th percentile of age-matched normal values preoperatively in 93%, showed a clear decrease postoperatively in all age groups. On surgical correction, however, performed as of six years of age, there was a continuously increasing rate of persistent arterial hypertension without recoarctation which ranged from about 10% in those undergoing surgery between the ages of six and eight years to 31% in patients operated at ages between 13 and 17 years. In consideration of the operative results only with respect to the response of the resting blood pressure, insight is enabled only into one aspect of the prognosis, since the latter is equally dependent on the postoperative systemic arterial resistance. The systemic arterial resistance is already slightly elevated at three to five years of age, significantly increased as of six years of age, and at surgery after the age of six, shows a further increasing tendency. Accordingly, patients with postoperative normal or borderline-elevated blood pressure and apparently adequate surgical results, may be at risk of subsequent development of hypertension due to persistence of increased resistance, in particular, if surgery is performed after the age of five years.(ABSTRACT TRUNCATED AT 250 WORDS)

The treatment of tetralogy of Fallot: early repair or palliation?

Between 1974 and 1983, 380 consecutive patients with tetralogy of Fallot underwent surgery. In 73 patients, undergoing palliative surgery, the operative mortality and late mortality was 6.8%. Twenty-five of these patients have undergone subsequent complete repair, while 38 are awaiting correction. During the last 4 years we preferred operative procedures with enlargement of the right ventricular outflow tract (80%) to shunt operations (20%). In contrast to shunts, enlargement of the outflow tract induces a symmetrical growth of hypoplastic pulmonary arteries without the risk of acquired pulmonary atresia or peripheral stenosis at the site of anastomosis. Three hundred twenty-nine patients have undergone repair. In 101 patients, who had palliative operations before complete repair, the operative mortality was 12.9% and late mortality 2.9%. Those patients undergoing repair as a first operation had an operative mortality of 8.8%, and a late mortality of 1.3%. In the last 5 years the operative mortality for all patients, whether or not they had had previous palliative surgery, was 4.7%. The incidence of transannular outflow tract patching was not greater in children less than 2 years of age (16.9%) than in older children, 2 to 14 years of age (16.5%). However, in our experience the requirement for subvalvular outflow patches was higher in younger children (52.1%) than in the older children (34.1%). Because of this high incidence, particularly in younger patients, we have now begun to repair tetralogy of Fallot using the transatrial approach, thus reducing our use of subvalvular patches.(ABSTRACT TRUNCATED AT 250 WORDS)

Tetralogy of Fallot. Development of hypoplastic pulmonary arteries after palliation.

Thirty-one patients with tetralogy of Fallot were studied angiographically before and after palliation with Blalock-Taussig operation (n = 9), Brock operation (n = 12), or enlargement of the right ventricular outflow tract with extracorporeal circulation (ECC) (n = 10). The relative diameter of the pulmonary vascular ring (PVR), pulmonary trunk (PT), and right and left pulmonary arteries (RPA, LPA) were measured before and 24.8 +/- 20.9 months after palliation. Using correlation analysis, the magnitude of growth of the pulmonary artery system was inversely related to its initial size. Mean pressure in the pulmonary artery after palliation as well as the period between date of surgery and recatheterization did not correlate significantly with growth. After Blalock-Taussig operation, the ipsilateral pulmonary artery predominantly increased in size without significant growth of PVR and PT. Brock operation and enlargement of the right ventricular outflow tract with ECC, improving the flow in the pulmonary vascular system centrally, induced a significant, symmetrical growth of the pulmonary vascular system and, therefore, are our methods of choice for palliation in patients with tetralogy of Fallow combined with hypoplastic pulmonary arteries.