Anatomic and functional "obstruction" of the outflow tract in atrioventricular septal defects with separate valve orifices ("ostium primum atrial septal defect"): an echocardiographic study.

Left ventricular (LV) outflow tract (OT) obstruction can be treacherous in any form of atrioventricular (AV) septal defect. The properties of the LVOT were investigated echocardiographically in 64 patients with separate valve orifices ("ostium primum atrial septal defect") who had survived corrective surgery. M-mode and cross-sectional echocardiographic (echo) images were made of the LVOT. The degree of malalignment of the aorta with the ventricular septum, the left atrium-aortic ratio, the fractional LV shortening and the diameter of the LVOT were recorded. Fixed anatomical obstruction was found in 3 patients, consisting of muscular bands or abnormal attachment of tension apparatus. Malalignment of the aorta with the ventricular septum was found in 62% of the patients. The diameter of the LVOT was smaller than that of the aortic root in 71% of the cases. The mean diameter of the LVOT was 92 +/- 27% (range 35 to 143%) of the aortic root diameter. Because its walls are mainly muscular, the LVOT constricts during systole. The mean end-systolic diameter of the LVOT was 77 +/- 22% (range 23 to 129%) of the aortic root diameter. Sequential measurements showed that the LVOT constricted gradually, but the velocity of constriction in patients with the most severe narrowing showed a distinct maximum in the first fifth of systole. In conclusion, a series of elements contribute to a potentially perilous arrangement of the LVOT in patients with AV septal defect. This intrinsically narrow tunnel was constricted during systole by its muscular walls.(ABSTRACT TRUNCATED AT 250 WORDS)

Left atrioventricular valve after surgical repair in atrioventricular septal defect with separate valve orifices ("ostium primum atrial septal defect"): an echo-Doppler study.

Left atrioventricular (AV) valve dysfunction is the most frequent major postoperative hemodynamic complication in patients with AV septal defect. The anatomy and function of the left AV valve were investigated in 64 patients with separate valve orifices (ostium primum atrial septal defect) who had survived corrective surgery. M-mode and cross-sectional echocardiograms of the left AV valve were obtained. Doppler flow tracings were obtained at the left AV valve orifice to determine if regurgitation was present. The findings were related to the position of the commissures between the leaflets, the size of the 3 leaflets and the position of the papillary muscles. Left AV valve regurgitation was present in 29 of 51 patients (57%). These patients had a significantly different left AV valve leaflet configuration, characterized by a large mural leaflet and a small inferior bridging leaflet. The size of the superior bridging leaflet is not a determinant factor. Thus, the configuration of the left AV valve in AV septal defect is related to the postoperative functional result. Awareness of the echocardiographic anatomy may influence the surgical approach to this defect.

Growth of the hypoplastic aortic arch after simple coarctation resection and end-to-end anastomosis.

Surgical treatment of a hypoplastic aortic arch associated with an aortic coarctation is controversial. The controversy concerns the claimed need to surgically enlarge the diameter of the hypoplastic arch, in addition to resection and end-to-end anastomosis. The purpose of this prospective study is to determine the fate of the hypoplastic aortic arch after resection of the aortic coarctation and end-to-end anastomosis. Between July 1, 1988, and January 1, 1990, 15 consecutive infants less than 3 months of age with an aortic coarctation were evaluated echocardiographically. A Z-value was calculated, being the number of standard deviations the aortic arch differs from the expected value, derived from a control group. Eight of these 15 infants had a hypoplastic aortic arch with a mean Z-value of -7.14 +/- 1.39. The other seven infants had a "normal" aortic arch with a mean Z-value of -1.85 +/- 1.08. All 15 infants underwent simple coarctation resection and end-to-end anastomosis. Six months after operation the mean Z-value increased significantly in those with a hypoplastic arch to -1.08 +/- 0.69 (p less than 0.0001) and in those with a "normal" aortic arch to 0.106 +/- 0.99 (p = 0.004). No infant died in our series (0%; CL 0% to 12%) and a recoarctation developed once (12.5%; CL 2% to 36%). Therefore we believe that simple resection and end-to-end anastomosis is the operation of choice for aortic coarctation associated with a hypoplastic aortic arch despite the presence of a ventricular septal defect and that enlargement of the hypoplastic aortic arch is not necessary.

Influence of age on survival, late hypertension, and recoarctation in elective aortic coarctation repair. Including long-term results after elective aortic coarctation repair with a follow-up from 25 to 44 years.

The optimal age for elective repair of aortic coarctation is controversial. The optimal age should be associated with a minimal risk of recoarctation, late hypertension, and other cardiovascular disorders. The purpose of this retrospective study is to determine the actuarial survival after aortic coarctation repair 25 years or more after operation and to calculate the optimal age for elective aortic coarctation repair. From 1948 to 1966, 120 consecutive patients underwent aortic coarctation repair. Eighty-seven were male (72.5%). The mean age at operation was 15.5 years (SD +/- 9.1 years). Resection and end-to-end anastomosis was performed in 103 patients (85.8%). Early mortality occurred in 6 patients as a result of surgical problems, whereas late mortality in 15 patients was predominantly caused by cardiac causes. The mean follow-up period was 32 years (range 25 to 44.2 years). Ninety-two patients 96.8%) were in New York Heart Association class I. The probability of survival 44 years after operation was 73%. Patients younger than 10 years at operation had the highest probability of survival at 97%. Multivariate analysis produced age at operation as the only incremental risk factor for the occurrence of recoarctation, of late hypertension, and of premature death. So that these sequelae can be avoided, elective aortic coarctation repair should be performed around 1.5 years of age. At that age, the probability of recoarctation will have decreased to less than 3%, and the probability of upper body normotension and long-term survival will be optimal.

Repair of aortic coarctation in infants.

Fifty-three consecutive infants younger than 2 years underwent coarctation repair. A recoarctation occurred in 11 infants (21%). To determine variables associated with recoarctation, we entered preoperative and operative data into a multivariate stepwise logistic regression analysis. Patient weight was an incremental risk factor for recoarctation instead of age, in contrast to previously published studies. Furthermore, the residual gradient after the operation was a strong incremental risk factor. This risk factor was even more significant when expressed as a ratio of the systolic arm pressure, which takes background hemodynamics into account. Because weight is a more significant risk factor than age, we conclude that deferring operation is indicated only when the infant gains weight. Furthermore, a residual gradient is more important in the hemodynamic setting of a lower systolic arm pressure.

Tissue-type plasminogen activator and fibrin monomers synergistically cause platelet dysfunction during retransfusion of shed blood after cardiopulmonary bypass.

Reduced hemostasis and bleeding tendency after cardiopulmonary bypass results from platelet dysfunction induced by the bypass procedure. The causes of this acquired platelet dysfunction are still subject to discussion, although, recently, greater emphasis has been placed on an overstimulated fibrinolytic system as a probable cause. In the first part of this study we assessed the effects of postoperative retransfusion of shed blood on blood loss to patients undergoing cardiopulmonary bypass. We observed that increasing concentrations of fibrinogen degradation products and tissue-type plasminogen activator stimulating activity in shed blood correlated significantly with a higher postoperative bleeding tendency (p < 0.05 for both). We further noted that retransfusion of shed blood increased the total postoperative blood loss by 43% (925 versus 1320 ml, p < 0.05). On the basis of these clinical observations, we hypothesized that the increased bleeding tendency was caused by fibrinolysis. In the second part of this study we collected evidence in support of this hypothesis by an in vitro study, in which we introduced similar (pro)fibrinolytic activity to platelet-rich plasma and measured the influence of this treatment on platelet function indicated by ristocetin agglutination. Tissue-type plasminogen activator and fibrin monomers (tissue-type plasminogen activator stimulator) together induced severe platelet damage, resulting in a decreased ristocetin agglutination response. Therefore, we propose a fibrinolysis-related mechanism for platelet dysfunction during cardiopulmonary bypass, dependent on fibrinolytic factors such as fibrin monomers, D-dimers, and tissue-type plasminogen activator.

Decision making for the surgical management of aortic coarctation associated with ventricular septal defect.

Coarctation of the aorta and associated ventricular septal defect may be repaired simultaneously or by initial coarctation repair with or without banding of the pulmonary artery. The question is whether specific preoperative criteria can enable the surgeon to choose the optimal surgical management. Between 1980 and 1993, 80 infants younger than 3 months with coarctation and ventricular septal defect were treated surgically. In 64 infants (multistage group), simple coarctation repair was performed through a posterolateral approach, with concomitant banding of the pulmonary artery in 10 infants. Twenty ventricular septal defects were closed as a secondary procedure and four were closed as a tertiary procedure. Sixteen infants (single-stage group) underwent one-stage repair through an anterior midline approach. The total in-hospital mortality rate was 7.5%. Freedom from recoarctation after 5 years was 91.3% in the multistage group versus 60.0% in the single-stage group (p = 0.018). Freedom from secondary ventricular septal defect treatment in the multistage group after 5 years was 40.7%, versus 100% in the single-stage group (p = 0.016). Thirty-seven ventricular septal defects (47.8%) closed spontaneously. In particular, the preoperative left-to-right shunt and extension of the perimembranous VSD into the inlet or outlet were risk factors for the need for eventual surgical ventricular septal defect closure after initial coarctation repair. On the basis of these two risk factors, the probability of the need for eventual surgical treatment of ventricular septal defect after initial coarctation repair can be calculated. This policy offers a well-considered choice between single-stage and multistage repair, weighing the risk of secondary ventricular septal defect treatment versus the risk of recoarctation. Finally, the number of surgical procedures per infant will be as low as possible.

Porcine-valved Dacron conduits in Fontan procedures.

From a series of 52 Fontan procedures between 1976 and 1984, the cases of the 27 consecutive patients who received a porcine-valved conduit were reviewed. There were 5 hospital deaths among these 27 patients. Follow-up ranges from 11 years 9 months to 3 years 9 months. At follow-up, no conduit-related complications could be demonstrated. There were no signs of valvular stenosis, exuberant peel formation, or calcification of the conduit in any of the patients. To date, there has been no need to replace any of the porcine-valved conduits. Cumulative survival (including hospital deaths) is 71% at 10 years. In conclusion, we believe that the porcine-valved conduits have functioned very satisfactorily over time.

The surgical anatomy of the left ventricular outflow tract in atrioventricular septal defect.

The left ventricular (LV) outflow tract (OT) in atrioventricular (AV) septal defect is an important structure that paradoxically is hardly ever seen by a surgeon. The LVOT is prone to develop obstruction following surgical procedures, such as left AV valve replacement, that seemingly do not affect the LVOT itself. We examined 15 hearts with AV septal defects and noted the anatomical boundaries of the LVOT. Additionally, the LVOT was examined microscopically, and it was sectioned to replicate echocardiographic images. A sham operation was performed to show the extent of the proposed resection for AV valve replacement. The mean length of this area was 91.8 +/- 35.5% (range, 28.6 to 167.0%) of the diameter of the ascending aorta in our specimens of the Rastelli A variety. The mean diameter of the LVOT was 68.2 +/- 13.5% (range, 42.9 to 100.0%) of the diameter of the ascending aorta. The posterior wall of the OT can either be resected or widened. Resection seems to be opportune at AV valve replacement, whereas widening could be performed when the OT is intrinsically stenotic. When one fully appreciates the concept of a five-leaflet common valve, it is clear that the length of the OT depends on the extent of adherence between the superior bridging leaflet and the septal crest. In hearts that have two separate AV valve orifices, the OT is fully developed; there is no potential for interventricular shunting ("ostium primum defect"), because the superior bridging leaflet is always tightly adherent to the septal crest. AV valve replacement in these cases is especially hazardous.(ABSTRACT TRUNCATED AT 250 WORDS)

Intra-arterial digital subtraction angiography (DSA) as an outpatient screening method for the follow-up of graft patency in coronary surgery.

In order to determine whether intra-arterial digital subtraction angiography (= DSA) is a suitable screening method to visualise graft patency and to determine whether DSA can be performed on an outpatient basis, we studied 73 patients shortly after coronary bypass surgery. In two patients DSA was precluded due to technical problems. The patency of the grafts was adequately visualised by means of DSA in 68 of the remaining 71 patients. During the DSA procedure complications occurred in two patients without serious consequences and bleeding at the puncture site was not observed in any patient whether mobilised at 24 or only 2 hours. We conclude that intra-arterial DSA is a suitable screening method to visualise patency in coronary bypass grafts and that it can be performed safely on an outpatient basis.

Mitral valve replacement in infancy: haemodynamic factors.

Severe mitral valve regurgitation necessitated the insertion of a prosthetic valve (Björk-Shiley no. 21) in a girl 10 months of age. Control studies after the baby had doubled her body weight showed normal haemodynamic data. It is estimated that a valve of this size should allow the expected cardiac output for a patient of 9-12 years of age. Thrombosis and tissue overgrowth may result in "recurrent" mitral stenosis long before this date. Repeated haemodynamic studies at intervals of 2-3 years are therefore indicated, since clinical signs fail to show recurrent valve stenosis; should the latter develop there is the risk of irreversible pulmonary vascular resistance.

[Chondrosarcoma of the bony thoracic wall]. / Chondrosarcoom van de benige thoraxwand.

An 85-year-old man was admitted because of a slowly growing mass in his left flank, which had been present for 16 years. A very large chondrosarcoma was diagnosed, weighing 8.5 kg. After radical resection of the tumour the patient recovered well. No recurrences developed during follow-up.

[Use of the right gastroepiploic artery graft in revascularization of the myocardium]. / Vyuzití stepu pravé gastroepiploické artérie pri revaskularizaci myokardu.

The right gastroepiploic artery (rGEA) was used as a graft for revascularization of the heart muscle in 239 patients along with other arterial grafts obtained from the internal mammary arteries (IMA). The mean follow-up of patients after operation was 17 months, the longest period being 38 months with a total follow-up period of 4,063 months. The authors recorded a high percentage of patency of the graft during check-up catheterization after a 6-month interval (94.7%), a low incidence of early complications of minor importance (2.5%), a low percentage of anginose complaints (7.9%) and reoperations (5%), while the lethality was not higher than in the control group (2.51%). The use of a graft from the rGEA is very suited for surgical treatment of IHD, in particular in patients of younger age groups and in reoperations. The authors discuss contraindications, the majority being only relative contraindications. The authors deal also with the preparatory surgical technique and the use of the graft.