Incremental risk factors in hospital mortality rate after repair of ventricular septal defect.
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Biomedical subjects
Publications and source records attributed to L M Bargeron.
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Many congenital heart conditions that were previously considered to be inoperable are now able to be corrected surgically. As a result, a more accurate and refined "surgical diagnosis" than that offered by conventional angiography has been sought. Axial cineangiography is invaluable in establishing a specific diagnosis in such cases.
Recent advances in techniques of angiocardiography now allow highly accurate assessment of the anatomy of the left ventricle in patients with transposition of the great arteries. The cineangiograms of 225 children with transposition were reviewed using axial cineangiographic techniques and 33 percent were found to have significant left ventricular outflow tract obstruction with or without coexisting ventricular septal defects. The varieties of left ventricular outflow tract obstruction and the associated ventricular septal defects found in this group of patients are described, illustrated and correlated. The advantages of axial angiocardiography in the diagnosis of transposition are pointed out, and the techniques used to produce the axial angiocardiograms are reviewed.
Actuarial analysis based on postmortem examination of patients who had been treated nonsurgically for complete atrioventricular (A-V) canal defect shows that only 54% survive to 6 months of age, 35% to 12 months, 15% to 24 months, and 4% to 5 years of age. Our surgical experience since 1975 in 39 patients confirms the idea that primary repair is feasible in small infants. The highest risk of hospital death is when the operation is done in the early months of life; it falls to 17% by age 12 months. Between 1967 and October, 1976, the five-year survival rate among patients leaving the hospital alive after repair was 91%. The age-specific probability of "surgical cure" of patients operated upon for complete A-V canal (alive five years later with mean pulmonary artery pressure less than 25 mm Hg) is maximal at 73% when the operation is done at about 14 months of age. Urgent earlier repair is frequently necessitated by the life history of the disease.
Between January, 1967, and October, 1977, we performed intracardiac repair in 24 patients with unroofed coronary sinus. Eight patients had the so-called pure form of the syndrome, with the concomitant findings of total absence of the partition between the coronary sinus and left atrium, connection of the left superior vena cava (LSVC) to the upper left corner of the left atrium, and a coronary sinus type of atrial septal defect. In 2 additional patients this combination was repaired, along with repair of the common atrium in 1 and tetralogy of Fallot in the other. In 4 patients with either a partial or complete atrioventricular canal defect, only the distal or downstream portion of the sinus was unroofed so that the coronary sinus ostium was considerably to the left of its usual location. In 6 patients, 5 of whom had situs ambiguous, the unroofed coronary sinus was associated with complex congenital heart disease, and in 5 there was a L(contralateral) SVC. Three of these 6 patients died. The basic repair consists of roofing the coronary sinus from within the left atrium, so that the LSVC drains through the newly created coronary sinus and its ostium into the right atrium. In the absence of a LSVC, the basic repair is simply closure of the coronary sinus atrial septal defect. This leaves the coronary veins draining into the left atrium.
Fifteen of 194 patients (7.7%) with tetralogy of Fallot operated upon since January 1, 1972 under a protocol of routine primary repair despite young age died in-hospital. Most deaths were from low cardiac output. Young age and smallness of size increased the risk of operation. No deaths occurred among patients older than 4 years. High hematocrit was also a risk factor. Transannular patching has an independent effect in increasing risk. The post-repair ratio of peak pressure in the right ventricle to that in the left did not exert an independent effect. To project current risks of a two-stage approach, we determined that five of 158 patients (3.2%) died in-hospital after secondary intracardiac repair after a previous Blalock-Taussig or Waterston anastomosis between 1967--1978. Using these data and those we have published on the risk of shunting, we project that except in very small babies, the risks of hospital death of a two-stage approach are not less than those of primary repair done without a transannular patch, except when body surface area is less than about 0.35 m2. When a transannular patch is used in the primary repair, the two-stage approach is projected to be safer when the child has a body surface area of about 0.48 m2 or smaller.
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An angiographic analysis of 10 cases of univentricular heart of the right ventricular type is reported. This congenital malformation is characterized by a large chamber with right ventricular morphology that receives both atrioventricular valves, and a second, smaller chamber, a trabecular pouch, with left ventricular morphology. These chambers are separated by a posterior septum but are connected by an inlet septal defect. The angiographic studies were done using the angled angiographic techniques in three patients and the standard frontal and lateral angiographic views in seven cases. The atrial situs in seven patients was solitus, in one inversus, and in two it was ambiguus with left isomerism. In seven patients the usually large right ventricular chamber received two atrioventricular valves and in four patients, one atrioventricular valve was straddling. Three patients had atresia of one atrioventricular valve. The trabecular pouch was small in seven patients but relatively large in three. In six patients the trabecular pouch was located posterior and to the left of the right ventricular chamber and in four anterior and to the right. Double outlet right ventricle was present in all cases. The aorta arose anteriorly to the pulmonary artery in nine patients and posteriorly in one. An autopsy was performed in one case and its correlation with the angiographic findings was remarkable. The angiographic demonstration of the anatomicaly details of this entity and its associated anomalies was facilitated by angled angiography.
Sixty-two patients with double-outlet right ventricle (DORV) underwent complete intracardiac repair between 1967 and July, 1978. Five patients (three deaths) with DORV and complete atrioventricular (AV) canal are the subject of a separate report and are not discussed further here. Twenty-eight patients had relatively uncomplicated DORV (subaortic ventricular septal defect [VSD], doubly committed VSD, or 1-malposition of the aorta with subaortic VSD) with or without pulmonary stenosis. Two (14%) of 14 died after a completely intraventricular repair, and one (12%) of eight after repair including use of a valved external conduit. Use of a transannular patch in this group was an incremental risk factor, five (83%) dying among six treated in this way. Anterior enlargement of a restrictive or unfavorably located VSD did not increase risk. A noncommitted VSD was an incremental risk factor, two (50%) of four dying after repair. No instances of complete heart block occurred in these groups. Two late deaths occurred in the uncomplicated group, from persisting servere pulmonary hypertension. The late results were good in the remainder and in the two surviving patients with noncommitted VSD. After repair of the Taussig-Bing type of DORV, eight deaths (32%) occurred among 25 patients. Complete heart block developed in two patients. In the Taussig-Bing type of DORV, the 6 year actuarial survival rate was only 38%. Most late deaths were related to improtant pulmonary vascular disease. The surgical technique that has evolved for the basic tunnel repair in the various types of DORV is described.
To aid preoperative decision-making, we have related the ratio of postrepair peak pressure in the right and left ventricles (PRV/LV) to preoperative cineangiographic measurements in a retrospective study of 135 patients undergoing complete repair of tetralogy of Fallot or tetralogy of Fallot with pulmonary atresia. Postrepair PRV/LV was related to the preoperative diameter of right (DRPA) and left (DLPA) pulmonary arteries normalized to the descending thoracic aorta (DescThAo) in patients undergoing repair with transannular patching or a valved external conduit by the dquation: PRV/LV = 0.4840/(DRPA/DescThAO + DLPA/DesThAo) + 0.2007. Stenosis of the right pulmonary artery orifice and pulmonary artery arborization abnormalities incrementally increased postrepair PRV/LV. When a transannular patch was not used in classical tetralogy of Fallot, an increment of postrepair PRV/LV usually resulted, depending upon the size of the "anulus" measured intraoperatively: Incremental PRV/LV = 0.09437 . exp(-0.6344 . Z) where Z is a normalized expression in circumference terms of the diameter of the pulmonary arterial outflow tract (DPAOT) measured intraoperatively after infundibular dissection and valvotomy. DPAOT is itself related to the cineangiographically measured pulmonary valve anulus diameter (DPVA): DPAOT = 3.357 . DPVA0.5789 . BSA0.1551. In toto, these relations allow postrepair PRV/LV without transannular patching to be estimated from preoperative cineangiographic measurements. This allows preoperative predictiom in classical tetralogy of Fallot of the need for transannular patching, and in infants this can determine the choice between primary one-stage repair and two-stage repair. Prediction of postrepair PRV/LV when transannular patching or an external conduit is planned allows identification of patients in whom right and left pulmonary arteries are too small for safe complete repair, and in them an initial palliative operation should be done to enlarge the arteries.
Three cases of anatomically corrected malposition of the great arteries (ACM) are presented. The association of ACM with juxtaposition of the atrial appendages and right aortic arch is emphasized. The unique plain film findings of anatomically corrected malposition include: (a) 1-position of the ascending aorta; (b) discrete convexity of the mid-left heart border in association with findings of an abnormal right atrial border (left juxtaposition of the atrial appendages); and (c) right aortic arch. This unusual combination of findings on the postero-anterior chest should lead to a high degree of suspicion in regard to the diagnosis of anatomically corrected malposition.
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Thirteen (16 percent) of 80 patients with tetralogy of Fallot and pulmonary atresia undergoing corrective operations between Jan. 1, 1967, and Jan. 1, 1978, died in the hospital. The hospital mortality rate was 13 percent (10 deaths) among the 77 patients with confluent right and left pulmonary arteries. The risk of operation was not significantly affected by age at operation or by use of a valved external conduit versus a transannular outflow patch. It was affected (p = 0.008) by the ratio of peak right ventricular to left ventricular pressure (PRV/LV) immediately after repair. This (PRV/LV) was determined primarily by size of left and right pulmonary arteries. An equation was developed relating postrepair PRV/LV to diameter of right and left pulmonary artery (normalized by dividing by size of descending thoracic aorta), body surface area, and possible arborization abnormalities and stenoses of the right and left pulmonary arteries. Cardiac performance after repair was better in those in whom a transannular patch was used rather than a valved external conduit. Important pulmonary dysfunction postoperatively occurred more often in patients with large "bronchial" arteries than in those without them, but was less when these were not ligated. Four (8 percent) of 48 traced hospital survivors died late postoperatively. Reoperations late postoperatively were required only in patients receiving valved external conduits. Forty (91 percent) of 44 living traced patients are asymptomatic.
Four patients with tetralogy of Fallot, three of whom had congenital pulmonary atresia, were treated by initial palliative operations to enlarge left and right pulmonary arteries which were considered too small for complete repair. Two to four years later the right and left pulmonary arteries had enlarged sufficiently to allow complete repair.
The value of axial cineangiography in several forms of congenital heart disease serves as an illustrated supplement to Section I. These techniques visualize defects in the entire ventricular and atrial septum. In persistent atrioventricular (A-V) canal, it is possible to visualize all parts of both septa, status of the A-V valves (two valves versus a common A-V valve), and if a common A-V valve, its degree of override. In tetralogy of Fallot, the bifurcation of the pulmonary trunk, entire ventricular septum and coronary arteries are vividly shown. The presence of true and confluent pulmonary arteries versus systemic or bronchial arteries in pseudotruncus is clearcut. In double outlet right ventricle or in transpositions with or without double outlet right ventricle, the mitral valve-semilunar valve relationships, the left ventricular outflow tract, subpulmonary region and a straddling tricuspid valve are well demonstrated. In asymmetric septal hypertrophy, biventricular angiography may be circumvented.
Cineangiographic axial techniques were designed to overcome the limitations of conventional angiography in the diagnosis of congenital heart disease. Two basic patient (or equipment) maneuvers are involved; 1) long axis of the heart is aligned perpendicular to the X-ray beam, and 2) rotation of patient results in the heart being radiographically sectioned at 30 degree angles. To accomplish this with fixed vertical and horizontal X-ray tubes, three positions were developed: 1) "hepato-clavicular," "4 chamber," 2) "long axial oblique," 3) "anterior-posterior axial." A fourth, the "sitting-up" projection is discussed. The hepato-clavicular position profiles the posterior ventricular septum and atrial septum, separates the A-V valves, places the four cardiac chambers en face, and clarifies mitral valve-semilunar valve and outflow tract relationships. The long axial oblique profiles the anterior ventricular septum, left ventricular outflow tract, aortic valve-anterior mitral valve leaflet. The sitting-up view visualizes the bifurcation of the pulmonary trunk and separates true pulmonary arteries from systemic collaterals.
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Eighty-five patients operated on before January 1976 have survived at least 30 days after insertion of a valved external conduit by the venous ventricle (usually the right) and the pulmonary arteries as a part of the repair of their congenital heart disease. Follow-up information is available on 83 patients, and mean follow-up time has been 27.7 months. Seven patients have required reoperation because of obstructive complications. Actuarial analysis indicates that the proportion of patients requiring reoperation is 4.0 +/- 2.8% at 2 years, 12.9 +/- 5.5% at 3 1/2 years, and 30.4 +/- 12.1% at 5 years. In five patients compression of the conduit between the sternum and heart was the primary cause of the obstruction. The proportion requiring reoperation for conduit compression is 2.0 +/- 2.0% at 2 years, 8.6 +/- 4.9% at 3 1/2 years, and 27 +/- 12.4% at 5 years. Age at operation is not related to the proportion of patients requiring reoperation, nor is the type of conduit. No patients have yet required reoperation for conduit compression whose operation has been done since we began systematically to position the conduit well away from the sternum. By actuarial analysis the proportion of patients surviving 5 years is 76.7%. Of those alive at follow-up examination, 78% were without limitation of activity.