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Five patients with double outlet right ventricle, ventricular septal defect, pulmonary arterial hypertension and pulmonary vascular obstructive disease and three patients with complete d-transposition of the great arteries, ventricular septal defect, pulonary arterial hypertension and pulmonary vascular obstructive disease underwent an elective Mustard baffle operation. The ventricular septal defect was not closed. A large patent ductus arteriosus was divided in three patients. Seven of the eight patients are alive five to 32 months after surgery; one patient died 11 months after surgery. Cyanosis, dyspnea on exertion, and exercise limitation improved initially in all and has persisted in the survivors. In pre and postoperative hemodynamic studies in four patients, systemic arterial oxygen saturation and effective pulmonary blood flow increased from mean values of 70% to 90% and 1.7/min/m2 to 3.3 L/mon/m2, respectively. Absolute systemic and pulmonary flows, and pressures and resistances, were not significantly altered. Criteria for selection of patients with transposition of the great arteries of double outlet right ventricle who would benefit from a palliative Mustard procedure (Mustard atrial baffle without closure of the ventricular spetal defect) are: 1) severe symptoms; 2) pulmonary arteiral hypertension (75% systemic) with pulmonary vascular obstructive disease; and 3) pulmonary artieral oxygen saturation greater than systemic (ascending aorta) arterial oxygen saturation by approximately 10%.
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PMID:The palliative Mustard operation for double outlet right ventricle or transposition of the great arteries associated with ventricular septal defect, pulmonary arterial hypertension, and pulmonary vascular obstructive disease. A report of eight patients. 6 24

Left ventricular wall motion abnormalities, the extent and location of coronary artery stenoses, and the radiographic evidence of pulmonary venous hypertension were analyzed in a retrospective study of 40 patients who had surgically proven rupture of the interventricular septum after myocardial infarction. In 33 patients in whom chest films were available, interstitial or alveolar pulmonary edema was present in 78%, while left ventricular enlargement was present in 82%. Of 26 patients who had coronary angiography, complete occlusion of the right coronary artery, left anterior descending artery, or left circumflex artery was present in 92%, with few, if any, collateral vessels around the occlusion. The location of the rupture in the muscular septum was always in the region of akinesis or dyskinesis. Posterior defects were associated with posterobasal and diaphragmatic akinesis, and anterior defects with apical akinesis. Left ventricular aneurysms were adjacent to the septal rupture in 68%, and 74% had mitral regurgitation. The right ventricular diaphragmatic wall in posterior rupture was always akinetic, indicating right ventricular infarction. Thus ventricular septal defect after myocardial infarction (1) tends to occur with multiple coronary occlusions about which little collateral flow develops; (2) can accurately be localized anteriorly or posteriorly in the muscular septum by the location of the akinetic left ventricular wall segment; and (3) has an associated right ventricular infarct when rupture is posterior.
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PMID:Coronary, ventricular, and pulmonary abnormalities associated with rupture of the interventricular septum complicating myocardial infarction. 10 43

Evaluation was made of 17 patients who underwent conduit reconstruction of the right ventricular outflow tract (for anomalies other than truncus arteriosus) at the Texas Heart Institute between December, 1965, and June, 1974. Fifteen patients survived the operation and have shown substantial clinical improvement. Several different conduits were used, principally an allograft aorta with the aortic valve and mitral leaflet attached, a woven Dacron prosthesis containing a xenograft (porcine) valve, and a valveless Dacron tube graft. Allograft conduits are sometimes difficult to procure and keep and tend to calcify with passage of time. Woven Dacron prostheses are favored because they are readily available in a wide range of sizes. We believe it is not necessary for the conduit to contain a valve unless the patient has pulmonary hypertension, in which case we use a xenograft (porcine) valve because this valve does not require the long-term use of anticoagulants, a difficult regimen to manage in children. The largest possible prosthesis must be used; otherwise right ventricular hypertension will persist. Indications for conduit reconstruction include anomalous coronary arteries crossing the right ventricular outflow tract, discontinuity of the pulmonary arteries, and pulmonary atresia with a ventricular septal defect. Our current method of managing pulmonary atresia with ventricular septal defect (pseudotruncus arteriosus) includes palliative shunting to relieve hypoxemia during infancy and to permit full development of the pulmonary arteries for eventual total correction at a more optimal age when a larger conduit may be used.
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PMID:Conduit reconstruction of right ventricular outflow tract. Experience with 17 patients. 12 41

Definitive, if not curative surgery is available for the eight most common congenital cardiac defects-ductus arteriosus, ASD, coarctation, pulmonary valve stenosis, aortic valve stenosis, tetralogy of Fallot, and transposition. The results of surgery for uncomplicated cases of DA, ASD, VSD, and coarctation usually can be determined by clinical means (including chest radiogram and ECG). Postoperative heart catheterization is recommended for evaluation of the patient who has had surgery for pulmonary valve stenosis or artic stenosis and is necessary after tetralogy of Falot or transposition of the great arteries repair to identify the important postoperrative residua and sequelae. The term "curative" surgery probably shoud be reserved for operation for divion of ductus arteriosus unassociated with pulmonary hypertension and performed in childhood. After closure of ASD, patients should continue to be observed for late development of arrhythmias and persistent cardiac enlargement, although the incidence of these problems is low. After VSD closure the patient is still followed at intervals for possible ill effects of the ventriculotomy scar, manifest as arrhythmias, ventricular aneurysm or myocardial insufficiency. The patient with coarctation repair must be observed for a possible late complication from one of the several clinically silent cardiovascular or cerebrovascular anomalies as well as for the change of restenosis or unrelieved hypertension...
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PMID:Residuae, sequelae, and complications of surgery for congenital heart disease. 12 37

The clinical, hemodynamic, and angiographic findings were correlated with the heart size in 207 patients with proved coronary artery disease. Cardiomegaly was noted in 34 patients and normal heart size in 173. In these two groups, the patients' age range, duration of disease, and history of myocardial infarction were similar. There was no statistical difference in incidence of shortness of breath, hypertension, left ventricular hypertrophy, or abnormal glucose tolerance. Patients with cardiomegaly had a significantly higher incidence of congestive heart failure (26 per cent) as compared to patients with normal heart size (2.9 per cent) (P less than 0.001). Patients with enlarged heart presented a high incidence of anterior wall or multiple myocardial infarction (73 per cent) (P less than 0.001). The cardiomegaly group had a high incidence of elevated end-diastolic volumes, elevated end-diastolic pressures, and diminished ejection fractions when compared to patients with normal heart size (P less than 0.01). Double and triple coronary artery disease was more frequent in patients with cardiomegaly and total coronary score was also higher in this group (P less than 0.005). Asynergy was present in 55 per cent of patients with normal heart size but in 82 per cent of those with enlarged hearts (P less than 0.01). The group of patients with cardiomegaly and documented congestive heart failure had ejection fractions less than 0.30. Cardiac catheterization is probably not advisable in these patients in the absence of associated significant mitral regurgitation, ventricular septal defect, or ventricular aneurysm.
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PMID:Correlation of heart size with clinical and hemodynamic findings in patients with coronary artery disease. 12 83

Presented here is the clinical and hemodynamic profile of 147 patients, above the age of 18 with tetralogy of Fallot. Cardiac catheterization and selective cineangiocardiography were performed in all. Infundibular pulmonary stenosis, a subaortic large infracristal ventricular septal defect, mitral-aortic fibrous continuity and equal pressures in both the ventricles and aorta were considered mandatory for the diagnosis of tetralogy of Fallot. Cardiac enlargement was seen in 25.8 per cent of the patients, and 15.6 per cent were in congestive cardiac failure; 9.5 per cent had systemic hypertension, and aortic regurgitation was present in 6.7 per cent. A reticular pattern in the lung fields due to bronchial collaterals was seen in 23.1 per cent. The incidence of right aortic arch (19.9 per cent), absent left pulmonary artery (2.8 per cent), absent right pulmonary artery (0.7 per cent) and dextrocardia (1.4 per cent) is brought out. The right atrial mean pressure was increased in 4.8 per cent and a prominent "a" wave greater than 10 mm Hg was present in 10.9 per cent. The right ventricular end-diastolic pressure was increased in 23.8 per cent and the left ventricular end-diastolic pressure in 25.9 per cent of the patients.
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PMID:Tetralogy of Fallot in adults. A report on 147 patients. 15 88

The hemodynamic effects of nitroprusside and dopamine were studied in 28 children early after intracardiac repair. Children were placed in six groups, five according to their anatomic lesion and one made up of those who had postoperative pulmonary artery hypertension, to evaluate the possible differences in response of the pulmonary arterial vasculature to the drugs. Seven children had repair of an atrial septal defect; six, repair of tetralogy of Fallot; four, repair of ventricular septal defect; five, surgery for pulmonary stenosis; one, closure of a left ventricular to right atrial tunnel; and five, postoperative pulmonary artery hypertension. Dopamine was infused at 8 microgram/kg/min, and nitroprusside at 3 microgram/kg/min. With dopamine, the heart rate increased an average of 10% and the cardiac index 11%; both increases were statistically significant. Changes in systemic and pulmonary vascular resistance, however, were not. With nitroprusside, the heart rate increased an average of 9% and the cardiac index 5%, while there was a significant decrease in both systemic (-20%) and pulmonary (-27%) vascular resistance. With the combination of dopamine and nitroprusside, both the cardiac index (+13%) and heart rate (+20%) increased significantly while systemic vascular resistance fell an average of 23% from control, and the pulmonary vascular resistance decreased 21%. Drug response among all five anatomic subgroups tended to be similar. We conclude that an afterload-reducing agent, such as nitroprusside and an inotropic drug such as dopamine, may have potential clinical advantages when used together in children providing heart rate does not become excessive.
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PMID:Effects of nitroprusside and dopamine on pulmonary arterial vasculature in children after cardiac surgery. 44 65

The author had in his charge 76 patients over 16 years of age. In adults the congenital ventricular septal defect is of a relatively rare occurrence. The extent of the defect is largely determined by the evolution of the disease. In adult patients with minor defects there is no evidence of developing pulmonary hypertension, whereas in those with major defects in evidence is usually a well-marked hypertension. Surgery is indicated in patients with pulmonary hypertension of the I group exhibiting subjective symptoms and ECG and roentgenographic changes. Patients with pulmonary hypertension of the II-IIIa groups are also liable to undergo surgery, but here a relative contraindication is circulatory disturbance in the IIb stage.
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PMID:[Defect in the interventricular septum in adults and the indications for its operative treatment]. 59 20

Twenty-five infants under 1 year of age (mean, 10.3 weeks and 4.0 kg) underwent coarctation repair. Eight had ventricular septal defect (VSD), 3 had transposition of the great arteries with VSD, and 5 had severe tubular hypoplasia. One infant required mitral valve replacement, and 1 required repair of total anomalous pulmonary venous return. Fifteen had repair by primary anastomosis. Seven underwent Dacron or subclavian aortoplasty; the advantages and technique of angioplasty are reviewed. Three patients required bypass grafts. Seventeen patients survived operation. All 5 patients who had severe tubular hypoplasia died postoperatively. The mortality for repair of coarctation with VSD by simultaneous pulmonary artery banding was high; for coarctation with VSD we currently recommend repair without banding, followed by VSD closure if indicated. Three infants have been treated successfully in this manner, with early VSD closure in 1 and regression of the VSD during follow-up in 2. The 17 survivors have been followed for a mean of 41 months with 3 late deaths. Of the 17 survivors, all of whom had a primary anastomosis, 3 have residual gradients. Of the 11 survivors who had preoperative hypertension, 6 are still hypertensive; 3 of these have a gradient between the upper and lower extremities. It is striking that 3 have persistent hypertension despite repair under the age of 1 year.
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PMID:Repair of aortic coarctation in the first year of life. 61 13

Application of various methods of classification to a group of 70 cases of ventricular septal defect with high pulmonary artery hypertension allowed a comparative study between the various methods aiming at distinguishing the forms with low from high pulmonary artery resistance. The reference clinical classification provides supplementary informations derived from the natural or post-operative course and eventually from the microscopic examination. The first automatic classification relies on the study of a single criterion: the pulmonary arteriolar resistance and the systemic resistance ratio. A second classification is based on the attribution of points to some clinical or haemodynamic signs resulting in a score orienting the classification of every individual. Multifactorial analysis methods deal with all the available informations for the overall group, and suppose the use of a computer. The informatic methods make it possible to study the classifying value of every sign. Correlations were established between these various techniques and the medical classification.
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PMID:[Study of interventricular septal defects with equal aortic and pulmonary artery pressures. Classification by clinical and computer methods of 70 cases]. 81 62


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