Grady L. Hallman

Congenital cardiovascular anomalies in adults. Results of surgical treatment in 167 patients over age 35.

Abstract Congenital cardiovascular anomalies are an infrequent but important cause of heart disease in adults. Variations in blood volume during and after operation are less critical in adults than in children, and intracardiac repair is frequently easier from a technical standpoint in the larger adult heart. A series of 167 patients over age 35 is reviewed. All underwent operations for a variety of cardiovascular anomalies including (in decreasing order of frequency) atrial septal defect (secundum), coarctation of aorta, ventricular septal defect, pulmonary stenosis, atrial septal defect (primum), patent ductus arteriosus, tetralogy of Fallot, aortic stenosis, vascular ring, and total anomalous pulmonary venous drainage. The over-all mortality was 9 per cent and varied according to age, severity of pulmonary hypertension, and presence of congestive heart failure. Technical factors in which adults differ from children are chiefly those concerned with degenerative changes such as atherosclerosis. Mortality in this series of 167 adult patients is comparable to that encountered in a group of 2,353 patients less than 35 years of age undergoing operations for congenital cardiovascular anomalies. This study indicates that surgical correction of congenital cardiovascular defects may be accomplished in adults within an acceptable low mortality rate and with gratifying results.

Obstructive Lesions of the Left Ventricular Outflow Tract Surgical Treatment

Currently available diagnostic methods and surgical technics now allow accurate assessment and effective correction of the majority of lesions producing obstruction of the left ventricular outflow tract. On the basis of anatomic location these lesions can be divided into supravalvular, valvular, and subvalvular stenosis, and valvular lesions further can be subdivided into congenital and acquired categories. Temporary cardiopulmonary bypass with a pump oxygenator provides sustained support of the circulation for operation, while surgical technics employed are selected on the basis of etiology and location of the lesion. Experience gained in operations on 296 patients with obstructive lesions of the left ventricular outflow tract since 1956 now has demonstrated that these operations usually can be performed with acceptable risk and with excellent functional results.

Surgical Treatment of Cardiac Defects Associated with Corrected Transposition of the Great Vessels

Corrected transposition of the great vessels is characterized by transposition of the aorta in front and to the left of the pulmonary artery and inversion of the ventricles and atrioventricular valves. Unless it is associated with an intracardiac defect, the circulation of blood inside the heart is normal, and the anomaly may not be recognized clinically. The altered anatomy requires important modifications in the technical features of operative correction of associated cardiac defects.Among 53 patients with corrected transposition of the great vessels, 31 underwent operation for associated intracardiac defects. These were ventricular septal defects (11 patients), tetralogy of Fallot (seven), common ventricle (five), single ventricle (three), tetralogy of Fallot with pulmonary atresia (two), pulmonary stenosis (one), pulmonary atresia (one), and tetralogy of Fallot with common ventricle (one).Postoperative complete heart block, which usually leads to a fatal outcome, may follow closure of ventricular septal defect when conventional techniques are used. Although only one death from heart block occurred in seven patients operated on through a right ventriculotomy, we have employed left ventriculotomy in the last two patients to reduce the risk of heart block and to afford better exposure of the ventricular septal defect. The presence of preoperative complete heart block does not contraindicate correction of associated defects, but consideration should be given to insertion of a pacemaker at the time the lesion is corrected since these patients are likely to have Stokes-Adams attacks later.The anatomical features of associated common ventricle, single ventricle, and tetralogy of Fallot present such severe technical problems that complete correction of these anomalies is accompanied by a high risk. Total correction of tetralogy of Fallot was successfully carried out in only one of seven patients. Others were treated by some form of systemic-pulmonary shunt with good results. Two patients with tetralogy of Fallot and pulmonary atresia and one patient with pulmonary atresia were also treated by systemic-pulmonary shunts and obtained satisfactory palliation. One patient with pulmonary stenosis underwent successful transventricular pulmonary valvulotomy. A ventricular septum was created in five patients with common ventricle. There were only two survivors; the other three died of complete heart block.Recognition of corrected transposition of the great vessels associated with an intracardiac defect that requires operative repair is important because of technical problems that the morphological alterations of ventricular inversion impose.

Combined mitral and aortic valve disease

Abstract Surgical treatment of multiple valve disease requiring replacement of two valves is associated with a high mortality. Different combinations of mitral and aortic valve stenosis or incompetence result in different changes of adaptation by the heart. The left ventricular response to pure pressure overload, as in combined mitral and aortic stenosis, is concentric hypertrophy, whereas the changes that occur in response to pure volume overload, as in combined mitral and aortic regurgitation, are dilatation and hypertrophy. The records of 124 patients with a variety of combinations of mitral and aortic valve disease were reviewed. Surgical mortality could be directly related to the presence of left ventricular concentric hypertrophy and the small size of the left ventricular chamber. Surgical treatment was accomplished with a surgical mortality of 52.5 percent (21 of 40 patients) when both valves were stenotic; however, when botb valves were insufficient the surgical mortality was only 9 percent (3 of 32 patients). This presentation includes correlation of surgical results with the clinical, electrocardiographic roentgenographic and hemodynamic data. An attempt is made to clarify the indications for double valve replacement. The operative technique used in the treatment of these patients with combined mitral and aortic valve disease is described.

RELATIONSHIP BETWEEN POLYCYTHEMIA AND SURGICAL MORTALITY IN PATIENTS UNDERGOING TOTAL CORRECTION FOR TETRALOGY OF FALLOT.

“Complete” surgical correction of Fallots tetralogy carries a much higher risk in patients with a hemoglobin concentration greater than 18 Gm. per cent (31 per cent mortality) than in those whose hemoglobin concentration is less than 18 Gm. per cent (10 per cent mortality).The successful reduction of hemoglobin concentration from greater than 18 Gm. per cent to less than 18 Gm. per cent through systemic-pulmonary anastomosis greatly reduces the surgical risk associated with “complete correction.” The mechanism by which this reduces surgical mortality is not known but may be related to (1) decreasing the hemorrhagic tendency associated with polycythemia and (2) conditioning of the pulmonary vascular bed and “left heart” in preparation for their new role when definitive repair has been accomplished.The hemoglobin concentration can be used to grade the severity of Fallots tetralogy. Such grading would provide a basis for valid appraisal of surgical mortality rates among otherwise comparable series of cases.

Cardiac valve replacement for congenital mitral valvular disease in children

Abstract Certain congenital defects of the mitral valve causing severe disability during childhood can be managed only by valve excision and prosthetic substitution. Mitral valve replacement was performed in 21 children for correction of congenital mitral stenosis in 6, isolated congenital mitral insufficiency in 10, and complete atrioventricular canal defects in 5. Three patients underwent concomitant aortic valvulotomy. Fourteen patients had pulmonary hypertension and 3 had undergone previous repair of coarctation. Nine patients were in congestive heart failure. Three hospital deaths occurred in patients operated upon for intractable failure; one had associated endocardial fibroelastosis, another acute mitral insufficiency from Marfans syndrome, and another complete atrioventricular canal. Two late deaths occurred; one due to recurrent thrombus formation on the prosthesis, and the other from bacterial endocarditis and cardiac failure. The low-profile discoid valve with minimal protrusion into the left ventricle is particularly suited for mitral replacement in children. Small adult-sized prostheses were used permitting growth and development of the children. Cloth covering of the seating ring permits incorporation by ingrowth of autologous tissue and decreased thromboembolic complications. Although the long-term fate of prosthetic heart valves is uncertain, the management of severe congenital mitral deformities by valve replacement has provided remarkable clinical improvement in these children.

Criteria for recommending surgery in total anomalous pulmonary venous drainage

Abstract Patients with mild dyspnea and cyanosis due to total anomalous pulmonary venous return should be treated medically until later childhood or early adult life when open corrective surgery can be performed with the aid of extracorporeal circulation. Infants experiencing severe dyspnea, cyanosis and congestive failure soon after birth should be considered surgical emergencies, since medical therapy is usually inadequate to support life until childhood. Early diagnosis and aggressive surgical therapy are, therefore, mandatory for survival in the critically ill infant with total anomalous pulmonary venous drainage.

Criteria for recommending surgery in the tetralogy of Fallot

Abstract Selection of the proper operative procedure for patients with tetralogy of Fallot depends upon several factors including age of the patient and severity of symptoms and findings. Infants in the first two years of life with severe cyanosis and with “spells” of dyspnea and loss of consciousness should be treated initially by creation of a systemic-pulmonary shunt. Indeed, operation during the newborn period may be necessary on an emergency basis if symptoms are acute. Definitive repair of the anatomic lesion should be deferred until the patient has reached a more favorable age, more than 5 years old. If hypoxemia is mild to moderate only, surgical intervention should be delayed until the age of 6 to 8 years at which time a total correction may be done. In some older patients with severe cyanosis and polycythemia, a preliminary systemic-pulmonary shunt is often used to reduce the hematocrit and to develop and prepare the hypoplastic left side of the heart to support systemic circulation when a complete correction is accomplished. These criteria were developed through an experience with selection of indirect and direct surgical procedures in a series of 416 operations on tetralogy of Fallot.

BILATERAL PATENT DUCTUS ARTERIOSUS: CASE REPORT.

&dquo;B. S.&dquo; was a 4-day-old Latin American male admitted to the Texas Children’s Hospital on August 21, 1957 because of cardiac enlargement and edema. The mother was a 19-year-old Rh negative preeclamptic. Birth occurred after an 8-month gestation. The patient was the second born of apparently single placenta twins and weighed 4 lb, 8 oz. Delivery was accomplished without undue difficulty. The Coomb’s test

Surgery of the heart and great vessels in the newborn period. Review of 450 cases.

The authors review a series of 450 operations performed during the first year of life on infants with congenital defects of the heart and great vessels. Both closed and open-heart technics were used in various operations designed to correct the anomalies completely or to diminish their deleterious hemodynamic effects. The survival of 72 per cent of these infants demonstrates the advisability of surgical therapy when medical management is not effective in relieving hypoxemia and heart failure.