Anna J. Hauck

Ventricular Septal Defect with Aortic Regurgitation Medical and Pathologic Aspects

Thirty-four patients with ventricular septal defect and aortic regurgitation, representing less than 5 per cent of our patients with ventricular septal defect, are discussed.A loud, systolic murmur, characteristic of ventricular septal defect, is noted during infancy, whereas evidences of aortic regurgitation (protodiastolic murmur and wide pulse pressure) does not usually appear until sometime between 2 and 10 years of age.Clinical and catheterization data indicate that the principal hemodynamic load is aorticregurgitation, whereas the ventricular septal defect does not usually result in a large pulmonary blood flow or high pulmonary arterial pressure. In about 50 per cent of the patients, a significant pressure gradient across the right ventricular outflow tract exists.Detailed pathologic studies indicate that the ventricular septal defects are high and anterior and encroach to a greater or lesser degree on the membranous bulbar septum. The right coronary cusp is the one most severely involved, and, by its prolapse, causes aortic regurgitation; the noncoronary cusp is always less severely affected. The anatomic basis of the pressure gradient observed across the right ventricular outflow tract is not always clear.

Persistent Truncus Arteriosus A Clinical, Hemodynamic, and Autopsy Study of Nineteen Cases

The clinical, hemodynamic, and autopsy data on 19 patients with a diagnosis of persistent truncus arteriosus have been presented.A proposed clinical classification, dividing the patients into types I and II only, with exclusion of type IV, provides a uniform clinical profile. The patients, as a rule, are small infants with a large left-to-right shunt, minimal cyanosis, wide pulse pressure, left or combined ventricular hypertrophy, and congestive heart failure, and resemble patients with a ventricular septal defect or patent ductus arteriosus.At cardiac catheterization distinct but slight differences in pressures or saturations between pulmonary arteries and “ascending aorta” do not exclude the diagnosis of persistent truncus arteriosus.The prognosis is uniformly poor. Most infants die in congestive heart failure within the first 6 months of life, others develop pulmonary vascular obstructive disease in later years.

Endocardial Cushion Defect Preoperative and Postoperative Survey

Preoperative and postoperative data on 44 patients with endocardial defect, 32 with the incomplete and 12 with the Complete form are presented. Nineteen per cent of the incomplete form died with per cent of the deaths occurring early and 9.5 per cent late. The mortality rate for the complete form was 75 per cent. Late follow-up results were evaluated in 28 patients 1 to 2½ years following surgery. Catheterization data in nine of 10 and clinical observations in 18 indicated no residual shunt. One patient showed questionable evidence of a left-to-right shunt by cardiac catheterization. Evidence of mitral regurgitation was present in seven (25 per cent) of the survivors. Short chordae attaching the closed cleft margin of the mitral leaflet to the ventricular septum rendered the valve incompetent in nine of 12 autopsied specimens. Surgical exploration of the underside of the cleft margins for such chordae is recommended. No evidence of residual tricuspid regurgitation was found.

The use of digoxin in infants and children

Abstract 1. 1. Eighty-six children with congestive failure or arrhythmias were treated with digoxin. Two separate courses of therapy were given to 3 patients. 2. 2. Dosage for digitalization of patients up to 2 years of age was found to be approximately 0.04 mg. per pound of body weight in 24 hours. Children over 2 years of age required approximately 0.02 to 0.03 mg. per pound of body weight for digitalization. Maintenance in both groups could be achieved, generally, with the daily administration of one third of the digitalizing dose. 3. 3. Exceptions to this schedule made it mandatory that each patients digitalization be individualized. 4. 4. Definite clinical or electrocardiographic evidences of toxicity were observed in 22 patients, in many of whom excessive dosage was deliberately given in an attempt to achieve therapeutic results. The toxicity was mild in most and reversible in all.

The Clinical Usefulness of Hydrogen Gas as an Indicator of Left-to-Right Shunts

In 60 patients with congenital heart disease 210 observations with hydrogen gas as an indicator for left-to-right shunts were recorded at cardiac catheterization. All but nine had other definite evidence of the presence or absence of left-to-right shunting. In these nine, 21 early hydrogen arrival times formed the only conclusive evidence of left-to-right shunting.Although hydrogen and oxygen may form a highly explosive mixture, pure hydrogen used with proper precautions is a safe, reliable, and extremely simple indicator, eminently suited as a rapid screening procedure or as a supplementary tool in cases in which left-to-right shunts are difficult to prove.

Combined aortic and pulmonic stenosis.

Combined aortic and pulmonic stenosis is a rare lesion. The clinical, hemodynamic, and pathologic findings in four patients are presented. The presence of a forceful left as well as right ventricular impulse (in four patients), the difference in the character of the ejection murmur at the second left as opposed to the second right interspace (in two patients), and a diastolic rumble at the apex (in four patients) were characteristic. The electrocardiogram showed right ventricular hypertrophy in all patients; additional and definite left ventricular hypertrophy was found in one. Radiologically, there was evidence of right ventricular enlargement in all and suggestive left ventricular enlargement in three. Simple valvular stenosis of both valves was found in only one patient, and valvular pulmonary and supravalvular aortic stenosis in another. The two remaining patients, those with corrected transposition of the great arteries, had complicated lesions including a single ventricle in one and small ventricular septal defect in the other. Attempts at surgical correction were unsuccessful in all cases.

Endocardial Cushion Defect with Pulmonic Stenosis

Five patients with endocardial cushion defect, pulmonic stenosis, and right-to-left shunt have been presented. Valvular pulmonic stenosis was present in all, with additional infundibular stenosis in two. Four patients had complete atrioventricular canal defects. In two children both great arteries arose from the right ventricle. In the clinical differentiation of this entity from the common forms of cyanotic congenital heart disease, the electrocardiogram, characterized by left axis deviation and a counterclockwise inscription of the plane vector loop, is the most helpful tool. The surgical problems presented may be formidable and even impossible to solve, if both great arteries emerge from the right ventricle.

HEART FAILURE IN INFANTS AND CHILDREN. SURVEY OF RECENT LITERATURE, ETIOLOGIC CONSIDERATIONS AND RECOMMENDATIONS FOR THERAPY.

High lights of recent reviews are presented, and various causes of heart failure in infants and children are considered from the standpoint of the mechanisms of de-compensation. The clinical picture is reviewed and recommendations for treatment are given, including symptomatic therapy, digitalization, use of diuretic agents, and treatment of the underlying disease.

Pediatric Aspects of Congestive Heart Failur

F r o m time t o t ime this journal plans t o reprint material of particular interest and value to anesthesiologists which has appeared in other journals. These articles primarilg will contain fundamentally basic information which has been presented by anesthesiologists, physicians, pharmacologists and others and, o n occasion, b y commercial firms. I n the last instance, these reprintings of presentations are not t o be construed a s any special endorsement of t h e particular commercial firm or product by Anesthes i a a n d Analges ia C u r r e n t Researches.