Leonard Steinfeld

Endocardial cushion defects: Specific diagnosis by angiocardiography∗

Abstract 1. 1. The anatomy and embryology of endocardial cushion defects are reviewed. 2. 2. Angiocardiographic deformities of the left ventricular outflow region and of the mitral valve are described which we believe to be characteristic and diagnostic of endocardial cushion defects. This method appears to be the most accurate single diagnostic technic available for the identification of these lesions. 3. 3. This study is based on a review of 16 cases of cushion defects and over 200 left ventricular angiocardiograms in a wide variety of congenital cardiac disorders.

Intracardiac thrombi complicating central total parenteral nutrition: Resolution without surgery or thrombolysis

roid hormone and calcitonin in glucose regulation. Eur J Pediatr 1980;135:195-198. 14. Moore EW. lonized calcium in normal serum, uttrafiltrates, and whole blood determined by ion-exchange electrodes. J Clin Invest 1970;49:318-334. 15. Danowski TS, Gillespie HK, Fergus EB, Puntereri AJ. Significance of blood sugar and serum electrolyte changes in cirrhosis following glucose, insulin, glucagon or epinephrine. Yale J Biol Med 1956;29:361. 16. Rasmussen I4. Cell communication, calcium ion and cyclic adenosine monophosphate. Science 1970;170:404-412. 17. Kissebah AH, Clark O, Vydelingum N, ct al. The role of calcium in insulin action. Eur J Clin Invest 1975;5:339-349. 18. Lostroh A J, Krahl ME. Magnesium, a second messenger for insulin: ion translocation coupled to transport activity. Adv Enzyme Reg 1974;12:73-81. 19. Aikawa JK. Effect of glucose and insulin on magnesium metabolism in rabbits: a study with Mg28. Proc Soc Exp Biol Med 1959;103:363-366.

Left Ventricular Angiocardiography in the Study of Ventricular Septal Defects

Ventricular septal defect is reported to be the most common congenital cardiac anomaly (1). In many cases, the presence of such a defect can be recognized on the basis of clinical findings alone. The clinical and hemodynamic changes produced by septal defects vary considerably, however, depending upon their size and location. Moreover, the diagnosis may be obscured by the presence of associated cardiac malformations. Experience with 65 cases has demonstrated that left ventricular angiocardiography is a valuable adjunct in the study of ventricular septal defects both as an aid to diagnosis and in planning treatment. Ventricular defects with left-to-right shunts in excess of 25 per cent of the systemic blood flow are readily apparent from oxygen studies obtained during right heart catheterization. Smaller shunts require more sensitive technics for their detection, such as dye dilution (2), hydrogen (3), and isotope (4) studies. With left-sided angiocardiography, both small and large defects can be consisten...

Left Ventricle to Aorta Valved Conduit for Relief of Diffuse Left Ventricular Outflow Tract Obstruction

Operative relief of congenital tunnel subaortic stenosis by means of local incision or excision, or both, has generally been unsatisfactory. The use of a valve-bearing conduit between the left ventricular apex and thoracic aorta offers a predictable means of bypassing the left ventricular outflow obstruction. The procedure was used in a 17 year old girl with an excellent hemodynamic result. The history of operative management with diverting plantation of valved conduits in this position have not been defined, but use of these prostheses appears advisable in severe subvalvular, valvular and supravalvular obstructions that are not readily amenable to predictable and safe surgical palliation. The operation may prove useful in selected cases of idiopathic hypertrophic obstructive cardiomyopathy.

Treatment of recurrent paroxysmal ventricular tachycardia

Abstract This case report demonstrates the therapeutic effect of the combination of propranolol and procainamide upon recurrent paroxysmal ventricular tachycardia in a 10-year-old boy. When used as a single agent, procainamide, diphenylhydantoin, and propranolol proved ineffective in suppressing the arrhythmia. The child has been in normal sinus rhythm for 20 months without side effects, while receiving a combination of propranolol and procainamide. The etiology and therapy of this condition was discussed.

Aorticopulmonary septal defect: Diagnosis and report of case successfully treated∗

Abstract Aorticopulmonary septal defect is a rare congenital anomaly which is difficult to diagnose. The prognosis is poor if treatment is not instituted and, therefore, surgery should not be delayed but should be performed as soon as is feasible. Transection with or without the aid of hypothermia or pump oxygenator is the treatment of choice. A successful case is presented.

Histologic observations of the cardiac conduction system in a patient with postoperative bilateral bundle branch block

Abstract It has been suggested that the postoperative development of a right bundle branch block pattern is due to injury of peripheral rather than proximal parts of the right bundle branch and does not affect the prognosis for the later development of complete heart block. However, the occurrence of complete heart block in a patient with postoperative right bundle branch block who later has left bundle branch block has not previously been documented. The entire specialized cardiac conduction system of a patient who had two operations for repair of double outlet right ventricle and multiple ventricular septal defects was serially sectioned. In the first operation, which resulted in a right branch block pattern, a subaortic ventricular septal defect was repaired through a right ventriculotomy. In the second operation complete heart block occurred after multiple muscular ventricular septal defects were repaired with a patch inserted through a left ventriculotomy. Examination of the conduction system showed that the right bundle branch was completely interrupted by the patch used to repair the subaortic ventricular septal defect and the left bundle branch was completely severed by the patch used to repair the muscular ventricular septal defects. This case proves that some patients who have a right bundle branch block pattern after repair of a ventricular septal defect may later have complete heart block if left bundle branch block develops as a result of sclerodegenerative or atherosclerotic heart disease or other cause.

Electrocardiographic and vectorcardiographic changes following surgery in persistent common atrioventricular canal.

Abstract The electrocardiogram and vectorcardiogram are probably the most reliable tools in the diagnosis of atrioventricular cushion defects. Our observations require further evaluation. Evidence has been offered suggesting that the electrocardiogram and vectorcardiogram also may assist in the evaluation of the effectiveness of surgical repair. It appears that there is enough evidence to conclude that the electrocardiographic and vectorcardiographic patterns observed in atrioventricular cushion defects are due not only to a congenital aberration of the left bundle but also to cardiac enlargement.

CORRELATION OF THE MURMUR OF INTERVENTRICULAR SEPTAL DEFECT WITH PRESSURE DIFFERENCES BETWEEN THE VENTRICLES.

The systolic murmur associated with interventricular septal defect presents a number of variations which have been related to the location and size of the defect, the pulmonary vascular resistance, and to the pressure gradient between the ventricles. The purpose of this investigation was to study the relation of the pressure gradient in interventricular septal defect to the time-intensity characteristics of the murmur. This was accomplished by simultaneously ?ecording pressure pulses in both ventricles together with continuous pressure differences between the ventricles, and phonocardiograms.

CARDIOVASCULAR EFFECTS OF MATERNAL METHADONE ADDICTION IN THE NEWBORN

Systemic hypertension has been observed in a number of infants born to methadone-addicted mothers. A study was designed to determine the characteristics of this hypertensive state. Fifty newboms of mothers participating in the methadone program were selected. Blood pressures were obtained in both a sleeping and in a quiet, wakeful state. In 24 patients (48%) an abnormal elevation in the systolic blood pressure was documented (range: 90 to 120 mmHg.). The majority were normotensive at birth; hypertension usually developed during the third and fourth days of life. The average duration of hypertension was two weeks (range: 3 to 60 days). The following observations concerning the hypertensive state were noted: (1) hypertension was demonstrated both in the presence and absence of withdrawal symptoms. Normal blood pressures were observed in several patients with obvious withdrawal, (2) elevated blood pressure persisted several weeks after withdrawal symptoms ceased and was unaffected by usual treatment for withdrawal, (3) hypertension was unrelated to serum electrolytes, serum catecholamines, or maternal blood pressure. Electrocardiograms of 18 patients (36%) displayed abnormalities of the ST segments, T waves, and/or QT intervals. Congenital heart disease was documented in three patients (6%). Transient hypertension seems the most common cardiovascular abnormality in infants born to methadone-addicted mothers.