Gerold L. Schiebler

The radiographic appearance of the tracheobronchial tree as an indicator of visceral situs

Abstract To arrive at an accurate anatomic diagnosis in patients with complex cardiovascular malformations or abnormal position of the heart, the situs of the viscera, or more specifically, that of the atria, must be known. Since the situs of the atria always corresponds to that of the tracheobronchial tree, the latter is a very reliable indicator of visceral (atrial) situs. A chest roentgenogram taken in the anteroposterior or posteroanterior projection will show the anatomy of the air-filled trachea and main bronchi, thus indicating whether there is situs solitus, situs inversus or “situs ambiguus”. The latter term is introduced to indicate that in many patients with asplenia or polysplenia syndrome, the visceral situs cannot be determined because of symmetrical development of normally asymmetrical organs.

Cardiovascular Manifestations of the Hurler Syndrome Hemodynamic and Angiocardiographic Observations in 15 Patients

Hemodynamic observations in 15 cases of the Hunter-Hurler syndrome are reported. Required premedication was two to three times that usually needed. No major complications or deaths occurred. As a group, these individuals tend to have slight elevations of pulmonary artery pressures and more definite elevations of systemic arterial pressures. They generally have normal cardiac outputs and, thus, elevations of total systemic, total pulmonary, and pulmonary arteriolar resistances. In only one case was there clinical and angiocardiographic evidence of valvular involvement, that case having aortic insufficiency. In another case there was only angiocardiographic evidence of mitral insufficiency. Only one child was in congestive heart failure at the time of the study. There was no evidence of coronary artery disease. The ascending limb right ventricular pressure curve was frequently notched and the pressure rise time of the left ventricle (dp/dt) was low. Nocase had a congenital malformation of the heart.Those individuals who have normal vascular bed resistances appear to live longer than those who have elevated resistances early in life. The prognosis is also better for the male Hurler of the sex-linked variety than for those cases due to autosomal recessive inheritance.

Reversible pulmonary hypertension in a child with respiratory obstruction and cor pulmonale

Laryngotracheomalacia usually is a self-limited disease causing only noisy respiration. This report describes an infant born with a severe form of this anomaly who manifested cor pulmonale and congestive heart failure by 29 months of age. Suprasystemic pulmonary artery pressures were noted at the time of the initial cardiac catheterization. The child improved clinically after tracheostomy, and a repeat cardiac catheterization at 4 years of age showed a marked decrease in pulmonary artery pressure. Though a frequent cause of stridor in infancy, to our knowledge laryngotracheomalacia has not been reported as associated with cor pulmonale.

Heart disease in the Laurence-Moon-Biedl-Bardet syndrome: A review and a report of 3 brothers

A summary of heart disease in the Laurence-Moon-Biedl-Bardet syndrome has not been published previously. Three male siblings, two of whom had congenital heart disease, are discussed. A review of the literature indicates that a variety of congenital cardiac malformations, occurring predominantly in males, may be found in this syndrome. Acquired heart disease, primarily hypertrophy of the left ventricle, is noted most frequently in association with renal disease and systemic hypertension. Necropsy reports suggest that the incidence of congenital and acquired heart disease is greater than one would surmise from the clinical reports of this entity.

Disturbances in Atrial Rhythm and Conduction Following the Surgical Creation of an Atrial Septal Defect by the Blalock-Hanlon Technique

Disturbances in atrial conduction or rhythm, or both, were found in 16 of 27 patients undergoing the surgical creation of an atrial septal defect by the Blalock-Hanlon technique. These included P-wave aberrations of intra-atrial block and ectopic atrial rhythm, A-V rhythm, A-V dissociation, sinus bradycardia, atrial flutter, escapecapture bigeminy, and atrial premature beats. Some of these were transient and occurred within 2 weeks after surgery. It seems likely that tissue trauma engendered by the clamp and resection of the atrial septum with possible injury to the internodal conducting pathways may be the genesis of these early postoperative disturbances. Of the eight cases with transient disturbances, six occurred in this period.Later changes, such as intra-atrial block, sinus bradycardia, and atrial flutter, may be atttributed to changes in atrial size secondary to the postoperative pathophysiology. Digitalis intoxication and congestive heart failure cannot be excluded as contributory factors in either the early or the late disturbances.It was not possible to correlate the incidence and nature of these disturbances with morbidity and mortality. In such severely ill, cyanotic infants, irregularities, however slight, may have altered cardiac function significantly and contributed to their deaths.

Familial Ebstein's anomaly of the tricuspid valve

Abstract Ebsteins anomaly of the tricuspid valve has not been reported previously in two members of the same family. A 6-year-old boy and his 29-year-old maternal uncle are described, both of whom had the Ebstein type of tricuspid valve documented by cardiac catheterization. A review of the literature suggests that when multiple cases of congenital heart disease occur in a family in which one individual has Ebsteins malformation of the tricuspid valve, another member may have the same anomaly; or even more likely, an analagous anomaly of the left-sided atrioventricular valve with or without congenitally corrected transposition of the great vessels.

Combined ventricular hypertrophy in infancy

Abstract The vectorcardiograms and electrocardiograms in 10 infants with congenital malformations of the heart have been described. Each malformation would presumably result in combined ventricular hypertrophy. The results in 7 patients were compared with the previously established vectorcardiographic criteria for combined ventricular hypertrophy, and it was demonstrated that these criteria may be applicable to infants as young as 7 days. Three infants demonstrated vectorcardiographic patterns different from the previously described patients. One patient, Case 8, revealed a pattern which may indicate an additional criterion of biventricular hypertrophy in the newborn. One common denominator in each of the 10 patients was large diphasic QRS complexes observed in the mid-precordial leads of the electrocardiogram. The corresponding vectorcardiograms in each instance, regardless of the direction of inscription, revealed a QRS loop in the horizontal plane oriented in space so that it manifested itself electrically at the midprecordial leads as large diphasic QRS complexes over 50 mm. These tall mid-precordial QRS complexes have been called the Katz-Wachtel phenomenon and presumably reflect combined ventricular hypertrophy. The derivation of this term was traced.

Left Ventricular Function in Children Studied by Increasing Peripheral Resistance with Angiotensin

Response to angiotensin infusion was evaluated in 11 normal children and 24 patients with suspected left ventricular dysfunction. This included 12 patients with endocardial fibroelastosis, three with idiopathic myocardial hypertrophy, one with cardiac glycogenosis, and eight with the Hunter-Hurler syndrome. In 20 of these 24 patients, cardiac catheterization findings confirmed the clinical suspicion of decreased left ventricular function. In 11 instances left ventricular dysfunction was diagnosed by an elevation of resting left ventricular end-diastolic pressure. In eight instances this was ascertained by a decrease in resting left ventricular stroke work when compared to the expected range, derived by using a linear regression formula based on 24 normal subjects. In only four instances were both abnormal; that is, the resting left ventricular stroke work was decreased and the left ventricular end-diastolic pressure was increased. In five of these 20 patients, abnormal left ventricular function was elicited only on infusion of angiotensin. Thus this test is a significant adjunct in our ability to determine myocardial malfunction at cardiac catheterization.

Interruption of the aortic arch with complete transposition of the great vessels

Abstract This report concerns a Negro infant with interruption of the aortic arch and complete transposition of the great vessels. The presenting features were congestive heart failure and differential cyanosis. The marked cyanosis of the hands was in sharp contrast to the relatively normal color of the feet. The diagnosis was established during life by detailed hemodynamic and angiocardiographic studies. These are correlated with the salient clinical features of this case and with similar cases reported in the literature.

Delayed Onset of Hemolytic Anemia in a Child An Indicator of Ball Variance of Aortic Valve Prosthesis

Severe intravascular hemolysis is described as a complication of implantation of an aortic Starr-Edwards ball-valve prosthesis and insertion of a Teflon patch in the ascending aorta in a 9-year-old boy with severe aortic valvar stenosis. This progressive hemolysis of delayed onset was the sole indicator of extreme degeneration of the silicone rubber ball of the prosthetic aortic valve, a potentially lethal condition requiring surgical intervention. The phonocardiographic signs of aortic ball variance were not present, and there was no overt valvar incompetence.A lesser but noteworthy aspect in this case was the occurrence of an “aplastic crisis” superimposed on the severe hemolysis. This transient bone marrow, red cell aplasia, which was unrelated to drugs, precipitated an acutely severe anemia which accounted for the presenting symptoms of the patient and required transfusion.