Edward C. Lambert

Sudden unexpected death from cardiovascular disease in children: A cooperative international study☆

Abstract The results of an international cooperative study of sudden unexpected death from cardiovascular disease in children are reported. Sudden natural death was defined as death occurring instantaneously or within 24 hours of the onset of acute symptoms or signs in ambulatory, nonhospitalized children aged 1 to 21 years. Twenty institutions representing 10 countries contributed a total of 254 acceptable case reports. In 186 cases the child had had no cardiac surgery. Four heart conditions accounted for 53 percent of these “medical” deaths: congenital aortic stenosis (18 percent), Eisenmengers syndrome (15 percent), cyanotic congenital heart disease with pulmonary stenosis or atresia (10 percent) and hypertrophic obstructive cardiomyopathy (9 percent). A total of 33 types of cardiovascular disease were reported. Sixty-eight chlldren died months or years after cardiac surgery. In 28 of these 68 patients death appeared to be causally related to the preceding surgery rather than to the underlying cardiovascular disease. All but 2 of the 28 probably died of an arrhythmia, usually without warning. The period between the onset of acute signs and symptoms (terminal episode) was less than an hour (usually instantaneous) in 73 percent of all 254 patients. In 95 percent of patients in the “medical” group heart disease was known or suspected, but a majority of these patients were asymptomatic. Warning syncope occurred in only 16 percent. A majority of the patients (58 percent) were inactive at the time of the terminal episode (Standing, sitting, lying or sleeping). Only 10 percent were engaged in active sports. This large study appears to establish better than previous data the relative frequency of sudden unexpected death among children with heart disease in Western Europe and Central and North America. Surgical and medical preventive measures, and the indications for engaging in sports, are discussed.

RETROGRADE ARTERIAL CATHETERIZATION OF THE LEFT HEART. EXPERIENCE WITH 500 INFANTS AND CHILDREN.

HISTORICALLY, retrograde catheterization of the arterial circulation appears to have been the first method employed in the study of the left heart. Cournand1 cites the physiologic studies of Chauveau and Marey. They recorded pressure tracings obtained from the left ventricle of the erect, unanesthetized horse in 1861.2 3 Almost 100 years later, in 1950, three groups of workers reported application of this approach to man. Zimmerman, Scott, and Becker4 were first to publish. However, they were successful only in patients with aortic regurgitation, being unable to pass through the normal aortic valve. Furthermore, one of their 11 patients succumbed from ventricular fibrillation during the procedure and another died 4 days later. Limon, Rubio, and Bouchard5 were the first to establish this method as a safe, effective approach in man, allowing passage of the catheter across the normal aortic valve in 17 patients without complications. Later in that year Sodi-Pallares 6 and his group confirmed Limons success in 25 patients stud-

Percutaneous right and left heart catheterization in children. Experience with 1,000 patients.

One thousand patients, ranging in age from 4 months to 26 years, underwent percutaneous catheterization of the femoral artery and femoral vein. Cardiac catheterization was combined with selective angiocardiography in all patients.The catheter entered the right ventricle in 972 of 990 patients (98%) and the left ventricle in 832 of 856 (97%), including 82 of 89 patients (92%) with aortic stenosis.There were no deaths, perforations of the heart, or loss of tissue. The peripheral pulse was decreased in 6% (49 of 856) and absent in 2% (17 of 856). Other complications included two uneventful perforations of the aortic arch, two lacerations of the femoral artery, three lacerations of the femoral vein, a false aneurysm of the femoral artery, and a cerebrovascular accident.Our findings indicate that, in children more than 2 years old and weighing more than 10 kg, the percutaneous method is safe, informative, and associated with less arterial circulatory complications than the approach from the surgically exposed artery.

Idiopathic hypertrophic cardiomyopathy involving the right ventricle

Abstract A 16 year old boy has been followed up since 1952. Though he now experiences slight exertional dyspnea, his cardiac status has not changed during this 14 year period. The clinical and catheterization data indicate that this patient represents a case of idiopathic hypertrophic cardiomyopathy that involves the septum and has produced narrowing of the apex of the right ventricle. Marked blood oxygen desaturation and elevated pressures were found at the right ventricular apex and are believed to be due to systolic obliteration and isolation of the apex by muscular contraction. No obstruction to pulmonary flow was found, and there was no gradient across the left ventricular outflow tract at rest. Our findings are similar to some observations made by others in patients with idiopathic hypertrophic cardiomyopathy, and different from reported cases of double-chambered right ventricle. They indicate that idiopathic hypertrophic cardiomyopathy represents a wide spectrum of manifestations and may involve predominantly the right ventricle.

Comparison of Oxygen Saturation of Blood in Azygos Vein and Superior Vena Cava

Blood samples from the superior vena cava (SVC) are usually considered an acceptable representation of mixed systemic venous return in identifying and quantifying left-to-right atrial shunts. However, if during sampling from the SVC unrecognized entrance of the catheter into the azygos vein takes place, diagnostic errors may result. Blood samples from the azygos vein were collected from 60 children with congenital heart disease and the oxygen saturations ranged from 20% to 92%. These saturations were compared to those of samples taken from the superior vena cava above the entrance of the azygos vein. No substantial difference (saturation within ±4%) was found in 24 patients, while 28 patients had a significantly higher saturation in the azygos vein (from +5% to +21%) and eight patients had a lower saturation (from −5% to −16%). A significant left-to-right shunt may be overlooked or erroneously thought to be present when sampling of blood takes place unknowingly from the azygos vein. Examples are presented.Approaching from the groin it was possible to catheterize the azygos vein in two thirds of the cases when a deliberate attempt was made to do so.

Concomitant development of infundibular pulmonary stenosis and spontaneous closure of ventricular septal defect: An unusual variant in the natural history of ventricular septal defect☆

Abstract An unusual variation in the natural history of ventricular septal defect is described in 2 children, each of whom had evidence of a ventricular septal defect with a large left to right shunt at age of 10 weeks. In both patients severe infundibular pulmonic stenosis subsequently developed, as well as almost complete closure of the septal defect. These changes were documented at ages 8 and 6 years, respectively, by a second cardiac catheterization and at operation.

Differential diagnosis of ventricular septal defect in infancy: A common problem

Abstract An analysis was made of 105 proved cases of congenital cardiac defects in infants under 1 year of age who were seen between 1950 and 1960. Patients were selected who presented with the following clinical findings: (1) a loud or moderately loud systolic murmur maximal in the third and fourth interspaces along the left sternal border; (2) congestive heart failure; (3) absent or minimal cyanosis; and (4) roentgenographic evidence of a large heart with increased pulmonary vasculature. Sixty-five (62%) of the patients selected in this fashion were found to have large ventricular septal defects. This comprised nearly all of the sick infants (65 out of 69) with adequate clinical data and a proved diagnosis of a large ventricular septal defect seen during the 10 year period. Nine different malformations were found to have masqueraded as ventricular septal defects. Two others were added from the literature. The relative frequency of this occurrence is presented. Conversely, the frequency with which the individual defects failed to mimic clinically a ventricular septal defect is analyzed and discussed. The roentgenographic findings were nonspecific, even in retrospect, and were of little aid in all but 3 cases of truncus arteriosus. The electrocardiographic findings, when analyzed with regard to evidence of ventricular hypertrophy or overload, were of value in eliminating possible diagnoses only in cases of aortic stenosis and total aberrant pulmonary venous drainage. Further, the electrocardiographic findings were almost specific in the detection of persistent atrioventricular canal. At the present time it appears necessary to perform cardiac investigative studies (cardiac catheterization and angiocardiography) in a majority of infants who present with this syndrome in order to determine the diagnosis and proper treatment.

Detection of Small Ventricular Septal Defects Comparison of Selective Left Ventricular Cineangiography with Indicator-Dilution Techniques

The value of selective left ventricular cineangiography in the detection of small ventricular septal defects was assessed in 23 patients in whom no shunt was detected by blood oxygen saturation change. A comparison was made with selective indocyaninegreen indicator-dilution curves in 10 of these patients. A ventricular septal defect was demonstrated by both methods in 10. However, in one case, the indicator curve showed a left-to-right shunt whereas selective left ventricular cineangiography failed to demonstrate it. Conversely, in two other cases, selective left ventricular cineangiography demonstrated a small ventricular septal defect while a normal curve was obtained by indicator-dilution technique.Comparing our experience with cineangiography with that of others, using hydrogen ion and indocyanine-green indicator-dilution techniques for the detection of small left-to-right intracardiac shunts, it seems there is little difference in the sensitivity of the three methods. Each is capable of detecting ventricular septal defects which cause left-to-right shunts too small to produce measurable blood oxygen saturation variations.

The electrocardiogram in tetralogy of fallot

Summary The ECG in 100 patients with proven isolated tetralogy of Fallot and no surgery was analyzed. 96% had signs of right ventricular hypertrophy. No patient had left ventricular hypertrophy or a QRS axis in the left upper quandrant in the frontal plane. In order of decreasing frequency, the signs of right ventricular hypertrophy were: abnormal R/S ratio in V 1 , right axis deviation in the frontal plane, abnormally low R/S ratio in V 6 , abnormally deep S waves in V 6 , abnormal R wave in V 1 , abnormal T wave in the right precordium and q waves in V 1 . Slurring, notching or rsR′ complexes in V 1 were frequently found in tetralogies, but not more so than in pulmonic stenosis with intact septum. There is no correlation between the hemodynamic severity and the electrocardigraphic criteria for right ventricular hypertrophy. The electrocardiogram in patients with tetralogies changed very little as time elapsed. A lack of increase or a decrease in left ventricular precordial potentials was seen most frequently.

The pediatrician and congenital heart disease.

Cooperation between the pediatrician and the cardiologist is essential if patients with congenital heart disease are to be well cared for and adequately supervised during infancy and childhood. The purpose of this article is to elaborate on the role of the practicing pediatrician in recognizing and caring for the child with this disease and to set forth the required facts for his effective participation.