Coarctation of the aorta: difficulties in clinical recognition.

Coarctation can be recognized by physical examination alone early in a person's life, except in instances in which the obstruction is very mild. While some cases remain mild for a lifetime, others become progressively worse during adolescence or adulthood with typical manifestations of upper-extremity hypertension and imperceptible pulses in the lower extremities. It is especially urgent and sometimes difficult to recognize coarctation in infants with congestive cardiac failure just as it is to identify older children and adolescents with asymptomatic but severe systemic hypertension. There may be medical or legal implications in failing to do so.

Value and limitations of auscultation in the management of congenital heart disease.

With advances in technology, especially that of ultrasonography, the art and science of auscultation could become relegated to that of an obsolete technique. Physicians in a cardiovascular training program involved in learning modern instrumentation diagnostic techniques often become expert in these areas before they have mastered the use of the stethoscope. Although the technology of ultrasonic examination, cardiac catheterization, and angiocardiography must be learned, the value of auscultation of the heart cannot be ignored. Many highly competent physicians who are responsible for diagnosis and treatment of the pediatric patient, including some pediatric cardiologists, may not be aware that they have not yet become experts at auscultation. The skill of auscultation of the heart must be learned by supervised experience with patients in whom a wide variety of normal and abnormal heart sounds and murmurs can be heard. Once learned, the skill can be maintained only by frequent practice in hearing and evaluating both subtle and obvious sounds emanating from the heart and from the pulmonary and systemic blood vessels.

The adult with congenital heart disease.

Internal medicine cardiovascular specialists and internists are going to be seeing increasing numbers of adult patients with congenital heart malformations, and most of them will have had one or more surgical or therapeutic catheter procedures. The nonsurgical cases will have either benign defects or important but inoperable anomalies, or the physician may encounter the rare case which, though amenable to surgery, has escaped operation until adult life. Among patients with postoperative congenital heart disease, a few are totally cured and require no special follow-up or any special precautions, e.g., those with certain cases of atrial septal defect of ostium secundum types, patent ductus arteriosus without pulmonary hypertension, and fully repaired total anomaly of pulmonary venous return. Others who are very much improved by definitive repair have residual defect for which surgical treatment was not attempted, e.g., bicuspid aortic valve in the patient who had surgical repair of coarctation or the patient with persistent cleft of a mitral valve leaflet for which no repair was attempted at the time of closure of the ostium primum type of atrial septal defect. Some patients have had incomplete and unsuccessful repair of a defect but yet are symptomatically improved compared with their preoperative status, e.g., those with tetralogy of Fallot with loss of right-to-left shunting by closure of the ventricular septal defect but persistent right ventricular outflow tract or pulmonary artery branch obstruction. Patients with persistently high intracardiac pressure from unrelieved obstruction are at higher risk for sudden death than they were preoperatively, especially those with a high grade of ventricular ectopy. Despite symptomatic improvement compared with the preoperative status, such cases must have reoperation, if feasible, as a means to prevent a sudden catastrophe. Patients with congenital malformations have symptoms that are distinctly different from those with acquired heart disease. Furthermore, their reactions to their problems and to their lives and work in relation to others are often colored by their restricted and overprotected childhoods, which were often filled with doctor visits, several admissions to the hospital, one or more operations, and usually several heart catheterizations. Otherwise, these patients are capable of the same level of achievement intellectually, and only a few have important defects of other systems that would interfere with their roles as independent and productive adults. The problem arises as to what group of physicians will care for these patients in the future.(ABSTRACT TRUNCATED AT 400 WORDS)

Ethmozine (moricizine HCl): a promising drug for "automatic" atrial ectopic tachycardia.

Atrial ectopic tachycardia is an uncommon life-threatening supraventricular tachycardia in children and is resistant to usual antiarrhythmic drugs. Whereas the cellular mechanism of atrial ectopic tachycardia is unknown, atrial ectopic tachycardia may be due to a form of automaticity. Moricizine HCl has been used primarily for ventricular rhythm disturbances; the drug depresses abnormal automaticity and delayed after-depolarizations but has little effect on normal automaticity. Because of this property, we have used moricizine HCl in 4 patients with atrial ectopic tachycardia. As evidenced by continuous 24-hour Holter monitoring, moricizine HCl was successful in suppressing atrial ectopic tachycardia in each patient. During a limited follow-up (6 months) no side effects have occurred. Moricizine HCl is a promising primary drug for atrial ectopic tachycardia.

Surgery without catheterization for congenital heart defects: management of 100 patients.

Little information is available regarding the surgical mortality of children with congenital heart disease who undergo operation on the basis of clinical assessment and echocardiographic diagnosis without cardiac catheterization. If catheterization affects early surgical mortality then perhaps omitting it would improve survival. Early operative mortality of 100 patients managed with echocardiography without preoperative catheterization (Group I) was compared with that of 151 diagnosis-matched control patients who had catheterization (Group II). The catheterization (Group II) and echocardiographic (Group I) groups included patients with the following diagnoses: atrial septal defect (33 and 17 in Group II and Group I, respectively); pulmonary stenosis/pulmonary atresia (33 and 15), aortic stenosis (14 and 3), coarctation of aorta (36 and 29), patent ductus arteriosus (10 and 15) and miscellaneous (25 and 21). Age range was 1 day to 16 years (mean 3.5); 114 (45%) of the 251 patients were less than 1 year of age and 59 (29%) were less than 1 month of age. With one exception, echocardiographic diagnosis was correct when compared with findings at surgery or autopsy, or both. Surgical mortality was 18% in Group I and 9% in Group II; however, multiple logistic regression analysis adjusting for differences in age and preoperative condition showed no significant difference between the two groups with a trend toward lower mortality in Group I (p = 0.075). Echocardiography as the definitive imaging modality in selected patients with congenital heart disease is accurate and is comparable with conventional methods of preoperative diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Prenatal diagnosis and postnatal management of critical aortic stenosis.

Fetal echocardiography has yet to have an impact on the treatment of congenital heart disease. Critical aortic valve stenosis was diagnosed by echocardiography before birth in a 35 week gestation fetus. The risks to the fetus and mother associated with prolonged rupture of membranes prompted their transport to a hospital with cardiac surgical and high-risk perinatal facilities. Prenatal and postnatal echocardiographic findings agreed regarding the diagnostic criteria for critical aortic stenosis and primary forms of cardiomyopathy were excluded. Results of fetal Doppler examination were consistent with valvular aortic stenosis and excluded mitral regurgitation. Determination of the left ventricular size excluded ventricular hypoplasia. The infant was delivered by cesarean section and underwent successful emergency aortic valvotomy at 12 h of age. Fetal echocardiography, in combination with a multidisciplinary postnatal approach, can be used in the successful treatment of a severe form of congenital heart disease.

Reconstruction of coronary artery anomaly in an infant using the internal mammary artery: 10-year follow-up.

We report a case of tetralogy of Fallot with an unsuspected anomalous left anterior descending coronary artery arising from the right coronary artery and crossing the right ventricular outflow tract in a 16-month-old infant. During operation, the anomalous artery was severed. Successful repair of the intracardiac anomalies was performed, including left anterior descending-internal mammary artery reconstruction of the anomalous artery. This case illustrates the importance of delineation of coronary artery anatomy and even selective coronary arteriography in patients with tetralogy of Fallot, since anomalous coronary arteries occur most frequently in association with other cardiac anomalies. Arteriography 10 years later revealed a patent anastomosis to the left anterior descending artery, proving the durability of the internal mammary artery in a patient we believe to be the youngest to have undergone bypass with this particular conduit.

Application of information theory to decision analysis in potentially prostaglandin-responsive neonates.

To define settings in which use of prostaglandin E1 before transfer from a community hospital to a tertiary care center benefits neonates with possible heart disease, information theory was used to predict the probability of a favorable response to prostaglandin therapy from the limited information of clinical variables. Records of 250 patients, newborn to 7 days old, with suspected heart disease were reviewed to assess six clinical variables (cyanosis, respiratory distress, heart murmur, pulse contour, hepatomegaly and prematurity). According to the anatomic and hemodynamic cardiovascular condition, each case was categorized as to whether a favorable response to prostaglandin E1 could be anticipated. Information content of each clinical variable with respect to prostaglandin responsiveness was determined, and patients were classified according to the most informative clinical variable. Stepwise extraction of information proceeded until remaining clinical variables added no significant information. Bayes' rule gave estimates of probability of prostaglandin-responsive defect in final subgroups for use in decision analysis. Cyanosis, murmur, small volume pulses and prematurity gave information about prostaglandin-responsive defects. Decision analysis indicated that frequency of poor outcome is minimized by early prostaglandin treatment of cyanotic term infants with a murmur or poor pulses, regardless of how ill they appear, and by treating any critically ill term newborn who has either cyanosis or poor pulses. Acyanotic patients with normal pulses are best untreated with prostaglandin until after definitive diagnosis is made. Advantage to either course was not seen in some small subgroups. Information theory with decision analysis is a rigorous approach to identify relevant clinical variables and define their roles in critical decisions in pediatric cardiology.

Quantitative morphology of the aortic arch in neonatal coarctation.

It has been speculated that neonatal coarctation results from postnatal constriction of the aortic isthmus and ductus arteriosus. However, aortic arch hypoplasia is present in some neonates with coarctation and is presumed to be due to decreased aortic arch blood flow in utero. To measure the degree of aortic arch hypoplasia and to analyze the distribution of blood flow in neonatal coarctation, quantitative morphometric analysis of the great vessels from two-dimensional echocardiograms was performed in 14 neonates with isolated coarctation and 14 normal control neonates, all less than 1 month old. Measurements of the aortic valve, pulmonary valve, ascending aorta, transverse aortic arch between the carotid and subclavian arteries, aortic isthmus, descending aorta, main pulmonary artery and brachiocephalic vessels were obtained to the nearest 0.5 mm. In coarctation patients the transverse arch and isthmus were significantly smaller than in control subjects (p less than 0.001). In addition, pulmonary valve and main pulmonary artery diameters were significantly greater in neonates with coarctation than in normal neonates. Transverse arch hypoplasia and increased pulmonary valve and pulmonary artery diameters are present in neonates with coarctation. This suggests decreased aortic arch flow and increased pulmonary and ductus arteriosus flow in utero, which produce a characteristic echocardiographic appearance of transverse arch hypoplasia with a large main pulmonary artery.

Doppler echocardiography in the diagnosis and management of persistent fetal arrhythmias.

Thirteen fetuses with persistent arrhythmias underwent combined noninvasive echocardiographic evaluation utilizing M-mode, two-dimensional and pulsed Doppler echocardiography. This group (Group A) was compared with 14 fetuses in which only two-dimensional and M-mode echocardiographic evaluations were performed (Group B). In both groups correct prenatal interpretation of the arrhythmia was confirmed by postnatal electrocardiograms in all surviving fetuses. Although Doppler echocardiography was not more sensitive than M-mode echocardiography in the interpretations of the arrhythmia, Doppler tracings of sufficient quality to analyze rate and rhythm were easier to obtain in all cases and provided additional information about valvular incompetence and the functional state of the fetal heart. Cardiac malformations and hydrops fetalis were commonly associated with persistent arrhythmias. Congenital heart disease occurred frequently (6 of 11) with complete atrioventricular (AV) block. Pulsed Doppler echocardiography defined the AV contraction sequence, atrial and ventricular rates and AV valve insufficiency, allowing rapid interpretation of fetal arrhythmias.

Dysrhythmias after repair of atrial septal defect.

To determine the incidence of dysrhythmias following repair of isolated secundum atrial septal defect, postoperative electrocardiograms were reviewed in 292 patients. The PR interval was shortened by the 7th postoperative day (p <.05) as compared to the preoperative PR interval. Sinus rhythm only was observed in 228/292 (78%). Nine patients (3%) had a preoperative dysrhythmia. A postoperative dysrhythmia was observed in 64/292 (22%). Twenty-two (7.5%) had symptomatic postoperative dysrhythmias. A tachydysrhythmia was found in 16/64 (25%) of these. The latest occurrence of symptomatic dysrhythmia was 17 years after surgery. Currently, 7/292 (2.4%) of the patients reviewed have a pacemaker. Five of 292 (1.7%) had Holt-Oram syndrome. All five patients with Holt-Oram syndrome had postoperative dysrhythmias; two patients were symptomatic. Serious dysrhythmias may present years after repair of an atrial septal defect. Patients with Holt-Oram syndrome are at greater risk for development of postoperative dysrhythmias.

Restrictive interatrial communication in total anomalous pulmonary venous connection.

Restriction to systemic blood flow at the atrial level in total anomalous pulmonary venous connection (TAPVC) may play a role in the early development of congestive heart failure in some patients. Over a 4-year period, 21 patients with TAPVC without extracardiac obstruction presented from 1 day to 10 months of age (mean 2.6 months). Of 21 patients, 17 (81%) presented after 1 month of age with moderate to severe congestive heart failure. In the first week after birth 4 patients presented with minimal symptoms of congestive heart failure. A small patent foramen ovale (3 mm or less in diameter) was found in 19 of 21 patients (90%) by 2-dimensional echocardiography or angiography. Balloon or blade and balloon atrial septostomy was performed in these 19 patients and resulted in significant decreases in mean right to left atrial pressure gradient (from 2.8 to 0.25 mm Hg, p less than 0.001), systolic pulmonary to femoral artery pressure ratio (from 0.80 to 0.60, p less than 0.001), and systemic arterial oxygen saturation (from 84% to 79%, p less than 0.01). One patient had nonfatal complications. Surgery was performed in 19 patients from 2 weeks to 29 months (mean 12 months) after catheterization, with 1 operative death (5% mortality). Four patients required early total correction because of persistent heart failure; 15 had elective surgical repair. One of 2 unoperated patients died of pneumonia at 2.5 years of age. A restrictive interatrial communication develops after the first month of extrauterine life in most patients with TAPVC. Atrial septostomy results in improved hemodynamic conditions and clinical palliation.(ABSTRACT TRUNCATED AT 250 WORDS)

Repeated endomyocardial biopsy without complication in an infant after heart transplantation.

Endomyocardial biopsy is necessary for accurate diagnosis of rejection after heart transplantation. This case illustrates the safe use of repeated endomyocardial biopsies in an infant after heart transplantation.