Left atrioventricular valve atresia: clinical management.
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Adolescent↗
Biomedical subjects
C C Lenox
Publications and source records attributed to C C Lenox.
Iliac venous thrombosis in infants and children after cardiac catheterization.
Twenty-two patients developed thrombosis of the lower abdominal portion of the inferior vena cava, iliac or femoral vein (IVT) after cardiac catheterization. All patients has at least one previous study from 1 day to 11 months of age (mean, 2 months) and seven had two catheterizations before discovery of IVT. Transposition of the great arteries was the most common defect associated with IVT (12/22, 55%). From one to seven catheter changes were made during the time of venous cannulation (mean, 105 minutes). No clinical evidence of IVT was present immediately after the preceding cardiac catheterization. The discovery of significant IVT usually necessitated the use of a vein from the upper extremity to complete the cardiac catheterization. IVT was associated with previous balloon atrial septostomy using either a Fogarty or Rashkind septostomy catheter in 13/22 patients (59%). In addition, 86% of the patients had either of these balloon catheters or a Swan-Ganz catheter used during a previous study. The potential development of IVT should be considered especially in cyanotic infants and small children in whom balloon catheters are used.
Cardiac Catheterization↗
Anomalous left pulmonary artery: successful management.
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Adolescent↗
Pneumonectomy for intractable left bronchial compression in d-transposition of the great arteries.
In infants with transposition of the great arteries (TGA) tracheobronchial obstruction may occur as a result of compression by a dilated posterior pulmonary artery, especially if there is a large interventricular septal defect with its attendant large pulmonary blood flow and high pressure. Banding of the pulmonary artery may give temporary relief. A patient with TGA had collapse of the left lung at 2 months of age, following atrial septectomy. Bronchoscopy and bronchography revealed extrinsic compression of the left main bronchus. Banding of the pulmonary artery at 6 months of age relieved the respiratory distress. At 8 years of age atelectasis of the left lung recurred following a Mustard procedure. Two additional procedures were attempted to decrease the pressure of the pulmonary artery on the bronchus but these were unsuccessful. At age 9 years, pneumonectomy, her sixth thoracotomy, was performed as a final attempt to relieve the respiratory problem caused by persistent atelectasis. She is now 17 years of age and has remained asymptomatic during the last 8 years.
Adolescent↗
Echocardiographic and hemodynamic correlation in transposition of the great arteries.
Echocardiography was performed in 36 patients with transposition of the great arteries (TGA). Twenty patients were studied before a Mustard operation, 14 patients after operation and two patients both before and after operation. Right ventricular end-diastolic dimension (RVED) was larger than normal in each patient and tended to increase postoperatively. In contrast, the left ventricular end-diastolic dimension (LVED) was significantly reduced postoperatively in all patients. A linear relationship was demonstrated between the ratio of LVED/RVED and the ratio of peak systolic pressures in the left and right ventricles in studies both before and after Mustard operation. Systolic anterior motion of the mitral valve was observed in 18% of peroperative patients and increased to 44% postoperatively. The incidence of fluttering of the mitral valve increased from 50% to 94% after the operation. Abnormal septal motion was found in 39% of cases. Abnormal movement of the mitral valve and of the interventricular septum seems to be related to a reversed pressure relationship in the ventricles. Shifting of the ventricular septum toward the left ventricle and consequent distortion of the left ventricular cavity and mitral valve apparatus may be responsible for the abnormal echocardiographic findings. Serial echocardiographic studies may be useful as a noninvasive tool in the assessment of left ventricular pressure or the status of the pulmonary vascular bed in TGA.
Adolescent↗
Left ventricular anomalies associated with Ebstein's malformation of the tricuspid valve.
Seventeen patients with Ebstein's malformation of the tricuspid valve have been evaluated. The majority of patients (11/17) presented in the newborn period with cyanosis and cardiomegaly. The remainder (6/17) were referred because of cardiomegaly on chest X-ray and/or a heart murmur heard on routine examination. Three patients have died; one in the newborn period and two suddenly at the age of 11 and 15 years. Thirteen patients have undergone right and left heart cardiac catheterization within the last seven years. Twelve of these 13 patients (92%) had angiocardiographic left ventricular contraction abnormalities. Five patients also had mitral valve prolapse associated with left ventricular dysfunction. Since many patients with Ebstein's anomaly may have significant left ventricular abnormalities, careful and systematic evaluation of the left ventricle is warranted.
Adolescent↗
Clinical use of blade atrial septostomy.
A cardiac catheter enclosing an extensible blade was used to enlarge the interatrial opening in seven patients. Two patients with transposition of the great arteries who had balloon atrial septostomy as newborns subsequently presented with clinical evidence of a restrictive interatrial opening at 1 and 4 months of age. Cardiac catheterization confirmed restenosis of the interatrial opening and inadequate intracardiac mixing. After blade atrial septostomy the systemic arterial oxygen saturation increased by 20% and 30%, respectively. Five patients with mitral atresia complex, ages 2 months-9 1/2 years, had a restrictive interatrial communication and severe pulmonary venous hypertension (mean left atrial pressures ranged from 20-38 mm Hg). Following blade atrial septostomy, the pressure gradient between the atria was almost completely abolished and prompt clinical improvement was observed in each patient. All patients tolerated the procedure without complications. Blade atrial septostomy was a safe, effective procedure for enlarging the interatrial communication in this limited series of patients with an interatrial septum too thick to permit adequate rupture by conventional balloon atrial septostomy.
Age Factors↗
Bilateral ductus arteriosus in d-transposition of the great arteries with right aortic arch.
The rare anomaly of a right aortic arch, distal origin of the left subclavian artery, and posterior left ductus arteriosus (forming a vascular ring) plus a right ductus arteriosus is described in an infant with d-transposition of the great arteries with an intact septum. The presence of a right aortic arch and distal left subclavian artery with bilateral ductus arteriosus has not been described previously.
Aorta, Thoracic↗
Arrhythmias and Stokes-Adams attacks in acute rheumatic fever.
In spite of general complacency about first-degree heart block in acute rheumatic fever, abnormal conduction with dysrhythmias, occasional complete heart block, and, rarely, Stokes-Adams attacks are important early signs of acute rheumatic fever and may precede other signs. Every person with episodic fainting is entitled to an ECG, and frequent ECGs are imperative in any case of rheumatic fever with signs of arrhythmias. Changing atrioventricular block necessitates continuous monitoring for dysrhythmias. A 13-year-old boy who appeared with Stokes-Adams attacks secondary to acute rheumatic fever was successfully treated by temporary pacing.
Acute Disease↗
Double-outlet right ventricle with restrictive ventricular septal defect.
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Angiocardiography↗
Pulmonary perfusion defect and bronchial artery collateral blood flow.
A five-year-old child with severe congestive heart failure following repair of tetralogy of Fallot was examined for correction of a suspected left-to-right shunt. Lung scan and selective bronchial angiographs demonstrated greatly diminished perfusion to the right upper lobe, which was supplied by a large bronchial artery. Technetium microspheres were injected into the artery for quantification. Subsequently, the artery was occluded by injection of the tissue-adhesive bucrylate. During the following year, radionuclide scans showed improvement in the uniformity and percentage distribution of pulmonary perfusion by way of the pulmonary artery. The child has become asymptomatic following closure of the bronchial vessel.
Bronchi↗
Down syndrome with congenital heart malformation.
Two hundred fifty-one patients with Down syndrome and congenital heart disease was based on clinical (41%), catheterization (38%), surgical (11%), or autopsy data (10%). The most common lesions were endocardial cushion defect (43%), ventricular septal defect (32%), secundum atrial septal defect (10%), tetralogy of Fallot (6%), and isolated patent ductus arteriosus (4%). Thirty percent had multiple cardiac defects. The most common associated lesions were patent ductus arteriosus (16%) and pulmonic stenosis (9%). Twenty-five percent of the patients uncerwent cardiac surgery. Motality in the 68 patients undergoing surgery was 26% for open heart procedures and 11% for closed heart surgery. In 32% of nonsurgically treated patients with large left-to-right shunts, irreversible pulmonary vascular disease developed. Improved medical and surgical care have decreased morbidity and mortality in these patients in recent years.
Abnormalities, Multiple↗
Pulmonary artery wedge pressures in congenital heart disease.
The bilateral measurement of pulmonary artery wedge pressure is essential in the cardiac catheterization evaluation of all patients with pulmonary artery hypertension. Five cases of pulmonary venous obstruction are presented, 4 of whom had additional intracardiac defects. The pulmonary artery hypertension in these 4 patients was initially attributed to the associated cardiac anomalies and because the left atrium had been entered directly with the catheter in each case, pulmonary artery wedge to the left atrium pressure gradients were initially either not obtained or were discounted as being artificial. Pulmonary venous obstruction was recognized in all 5 cases on the basis of an elevated pulmonary artery wedge pressure, and the anatomic site of the obstruction was successfully documented. Unless bilateral pulmonary artery wedge pressures are measured in all patients with pulmonary artery hypertension regardless of the presence of additional cardiac anomalies, a surgically correctable cause of pulmonary hypertension may be overlooked.
Blood Pressure↗
Coarctation of the aorta with ventricular septal defect.
From 1965 to 1974, 53 children with coarctation of the aorta (COA) and an associated ventricular septal defect (VSD) underwent cardiac catheterization. Thirty-one patients presented with congestive heart failure. Twenty-five of 27 patients (92%) who underwent cardiac catheterization under age 3 months had either systemic hypertension, a systolic gradient across the coarctation greater than 20 mm Hg or both. Pulmonary hypertension was present in all 25 patients. COA repair was performed in 39 patients and there were seven deaths. Of the 32 survivors, 23 have no residual gradient; six are normotensive but have a mild residual gradient; three are hypertensive or have a gradient greater than 20 mm Hg. Repair of the VSD or pulmonary artery banding has been performed in 11 of 44 patients who survived infancy. Spontaneous closure of the VSD has occurred in three cases and 25 patients have a small VSD that does not warrant surgical repair. Surgical repair of COA during infancy may be unavoidable but conservative medical management of the associated VSD is often successful.
Aortic Coarctation↗
Tetralogy of Fallot: postoperative electrophysiologic studies.
Electrophysiologic studies performed during postoperative cardiac catheterization in 51 patients following repair of tetralogy of Fallot (TF) were compared with studies performed in a control group of 30 patients. These studies include His bundle electrograms, measurement of atrioventricular (A-V) conduction at progressively increasing atrial pacing rates, and evaluation of sinus node recovery time. More postoperative TF patients (20%) had prolonged H-V intervals compared to the controls (7%). With atrial pacing, no patient in the control group developed second degree A-V block below 160 beats/min, while 23% of the postoperative TF patients developed block below this level. Half of these patients had normal His bundle studies. Stress of the A-V conduction system by atrial pacing may unmask conduction abnormalities not present on the surface electrocardiogram or on His bundle electrogram. Two-thirds of the patients had some form of conduction abnormality on the basis of evaluation of one or more parameters but about half of the patients with a given abnormality had normal findings in other areas. The high incidence of electrophysiologic abnormalities following TF repair may have future prognostic significance. Systematic evaluation of atrioventricular conduction during preoperative and postoperative cardiac catheterization may be of value.
Adolescent↗
Valve replacement in children and adolescents.
Replacement of diseased heart valves has become the treatment of choice in many adults. Valve replacement in older children and adolescents has been done in our institution only when other procedures are ineffective. Over the past 10 years, 24 patients have received 28 artificial valves. Thirteen (54 per cent) had rheumatic heart disease; the remainder had congenital or acquired valvular abnormalities. The Björk-Shiley valve was most commonly used in the aortic position and the Beall prosthesis in the mitral position. Sixteen patients (67 per cent) survived operation and have been followed from 3 to 79 months, averaging 27 months. All are free of cardiac symptoms. Hemarthrosis occurred once in 2 survivors (13 per cent). Eight patients died (33 per cent), 5 immediately postoperatively and 3 within a year after the operation. The mortality rate has declined to 21 percent during the past 3 years. Our experience in the pediatric age group suggests that valve replacement is a serious undertaking with higher mortality rate than in adults. The operation should be reserved for those patients in whom valvuloplasty or valvulotomy is expected to be ineffective.
Adolescent↗
Management of bilateral ductus arteriosus in complex cyanotic heart disease.
Bilateral ductus arteriosus (BDA) usually is associated with complex cyanotic heart disease. Since pulmonary valve atresia often is part of the complex, hypoxia may necessitate emergency cardiac catheterization and surgery for these critically ill newborn infants. Optimum management depends on accurate delineation of the intracardiac and great vessel anatomy. Since the ductus arteriosus has a tendency to close spontaneously, the true anatomy of the fourth to sixth aortic arch connections should be determined on the first catheterization. An over-all plan for future care by the medical-surgical team should have been made at the time of the initial surgical procedure. The case histories of four newborn infants with BDA associated with cyanotic heart diseases are reported. The anatomy and basic embryology of the fourth to sixth arch system is reviewed and recommendations for long-term management are given.
Aorta, Thoracic↗
Continuous murmur following Mustard operation for transposition of the great arteries. A sign of pulmonary venous obstruction.
Three patients developed severe pulmonary venous obstruction following Mustard operation for transposition of the great arteries. Each patient had a soft continuous murmur with distinct diastolic accentuation at the low left sternal border or xiphoid area. Simultaneous recording of intracardiac sound and pressure in one patient showed that the murmur originated at the site of obstruction in the surgically-constructed pulmonary venous atrium. Selective cineangiograms demonstrated the baffle to be the cause of the obstruction. A continuous murmur in a patient following Mustard operation may suggest significant pulmonary venous obstruction.
Child, Preschool↗