Pulsed Doppler assessment of pulmonary vein obstruction.
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Publications and source records attributed to R D Rowe.
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Data derived from serial hemodynamic and angiocardiographic investigations on pediatric patients not subjected to intervening intracardiac operations support the view that subaortic stenosis in congenital heart disease tends to be a progressive disorder. Our data are obtained from two groups of patients. The first comprised 22 patients with discrete subaortic stenosis in relative isolation. The second was made up of 19 patients with the fibrous or fibromuscular forms of discrete subaortic stenosis associated with a perimembranous ventricular septal defect. The results from both groups support our initial contention. The progressive character of subaortic stenosis in these two situations illustrates the dynamic nature of congenital heart disease, and the tendency of a changing form and function.
In children with congenital aortic stenosis a modified mapping system was created to explore the electrocardiographic potentials on the chest surface from the left sternal edge (direct anterior), left axillary line (direct lateral) and midchest (45 degrees anterior to the lateral lead) in the third through the seventh intercostal spaces. Potentials were normalized according to chest size based on elliptical and cylindrical models of the chest with the heart at the center. The unadjusted and adjusted potentials were correlated with the peak systolic gradients across the left ventricular outflow tract and equations to predict the gradients were derived by stepwise multiple regression analysis. The best equation was: Gradient = -15.0 +(3.845 X MCT 4) +(0.474 X CD X LSS 3) + (0.138 X CD X MCS 3) where MCT 4 = T wave amplitude in the lead in the fourth interspace in the midclavicular line CD = AP chest diameter in cm LSS 3 = S wave amplitude in the lead in the third interspace at the left sternal border MCS 3 = S wave amplitude in the lead in the third interspace in the midclavicular line (R = 0.84, SEE = 24.3) There are areas on the chest surface that are unexplored by standard electrocardiography. The electrocardiographic potentials from these areas, when normalized for chest size, yield better estimates of transaortic gradients than previous estimates from the routine electrocardiogram.
Serial electrocardiograms and creatine kinase (CK) isoenzyme activities were studied prospectively in 20 asphyxiated term newborn infants and 43 normal neonates. By adapting a previously described grading system for ischaemic changes, a degree of electrocardiographic ischaemia was defined which occurred almost solely in asphyxiated infants. Infants with this degree of abnormality had significantly higher mean CK-MB and MM activities than other asphyxiated infants at 0, 8 and 28 hours. Histological changes of peripartum myocardial necrosis were seen in 4 of the 5 infants on whom an autopsy was performed, and either electrocardiogram or CK-MB was abnormal in all four. It is concluded that myocardial injury in the newborn period is often associated with CK-MB release, but in view of the lack of cardiac-specificity of CK-MB in newborn infants, caution is urged in the interpretation of elevated isoenzyme activity in the neonate.
Since the Fontan procedure results in low pulsatile pulmonary blood flow similar to that seen in patients with a Glenn shunt, it may also be associated with abnormal distribution of flow to the lower lung lobes and with the development of pulmonary arteriovenous fistulae (PAVF). In 12 patients 0.8 to 4.5 years after Fontan procedure and in 20 patients 0.2 to 18 years after receipt of Glenn shunts we assessed ventilation (with 133Xe) and perfusion (after a peripheral injection of 99mTc-macroaggregated albumin) to compare upper to lower lobe distribution of blood flow with that in a control group. The presence of PAVF was assessed by radionuclide activity in kidneys and the brain and by a two-dimensional echocardiographic contrast study. A decreased upper/lower lobe perfusion ratio was noted in 13 of 20 patients with Glenn shunts (65%) and correlated with the time after surgery (p less than .05). Despite the shorter follow-up period, two of 12 (16%) patients who had undergone the Fontan procedure also had a decreased upper/lower lobe perfusion ratio, and one of these developed right heart failure. Brain and kidney radionuclide counts above control values were observed in all patients with Glenn shunts and in 11 of 12 patients who had the Fontan operation. However, in only five of 20 (25%) patients with Glenn shunts were PAVF confirmed by the two-dimensional echocardiographic contrast study. Three of the five patients with PAVF had Glenn shunts of long duration.(ABSTRACT TRUNCATED AT 250 WORDS)
Physical fitness was assessed in children with congenital heart disease using seven tests designed to measure cardiovascular endurance, strength, flexibility and co-ordination. Test scores for age-matched healthy children were significantly higher than preoperative scores for patients. Postoperatively, the patients were divided into a control group and a physical training group. The training group was subsequently divided into compliant and non-compliant subgroups. Patients who complied with a simple six-week home program of physical activity training after surgery improved their test scores significantly (p less than .001) and were not significantly different from normal children. The scores of the non-compliant group remained below normal (p less than .01). When retested six months postop, the scores of all patient groups had improved; however, while there was no significant difference between the healthy and compliant groups, the scores of the non-compliant and control groups remained significantly below those of the healthy children (p less than .01).
Fifteen preterm infants who weighed 0.7 to 2.0 kg and had clinical evidence of a patent ductus arteriosus (PDA) were studied by combined 2-dimensional and Doppler echocardiography before and after the administration of indomethacin. In 10 patients the PDA was widely patent at the time of the study and in 5 the lumen was narrow. In this latter group, the PDA was narrow at the pulmonary artery end in 2 patients, in the middle in 2 patients and at the aortic end and the middle in 1 patient. After the administration of intravenous indomethacin, the PDA closed completely in 12 patients and constricted in 3. The patterns of closure could be documented in those in whom serial studies were performed. In 3 patients, closure occurred after a single dose of indomethacin, in 3 after 2 doses and in the rest after a full course of 3 doses. Doppler interrogation at the aortic and pulmonary artery end of the PDA demonstrated the shunting patterns and provided a reliable assessment of patency after the ductal lumen was outside the range of lateral resolution following constriction. In no case did the PDA reopen after the course of indomethacin. This combined approach is a reliable method of assessing a PDA before and after a course of indomethacin. It should provide the means to answer many of the questions regarding the effect of various manipulations on the PDA in the preterm infant.
Four patients with transposition of the great arteries and unilateral pulmonary vein (PV) stenosis, all left-sided, were studied. Two patients had an intact ventricular septum (1 with a patent ductus arteriosus), 1 patient had a ventricular septal defect and 1 had a ventricular septal defect with pulmonary stenosis. Clinical signs, such as recurrent pneumonia, unilateral pulmonary edema and pleural effusion, were present preoperatively in 2 patients. Diagnosis was made at cardiac catheterization by cineangiography in 2 patients and at Mustard operation when the PV orifices were inspected in the other 2. PV dilatation was attempted in 3 patients, 1 before correction (age 6 months), 1 during and after it (ages 1 and 3 years, respectively) and 1 during corrective surgery (age 15 months). In the fourth patient only the intracardiac baffle was enlarged near the left PV orifices. In the first patient, at Mustard operation (age 18 months), only a fibrotic PV without an orifice was found; this patient died after surgery. The mean follow-up in the 3 survivors was 3.2 years (range 2 months to 7.6 years). All have severe residual PV obstruction documented by technetium-99m lung perfusion scans that show decreased flow to the left lung (0 to 16% total counts; normal 45%); 2 have unilateral pulmonary edema and 1 has pulmonary artery pressure at systemic level. It is believed that in patients with transposition of the great arteries, left-sided unilateral PV stenosis is a congenital anomaly that becomes progressive as a result of postnatal preferential flow to the right lung.
Thirty-two patients with complete transposition of the great arteries (TGA) and coarctation of the aorta (C of A) were seen at The Hospital for Sick Children, Toronto, Canada, between 1963 and 1983. Three patients had only mild C of A and have not required coarctectomy (Group I); 29 had a severe form of C of A (Group II). Two patients in Group I and 21 in Group II had a ventricular septal defect. Subaortic obstruction was present in 5 patients in Group II. The mechanisms included anterior deviation of the infundibular ventricular septum, anomalous right ventricular muscle bundles, and abnormal ventriculoinfundibular fold. Five patients in Group II had a hypoplastic right ventricle. Coarctectomy was performed in 25 patients, and 5 died (20% mortality rate). Sixteen patients had repair for TGA (13 Mustard, 2 Jatene , 1 Rastelli), and 2 died (12% mortality rate). Life-table analysis shows that only 68% of the patients with TGA and C of A survived the first month of life. The 5-year survival in this group was 57%. In the same period, 94% of patients with uncomplicated TGA survived the first month of life and the 5-year survival rate was 89%.
Thirty patients are reported with atrioventricular (AV) septal defect and either coarctation of the aorta (C of A) or subaortic stenosis (SAS) or both. All patients had normal left ventricles as assessed by angiography (21 of 30 patients) or necropsy (9 of 30). Three groups were recognized. Groups I and II included 19 patients with AV septal defect (12 complete, 7 partial) and C of A with or without SAS, 11 patients with AV septal defect (5 complete, 6 partial) and SAS. In Group I, preductal C of A was diagnosed in 16 of 19 patients. Concomitant angiographic evidence of SAS was present in 2 cases, the mechanism being exaggerated anterior displacement of the left AV valve. In Group III, at the time of diagnosis left ventricular-aortic peak systolic pressure gradients of greater than 20 mm Hg were present in 9 patients, 2 of whom had gradients greater than 50 mm Hg. Angiographic diagnoses were: discrete fibrous diaphragm in 4, fibromuscular obstruction in 5, dynamic tunnel in 1, and chordae from left AV valve to LV outflow tract in 1. Thus, SAS in AV septal defect is most often due to a discrete anatomic lesion. Hemodynamic data show that SAS can be progressive, both before and after the surgical management of the AV septal defect.
Bilateral ductus arteriosus (DA) was clinically recognized in 27 patients studied angiographically from 1963 through May 1983. Distal bilateral DA origin of non-confluent pulmonary arteries was identified in 15 patients, ectopic or distal ductal origin of 1 pulmonary artery in 9 patients (5 without evidence of intracardiac disease) and isolation of the left subclavian artery in 3 (all 3 of whom had a right aortic arch). Other conditions reported to be associated with bilateral DA include interruption of the aortic arch with isolation of a subclavian artery, aortic atresia with interruption of the aortic arch in which bilateral DA supports the entire systemic circulation, bilateral DA complicating forms of congenitally malformed hearts other than those just stated, and, rarely, bilateral DA in isolation. Understanding the symmetric or paired nature of the primitive aortic arch system in the developing human heart facilitates recognition of the patterns of fourth and sixth arch anomalies seen with bilateral DA.
Seventeen patients with a univentricular atrioventricular connection and subaortic stenosis at the outlet foramen level were seen from 1974 until 1983. The ventricular anatomy was of a single left ventricle with an outlet chamber, and the great arteries were transposed. This condition often followed pulmonary artery banding (15 cases) but also occurred with pulmonic stenosis (one) or pulmonary atresia (one). The median interval to the detection of subaortic stenosis after banding was 2.3 years (range 11 days to 15 years). Thirteen patients had a resting gradient to 118 mm Hg and three a gradient only with isoprenaline infusion. The following surgical procedures have been performed: (1) insertion of a conduit between the left ventricle and aorta (one patient); (2) creation of an aorticopulmonary window proximal to the band and tightening of the band (three patients); (3) enlargement of the ventricular septal defect (three patients); (4) Fontan procedure (five patients); and (5) Fontan procedure plus arterial switch (two patients). All of the procedures but the last one have met with significant mortality in this difficult group of patients.
An enlarged right ventricle and abnormal ventricular septal motion are characteristic echocardiographic features of atrial septal defect and often persist after the defect has been completely closed, even when the operation clinically is judged to be successful. These features were examined retrospectively 15 to 21 months after operation in a group of children whose atrial septal defect had been closed between January 1976 and July 1979. Despite satisfactory postoperative results in all, about two thirds had an enlarged right ventricular dimension and about the same number had abnormal septal motion when examined echocardiographically an average of 18 months after operation. The best operative strategy seems to be to operate while the right ventricular end-diastolic dimension is still relatively small in echocardiographic terms.
Between June, 1977, and December, 1982, 106 infants underwent subclavian flap arterioplasty for repair of coarctation of the aorta in the first year of life. This technique was utilized because of our previous experience with the end-to-end technique in 181 infants, in whom there was a recurrence rate of 42% at 5 years. The recurrence rate in 78 of the 81 survivors who have been followed up for a mean of 18.8 months +/- 15.9 months (standard error) is 17.3% at 1 year and 11.1% at 2 and 3 years postoperatively. The presence of associated disease affects mortality: only one death in 34 patients with isolated coarctation; three deaths in 24 with coarctation plus ventricular septal defect; and 21 deaths in 45 infants with coarctation and complex lesions. Our present approach in the group with coarctation plus ventricular septal defect is to repair the coarctation without banding the pulmonary artery and then to close the defect if the infant remains in failure. Operative survival was 13 of 13 without pulmonary artery banding and 11 of 14 with pulmonary artery banding.
Forty-one patients with subaortic stenosis (SAS) and ventricular septal defect (VSD) were identified from the cardiac records of the Hospital for Sick Children, Toronto, Ontario. The diagnosis of an associated SAS was made clinically in only 1 patient, who had findings of left ventricular (LV) hypertrophy with strain on the electrocardiogram. There was a delay of 3.1 years between initial presentation and detection of SAS. The SAS was not diagnosed at initial catheterization in 17 patients and was confirmed at subsequent catheter studies in 8 patients, surgery in 5 and autopsy in 4. Associated defects included coarctation of the aorta in 12 patients, mitral valve abnormalities in 4, and right-sided obstructions, including anomalous right ventricular muscle bundles in 6 patients, tetralogy in 4 and pulmonic stenosis in 1 patient. The mean gradient across the LV outflow tract was 25 mm Hg. Nineteen patients had serial catheters without intervening surgery, and the outflow gradient increased from a mean of 9 to 36 mm Hg. The mechanism of SAS consisted of fibrous diaphragm and fibromuscular obstruction in 31 cases, muscular narrowing in 4, protruding tricuspid valve leaflet in 2, hypertrophic cardiomyopathy in 2, anterolateral twist in 1 patient and redundant tissue tag in 1. Thirty-eight patients had a perimembranous VSD, 19 of whom had an associated so-called aneurysm of the membranous septum; 2 had an infundibular VSD and 1 patient had a central muscular defect. Although the SAS was located below the VSD in 30 cases, the associated heart failure and reduced cardiac output can mask the presence or severity of associated SAS.(ABSTRACT TRUNCATED AT 250 WORDS)
Pulmonary vein wedge angiography was applied to 25 patients ranging in age from 4 months to 16 years. The indications for this technique include (1) determination of the presence or absence of central (intrapericardial or mediastinal) pulmonary arteries in patients with pulmonary atresia where standard anterograde injections of contrast medium do not demonstrate these, (2) determination of the patency and caliber of a nonvisualized pulmonary artery when there is distal "occlusion" by a systemic-to-pulmonary artery anastomosis, (3) determination of the patency and caliber of a nonvisualized pulmonary artery when previous pulmonary arterial banding has led to acquired pulmonary artery atresia, and (4) determination of the presence of a mediastinal pulmonary artery in patients with so-called isolated congenital unilateral absence of a pulmonary artery. Surgical correlates to the interpretation of pulmonary vein wedge angiograms are provided in 18 patients. The present data suggest that pulmonary vein wedge angiography is a safe technique that provides considerable information about (1) the caliber of the parenchymal pulmonary arteries, (2) their sizes at the hilum of the lung, and (3) the presence or absence of a mediastinal confluence of pulmonary arteries. This technique may also complement those anatomic findings derived from anterograde injection of contrast material in patients with pulmonary atresia of a congenital or acquired nature.
Two-dimensional echocardiography (2DE) was used to study 51 neonates in whom coarctation of the aorta had been diagnosed clinically. In 40 patients, studies showed coarctation or arch interruption that was later confirmed at angiography, surgery, or autopsy. Of the remaining 11 studies, one gave a false positive result, one was technically poor, three had echocardiographic evidence of only mild arch narrowing and are being followed clinically, and six were negative. There were two false negative studies. Four studies were apparently true negatives. Many additional lesions were correctly identified by echocardiography although some, such as patent ductus arteriosus and small ventricular septal defect, were frequently missed. Twelve patients underwent surgery without preoperative catheterization. 2DE has proven extremely useful in confirming the diagnosis of neonatal coarctation of the aorta and sometimes obviates the need for invasive catheterization.
A case report details the operative technique used in the repair of complete transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis in a patient who had had Baffes procedure previously. Experience in managing 10 patients with complete TGA who had undergone a Baffes procedure is discussed. Four patients with a large VSD had pulmonary vascular disease. The condition of 1 of them was improved by a palliative Mustard operation. Four children with isolated TGA underwent a successful modified Mustard repair. The 2 remaining patients had a VSD and pulmonary stenosis; in 1 the condition was palliated by a Glenn shunt. The other is the subject of the case report. The mean interval between the Baffes procedure and the second operation was 11 years. There was 1 late death after secondary repair. Follow-up in the remaining 6 patients at a mean of 10.6 years indicates a favorable outcome.