Biomedical subjects
J A Ettedgui
Publications and source records attributed to J A Ettedgui.
Echocardiographic-morphologic correlations in tricuspid atresia.
OBJECTIVES: Our aim was to clarify the anatomic substrate in hearts diagnosed as having tricuspid atresia by studying autopsy specimens and comparing the findings with those in two-dimensional echocardiograms. BACKGROUND: Traditionally, tricuspid atresia was thought, and is still believed by some, to be due to an imperforate valvular membrane interposed between the floor of the blind-ending right atrium and the hypoplastic right ventricle. Others argued that the dimple, when present, pointed to the outflow tract of the left ventricle rather than to the right ventricle, making the lesion more akin to double-inlet left ventricle. METHODS: We examined 39 autopsy specimens catalogued as having tricuspid atresia. We then studied 24 two-dimensional echocardiograms from patients with a primary diagnosis of tricuspid atresia. RESULTS: Of the 39 specimens, 37 had a completely muscular floor to the right atrium (absent right atrioventricular [AV] connection). The dimple, when identified, was (except in one case) directed to the left ventricular outflow tract. Only two hearts had an imperforate tricuspid valve. Two-dimensional echocardiograms in all cases showed an echo-dense band, produced by the fibrofatty tissue of the AV groove and representing absence of the right AV connection, between the muscular floor of the morphologically right atrium and the ventricular mass. CONCLUSIONS: Tricuspid atresia is usually, but not always, due to morphologic absence of one AV connection. In most cases, the ventricular mass then comprises a dominant left ventricle together with a rudimentary and incomplete right ventricle.
Is a cleft in the anterior leaflet of an otherwise normal mitral valve an atrioventricular canal malformation?
OBJECTIVES: This study sought to ascertain the surgical anatomy of a cleft in the left atrioventricular (AV) valve. BACKGROUND: Important morphologic differences exist between hearts with a cleft in the anterior leaflet of an otherwise normal mitral valve and those with a so-called cleft in the left AV valve when there is an AV septal defect, but it has been customary to link the lesions together on developmental grounds. METHODS: Eight autopsied specimens with a cleft in the aortic (or anterior) leaflet of the mitral valve were studied in detail, and echocardiograms from 21 patients with such a cleft were compared with the specimens and with findings typical of the so-called partial AV canal and other forms of AV septal defect. RESULTS: The structure and direction of the cleft, location of the papillary muscles within the left ventricle and AV junctional morphology of hearts with an otherwise normally structured mitral valve were significantly different from typical findings in hearts with AV septal defects. CONCLUSIONS: It is necessary to distinguish morphologically a cleft in an otherwise normally structured mitral valve in hearts with separate right and left AV junctions from the trifoliate left component of a common AV valve in hearts with an AV septal defect and a common AV junction because the disposition of the AV conduction tissues varies markedly between the lesions.
Transatrial lead placement for endocardial pacing in children.
Transvenous placement of endocardial leads in children may be difficult due to restrictions and complications of vascular access. We have placed endocardial leads from a transatrial approach in 5 children with various cardiac malformations. The usual surgical approach involved an anterolateral thoracotomy and, under fluoroscopic guidance, passage of the lead tip directly through the right atrial wall and across the tricuspid valve to the apex of the right ventricle. At a mean follow-up time of 23.2 months (range, 12.0 to 27.9 months), all patients have low thresholds for myocardial capture, and there have been no complications. We conclude that placement of endocardial leads by a transatrial approach provides an excellent alternative to an epicardial system in children destined for lifelong pacing.
Images in cardiovascular medicine. Profiles of sinus venosus atrial septal defect.
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Long-term results of survivors of surgical valvotomy for severe aortic stenosis in early infancy.
Long-term morbidity and mortality were evaluated in the 21 survivors of a cohort of 51 consecutive infants with severe aortic valve stenosis who underwent surgical treatment in the first 3 months of life during the period from 1958 to 1988. The 21 early survivors have been followed up from 3 to 27 years (median 7.5 years). There have been two late deaths: one at age 13 year from bacterial endocarditis and the other at age 14 years after dislodgment of a prosthetic valve. The calculated 10-year actuarial survival for this group is 100%, with a 15-year actuarial survival of 75% (standard error 15%). Seven repeat operations have been performed in six patients: Three had persistent stenosis and a repeat valvotomy was performed in two of them, aged 2 years and 15 years. The other underwent placement of a conduit from the left ventricle to the descending aorta at 2 years of age. Replacement of the aortic valve has been performed in four patients because of severe valvular insufficiency 13 to 27 years after the initial operation. One of these had required a repeat valvotomy at the age of 15 years. The calculated actuarial freedom from reoperation at 10 years is 90% (standard error 6%) and at 15 years, 67% (standard error 15%). Aortic insufficiency was progressive throughout the period of follow-up. No patient had more than moderate aortic insufficiency 3 to 5 years after the initial valvotomy, whereas aortic insufficiency was severe in five of the eight patients followed up for 11 or more years. Progression of aortic insufficiency and the need for reoperation were not related to the age at initial valvotomy. Survivors of surgical aortic valvotomy in early infancy have a relatively good long-term prognosis and a high freedom from reoperation in the period leading to adolescence. Aortic insufficiency in these patients is progressive, and valve replacement eventually may be required.
Experience with a newly developed pericardiocentesis set.
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Absent pulmonary valve syndrome with ventricular septal defect: role of the arterial duct.
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Usefulness of the internal jugular venous route for cardiac catheterization in children.
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Carotid artery approach for balloon dilation of aortic valve stenosis in the neonate: a preliminary report.
Balloon valvuloplasty in neonates with severe aortic valve stenosis is limited by difficulties in catheter manipulation around the arch and across the valve and by the risk of femoral artery complications. A right common carotid artery cutdown was utilized for balloon aortic valvuloplasty in five neonates 1 to 20 days of age, weighing 3.1 to 3.9 kg. Standard balloon valvuloplasty was performed through a 6F sheath inserted in the right carotid artery. The arteriotomy was repaired at the end of the procedure. Mean left ventricular systolic pressure was reduced from 142 to 97 mm Hg, with a decrease in mean peak systolic pressure gradient from 76 to 33 mm Hg. Only one patient developed mild aortic regurgitation. One patient with a hypoplastic left ventricle died, and one patient required open valvotomy. All four survivors have a normal carotid pulse and no neurologic sequelae. Two of these patients required repeat balloon dilation to treat residual aortic valve stenosis at 8 and 10 months of age, respectively. Balloon valvuloplasty using a carotid artery approach is feasible and was safe in five neonates with severe aortic valve stenosis.
Diagnostic echocardiographic features of the sinus venosus defect.
To establish the diagnostic criteria for a sinus venosus atrial septal defect cross sectional echocardiograms, cineangiograms, and surgical notes of all patients with this diagnosis seen at the Children's Hospital of Pittsburgh between 1986 and 1988 were reviewed. Seven patients were identified. In each the extent of the atrial septum and the nature of the junction of the superior vena cava with the atria were evaluated echocardiographically from the subcostal position. All had overriding of the superior vena cava and abnormally connected right pulmonary veins. Six patients had undergone cardiac catheterisation and cineangiography. Five patients underwent surgical repair. The operative findings were consistent with the expected morphology in all five, and these features were additionally confirmed in a specimen from the cardiopathological museum. Therefore, the basic anatomical feature of a superior sinus venosus interatrial communication is a biatrial connection of the superior vena cava. This, together with anomalous drainage of the right sided pulmonary veins, results in an interatrial communication outside the confines of the true atrial septum. Overriding of the superior vena cava across the upper rim of the oval fossa is suggested as the pathognomonic diagnostic feature that can clearly be demonstrated echocardiographically from the subcostal position. In essence the lesion is an interatrial communication rather than an atrial septal defect.
An evaluation of right and left ventricular function after anatomical correction and intra-atrial repair operations for complete transposition of the great arteries.
Anatomical correction of complete transposition of the great arteries has the potential advantage over intra-atrial repair in that the left ventricle becomes the systemic pump. To investigate the importance of this, we evaluated right and left ventricular function in 21 patients after anatomical correction and in 21 patients after Mustard or Senning operations. First-pass and equilibrium-gated radionuclide angiography were used to measure right and left ventricular ejection fractions between 17 and 78 (mean, 47) months after anatomical correction and between 3 and 187 (mean, 67) months after intra-atrial repair. The mean age of the patient groups at the time of study was 52 and 84 months, respectively. The right ventricular ejection fraction ranged from 35% to 78% (mean, 58%) in patients after anatomical correction and from 27% to 68% (mean, 51%) after intra-atrial repair (p = 0.066). The left ventricular ejection fraction ranged from 39% to 74% (mean, 58%) after anatomical correction and from 35% to 74% (mean, 58%) after intra-atrial repair (p = 0.86). The mean right and left ventricular ejection fractions of both groups were significantly lower than those of normal children. Individuals with systemic ventricular dysfunction were identified after both types of operations; however, symptomatic dysfunction occurred only after intra-atrial repair (p = 0.24).
Noninvasive diagnostic features of partial absence of the pericardium.
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Corrected transposition presenting as aortic stenosis in an infant.
Associated intracardiac lesions have been well documented in congenitally corrected transposition. Most often these include ventricular septal defects, dysplasia of the morphologically tricuspid valve and subpulmonic stenosis. Aortic stenosis as the only isolated intracardiac defect in association with corrected transposition has not been described previously. We present such a patient and review the clinical features of corrected transposition.
A quantitative evaluation of aortic regurgitation after anatomic correction of transposition of the great arteries.
Twenty patients who had undergone anatomic correction of transposition of the great arteries were assessed by Doppler echocardiography or cardiac catheterization, or both, to identify the presence of aortic regurgitation. The severity of aortic regurgitation was evaluated by radionuclide angiographic measurement of the stroke volume index a mean of 47.1 months postoperatively. The stroke volume index was defined as the ratio of the stroke counts between the left and right ventricles. A value greater than 1.8 was considered to indicate significant left ventricular volume overload. Eight patients (40%) were shown to have various degrees of aortic regurgitation by Doppler echocardiography or cardiac catheterization, or both. The mean (+/- SD) stroke volume index was 1.03 +/- 0.15 in these patients and 1.01 +/- 0.21 in the 12 patients without aortic regurgitation (p = NS). The stroke volume index was not above the normal range in any patient, indicating that the degree of aortic regurgitation present was trivial. This medium-term study indicates that trivial or mild aortic regurgitation is a frequent finding after anatomic correction of transposition of the great arteries. However, it rarely results in an audible cardiac murmur or significant left ventricular volume overload. Long-term evaluation is required to determine its importance.
The pathology of balloon pulmonary valvoplasty.
The pulmonary valve was examined following balloon dilation of pulmonary valve stenosis in six children. Stenosis was due to commissural fusion in two. In these patients, cineangiography showed that the valve was slightly thickened and "domed" during systole. Balloon valvoplasty produced complete separation of fused commissures in one and partial separation in the other. Commissural splitting is likely to be the most common mechanism of relief of pulmonary stenosis by balloon dilation. Incomplete separation could account for the partial reduction of the gradient in most children treated with this technique. Four children had pulmonary valve dysplasia. Balloon valvoplasty did not produce haemodynamic improvement or morphological changes that were identifiable at the time of surgical correction. Tearing of pulmonary valve tissue and avulsion of pulmonary cusps were not seen in this series.
Complete heart block as a complication of varicella.
A case of varicella myocarditis complicated by permanent complete heart block requiring treatment with a permanent pacemaker is presented. Antiviral therapy with Acyclovir from the moment of presentation had no effect on the course of the heart block, but may have induced recovery of myocardial contractile dysfunction.
The value of counter-current aortography in infants.
Counter-current aortograms were performed on five infants weighing from 1.6 to 2.8 kg. All had symptoms suggestive of congenital heart disease. The images obtained were all of diagnostic quality. In only one, who had anomalous origin of the subclavian artery on the side chosen for the injection, was the diagnosis not made by this technique. No complications directly attributable to the procedure occurred. Counter-current aortography is a safe and effective minimally invasive method of elucidating the anatomy of the aortic arch in young infants.