1979
DOI: 10.1136/hrt.41.3.281
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Morphological variations in pulmonary atresia with intact ventricular septum.

Abstract: SUMmARY The morphological features of a series of 37 specimens of pulmonary atresia with intact ventricular septum were reviewed with particular emphasis on features which might influence the results of pulmonary valvotomy. The

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Cited by 133 publications
(49 citation statements)
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“…Fenton and associates [20] attributed the most common cause of interim mortality to abnormal myocardial perfusion. Others have reported myocardial abnormalities including myocardial noncompaction, disarray [22], and endocardial fibroelastosis [10]. These findings suggest that the disease is not limited to the right side, which may explain the poor prognosis of PAIVS.…”
Section: Discussionmentioning
confidence: 76%
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“…Fenton and associates [20] attributed the most common cause of interim mortality to abnormal myocardial perfusion. Others have reported myocardial abnormalities including myocardial noncompaction, disarray [22], and endocardial fibroelastosis [10]. These findings suggest that the disease is not limited to the right side, which may explain the poor prognosis of PAIVS.…”
Section: Discussionmentioning
confidence: 76%
“…Commonly, these patients have a small, hypertrophied right ventricle (RV) with supra-systemic pressure and sinusoidal communication between the RV and the coronary artery [1,2]. Many authors have noticed the possible deleterious effects associated with this situation, i.e., the progression of RV-to-coronary connections and subsequent coronary artery stenosis [3][4][5][6], potential RV 'steal' and coronary ischemia after RV decompression [4,7], impaired left ventricular compliance [8], left ventricular hypertrophy with a prominent subaortic septal bulge [9,10], fibroelastosis of the left ventricle (LV) [10], and an impaired LV function after a Fontan procedure [11] or even after biventricular repair [12]. A surgical trial to prevent these deleterious effects was first described by Waldman and associates [13].…”
Section: Introductionmentioning
confidence: 99%
“…4 The semilunar valve primordia do not appear until after the partitioning of the truncus and the completion of ventricular septation. '5 The stage at which the cusps fuse is unknown and may be variable.2' Although it must occur late relative to the development of the flow pathways of the heart, which are complete within the first month or so of fetal life, fusion may occur early enough to impair the development and differentiation of the substance of the heart the myocardium, connective tissue and blood supply.…”
Section: Origins Of Right Ventricular Dysplasiamentioning
confidence: 99%
“…In contrast to PA associated with ventricular septal defect, when the ventricular septum is intact the pulmonary annulus is almost always adequate. 4 The tricuspid annulus and hypoplastic right ventricular cavity thus provide the main restriction to forward flow after valvotomy.…”
Section: Right Ventricular Cavity and Wall Thicknessmentioning
confidence: 99%
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