Postoperative pathology of congenital heart disease.
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Biomedical subjects
Publications and source records attributed to G Thiene.
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Cardiac valvular involvement in patients with rheumatoid arthritis is rare and seldom so severe as to require surgery. The present report is concerned with a case of rheumatoid aortic incompetence successfully treated by prosthetic valve replacement. As suggested by previous similar reports, surgery is the most effective therapy for clinically significant rheumatoid endocarditis.
Sixty-two hearts without a patent exit from the left atrium to the ventricular mass have been studied. All had situs solitus and laevocardia. The material consisted of 32 cases with coexistent aortic atresia and 30 cases with patent aortic root. Five hearts with aortic atresia were biventricular with atrioventricular concordance and imperforate left atrioventricular valves, and 27 hearts were univentricular of right ventricular type, with absent left atrioventricular connection. The anatomy of this group was uniform, with extreme hypoplasia of all the left cardiac segments. Among the cases with patent aortic root, five were biventricular, with atrioventricular concordance and imperforate left atrioventricular valves, and 24 had absent left atrioventricular connection, 15 with univentricular heart of right ventricular type and nine with univentricular heart of left ventricular type. The final heart had double inlet univentricular of left ventricular type with an imperforate left atrioventricular valve. In this second group the aorta was larger in cases with discordant ventriculoarterial connection or in those with double outlet from the main ventricular chamber. A normal sized aorta without aortic arch obstruction was observed in nine instances. These are of great interest in terms of surgical anatomy since definitive palliation may be feasible.
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The case is presented of a 31 year old man with anteroseptal myocardial infarction, whose chest X-ray showed an anomalous protrusion along the left cardiac border. After 6 months, the increase in size of such protrusion lead to left ventriculographic and coronariographic examination: the presence of a large nonfistulous bisaccular coronary aneurysm was documented along the anterior descending branch of the left coronary artery. Cardiac surgery consisted in resection of the coronary aneurysm and of the left ventricular post-infarction area, and was followed by good surgical and angiocardiographic result. Histological examination showed the wall of the coronary aneurysm to be composed of collagenous bands in the absence of elastic components, with hemosiderin and fibrino-hematic deposits and scattered granulocytic infiltrates. An infective etiology is hypothesized for the coronary parietal lesion, leading to a coronary pseudo-aneurysm an subsequent myocardial infarction.
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Several investigations have recently been published which suggest that classical tricuspid atresia is produced by an imperforate valve membrane interposed between the right atrium and the underlying hypoplastic right ventricle. Since this is contrary to our understanding of the anatomy of the majority of examples of tricuspid atresia, and since a knowledge of the basic anatomy is vital for correct clinical diagnosis, particularly using two-dimensional echocardiography, we have reviewed the morphology of the lesions which can produce "tricuspid" atresia. For this purpose, we have considered all those lesions which produce blockage of direct exist from the morphologically right atrium in patients with situs solitus. In some instances, the lesion may not in fact affect a morphologically tricuspid valve, and for this reason right atrioventricular valve atresia is a more accurate term for the group. But because of the widespread clinical use and understanding of tricuspid atresia as describing anomalies with no other exit from the morphologically right atrium, we have used this term. In essence, tricuspid atresia as thus defined can be due to either an imperforate atrioventricular valve membrane or to absence of the right atrioventricular connexion. Contrary to widely held opinion, the commonest type of classical tricuspid atresia is due to absent right connexion. In this form, atrioventricular sulcus tissue extends in to the septal atrioventricular junction, completely separating the floor of the right atrium from the ventricular myocardium. The right ventricular chamber is rudimentary, lacking an inlet. Cases with atrioventricular concordance and an imperforate tricuspid valve can exist but are rare. Such cases, when associated with Ebstein's malformations, may have right atrioventricular annuli sufficiently large to permit insertion of a prosthetic valve, so are of major clinical significance. Rarer forms of tricuspid atresia can be produced with either absent connexions or imperforate membranes in association with other chamber combinations. We have sectioned heart specimens to show the salient clinical anatomy of these various forms, and constructed diagrams to show the features of the rarer types. Finally, we have discussed the relationship of these various forms of tricuspid atresia to the univentricular heart, showing how most disagreements relate to semantics.
In this paper we have reviewed the salient features of the anatomy of mitral atresia which should be of interest to the clinician. In the preceding paper, we reviewed the comparable features of tricuspid atresia, defined as lack of exit from the systemic venous atrium, even though some of the lesions may have affected a morphologically mitral valve. In this paper, we consider lesions producing lack of exit from the pulmonary venous atrium even though some of them may affect a morphologically tricuspid valve. Left atrioventricular valve atresia is a more accurate term, but mitral atresia is used because of its clinical applications. As with tricuspid atresia, mitral atresia may be due to either absence of an atrioventricular connexion or to presence of an imperforate valve membrane. In the setting of mitral atresia, the distinction is rarely if ever of practical clinical significance but understanding of the difference is essential for correct interpretation of the anatomy. When the left connexion is absent, the atrioventricular sulcus tissue completely separates the muscular floor of the left atrium from the underlying ventricular myocardium. When an imperforate valve membrane is present, it separates the cavity of the left atrium from an underlying ventricular cavity. Either of these lesions can be found with all the various forms of mitral atresia. The major anatomical subdivision is into groups with co-existing aortic atresia and with a patent aortic root. With aortic atresia, almost invariably the right atrium is connected to a right ventricular chamber. When there is a patent aortic root, the right atrium can be connected to a right ventricular chamber, a left ventricular chamber or rarely an indeterminate ventricular chamber, and these forms can all be found with various ventriculo-arterial connexions. The anatomical features are illustrated with diagrams and with hearts sectioned so as to simulate diagnostic planes used during two-dimensional echocardiographic examination. The convention used for categorizing these hearts as either biventricular or univentricular is discussed, emphasizing how disagreements about such nosological matters should be subordinate to accurate description of the underlying anatomy.
A pathological study has been performed on 10 infected Hancock bioprostheses removed from nine patients who died of prosthetic endocarditis. The devices had been in place from 2 to 87 months (average 37.5), the interval between operation and onset of infection averaging 30 months. The offending organisms were Gram negative bacteria in three patients (Klebsiella pneumoniae, Enterobacter cloacae, and Serratia marcescens), Gram positive bacteria in two (Staphylococcus aureus and Streptococcus viridans), and fungi in four (Candida species in three and Aspergillus species in one). Gross examination of the explants revealed in most cases a vegetative endocarditis of one porcine valve leaflets. Septic embolization occurred in five cases owing to the high friability of the vegetations. Prosthetic valve incompetence was the commonest type to dysfunction observed because of tears, perforations, and even complete destruction of the cusps. Prosthetic valve stenosis following obstruction of the valve orifice by infected polypous masses was noted in two cases. Clumps of infective organisms were detected deep in the cusp tissue in most cases on histologic examination. Infection located on the paraprosthetic tissues, associated with ring abscess, valve detachment, and insufficiency, was observed only once. According to the results of investigation, endocarditis on porcine bioprostheses is associated with a better preserved native valvular ring as viewed at reoperation. Therefore surgical intervention appears appropriate in the presence of severe hemodynamic complications after adequate antibiotic treatment. However, infection of these particular prostheses still carries an extremely high mortality. In the present series, this poor outcome might be explained by the frequently associated septic and thromboembolic events.
Electrophysiologic and histopathologic correlation has been carried out in a patient with scleroderma heart disease, affected by syncopal seizures, who died of recorded ventricular fibrillation. The electrophysiological investigation disclosed dysfunction of sinoatrial conduction, revealed by sinoatrial blocks and by an abnormal return cycle pattern after premature atrial beats. Atrial effective and functional refractory periods were increased and an unusual 'pseudo-Wenckebach' phenomenon between artificial stimulus and atrium was observed during atrial pacing. Intra-AV nodal conduction time was at normal upper limits and Wenckebach-type AV block was obtained on pacing the atrium at 100 beats/min. HV conduction was moderately prolonged in the presence of left anterior hemiblock. The histopathologic substrates of these electrophysiologic disturbances were fibrosis of the sinus node, disrupted internodal pathways and atrio-AV nodal connections, and left bundle branch atrophy. As far as fatal tachyarrhythmia is concerned, myofibrillar degeneration may have contributed to its pathogenesis. It is suggested that both lesions of the ordinary myocardium and specialized conduction system account for the electrical instability of sclerodermic patients.
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Though atresia of the left atrioventricular (av) orifice can show multiple types of ventricular morphology and different ventriculo-arterial (va) connections, clinical prognosis and surgical reliability depend upon the size of the aorta. Two cases with "normal" aorta are reported. The first was a female baby, died at four months, who had atresia of the left av orifice with right main ventricular chamber, va discordance and moderate pulmonary stenosis. The fatal outcome was due to progressive restriction of the foramen ovale. The second patient was studied at six months, is still alive, and showed atresia of the left av orifice with left main ventricular chamber, va discordance and pulmonary blood overload. According to this type of anatomy, modified Fontan procedure is postulated as possible and reliable surgical correction.
Long-term clinical results and pathologic features, both macroscopic and microscopic, of 24 Sparks-Mandril grafts as femoro-popliteal substitutes are reviewed. High rate of complications and low percentage of patency are stressed. Further use of this graft is not recommended.
Fourteen patients with double-chambered right ventricle underwent surgical treatment and repair of associated anomalies. The anomalous muscle band was isolated in 5 cases, associated with membranous ventricular septal defect in 7, with discrete sub-aortic stenosis in one and with double outlet right ventricle in one. All patients survived. The obstructing muscular band was a hypertrophic structure identifiable either with a displaced moderator band, still related to the anterior papillary muscle, or with a giant septoparietal band, inserting to the anterior free wall and occasionally present in normal hearts. According to this interpretation, referring the obstructive band to the septomarginal complex, double-chambered right ventricle should not be regarded strictly as a truncoconal malformation.