Nonobstructing accessory mitral valve tissue and ventricular septal defect

A 4-month-old boy with ventricular septal defect was found to have accessory mitral valve tissue attached to the anterior leaflet of the mitral valve. Operation was successfully performed to excise the accessory mitral tissue in the left ventricular outflow tract and close the ventricular septal defect. Most previously reported cases with accessory mitral valve tissue were associated with left ventricular outflow tract obstruction. This boy had no pressure gradient across the left ventricular outflow tract. The indications for prophylactic excision of nonobstructing accessory mitral valve tissue in a patient with other forms of congenital cardiac disease are discussed.     

Association between parachute mitral valve and Down's syndrome. Report of a case

The trisomy 21 form of Down's syndrome is the most common human chromosomal aberration. Congenital heart disease is found in as many as 50 per cent of patients with this disorder. The two most common cardiac lesions in Down's syndrome are septal ventricular defect and endocardial cushion defect. Secundum atrial septal defect, tetralogy of Fallot and isolated patent ductus arteriosus are also observed in these Down's patients. Transposition of great arteries and coarctation of the aorta are rarely seen. Most patients having Down's syndrome with congenital heart disease have a single lesion. However, as many as 30 per cent may have multiple cardiac defects. Parachute mitral valve is a rare congenital mitral defect: a single papillary muscle in the left ventricle is the hallmark of this lesion. A parachute mitral valve is frequently associated with other left heart disorders such as supravalvular mitral ring, abnormal and stenosed mitral valve, subaortic stenosis and coarctation of the aorta, thus constituting either a complete form of Shone's complex (when all 4 components are present) or an incomplete form when there are fewer. The aim of the present report is to describe the connection between Down's syndrome and isolated, non-stenosed parachute mitral valve, which has never been reported before.     

Refractory head and neck pain. A difficult problem and a new alternative therapy

Mitral valve repair was performed in six patients by transferring the posterior tricuspid leaflet with its sub-valvular apparatus onto the mitral valve. This new technique considers the tricuspid valve as the patients own tissue bank where the posterior leaflet and eventually the adjacent part of the anterior leaflet is used as a "donor" valve, based on the knowledge that the right atrio-ventricular valve can be efficiently repaired with a very low risk of significant dysfunction. The mitral repair consists of incorporating the tricuspid autograft by securing the tricuspid papillary muscle to the mitral papillary muscle and by suturing the leaflet tissue where required. A mitral annuloplasty ring reinforces the repair. The tricuspid valve is subsequently repaired by annular plication and leaflet suture. A tricuspid ring is necessary to maintain efficient remodeling. The six patients ages ranged from 20 to 70 years. A etiology, was rheumatic in the first case and degenerative in the following. In three cases, sterilised endocarditis was responsible for ruptured chordae and leaflet destruction. The mitral insufficiency was located in a commissural area in 4 cases, and was due to a widespread posterior prolapse in 2. Post-operative control transesophageal echocardiography confirmed the excellent results of the repair and proved that, in selected cases, the tricuspid leaflet inserted onto the mitral apparatus is very efficient in correcting mitral insufficiency, without causing significant tricuspid impairment. With a 3 to 7 month follow-up, the results are stable.     

Mechanisms of antitumor activity of carotenoids

Two cases are presented in which a pigtail catheter was entrapped by the chordae tendineae of the tricuspid valve during pulmonary arteriography. A technique for removal of the catheter from its entanglement by the chordae tendineae is described. Caution must be taken when advancing through the right ventricle a catheter that appears to be entrapped by the chordae tendineae. When such an entanglement occurs, measures to reduce the risk of rupturing a papillary muscle must be taken.     

Use of bovine heterografts in intravenous therapy

Severe prolapse of the mitral valve leaflets was seen at left ventricular angiography in 16 of 92 patients with a secundum type atrial septal defect studied prospectively from 1970 to 1974. The patients were aged 15 to 69 years; angioplasty or mitral valve replacement was carried out in nine. In 9 of 122 patients aged 15 to 55 years who were operated on for closure of a secundum type atrial septal defect between 1956 and 1969, mitral regurgitation due to prolapse but with intact chordae tendineae was seen at operation. In three of these patients chordal rupture was seen at a second operation 2 to 6 years later. The outlook in the syndrome of mitral valve prolapse may be less benign than is usually believed.     

Pulsatile total cavopulmonary shunt for hypoplastic right heart syndrome with abnormal systemic venous return--a case report

A pulsatile total cavopulmonary shunt was successfully performed on a 5-year-old girl with hypoplastic right heart syndrome associated with abnormal systemic venous return; at the same time, modified mitral valve replacement was performed for mitral regurgitation. The right atrium, tricuspid valve and right ventricle were all extremely dimunitive. The diameter of the tricuspid valve was 50% of normal and the volume of the right ventricle was 8.6% of normal. In addition, there were severe subpumonary stenosis, a restrictive ventricular septal defect (VSD) and an atrial septal defect (ASD). The bilateral superior venae cavae (SVCs) and the hepatic vein drained to the left atrium, and the inferior vena cava was infrahepatically interrupted with a hemiazygos connection to the left superior vena cava. At the operation, each SVC was anastomosed end-to-side to each branch of the pulmonary artery (PA). The restrictive ventricular septal defect and stenotic subpulmonary lesion were left. The diameter of the ASD was reduced from 12 mm to 7 mm. The main PA was neither divided nor banded. The pulsatile blood flow from the left heart to the PA was regurated by a native restrictive VSD and stenotic subpulmonary lesion, and that from the right heart via the ASD was limited by reducing the size of the ASD. These described anatomic arrangements produced adequate antegrade pulsatile flow in the PA, which might prevent the development of pulmonary arteriovenous fistulae and, besides permit transfer of drainage of the hepatic vein from the left to the right atrium via the ASD in future.     

A case report of incomplete endocardial cushion defect with mitral stenosis in an elderly patient

The following paper describes a mitral valve replacement (SJM 27 mm), the patch closure (EPTFE) of an ostium primum atrial septal defect and tricuspid annuloplasty (De Vega's method) in a 64-year-old female patient with an incomplete endocardial cushion defect and mitral stenosis. Surgery revealed thickened, mitral valve leaflets and the presence of a cleft, findings similar to those observed in case of rheumatic degeneration. Investigation of patient hemodynamics confirmed a diagnosis of Lutembacher syndrome and a lower with left ventricle volume. After surgery, the volume of left ventricle increased and the patients clinical course was uneventful.     

Motion of mitral apparatus in hypertrophic cardiomyopathy with obstruction

Motion of the mitral apparatus in hypertrophic cardiomyopathy with obstruction was investigated by conventional single dimensional and multidimensional echocardiography. In systole, anterosuperior displacement of the posterior papillary muscle, failure of mitral valve closure, and anterior motion of both mitral leaflets were shown. The anterior leaflet was seen to impinge on the posterior papillary muscle but not on the interventricular septum in systole. The abnormality of the single dimensional mitral echogram, previously ascribed to systolic anterior motion of the mitral anterior leaflet, was found to be a complex of echoes from the chordae tendineae, the papillary muscle, and, furthest from the septum, the mitral anterior leaflet. It is concluded that systolic anterior motion of the mitral anterior leaflet is of smaller amplitude than others have suggested, and that obstruction to left ventricular outflow in hypertrophic cardiomyopathy is produced by systolic contact between the mitral anterior cusp and the posterior papillary muscle. The theory is put forward that displacement of the posterior papillary muscle above and in front of the mitral leaflets produces chordal slackening, and that it is displacement of the chordae tendineae by the blood flowing to the aortic root during left ventricular ejection, which is responsible for systolic anterior motion of the mitral leaflets.     

Experimental principles for preserving annulo-ventricular integrity of the mitral valve

Despite numerous improvement in cardiac surgery the results in mitral valve replacement are still not satisfactory, since impaired left ventricular function continues to be a problem during the postoperative course. In order to investigate the effect of mitral valve replacement on left ventricular function canine experiments were performed: During extracorporeal circulation bileaflet mitral valve prostheses were implanted preserving the ventriculo-annular continuity. Flexible wires were slung around the chordae of the subvalvular mitral apparatus and brought to the outside through the left ventricular wall. Left ventricular diameters were measured by sonomicrometry, left ventricular stroke volume, left ventricular enddiastolic volume and ejection fraction by dye dilution technique as well as left ventricular and aortic pressure by catheter tip manometers. After finishing cardiopulmonary bypass control values were registered and different preload values achieved by volume loading with blood transfusions to left ventricular enddiastolic pressures of 12 mm Hg. Subsequently under normovolumic conditions the chordae tendineae of the anterior and posterior papillary muscles of the mitral valve were cut from the outside, while the heart was beating, by application of electrocautery on the steel wires. Following severance of the ventriculo-annular continuity of the mitral valve again function curves of left ventricular hemodynamics were made during volume transfusions. When the chordae had been divided the left ventricular enddiastolic diameter increased by 10% in the major axis, while in the minor axis no significant changes occurred. The systolic shortening was impaired substantially by reduction of 43% during the ejection phase when the subvalvular mitral apparatus had been severed. Left ventricular enddiastolic volume was increased by 18% at any preload level, while left ventricular ejection fraction was reduced by 16%. Consequently left ventricular stroke volume was decreased by 24% at any left ventricular enddiastolic volume, when the chordae had been divided. It can be concluded that left ventricular geometry is changed when the annulo-ventricular continuity has been interrupted at mitral valve replacement: The major axis of the left ventricle is increased and the enddiastolic volume is augmented. The left ventricle is only able to eject the same stroke volume at higher preload levels when the chordae tendineae have been divided. The same cardiac performance can only be achieved by volume loading and at the expense of higher wall tension, which leads to unfavorable conditions in terms of cardiac muscle mechanics with reduced exercise tolerance. These data speak for preservation of the annulo-ventricular continuity in mitral valve replacement. Provided that these results from acute canine experiments can be transferred to humans, one would suggest that preservation of the mitral subvalvular apparatus is of importance in patients with dilated hearts and with impaired left ventricular function.     

Definition of inseparably fused ventricular myocardium in thoracopagus: fetal echocardiographic utility and pathologic refinement

Correlative echocardiographic and pathological findings in a thoracopagus with conjoined hearts are reported. One twin had tricuspid atresia with discordant atrioventricular connections and concordant ventriculoarterial connections. The morphologic right ventricle was hypoplastic and there was a large muscular ventricular septal defect. The other twin had hypoplasia of the mitral valve anulus and left ventricle with double-outlet right ventricle and pulmonary valve atresia. The tricuspid valve was severely insufficient in part because of a large orifice and redundant, elongated leaflets with abnormal chordal attachments. The left ventricles of these two twins shared a perforated common "free wall" with at least two large defects allowing mixing of the circulations at that level. Not all anatomic details were established conclusively by fetal echocardiography; however, sufficient diagnostic information was obtained to support a decision not to aggressively resuscitate these twins after elective cesarean delivery at 31 weeks' gestation.     

Cineangiographic diagnosis of papillary muscle involvement in rheumatic mitral stenosis

Left ventricular cineangiography was performed in 25 patients with normal mitral valves and in 49 patients with rheumatic mitral valve disease to estimate the spatial relations between the mitral valve leaflets and papillary muscles. The findings in the pathologic group were compared with observations at operation or with the excised mitral valves. It is concluded that fusion of the papillary muscles with the mitral valve leaflet or severe shortening of the chordae is predicted with a reasonable accuracy preoperatively by means of left ventricular cineangiography. The surgical implications are discussed.     

Ventricular buckling: a factor in the abnormal ventriculogram and peculiar hemodynamics associated with mitral valve prolapse

Because of intractable ventricualr arrhythmias after a near-fatal episode of ventricular fibrillation, a patient with idiopathic mitral valve prolapse was subjected to mitral valve replacement. Vector analysis and intraoperative epicardial mapping localized the ectopic focus to the region of the posterior papillary muscle. The patient is alive and well two years after surgery; chronically inverted T waves have become upright. But propranolol and diphenylhydantoin are needed to prevent arrhythmias and T wave abnormalities during standing and exercise. Preoperatively, with the onset of mitral regurgitation and a second rapid phase of prolapse, the ventriculogram was deformed by abnormal midsystolic hyperkinesis at both sites of papillary muscle insertion. Postoperatively, focal hypokinesis appeared in the same areas, implying that they had been retracted by the prolapsing valve. Preoperatively, a papillary tip could be seen entering the mitral ring while coronary arteriography showed late systolic elongation of a small vessel feeding the anterior papillary muscle, suggesting that the papillary apparatus was indeed subject to damaging stress during the abnormal basal movement. Three other persons with severe mitral prolapse (but intact chordae) have had valve repacement and developed qualitatively similar changes in the ventriculogram. Papillary speciments in two showed significant fibrosis. Indication for operation in one of these was edpisodic ventricular fibrillation, which has not recurred. A spectrum of ventriculographic abnormality associated with mitral prolapse could be partly explained by hypokinesis of the papillary loops, variably disguised by retraction stress tansmitted from the billowing leaflets, translocation of blood into the expanding valve sail, and various degrees of unloading into the left atrium. Abnormal intraventicular flow may probably result from associated prolapse of the anterior leaflet and from buckling of the papillary sties toward the mitral annulus. Unusual physical findings in the operated cases and in eight other patients define a clinically recognizable syndrome in which severe prolapse abbreviates left ventricular ejection. Liability to symptoms and to progression of disease seems high in this group.     

Mitral valve anomalies obstructing left ventricular outflow

This paper reports on two cases of more uncommon types of subaortic stenosis. A 2-year-old boy was found with accessory mitral valve leaflet (AMVL) attaching to the anterior leaflet, ballooning into the subaortic ventricular septum associated with a discrete subaortic membrane. The obstruction was successfully relieved by removal of the AMVL and resection of the membrane. A 19-day-old newborn with accessory tissue on the mitral valve (AMVT) causing subaortic stenosis, subaortic ventricular septal defect (VSD) and patent ductus arteriosus was operated on successfully. Accessory tissue excision through the VSD, VSD patch closure and ductus ligation were performed.     

Traumatic tricuspid valve insufficiency

We report a case of tricuspid insufficiency in connection with blunt chest trauma. The patient was involved in a car accident. The central venous catheter showed a right ventricular pressure curve, suggestive of a tricuspid valve insufficiency. A transoesophageal echocardiographic examination supported this by revealing a papillary muscle rupture. This kind of injury has been seen more frequently during the last 35 years, partly because of better diagnostic procedures and a better understanding of the pathology. The decelerating force in the right ventricular chamber produces a regurgitation thereby initiating rupture of the papillary muscle and/or the chordae tendinae. In a literature study twelve out of thirteen patients were involved in car accidents. Some of them had a dominant V-wave in the venous pulse curve, but no clinical observation is directly diagnostic. Therefore, cardiac lesions should be kept in mind whenever there is a history of blunt chest trauma. The best diagnostic approach is echocardiography.     

A case report of mitral valve repair of broad posterior leaflet prolapse due to ruptured chordae tendinea by replacement of chordae tendineae with EPTFE sutured and Carpentier-Edwards ring techniques]

A 68-year-old woman with mitral valve regurgitation due to ruptured chordae tendineae of posterior leaflet underwent mitral valve repair by replacement of chordae tendineae with EPTFE sutures and Carpentier-Edwards ring techniques. Preoperative study showed massive mitral regurgitation and moderate tricuspid regurgitation with CTR 54% of chest X-ray. The postoperative course was not eventful. Postoperative study showed trivial mitral and trivial tricuspid regurgitation. Postoperative CTR was 45%. Mitral valve repair by these techniques could be modified and applicable to mitral valve regurgitation due to ruptured chordae tendineae of posterior leaflet. There was no complication during follow-up period of 8 months.     

Pitfalls in clinical recognition and a novel operative approach for hypertrophic cardiomyopathy with severe outflow obstruction due to anomalous papillary muscle

BACKGROUND: Ventricular septal myotomy/myectomy (Morrow procedure) is the standard surgical option for severely symptomatic patients with hypertrophic cardiomyopathy (HCM) and marked basal obstruction to left ventricular outflow due to mitral valve systolic anterior motion. In some patients, however, congenital malformations of the mitral apparatus may be responsible for outflow obstruction; the failure to recognize this morphology before operation could have adverse consequences. METHODS AND RESULTS: We recently evaluated 2 patients with obstructive HCM operated on at Mayo Medical Center in 1997 who demonstrated direct anomalous papillary muscle insertion into the anterior mitral leaflet, producing muscular midcavity obstruction. This anomaly is potentially identifiable with echocardiography by exaggerated anterior displacement of hypertrophied papillary muscles within the left ventricular cavity and the direct continuity between papillary muscle and anterior leaflet associated with a rigid motion pattern of the mitral apparatus. Echocardiographic diagnosis, however, was confused in both patients by the association of systolic anterior motion of the mitral valve, probably produced by freely mobile margins of the mitral leaflet unencumbered by papillary muscle insertion, and in 1 patient probably representing a second and more basal level of obstruction. Because outflow tract morphology was judged unsuitable for conventional myotomy/myectomy, a novel surgical strategy was designed to remove the outflow gradient in which an extensive myectomy trough (wider at its apical than basal extent) was created within the ventricular septum to papillary muscle level; also, in 1 patient, attachment of anterolateral papillary muscle with the lateral free wall was partially severed to increase mobility of the mitral apparatus. After surgery, both patients reported substantial relief of symptoms and improved exercise tolerance and also showed reduced or abolished basal outflow obstruction. CONCLUSIONS: In HCM, outflow obstruction due to anomalous papillary muscle insertion directly into anterior mitral leaflet is challenging to identify but should always be contemplated before operative intervention. This important (but often unsuspected) congenital malformation may require alternative surgical strategies to standard myotomy/myectomy, similar to those described here.     

Idiopathic mitral valve prolapse-analysis by real-time two-dimensional echocardiography

Mitral valve prolapse is diagnosed in real-time two-dimensional echocardiograms when there are discrepancies in the coaptation zone of the anterior mitral leaflet and the posterior mitral leaflet. Out of the 100 cases of mitral valve prolapse diagnosed in this way, 65 had prolapsed anterior mitral leaflets, 28 prolapsed posterior mitral leaflets and 7 prolapses of both the anterior and posterior mitral leaflets. In addition to the cases with mitral valve prolapse 23 cases of ruptured chordae tendineae of the mitral valve, including 15 cases which had undergone surgery, were investigated. The frequent site of mitral valve prolapse was the posteromedial commissure in the anterior leaflet and the posteromedial and anterolateral commissures in the posterior leaflet. These sites coincide with those where rupture of the chordae tendineae of the mitral valve was apt to occur. An investigation of the relation between age and mitral valve prolapse showed that the number of cases of prolapsed anterior leaflet did not increase with age, but there was an age-related increase in the number of cases of prolapsed posterior leaflets. It was also found that the degree of the prolapse progressed with age. Many of the cases of ruptured chordae tendineae of the mitral valve were in their forties or fifties, and there appeared to be some relation between the progress of the prolapse and age. Mitral regurgitant murmurs were recorded on phonocardiograms, and the severer the degree, the wider the range of the prolapse. Mitral regurgitation was more likely to occur in cases of prolapsed posterior leaflets than in those with prolapsed anterior leaflets, even if the degree and the range of the prolapse were mild.     

Increased excretion of urinary transforming growth factor beta 1 in patients with diabetic nephropathy

Two cases of double outlet right ventricle with restrictive ventricular septal defect are described. This is an uncommon presentation that causes left ventricular dysfunction because of left ventricular outflow tract obstruction. The presence of an intact atrial septum leads to severe pulmonary hypertension, which tends to aggravate the right ventricular output. In the presence of a normal left ventricle, the authors suggest the possibility of enlargement of the ventricular septal defect in order to perform a biventricular repair. The association of a supramitral valve ring in both cases, and the isolation of the left subclavian artery and an aortopulmonary fenestration in one of these cases, are also discussed. In addition we explore factors that cause restrictive ventricular septal defects as well as the mechanisms that may lead to spontaneous closure of ventricular septal defect in a double outlet right ventricle.     

Anatomic variations in underdeveloped right ventricle related to tricuspid atresia and stenosis

In the anatomy of 416 hearts, the seat of tricuspid stenosis or atresia is examined, with special reference to Fontan-like surgical procedures. A classification is offered which includes cases with and without regular or inverted transposition, and with decreased or increased pulmonary flow. The size and thickness of the right atrium, the size and architecture of the right ventricle, the size of the pulmonary tree, the types of atrial and ventricular septal defects, the condition of the mitral valve, and the size and thickness of the left atrium and left ventricle are analyzed. In addition the various intracardiac and extracardiac abnormalities are enumerated. Reference is also made to the tendency of the aorta and pulmonary trunk to override the septum, in some cases producing double-outlet left ventricle. It is considered that many cases of tricuspid valve atresia and stenosis with or without transposition may be amenable to Fontan-like procedures in the proper age group. All the above anatomic considerations have a bearing on the suitability and type of operative tricuspid bypass procedures, and they may influence the prognosis of surgical therapy.     

A case report on a reconstructive operation of a traumatic tricuspid regurgitation with a congenital defect of the left pericardium

We reported a successful tricuspid valve replacement in a 58-years old man, who had easy fatiguability after 14 years of a blunt chest trauma. The preoperative examination revealed a marked cardiomegaly with deformation of both ventricles and grade 4 tricuspid regurgitation caused by the prolapse of the anterior leaflet. The operative inspection revealed a left pericardial defect with a diameter of 10 cm and a torn anterior papillary muscle. Since a usual plastic procedure did not improve the regurgitation, a Carpentier-Edward bioprosthetic valve was implanted in the supra annular position. Atrioventricular conduction was preserved. The tricuspid valve was not resected to preserve the ventricular function. The patient recovered his own activity.