SIOP Working Committee on psychosocial issues in pediatric oncology: guidelines for communication of the diagnosis

A 44-year-old man with a St. Jude mitral valve was admitted because of progressive pulmonary edema. He was diagnosed with prosthetic heart valve thrombosis (PHVT) based on the findings of "muffled" prosthetic valve clicks. Doppler echocardiographic evidence of severe mitral stenosis and transesophageal echocardiographic evidence of limited mitral valve motility. Because the patient hesitated to undergo our recommended surgical treatment, he was immediately treated with intravenous recombinant tissue plasminogen activator (100 mg over 3 h) followed by heparinization. Two hours after the thrombolytic therapy, the prosthetic valve clicks became clearly audible and his congestive symptoms were dramatically improved. Follow-up echocardiography no longer-showed significant mitral valve obstruction. A transient cerebral ischemic attack occurred at the end of thrombolytic therapy but there were no neurologic sequalae. The patient, on warfarin therapy, was well at follow-up 8 months after discharge. Surgical intervention has long been the standard therapy for patients with PHVT. Our case experience suggests that thrombolytic therapy may be considered as an effective alternative to surgical intervention for selected patients with PHVT. In this report, we also review the current literature regarding the indications, effectiveness and safety of thrombolytic therapy in PHVT.     

Three-dimensional transesophageal echocardiographic assessment for prosthetic valves

We evaluated three-dimensional transesophageal echocardiographic assessment of the implanted mechanical valves by rotational scanning method. Patients were 7 mitral valve replacement and one aortic valve replacement, 2 mitral and aortic valve replacement. In 2 cases of 7 mitral valve replacement, the prosthetic valve regurgitation was evaluated using by color Doppler echocardiography. In this study, multiplane transesophageal probe was used. It rotated at 2-degree intervals from 0 to 180 degrees and the three-dimensional reconstruction was performed by echo scan workstation system (TOMTEC Inc, Munich, Germany). In mitral valve replacement cases, the valve motion of bileaflet valve (St. Jude Medical valve and ATS valve) were showed very clearly. The struts was easily recognized in the St. Jude Medical valve (SJM valve) cases. In one of SJM valve cases, the pannus formation was revealed, but it did not disturb valve motion. The prosthetic valve regurgitation can be seen accurately. However, aortic valve and ball valve cases cannot be reconstructed because of artifact from prosthetic valve and ultrasonic direction. The prosthetic valve regurgitation, pannus formation which was difficult to be showed in two-dimensional echocardiography and relationship between annulus and sewing cuff can be evaluated by this three-dimensional echocardiography. In this study, this system has some problems, for example real-time evaluation is impossible, aortic valve and ball valve cases cannot be reconstructed. However, we think that this new technology is suitable for evaluating valve thrombus, valve dysfunction and paravalvular leakage. In conclusion, the three-dimensional echo-cardiography demonstrated, reliable and accurate examination, and it can evaluate various complications of prosthetic valve.     

Report of a case with re-mitral valve replacement for Bj?rk-Shiley Delrin disc valve prosthesis--disc wear of the Delrin disc 20 years after implantation

We reported a patient in whom slight congestive heart failure gradually developed 20 years after mitral valve replacement with a Delrin disc Bj?rk-Shiley valve prosthesis. Although no evident cause of prosthetic valve malfunction could be detected preoperatively, the mitral prosthesis was excised and replaced uneventfully with a 29 mm St. Jude Medical valve prosthesis. At gross inspection, marked wears of the Delrin disc surface and strut shaped indentations were present. These disc variance, occurred much earlier than initially predicted by Dr. Bj?rk, allowed prosthetic valve malfunction. The patient with this specific model should be carefully followed-up, if necessary, performed reoperation.     

A case of left atrial free-floating ball thrombus

A rare case of left atrial free floating ball thrombus with mitral stenosis is reported. A 66-year-old woman was admitted for epigastralgia and acute heart failure without atrial fibrillation and previous embolization. The patient was treated successfully with removal of ball thrombus and mitral valve replacement. Intraoperative transesophageal echocardiography was effective for monitoring of mobile left atrial thrombus.     

Accurate localization of mitral regurgitant defects using multiplane transesophageal echocardiography

BACKGROUND: Appropriate patient selection for surgical repair of the mitral valve depends on the specific location and mechanism of regurgitation, which, in turn, has necessitated a more detailed method to accurately describe mitral pathology. This study tests a strategy of using multiplane transesophageal echocardiography to systematically localize mitral regurgitant defects and compares these results with the surgical findings. METHODS: Fifty patients with mitral regurgitation underwent intraoperative transesophageal echocardiography for the evaluation of mitral pathology and potential repair. Mitral regurgitant defects were localized using a systematic strategy and a simple nomenclature that divides each mitral valve into six sections (three sections per leaflet) and each prosthetic sewing ring into six sections (60 radial degrees = one section). RESULTS: Thirty-nine patients with native mitral valves were studied, for a total of 234 sections evaluated. Eighty-seven of these sections contained regurgitant defects by transesophageal echocardiography (mean number of regurgitant defects per valve, 2.2; range, 1 through 6). There was agreement between the transesophageal echocardiographic and surgical localizations in 96% (224/234; p < 0.0001) of the sections. Eleven patients with prosthetic mitral valves were studied, for a total of 66 sections evaluated. Twenty-three of these sections contained paravalvular leaks by transesophageal echocardiography (mean number of leaks per prosthesis, 2.1; range, 1 through 6). There was agreement between the transesophageal echocardiographic and surgical localizations in 88% (58/66; p < 0.001) of the sections. CONCLUSIONS: This transesophageal echocardiographic strategy provides a systematic method to accurately localize mitral regurgitant lesions and has the potential to improve the preoperative assessment of patients with significant mitral regurgitation.     

Clinico-morphological comparisons in thrombosis and insufficiency of mitral valve prosthesis]

Clinical and morphological comparisons were conducted in 31 patients with thrombosis and insufficiency of mitral valve prostheses. The morphology of these complications was studied on the basis of autopsy data in 19 cases, and in 12 reoperations. Prosthetic thrombosis was observed in 18 patients. The leading causes of the functional disorders in the prosthesis were septic complications, persisting hypotension in the early postoperative period, errors of anticoagulation therapy. The clinical manifestations of thrombosis of a prosthesis depend on the extent and localization of the thrombus. An important role in the diagnosis of such complications belongs to a dynamic observation, especially to repeated phonocardiographic examinations. A phonocardiographic semiotics of the complications is presented. The results of reoperations for thrombosis of a mitral valve prosthesis and its insufficiency are also presented. The rationale of secondary interventions is demonstrated.     

Late, intermittent obstruction of a mitral prosthesis by chordal remnants

A 59 year old male was admitted 10 weeks following insertion of a Medtronic Hall mitral prosthesis. He suffered recurrent episodes of electromechanical dissociation (EMD). Transthoracic echocardiography demonstrated that during the times of haemodynamic compromise, the mitral prosthesis was intermittently obstructed. Emergency surgical intervention revealed that chordae tendineae had prolapsed through the lesser orifice, obstructing the valve mechanism. The mitral remnants were excised, and as the valve functioned normally, it was not replaced. Postoperatively, the patient made an uneventful recovery. This case illustrates the Doppler echocardiographic features associated with extrinsic obstruction of a mitral prosthesis, and demonstrates that this unusual complication can be responsible for late valve dysfunction.     

Differentiating thrombus from pannus formation in obstructed mechanical prosthetic valves: an evaluation of clinical, transthoracic and transesophageal echocardiographic parameters

OBJECTIVES: We sought to determine the clinical and echocardiographic parameters that differentiate thrombus from pannus formation as the etiology of obstructed mechanical prosthetic valves. BACKGROUND: Distinction of thrombus from pannus on obstructed prosthetic valves is essential because thrombolytic therapy has emerged as an alternative to reoperation. METHODS: We analyzed clinical, transthoracic and transesophageal echocardiography (TEE) data in 23 patients presenting with 24 obstructed prosthetic valves and compared the findings to pathology at surgery. RESULTS: Fourteen valves had thrombus and 10 had pannus formation. Patients with thrombus had a shorter duration from time of valve insertion to malfunction, shorter duration of symptoms, but similar New York Heart Association functional class at the time of operation. Patients with thrombus had a lower rate of adequate anticoagulation (21% vs. 89%; p=0.0028). Pannus formation was more common in the aortic position (70% vs. 21%; p=0.035). Abnormal prosthetic valve motion was detected by TEE in all cases with thrombus formation but in 60% with pannus (p=0.0198). Thrombi were larger than pannuses (total length 2.8+/-2.47 cm vs. 1.17+/-0.43 cm; p=0.038). This was mostly due to extension of thrombi into the left atrium in prosthetic mitral valves. Thrombi appeared as a soft mass on the valve in 92% of cases, whereas 29% of pannuses had a soft echo density (p= 0.007). Ultrasound video intensity ratio, derived as the videointensity of the mass to that of the prosthetic valve, was lower in the thrombus group (0.46+/-0.14 vs. 0.71+/-0.17, p=0.006). A videointensity ratio of <0.70 had a positive predictive value of 87% and a negative predictive value of 89% for thrombus. Duration from onset of symptoms to reoperation of <1 month separated thrombus from pannus formation. The best objective clinical parameter for prediction of thrombus was inadequate anticoagulation, whereas the best TEE parameters were qualitative and quantitative ultrasound intensity of the mass. The presence of either inadequate anticoagulation or a soft mass by TEE improved the predictive power of either parameter alone and was similar to that of ultrasound videointensity ratio. CONCLUSIONS: Duration of symptoms, anticoagulation status and qualitative and quantitative ultrasound intensity of the mass obstructing a mechanical prosthetic valve can help differentiate pannus formation from thrombus and may therefore be of value in refining the selection of patients for thrombolytic therapy of prosthetic valve obstruction.     

DNA repair in baboon alveolar macrophages: a system for assessing biohazardous materials

The influence of transducer position and angulation upon the mitral systolic echo was studied in 100 presumably healthy females. Echocardiographic studies were performed from the second, third, fourth and fifth intercostal spaces (ICS). The role of the sound beam's path relative to cardiac motion was assessed by analyzing the recorded mitral valve pattern as a function of transducer orientation, independent of the absolute ICS used. With the transducer directed caudally when both mitral leaflets and left atrium were recorded, holosystolic or midsystolic posterior motion of the mitral valve leaflet echo was seen in 59% of the subjects. These patterns, recorded this way, were not related to phonocardiographic signs suggesting mitral valve prolapse. Best correlation with phonocardiographic findings was obtained when the echocardiographic examination was performed with the transducer either perpendicular to the chest in the sagittal plane, or pointing slightly cephalad ('perpendicular' position). With the transducer in 'perpendicular' position, both holosystolic and midsystolic posterior motion of the mitral systolic echo, deviating more than 2 mm from a line joining the C and D points, were highly related statistically to phonocardiographic findings suggesting mitral valve prolapse. This study demonstrates that transducer position and angulation on the chest wall are important determinants of echocardiographic appearance of mitral valve during systole. Only the 'perpendicular' transducer position should be used when analyzing echocardiograms for the presence of mitral valve prolapse.     

DNA ploidy and expression of p53 and C-erbB-2 in extramammary Paget''s disease of the vulva

We report a case of total pulse alternans in a patient with paroxysmal ectopic atrial tachycardia and echocardiographic findings obtained before and after radiofrequency catheter ablation (RFCA) that terminated the arrhythmia. The patient was a 27-year-old man with history of paroxysmal palpitations with worsening episodic dizziness, chest tightness, and dyspnea. Electrocardiography (ECG) showed atrial tachycardia at 160 to 170 beats/min while the simultaneous pulse was in the 80s beats/min. Echocardiogram showed that aortic and mitral valves opened with alternating excursions and outflow velocities. Furthermore, despite similar ventricular wall thickening during systole of consecutive cardiac cycles, there was alternating mitral valve opening during diastole of the same cycles, providing direct evidence that ineffective diastolic filling and mitral valve opening may play a role in the pathogenesis of pulse alternans. Repeat ECG and echocardiography after the successful RFCA showed normal sinus rhythm and normal opening excursion and the velocity across the aortic and mitral valves.     

Predisposing factors for severe mitral regurgitation in idiopathic mitral valve prolapse

To elucidate predisposing factors for severe mitral regurgitation (MR) in idiopathic mitral valve prolapse (MVP), 124 MVP patients were classified into the following categories: 55 with isolated clicks (click group), 35 with a late-systolic murmur (late-SM group), and 34 with a holosystolic murmur (holo-SM group). Their clinical and echocardiographic findings were compared with those of 26 patients with spontaneous chordal rupture (rupture group). In 22 patients in the click group, 24 in the late-SM group, and 22 in the holo-SM group, follow-up studies were performed for a mean of 4.5 years (range 1 to 13.5). The mean age was youngest in the click group and oldest in the rupture group. The click and late-SM groups showed a female predominance, but the holo-SM and rupture groups showed a male predominance. There was no difference in the incidence of systemic hypertension among the 4 groups. Most patients in the click and late-SM groups had anterior leaflet prolapse. In the holo-SM and rupture groups, however, the incidence of posterior leaflet involvement was significantly increased. The incidence of thickened mitral valve increased in order of the click (8%), late-SM (21%), holo-SM (38%), and rupture (50%) groups. Six patients in the holo-SM group developed chordal rupture with severe MR during the follow-up period. In the click and late-SM groups, however, there were no complications and no development into a holo-SM. Thus, aging, male sex, posterior leaflet prolapse, thickened mitral valve, and holo-SM were found to be important predisposing factors for severe MR in idiopathic MVP.     

Normal echocardiographic characteristics of the Sorin Bicarbon bileaflet prosthetic heart valve in the mitral and aortic positions

Doppler echocardiographic characteristics of normally functioning Sorin Bicarbon prostheses were prospectively assessed in 226 consecutive patients (135 male and 91 female patients, mean age 61 +/- 10 years) with 233 valves in the mitral (n = 67) and aortic (n = 166) positions whose function was considered normal by clinical and echocardiographic evaluation. Patterns of "normal" transprosthetic leakage were assessed with transthoracic echocardiography in all valves and with transesophageal echocardiography in six selected mitral valve prostheses. For the mitral valve prostheses, we found that peak and mean gradient, as well as pressure half-time, were not significantly different in either the 25 or the 31 mm valves (median values from 15 to 10 mm Hg, from 4 to 4 mm Hg, and from 70 to 83 ms; p = Not significant for all). On transthoracic study, 12 patients (17%) with a Sorin Bicarbon valve in the mitral position showed minimal transprosthetic leakage. On transesophageal study, all patients showed a transprosthetic leakage whose spatial distribution had a complex pattern: in planes orthogonal to the leaflet axis, two to four jets arising from the hinge points and converging toward the center of the valve plane could be visualized; in planes parallel to the leaflet axis, there were three jets, the two lateral ones diverging and the central one perpendicular to the valve plane. For the aortic valve prostheses, there was a significant decrease in transprosthetic gradients and an increase in effective orifice areas as prosthesis size increased. Peak and mean gradients decreased from a median value of 25 and 13 mm Hg in the 19 mm valves to 9 and 5 mm Hg in the 29 mm valves, respectively. Effective prosthetic valve area calculated with the continuity equation increased from a median value of 0.97 cm2 for the 19 mm size valves to 3.45 cm2 for the 29 mm size. With analysis of variance, effective prosthetic aortic valve area differentiated various valve sizes (F = 40.9, p < 0.0001) better than peak (F = 10.3, p < 0.0001) or mean (F = 8.04, p < 0.0001) gradients alone did. Furthermore, effective prosthetic aortic valve area correlated better than peak and mean gradients with prosthetic size (r = 0.76, r = -0.45, and r = -0.39, respectively). On transthoracic study, 109 patients (66%) showed minimal transprosthetic leakage. These normal values, obtained in a large number of patients with normofunctioning mitral and aortic Sorin Bicarbon valves, may help to identify Sorin Bicarbon prosthesis dysfunction.     

Phonomechanocardiographic diagnosis of the apparently "silent" mitral valve prolapse syndrome (author's transl)]

Five cases with angiocardiographically diagnosed posterior mitral valve prolapse and without ausculatotory findings of midsystolic click or late systolic murmur were studied with phonocardiographic technique employing pharmacological tests and postural changes. A midsystolic click was thus obtained. Isoprenaline was the must helpful drug to achieve it. The accompanying electrocardiographic and coronarographic features of this syndrome are described and discussed.     

The recurrence of an atrial myxoma in a patient with a mechanical mitral prosthesis--apropos a case

Recurrence of cardiac myxoma after surgery is an uncommon situation, particularly if a wide excision of the tissue under the tumour has been done. The authors report a case of a 54-year-old male presenting with a left atrial myxoma near the mitral valve, which had to be replaced by a mechanical prosthetic valve during the removal of the tumour. One year later, he was admitted to hospital with persistent fever, weight loss, and congestive heart failure. After a positive hemoculture, intravenous antibiotherapy was initiated, and twice modified because of relapsing fever. Six weeks later, he was transferred to our institution, after an episode of severe acute pulmonary edema. 2D-Doppler echocardiography suggested the possibility of prosthesis dysfunction, revealing a transprothetic diastolic flow with a high peak velocity and moderately elevated pressure half-time. No intra-atrial masses were visualized. Computed tomography was also inconclusive, because of multiple artifacts produced by the prosthesis. These results led to the performance of a cardiac catheterization with contrast ventriculography, which revealed the presence of a transprothetic gradient, and mild mitral regurgitation. The patient was submitted to cardiac surgery, which revealed a recurrent pedunculated left atrial myxoma, with mechanical obstruction of the mitral prosthetic valve. No signs of endocarditis were found. Recurrent cardiac myxomas are reviewed and discussed, as well as the specific problems of the present case, namely the presence of a mechanical prosthetic mitral valve and the initial hemoculture results, with consequent diagnostic delay.     

A rare case of 2 free thrombi in left atrium with mitral stenosis

A 64-year-old female was admitted with general fatigue and orthopnea. Preoperative echocardiography showed a free ball thrombus in the left atrium, mitral stenosis and severe tricuspid regurgitation. To avoid a herniation of thrombus to the mitral orifice, an emergency operation was performed. Two free and small mural thrombi were found in the left atrium. Thrombectomy, mitral valve replacement and tricuspid annuloplasty were performed successfully. Postoperative course was uneventful, and she was discharged in good condition on the 21st postoperative day.     

Aorto-left atrial fistula in prosthetic aortic endocarditis

Aortic valve endocarditis commonly leads to the formation of a root abscess, but fistulae are uncommon. The echocardiographic findings in a patient with Streptococcus viridans endocarditis of a prosthetic aortic valve associated with a fistula between the aorta and the left atrium are presented. The diagnosis was made by transthoracic echocardiography, although the transesophageal study gave higher resolution views and allowed a more confident exclusion of mitral valve involvement.     

Long-lasting mnemotropic effect of substance P and its N-terminal fragment (SP1-7) on avoidance learning

Obstruction of a prosthetic valve by an infective vegetation is a rare and life-threatening complication of endocarditis that demands emergent surgical intervention. In our patient's case, transthoracic echocardiography showed the large vegetation, transthoracic Doppler imaging showed severe obstruction of diastolic flow through the bioprosthetic valve, and transesophageal echocardiography showed that no perivalvular abscess was present. Rapid diagnosis of prosthetic valve infection and obstruction demanded application of all three major echocardiographic modalities and proved critical to the patient's recovery.     

Protein electrolyte spectrum of the blood in patients with chronic suppurative inflammatory diseases of the lungs and pleura

A second case of malfunction of a Harken disk valve due to undue disk wear is reported. Two and one-half years after aortic and mitral valvular replacement, the patient had paraprosthetic aortic insufficiency and physical findings suggesting intermittent dysfunction of his prosthetic mitral valve. Catheterization showed intermittent hemodynamic abnormalities; fluoroscopic and cineangiographic findings indicated intermittent mitral regurgitation secondary to undue mitral disk wear. At operation, the excised valve showed normal struts and sewing ring but severe disk wear. There was loss of disk substance and rim notching.     

Mitral valve insufficiency caused by tendinous cord rupture and mitral valve aneurysm. Significance of the echocardiographic study

Mitral valve aneurysm is a rare complication that may occur in a myxomatous valve. We report the case of a 73 year old male patient with severe mitral regurgitation and heart failure-class IV NYHA. Echocardiography showed perforation of an aneurysm of the anterior leaflet of the mitral valve associated with rupture of tendinous cords of the posterior mitral leaflet. Diagnosis was made by transthoracic echocardiography and confirmed by transesophageal echocardiography. The patient was urgently operated with success and a mitral valve prosthesis was implanted.     

Hereditary occurrence of the mesotelesystolic click syndrome: a study of 9 families (author's transl)

Nine families affected by the mid-systolic click syndrome were studied. Of the one hundred and forty-four first-degree relatives, 117 of whom were living, eighty-four were examined. Thirty-five were found to be affected by the syndrome. Twenty-six were females and nine were males. Auscultatory and phonocardiographic findings consisted either of isolated mis-systolic clicks or systolic murmurs or a combination of the two. Electrocardiograms revealed changes of various types, most commonly of the ST-T segment. About seventy per cent of the patients were symptomatic. Nine members, not examined by the authors, had died suddenly; all had a previous history of "cardiopathy". Progressivity of mitral valve disease with age is not confirmed by the present study. It is suggested that the mode of inheritance of the defect might be that of an autosomal dominant form of Mendelian type with delayed expression of the defect. An alternatove hypothesis of a multifactorial inheritance mechanism, which is more stimulating for future studies on the cause of the syndrome, is also taken into consideration.