Marfan's syndrome is an inherited connective tissue defect that affects many organs, especially of the musculoskeletal, ophthalmic and cardiovascular systems, and may be associated with some rare conditions. Here, we report the first known case of Marfan's syndrome, combined with situs inversus totalis with dextrocardia and discrete subaortic stenosis and aortic insufficiency in a 22-year-old woman.

Background and aim of the study: Magnetic resonance (MR) imaging is used in an increasing number of patients, and not only after cardiac valve replacement. However, ferromagnetic biomedical implants are often considered a contraindication for MR imaging because of the potential hazards with respect to their movement, dislodgement, or heating effects during the procedure. The purpose of this study was to assess ferromagnetism, attraction forces, heating effects, and artifacts associated with prosthetic heart valve implants. Methods: Seventeen common heart valve prostheses (12 mechanical, five biological) were examined in vitro using a high-field-strength 1.5 Tesla (T) MR system. Attractive forces, temperature changes and the amount of artifacts were assessed by applying turbo-spin and gradient-echo sequences. Results: The maximal calculated corresponding ferromagnetic force was (0.22 x 10(-3) N) in the static magnetic field. The temperature changes ranged from 0 to 0.5 degrees C maximum. Artifacts produced by the presence of the heart valve prostheses were less evident using a spin-echo sequence than a gradient-echo sequence. Conclusion: MR imaging exerted no significant force on the examined heart valve prostheses, and did not result in significant biological relevant temperature increase. None of the associated artifacts is considered to pose a substantial risk on MR imaging. MR procedures performed with a 1.5 T MR system can be applied safely in patients with heart valve prostheses evaluated in this study.

Background and aim of the study: Whether the St. Jude Medical (SJM), Medtronic Hall (MH) or CarboMedics (CM) heart valves confer any relative benefits to patient outcome remains controversial. While numerous studies have analyzed clinical results with a single brand, and a few studies have compared two brands, there are no single-center trials comparing all three valves.  Methods: Our experience with patients who had either a SJM, MH or CM mechanical valve in isolated aortic valve (AVR) or mitral valve (MVR) replacement was reviewed. AVR was performed in 953 patients (SJM = 394, MH = 314, CM = 245) and MVR in 591 patients (SJM = 193, MH = 264, CM = 134). Survivors were assessed annually; follow up consisted of 3336 patient-years (pt-yr) after AVR and 1693 pt-yr after MVR. Results: Preoperatively, in the AVR group, more MH patients had previous valve surgery (p = 0.001) or were in NYHA class III/IV (p = 0.03), and more CM patients had a concomitant surgical procedure (p = 0.005). The hospital mortality after AVR with SJM, MH and CM valves was 3.8, 4.7 and 5.3%, respectively (p = 0.65). In the MVR group, there were more males in the CM group (p = 0.011), more CM patients had concomitant surgery (p = 0.001), and more MH patients had previous surgery (p = 0.006). The hospital mortality after MVR with SJM, MH and CM valves was 8.3, 10.2 and 6.0%, respectively (p = 0.35). There was no late survival advantage in either the AVR or MVR group according to the valve used (p = 0.24 and p = 0.90, respectively). For the AVR group the five-year actuarial freedom from thromboembolism was: SJM 85.8 +/- 2.5%, MH 80.1 +/- 2.7% and CM 85.9 +/- 3.5% (p = 0.04), and for MVR it was: SJM 84.2 +/- 4.0%, MH 77.5 +/- 3.4% and CM 86.9 +/- 5.2% (p = 0.27). Bleeding occurred with a similar frequency in the AVR (p = 0.36) and MVR (p = 0.70) groups. No cases of structural failure were identified in this study. At follow up, among AVR patients NYHA class III/IV was present in: SJM 5%, MH 6% and CM 3% (p = 0.50), while among MVR patients this was identified in: SJM 7%, MH 10% and CM 4% (p = 0.22). Conclusion: It is concluded that the SJM, MH and CM mechanical valves offer similar clinical results when used for isolated AVR or MVR. While there is a suggestion of an advantage with bileaflet valves, any differences detected may simply reflect differences in the preoperative patient variables.

A 29-year-old woman presented with shortness of breath, vague chest pain, and prominent intermittent ejection systolic murmur. Transthoracic echocardiography showed a large mass in the right ventricular outflow tract. Transesophageal echocardiography demonstrated two masses that were adherent to the tricuspid valve and intermittently prolapsed through the pulmonary valve. Computed tomography of the chest corroborated the echocardiographic findings. Currently, there are no definitive guidelines regarding the optimal management of right heart thrombi in patients with antiphospholipid syndrome. Our patient did not respond to a standard dose of rt-PA used in the treatment of pulmonary embolus. She underwent successful surgical resection of the thrombi without complications.

Background and aim of the study: The study aim was to summarize the authors' experience in corrective surgery of Ebstein's anomaly. Methods: A total of 139 patients operated on between June 1980 and January 2000 was studied retrospectively. Among these patients, 111 underwent atrialized ventricle plication, tricuspid valve reconstruction and DeVega tricuspid annuloplasty, 27 underwent tricuspid valve replacement, and one patient with right ventricular hypoplasia underwent an additional total cavopulmonary connection. Results: Overall, there were 12 operative deaths (mortality rate 8.6%); however, between 1990 and 2000, the mortality rate was 3.3%. Among the reconstruction patients, 10 cases were reoperated on for valve replacement, and all survived. Conclusion: Surgery for Ebstein's anomaly should be defined according to the pathologic/anatomic features of the condition. Tricuspid valve reconstruction should be performed in the mild condition; in medium A type, reconstruction should be performed, while for medium B type, reconstruction or valve replacement should be selected, albeit with caution. Valve replacement should be performed in the severe conditions.

Prosthetic valve thrombosis occurring during pregnancy is a life-threatening complication. Surgical treatment requires clot removal or valve replacement under cardiopulmonary bypass, and carries a high mortality. We report successful thrombolytic therapy with streptokinase for prosthetic valve thrombosis in a pregnant, 28-year-old woman. The patient, who had undergone mitral valve replacement (St. Jude Medical prosthesis) two years previously for restenosis after closed mitral valvotomy, was successfully thrombolyzed during the first trimester (6-8 weeks) for prosthetic valve thrombosis, and without any complication. The patient delivered a normal healthy child at nine months' gestation. Although thrombolysis in pregnancy has been reported previously, this is the first case in which it was performed during the first trimester for prosthetic valve thrombosis.

Background and aim of the study: The carinactivase-1 (CA-1) test is a new method for monitoring plasma prothrombin levels during warfarin anticoagulation therapy. Methods: A total of 192 patients were allocated to two groups. Group A patients (n = 42) were controls (no warfarin); group B patients (n = 150) received warfarin. A Ca2+-ion and Boc-Val-Pro-Arg-pNA (a chromogenic substrate for thrombin) were added to 10-fold diluted plasma, after which prothrombin was activated with CA-1. Prothrombin levels were determined by measuring the extent of p-nitoroaniline liberation. Results: The mean prothrombin level was 112.8 +/- 20.0 microg/ml in group A (Gaussian distribution), and 53.3 +/- 19.6 microg/ml in group B. In group B, correlations were found between the CA-1 test and prothrombin levels measured by prothrombin time (PT; r = 0.61, p <0. 01), PT-INR (r = 0.61, p <0.01), Thrombotest (TT; r = 0.57, p <0.01) and Hepaplastin test (HPT; r = 0.69, p <0.01). Conclusion: The CA-1 test represents a viable method of monitoring the coagulation system. CA-1 recognized the Gla-domain of prothrombin, and activated prothrombin. The CA-1 test required only 10 microl of diluted blood plasma, and took approximately 30 min to complete. The CA-1 test also measures prothrombin levels, correlates excellently with other tests for coagulation, and compares well with currently available methods for determining the efficacy of warfarin.

A wide array of recommendations is available for the management of anticoagulation in patients with a prosthetic heart valve scheduled for non-cardiac surgery, ranging from avoidance of replacement anticoagulant therapy in all cases (excluding those with a recent thromboembolic event), to replacement anticoagulant therapy in all, without risk stratification. These guidelines are derived from only a few small- to medium-sized, non-randomized and often methodologically flawed studies conducted during the late 1970s, and applies mainly to caged-ball and caged-disc valves. Furthermore, extrapolation of the thromboembolic risk from data on patients not receiving oral anticoagulants at all is based on assumptions that are not necessarily valid. In this review, the direct and indirect evidence on which these guidelines are based is examined critically. Their applicability to the newer, less thrombogenic valve models is questionable. The need for further prospective, randomized studies is emphasized by the failure of existing studies to adjust properly for the main known or presumed thromboembolic risk factors, and their low statistical power to detect significant differences between protocols in an intention-to-treat manner. The evaluation of obstructive and non-obstructive thrombosis should serve as a secondary outcome measure in the assessment of anticoagulation management before non-cardiac surgery.

Congenital factor XII deficiency is a rare condition. We report a case of aortic valve replacement (AVR) in a 63-year-old man with factor XII deficiency. On admission, the patient's activated partial thromboplastin time (aPTT) was prolonged (271 s), and activated clotting time was 500 s. His factor XII level was <3%. The Sonoclot signature showed an abnormal pattern. AVR with a prosthetic valve (St. Jude Medical) was performed safely after the normalization of aPTT and the Sonoclot signature by frozen plasma transfusion. The perioperative management in patients with factor XII deficiency is discussed.

Reports of aortic regurgitation due to rupture of the aortic valve commissures are rare. Prompt surgical intervention is necessary, as the condition results in rapid, progressive heart failure and subsequent death. We report the case of a 78-year-old man who presented with aortic laceration and cardiac tamponade that was probably induced by prolapse of the bicuspid aortic valve. We speculate that prompt initial surgery may have prevented aortic laceration and cardiac tamponade in this patient. Thus, in order to optimize clinical outcome, clinicians must consider early, precautionary surgical management in patients who have sudden cardiac failure due to aortic regurgitation associated with prolapse of the bicuspid aortic valve.

Background and aim of the study: Aortic valve disease is presently the number one indication for valve replacement in the United States, yet its molecular mechanisms remain unknown. As apoptosis (programmed cell death) occurs in degenerative disease states, it was postulated that experimental hypercholesterolemia is associated with apoptosis in rabbit aortic valves. Methods: New Zealand White rabbits (n = 8) were fed a 1% cholesterol diet for 12 weeks; control rabbits (n = 8) were fed a normal diet. After sacrifice of the animals, the aortic valves were dissected. Apoptosis was identified in the valvular lesion by TdT-mediated dUTP-biotin nick end-labeling (TUNEL) technique, and confirmed with transmission electron microscopy. The number of apoptotic cells was measured by computed morphometry. Results: Valves from hypercholesterolemic rabbits showed an increase in apoptosis. TUNEL staining was identified in the atherosclerotic layer of hypercholesterolemic valves (0.1% of cells), but not in the cells of controls (p <0.0001). Conclusion: Apoptosis is increased in rabbit aortic valves during experimental hypercholesterolemia. If fatal cellular degeneration occurs in hypercholesterolemic valve disease, these data suggest that apoptosis may play a role in the mechanism of valvular disease.

Background and aim of the study: Aortic prosthetic valve endocarditis (PVE) with annular destruction presents a challenge that requires techniques to eradicate the infection and correct the hemodynamic abnormality. Methods: Between July 1, 1996 and March 31, 2000, six patients with native or PVE of the aortic valve and aortic annular destruction underwent surgical treatment. Of these patients, three (two men, one woman; mean age 71.0 years) had circumferential annular destruction of the aortic annulus, and formed the basis of this study. The microorganisms responsible for the infection were Streptococcus spp. in two patients and Staphylococcus aureus in one patient. In addition to aggressive debridement of the infected tissue, repair was achieved by reconstruction of the left ventricular outflow tract with a xenopericardial conduit and fixation of the new prosthetic valve to the conduit. Results: One patient with ventricular septal perforation, multiple systemic embolism and sepsis died of low cardiac output syndrome soon after surgery. Two operative survivors were followed up for 9 and 51 months, with no late deaths. No patient has experienced recurrent infection, pericardial patch aneurysm, or prosthetic valve detachment. Conclusion: These operative procedures provide easy and secure fixation of the pericardial patch to the healthy tissue under excellent operative view, as well as a sturdy structure for the fixation of the new prosthesis, and complete exclusion of the abscess cavity from the blood stream.

Background and aim of the study: Calcific aortic stenosis is common in the elderly; indeed, 30-60% of patients with mild 'senile' aortic stenosis will progress to severe obstruction. Nonetheless, predictors of progression are incompletely defined, and non-invasive technologies capable of quantifying aortic valve calcium are needed. The reliability of electron beam computed tomography (EBCT) was evaluated for quantification of aortic valve calcium content. Methods: Nineteen patients with and without restrictive valve calcification underwent EBCT scanning. Separate calcium scores, 30 s apart, were obtained in all patients, and the Spearman correlation coefficient was calculated between measurements. The relationship between dichotomized mean calcium score and aortic valve area was also investigated. Results: There was excellent correlation between calcium scores (R = 0.99, p = 0.0001), as well as a significant inverse relationship between calcium scores in the upper and lower ranges and aortic valve area (p = 0.002). Conclusion: EBCT can be used for reproducible quantitation of aortic valve calcification. While at their extremes, calcium scores are inversely related to aortic valve area, further evaluation is needed to define the precise nature of this relationship throughout the spectrum of stenosis severity. EBCT holds promise in the longitudinal assessment of valvular calcification progression and its response to potential medical therapies.

Background and aim of the study: The effect of concurrent disease and cardiac comorbidity on survival after bioprosthetic valve replacement in elderly patients was assessed retrospectively. Risk factors were categorized as general, non-cardiac (age, diabetes, previously treated carcinoma) and cardiac (LVEF, three-vessel disease, previous CABG or valve replacement, and endocarditis). Methods: A total of 400 elderly patients (median age 73 years; range: 71-76 years) was studied. Medical history included diabetes, previous CABG or aortic valve replacement (AVR), endocarditis and treatment of previous carcinoma. A left ventricular ejection fraction (LVEF) of <0.66 and presence of three-vessel disease were also investigated. Hospital deaths (and cause) were recorded; survival or date and cause of death after discharge were obtained by questionnaire. Kaplan-Meier univariate and Cox proportional hazards multivariate regression analyses were carried out. Results: Mortality during follow up was 28.3%; hospital mortality was 3.8%. Univariate analysis showed five factors significantly to affect survival: LVEF, history of endocarditis, carcinoma, age and three-vessel disease. Fifteen of 38 patients with history of carcinoma died, 10 due to a malignancy. Of 76 patients with three-vessel disease, 26 died. A history of diabetes and previous CABG did not influence survival significantly. Four of eight patients with preoperative endocarditis died, all in hospital. Six of 11 patients died after redo-AVR, none before 36 months follow up. By Cox regression analysis, LVEF and histories of carcinoma and endocarditis remained significant. Conclusion: AVR should be performed before ventricular deterioration occurs. Previous CABG is not a contraindication for AVR. Endocarditis impaired survival. Long-term mortality after redo-AVR in this population was relatively high, but acceptable. AVR should also be performed in elderly patients with aortic valve disease. Since prognosis of symptomatic aortic valve disease is poor in the short term, AVR is indicated in patients treated for carcinoma.

Background and aim of the study: After aortic valve replacement (AVR), 90% of survivors are in NYHA class I or II, and most return to work after three months. It is unclear, however, whether at that time patients have an improved quality of life (QOL), are able to perform physical and social activities, and live independently. Methods: To assess this situation, 62 patients (39 males, 23 females; mean age 68.5 +/- 10 years) who underwent primary AVR completed the validated Short Form 36 (SF-36) questionnaire. QOL parameters were determined before and three months after surgery prospectively, and analyzed using the Wilcoxon matched pairs rank test. Results: All patients showed significant improvement in all eight QOL parameters (score 0-100): (i) physical function (67 +/- 26 versus 37 +/- 28; p <0.00001); (ii) role limitation due to physical function (52 +/- 43 versus 20 +/- 37; p <0.0001); (iii) social function (80 +/- 25 versus 59 +/- 30; p <0.0001); (iv) role limitation due to emotional problems (64 +/- 41 versus 41 +/- 46; p = 0.01); (v) energy (62 +/- 22 versus 42 +/- 23; p <0.00001); (vi) mental health (78 +/- 19 versus 63 +/- 22; p <0.00001); (vii) pain (78 +/- 27 versus 67 +/- 32; p = 0.02); and (viii) general health perception (72 +/- 20 versus 58 +/- 21; p <0.00001). Significant improvements in QOL parameters were: (i) after mechanical and bioprosthetic AVR, seven of eight QOL parameters were improved; (ii) patients aged < 70 years had pronounced improvement in six of seven parameters, while those aged > or = 70 years had limited improvement in five of eight parameters; (iii) patients with valve sizes 19 and 21 mm improved in five of eight parameters, while those with > or = 23 mm implants improved in all eight parameters, regardless of post-AVR peak gradient of < 20 or > or = 20 mmHg; and (iv) patients with left ventricular end-diastolic dimension (LVEDD) < 55 mm improved in all eight parameters while those with LVEDD > or = 55 mm improved in only five parameters. Conclusion: There was significant improvement in patients' QOL at three months after AVR, regardless of the type of aortic implant used; improvement was greatest in those aged < 70 years. The results also suggest that patients with smaller implants (who were older) and those with LVEDD > or = 55 mm were less likely to show significant improvement in all QOL parameters at three months after AVR.

Background and aim of the study: The study aim was to elucidate the advantages and limitations of the homograft aortic valve for aortic valve replacement over a 29-year period.

Methods: Between December 1969 and December 1998, 1,022 patients (males 65%; median age 49 years; range: 1-80 years) received either a subcoronary (n = 635), an intraluminal cylinder (n = 35), or a full root replacement (n = 352). There was a unique result of a 99.3% complete follow up at the end of this 29-year experience. Between 1969 and 1975, homografts were antibiotic-sterilized and 4 degrees C stored (124 grafts); thereafter, all homografts were cryopreserved under a rigid protocol with only minor variations over the subsequent 23 years. Concomitant surgery (25%) was primarily coronary artery bypass grafting (CABG; n = 110) and mitral valve surgery (n = 55). The most common risk factor was acute (active) endocarditis (n = 92; 9%), and patients were in NYHA class II (n = 515), III (n = 256), IV (n = 112) or V (n = 7). Results: The 30-day/hospital mortality was 3% overall, falling to 1.13 +/- 1.0% for the 352 homograft root replacements. Actuarial late survival at 25 years of the total cohort was 19 +/- 7%. Early endocarditis occurred in two of the 1,022 patient cohort, and freedom from late infection (34 patients) actuarially at 20 years was 89%. One-third of these patients were medically cured of their endocarditis. Preservation methods (4 degrees C or cryopreservation) and implantation techniques displayed no difference in the overall actuarial 20-year incidence of late survival endocarditis, thromboembolism or structural degeneration requiring operation. Thromboembolism occurred in 55 patients (35 permanent, 20 transient) with an actuarial 15-year freedom in the 861 patients having aortic valve replacement +/- CABG surgery of 92% and in the 105 patients having additional mitral valve surgery of 75% (p = 0.000). Freedom from reoperation from all causes was 50% at 20 years and was independent of valve preservation. Freedom from reoperation for structural deterioration was very patient age-dependent. For all cryopreserved valves, at 15 years, the freedom was 47% (0-20-year-old patients at operation), 85% (21-40 years), 81% (41-60 years) and 94% (>60 years). Root replacement versus subcoronary implantation reduced the technical causes for reoperation and re-replacement (p = 0.0098). Conclusion: This largest, longest and most complete follow up demonstrates the excellent advantages of the homograft aortic valve for the treatment of acute endocarditis and for use in the 20+ year-old patient. However, young patients (< or = 20 years) experienced only a 47% freedom from reoperation from structural degeneration at 10 years such that alternative valve devices are indicated in this age group. The overall position of the homograft in relationship to other devices is presented.

Background and aim of the study: Chordal rupture in myxomatous mitral valves is the leading cause of leaflet prolapse and regurgitation. Increased glycosaminoglycan (GAG) content has been reported in these valves. Therefore, the biochemical differences between myxomatous and control mitral valve chordae were investigated. Methods: The contents of hexuronic acid, DNA, water, and collagen in chordae from 45 myxomatous valves and 10 control valves were measured. Collagen and hexuronic acid quantities were normalized to wet and dry weights, and to DNA content. Different GAG classes were measured using fluorophore-assisted carbohydrate electrophoresis (FACE). Results: Myxomatous chordae contained significantly more GAGs than controls after quantities were normalized for wet weight, dry weight, and DNA content. The FACE assay showed that the myxomatous chordae contained significantly more chondroitin/dermatan 6-sulfate when normalized to both wet and dry weight, and slightly more hyaluronan. In contrast to leaflets, which contain predominantly hyaluronan, the predominant GAG class in chordae was chondroitin/dermatan sulfate. Keratan sulfate, a GAG class previously unreported in valve tissues, was also discovered in the chordae. Myxomatous chordae contained more water and less collagen than control chordae, but equal quantities of DNA when normalized for wet weight. Conclusion: Cells in the chordae of myxomatous valves may produce more GAGs than cells in the chordae of control valves. The resulting accumulation of GAGs and bound water likely gives myxomatous valves their characteristic thickening and floppy, gelatinous nature, and may account for their reported mechanical weaknesses.

Background and aim of the study: Chordal rupture is the most common reason for severe mitral regurgitation requiring surgery. The features that predispose myxomatous chordae to rupture, however, have not been studied. Thus, the physical and mechanical properties of normal and myxomatous mitral valve chordae were measured. Methods: Chordae from 24 normal and 59 myxomatous mitral valves were cut into 10 mm-long segments and mechanically tested to measure extensibility, modulus, failure stress, failure strain, and failure load. After testing, the specimens were weighed and their cross-sectional area and volume measured. Results: Chordae from myxoid mitral valves were larger (1.9 +/- 0.1 mm2 versus 0.8 +/- 0.1 mm2, p < or = 0.001) and heavier (16.6 +/- 1.0 mg versus 6.5 +/- 0.4 mg, p < or = 0.001) than normal chordae. Myxoid chordae had significantly lower moduli (40.4 +/- 10.2 MPa versus 132 +/- 15 MPa, p < or = 0.001) and failed at significantly lower tensile stress (6.0 +/- 0.6 MPa versus 25.7 +/- 1.8 MPa, p < or = 0.001) and absolute load (728 +/- 50 g versus 1,450 +/- 135 g, p < or = 0.001) than normal chordae. Normal and myxoid chordae had similar measurements of extensibility and failure strain. Conclusion: Myxomatous degeneration severely affects the mechanical properties of mitral valve chordae. Most notably, myxoid chordae failed at loads one-half of those of normal chordae. This may explain why chordal rupture is the main indication for repair of myxoid mitral valves. These findings also suggest that chordal preservation should be carried out with caution, as myxoid chordae are clearly abnormal with compromised mechanical strength.

Background and aim of the study: Ovine mitral valve closure is associated with presystolic mitral annular reduction coincident with atrial contraction, which is abolished with ventricular pacing. Whether lack of properly timed atrial contraction influences mitral valve closure or competence, however, is not known. Methods: Eight sheep underwent myocardial marker implantation on the left ventricle, mitral annulus (MA), and mitral leaflets. After 7-10 days, the animals were studied with biplane videofluoroscopy at baseline and during ventricular or atrioventricular (AV) sequential pacing. Valve closure was timed from end-diastole (ED) and defined as minimum distance between two leaflet edge markers. ED was defined as peak of ECG R wave, end-systole as peak negative left ventricular (LV) dP/dt, and end-isovolumic contraction (EIVC) as 83.5 ms after ED. Septal-lateral (S-L) annular diameter was defined as distance between two markers at the middle of the anterior and posterior annulus. Regurgitant volume (RV) was calculated as relative volume change between ED and EIVC. Results: V-pacing was associated with delayed leaflet closure (65 +/- 5 versus 29 +/- 10 ms, p = 0.008); moreover, RV (4.1 +/- 0.5 versus 1.4 +/- 0.5 ml, p = 0.02), end-diastolic S-L diameter (2.87 +/- 0.10 versus 2.67 +/- 0.09 cm, p = 0.0005), and MA area (8.12 +/- 0.37 versus 7.26 +/- 0.31 cm2, p = 0.009) all increased. RV and leaflet and annular dynamics during AV-pacing were similar to baseline. Conclusion: V-pacing increased S-L MA diameter by only 8 +/- 1%, but this change was associated with delayed leaflet coaptation and a 16 +/- 1% regurgitant fraction. These findings provide direct evidence that a properly timed atrial contraction is functionally important for effective mitral leaflet closure.

Systolic anterior motion (SAM), a recognized complication of mitral valve repair, is often associated with left ventricular outflow gradient and mitral regurgitation. Current surgery to prevent these conditions is to perform sliding annuloplasty to reduce the posterior mitral leaflet (PML) height and to oversize the annuloplasty ring. However, these techniques do not consistently eliminate post-repair SAM, and removal of excess tissue and reduction of anterior mitral leaflet (AML) height may be more effective; this is the 'Pomeroy procedure'. Here, we report a patient in whom all standard procedures to prevent SAM were performed, but the condition still developed. This was corrected on a second pump run, using the Pomeroy procedure.

Background and aim of the study: Systolic anterior motion (SAM) of the mitral valve resulting in left ventricular outflow obstruction is a well-recognized complication of repair of the degenerative myxomatous mitral valve. A precise mechanism is unknown. A current approach consists of sliding annuloplasty of the posterior leaflet. It was postulated that excess tissue of the anterior mitral leaflet (AML) was as equally (or more) important as the excess posterior mitral leaflet (PML) tissue in the development of SAM subsequent to valve repair. Methods: Thirty-two patients without post-repair SAM (No-SAM group) were compared with eight patients with SAM (SAM group). The AML and PML heights and the mitral annulus diameter were measured by TEE using mid-esophageal four-chamber and long-axis planes. Results: Pre-repair TEE showed the AML height to be greater in the SAM group (p = 0.04), and that of the posterior leaflet tended to be greater (p = 0.08), whilst the annular dimensions were similar in both groups. In the post-repair status, the AML height was markedly greater (p = 0.005) and the annulus markedly smaller (p = 0.001) in the SAM group. Post-repair assessment showed the relative difference between AML height and annular dimension (AML - Ann) as well as the difference between combined leaflet heights and annular dimension (AML + PML - Ann) to be strikingly greater in the SAM group as compared with the No-SAM group (p = 0.001). Conclusion: A disparity between dimension of the annulus following mitral valve repair and combined heights of the two leaflets explains post-repair SAM. The AML height is a more important factor in the development of SAM. Thus, surgical techniques to reduce AML heights should be considered in patients with disproportionately large anterior leaflets in order to prevent SAM. Selection of size of the annuloplasty ring should take into consideration the height of the AML.

Background and aim of the study: Impairment of left ventricular (LV) function after mitral valve replacement (MVR) has been the most important factor to determine morbidity and mortality. With this in mind, LV performance in the postoperative period was assessed with and without preservation of papillo-annular continuity in MVR. Methods: Between March 1994 and August 1998, a total of 383 valve prostheses (202 MVR, 65 AVR, 58 MVR+AVR) were implanted in 325 patients, 177 of whom underwent MVR with Starr Edwards ball cage prostheses (the study group). Of these 177 patients, 105 had MVR with preservation of the posterior mitral leaflet (group I), and 72 had conventional MVR (group II). Predominant lesions were mitral stenosis in 81, mitral regurgitation in 42, and mixed mitral lesion (MS/MR) in 54. Concomitant tricuspid valve annuloplasty was performed in 13, and atrial septal defect repair in five. Sixteen patients underwent MVR for mitral restenosis. In-vivo performance of the prostheses and LV function was evaluated by M-mode and Doppler echocardiography. Results: At 3-6 months clinical improvement was seen in NYHA class, with reduction in cardiothoracic ratio among patients with preserved papillo-annular continuity, irrespective of lesion type. Significant reductions (p <0.05) were seen in left atrial dimensions (54.10 +/- 8.79 preop. versus 44.64 +/- 8.54 postop.; p <0.05), left ventricular end-diastolic dimensions (LVEDD) (50.84 +/- 10.42 preop. versus 41.21 +/- 7.16 postop.; p <0.05) and end-systolic dimensions (LVESD) (34.76 +/- 7.94 preop. versus 28.81 +/- 5.79 postop.; p <0.05) in patients who had their posterior mitral leaflet preserved with significant improvement in ejection fraction (60.31 +/- 8.22 versus 64.47 +/- 7.93; p <0.05). Further analysis of data in group I patients showed significant reductions in left atrial dimensions, LVESD and peak gradient, along with improved ejection fraction compared with conventional (group II) patients. Conclusion: Deterioration in LV function in patients undergoing conventional MVR indicates chordal resection as a putative mechanism. This study supports the concept that maintenance of continuity between the mitral annulus and papillary muscles has a beneficial effect on postoperative LV function, and is particularly important in patients with mitral stenosis with depressed preoperative LV systolic function.

Background and aims of the study: Closed mitral commissurotomy (CMC) is a well-established method for treatment of rheumatic mitral stenosis, but outcome in patients with severe pulmonary arterial hypertension (PAH) has not been clearly documented. Methods: Between April 1996 and October 1999, among 61 patients who underwent CMC, 27 had severe PAH (systolic pressure > 100 mmHg). Of these patients, 11 were in NYHA class III, and 16 were in class IV. Preoperatively, the mean pulmonary artery (PA) pressure was 107.85 +/- 5.74 mmHg (range: 100-118 mmHg), mitral valve area (MVA) 0.704 +/- 0.106 cm2 (range: 0.5-0.91 cm2), and transmitral gradient 11.93 +/- 1.54 mmHg (range: 10-15 mmHg). The echocardiographic mitral valve score was 6.37 +/- 1.11 (range: 6-10). Results: There was no operative mortality or incidence of significant (> or = 2+) post-CMC mitral regurgitation or cerebrovascular accident. The MVA increased to 2.385 +/- 0.248 cm2 (range: 1.9-2.8 cm2), the transmitral gradient fell to 2.44 +/- 0.51 mmHg (range: 2-3 mmHg), and postoperative PA systolic pressure fell to 33.33 +/- 8.20 mmHg (range: 30-60 mmHg). During a mean follow up of 26.9 months (range: 11-51 months), 23 patients were in NYHA class I and four were in class II. There were no significant differences in parameters between sexes, but mean male age was five years less than mean female age. Conclusion: In the subset of patients with severe PAH, surgical CMC is a safe and effective procedure that results in greater MVA and a more significant and sustained fall in PA pressure compared with reported series of percutaneous balloon mitral valvuloplasty.

Background and aim of the study: Closed mitral commissurotomy (CMC) and percutaneous mitral balloon valvuloplasty (PMBV) were compared by their initial results and Doppler echocardiographic data obtained at one week and one year after the procedure. Methods: Of 580 patients with severe rheumatic mitral stenosis, 280 underwent CMC and 300 PMBV. The mean pre-procedural transmitral gradient (TMG) was 21 +/- 6 mmHg in the CMC group and 20 +/- 5 mmHg in the PMBV group (p = 0.6); the mean mitral valve area (MVA) was 1.1 +/- 0.2 cm2 in both groups. Results: Mortality was 0.7% after CMC and 0.3% after PMBV; the primary success rates were 98.3% and 89% respectively (p <0.0001). Two CMC patients and three PMBV patients underwent emergency mitral valve replacement. At the first week, the mean TMG was decreased to 4 +/- 3 mmHg in the CMC group, and to 5.8 +/- 2 mmHg in the PMBV group (p <0.0001). The mean MVA was increased to 2.5 +/- 0.5 cm2 after CMC, and to 2.1 +/- 0.4 cm2 after PMBV (p <0.0001). After one year, TMG was 5.4 +/- 4 mmHg in the CMC group (p <0.0001) and 7.1 +/- 3 mmHg in the PMBV group (p <0.0001); MVA was 2.3 +/- 0.5 cm2 (p <0.0001) and 1.9 +/- 0.4 cm2 (p <0.0001), respectively. The results of CMC were significantly better (p <0.0001) with regard to TMG and MVA at these times. A significant decrease was also seen in mean left atrial diameter and pulmonary artery pressure in both groups (p <0.0001). Conclusion: Although satisfactory results can be achieved using either approach, CMC provides a higher primary success rate, greater MVA augmentation, and better technical control during the procedure, while reducing the cost. PMBV shortens in-hospital stay and eliminates the risk imposed by thoracotomy and anesthesia. Therefore, in our practice, when surgical intervention is contraindicated due to associated problems, PMBV may be the preferred approach, but exposure to radiation may be of concern in pregnant patients.