Ebstein's anomaly with imperforate tricuspid valve. Prenatal diagnosis

Ebstein's anomaly is an uncommon congenital heart defect, with a prevalence of 0.3-0.5%. Its association with an imperforate tricuspid valve is an even more rare situation (less than 10% of cases). Prenatal diagnosis of this association by means of fetal echocardiography has not been reported. We describe here this association diagnosed before birth and confirmed after birth. The diagnostic potential and importance of fetal echocardiography during prenatal evaluation of cardiac malformations allows for adequate perinatal planning and management, with an obvious impact on morbidity and mortality.

Mitral valve repair with a malleable bovine pericardium ring

OBJECTIVE: To describe a surgical procedure utilizing a malleable bovine pericardium ring in mitral valve repair and clinical and echodopplercadiographic results. METHODS: Thirty-two (25 female and 7 male) patients, aged between 9 and 66 (M=36.4±17.2) years, were studied over a 16-month period, with 100% follow-up. In 23 (72%) of the patients, the mitral approach was the only one applied; 9 patients underwent associated operations. The technique applied consisted of measuring the perimeter of the anterior leaflet and implanting, according to this measurement, a flexible bovine pericardium prosthesis for reinforcement and conformation of the posterior mitral annulus, reducing it to the perimeter of the anterior leaflet with adjustment of the valve apparatus. RESULTS: The patient survival ratio was 93.8%, with 2 (6.2%) fatal outcomes, one from unknown causes, the other due to left ventricular failure. Only one reoperation was performed. On echodopplercardiography, 88% of the patients had functional recovery of the mitral valve (50% without and 38% with mild insufficiency and no hemodynamic repercussions). Of four (12%) of the remaining patients, 6% had moderate and 6% had seigre insufficiency. Twenty-eight percent of class II patients and 72% of class III patients passed into classes I (65%), II (32%), and III (3%), according to NYHA classification criteria. CONCLUSION: Being flexible, the bovine pericardium ring fit perfectly into the valve annulus, taking into account its geometry and contractility. Valve repair was shown to be reproducible, demonstrating significant advantages during patient evolution, which did not require anticoagulation measures.

Changes in the profile of lipoprotein subfractions associated with hormone replacement therapy

OBJECTIVE: To report the effects of 2 regimens of hormone replacement therapy during the postmenopausal period on the profile of the major lipoprotein subfractions (HDL, LDL, and VLDL). METHODS: We carried out a cohort study in 38 postmenopausal patients who were starting their hormone replacement therapy due to gynecological indications, for a period of 12 weeks. Analysis of lipoprotein subclasses was performed through nuclear magnetic resonance spectroscopy. RESULTS: Hormone replacement therapy cause an increase in the proportion of larger subfractions of VLDL and HDL (p=0.008 and 0.03, respectively) and in the proportion of larger particles of VLDL due to a 36% increase in the levels of larger particles (p=0.004), concomitantly with a 15% reduction in the levels of smaller particles (p=0.04). In regard to HDL, the increase occurred only a 17% increase in the levels of larger particles (p=0.002). No significant change occurred in the distribution pattern of LDL subfractions. CONCLUSION: The proportion of larger subfractions of VLDL and HDL increases after hormone replacement therapy. The increase in the proportion of larger particles of VLDL occurs due to an increase in the levels of the larger subclasses concomitantly with a reduction in the smaller particles. However, an increase in the proportion of larger particles of HDL occurs only due to an increase in the levels of the larger subfractions.

Unsupported valvuloplasty in children with congenital mitral valve anomalies. Late clinical results

OBJECTIVE: To analyze late clinical evolution after surgical treatment of children, with reparative and reconstructive techniques without annular support. METHODS: We evaluated 21 patients operated upon between 1975 and 1998. Age 4.67±3.44 years; 47.6% girls; mitral insufficiency 57.1% (12 cases), stenosis 28.6% (6 cases), and double lesion 14.3% (3 cases). The perfusion 43.10±9.50min, and ischemia time were 29.40±10.50min. The average clinical follow-up in mitral insufficiency was 41.52±53.61 months. In the stenosis group (4 patients) was 46.39±32.02 months, and in the double lesion group (3 patients), 39.41±37.5 months. The echocardiographic follow-up was in mitral insufficiency 37.17±39.51 months, stenosis 42.61±30.59 months, and in the double lesion 39.41±37.51 months. RESULTS: Operative mortality was 9.5% (2 cases). No late deaths occurred. In the group with mitral insufficiency, 10 (83.3%) patients were asymptomatic (p=0.04). The majorit y with mild reflux (p=0.002). In the follow-up of the stenosis group, all were in functional class I (NYHA); and the mean transvalve gradient varied between 8 and 12mmHg, average of 10.7mmHg. In the double lesion group, 1 patient was reoperated at 43 months. No endocarditis or thromboembolism were reported. CONCLUSION: Mitral stenosis repair has worse late results, related to the valve abnormalities and associated lesions. The correction of mitral insufficiency without annular support showed good long-term results.

Fibroelastoma of the mitral valve as a cause of transient ischemic stroke

A 44-year-old woman had a transient ischemic stroke, fibroelastoma of the mitral valve being the source of the embolus. The patient evolved with neutropenia induced by ticlopidine after 10 days of treatment. We report the major clinical features, therapeutical options, and medicamentous toxicity resulting from the use of antiplatelet drugs.

Mini-sternotomy for the treatment of aortic valve lesions

OBJECTIVE: To compare inverted-L mini-sternotomy performed above the sternal furcula with conventional sternotomy in patients with aortic valve diseases who undergo surgical treatment. METHODS: We operated upon 30 patients who had aortic valve lesions that had clinical and hemodynamic findings. All patients underwent inverted-L sternotomy, which extended from above the manubrium of the sternum to the 3rd right intercostal space, without opening the pleural cavity. Their ages ranged from 32 to 76 years, and 18 were males and 12 were females. We used negative pressure in a venous ¼-inch cannula, and the patients were maintained in Trendelemburg's position. Twenty-seven patients received bioprostheses with diameters ranging from 23 to 29mm. Three patients underwent only removal of the calcifications of the aortic valve leaflets and aortic commissurotomy. RESULTS: The mean duration of anoxic cardiac arrest was 63.11min. Access was considered good in all patients. One death was due to pulmonary and renal problems not related to the incision. All patients had a better recovery in the intensive care unit, got out of bed sooner, coughed more easily, and performed prophylactic physiotherapeutic maneuvers for respiratory problems more easily and with less pain in the incision. Early ambulation was more easily carried out by all patients. CONCLUSION: Mini-sternotomy proved to be better than the conventional sternotomy because it provided morecomfort for the patients in the early postoperative period, with less pain and greater desire for early ambulation and all its inherent advantages.

New percutaneous techniques for perforating the pulmonary valve in pulmonary atresia with intact ventricular septum

We report new percutaneous techniques for perforating the pulmonary valve in pulmonary atresia with intact ventricular septum, in 3 newborns who had this birth defect. There was mild to moderate hypoplastic right ventricle, a patent infundibulum, and no coronary-cavitary communications. We succeeded in all cases, and no complications related to the procedure occurred. The new coaxial radiofrequency system was easy to handle, which simplified the procedure. Two patients required an additional source of pulmonary flow (Blalock-Taussig shunt) in the first week after catheterization. All patients had a satisfactory short-term clinical evolution and will undergo recatheterization within 1 year to define the next therapeutic strategy. We conclude that this technique may be safely and efficiently performed, especially when the new coaxial radiofrequency system is used, and it may become the initial treatment of choice in select neonates with pulmonary atresia and intact ventricular septum.

Assessment of Thromboembolism After the Cox-Maze Procedure for Chronic Atrial Fibrillation Secondary to Mitral Valve Lesion

OBJECTIVE: To assess the occurrence of late thromboembolism after surgical repair of chronic atrial fibrillation (AF) simultaneously with repair of mitral valve using the Cox-Maze procedure. METHODS: 69 patients underwent Cox 3 procedure, with no cryoablation simultaneously with mitral valvuloplasty or prosthesis. Mean age was 49.9±13.2 years. Mean follow-up was of 31.7±19 months. Types of lesion were as follows: 33 (48%) stenoses, 23 (33%) insufficiencies, and 13 (19%) double lesions. Procedures were: 64 (93%) valvuloplasties, 3 (4%) biological and 2 (3%) mechanical prosthesis placement. There were 9 (13%) patients with previous systemic embolism and 2 (3%) had left atrial thrombi. RESULTS: Early mortality was 7% and late 1%. 2 patients (3%) were reoperated for mitral placement. At last evaluation, 10 patients (15%), were in AF. The remaining 59 (85%) were either in sinus / atrial rythm (74%) or under pacing (12%). There were no occurrence of early or late, systemic or pulmonary embolism. Permanent anticoagulation was employed in 16 cases, 10 in regular rythm and 6 in AF. The remaining 47 (75%), 2 in AF and 45 in regular rythm, did not receive anticoagulants. CONCLUSIONS: These results are in accordance with others series, where the occurrence of embolism was rare after maze procedure. Permanent systemic anticoagulation seems to be unnecessary in those cases.

Surgical treatment of partial atrioventricular septal defect: functional analysis of the mitral valve in the postoperative period

OBJECTIVE: To study mitral valve function in the postoperative period after correction of the partial form of atrioventricular septal defect. METHODS: Fifty patients underwent surgical correction of the partial form of atrioventricular septal defect. Their mean age was 11.8 years and 62% of the patients were males. Preoperative echocardiography showed moderate and severe mitral insufficiency in 44% of the patients. The mitral valve cleft was sutured in 45 (90%) patients (group II - GII). Echocardiographies were performed in the early postoperative period, and 6 and 12 months after hospital discharge. RESULTS: The patients who had some type of arrhythmia in the postoperative period had ostium primum atrial septal defect of a larger size (2.74 x 2.08 cm). All 5 patients in group I (GI), who did not undergo closure of the cleft, had a competent mitral valve or mild mitral insufficiency in the preoperative period. One of these patients began to have moderate mitral insufficiency in the postoperative period. On the other hand, in GII, 88.8% and 82.2% of the patients had competent mitral valve or mild mitral insufficiency in the early and late postoperative periods, respectively. CONCLUSION: The mitral valve cleft was repaired in 90% of cases. Echocardiography revealed competent mitral valve or mild mitral insufficiency in 88.8% and 82.2% of GII patients in the early and late postoperative periods, respectively.

Evaluation of the marker of hypercoagulability prothrombin fragment F 1+2 in patients with mechanical or biological heart valve prostheses

OBJECTIVE: To investigate whether patients with heart valve prostheses and similar International Normalized Ratios (INR) have the same level of protection against thromboembolic events, that is, whether the anticoagulation intensity is related to the intensity of hypercoagulability supression. METHODS: INR and plasma levels of prothrombin fragment 1+2 (F1+2) were assessed in blood samples of 27 patients (7 with mechanical heart valves and 20 with biological heart valves) and 27 blood samples from healthy donors that were not taking any medication. RESULTS: Increased levels of F1+2 were observed in blood samples of 5 patients with heart valve prostheses taking warfarin. These findings reinforce the idea that even though patients may have INRs, within the therapeutic spectrum, they are not free from new thromboembolic events. CONCLUSION: Determination of the hypercoagulability marker F1+2 might result in greater efficacy and safety for the use of oral anticoagulants, resulting in improved quality of life for patients.

Debranching Solutions in Endografting for Complex Thoracic Aortic Dissections

Background: Conventional surgical repair of thoracic aortic dissections is a challenge due to mortality and morbidity risks. Objectives: We analyzed our experience in hybrid aortic arch repair for complex dissections of the aortic arch. Methods: Between 2009 and 2013, 18 patients (the mean age of 67 ± 8 years-old) underwent hybrid aortic arch repair. The procedural strategy was determined on the individual patient. Results: Thirteen patients had type I repair using trifurcation and another patient with bifurcation graft. Two patients had type II repair with replacement of the ascending aorta. Two patients received extra-anatomic bypass grafting to left carotid artery allowing covering of zone 1. Stent graft deployment rate was 100%. No patients experienced stroke. One patient with total debranching of the aortic arch following an acute dissection of the proximal arch expired 3 months after TEVAR due to heart failure. There were no early to midterm endoleaks. The median follow-up was 20 ± 8 months with patency rate of 100%. Conclusion: Various debranching solutions for different complex scenarios of the aortic arch serve as less invasive procedures than conventional open surgery enabling safe and effective treatment of this highly selected subgroup of patients with complex aortic pathologies.

Multimodality Imaging of Heart Valve Disease

Unidentified heart valve disease is associated with a significant morbidity and mortality. It has therefore become important to accurately identify, assess and monitor patients with this condition in order that appropriate and timely intervention can occur. Although echocardiography has emerged as the predominant imaging modality for this purpose, recent advances in cardiac magnetic resonance and cardiac computed tomography indicate that they may have an important contribution to make. The current review describes the assessment of regurgitant and stenotic heart valves by multimodality imaging (echocardiography, cardiac computed tomography and cardiac magnetic resonance) and discusses their relative strengths and weaknesses.