Pediatric valve replacement, 15 years experience in Oman

Background Despite improving surgical techniques, treatment of heart valve disease in children remains controversial. Somatic growth and adequate anticoagulation are of concern when children undergo valve replacement. We conducted this study to evaluate the performance of valves in this age group. Methods 42 children under the age of 13 years who underwent valve replacement were included in this study. Totally, 50 valves were implanted in 42 patients: 48 were mechanical prostheses, two were bioprosthetic both in pulmonary position. 37 (74%) valves were implanted in mitral position, 10 (20%) in aortic position, 1 (2%) in tricuspid position and 2 (4%) in pulmonary position. Preoperatively, 14 (33,3%) patients were in New York Heart Association (NYHA) class IV, while 27 (64.2%) were in NYHA class III. Results There were 2 (4.7%) hospital deaths and 2 (4.7%) late deaths while 2 (4.7%) patients were lost to follow up. The mean follow up period was 9.4 yrs. 35 (83.3%) patients are in NYHA Class I and free of all medications except warfarin. 3 (7.1%) patients have undergone 5 successful pregnancies. The median INR was 2.23. Major thrombo-embolic episode occurred in 1 (2.3%) patient. Conclusions In view of the problems of sizing, anticoagulation and need for re-operation at an early age, there is a reluctance to replace valves in children. This study shows that despite these problems, valve replacement can be undertaken safely and successfully in children, when repair has failed or not technically feasible.     

计算机技术定量分析三尖瓣返流患者右室收缩和舒张功能的研究

本研究对３０例三尖瓣返流患者同步进行了多普勒超声心动图和右心导管检查，应用计算机软件程序对三尖瓣返流频谱曲线进行微分处理。结果表明，两种技术测量的右室压力最大上升速率、右室心肌最大生理缩短速度、右室压力最大下降速率以及右室心肌松驰时间常数均高度相关（ｒ分别为０．９３，０．８６，０．９４，０．９５），表明三尖瓣返流压差法能够无创性估测右室收缩和舒张功能，应用计算机软件程序能够使测量方法简便、准确。     

Factors influencing the variations of ejection fraction during exercise in chronic aortic regurgitation

The influence of left ventricular volume variations and regurgitant fraction variations upon left ventricular ejection fraction, during exercise was examined using equilibrium radionuclide angiography in patients suffering from aortic regurgitation. Ejection fraction (EF), regurgitant fraction (RF), end diastolic volume (EDV) and end systolic volume (ESV) variations from rest to peak exercise were determined in 44 patients suffering from chronic aortic regurgitation (AR) and in 8 healthy volunteers (C). In C, EF increased (+0.10±0.03, P<0.01) and ESV decreased significantly (-23%±12%, P<0.01) RF and EDV did not vary significantly. In AR patients, EF, EDV and ESV did not vary significantly because of important scattering of individual values. Changes in EF and ESV were inversely correlated (r=-0.79, P<0.01) and RF decreased significantly (-0.12±0.10, P<0.01). Volumes and EF changes during exercise occurred in three different ways. In a 1st subgroup of 7 patients, EF increased (+0.09±0.03, P<0.05) in conjunction with a reduction of ESV (-24%±12%, P<0.05) without a significant change in EDV. In a 2nd group of 22 patients. EF decreased (-0.04±0.07, P<0.01) in association with an increase in ESV (+17%±16%, P<0.01) and no change in EDV. In a 3rd subgroup of 15 patients, EF decreased (-0.02±0.06, P<0.01) despite a reduction in ESV (-7%±6%, P<0.01) because of a dramatic EDV decrease (-10%±6%, P<0.05). In this subgroup, changes in EF were inversely correlated with changes in ESV (r=-0.55, P<0.01) and positively related to EDV variations (r=0.42, P=0.02). EDV related to EDV variations (r=0.42, P=0.02). EDV changes were weakly, but significantly, correlated to RF decrease (r=0.39, P<0.05). We conclude that changes in left ventricular ejection fraction during exercise in patients with chronic aortic regurgitation are significantly related in some patients to changes in ventricular loading conditions as well as contractile state. Therefore, a correct interpretation of EF changes during exercise requires the simultaneous determination of changes in LV volumes.Abbreviations EDV end diastolic volume - EF ejection fraction - ESV end systolic volume - LV left ventricle - RV right ventricle     

彩色多普勒在心脏起搏器综合征诊断中的应用价值

目的 :探讨运用彩色多普勒血流显像 (CDFI)并结合心电图 (ECG)诊断起搏器综合征 (PMS)的临床价值。方法 :对具有详实临床及ECG资料的心室按需 (VVI)起搏器安置患者 6 0例及对照组 4 8例进行CDEI检查 ,观察起搏组与对照组的三尖瓣返流 (TR)的发生率、返流程度 ,并分析二者之间的相关性。结果 :起搏组出现逆传P波 (即PMS阳性 )者 15例 ,CDFI检测出现TR者 2 0例 ,明显高于对照组 ,且PMS阳性者TR发生率明显高于PMS阴性者 ,表明安置VVI起搏器后出现逆传P波与TR的发生相关性良好。结论 :CDFI对PMS的诊断具有重要意义 ,尤其将ECG与CDFI结合应用有助于诊断率的提高。     

Role of Papillary Muscle Dysfunction in the Pathogenesis of Mitral Regurgitation in Myocardial Infarction:

To elucidate the pathogenesis of mitral regurgitation (MR) after myocardial infarction (MI), the incidence of papillary muscle dysfunction (PMD), mitral annular size, and the extent of wall-motion abnormalities were examined in 81 patients with previous MI by two-dimensional echocardiography and real-time two-dimensional Doppler flow imaging. The prevalence of pathological MR was lower in patients with anterior MI (36%) than in those with inferior (65%) or anterior and inferior MI (88%) (P < 0.01 vs anterior MI group). The incidence of PMD in patients with MR in the anterior MI group (15%) was lower than that in the inferior (50%, P < 0.01) or anterior and inferior MI group (43%, P < 0.05). The mitral annular dimension in patients with MR was significantly greater than in those without MR, but it was similar among the three groups. The extent of wall-motion abnormality correlated significantly with the area of MR jet in the anterior MI group (y = 3.1x + 15.5, r = 0.52, P < 0.01) and in the inferior MI group (y = 8.3x + 32.7, r = 0.57, P < 0.01). However, the slope of this relationship was significantly steeper in the inferior MI group than in the anterior MI group (P < 0.05). These results indicated that the degree of MR with inferior MI was greater than with anterior MI for a given MI area. PMD may play an important role in the higher prevalence and greater degree of MR in inferior MI.     

球囊二尖瓣扩张术后心房纤颤复律的临床研究

目的探讨二尖瓣狭窄合并心房纤颤患者在球囊二尖瓣扩张后,转复心房纤颤的临床效果及影响心房纤颤转复后窦性心律维持的相关因素.方法 206例二尖瓣狭窄合并心房纤颤患者在球囊扩张术后,采用口服胺碘酮及电复律治疗心房纤颤,对复律成功的患者给予小剂量胺碘酮维持窦性心律并随访1年.结果服用胺碘酮者58例,17例转复成功(29.3%);189例行电转复,178例转复成功(94.2%),电转复成功率明显高于胺碘酮转复(P＜0.01).心房纤颤复发组与保持窦性心律组相比,左心房内径明显扩大(P＜0.01),心房纤颤持续时间明显长(P＜0.01),中度二尖瓣关闭不全人数的比率明显高(P＜0.05).随访1年,有45例患者心房纤颤复发.影响心房纤颤转复后维持窦性心律的相关因素有左心房内径扩大、心房纤颤持续时间长及明显的二尖瓣关闭不全.结论对左心房内径明显扩大、心房纤颤持续时间过长及有明显的二尖瓣关闭不全的患者,在选择心房纤颤转复时应慎重.     

Concomitant mitral valve repair and left ventricular reconstruction in a patient with chronic ischemic mitral regurgitation and inferior left ventricular aneurysm

The surgical approach to ischemic mitral regurgitation with concomitant inferior left ventricular aneurysm remains uncertain in terms of the indication for operation and the short-and long-term outcomes. We performed concomitant mitral valve repair, left ventricular reconstruction, and aortic valve replacement on a 71-year-old male with severe ischemic mitral regurgitation, inferior left ventricular aneurysm, and degenerative aortic regurgitation. Postoperative status was in New York Heart Association functional class I without mitral regurgitation 8 months after operation. We discuss, and review the procedures reported in the literature.     

Unusual presentation and clinical variability in Belgian pedigrees with progressive external ophthalmoplegia and multiple deletions of mitochondrial DNA

We studied 14 patients from three unrelated Belgian pedigrees with a familial mitochondrial disorder and multiple deletions of mitochondrial DNA (mtDNA). In one family with an oculopharyngeal presentation there is a clear autosomal dominant inheritance. Progressive external ophthalmoplegia (PEO), “ragged red fibres” (RRF) and multiple deletions of mtDNA are common to all three families. Therefore a diagnosis of autosomal dominant progressive ophthalmoplegia with multiple deletions of mtDNA (adPEO) was made in one family at least. Our data confirm the previous observations that adPEO is a systemic disorder rather than a pure myopathy. In our pedigrees frequently associated features include axonal peripheral neuropathy, dysphagia, psychiatric illness, and sudden death. Mild ataxia, pes cavus and mitral valve prolapse with associated mitral insufficiency also occur. In some cases onset is atypical with neuropathy, adolescent onset myopathy or psychiatric illness. In such cases the common features of PEO and muscle weakness always complete the clinical phenotype later during the course of the disease. Biochemical studies on mitochondrial fractions prepared from one patient's muscle, revealed no abnormalities of respiratory chain enzyme activities.