Clinical Presentation and Therapy of Atrioventricular Septal Defect.

Atrioventricular septal defects (AVSDs) consist of a number of cardiac malformations that result from abnormal development of the endocardial cushions. AVSDs occur in 0.19 of 1000 live births and constitute 4-5 % of congenital heart defects. AVSDs can be categorized as incomplete (or partial) or complete, and intermediate or transitional.

Percutaneous Mitral Valve Repair in Pediatric Patients.

Rigorous clinical trials have demonstrated the safety and efficacy of Transcatheter Edge-to-Edge Repair to treat severe secondary mitral regurgitation (MR) in adults with primary cardiomyopathy who have failed guideline-directed medical therapy, as well as those with primary MR at high surgical risk. To date, there are only three case reports describing this procedure in the pediatric population. We report a case series of four pediatric patients, including the youngest and smallest reported, who underwent this procedure.

[Acute mitral insufficiency revealed by unilateral Flash Pulmonary Edema]. / L'image du mois. Insuffisance mitrale aiguë révélée par un œdème aigu du poumon unilatéral.

Flash pulmonary edema (2 % of FPE cases) is observed in 25 % of cases of acute mitral insufficiency. This clinical disorder is often mistaken for infectious pneumonitis and treated as such, with as consequence an increased mortality of these patients. The diagnosis of acute mitral insufficiency is therefore essential for the optimal management of these patients. The etiologies of mitral insufficiency can be of primary or secondary origin. Cardiac ultrasound (trans-thoracic and trans-esophageal) represent the key to the diagnosis of acute mitral insufficiency and to ensure an adapted management. It consists initially in stabilizing the patient's condition (which may be critical) via pharmacological treatments and/or mechanical support. Once stabilized, the patient can benefit from a definitive treatment of the mitral insufficiency either via valve repair (plasty) or its complete replacement.

Un oedème aigu du poumon unilatéral (2 % des cas) est retrouvé dans 25 % des cas d'insuffisance mitrale aiguë. Celui-ci est souvent confondu à tort avec une pneumopathie infectieuse et traité comme telle avec, pour conséquence, une augmentation de la mortalité. Le diagnostic d'une insuffisance mitrale aiguë est donc primordial pour une prise en charge optimale du patient. Les étiologies de celle-ci peuvent être d'origine primaire ou secondaire. L'échographie cardiaque (trans-thoracique et trans-oesophagienne) sera la clef du diagnostic d'insuffisance mitrale aiguë pour assurer une prise en charge adaptée. Celle-ci consiste, dans un premier temps, à stabiliser l'état du patient (qui peut être critique) via des traitements médicamenteux ou/et un support mécanique. Ceci permettra d'amener le patient dans des conditions optimales afin qu'il puisse bénéficier d'un traitement définitif de l'insuffisance mitrale, que ce soit via une plastie ou un remplacement complet de la valve.

[Mitral valve replacement in patients with obstructive hypertrophic cardiomyopathy]. / Protezirovanie mitral'nogo klapana u bol'nykh obstruktivnoi gipertroficheskoi kardiomiopatiei.

OBJECTIVE: To analyze the incidence and causes of mitral valve replacement in patients with obstructive hypertrophic cardiomyopathy (HCM). MATERIAL AND METHODS: There were 172 patients (98 women and 74 men) with obstructive HCM and severe mitral valve insufficiency between November 2017 and May 2023. All patients complained of chronic heart failure NYHA class III. Surgical correction of left ventricular outflow tract (LVOT) obstruction and mitral valve repair with elimination of systolic anterior motion were technically successful in 160 (93.0%) patients. RESULTS: The need for mitral valve replacement was noted in 12 (7.0%) patients. Mean cardiopulmonary bypass time was 83.5±19.2 min (94; 127), aortic cross-clamping - 62.8±14.3 min (70; 102). In 5 cases, primary mitral valve replacement was scheduled due to obvious organic lesion of the mitral valve (tearing of chords, rheumatic lesion with leaflet restriction). In 7 patients, valve replacement was forced after ineffective primary septal myectomy (LVOT pressure gradient, severe mitral insufficiency). CONCLUSION: Mitral valve replacement is an involuntary strategy after ineffective myectomy with severe mitral insufficiency and high LVOT pressure gradient.

Post-infarct mitral insufficiency: when to resort to reparative surgery, when to the mitral clip.

Severe mitral insufficiency is a feared complication that develops in 10-12% of patients with myocardial infarction and ST elevation. It results from the rupture of the papillary muscle or is secondary to an acute remodelling of the left ventricle in its entirety or of the infarcted region. The mortality of patients with acute clinical onset reaches 50%. The ideal treatment of acute mitral insufficiency is controversial and still a source of debate. Most of these patients are at high surgical risk or inoperable; therefore, they are treated conservatively and have a poor prognosis. In these candidates, percutaneous treatment of insufficiency with percutaneous edge to edge can be considered an alternative.

Prevalence and follow-up of subclinical rheumatic heart disease among asymptomatic school children in a north-western district of India based on the World Heart Federation echocardiographic criteria.

BACKGROUND: The present study is based on the World Heart Federation (WHF) echocardiographic criteria to assess the prevalence of subclinical rheumatic heart disease (RHD) and elucidate evolution of the disease when the cases were placed on appropriate antibiotic prophylaxis and regular follow-up. The prevalence of subclinical RHD reported by previous active surveillance studies among asymptomatic school children is not comparable to our study because of major differences in screening methods. METHODS: A random inclusion strategy was adopted to recruit urban and rural school children of Bikaner district in the state of Rajasthan, India. The diagnosis of RHD was based on the echocardiographic criteria proposed by the WHF. All studies were reported on-site by a single experienced cardiologist and the digitally preserved studies were reported by a second cardiologist off-site. The final diagnosis was made by consensus. The second echocardiogram was performed for cases diagnosed with RHD after two years from start of study to document early evolution of the disease with ongoing antibiotic prophylaxis. RESULTS: A high prevalence of subclinical RHD was observed in the study population. Pathological mitral and/or aortic valve regurgitation was the commonest lesion, and a significant proportion of cases improved while on regular antibiotic prophylaxis. No case showed fixity of leaflets/ stenosis. CONCLUSION: The prevalence of subclinical RHD is high in the study population, and the disease seems to regress over time in the presence of appropriate antibiotic prophylaxis.

Himalayan P wave in a patient admitted for cardiac surgery.

Giant or Himalayan P waves are tall and peaked, the most prominent in lead II, and generally indicate enlargement of the right atrium. It has been rarely seen in clinical practice. The mechanism of Himalayan P wave formation is most likely related with prolonged conduction of electrical impulses through the enlarged right atrium. We describe the case of a patient with Himalayan P wave admitted for cardiac surgery.

Elevated antistreptolysin O titer is closely related to cardiac mitral insufficiency in untreated patients with Takayasu arteritis.

BACKGROUND: The etiology of Takayasu arteritis (TA) is unknown; however, a possible relationship between streptococcal infection and TA has been proposed. This study aimed to identify the clinical features and cardiac valvular involvement in untreated TA patients with an elevated antistreptolysin O (ASO) titer. METHODS: In this retrospective study, the clinical characteristics and features of valvular involvement were compared in TA patients with or without an elevated ASO titer. RESULTS: Of the 74 untreated TA patients, 13 patients were found have elevated ASO titers (17.6%). Mitral insufficiency was the most common in patients with elevated ASO (69.2%, 9/13), followed by aortic valve insufficiency (46.2%, 5/13) and tricuspid insufficiency (46.2%, 5/13), which were no significantly different than that in normal ASO group. The proportions of moderate to severe mitral (30.8% vs 1.6%, p = 0.000) and tricuspid valve (15.4% vs 1.64%, p = 0.023) insufficiency in the ASO positive group were significantly higher than those in the ASO negative group. The odds of mitral regurgitation in patients with elevated ASO titers were 3.9 times higher than those in the group with normal ASO titers (p = 0.053, OR = 3.929, 95% confidence interval [CI]: 0.983-15.694). Furthermore, the risk of moderate to severe mitral insufficiency in patients with elevated ASO titers was 41.6 times higher than that in patients with normal ASO titers (p = 0.002, OR = 41.600, 95% CI: 3.867-447.559). CONCLUSIONS: An increase in ASO titer is related to valvular involvement in TA and is closely linked to mitral insufficiency.

The treatment of mitral insufficiency in refractory heart failure.

Secondary mitral insufficiency (SMI) is caused by dilatation and left ventricular dysfunction and is a frequent finding in patients with heart failure (HF). It is associated with a mortality of between 40% and 50% at 3 years. The first-line treatment is represented by medical therapy, possibly associated, when indicated, with cardiac re-synchronization. If the patient remains symptomatic, corrective action should be considered. Surgery is indicated in cases of severe SMI with ejection fraction >30% and the need for myocardial revascularization. The management of patients in whom revascularization is not an option remains extremely complex and the evidence in this field is extremely limited. Percutaneous transcatheter therapies, reparative or replacement, are rapidly emerging as valid alternatives in cases of patients at high surgical risk. In particular, edge-to-edge repair (MitraClip) has proven effective in improving symptoms and reducing hospitalizations for HF. However, neither transcatheter nor surgical mitral repair or replacement has been shown to significantly improve prognosis, with mortality remaining high (14-20% at 1 year). Randomized trials aimed at assessing the effect of these treatments and establishing their long-term outcomes are urgently required.

Effect of expiratory phase for radiographic detection of left heart enlargement in dogs with mitral regurgitation.

Radiography is a standard diagnostic test for characterizing left heart enlargement in dogs however limited information is available on the effects of respiratory phases. This prospective and retrospective method comparison study investigated the respiratory effect on the size and shape of the left heart in dogs to determine the usefulness of expiratory radiographs to detect enlargements in the left atrium (LA) and left ventricle (LV). Thoracic radiographs taken at full inspiration and expiration were evaluated in 20 normal beagles and 100 dogs diagnosed with mitral regurgitation (MR). Vertebral heart score (VHS), vertebral left atrial size, elevation of the carina, and dorsal bulging of LA on lateral view and lateral bulging of the left auricular appendage and LV on ventrodorsal view were assessed. In normal dogs, there were no significant differences in the evaluative factors between inspiration and expiration. In dogs with MR, VHS did not change according to respiration. However, bulging of the LA, left auricular appendage, and LV had sharp margin during expiration compared with inspiration. The expiratory radiographic finding of LA bulging had a higher correlation with the LA to aorta ratio compared with LA bulging in the inspiratory radiography. Using a LA to aorta echocardiographic ratio greater than 1.5 as the gold standard, the radiographic sensitivity for LA enlargement was higher during expiration than inspiration. These findings of our study indicated that expiratory radiography can be helpful to support the detection of left heart enlargement, although it can overestimate LA enlargement in dogs with MR.

Clinical outcomes of percutaneous mitral valve repair with MitraClip for the management of functional mitral regurgitation.

OBJECTIVES: To evaluate the safety and efficacy of percutaneous mitral valve repair for the management of functional mitral insufficiency. BACKGROUND: Severe FMR is present in 25-30% of patients with heart failure and is an independent predictor of mortality and hospitalizations in patients with both ischemic and nonischemic cardiomyopathy. MitraClip therapy has been approved for high surgical risk patients with primary mitral regurgitation. Recent studies including two randomized trials have yielded conflicting results in terms of its clinical efficacy and outcomes for FMR. A quantitative evaluation and synthesis of this information are essential in elucidating the role of MitraClip repair for FMR. METHODS: We performed a literature search using PubMed, EMBASE, and Cochrane Central Register of Controlled Trials from September 2008 to September 2018. Studies comparing percutaneous mitral valve repair using the MitraClip device against conservative therapy for the management of functional mitral regurgitation were included. RESULTS: Seven studies with 1,174 patients in MitraClip group and 1,015 patients in medical therapy group met inclusion criteria. The 12-month mortality in the MitraClip group was 18.4% compared with 25.9% in the medical therapy group (odds ratio [OR]: 0.65 [0.50, 0.86]; p < .002). The rate of readmission at 12 months was 29.9% in the MitraClip group compared with 54.1% in the medical therapy group (OR: 040 [0.32-0.49]; p < .0001. The prognostic efficacy of MitraClip repair appears to be more substantial over longer follow-up period over medical therapy alone. CONCLUSIONS: Based on the results of this meta-analysis, percutaneous mitral valve repair with MitraClip appears to be superior to medical therapy for symptomatic moderate-to-severe functional mitral insufficiency. Further clinical research is needed to identify the ideal patient subgroups who receive maximum benefit with the MitraClip therapy.

Comparison of the newer proposed diagnostic score with the World Heart Federation criteria for echocardiographic detection of rheumatic heart disease.

INTRODUCTION: Echocardiography has been found to be a much better screening tool compared to clinical examination for the detection of rheumatic heart disease (RHD) in asymptomatic school children living in the RHD endemic areas. Recently, World Heart Federation (WHF) published echocardiographic criteria for the diagnosis of RHD. The present study was done to compare the performance of the newer proposed, quantitative diagnostic score against the qualitative WHF criteria in a field survey of asymptomatic school children belonging to the district having high prevalence of RHD. METHODS: 3000 asymptomatic school children studying in rural and urban schools of Bikaner district were screened both by clinical examination and echocardiography performed in parallel. The WHF criteria and the proposed diagnostic score were applied simultaneously for the diagnosis of RHD. RESULTS: A high prevalence of subclinical RHD was found. There was complete agreement between the two sets of criteria for the diagnosis of RHD. However, there was discrepancy in grading the severity of disease. The diagnostic score proved superior to the WHF criteria in grading the disease severity accurately. CONCLUSIONS: Diagnostic score captures the disease spectrum of RHD better than WHF criteria and reduces the subjectivity in the diagnosis of RHD.

[Surgical treatment of patients with obstructive hypertrophic cardiomyopathy]. / Khirurgicheskie i anesteziologicheskie aspekty lecheniia bol'nykh s gipertroficheskoi kardiomiopatiei.

OBJECTIVE: To analyze in-hospital results of surgical treatment of patients with obstructive hypertrophic cardiomyopathy. MATERIAL AND METHODS: There were 15 patients (8 women and 6 men) with obstructive hypertrophic cardiomyopathy and severe mitral insufficiency for the period from November 2017 to March 2019. Mean age of patients was 55.9±7.1 years. All patients had chronic heart failure NYHA class III and secondary coronary insufficiency. RESULTS: Surgical correction of LVOT obstruction and mitral valve repair with elimination of systolic anterior motion were technically successful in all cases. There was no need for mitral valve replacement in all patients. Heart failure NYHA class I-II in early postoperative period was observed in all cases. CONCLUSION: Careful preoperative planning with a comprehensive analysis of hemodynamic and anatomical features of LV and certain surgical experience are essential for adequate correction of LVOT obstruction without atrioventricular node injury and mitral valve repair.

The Effect of LVAD Pressure Sensitivity on the Assisted Circulation Under Consideration of a Mitral Insufficiency: An In Vitro Study.

Current left ventricular assist devices (LVADs) differ with respect to their pump characteristics as described by the pump characteristic curve (also called HQ-curve). Pressure sensitive LVADs depict a flat characteristic curve while most available LVADs have a steep, less pressure sensitive characteristic curve. This in vitro study investigated the effect of LVAD pressure sensitivity with a focus on the afterload of the right ventricle (RV) which is one out of many factors influencing right heart failure (RHF). To this end, two laboratory pumps differing in pressure sensitivity were tested as LVAD in an established, active mock circulation loop (MCL). The MCL represented patients with left heart failure and mitral insufficiency as another contributing factor to RV afterload. The results show that the pressure-volume loop (PV-loop) of the left ventricle (LV) undergoes a leftward and thus somewhat of a downward-shift for highly pressure sensitive support. Consequently, the LV is unloaded to a higher degree at comparable arterial blood pressure and identical cardiac output, pulmonary and systemic vascular resistance and ventricular contractility. This causes a concomitant decrease of RV afterload. This effect seems to be due to increased unloading during systole. In case of a severe concomitant mitral insufficiency and looking at left atrial pressure, the difference is 18.5%. Without mitral insufficiency, the difference is reduced to 10.2%.

Repair Techniques for Mitral Valve Insufficiency in Children.

Congenital mitral valve (MV) dysplasia is a relatively rare and highly complex cardiac disease. We sought to provide a comprehensive analysis of the current surgical techniques for treating mitral valve insufficiency and the results of mitral valve repair at our institution. Between 1972 and 2017, 104 consecutive patients underwent surgical repair of congenital MV dysplasia-insufficiency at our institution. Among these, 59 patients presented with MV insufficiency (or prevalent MV insufficiency) and were part of the study. There was 1 early (1.7%) and 1 late death (1.7%). Survival at 5, 10 and 20 years was 98%, 98% and 94%, respectively. Eight patients (14%) required MV replacement for residual significant MV insufficiency. Freedom from re-intervention for MV dysfunction at 5, 10 and 20 years were 89%, 84% and 79%, respectively. Surgical techniques for treating mitral valve insufficiency must be tailored for each patient with the main goal of achieving a proper valve function, rather than a "normal" anatomy. The mechanism underlying valve dysfunction in congenital mitral valve insufficiency is multifactorial and requires the application of a variety of surgical techniques in each case.

A late presentation of congenital cardiac anomaly: Accessory chordae from the left atrium causing severe mitral regurgitation.

Mitral regurgitation secondary to accessory mitral valve (MV) chordae of the left atrium is an extremely rare congenital disease. A 85-year-old female (NYHA I-II) was hospitalized for investigations. An echocardiogram showed calcification of the MV with mild stenosis and moderate regurgitation. Transesophageal three-dimensional echocardiogram revealed a band-like structure extending from the distal third of the anterior wall of the left atrium to the MV. This accessory chordae determined severe systolic regurgitation and mild mitral stenosis. The patient was referred for consideration of cardiac surgery but was refused for comorbidities and anatomy. Usually aberrant chordae determinant valvulopathies are detected and treated at a much younger age. The delay of the symptoms could be explained in our case with the progressive growth and dilatation of the left atrium causing traction of the aberrant chord resulting in an increase in the leaflet prolapse and regurgitation.

[Phenomenological Regularities of Assessment of Left Ventricle Function in Mitral Valve Insufficiency].

OBJECTIVE: to examine relationship between anatomical changes of the left ventricle (LV), dynamics of velocity of its volume modification, and blood flows in the LV in patients with mitral regurgitation (MR) before and after surgical treatment. MATERIALS AND METHODS: We included into this study 58 patients with severe 3-4 degree MR (38 men, 20 women aged 24-69 [mean age 51±9] years) in sinus rhythm (96 %) or atrial fibrillation (4 %). The control group included 86 healthy volunteers, mean age 39±7 years. Transthoracic echocardiographic studies were performed in both groups by standard technique at rest using a high-quality echocardiograph Vivid E9, equipped with a 3.5-4.6 MHz multi frequency transducer (in patients before and after surgical repair - mitral valve [MV] replacement and MV reconstruction with annuloplasty ring). The analysis of files recorded was performed off-line by vector analysis technique including estimation of myocardial deformation velocities and dynamics of LV volume modification, construction of "flow-volume" diagram, calculation of the expended kinetic energy, and registration of intraventricular blood flows. RESULTS: End diastolic volume (EDV), end systolic volume (ESV) and total stroke volume (TSV) (effective + retrograde) were significantly increased in patients with severe LV volume overload before surgery in comparison with the control group (p.

The association between systemic lupus erythematosus and valvular heart disease: an extensive data analysis.

BACKGROUND: Association between antiphospholipid syndrome in systemic lupus erythematosus (SLE) and valvular heart disease (VHD) is well reported, but relatively few studies have been carried out to establish the linkage between VHD and SLE itself. We aimed to investigate link between VHD and SLE and to evaluate the association of diverse factors with VHD among these patients in a large-scale population-based study. MATERIALS AND METHODS: We used the databases of the largest state-mandated health service organization in Israel. All SLE patients were included (n = 5018) as well as their age and sex-matched controls (n = 25 090), creating a cross-sectional population-based study. Medical records of all subjects were analysed for documented VHD and the presence of antiphospholipid antibodies (aPLs). A logistic regression model was carried out to evaluate the diverse factors including SLE and aPLs as independent risk factors for VHD. RESULTS: Valvular heart disease were found to be more frequent among SLE group when compared to controls (aortic stenosis, 1·08% vs. 0·35% respectively, P < 0·001; aortic insufficiency, 1·32% vs. 0·29% respectively, P < 0·001; mitral stenosis, 0·74% vs. 0·21% respectively, P < 0·001; mitral insufficiency, 1·91% vs. 0·39% respectively, P < 0·001). Male sex, hypertension, aPLs and SLE were found to be significant independent risk factors for VHD. CONCLUSION: All VHD are more prevalent among SLE patients when compared to controls. SLE and aPLs are independent risk factor for VHD (OR of 2·46 and 1·7, respectively). Physicians must be aware of such significant association, and routine echocardiography should be considered in SLE patients regardless of their aPL status.

Mitral Regurgitation After Orthotopic Lung Transplantation: Natural History and Impact on Outcomes.

OBJECTIVE: Progression of mitral regurgitation (MR) after orthotopic lung transplantation (OLT) may be an underrecognized phenomenon due to the overlapping symptomatology of pulmonary and valvular disease. Literature evaluating the progression of MR after OLT currently is limited to case reports. Therefore, the hypothesis that MR progresses after OLT was tested and the association of preprocedure MR with postoperative mortality was assessed. DESIGN: A retrospective cohort. SETTING: A tertiary-care hospital. PARTICIPANTS: Patients who underwent OLT between January 1, 2003 and February 4, 2012. INTERVENTIONS: After receiving institutional review board approval, a preprocedure transesophageal echocardiogram was compared with a postoperative transthoracic echocardiogram (TTE) to determine the progression of MR. Univariate and multivariate association between preprocedure MR grade and 1- and 5-year mortality was assessed. A p value of<0.05 was considered statistically significant. MEASUREMENTS AND MAIN RESULTS: From 715 patients who underwent OLT, 352 had a postoperative TTE and were included in the evaluation of progression of MR. Five patients had progression of MR postoperatively, and the mean change in MR score of -0.04 was found to be nonsignificant (p = 0.25). Mortality data were available for 634 of the 715 patients. After covariate adjustment, there was no significant association between MR grade and 1-year mortality (p = 0.20) or 5-year mortality (p = 0.46). CONCLUSIONS: This study rejected the hypothesis that primary and secondary MR progresses after OLT and found that preprocedure MR was not associated with increased postoperative mortality. Despite the findings that MR does not progress in all patients, there is a subset of patients for whom MR progression is clinically significant.