Acute inferior myocardial infarction combined with papillary muscle rupture: A case report / 中南大学学报(医学版)

The incidence of acute myocardial infarction (AMI) is increasing. Acute papillary muscle rupture is one of the serious and rare mechanical complications of AMI, which occurs mostly in inferior and posterior myocardial infarction. A patient with acute inferior myocardial infarction developed pulmonary edema and refractory shock, followed by cardiac arrest. After cardiopulmonary resuscitation (CPR), revascularization of criminal vessels was carried out by emergency percutaneous transluminal coronary angioplasty (PTCA) under the support of intra-aortic balloon pump (IABP) and extra corporeal membrane oxygenation (ECMO). Although the patient was given a chance for surgery, his family gave up treatment due to unsuccessful brain resuscitation. It reminds that mechanical complications such as acute papillary muscle rupture, valvular dysfunction and rupture of the heart should be highly suspected when cardiogenic pulmonary edema and cardiogenic shock are difficult to correct in acute inferior myocardial infarction. Echocardiogram and surgery should be put forward when revascularization of criminal vessels is available.

Cardiac anomalies in pediatric patients with pectus excavatum

SUMMARY OBJECTIVE: Pectus excavatum is the most prevalently encountered deformity of the thoracic wall. It can be accompanied by congenital anomalies. METHODS: The cardiac findings of 36 children who were diagnosed at the Thoracic surgery outpatient clinic of our university between 10 February 2021 and 1 October 2021 and 57 healthy children in a similar age group were analyzed. RESULTS: We determined that the pectus excavatum patients in our study had a higher risk of having mitral insufficiency, mitral valve prolapse, tricuspid valve prolapse, cardiac malposition, and congenital heart disease. CONCLUSION: Our study showed that the prevalence of cardiac pathologies was higher in pediatric pectus excavatum patients than in healthy children in the control group. Thus, we recommend clinicians to refer pediatric pectus excavatum patients to pediatric cardiology outpatient clinics for the early diagnosis of potential cardiac pathologies.

Presentación inusual del síndrome de Lutembacher / Unusual presentation of Lutembacher’s syndrome

Resumen Introducción: El síndrome de Lutembacher corresponde a la asociación de un defecto interauricular (congénito o iatrogénico) e insuficiencia o estenosis mitral (congénita o adquirida). La etiología reumática es la causa más frecuente del compromiso mitral. Tiene una prevalencia reportada de 0.001 por cada 1.000.000 habitantes. Caso clínico: Paciente femenina de 6 años con cuadro de 10 meses de palpitaciones asociadas a dolor torácico. Se realizó un electrogardiograma en el que se evidenció bloqueo incompleto de la rama derecha del haz de His y prolongación del intervalo PR, además de un ecocardiograma en el que se visualizó una comunicación interauricular tipo ostium secundum no restrictiva de 28 mm, con cortocircuito de izquierda a derecha, dilatación del ventrículo derecho, prolapso de válvula mitral, valvas engrosadas e insuficiencia mitral moderada a grave. Se realizó plastia de válvula mitral y cierre quirúrgico de la comunicación interauricular, sin complicaciones. Durante el seguimiento se encuentra asintomática desde el punto de vista cardiovascular, en manejo farmacológico. Conclusiones: La asociación Lutembacher tiene una prevalencia de 0.001/1.000.000 habitantes; la mayoría tiene etiología reumática. La paciente no tiene historia de fiebre reumática y sería la paciente más joven reportada en la literatura con síndrome de Lutembacher.

Abstract Introduction: Lutembacher’s syndrome corresponds to the association of an atrial septal defect (congenital or iatrogenic) and mitral regurgitation or stenosis (congenital or acquired), with rheumatic etiology being the most-frequent cause of mitral regurgitation. It has a reported prevalence of 0.001 for every 1,000,000 inhabitants. Clinical case: Female patient six years of age with a 10-month condition of palpitations associated with chest pain. An electrocardiogram was performed with evidence of incomplete right His bundle branch block and PR interval prolongation; additionally, an echocardiogram showed 28-mm non-restrictive ostium secundum atrial septal defect, with left-to-right shunt, right ventricular dilation, mitral valve prolapse, thickened valves, and moderate-to-severe mitral regurgitation. Mitral valve plasty and surgical closure of the atrial septal defect were performed, without complications. During follow-up, she was asymptomatic from the cardiovascular point of view, under pharmacological management. Conclusions: Lutembacher’s association has a prevalence of 0.001/1´000.000 inhabitants; the majority with rheumatic etiology. Our patient has no history of rheumatic fever and would be the youngest patient reported in the literature with Lutembacher’s syndrome.

Disjunção do anel mitral: modalidades diagnósticas, implicações clínicas e evolução prognóstica / Mitral annulus disjunction: diagnostic modalities, clinical implications, and prognostic progression

A disjunção do anel mitral (DAM) é uma inserção anormal da linha de flexão do anel mitral na parede atrial. O anel mostra uma separação (disjunção) entre a junção folheto posterior-parede atrial e a crista miocárdica ventricular esquerda.1 A DAM foi descrita pela primeira vez há mais de 30 anos em estudo de autópsia, estando relacionada com prolapso da valva mitral (PVM) em 92% dos casos.2 Desde então, foram realizados diversos estudos, sendo a prevalência de DAM em pacientes com PVM reportada de forma variável, podendo ou não estar associada à insuficiência mitral. O ecocardiograma transtorácico (ETT) faz parte da avaliação inicial do prolapso valvar mitral, permitindo o diagnóstico e a avaliação de complicações relacionadas. Com a evolução de novos métodos diagnósticos, a ressonância magnética cardíaca (RMC) e o ecocardiograma transesofágico (ETE) passaram a aprimorar a avaliação dessa patologia, bem como de sua extensão e localização. Contudo, as características fenotípicas do PVM que estão mais associadas a DAM permanecem incertas, sobretudo devido ao número limitado de pacientes, nos estudos clássicos sobre o tema. Portadores de DAM podem desenvolver sintomas relacionados a arritmias ventriculares, configurando a síndrome arrítmica da DAM (SDAM), podendo evoluir para morte súbita. Na literatura, os dados prognósticos ainda são conflitantes entre os diversos estudos acerca do tema, indo desde critérios claros de diagnóstico, o melhor método de imagem a ser aplicado, o tratamento e o prognóstico. Esta revisão descreve as características da DAM associada ou não ao prolapso valvar, auxiliando no diagnóstico e na conduta dessa importante patologia. (AU)

Mitral annulus disjunction (MAD) is an abnormal insertion of the flexion line of the mitral annulus into the atrial wall. The annulus presents a separation (disjunction) between the posterior leaflet­atrial wall junction and the left ventricular myocardial crest.1 MAD was first described more than 30 years ago in an autopsy study and is reportedly related to mitral valve prolapse (MVP) in 92% of cases.2 Since then, several studies have been conducted, and reports on the prevalence of MAD in patients with MVP have varied. Ultimately, it may or may not be associated with mitral regurgitation. Transthoracic echocardiography is part of initial MVP assessment, allowing its diagnosis and the assessment of related complications. As new diagnostic methods emerged, cardiac magnetic resonance imaging and transesophageal echocardiography improved the assessment of this pathology in terms of its diagnosis, extension, and location. However, the phenotypic characteristics of MVP that are more closely associated with MAD remain uncertain mainly due to the limited number of patients in classic studies on the subject. Patients with MAD may develop symptoms related to ventricular arrhythmias, configuring the MAD arrhythmic syndrome, which may progress to sudden death. The literature presents conflicting prognostic data among several studies on the subject from clear diagnostic criteria and best imaging method to be used to treatment and prognosis. This review describes MAD characteristics associated (or not) with valve prolapse to improve the diagnosis and management of this important pathology. (AU)

Síndrome de Peutz-Jeghers, telangiectasias y prolapso de la válvula mitral. Presentación de caso y asesoramiento genético / Peutz-Jeghers syndrome, telangiectasias, and mitral valve prolapse. Case presentation and genetic counseling

Introducción: El síndrome de Peutz-Jeghers se caracteriza por hiperpigmentación mucocutánea y hamartomas gastrointestinales que pueden aparecer desde el estómago hasta el ano. Tiene un patrón de herencia autosómico dominante y expresividad variable. El diagnóstico se basa en los hallazgos clínicos y la apariencia histológica de los pólipos. No ha sido reportado hasta ahora asociación de esta entidad a telangiectasias y prolapso de la válvula mitral. Objetivo: Describir los hallazgos que permitieron establecer el diagnóstico de Síndrome de Peutz-Jeghers en un paciente y brindar asesoramiento genético. Presentación del caso: Paciente masculino de 36 años de edad con antecedentes de prolapso de la válvula mitral que acude a consulta de genética clínica con su esposa para solicitar asesoramiento genético, debido a que tienen una hija con diagnóstico de Síndrome de Peutz-Jeghers y desean conocer el riesgo de tener otro hijo afectado. Al examen físico se observa mácula hiperpigmentada en labio inferior y varias de estas en encías. Con tales hallazgos y el antecedente de tener la hija Síndrome de Peutz-Jeghers se emite el mismo diagnóstico en el padre. Como dato de interés se constatan en este individuo múltiples telangiectasias en tórax, cuello y espalda. Los estudios realizados en busca de la causa de estas fueron negativos. Conclusiones: Los antecedentes y los hallazgos encontrados en el paciente permitieron realizar el diagnóstico de Peutz-Jeghers y brindar asesoramiento genético. Se presenta el primer reporte de esta enfermedad asociada a telangiectasias y prolapso de la válvula mitral en la literatura científica(AU)

Introduction: Peutz-Jeghers syndrome is characterized by mucocutaneous hyperpigmentation and gastrointestinal hamartomas that can appear from the stomach to the anus. It has an autosomal dominant inheritance pattern and variable expressiveness. The diagnosis is based on clinical findings and histological appearance of the polyps. No association between this entity and telangiectasias and mitral valve prolapse has been reported so far. Objective: To describe the findings that made it possible to establish the diagnosis of Peutz-Jeghers syndrome in a patient and to provide genetic counseling. Case presentation: Thirty-six-year-old male patient with a history of mitral valve prolapse who attends a clinical genetics consultation with his wife to request genetic counseling due to the fact that their daughter was diagnosed with Peutz-Jeghers Syndrome and they want to know about the risk of having another affected child. On physical examination, a hyperpigmented macule on the lower lip and several of these on the gums were observed. With such findings and the antecedent of having a daughter with Peutz-Jeghers syndrome, the same diagnosis is made in the father. As data of interest, multiple telangiectasias on the thorax, neck and back were found in this individual. The studies carried out to identify the same cause were negative. Conclusions: The history and findings in this patient allowed us to make the diagnosis of Peutz-Jeghers syndrome as well as to provide genetic counselling. The first report of this disease associated with telangiectasias and mitral valve prolapse is presented in the scientific literature(AU)

Endocarditis infecciosa con hemocultivos negativos en prolapso de la válvula mitral. Presentación de caso / Infective endocarditis with negative blood cultures in mitral valve prolapse. Case presentation

Introducción: La Endocarditis infecciosa sigue desafiando a la Medicina moderna a pesar de no ser una entidad frecuente. Objetivo: Se presenta un caso con una lesión valvular previa no diagnosticada antes, y sin síntomas, y que se consideró el diagnóstico tempranamente de endocarditis en el nivel hospitalario. Presentación del caso: Paciente de 20 años, mujer, con antecedentes de salud referidos, fumadora. Ingresa en sala del Servicio de Medicina el 21 de enero de 2020 por fiebres que se mantienen todo el día de 38-38,50 C, con picos que alcanzan los 400 C con escalofríos en determinados momentos. Desde hace un mes presenta esta sintomatología. Ruidos cardiacos rítmicos, taquicárdicos, de buena intensidad. Clic sistólico con arrastre sistólico fuerte de regurgitación IV/VI audible en foco mitral con frémito que se irradia a la axila, anemia, VSG acelerada, leucocitosis con desviación izquierda, hemocultivos negativos y en ecocardiograma prolapso de válvula mitral, valva anterior y posterior, con regurgitación que ocupa toda la aurícula izquierda hasta el techo de la misma. Múltiples vegetaciones en cara auricular de valva posterior de válvula mitral, la mayor de 7 x 3 mm. Conclusiones: El método clínico es fundamental en el proceso diagnóstico en la práctica clínica secundado por los medios diagnósticos como en la enfermedad que nos ocupa(AU)

Introduction: Infective endocarditis continues to be a great challenge for modern medicine although it is not a frequent entity. Objective: We present a case of an undiagnosed previous valve lesion without symptoms. The early diagnosis of endocarditis was made at the hospital level. Case Presentation: A 20-year-old woman, smoker, with previous history of good health was admitted to the medical ward on January 21, 2020. The patient reported continuous fever (38-38,50 C) throughout the day, with spikes up to 400 C and intermittent chills. She has been having these symptoms for a month. Rhythmic heart sounds and high intensity tachycardia and systolic click with strong systolic displacement of regurgitation grade IV/VI audible in mitral area with fremitus radiating to the armpit were heard. Anemia, accelerated ESR, leukocytosis with left deviation, and negative blood cultures were confirmed. The echocardiogram showed a mitral valve prolapse with regurgitation of anterior and posterior valves that occupies all the left atrium until its top. There was multiple vegetation in the atrial side of the posterior leaflet of the mitral valve; the greatest is 7 x 3 mm. Conclusions: The clinical method is essential in the diagnostic process performed in clinical practice supported by diagnostic means, as in the current case(AU)

Research progress on effects of different valvuloplasty techniques on posterior mitral valve prolapse / 中国胸心血管外科临床杂志

@#Mitral valvuloplasty is a more suitable surgical procedure than mitral valve replacement in the case of mitral valve degeneration. Quadrangular resection and artificial chordae plantation, considered to be classical procedures, are widely employed in posterior mitral valve prolapse, and have prominent long-term effects during the follow-up. However, is there any difference in mitral valve reconstruction due to completely different surgical methodology and concepts of the two procedures? Every surgeon has his own ideas and preferences for mitral valvuloplasty, and the choice of surgical procedures mostly depends on experience of surgeons. The article generally reviews variances in intraoperative and long-term clinical outcomes of both rectangular excision and artificial chordae plantation in posterior leaflet valvuloplasty, hoping to provide references for clinical decision.

Genome-wide association study of degenerative mitral valve disease in Maltese dogs

Genome-wide association study (GWAS) is a powerful tool for identifying the genetic causes of various diseases. This study was conducted to identify genomic variation in Maltese dog genomes associated with degenerative mitral valve disease (DMVD) development and to evaluate the association of each biological condition with DMVD in Maltese dogs. DNA was extracted from blood samples obtained from 48 Maltese dogs (32 with DMVD and 16 controls). Genome-wide single nucleotide polymorphism (SNP) genotyping was performed. The top 30 SNPs from each association of various conditions and genetic variations were mapped to their gene locations. A total of 173,662 loci were successfully genotyped, with an overall genotype completion rate of 99.41%. Quality control analysis excluded 46,610 of these SNPs. Manhattan plots were produced using allelic tests with various candidate clinical conditions. A significant peak of association was observed between mitral valve prolapse (MVP) and SNPs on chromosome 17. The present study revealed significant SNPs in several genes associated with cardiac function, including PDZ2, Armadillo repeat protein detected in velo-cardio-facial syndrome, catenin (cadherin-associated protein) alpha 3, low-density lipoprotein receptor class A domain containing protein 4, and sterile alpha motif domain containing protein 3. To our knowledge, this is the first study of a genetic predisposition to DMVD in Maltese dogs. Although only a limited number of cases were analyzed, these data could be the basis for further research on the genetic predisposition to MVP and DMVD in Maltese dogs.

Utility of Cardiac CT for Preoperative Evaluation of Mitral Regurgitation: Morphological Evaluation of Mitral Valve and Prediction of Valve Replacement

OBJECTIVE: We aimed to investigate the diagnostic accuracy of cardiac computed tomography (CT) for the detection of mitral valve (MV) prolapse in mitral regurgitation (MR) with surgical findings as the standard reference, and to assess the predictability of MV replacement based on morphologic CT findings. MATERIALS AND METHODS: A total of 156 patients who had undergone preoperative cardiac CT and subsequently received MV surgery due to severe MR were retrospectively enrolled. Non-repairable MV was defined when at least one of the following conditions was met: 1) anterior leaflet prolapse, 2) bi-leaflet prolapse, or 3) valve morphology (leaflet thickening, calcification, or mitral annular calcification [MAC]). Diagnostic performance of CT for the detection of the prolapsed segment was assessed with surgical findings as the standard reference. Logistic regression analysis was performed to evaluate the value of CT findings to predict actual valve replacement. RESULTS: During surgery, MV prolapse was identified in 72.1%. The sensitivity, specificity, and diagnostic accuracy for the detection of MV prolapse were 99.1%, 81.4%, and 94.2%, respectively, per patient. One-hundred eighteen patients (75.6%) underwent MV repair and the remaining 38 patients received MV replacement. Bi-leaflet prolapse and valve morphology were independent predictors of valve replacement after adjusting for clinical variables (adjusted odds ratio, [OR] 8.63 for bi-leaflet prolapse; OR, 4.14 for leaflet thickening; and OR, 5.37 for leaflet calcium score > 5.6; p < 0.05). CONCLUSION: Cardiac CT can have high diagnostic performance for detecting the prolapsed segment of the MV and predictability of valve replacement before surgery. Bi-leaflet prolapse and valve morphology, such as leaflet thickening, or calcification or MAC, are the most important predictors of valve replacement.

Preliminary study on the application of mitral valve prolapse model made by three‐dimensional printing in mitral valvuloplasty / 中华超声影像学杂志

Objective To evaluate the feasibility and accuracy of mitral valve prolapse( M VP) model made by three‐dimensional( 3D) printing based on three‐dimensional transesophageal echocardiography ( 3D‐T EE) data and the application value for mitral valvuloplasty . Methods 3D‐T EE volumetric data of 28 patients with M VP were acquired and postprocessed ,13 patients underwent mitral valve replacement and 15 patients underwent mitral valvuloplasty . A flexible material was used to made the valve 3D model by molding . T he areas of M VP identified by models were compared with surgical findings ,the circumference and the length and thickness of anterior and posterior mitral leaflets obtained from the valve specimens and the models were compared in the mitral valve replacement group . T he diameter between anterior and posterior ,the diameter between anterolaterior and posteromedial ,annulus area ,height of prolapsed leaflet and area of prolapsed leaflet were measured from 3D models and 3D‐T EE images in mitral valvuloplasty group . Surgical simulations were performed on the 3D models of the mitral valvuloplasty group ,and the water injection test was used to evaluate the surgical results and compared with the surgical results . Results 3D‐T EE volumetric data were successfully postprocessed and made as 3D M VP models in all patients . T he consistency of M VP location based on 3D models and surgical findings was 0 .92 . T he differences between the mitral valve replacement group and mitral valvuloplasty group were not significant ( P＞ 0 .05 ) . A simulation valvuloplasty was successfully performed on the 3D model in mitral valvuloplasty group ,2 patients underwent mitral valve replacement after water injection test . T he remaining 3D models successfully simulated the operation . Conclusions The M VP model made by 3D‐T EE and 3D printing technique has high feasibility and accuracy ,w hich may be promising for the mitral valvuloplasty of M VP .

Changes of left ventricular structure and function after minimally invasive repair of mitral valve prolapse / 中国胸心血管外科临床杂志

@#Objective    To evaluate the changes of left ventricular structure and function by echocardiography and its grading of left ventricular diastolic function in patients with mitral valve prolapse treated by minimally invasive mitral valve repair. Methods    By retrospective analysis, 37 patients including 25 males and 12 females aged 53.49±11.02 years with mitral valve prolapse who underwent minimally invasive mitral valve repair were as an operation group, and 34 healthy persons including 19 males and 15 females aged 54.26±8.33 years matched by age and sex were selected as a control group. Ultrasound parameters of every participant were routinely collected before operation, 1 month, 3 months, 6 months and 1 year after operation, and left ventricular diastolic function was graded. The ultrasound parameters between the two groups were compared. Results    The diameters of left ventricular end systolic and diastolic phase, left atrial diameter and left ventricular volume in the operation group were significantly smaller than those before operation. The diameters of left ventricle and left atrium after operation were significantly shorter than those before operation, but they were still larger than those of the control group. The ejection fraction value decreased significantly at one month after the operation and then returned to normal level. The incidence of left ventricular diastolic dysfunction at 6 months and 1 year after operation was significantly lower than that before operation (P<0.05). Conclusion    Minimally invasive repair for patients with mitral valve prolapse can significantly improve systolic and diastolic functions of left ventricle while reconstructing left atrial and left ventricular structures.

Clinical effect of loop-in-loop technique and annuloplasty ring for the treatment of mitral valve prolapse under total thoracoscopy / 中国胸心血管外科临床杂志

@#Objective    To analyze the effect of loop-in-loop technique and annuloplasty ring for the treatment of mitral valve prolapse (MVP) under total thoracoscopy. Methods    Between May 2012 and May 2017, 21 patients with MVP underwent mitral valve repair in our hospital. There were 12 males and 9 females with a mean age of 50.90±9.66 years and the mean weight of 64.81±11.56 kg. Loop-in-loop artificial chordae tendonae reconstruction and mitral annuloplasty were performed through the right atrial-atrial septal incision under total thoracoscopy. The water test and transesophageal echocardiography were performed during the operation to evaluate the effect of mitral annuloplasty. Data of echocardiography and chest radiography were collected postoperatively one week, before discharge and after discharge. Results    All the operations were successful without re-valvupoplasty or valve replacement, conversion to median thoracotomy, malignant arrhythmia, perioperative death or wound infection. Among them, 10 patients underwent tricuspid valvuloplasty, 1 patient underwent tricuspid valvuloplasty plus radiofrequency ablation simultaneously. The mean cardiopulmonary bypass time was 255.57±37.24 minutes, aortic occlusion time was 162.24±19.61 minutes, the number of loop was 2–5 (3.29±0.78), the size of ring was 28–34 (31.11±1.88) mm, ventilator assistance time was 19.43±14.68 hours, ICU time was 58.45±24.60 hours and postoperative hospital stay was 12.28±3.61 days. Transthoracic echocardiography was re-examined postoperatively. Mild-mitral regurgitation was found in 3 patients. Warfarin anticoagulant therapy was given orally for 6 months postoperatively. The patients were followed up regularly for 2–51 months at 1, 3, 6 and 12 months postoperatively. Left ventricular end-diastolic diameter (LVEDD) was 45.06±2.96 mm, left ventricular end-diastolic volume 108.11±17.09 mL, left atrial diameter (LAD) 35.56±6.93 mm and cardiothoracic ratio 0.53±0.13 at discharge which were significantly smaller than those at admission (P<0.05). Pulmonary artery pressure was 19.22±6.38 mm Hg which was significantly lower than that at admission (P<0.05), but left ventricular ejection fraction (62.33%±4.00%) had no significant change (P>0.05). The LAD and LVEDD were significantly smaller than those before operation, and the cardiac function improved to some extent during the follow-up. No new mitral valve prolapse, increased regurgitation, infective endocarditis, thromboembolism or anticoagulation-related complications were found during the follow-up. Conclusion    Loop-in-loop artificial chordae tendon implantation combined with mitral annuloplasty is a safe and effective method for MVP under total thoracoscopy with minimal trauma, satisfactory cosmetic effect, and good early- and medium-term results. It is worth of popularizing. However, the operation time needs to be further shortened, and its long-term clinical effect needs further follow-up and other researches to confirm.

Marfan Syndrome: Clinical Presentation with Congenital Glaucoma? / Síndrome de Marfan: ¿presentación clínica con glaucoma congénito?

ABSTRACT Congenital glaucoma (CG), diagnosed in the first year of life, can fall into three main groups: primary CG, glaucoma associated with congenital anomalies, and secondary glaucoma of infants. The associated congenital anomaly of CG includes the autosomal dominant: Marfan Syndrome (MS), the phenotypic features of which would rarely be evident in the first year of life. Multiple other associated autosomal dominant, autosomal recessive, X-linked and chromosomal conditions that can present with CG need to be excluded. Hence, this is a retrospective diagnosis and is the first known documented case report in an Afro-Caribbean with MS presenting with CG at six weeks of age. The Marfanoid features became apparent in late childhood and adolescence.

RESUMEN El glaucoma congénito (GC), diagnosticado en el primer año de vida, puede clasificarse en tres grupos principales: GC primario, glaucoma asociado con anomalías congénitas, y glaucoma secundario de lactantes. La anomalía congénita asociada con el GC incluye el trastorno autosómico dominante conocido como síndrome de Marfan (SM), cuyas características fenotípicas serían raramente evidentes en el primer año de vida. Otras múltiples condiciones asociadas - autosómicas dominantes, autosómicas recesivas, ligadas al cromosoma X, y cromosómicas - que pueden presentarse con el GC, necesitan ser excluidas. Por lo tanto, éste es un diagnóstico retrospectivo, a la par que el primer reporte de caso documentado de que se tenga noticia en relación un bebé afrocaribeño con SM, que presentaba GC a las seis semanas de la edad. Las características marfanoides se hicieron evidentes en la última etapa de la niñez y en la adolescencia.

Long-term results of mitral valve repair

Abstract Introduction: Current guidelines state that patients with severe mitral regurgitation should be treated in reference centers with a high reparability rate, low mortality rate, and durable results. Objective: To analyze our global experience with the treatment of organic mitral regurgitation from various etiologies operated in a single center. Methods: We evaluated all surgically treated patients with organic mitral regurgitation from 2004-2017. Patients were evaluated clinically and by echocardiography every year. We determined early and late survival rates, valve related events and freedom from recurrent mitral regurgitation and tricuspid regurgitation. Valve failure was defined as any mitral regurgitation ≥ moderate degree or the need for reoperation for any reason. Results: Out of 133 patients with organic mitral regurgitation, 125 (93.9%) were submitted to valve repair. Mean age was 57±15 years and 52 patients were males. The most common etiologies were degenerative disease (73 patients) and rheumatic disease (34 patients). Early mortality was 2.4% and late survival was 84.3% at 10 years, which are similar to the age- and gender-matched general population. Only two patients developed severe mitral regurgitation, and both were reoperated (95.6% at 10 years). Freedom from mitral valve failure was 84.5% at 10 years, with no difference between degenerative and rheumatic valves. Overall, late ≥ moderate tricuspid regurgitation was present in 34% of the patients, being more common in the rheumatic ones. The use of tricuspid annuloplasty abolished this complication. Conclusion: We have demonstrated that mitral regurgitation due to organic mitral valve disease from various etiologies can be surgically treated with a high repair rate, low early mortality and long-term survival that are comparable to the matched general population. Concomitant treatment of atrial fibrillation and tricuspid valve may be important adjuncts to optimize long-term results.

Advances in surgical diagnosis and treatment of mitral valve prolapse in children / 中华实用儿科临床杂志

Mitral valve prolapse is a disease which causes mitral regurgitation,which is one of the common causes of mitral insufficiency.With the aggravation of blood reflux,mitral valve prolapse will eventually lead to pulmo-nary hypertension,heart failure and even death in children.The main diagnostic methods include chest X－ray,echocar-diography,spiral CT and magnetic resonance imaging.Mitral valvuloplasty is the main surgical method for mitral valve prolapse in children.It includes valvuloplasty,annuloplasty,tendon chordoplasty,edge－to－edge mitral valve repair, and so on.Now,the progress in surgical diagnosis and treatment of mitral valve prolapse in children were reviewed in or-der to provide a meaningful reference for its individual treatment.

Cardiovascular abnormalities in patients with oral cleft: a clinical-electrocardiographic-echocardiographic study

OBJECTIVES: The present study aims to describe the clinical, electrocardiographic, and echocardiographic cardiological findings in a group of patients with oral clefts. METHODS: This is a prospective cross-sectional study on 70 children (age range from 13 days to 19 years) with oral clefts who attended the multidisciplinary program of a university hospital from March 2013 to September 2014. The patients were evaluated by a pediatric cardiologist and underwent detailed anamnesis, physical examination, electrocardiogram, and echocardiogram. RESULTS: Sixty percent of the patients were male; 55.7% presented with cleft lip and palate, and 40.0% presented with health complaints. Comorbidities were found in 44.3%. Relevant pregnancy, neonatal, family and personal antecedents were present in 55.7%, 27.1%, 67.2%, and 24.3% of the patients, respectively. Regarding the antecedents, 15.2% of the patients presented with a cardiac murmur, 49.0% with a familial risk of developing plurimetabolic syndrome, and 6% with family antecedents of rheumatic fever. Electrocardiographic evaluation showed one case of atrioventricular block. Echocardiograms were abnormal in 35.7% of the exams, including 5 cases of mitral valve prolapse — one of which was diagnosed with rheumatic heart disease. CONCLUSION: The finding of a family risk of developing plurimetabolic syndrome and a diagnosis of rheumatic heart disease indicates that patients with oral clefts may be more prone to developing acquired heart disease. Thus, our findings highlight the importance of anamnesis and methodological triangulation (clinical-electrocardiographic-echocardiographic) in the investigation of patients with oral clefts and emphasize that cardiological follow-up to evaluate acquired and/or rhythm heart diseases is necessary. This strategy permits comorbidity prevention and individualized planned treatment.

Long term outcomes of posterior leaflet triangular resectionvalvuloplasty formitral valve regurgitation / 中华胸心血管外科杂志

Objective To explorethe long-term prognosis of the triangular resectionvalvuloplasty for posterior mitral valve prolapse and provide a reference for the clinical treatment.Methods We retrospectively analyzed the clinical data of 61 pa-tients with posterior mitral valve prolapse underwent the mitral valvuloplasty in our hospital from Match 2008 to December 2016.30 patients who underwent triangular resection for posterior mitral valve prolapse.31 patients who underwentstandard quadrangular resection.The cardiac functional parameters and condition of mitral regurgitation were compared among the period of preoperation,discharging and follow-up.Results The mean follow-up period was 42 ±34(3-106)months.left atrium and left ventricular diameter were decreased(P<0.05).Inresrarch group,there was 1 patient who have moderate regurgitation,ar-rhythmia is one.In control group,there was arrhythmia in 1 cases,moderate regurgitation in 2 cases and death in 1 cases.Rate of freedom from reregurgitation was(97 ±6)% in research group and was(97 ±4)% in control group.There was no SAM in two groups.There were no significant differences of survival or risk of reoperation.There was a statistic difference of NYHA classification of cardiac function in the phase of follow-up than that of discharging(P<0.05).Conclusion Triangular resec-tion of a prolapsed posterior mitral leaflet is a simplified,reliable, reproducible, and durable procedure,like quadrangular re-section of a prolapsed posterior leaflet.

Analysis on clinical characteristics and prognosis of patients with mitral valve prolapse / 中华心血管病杂志

Objective@#To evaluate the clinical characteristics and prognosis of patients with mitral valve prolapse (MVP).@*Methods@#We retrospectively analyzed the clinical characteristics and prognosis of 148 MVP patients who underwent mitral valve surgery in Fuwai hospital from January 2012 to December 2015.The patients were divided into mucoid degeneration group (52 cases) and without mucoid degeneration group(19 cases) according to pathological examination of leaflets and chordate.@*Results@#The clinical symptoms of MVP patients included dyspnea (59.5%(88/148)), chest distress and pain (52.7%(78/148)), and palpitations (36.5% (54/148)). Mitral valve repair was performed in 144 cases (97.3%), and mitral valve replacement was performed in 4 cases (2.7%). Posterior leaflet prolapse was the most common form of MVP (68.9%, 102/148). Pathological examination revealed myxomatous degeneration in 73.2% patients (52/71), fibrosis in 8.5% patients (6/71), and fibrinoid necrosis in 8.5% patients (6/71). Patients with mucoid degeneration had less atrial fibrillation before surgery (5.8%(3/52) vs. 42.1%(8/19), P<0.01), smaller preoperative left atrium diameter ((43.2±6.5) mm vs. (48.2±8.9) mm, P<0.05), more posterior leaflet prolapse (94.2%(49/52) vs. 63.2%(12/19), P<0.01), redundant chordae (26.9%(14/52) vs. 0, P<0.05) and leaflet thickening (76.9%(40/52) vs. 52.6%(10/19), P<0.05) when compared with patients without mucoid degeneration.Echocardiography examination at the postoperative follow-up of 39.0(22.3, 57.0) months revealed smaller left atrium diameter((38.5±7.1) mm vs. (45.3±8.3) mm, P<0.01), left ventricular end-diastolic diameter ((48.9±6.2) mm vs. (57.5±7.6) mm, P<0.01), reduced left ventricular ejection fraction ((61.2±7.1)% vs. (65.1±6.2)%, P<0.01) and less moderate or severe mitral regurgitation (1.4%(2/148) vs. 100.0%(148/148), P<0.01) compared with the corresponding preoperative values.@*Conclusions@#Dyspnea is the main symptom, and mucoid degeneration characterized by redundant chordae and leaflet thickening are the main pathological features of MVP patients.The surgical treatment of MVP patients is related with satisfactory outcome results.

Mitral valve prolapse in pregnancy

Abstract Mitral valve prolapse is a benign condition. Mitral regurgitation is only complicated in patients with severe mitral valve prolapse. Women with mitral valve prolapse in the absence of other cardiovascular disorders tolerate pregnancy well and do not develop remarkable cardiac complications. Nevertheless, serious complications of mitral valve prolapse, including arrhythmia, infective endocarditis and cerebral ischemic events, can be present in pregnancy. Debates remain with regard to the use of prophylactic antibiotics and β-blockers in the pregnant women with mitral valve prolapse. The prognosis of the pregnant patients might be closely related to the pathological and (or) functional changes of the mitral valve. Non-myxomatous mitral valve prolapse poses no or little obstetric risks in terms of pregnancy, labor and neonatal complications; whereas myxomatous mitral valve prolapse is a major etiology of valvular heart disease in women of childbearing age. In the pregnant patients with mitral valve prolapse progressing into major complications, surgical interventions are considered. Medicinal treatment of such patients with β-blockers should be a concern for the fetal safety.