-
Revista Espanola de Cardiologia Apr 2007
Review
Topics: Antibiotic Prophylaxis; Aortic Valve; Aortic Valve Insufficiency; Aortic Valve Stenosis; Endocarditis, Bacterial; Heart Valve Diseases; Heart Valve Prosthesis; Humans; Mitral Valve Insufficiency; Mitral Valve Stenosis; Thrombosis; Tricuspid Valve Insufficiency; Ultrasonography
PubMed: 17521540
DOI: No ID Found -
Journal of Cardiothoracic Surgery Apr 2021Parachute mitral valve with reticular chordae tendineae is an extremely rare anomaly.
BACKGROUND
Parachute mitral valve with reticular chordae tendineae is an extremely rare anomaly.
CASE PRESENTATION
We present a case of parachute mitral valve associated with distinctive reticular chordae tendineae in an adult. It was diagnosed from the echocardiogram. The patient was referred for surgery. Valve analysis showed thickened mitral valve leaflets and commissures. The chordae tendinae were lengthy and thick. All the chordae tendinae merged into a solitary papillary muscle. A distinctive reticular fibrous tissue was found on mitral valve apparatus as the chordae tendinae intermixed each other. The only functional communication between the left atrium and the left ventricle was through the reticular spaces. This anomaly was considered to be unrepairable and was replaced with a mechanical valve.
CONCLUSIONS
An extremely rare and unique case of parachute mitral valve associated with reticular chordae tendineae was reported. Mitral valve replacement is a reasonable choice in patients with parachute mitral valve with reticular chordae tendineae.
Topics: Adult; Chordae Tendineae; Echocardiography; Heart Valve Prosthesis; Humans; Male; Mitral Valve Insufficiency; Papillary Muscles
PubMed: 33836801
DOI: 10.1186/s13019-021-01448-4 -
Circulation Sep 2010Analyzing the determinants of systolic anterior motion of the mitral valve and consequent left ventricular outflow tract (LVOT) obstruction in patients with asymmetrical...
In vivo measurement of mitral leaflet surface area and subvalvular geometry in patients with asymmetrical septal hypertrophy: insights into the mechanism of outflow tract obstruction.
BACKGROUND
Analyzing the determinants of systolic anterior motion of the mitral valve and consequent left ventricular outflow tract (LVOT) obstruction in patients with asymmetrical septal hypertrophy requires a comprehensive 3-dimensional analysis of mitral leaflet (ML) area, papillary muscle (PM) geometry, and the distribution of left ventricular hypertrophy.
METHODS AND RESULTS
Real-time 3-dimensional echocardiography was performed in 47 patients with asymmetrical septal hypertrophy and 32 normal controls. Patients included 20 with resting LVOT obstruction (group I) and 27 without (group II). Customized software (Omni 4D) provided a validated measure of ML surface area, LVOT area, mitral annular area and nonplanarity, LVOT hypertrophy index by topography (percent area with wall thickness >16 mm), and 3-dimensional PM positions relative to annulus. ML area was more than twice as large in group I than normal and 1.4 times normal in group II (P<0.001). Group I patients were also characterized by higher LVOT hypertrophy index and medial and anterior displacements of both PMs, resulting in a shorter inter-PM distance. Independent determinants of LVOT obstruction were indexed total ML area (adjusted odds ratio, 5.651; 95% confidence interval, 1.573 to 20.304; P=0.008) and inter-PM distance (adjusted odds ratio, 0.416; 95% confidence interval, 0.203 to 0.854; P=0.0169). Minimal LVOT area during systole correlated well with peak LVOT pressure gradient (R(2)=0.83, P<0.001); its independent determinants were left ventricular end-systolic volume (P=0.0183), indexed total ML area (P=0.0108), inter-PM distance (P=0.0378), annular height (P=0.0047), and LVOT hypertrophy index (P=0.0098).
CONCLUSIONS
Myocardium is not the only tissue affected in patients with asymmetrical septal hypertrophy, and primary changes of the mitral apparatus, including ML area increase and PM displacement, are independent determinants of LVOT obstruction and provide a comprehensive mechanism that determines leaflet slack and anteriorly directed motion. Abnormal PM-mitral valve geometry assessed by real-time 3-dimensional echocardiography can provide reasonable new targets for individualized intervention.
Topics: Echocardiography, Three-Dimensional; Female; Heart Rate; Heart Septum; Heart Ventricles; Humans; Male; Middle Aged; Mitral Valve; Ventricular Dysfunction, Left; Ventricular Outflow Obstruction
PubMed: 20837895
DOI: 10.1161/CIRCULATIONAHA.109.935551 -
Texas Heart Institute Journal 2011WEB SITE FEATURE
WEB SITE FEATURE
Topics: Adult; Coronary Disease; Coronary Sinus; Dilatation, Pathologic; Echocardiography, Doppler, Color; Female; Humans; Mitral Valve Stenosis; Predictive Value of Tests; Tricuspid Valve Insufficiency; Vascular Malformations; Vena Cava, Inferior
PubMed: 21720482
DOI: No ID Found -
Turk Kardiyoloji Dernegi Arsivi : Turk... Oct 2011
Topics: Adult; Angina Pectoris; Diagnosis, Differential; Dyspnea; Echocardiography, Doppler; Female; Humans; Mitral Valve; Mitral Valve Insufficiency
PubMed: 21983777
DOI: 10.5543/tkda.2011.01604 -
Brazilian Journal of Cardiovascular... Dec 2019This study aimed to evaluate the early operative outcomes and to compare the left ventricle and mitral valve functions after initial Takeuchi repair in patients with...
OBJECTIVE
This study aimed to evaluate the early operative outcomes and to compare the left ventricle and mitral valve functions after initial Takeuchi repair in patients with anomalous left coronary arising from pulmonary artery (ALCAPA).
METHODS
Fourteen patients (5 males, 9 females; mean age 4.3 years, ranging from 25 days to 34 years) who were operated for ALCAPA between 2007 and 2018 were included in this study. Data were evaluated retrospectively based on our medical records.
RESULTS
Hospital mortality rate was 7.1% (n=1). Thirteen surviving patients were kept in follow-up mean 4.3±3.05 years. When compared to preoperative measurements, both left ventricular ejection fraction (LVEF), (P=0.007) and mitral regurgitation (MR) (P=0.001) significantly improved before discharge. Moreover, LVEF values were improved in the late follow-up, considering early postoperative outcomes, and this alteration was significant (P=0.014). Nevertheless, alteration in the degree of MR among patients did not differ in the long-term follow-up (P=0.180). There was no late-term mortality or need for reoperation among patients.
CONCLUSION
Although some centers prefer to direct implantation in ALCAPA, Takeuchi procedure can be accepted as a reliable method that provides satisfactory long-term results, considering that it aids to improve left ventricle ejection fraction and reduced mitral valve regurgitation.
Topics: Adolescent; Adult; Bland White Garland Syndrome; Cardiac Surgical Procedures; Child; Child, Preschool; Female; Hospital Mortality; Humans; Infant; Male; Mitral Valve; Mitral Valve Insufficiency; Pulmonary Artery; Retrospective Studies; Treatment Outcome; Vascular Surgical Procedures; Young Adult
PubMed: 31364346
DOI: 10.21470/1678-9741-2018-0376 -
Journal of the American College of... Jun 1991The clinical, surgical and morphologic findings in five cases of a rare form of straddling mitral valve are presented. Three patients were diagnosed by two-dimensional...
Straddling mitral valve with hypoplastic right ventricle, crisscross atrioventricular relations, double outlet right ventricle and dextrocardia: morphologic, diagnostic and surgical considerations.
The clinical, surgical and morphologic findings in five cases of a rare form of straddling mitral valve are presented. Three patients were diagnosed by two-dimensional echocardiography, cardiac catheterization and angiocardiography and two had diagnostic confirmation at autopsy. All five cases shared a distinctive and consistent combination of anomalies: 1) dextrocardia; 2) visceroatrial situs solitus, concordant ventricular D-loop and double outlet right ventricle with the aorta positioned to the left of and anterior to the pulmonary artery; 3) hypoplasia of right ventricular inflow (sinus) with tricuspid valve stenosis or hypoplasia; 4) large right ventricular infundibulum (outflow); 5) malalignment conoventricular septal defect; 6) straddling mitral valve with chordal attachments to the left ventricle and right ventricular infundibulum; 7) severe subpulmonary stenosis with well developed pulmonary arteries; and 8) superoinferior ventricles with crisscross atrioventricular (AV) relations. The degree of malalignment between the atrial and ventricular septa was studied quantitatively by measuring the AV septal angle projected on the frontal plane. The AV septal angle in the two postmortem cases was 150 degrees, reflecting marked malalignment of the ventricles relative to the atria. This AV malalignment appears to play an important role in the morphogenesis of straddling mitral valve. As judged by a companion study of seven postmortem cases, the more common form of straddling mitral valve with a hypertrophied and enlarged right ventricular sinus had less severe ventricular malposition than did the five rare study cases with hypoplastic right ventricular sinus. A competent mitral valve, low pulmonary vascular resistance and low left ventricular end-diastolic pressure were found at cardiac catheterization in the three living patients who underwent a modified Fontan procedure and are doing well 2.2 to 5.8 years postoperatively.
Topics: Adult; Angiocardiography; Cardiac Catheterization; Child; Echocardiography; Female; Heart Defects, Congenital; Humans; Male; Mitral Valve
PubMed: 2033193
DOI: 10.1016/0735-1097(91)90655-s -
Annals of Pediatric Cardiology 2020Unguarding of an atrioventricular valvar orifice is a rare form of congenital heart disease that requires staged functionally univentricular palliation. Unguarding of...
Unguarding of an atrioventricular valvar orifice is a rare form of congenital heart disease that requires staged functionally univentricular palliation. Unguarding of the mitral valvar orifice has previously been reported in the setting of mirror-imaged atrial arrangement. We report a neonate with unguarding of the mitral valvar orifice in the setting of usual atrial arrangement, but with discordant atrioventricular connections and pulmonary atresia.
PubMed: 32030042
DOI: 10.4103/apc.APC_4_19 -
American Journal of Medical Genetics.... Mar 2021Xia-Gibbs syndrome (XGS) is a very rare genetic condition. The clinical spectrum is very broad and variable. The phenotype and evolution in a Congolese boy with XGS have... (Review)
Review
Xia-Gibbs syndrome (XGS) is a very rare genetic condition. The clinical spectrum is very broad and variable. The phenotype and evolution in a Congolese boy with XGS have been reported. At 6 years he had speech delay, drooling, marked hyperactivity, attention deficit, aggressive behavior, and intellectual disability. Dysmorphological evaluation revealed strabismus, mild unilateral ptosis, uplifted ear lobes, flat philtrum, thin upper lip vermillion, high arched palate, and flat feet. Patient-only whole exome sequencing identified a known pathogenic frameshift variant in the AHDC1 gene [NM_001029882.3(AHDC1):c.1122dupC;(p.Gly375ArgfsTer3)]. The clinical follow-up revealed the deterioration of his fine motor skills and significant cerebellar phenotype including tremor, pes cavus, and gait instability at the age of 12 years. This patient was compared with three previously reported patients with the same variant but did not identify a consistent pattern in the evolution of symptoms with age.
Topics: Abnormalities, Multiple; Agenesis of Corpus Callosum; Attention Deficit Disorder with Hyperactivity; Child; DNA-Binding Proteins; Democratic Republic of the Congo; Developmental Disabilities; Face; Frameshift Mutation; Humans; Intellectual Disability; Language Development Disorders; Male; Mitral Valve Insufficiency; Palate; Syndrome; Talipes Cavus; Exome Sequencing
PubMed: 33372375
DOI: 10.1002/ajmg.a.62049 -
The Journal of Thoracic and... Feb 2020
Topics: Aortic Valve; Bicuspid Aortic Valve Disease; Child; Heart Valve Diseases; Humans; Mitral Valve
PubMed: 31447133
DOI: 10.1016/j.jtcvs.2019.07.044