-
General Thoracic and Cardiovascular... Jul 2012In this report we review our experience of operations on mitral regurgitation associated with abnormal papillary muscles/chordae tendineae of the mitral valves and...
PURPOSE
In this report we review our experience of operations on mitral regurgitation associated with abnormal papillary muscles/chordae tendineae of the mitral valves and discussed the clinical characteristics, operative findings, and treatment strategies.
METHODS
Undifferentiated papillary muscle was defined as a hypoplastic chordae tendineae with anomalous formation of papillary muscles attached to the mitral valves directly. Consecutive 87 patients undergoing surgery for mitral regurgitation at our institution were reviewed and 6 of them had undifferentiated papillary muscle.
RESULTS
The underlying mechanism of regurgitation was prolapse at the center of the anterior leaflet in 3 cases and tethering, a wide area of myxomatous degeneration, and annular dilatation in one case, respectively. Five patients underwent mitral valve plasty and 1 patient received replacement. Anomalous formation of chordae tendineae was corrected by resection and suture with transplantation at the tip of the leaflet to which abnormal chordae were attached in 2 cases, while resection and suture with chordal shortening was performed in 1 case, and chordal reconstruction using artificial chordae was employed in 2 cases. There was no operative death, and postoperative echocardiography showed no residual regurgitation in any of the cases.
CONCLUSIONS
Mitral regurgitation associated with undifferentiated papillary muscle resulted from prolapse or tethering and impaired flexibility of leaflets. It was possible to successfully treat the patients by mitral valve plasty unless complex congenital cardiac malformation coexisted. Detailed examinations of attached papillary muscle by echocardiography and intraoperative inspection are necessary and surgical techniques should be selected appropriately in each case.
Topics: Adolescent; Aged; Cell Differentiation; Chordae Tendineae; Female; Heart Defects, Congenital; Heart Valve Prosthesis Implantation; Humans; Japan; Male; Middle Aged; Mitral Valve; Mitral Valve Annuloplasty; Mitral Valve Insufficiency; Mitral Valve Prolapse; Papillary Muscles; Treatment Outcome; Ultrasonography
PubMed: 22576650
DOI: 10.1007/s11748-012-0055-x -
Re-evaluation of hypoplastic left heart syndrome from a developmental and morphological perspective.Orphanet Journal of Rare Diseases Aug 2017Hypoplastic left heart syndrome (HLHS) covers a spectrum of rare congenital anomalies characterised by a non-apex forming left ventricle and stenosis/atresia of the...
BACKGROUND
Hypoplastic left heart syndrome (HLHS) covers a spectrum of rare congenital anomalies characterised by a non-apex forming left ventricle and stenosis/atresia of the mitral and aortic valves. Despite many studies, the causes of HLHS remain unclear and there are conflicting views regarding the role of flow, valvar or myocardial abnormalities in its pathogenesis, all of which were proposed prior to the description of the second heart field. Our aim was to re-evaluate the patterns of malformation in HLHS in relation to recognised cardiac progenitor populations, with a view to providing aetiologically useful sub-groupings for genomic studies.
RESULTS
We examined 78 hearts previously classified as HLHS, with subtypes based on valve patency, and re-categorised them based on their objective ventricular phenotype. Three distinct subgroups could be identified: slit-like left ventricle (24%); miniaturised left ventricle (6%); and thickened left ventricle with endocardial fibroelastosis (EFE; 70%). Slit-like ventricles were always found in combination with aortic atresia and mitral atresia. Miniaturised left ventricles all had normally formed, though smaller aortic and mitral valves. The remaining group were found to have a range of aortic valve malformations associated with thickened left ventricular walls despite being described as either atresia or stenosis. The degree of myocardial thickening was not correlated to the degree of valvar stenosis. Lineage tracing in mice to investigate the progenitor populations that form the parts of the heart disrupted by HLHS showed that whereas Nkx2-5-Cre labelled myocardial and endothelial cells within the left and right ventricles, Mef2c-AHF-Cre, which labels second heart field-derived cells only, was largely restricted to the endocardium and myocardium of the right ventricle. However, like Nkx2-5-Cre, Mef2c-AHF-Cre lineage cells made a significant contribution to the aortic and mitral valves. In contrast, Wnt1-Cre made a major contribution only to the aortic valve. This suggests that discrete cardiac progenitors might be responsible for the patterns of defects observed in the distinct ventricular sub-groups.
CONCLUSIONS
Only the slit-like ventricle grouping was found to map to the current nomenclature: the combination of mitral atresia with aortic atresia. It appears that slit-like and miniature ventricles also form discrete sub-groups. Thus, reclassification of HLHS into subgroups based on ventricular phenotype, might be useful in genetic and developmental studies in investigating the aetiology of this severe malformation syndrome.
Topics: Animals; Endocardial Fibroelastosis; Heart Defects, Congenital; Heart Ventricles; Homeobox Protein Nkx-2.5; Hypoplastic Left Heart Syndrome; Immunohistochemistry; MEF2 Transcription Factors; Mice; Mitral Valve; Myocardium
PubMed: 28793912
DOI: 10.1186/s13023-017-0683-4 -
Journal of the American College of... Sep 2018
Topics: Aortic Valve; Bicuspid Aortic Valve Disease; Endocarditis; Endocarditis, Bacterial; Heart Valve Diseases; Humans; Mitral Valve Prolapse
PubMed: 30236319
DOI: 10.1016/j.jacc.2018.06.073 -
The Pan African Medical Journal 2016A 4 year-old male presented with effort dyspnea, and was diagnosed as atrioventricular canal defects. This finding was confirmed by open heart surgery, and a congenital... (Review)
Review
A 4 year-old male presented with effort dyspnea, and was diagnosed as atrioventricular canal defects. This finding was confirmed by open heart surgery, and a congenital double orifice mitral valve was discovered. The septal defect was closed but the double orifice mitral valve was respected because of the absence of hemodynamic disturbance. We report this case with review of literature.
Topics: Cardiac Surgical Procedures; Child, Preschool; Dyspnea; Heart Septal Defects; Heart Valve Diseases; Humans; Male; Mitral Valve
PubMed: 27347288
DOI: 10.11604/pamj.2016.23.199.8676 -
Indian Heart Journal 2014Hammock valve, also known as anomalous mitral arcade is a rare mechanism for congenital mitral insufficiency. We report a case of a two-week-old neonate who presented... (Review)
Review
Hammock valve, also known as anomalous mitral arcade is a rare mechanism for congenital mitral insufficiency. We report a case of a two-week-old neonate who presented with features of heart failure and an apical systolic murmur. Echocardiogram showed severe mitral regurgitation and abnormal mitral valve with direct attachment of mitral leaflets to papillary muscle without intervening chordae tendinae, typical of hammock valve. Heart failure was controlled with ionotrpes and diuretics. The literature on the hammock mitral valve is reviewed.
Topics: Echocardiography; Heart Failure; Heart Valve Prosthesis Implantation; Humans; Infant, Newborn; Mitral Valve; Mitral Valve Insufficiency
PubMed: 24973848
DOI: 10.1016/j.ihj.2014.03.008 -
Journal of Cardiology Cases Jan 2017We report the case of a newborn baby with an unguarded mitral orifice associated with asplenia syndrome, double-outlet right ventricle, dysplastic tricuspid valve, and...
We report the case of a newborn baby with an unguarded mitral orifice associated with asplenia syndrome, double-outlet right ventricle, dysplastic tricuspid valve, and pulmonary stenosis. This case was accompanied by severe tricuspid regurgitation and severe right ventricular hypertrophy. The patient had a fatal clinical course due to severe hypoxia and congestive heart failure. Unguarded mitral orifice is a rare disease in which there has been no previous report of lethal clinical course during the neonatal period. Prior reports stated that unguarded mitral orifice was a new constellation of defects and that its etiology and embryology could be classified in the same category because of similar associated malformations of double-outlet right ventricle and pulmonary stenosis or atresia. However, the present case was diagnosed on autopsy as also having asplenia syndrome. Therefore, it is possible that the genetic etiology of unguarded mitral orifice in this case was different from cases of non-heterotaxy. < Unguarded mitral orifice is a rare disease that might be associated with asplenia syndrome and dysplastic tricuspid valve. If unguarded mitral orifice is associated with such defects, the clinical course can be fatal. Therefore, when this diagnosis is recognized, the physician should explain the possibility of neonatal death and plan the treatment of such a case to include grief therapy for the family.>.
PubMed: 30524572
DOI: 10.1016/j.jccase.2016.09.001 -
The Journal of Cardiovascular Surgery Oct 2004Congenital malformations of the mitral valve are numerous and can be confusing. The functional expression is an insufficiency, a stenosis or a combined lesion. The... (Comparative Study)
Comparative Study Review
Congenital malformations of the mitral valve are numerous and can be confusing. The functional expression is an insufficiency, a stenosis or a combined lesion. The anatomic classification is replaced by a functional approach. Anatomy and functional manifestations are analyzed by echocardiography pre-, intra- and postoperatively. Conservative surgery is the goal but is not always possible. Surgery of mitral valve insufficiency has a better prognosis than stenosis. The most difficult malformations are parachute and hammock mitral valves. When the mitral lesion is associated with another cardiac defect, the trend is to treat all the lesions in the same operation.
Topics: Cardiac Surgical Procedures; Child, Preschool; Female; Follow-Up Studies; Heart Defects, Congenital; Heart Valve Diseases; Hemodynamics; Humans; Infant; Infant, Newborn; Male; Mitral Valve; Postoperative Complications; Risk Assessment; Survival Rate; Suture Techniques; Treatment Outcome; Ultrasonography
PubMed: 15736569
DOI: No ID Found -
The Thoracic and Cardiovascular Surgeon Jan 2022Surgical correction of an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) has been associated with excellent survival during recent...
BACKGROUND
Surgical correction of an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) has been associated with excellent survival during recent years. The purpose of this study was to evaluate the effectiveness of reimplantation of the coronary artery and to investigate the recovery of postoperative cardiac and mitral valve (MV) function.
METHODS
From 2005 to 2015, 80 patients who had ALCAPA received surgical correction. Among them, 49 were infants. The median patient age was 7.8 months. Operative strategies included reimplantation of the coronary artery in 71 patients, the Takeuchi procedure in another 7 patients, and coronary artery ligation in the remaining 2 patients.
RESULTS
There were 11 hospital deaths and 2 late deaths. Six patients required intraoperative or postoperative mechanical circulatory support. A significant improvement in the ejection fraction (EF) and shortening fraction (SF) was present in all surviving patients at discharge, at a 3-month follow-up and at a 1-year follow-up. MV function improved gradually after surgical repair with no late secondary intervention.
CONCLUSIONS
The repair of ALCAPA can be accomplished by establishment of a dual-coronary system, which offers an acceptable mortality rate and will rarely require a second surgery. Left ventricular (LV) recovery is a progressive process, especially for infants with impaired LV function. Concomitant MV annuloplasty is safe and reliable and can be performed as necessary in patients with moderate or severe mitral valve regurgitation.
Topics: Bland White Garland Syndrome; Child; Coronary Vessel Anomalies; Humans; Infant; Mitral Valve Insufficiency; Pulmonary Artery; Retrospective Studies; Treatment Outcome
PubMed: 33851407
DOI: 10.1055/s-0041-1725978 -
Journal of the American College of... Oct 1986Mitral valve prolapse, the most common inherited cardiovascular condition, has been associated with a variety of signs, symptoms and electrocardiographic abnormalities,... (Comparative Study)
Comparative Study
Mitral valve prolapse, the most common inherited cardiovascular condition, has been associated with a variety of signs, symptoms and electrocardiographic abnormalities, but the true spectrum of the mitral prolapse syndrome remains in doubt because clinical findings often contribute to patient identification and their prevalence in patient groups may be overstated because of ascertainment bias. Accordingly, clinical findings in 88 patients with echocardiographic mitral prolapse were compared with those in 81 of their adult first degree relatives with mitral prolapse (a group free of ascertainment bias) and in two control groups without mitral prolapse: 172 first degree relatives and 60 spouses. Comparison of relatives with and without mitral prolapse demonstrated true associations between mitral prolapse and clicks or murmurs, or both (67 versus 9%, p less than 0.001), thoracic bony abnormalities (41 versus 16%, p less than 0.001), systolic blood pressure less than 120 mm Hg (53 versus 31%, p less than 0.001), body weight 90% or less of ideal (31 versus 14%, p less than 0.005) and palpitation (40 versus 24%, p less than 0.01). In contrast, relatives with mitral prolapse showed no significant increase over normal relatives or spouses without mitral prolapse in prevalence of chest pain, dyspnea, panic attacks, high anxiety or repolarization abnormalities, but these features were all more common in women than in men (p less than 0.01 to less than 0.001). Thus, the true spectrum of the mitral prolapse syndrome encompasses a midsystolic click and late systolic murmur, thoracic bony abnormalities, low body weight and blood pressure and palpitation. Other suggested clinical features, including nonanginal chest pain, dyspnea, panic attacks and electrocardiographic abnormalities, have appeared to be associated with mitral valve prolapse because of ascertainment bias and an erroneous classification of differences between men and women as being due to mitral valve prolapse.
Topics: Adolescent; Adult; Anxiety; Arrhythmias, Cardiac; Body Weight; Echocardiography; Electrocardiography; Female; Humans; Hypotension; Male; Middle Aged; Mitral Valve Prolapse; Syndrome; Thorax
PubMed: 3760352
DOI: 10.1016/s0735-1097(86)80415-6 -
The Journal of Thoracic and... Feb 2009In "true" parachute mitral valve, mitral valve chordae insert into one papillary muscle. In parachute-like asymmetric mitral valve, most or all chordal attachments are... (Comparative Study)
Comparative Study
OBJECTIVE
In "true" parachute mitral valve, mitral valve chordae insert into one papillary muscle. In parachute-like asymmetric mitral valve, most or all chordal attachments are to one papillary muscle. This study compared morphologic features, associated lesions, and palliation strategies of the two parachute mitral valve and dominant papillary muscle types and examined interventions and midterm outcomes in patients with biventricular circulation.
METHODS
Echocardiography and autopsy databases were reviewed to identify patients with "true" parachute mitral valve or parachute-like asymmetric mitral valve from January 1987 to January 2006. Predictors of palliation strategy in the entire cohort, mitral stenosis on initial echocardiogram, and mortality in the biventricular cohort were determined with logistic regression.
RESULTS
Eighty-six patients with "true" parachute mitral valve (n = 49) or parachute-like asymmetric mitral valve (n = 37) were identified. Chordal attachments to the posteromedial papillary muscle were more common (73%). The presence "true" parachute mitral valve (P = .008), hypoplastic left ventricle (P < .001), and two or more left-sided obstructive lesions (P = .002) predicted univentricular palliation. Among 49 patients maintaining biventricular circulation at follow-up, 8 died median follow-up 6.4 years (7 days-17.8 years). Multivariate analysis revealed that "true" parachute mitral valve was associated with mitral stenosis on initial echocardiogram (P = .03), and "true" parachute mitral valve (P = .04) and conotruncal anomalies (P = .0003) were associated with mortality. Progressive mitral stenosis was found in 11 patients; 2 underwent mitral valve interventions, and 1 died.
CONCLUSION
Nearly two thirds of this parachute mitral valve cohort underwent biventricular palliation. Some progression of mitral stenosis occurred, although mitral valve intervention was rare. "True" parachute mitral valve was associated with mitral stenosis on initial echocardiogram. "True" parachute mitral valve and conotruncal anomalies were associated with mortality in the biventricular population.
Topics: Adolescent; Cardiac Surgical Procedures; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Mitral Valve; Mitral Valve Stenosis; Multivariate Analysis; Papillary Muscles; Retrospective Studies
PubMed: 19185158
DOI: 10.1016/j.jtcvs.2008.09.016