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Journal of the American College of... Jun 1992In mitral atresia with a large left ventricle, the tricuspid valve is either straddling and biventricular or entirely left ventricular. To learn how to assess the...
In mitral atresia with a large left ventricle, the tricuspid valve is either straddling and biventricular or entirely left ventricular. To learn how to assess the identity of the tricuspid valve in such cases 15 heart specimens were examined as well as the echocardiograms of 10 living patients. When the right ventricular sinus was underdeveloped (11 cases), a straddling tricuspid valve was present; when it was absent (14 cases), the tricuspid valve was entirely left ventricular. Regardless of biventricular or exclusively left ventricular attachments, the tricuspid valve was tricommissural (at postmortem examination or on echocardiography) in 22 cases (88%). Its chordal attachments showed considerable variations but were usually paraseptal or on the ventricular septal crest or conal septum. When biventricular, the tricuspid valve straddled through an inlet ventricular septal defect. Clinical or anatomic evidence, or both, of tricuspid regurgitation was present in 14 cases (56%). It is concluded that 1) the identity of the atrioventricular valves is reflected in their chordal attachments more accurately than in their leaflet morphology and depends primarily on the type of ventricular loop present; 2) as a rule, the tricuspid valve is right-sided in D-looped and left-sided in L-looped ventricles; 3) valve identity expressed as the number and position of the papillary muscle attachments is generally recognizable echocardiographically and can be used to diagnose the type of ventricular loop that is present; and 4) the presence and degree of tricuspid regurgitation deserve attention when choosing optimal palliative surgery.
Topics: Adult; Cardiac Catheterization; Child; Child, Preschool; Echocardiography; Female; Heart Defects, Congenital; Humans; Infant; Infant, Newborn; Male; Mitral Valve; Tricuspid Valve; Tricuspid Valve Insufficiency
PubMed: 1593052
DOI: 10.1016/0735-1097(92)90619-x -
Cardiology in the Young Jan 2000
Review
Topics: Humans; Mitral Valve; Tricuspid Atresia
PubMed: 10695538
DOI: 10.1017/s1047951100006351 -
A.M.A. American Journal of Diseases of... Aug 1955
Topics: Cardiovascular Abnormalities; Cardiovascular System; Humans; Hypoplastic Left Heart Syndrome
PubMed: 14397834
DOI: 10.1001/archpedi.1955.04030010178009 -
Pediatric and Developmental Pathology :... 1998A newborn female infant was found to have a unique and previously unreported group of anomalies: (1) mitral and aortic atresia with a highly obstructive atrial septum;...
Mitral and aortic atresia associated with hypoplastic right lung, crossover segment of right lower lobe, and anomalous scimitar-like right pulmonary venous connection with inferior vena cava: clinical, angiocardiographic, and autopsy findings in a rare case.
A newborn female infant was found to have a unique and previously unreported group of anomalies: (1) mitral and aortic atresia with a highly obstructive atrial septum; (2) hypoplasia of the right lung with a crossover segment involving the right lower lobe; (3) normally connected pulmonary veins, two from the left lung and one from the right; and (4) a large anomalous branch of the right pulmonary vein of scimitar configuration that anastomosed with the normally connected right pulmonary vein and with the inferior vena cava (IVC). The scimitar vein appeared obstructed at its junction with the right pulmonary vein and at its junction with the inferior vena cava within the hepatic parenchyma. To our knowledge, this is the first report of a scimitar-like vein coexisting with mitral and aortic atresia and connecting both with the right pulmonary vein and with the inferior vena cava. The highly obstructed left atrium was partially decompressed by retrograde blood flow via the normally connected right pulmonary vein to the anomalous scimitar venous pathway and thence to the inferior vena cava via a pulmonary-to-IVC collateral vein.
Topics: Abnormalities, Multiple; Angiocardiography; Aortic Valve; Fatal Outcome; Female; Heart Defects, Congenital; Humans; Infant, Newborn; Lung; Mitral Valve; Pulmonary Veins; Vena Cava, Inferior
PubMed: 9688765
DOI: 10.1007/s100249900056 -
Pediatric Cardiology 1982The pathological anatomy of 109 specimens of aortic valvular atresia was reviewed for the purpose of identifying the cardiovascular anomalies associated with that...
The pathological anatomy of 109 specimens of aortic valvular atresia was reviewed for the purpose of identifying the cardiovascular anomalies associated with that condition. We found the most commonly associated anomaly to be coarctation of the aorta, which was present in 71 percent of our cases and judged to be hemodynamic significance in one-third of the involved cases. Other associated anomalies, in order of decreasing frequency, were mitral atresia, anomalous systemic and pulmonary venous connections, abnormalities of branching of the aortic arch, and ventricular septal defect. The study demonstrated that aortic atresia is associated with a significant incidence of other cardiovascular anomalies. Additional anomalies, when present, may complicate emerging attempts at surgical correction of this condition.
Topics: Aorta; Aortic Coarctation; Aortic Valve; Diseases in Twins; Female; Heart Atria; Heart Septal Defects, Atrial; Heart Septal Defects, Ventricular; Heart Ventricles; Humans; Infant, Newborn; Male; Pulmonary Valve; Pulmonary Veins; Spleen; Tricuspid Valve
PubMed: 7088729
DOI: 10.1007/BF02424944 -
Journal of Ultrasound in Medicine :... Apr 2012
Topics: Abnormalities, Multiple; Heart Ventricles; Humans; Mitral Valve; Tricuspid Atresia; Ultrasonography, Prenatal
PubMed: 22441924
DOI: 10.7863/jum.2012.31.4.654 -
Archives Des Maladies Du Coeur Et Des... Apr 1987This study is devoted to a series of 30 anatomical cases of mitral atresia. The left atrium was dilated in 5 cases and hypoplastic in 25. The interatrial ostium was...
This study is devoted to a series of 30 anatomical cases of mitral atresia. The left atrium was dilated in 5 cases and hypoplastic in 25. The interatrial ostium was small in 50% of the hearts. Left atrio-ventricular concordance was present in 29 cases, as against 1 case of discordance. There was no atrio-ventricular connection in 29 cases, and the mitral valve was imperforate in 1 case. Connections between the tricuspid valve and the main ventricle were normal in 26 hearts. The tricuspid valve straddled the septum in 4 cases. Five hearts had two ventricles, 25 had a single ventricle. Nine hearts showed no ventriculo-atrial atresia. The main vessels were normally located in 1 case, transposed in 2 and originated in the right ventricle in 2 cases; in the 5 hearts with single ventricle the two main vessels arose from the main cavity. Seventeen hearts had an atresic aortic valve and a hypoplastic ascending aorta; the pulmonary artery arose from the right ventricle in 1 case and from the main chamber of a single ventricle heart in 15 cases. The pulmonary valve was atresic and the aorta arose from the main chamber of a single ventricle heart in 4 cases.
Topics: Aortic Valve; Heart Atria; Heart Defects, Congenital; Heart Ventricles; Humans; Infant; Infant, Newborn; Mitral Valve; Pulmonary Valve
PubMed: 3113363
DOI: No ID Found -
Journal of the American College of... Jun 2018
Topics: Antibiotic Prophylaxis; Aortic Valve; Bicuspid Aortic Valve Disease; Endocarditis; Endocarditis, Bacterial; Heart Valve Diseases; Humans; Mitral Valve Prolapse
PubMed: 29903347
DOI: 10.1016/j.jacc.2018.04.021 -
British Heart Journal Mar 1975Although Fontan and Baudet in 1971 described a physiological correction procedure for tricuspid atresia, very few successful operations have been reported. Two patients...
Although Fontan and Baudet in 1971 described a physiological correction procedure for tricuspid atresia, very few successful operations have been reported. Two patients corrected 20 and 10 months ago at the Brompton Hospital are presented. These two patients exhibit many of the problems in the management of tricuspid atresia. The first patient aged 20 had undergone three previous palliative operations, a Blalock-Taussig shunt, a Glenn procedure, and an infundibular resection, and therefore presented a number of operative problems. In contrast the second patient, aged 8, whose condition had deteriorated considerably over the previous year, had had no previous surgical treatment. At operation he was found, in addition, to have a partial atrioventricular canal. Details of the operative procedures and the patients' postoperative course are described. The criteria for selection of patients for the Fontan operation are discussed as are the possible long-term hazards of homograft failure, atrial dysrhythmias, and hepatic dysfunction. The successful outcome of these two patients suggests that palliative surgery in infancy should allow for this form of correction in later life.
Topics: Adult; Aorta; Aortic Valve; Child; Heart Defects, Congenital; Heart Septal Defects, Atrial; Humans; Ligation; Male; Mitral Valve; Palliative Care; Pulmonary Artery; Transplantation, Homologous; Tricuspid Valve
PubMed: 49188
DOI: 10.1136/hrt.37.3.282 -
The Journal of Thoracic and... Feb 2008We sought to identify and characterize a subgroup of patients with hypoplastic left heart syndrome who might be at higher risk for stage I failure.
OBJECTIVE
We sought to identify and characterize a subgroup of patients with hypoplastic left heart syndrome who might be at higher risk for stage I failure.
METHODS
From January 2001 through December 2006, all patients with hypoplastic left heart syndrome who underwent stage I palliation at Children's Hospital Boston were retrospectively reviewed. The subgroup with the mitral stenosis-aortic atresia variant was studied separately. We evaluated preoperative echocardiographic data, operative characteristics, and postoperative factors associated with death or the need for transplantation. The Kaplan-Meier method was used to assess survival.
RESULTS
Thirty-eight (23%) of 165 patients had mitral stenosis-aortic atresia. Hospital mortality or need for transplantation for patients with mitral stenosis-aortic atresia was significantly higher than for other anatomic subgroups (29% vs 7.9%, P = .002). Left ventricle-subepicardial coronary artery communications were present in 20 (53%) patients with mitral stenosis-aortic atresia and were associated with a significantly higher hospital mortality (50% vs 6%, P = .004). No difference in outcome was demonstrated between different sources of pulmonary blood flow. A longer cardiopulmonary bypass time (P = .02) and the need for postoperative extracorporeal membrane oxygenation support (P < .001) were associated with a higher mortality rate.
CONCLUSIONS
With improved outcomes in the management of neonates with hypoplastic left heart syndrome, those with the mitral stenosis-aortic atresia variant and left ventricle-subepicardial coronary artery fistulae have emerged as a higher-risk subgroup for failure of stage I palliation. Further investigation is required, and a change in clinical management strategy for this particular subgroup might be warranted.
Topics: Abnormalities, Multiple; Aorta; Cardiac Surgical Procedures; Cause of Death; Female; Follow-Up Studies; Heart Defects, Congenital; Hospital Mortality; Humans; Hypoplastic Left Heart Syndrome; Infant, Newborn; Male; Mitral Valve Stenosis; Palliative Care; Probability; Retrospective Studies; Risk Assessment; Survival Analysis; Treatment Outcome
PubMed: 18242265
DOI: 10.1016/j.jtcvs.2007.09.007