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British Heart Journal Mar 1984The morphological characteristics of mitral atresia were studied in 30 hearts to determine the presence or absence of a morphological rudiment of the atretic valve and...
The morphological characteristics of mitral atresia were studied in 30 hearts to determine the presence or absence of a morphological rudiment of the atretic valve and the relation of this rudiment, if found, to any chamber in the ventricular mass. All the hearts showed atrial situs solitus and no ventricular inversion; consequently all had left atrioventricular atresia. In all instances dense fibrous tissue connected the floor of the left atrium to the left ventricle. This connective tissue is considered to be the morphological rudiment of the atretic mitral valve. In several hearts the intervening fibrous tissue varied from a thick fibrous membrane to a tiny fibrous cord; it is impossible to detect these variations clinically. It is, therefore, more practical to classify those hearts which have a detectable fibrous membrane macroscopically as having an "imperforate membrane" and those with a fibrous strand detectable only microscopically as having an "absent atrioventricular connection."
Topics: Heart Defects, Congenital; Heart Ventricles; Humans; Mitral Valve; Terminology as Topic
PubMed: 6696802
DOI: 10.1136/hrt.51.3.252 -
BMJ Case Reports Jun 2024Anomalous mitral arcade (MA) is a rare congenital anomaly. We report a case of MA in a newborn who presented with hydrops fetalis due to severe mitral regurgitation....
Anomalous mitral arcade (MA) is a rare congenital anomaly. We report a case of MA in a newborn who presented with hydrops fetalis due to severe mitral regurgitation. After birth, he developed severe respiratory failure, congestive heart failure and airway obstruction because an enlarged left atrium from severe mitral regurgitation compressed the distal left main bronchus. There is limited experience in surgical management of this condition in Thailand, and the patient's mitral valve was too small for replacement. Therefore, he was treated with medication to control heart failure and supported with positive pressure ventilation to promote growth. We have followed the patient until the current time of writing this report at the age of 2 years, and his outcome is favourable regarding heart failure symptoms, airway obstruction, growth and development. This case describes a challenging experience in the non-surgical management of MA with severe regurgitation, which presented at birth.
Topics: Humans; Mitral Valve Insufficiency; Hydrops Fetalis; Male; Infant, Newborn; Mitral Valve; Echocardiography; Heart Failure; Heart Defects, Congenital; Positive-Pressure Respiration
PubMed: 38866580
DOI: 10.1136/bcr-2023-259272 -
The Journal of Thoracic and... Oct 2008Successful mitral valve replacement in young children is limited by the lack of small prosthetic valves. Supra-annular prosthesis implantation can facilitate mitral...
Mitral valve replacement in infants and children 5 years of age or younger: evolution in practice and outcome over three decades with a focus on supra-annular prosthesis implantation.
OBJECTIVE
Successful mitral valve replacement in young children is limited by the lack of small prosthetic valves. Supra-annular prosthesis implantation can facilitate mitral valve replacement with a larger prosthesis in children with a small annulus, but little is known about its effect on the outcomes of mitral valve replacement in young children.
METHODS
One hundred eighteen children underwent mitral valve replacement at 5 years of age or younger from 1976-2006. Mitral valve replacement was supra-annular in 37 (32%) patients.
RESULTS
Survival was 74% +/- 4% at 1 year and 56% +/- 5% at 10 years but improved over time (10-year survival of 83% +/- 7% from 1994-2006). Factors associated with worse survival included earlier mitral valve replacement date, age less than 1 year, complete atrioventricular canal, and additional procedures at mitral valve replacement, but not supra-annular mitral valve replacement. As survival improved during our more recent experience, the risks of supra-annular mitral valve replacement became apparent; survival was worse among patients with a supra-annular prosthesis after 1991. A pacemaker was placed in 18 (15%) patients within 1 month of mitral valve replacement and was less likely in patients who had undergone supra-annular mitral valve replacement. Among early survivors, freedom from redo mitral valve replacement was 72% +/- 5% at 5 years and 45% +/- 7% at 10 years. Twenty-one patients with a supra-annular prosthesis underwent redo mitral valve replacement. The second prosthesis was annular in 15 of these patients and upsized in all but 1, but 5 required pacemaker placement for heart block.
CONCLUSIONS
Supra-annular mitral valve replacement was associated with worse survival than annular mitral valve replacement in our recent experience. Patients with supra-annular mitral valve replacement were less likely to have operative complete heart block but remained at risk when the prosthesis was subsequently replaced.
Topics: Age Factors; Child, Preschool; Cohort Studies; Female; Follow-Up Studies; Heart Defects, Congenital; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Infant; Infant, Newborn; Kaplan-Meier Estimate; Logistic Models; Male; Mitral Valve; Mitral Valve Insufficiency; Mitral Valve Stenosis; Multivariate Analysis; Postoperative Complications; Probability; Proportional Hazards Models; Prosthesis Design; Registries; Retrospective Studies; Risk Assessment; Survival Rate; Time Factors; Treatment Outcome
PubMed: 18954636
DOI: 10.1016/j.jtcvs.2007.12.076 -
Journal of the American College of... Jan 2011The objective of this article is to review anatomic, physiologic, and clinical features of fetuses and neonates with severe mitral regurgitation (MR) in conjunction with... (Comparative Study)
Comparative Study
Aortic stenosis and severe mitral regurgitation in the fetus resulting in giant left atrium and hydrops: pathophysiology, outcomes, and preliminary experience with pre-natal cardiac intervention.
OBJECTIVES
The objective of this article is to review anatomic, physiologic, and clinical features of fetuses and neonates with severe mitral regurgitation (MR) in conjunction with aortic stenosis (AS) and left ventricular (LV) and left atrial (LA) dilation and to present preliminary results of pre-natal intervention for this condition.
BACKGROUND
Severe fetal valvar AS with an abnormal mitral valve (MV) and MR can lead to left heart dilation, with consequent compression of the right ventricle (RV); hydrops and low cardiac output are often associated.
METHODS
This is a retrospective review of fetuses diagnosed with AS, severe MR, and LA dilation (2002 to 2009) and neonates with the same combination of abnormalities (1988 to 2009).
RESULTS
Fourteen fetuses and 7 neonates were investigated. Eleven fetuses had severe hydrops; all had polyhydramnios and a structurally abnormal MV, abnormal MV inflow pattern, restrictive/intact atrial septum, retrograde flow in the transverse aortic arch, and compression of the right heart. The mean indexed RV output was 326 ± 160 ml/kg/min, lower than the normal average fetal combined ventricular output of 550 ± 150 ml/kg/min. Ten fetuses underwent pre-natal cardiac intervention: aortic valvuloplasty (n = 8) and/or atrial septal dilation/stenting (n = 5). Seven of these, and 11 overall, were live born. Nine patients died (median age 6 days), and 2 patients are currently alive. All 7 patients diagnosed in the neonatal period died (median age 1 day).
CONCLUSIONS
Aortic stenosis associated with significant MR in the fetus can cause severe LA and LV enlargement, leading to low cardiac output and hydrops. Despite the potential advantages of early pre-natal diagnosis and both fetal and neonatal interventions, this rare complex of anomalies carries a poor prognosis.
Topics: Aortic Valve Stenosis; Child, Preschool; Cohort Studies; Female; Heart Atria; Humans; Hydrops Fetalis; Infant, Newborn; Mitral Valve Insufficiency; Pregnancy; Retrospective Studies; Treatment Outcome; Ultrasonography, Prenatal
PubMed: 21232673
DOI: 10.1016/j.jacc.2010.08.636 -
The Journal of Thoracic and... Jun 2011Our objective was to determine the feasibility and early to medium-term outcome of stenting the patent ductus arteriosus at the time of radiofrequency valvotomy in the...
Concomitant stenting of the patent ductus arteriosus and radiofrequency valvotomy in pulmonary atresia with intact ventricular septum and intermediate right ventricle: early in-hospital and medium-term outcomes.
OBJECTIVES
Our objective was to determine the feasibility and early to medium-term outcome of stenting the patent ductus arteriosus at the time of radiofrequency valvotomy in the subgroup of patients with pulmonary atresia with intact ventricular septum and intermediate right ventricle.
BACKGROUND
Stenting of the patent ductus arteriosus and radiofrequency valvotomy have been proposed as the initial intervention for patients with intermediate right ventricle inasmuch as the sustainability for biventricular circulation or 1½-ventricle repair is unclear in the early period.
METHODS
Between January 2001 and April 2009, of 143 patients with pulmonary atresia and intact ventricular septum, 37 who had bipartite right ventricle underwent radiofrequency valvotomy and stenting of the patent ductus arteriosus as the initial procedure. The mean tricuspid valve z-score was -3.8 ± 2.2 and the mean tricuspid valve/mitral valve ratio was 0.62 ± 0.16.
RESULTS
Median age was 10 days (3-65 days) and median weight 3.1 kg (2.4-4.9 kg). There was no procedural mortality. Acute stent thrombosis developed in 1 patient and necessitated emergency systemic-pulmonary shunt. There were 2 early in-hospital deaths owing to low cardiac output syndrome. One late death occurred owing to right ventricular failure after the operation. Survival after the initial procedure was 94% at 6 months and 91% at 5 years. At a median follow-up of 4 years (6 months to 8 years), 17 (48%) attained biventricular circulation with or without other interventions and 9 (26%) achieved 1½-ventricle repair. The freedom from reintervention was 80%, 68%, 58%, and 40% at 1, 2, 3, and 4 years, respectively.
CONCLUSIONS
Concomitant stenting of the patent ductus arteriosus at the time of radiofrequency valvotomy in patients with pulmonary atresia with intact ventricular septum and intermediate right ventricle is feasible and safe with encouraging medium-term outcome.
Topics: Abnormalities, Multiple; Cardiac Catheterization; Catheter Ablation; Ductus Arteriosus, Patent; Feasibility Studies; Heart Ventricles; Hospital Mortality; Humans; Infant; Infant, Newborn; Kaplan-Meier Estimate; Malaysia; Pulmonary Atresia; Radiography, Interventional; Recovery of Function; Stents; Survival Rate; Time Factors; Treatment Outcome; Ultrasonography; Ventricular Septum
PubMed: 21227471
DOI: 10.1016/j.jtcvs.2010.08.085 -
California Medicine Mar 1968The death rate among neonates with cardiovascular disease is 50 percent during the first six months, with the majority dying during the first month. With early diagnosis... (Review)
Review
The death rate among neonates with cardiovascular disease is 50 percent during the first six months, with the majority dying during the first month. With early diagnosis most of these babies could be saved. In approaching the diagnosis of cardiac distress in the newborn, it is important to remember that the types of cardiovascular disease which cause symptoms and death early in life are quite different from those in older children. Lesions such as hypoplasia of the left heart, transposition of the great arteries, endocardial fibroelastosis, pulmonary atresia, mitral atresia, tricuspid atresia and truncus arteriosus are common, not rare, causes of cardiac distress in the newborn.A classification of neonatal cardiovascular diseases into seven pathophysiological groups is presented as a basis for an effective, practical approach to the differential diagnosis of the potentially lethal lesions. This approach is simplified further since over 90 percent of babies with cardiac distress have one of three lesions: (1) Large left-to-right shunt (characterized by the presence of massive plethora on the chest roentgenogram), (2) Large right-to-left shunt (association with intense cyanosis) or (3) Severe obstruction (including hypoplasia of the left heart, which is the most common cause of death due to cardiac distress during the first week of life).
Topics: Aortic Valve Insufficiency; Arrhythmias, Cardiac; Dextrocardia; Electrocardiography; Endocardial Fibroelastosis; Heart Block; Heart Defects, Congenital; Humans; Infant, Newborn; Myocarditis; Transposition of Great Vessels
PubMed: 4867629
DOI: No ID Found -
Indian Heart Journal 201462-year-old female presented with progressive dyspnea NYHA class III for six months. Echocardiography showed normal left ventricular (LV) systolic function, mild...
62-year-old female presented with progressive dyspnea NYHA class III for six months. Echocardiography showed normal left ventricular (LV) systolic function, mild biatrial enlargement, an L wave in pulse wave Doppler at mitral inflow and in M mode echocardiography across mitral valve. Tissue Doppler imaging at medial mitral annulus showed an L' wave in mid diastole in addition to E' and A' wave. An L wave in pulse wave Doppler and M mode echocardiography represents continued pulmonary vein mid diastolic flow through the left atrium in to LV across mitral valve after early rapid filling. Presence of an L' wave in these patients associated with higher E/E' is indicative of advance diastolic dysfunction with elevated filling pressures.
Topics: Echocardiography, Doppler; Female; Humans; Middle Aged; Mitral Valve; Mitral Valve Insufficiency; Ventricular Dysfunction, Left; Ventricular Function, Left
PubMed: 24973854
DOI: 10.1016/j.ihj.2014.03.022 -
The Journal of Thoracic and... Jun 2017
Topics: Aorta; Aortic Valve; Bicuspid Aortic Valve Disease; Constriction, Pathologic; Heart Valve Diseases; Humans; Mitral Valve
PubMed: 28377120
DOI: 10.1016/j.jtcvs.2017.03.018 -
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... Oct 1999Aortic valve atresia or hypoplasia can present with a ventricular septal defect and a normal mitral valve and left ventricle. These patients may be suitable for...
OBJECTIVE
Aortic valve atresia or hypoplasia can present with a ventricular septal defect and a normal mitral valve and left ventricle. These patients may be suitable for biventricular repair, although the optimal initial management strategy remains unknown.
METHODS
From January 1991 through March 1999, 20 patients with aortic atresia or hypoplasia and ventricular septal defect underwent operation with the intent to achieve biventricular repair. Aortic atresia was present in 7 patients, and aortic valve hypoplasia was present in 13 patients. Among those patients with aortic hypoplasia, Z-scores of the aortic valve anulus ranged from -8.8 to -2.7. Associated anomalies included interrupted aortic arch (n = 12 patients), coarctation (n = 6 patients), aortopulmonary window (n = 1 patient), and heterotaxia (n = 1 patient). Nine patients were staged with an initial Norwood procedure followed by biventricular repair in 8 patients. One patient awaits biventricular repair after a Norwood procedure. The conditions of 11 patients were corrected with a single procedure.
RESULTS
Among the 9 patients who underwent staged repair, there were no deaths after the Norwood procedure and 1 death after biventricular repair. For the 11 patients who underwent a primary biventricular repair, there was 1 early death and 2 late deaths from noncardiac causes. Follow-up ranged from 1 to 85 months (mean, 28 months). Actuarial survival for the entire group was 78% +/- 10% at 5 years and was not significantly different between staged repair (89%) and primary biventricular repair (73%).
CONCLUSIONS
Both primary and staged biventricular repair for patients with aortic atresia or hypoplasia and ventricular septal defect may be performed with good late survival. Refinements in technique of conduit insertion and arch reconstruction have resulted in primary biventricular repair becoming our preferred approach.
Topics: Actuarial Analysis; Anastomosis, Surgical; Aorta; Aortic Coarctation; Aortic Valve; Blood Vessel Prosthesis Implantation; Cardiac Surgical Procedures; Cause of Death; Dextrocardia; Echocardiography, Doppler; Female; Follow-Up Studies; Heart Septal Defects, Ventricular; Heart Ventricles; Humans; Infant; Infant, Newborn; Male; Pulmonary Artery; Surgical Wound Infection; Survival Rate; Transposition of Great Vessels; Treatment Outcome
PubMed: 10504629
DOI: 10.1016/S0022-5223(99)70010-3