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Circulation Jun 1976Eighteen cases of mitral atresia with normal aortic valve plus 68 cases from the literature are analyzed. A new classification based on anatomical findings is proposed....
Eighteen cases of mitral atresia with normal aortic valve plus 68 cases from the literature are analyzed. A new classification based on anatomical findings is proposed. Pulmonary stenosis or atresia is frequent in the type with transposition of the great arteries. The rare instances of normal or large left ventricle are due to a large ventricular septal defect, or to straddling or displaced tricuspid valve. The clinical, radiologic and electrocardiographic findings have been summarized and correlated with the different physiopathological situations. In our catheterized cases an oxygen saturation step-up was found in the right atrium together with left atrial hypertension. In seven cases mitral atresia was demonstrated by selective left atrial contrast injection. The average age at death was six months. Among the cases surviving one year or more, the association of atrial septal defects or pulmonary stenosis was frequent. Enlargement of the interatrial communication, accompanied by other palliative measures, is the only available surgical procedure.
Topics: Angiocardiography; Aortic Valve; Arteries; Electrocardiography; Female; Heart Atria; Heart Defects, Congenital; Heart Ventricles; Hemodynamics; Humans; Infant; Infant, Newborn; Male; Mitral Valve; Transposition of Great Vessels
PubMed: 1269115
DOI: 10.1161/01.cir.53.6.1004 -
Ryoikibetsu Shokogun Shirizu 1996
Review
Topics: Diagnosis, Differential; Humans; Mitral Valve; Mitral Valve Insufficiency; Mitral Valve Stenosis
PubMed: 9117607
DOI: No ID Found -
Acta Cardiologica Jun 2020
Topics: Cardiac Imaging Techniques; Computed Tomography Angiography; Double Outlet Right Ventricle; Echocardiography; Heart Septal Defects, Ventricular; Humans; Infant; Male; Mitral Valve; Prognosis; Pulmonary Arterial Hypertension; Pulmonary Valve; Transposition of Great Vessels
PubMed: 30925228
DOI: 10.1080/00015385.2019.1572961 -
The Thoracic and Cardiovascular Surgeon Aug 2015Restrictive atrial septal defect (ASD) is described as risk factor for Norwood procedure because of elevated pulmonary resistance. We hypothesized that it invariably... (Comparative Study)
Comparative Study
BACKGROUND
Restrictive atrial septal defect (ASD) is described as risk factor for Norwood procedure because of elevated pulmonary resistance. We hypothesized that it invariably could not cause pulmonary hypertension, unless it was combined with mitral valve or aortic valve atresia. We investigated how restrictive ASD influenced survival of patients with hypoplastic left heart syndrome (HLHS) who underwent Norwood operation.
PATIENTS AND METHODS
A total of 118 HLHS patients who underwent surgery between January 2005 and December 2012 were grouped into three groups. Group 1 included 31 patients with restrictive ASD combined with mitral or aortic atresia; Group 2 composed of 12 patients with restrictive ASD and mitral and aortic stenosis; Group 3 (n = 75) had no ASD restriction. Survival was determined for each group. Multivariate analysis was conducted to test risk factors for mortality.
RESULTS
Mean follow-up was 26.3 ± 24.1 months. Survival was 78.7% ± 4.2% at 30-month interval and onward after Norwood procedure for the whole cohort; it was 43.8% ± 10.0%, 91.7% ± 8.0%, and 77.3% ± 5.0% for Group 1, 2, and 3, respectively. The difference was significant between Group 1 and Group 2 and 3: p < 0.001. Survival was similar for Group 2 and Group 3: p = 0.45. Combination of restrictive ASD and mitral or aortic atresia was found to be the sole risk factor for early and late mortality (odds ratio: 3.5, 95% confidence interval: 1.8-7.1, p < 0.001).
CONCLUSION
Restrictive ASD only affects survival of HLHS patients following Norwood procedure if it is associated with mitral or aortic atresia.
Topics: Abnormalities, Multiple; Aortic Valve; Cohort Studies; Confidence Intervals; Female; Follow-Up Studies; Heart Defects, Congenital; Heart Septal Defects, Atrial; Humans; Hypoplastic Left Heart Syndrome; Infant; Kaplan-Meier Estimate; Male; Mitral Valve; Multivariate Analysis; Norwood Procedures; Retrospective Studies; Risk Assessment; Statistics, Nonparametric; Survival Analysis; Time Factors; Treatment Outcome
PubMed: 24788706
DOI: 10.1055/s-0034-1374060 -
Journal of Cardiovascular Development... Aug 2022Hypoplastic left heart syndrome (HLHS) is a collective term applied to severe congenital cardiac malformations, characterised by a combination of abnormalities mainly... (Review)
Review
Hypoplastic left heart syndrome (HLHS) is a collective term applied to severe congenital cardiac malformations, characterised by a combination of abnormalities mainly affecting the left ventricle, associated valves, and ascending aorta. Although in clinical practice HLHS is usually sub-categorised based on the patency of the mitral and aortic (left-sided) valves, it is also possible to comprehensively categorise HLHS into defined sub-groups based on the left ventricular morphology. Here, we discuss the published human-based studies of the ventricular myocardium in HLHS, evaluating whether the available evidence is in keeping with this ventricular morphology concept. Specifically, we highlight results from histological studies, indicating that the appearance of cardiomyocytes can be different based on the sub-group of HLHS. In addition, we discuss the histological appearances of endocardial fibroelastosis (EFE), which is a common feature of one specific sub-group of HLHS. Lastly, we suggest investigations that should ideally be undertaken using HLHS myocardial tissues at early stages of HLHS development to identify biological pathways and aid the understanding of HLHS aetiology.
PubMed: 36005443
DOI: 10.3390/jcdd9080279 -
International Journal of Cardiology Apr 1990We report a 20-year-old male patient with mitral atresia, double outlet right ventricle, subaortic ventricular septal defect, valvar pulmonary stenosis and an aneurysm...
We report a 20-year-old male patient with mitral atresia, double outlet right ventricle, subaortic ventricular septal defect, valvar pulmonary stenosis and an aneurysm of atrial septum who presented with minimal effort tolerance and cyanosis. The morphological features were confirmed by cross-sectional echocardiography and cine-angiocardiography.
Topics: Adult; Cineangiography; Double Outlet Right Ventricle; Echocardiography, Doppler; Electrocardiography; Humans; Male; Mitral Valve
PubMed: 2335403
DOI: 10.1016/0167-5273(90)90198-e -
Turk Kardiyoloji Dernegi Arsivi : Turk... Jan 2014Congenital anomalies of the mitral valve apparatus are rare. Of such cases, congenital mitral stenosis, atresia, accessory valvular tissue, and cleft mitral valve are...
Congenital anomalies of the mitral valve apparatus are rare. Of such cases, congenital mitral stenosis, atresia, accessory valvular tissue, and cleft mitral valve are more common. Descriptions of unileaflet mitral valves (either partial or complete leaflet agenesis/hypoplasia) are extremely rare and largely limited to a few case reports. We report herein a 45-year-old nulliparous woman presenting to our outpatient clinic with chest discomfort and dyspnea on minimal exertion. Mitral valve with a single leaflet leading to a significant obstruction in left ventricular outflow was seen on transthoracic echocardiography and confirmed with transesophageal two- (2D) and three-dimensional (3D) echocardiography. Metoprolol was prescribed to relieve obstruction and symptoms.
Topics: Adrenergic beta-1 Receptor Antagonists; Echocardiography, Transesophageal; Female; Heart Defects, Congenital; Humans; Metoprolol; Middle Aged; Mitral Valve; Ventricular Outflow Obstruction
PubMed: 24481102
DOI: 10.5543/tkda.2014.44380 -
European Journal of Cardio-thoracic... Feb 2022Coronary artery atresia is a rare coronary artery anomaly in children and has a high rate of misdiagnosis. We aimed to summarize the profile and early outcomes after the...
OBJECTIVES
Coronary artery atresia is a rare coronary artery anomaly in children and has a high rate of misdiagnosis. We aimed to summarize the profile and early outcomes after the surgical reconstruction of coronary artery atresia in children.
METHODS
A retrospective analysis was performed in 12 consecutive patients with coronary artery atresia who were admitted to the Department of Paediatric Cardiac Surgery of Fuwai Hospital between October 2016 and September 2020. Ten patients underwent surgical reconstruction of the coronary artery with the pulmonary artery anterior wall, and 8 patients underwent concomitant mitral valvuloplasty.
RESULTS
There were 6 females and 6 males, with an age of 1.75 years [interquartile range (IQR), 1.0-3.5] and weight of 10.0 kg (IQR, 8.9-14.75). There were 10 cases of left coronary artery atresia and 2 cases of right coronary artery atresia. All the patients were initially misdiagnosed in the outpatient clinic, but further nonselective coronary angiography confirmed the diagnosis of coronary artery atresia. In all 10 patients with mitral regurgitation, echocardiogram of the mitral valve chordae and papillary muscle revealed ischaemic changes. The clamp time was 89.0 min (IQR, 75.0-101.0), the pump time was 126.0 min (IQR, 119.0-132.0) and the intensive care unit stay time was 1.5 days (IQR, 1.0-3.0). No perioperative deaths were noted. After 9.5 months (IQR, 5.5-13.5) of follow-up, 2 patients with fractional shortening that significantly decreased to 14.1% and 14.8% died and 1 patient had moderate pericardial effusion that improved after treatment with oral diuretics. Coronary artery ultrasound and enhanced computed tomography showed a patent main coronary artery in all the patients.
CONCLUSIONS
Coronary artery atresia in children is often associated with mitral regurgitation, and mitral valve chordae and papillary muscle exhibit ischaemic changes. Coronary artery reconstruction is safe and effective in children with coronary artery atresia.
Topics: Child; Coronary Vessel Anomalies; Female; Humans; Infant; Male; Mitral Valve; Mitral Valve Insufficiency; Retrospective Studies; Treatment Outcome
PubMed: 34459900
DOI: 10.1093/ejcts/ezab319 -
Cardiology in the Young Mar 2023The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary...
The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary artery atresia. Three patients had heart murmurs due to mitral regurgitation at less than 1 year old. Their myocardial ischaemia worsened on exercise with aging. In the fourth patient, hypertrophic cardiomyopathy and Noonan syndrome were suspected at 1 year old. The development of communicating arteries between the conus branch and the left anterior descending artery was detected at 7 years old. The left main coronary artery atresia was confirmed by a selective coronary angiogram at 15 years old. Congenital left main coronary artery atresia could not be diagnosed by two-dimensional echocardiography; however, the left coronary arteries were small. Two patients underwent coronary artery bypass grafting of the left anterior descending artery using the left internal thoracic artery at 3 years and 6 years old, respectively. Two patients had an angioplasty with a cut back at the orifice of the left coronary artery at 2 years old and 17 years old, respectively. Two patients had no cardiac events without medication for more than 30 years after the operation. We must differentiate the diagnosis of left main coronary artery atresia in the small left coronary arteries with mitral regurgitation during the first year. Coronary artery revascularisation and mitral annuloplasty are needed. The long-term outcome of both coronary artery bypass grafting and angioplasty were good. The degree of mitral regurgitation after surgery may affect the prognosis.
Topics: Child; Humans; Adolescent; Child, Preschool; Infant; Mitral Valve Insufficiency; Coronary Artery Bypass; Myocardial Ischemia; Coronary Artery Disease; Treatment Outcome
PubMed: 35351222
DOI: 10.1017/S104795112200083X -
Nihon Rinsho. Japanese Journal of... May 1978
Topics: Humans; Mitral Valve
PubMed: 691603
DOI: No ID Found