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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 -
World Journal For Pediatric &... Sep 2022Left ventricular hypoplasia is a common finding in various forms of congenital heart disease. Echocardiography in the setting of left ventricular hypoplasia must...
Left ventricular hypoplasia is a common finding in various forms of congenital heart disease. Echocardiography in the setting of left ventricular hypoplasia must comprehensively assess the size and function of all left-sided structures including the mitral valve, left ventricular outflow tract, aortic valve and aortic arch. Of most importance in any variation of left ventricular hypoplasia is the left ventricular inlet. In neonates, the left ventricular inlet often determines the adequacy of the left ventricle and is the most difficult component to treat surgically.
Topics: Echocardiography; Heart Defects, Congenital; Heart Ventricles; Humans; Hypoplastic Left Heart Syndrome; Infant, Newborn; Mitral Valve
PubMed: 36053101
DOI: 10.1177/21501351221114767 -
Clinical Radiology Jan 2015Pulmonary arteriovenous malformations (PAVMs) are abnormal communications between the pulmonary arteries and veins, which result in a right-to-left (R-L) shunt with... (Review)
Review
Pulmonary arteriovenous malformations (PAVMs) are abnormal communications between the pulmonary arteries and veins, which result in a right-to-left (R-L) shunt with resultant hypoxemia, the severity of which will depend upon the size and number of lesions. Most PAVMs occur in individuals with hereditary haemorrhagic telangiectasia (HHT) and are a cause of serious morbidity and mortality largely related to cerebrovascular complications secondary to paradoxical embolization. The importance of their recognition and treatment by embolization, even in the absence of symptoms, is well known. Their appearances on chest radiographs are often, but not always, characteristic and the CT appearances are diagnostic; however, there are a number of both vascular and non-vascular diseases that can cause confusion. This review serves to highlight these PAVM "mimics".
Topics: Aneurysm; Aneurysm, False; Arteriovenous Malformations; Diagnosis, Differential; Humans; Mitral Valve; Pulmonary Artery; Pulmonary Veins; Tomography, X-Ray Computed; Varicose Veins; Vascular Neoplasms
PubMed: 25443645
DOI: 10.1016/j.crad.2014.09.003 -
Seminars in Thoracic and Cardiovascular... 2023Mitral valve replacement (MVR) in the very young is an imposing clinical challenge. Early and late mortality risk is substantial, severe adverse events are common, and... (Review)
Review
Mitral valve replacement (MVR) in the very young is an imposing clinical challenge. Early and late mortality risk is substantial, severe adverse events are common, and redo mitral valve replacement is inevitable. Therapeutic options are limited. In the older infant with an annulus of 17mm or larger, mechanical MVR is associated with low risk of mortality and predictable durability. For the very young with annular hypoplasia, bovine jugular vein conduit MVR appears to offer equivalent or better early outcomes with the possibility of subsequent valve expansion, potentially prolonging the interval to redo MVR. Experience with cylinder MVR and other forms of surgeon-manufactured MVR is quite limited, and there is currently no information on late outcomes or durability.
Topics: Humans; Infant; Animals; Cattle; Mitral Valve; Heart Valve Prosthesis Implantation; Heart Valve Prosthesis; Reoperation; Catheters; Treatment Outcome; Retrospective Studies
PubMed: 36842801
DOI: 10.1053/j.pcsu.2023.01.001 -
Progress in Cardiovascular Diseases 2017Mitral valve prolapse (MVP) is a common heritable valvulopathy affecting approximately 2.4% of the population. It is the most important cause of primary mitral... (Review)
Review
Mitral valve prolapse (MVP) is a common heritable valvulopathy affecting approximately 2.4% of the population. It is the most important cause of primary mitral regurgitation (MR) requiring surgery. MVP is characterized by fibromyxomatous changes and displacement of one or both mitral leaflets into the left atrium. Echocardiography represents the primary diagnostic modality for assessment of MVP. Accurate quantitation of ventricular volumes and function for surgical planning in asymptomatic severe MR can be provided with both echocardiography and cardiac magnetic resonance. In addition, assessment of myocardial fibrosis using late gadolinium enhancement and T1 mapping allows better understanding of the impact of MVP on the myocardium. Imaging in MVP is important not only for diagnostic and prognostic purposes, but is also essential for detailed phenotyping in genetic studies. Genotype-phenotype studies in MVP pedigrees have allowed the identification of milder, non-diagnostic MVP morphologies by echocardiography. Such morphologies represent early expression of MVP in gene carriers. This review focuses on multimodality imaging and the phenotypic spectrum of MVP. Moreover, the review details the recent genetic discoveries that have increased our understanding of the pathophysiology of MVP, with clues to mechanisms and therapy.
Topics: Echocardiography; Genetic Predisposition to Disease; Hemodynamics; Humans; Magnetic Resonance Imaging; Mitral Valve; Mitral Valve Insufficiency; Mitral Valve Prolapse; Multimodal Imaging; Phenotype; Predictive Value of Tests; Prognosis; Risk Factors; Tomography, X-Ray Computed
PubMed: 29122631
DOI: 10.1016/j.pcad.2017.10.007 -
Cardiology in the Young Sep 2023Congenital mitral stenosis is a broad-spectrum pathology in which blood flow to the left ventricle is obstructed both functionally and anatomically. Hammock mitral...
Congenital mitral stenosis is a broad-spectrum pathology in which blood flow to the left ventricle is obstructed both functionally and anatomically. Hammock mitral valve, also known as anomalous mitral arcade, is a rare congenital anomaly particularly in infants and children. Hammock mitral valve may not be suitable for repair regarding the advanced dysplastic mitral valve structure. Aortopulmonary window is an unusual cardiac anomaly which is defined as a communication between the main pulmonary artery and the ascending aorta. As a result of the excessive left-to-right shunt, early intervention and surgical closure deemed mandatory to avoid development of severe pulmonary hypertension and its consequences. All patients with an aortopulmonary window necessitates prompt repair immediately. In this brief report, mitral valve replacement with a mechanical valve and repair of aortopulmonary window with a Dacron patch were performed simultaneously in a 5-month-old patient with a hammock mitral valve and accompanying aortopulmonary window.
Topics: Infant; Child; Humans; Mitral Valve; Aortopulmonary Septal Defect; Mitral Valve Stenosis; Pulmonary Artery; Mitral Valve Insufficiency
PubMed: 37092647
DOI: 10.1017/S1047951123000914 -
Journal of Cardiac Surgery Jul 2022The present study sought to study the imaging associations of left atrioventricular valve (mitral) atresia and patent aortic root in patients with isomerism of atrial...
AIM
The present study sought to study the imaging associations of left atrioventricular valve (mitral) atresia and patent aortic root in patients with isomerism of atrial appendages as detected on multidetector computed tomography (CT) angiography MATERIALS AND METHODS: The CT angiography studies performed at a tertiary medical center from January 2014 to December 2021 were retrospectively evaluated to identify patients with left atrioventricular valve atresia and patent aortic root with isometric atrial chambers. The morphology of the atrial appendage and extent of pectinate muscles was used to identify the type of isomeric atrial chambers. A comprehensive assessment of discordant arrangement of organs and the variations in venoatrial connections was done.
RESULTS
Left atrioventricular valve atresia in the presence of patent aortic root and isomeric atrial appendages was identified in eight patients (four males). The mean age at diagnosis was 7 years. Right isomerism of the atrial appendages was seen in five patients while three patients had left isomerism. Disharmonious patterns of abdominal visceral arrangement were seen in two patients. Atrial and ventricular septal defects were seen in all patients. The most common pattern of ventricular arterial connection was DORV (double outlet right ventricle) (n = 4). While five patients had normally related great arteries, three had aorta anterior to the pulmonary trunk. Right ventricular outflow obstruction was seen in seven patients. Right aortic arch with mirror image branching was seen in four patients. Coronary artery anomalies were seen in four patients.
CONCLUSIONS
The presence of left atrioventricular valve atresia and patent aortic root with isomeric atrial appendages is a rare congenital anomaly frequently associated with anomalous systemic and pulmonary drainage, conotruncal anomalies, and right ventricular outflow obstruction. Hence, comprehensive CT-based evaluation is indispensable in providing detailed anatomy and aids in presurgical planning.
Topics: Aorta; Atrial Appendage; Double Outlet Right Ventricle; Heart Defects, Congenital; Humans; Male; Multidetector Computed Tomography; Retrospective Studies; Ventricular Outflow Obstruction
PubMed: 35396763
DOI: 10.1111/jocs.16468 -
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 -
Journal of Cardiac Surgery Jun 2022To study the imaging characteristics and the associations of left atrioventricular valve atresia with patent aortic root and lateralized atrial chambers on multidetector...
AIM
To study the imaging characteristics and the associations of left atrioventricular valve atresia with patent aortic root and lateralized atrial chambers on multidetector computed tomography (CT) angiography.
MATERIALS AND METHODS
We retrospectively evaluated all the CT angiography studies performed for the evaluation of various congenital heart diseases at our center from January 2014 to December 2021. Cases with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers were identified. The types of cardiovascular involvement as identifiable on CT angiography were studied and compiled.
RESULTS
A total of 38 patients with left atrioventricular valve atresia and patent aortic root were included (median age: 5 months, males: 31). A total of 89.5% had usual atrial arrangement and 10.5% had mirror imagery of atria. Normal drainage of right and left superior caval veins and inferior caval vein into systemic venous atrium was seen in 77.8%, 61.5%, and 86.6%, respectively. Anomalous pulmonary venous drainage was seen in 3 (7.9%) patients. Atrial and ventricular septal defects were the most common associations. Imperforate left atrioventricular membrane was seen in 9 (23.7%) patients and absent left atrioventricular connection in 27 (71.1%). Discordant right atrioventricular connection was seen in 5 (13.1%) patients. While a double outlet right ventricle (76.3%) was the most common ventriculoarterial connection, discordant ventriculo-arterial connection (transposition) in 4 (10.5%). Most patients had good sized pulmonary arteries. Patent arterial duct and right aortic arch were seen in 29% and 21%, respectively. Aberrant right subclavian artery was the most common arch vessel anomaly. Coronary anomalies were seen in 7 patients (19%); single coronary artery being the most common.
CONCLUSIONS
Majority of the patients with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers have usual atrial arrangement, normal right atrioventricular connections and a double outlet configuration of the right ventricle. Absent left atrioventricular connection is a more common than imperforate left atrioventricular valve membrane. Atrial and ventricular septal defects are the most common associations. Comprehensive CT based evaluation of vascular and nonvascular thoracic structures is imperative before surgery or interventions in patients with mitral atresia.
Topics: Heart Atria; Heart Defects, Congenital; Heart Septal Defects, Ventricular; Heart Ventricles; Humans; Infant; Male; Multidetector Computed Tomography; Retrospective Studies; Situs Inversus; Vena Cava, Superior
PubMed: 35301758
DOI: 10.1111/jocs.16418 -
Swiss Medical Weekly Nov 2018BACKGROUND The term “predisposition” is used as an indication of antimicrobial prophylaxis to prevent infective endocarditis and as a criterion for diagnosing... (Review)
Review
BACKGROUND The term “predisposition” is used as an indication of antimicrobial prophylaxis to prevent infective endocarditis and as a criterion for diagnosing infective endocarditis according to the modified Duke criteria. The criterion for diagnosing infective endocarditis in native valves is not well defined. OBJECTIVES To identify conditions that increase the risk for infective endocarditis in native valves, for the diagnosis of infective endocarditis according to the modified Duke criteria. In parallel, we compared the results with the year of patient inclusion for each study and echocardiographic techniques. RESULTS Our systematic review included 207 studies published from January 1970 to August 2015. Studies that focused on mitral valve prolapse (112 studies), prior infective endocarditis (96) and bicuspid aortic valve (78) provided the most data. However, only six (5.3%), three (3.1%) and one (1.3%) of these studies, respectively, used analytical statistical methods. Three (2.7%), two (2.1%) and one (1.3%), respectively, were graded as good quality studies. Odds ratios (ORs) for developing infective endocarditis were 3.5–8.2 for mitral valve prolapse, and 2.2 and 2.8 for prior infective endocarditis. The hazard ratio for developing infective endocarditis was 6.3 for bicuspid aortic valve. The mean prevalence proportion of infective endocarditis in patients with these three heart conditions were 8.5% (mitral valve prolapse), 8.3% (prior infective endocarditis) and 8.8% (bicuspid aortic valve). The proportions of publications prior to the publication of the modified Duke criteria were 81.8, 75.6 and 74%, respectively. Evolution of the imaging method and echocardiographic technique was estimated to be considerable for mitral valve prolapse. The literature review on aortic valve stenosis (46 studies), mitral valve insufficiency (41) and aortic valve insufficiency (39) provided two analytical studies for aortic stenosis. One study was graded as good quality and reported a hazard ratio 4.9. The mean prevalence of these heart conditions in patients with infective endocarditis were 7.3, 19.9 and 10.2%, respectively. The proportions of publications prior to the publication of the modified Duke criteria were 78, 75.6 and 79.5%, respectively. The evolution of both the echocardiographic technique and the categorisation of valve disease severity was considerable for all three entities. CONCLUSIONS The evidence for native valve heart conditions predisposing to infective endocarditis is mainly based on studies with only descriptive statistics published prior to the release of the modified Duke criteria. Mitral valve prolapse, prior infective endocarditis and bicuspid aortic valve are frequently cited as predisposing heart conditions for infective endocarditis. The evolution in echocardiographic techniques over the past decades and its influence on diagnosis was considerable for mitral valve prolapse, aortic stenosis, mitral insufficiency and aortic insufficiency.
Topics: Aortic Valve; Aortic Valve Insufficiency; Bicuspid Aortic Valve Disease; Echocardiography; Endocarditis; Guidelines as Topic; Heart Valve Diseases; Humans; Mitral Valve Prolapse; Risk Factors
PubMed: 30440064
DOI: 10.4414/smw.2018.14675