-
Children (Basel, Switzerland) May 2021In this paper, the author enumerates cardiac defects with a functionally single ventricle, summarizes single ventricle physiology, presents a summary of management... (Review)
Review
In this paper, the author enumerates cardiac defects with a functionally single ventricle, summarizes single ventricle physiology, presents a summary of management strategies to address the single ventricle defects, goes over the steps of staged total cavo-pulmonary connection, cites the prevalence of inter-stage mortality, names the causes of inter-stage mortality, discusses strategies to address the inter-stage mortality, reviews post-Fontan issues, and introduces alternative approaches to Fontan circulation.
PubMed: 34073809
DOI: 10.3390/children8060441 -
World Journal of Cardiology Feb 2019The incidence of heart valve disease increases significantly with age. Degenerative abnormalities associated with severe aortic stenosis and mitral and tricuspid... (Review)
Review
The incidence of heart valve disease increases significantly with age. Degenerative abnormalities associated with severe aortic stenosis and mitral and tricuspid regurgitation are found in not less than 10% of the population aged ≥ 75 years. Surgical treatment has been considered for years to be the treatment of choice. However, it was not uncommonly associated with high perioperative morbidity and mortality due to frequent comorbidities and overall frailty conditions of these patients. Conventional risk scores such as Society of Thoracic Surgeons and European System for Cardiac Operative Risk Evaluation may underestimate the risk of surgery in elderly patients, leading to inappropriate surgical indication. On the other hand, at least 30% of patients with severe conditions are left untreated due to prohibitive surgical risk. Interventional procedures, which are in continuous development, may be actually considered for high risk patients and, as recent results suggest, also for intermediate risk patients.
PubMed: 30820277
DOI: 10.4330/wjc.v11.i2.71 -
Internal Medicine (Tokyo, Japan) May 2021
Topics: Abnormalities, Multiple; Heart Defects, Congenital; Heart Septal Defects, Atrial; Humans; Lower Extremity Deformities, Congenital; Upper Extremity Deformities, Congenital
PubMed: 33281169
DOI: 10.2169/internalmedicine.6390-20 -
Frontiers in Cardiovascular Medicine 2023Valvular heart disease (VHD) has been a significant health problem, particularly in developed countries, in relation to the aging population. Recent developments in the... (Review)
Review
Valvular heart disease (VHD) has been a significant health problem, particularly in developed countries, in relation to the aging population. Recent developments in the management of VHD require a more accurate assessment of disease severity to determine the need for transcatheter interventions or open heart surgery. Stress echocardiography is a crucial imaging modality for identifying the underlying pathology of VHD. Optimal administration of exercise or intravenous drugs may reveal hemodynamic abnormalities under stress without posing an invasive risk. Therefore, the implementation of stress echocardiography is recommended for determining interventional indications and risk stratification in mitral regurgitation and aortic stenosis. In addition, recent evidence has accumulated regarding the usefulness of stress echocardiography in various conditions including mitral stenosis, aortic regurgitation, and post-interventional VHD. Here, we summarize the current evidence and future perspectives on stress echocardiography in VHD.
PubMed: 38162127
DOI: 10.3389/fcvm.2023.1233924 -
Journal of Cardiovascular Development... Mar 2023As yet, there is no agreed definition for the so-called "hypoplastic left heart syndrome". Even its origin remains contentious. Noonan and Nadas, who as far as we can... (Review)
Review
As yet, there is no agreed definition for the so-called "hypoplastic left heart syndrome". Even its origin remains contentious. Noonan and Nadas, who as far as we can establish first grouped together patients as belonging to a "syndrome" in 1958, suggested that Lev had named the entity. Lev, however, when writing in 1952, had described "hypoplasia of the aortic outflow tract complex". In his initial description, as with Noonan and Nadas, he included cases with ventricular septal defects. In a subsequent account, he suggested that only those with an intact ventricular septum be included within the syndrome. There is much to commend this later approach. When assessed on the basis of the integrity of the ventricular septum, the hearts to be included can be interpreted as showing an acquired disease of fetal life. Recognition of this fact is important to those seeking to establish the genetic background of left ventricular hypoplasia. Flow is also of importance, with septal integrity then influencing its effect on the structure of the hypoplastic ventricle. In our review, we summarise the evidence supporting the notion that an intact ventricular septum should now be part of the definition of the hypoplastic left heart syndrome.
PubMed: 37103012
DOI: 10.3390/jcdd10040133 -
The Journal of Thoracic and... Aug 2018
Topics: Aortic Valve; Bicuspid Aortic Valve Disease; Heart Valve Diseases; Humans; Mitral Valve; Tricuspid Valve
PubMed: 29666012
DOI: 10.1016/j.jtcvs.2018.03.069 -
The Turkish Journal of Pediatrics 2017Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232. Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare... (Review)
Review
Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232. Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare genetic disorder involving multiple systems including the circulatory system. However, the etiologies of the associated congenital heart defects in WS patients have not been sufficiently elucidated and represent therapeutic challenges. The typical congenital heart defects in WS were supravalvar aortic stenosis, pulmonary stenosis (both valvular and peripheral), aortic coarctation and mitral valvar prolapse. The atypical cardiovascular anomalies include tetralogy of Fallot, atrial septal defects, aortic and mitral valvular insufficiencies, bicuspid aortic valves, ventricular septal defects, total anomalous pulmonary venous return, double chambered right ventricle, Ebstein anomaly and arterial anomalies. Deletion of the elastin gene on chromosome 7q11.23 leads to deficiency or abnormal deposition of elastin during cardiovascular development, thereby leading to widespread cardiovascular abnormalities in WS. In this article, the distribution, treatment and surgical outcomes of typical and atypical cardiac defects in WS are discussed.
Topics: Child; Child, Preschool; Heart Defects, Congenital; Humans; Williams Syndrome
PubMed: 29376566
DOI: 10.24953/turkjped.2017.03.001 -
Methodist DeBakey Cardiovascular Journal 2021An 89-year-old female with a history of hypertension presented to the hospital with symptoms of fatigue. Her electrocardiogram (ECG) showed high-grade atrioventricular...
An 89-year-old female with a history of hypertension presented to the hospital with symptoms of fatigue. Her electrocardiogram (ECG) showed high-grade atrioventricular (AV) block, so a transthoracic echocardiogram was obtained to assess for structural heart abnormalities (Figure 1). Color Doppler showed mild mitral regurgitation (MR) extending into diastole. Temporal interrogation of the MR jet using continuous wave Doppler confirmed the diastolic component. Diastolic MR is generally described in the setting of AV dissociation. In patients with high-degree AV block and underlying sinus rhythm, the prolonged diastolic time with accompanying superimposed left atrial (LA) contractions will lead to a significant elevation in left ventricular end-diastolic pressure (LVEDP), creating a reverse gradient favoring flow from the left ventricle back into the LA during diastole. Diastolic MR also can occur with substantial elevations in LVEDP in restrictive cardiomyopathies and acute severe aortic regurgitation.
Topics: Aged, 80 and over; Aortic Valve Insufficiency; Atrioventricular Block; Diastole; Echocardiography; Female; Humans; Mitral Valve Insufficiency
PubMed: 34992727
DOI: 10.14797/mdcvj.1054