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JACC. Advances Apr 2024Neoaortic root dilatation (NeoARD) and neoaortic regurgitation (NeoAR) are common sequelae following the arterial switch operation (ASO) for transposition of the great...
BACKGROUND
Neoaortic root dilatation (NeoARD) and neoaortic regurgitation (NeoAR) are common sequelae following the arterial switch operation (ASO) for transposition of the great arteries.
OBJECTIVES
The authors aimed to estimate the cumulative incidence of NeoAR, assess whether larger neoaortic root dimensions were associated with NeoAR, and evaluate factors associated with the development of NeoAR during long-term follow-up.
METHODS
Electronic databases were systematically searched for articles that assessed NeoAR and NeoARD after ASO, published before November 2022. The primary outcome was NeoAR, classified based on severity categories (trace, mild, moderate, and severe). Cumulative incidence was estimated from Kaplan-Meier curves, neoaortic root dimensions using Z-scores, and risk factors were evaluated using random-effects meta-analysis.
RESULTS
Thirty publications, comprising a total of 6,169 patients, were included in this review. Pooled estimated cumulative incidence of ≥mild NeoAR and ≥moderate NeoAR at 30-year follow-up were 67.5% and 21.4%, respectively. At last follow-up, neoaortic Z-scores were larger at the annulus (mean difference [MD]: 1.17, 95% CI: 0.52-1.82, < 0.001; MD: 1.38, 95% CI: 0.46-2.30, = 0.003) and root (MD: 1.83, 95% CI: 1.16-2.49, < 0.001; MD: 1.84, 95% CI: 1.07-2.60, < 0.001) in patients with ≥mild and ≥moderate NeoAR, respectively, compared to those without NeoAR. Risk factors for the development of any NeoAR included prior pulmonary artery banding, presence of a ventricular septal defect, aorto-pulmonary mismatch, a bicuspid pulmonary valve, and NeoAR at discharge.
CONCLUSIONS
The risks of NeoARD and NeoAR increase over time following ASO surgery. Identified risk factors for NeoAR may alert the clinician that closer follow-up is needed. (Risk factors for neoaortic valve regurgitation after arterial switch operation: a meta-analysis; CRD42022373214).
PubMed: 38939665
DOI: 10.1016/j.jacadv.2024.100878 -
Journal of Clinical Medicine Jun 2024An aortopulmonary septal defect or aortopulmonary window (APW) is a rare cardiovascular anomaly with direct communication between the ascending aorta and the main... (Review)
Review
An aortopulmonary septal defect or aortopulmonary window (APW) is a rare cardiovascular anomaly with direct communication between the ascending aorta and the main pulmonary artery leading to a left-to-right shunt. It is accompanied by other cardiovascular anomalies in approximately half of patients. In order to avoid irreversible sequelae, interventional or surgical treatment should be performed as soon as possible. Cardiovascular CT, as a fast, non-invasive technique with excellent spatial resolution, has an increasing role in the evaluation of patients with APW, enabling precise and detailed planning of surgical treatment of APW and associated anomalies if present. This article aims to review the anatomical and clinical features of aortopulmonary septal defect with special emphasis on its detection and characterization by a CT examination.
PubMed: 38930042
DOI: 10.3390/jcm13123513 -
Advances in Experimental Medicine and... 2024In normal cardiovascular development in birds and mammals, the outflow tract of the heart is divided into two distinct channels to separate the oxygenated systemic blood... (Review)
Review
In normal cardiovascular development in birds and mammals, the outflow tract of the heart is divided into two distinct channels to separate the oxygenated systemic blood flow from the deoxygenated pulmonary circulation. When the process of outflow tract septation fails, a single common outflow vessel persists resulting in a serious clinical condition known as persistent truncus arteriosus or common arterial trunk. In this chapter, we will review molecular pathways and the cells that are known to play a role in the formation and development of the outflow tract and how genetic manipulation of these pathways in animal models can result in common arterial trunk.
Topics: Animals; Humans; Disease Models, Animal; Signal Transduction; Truncus Arteriosus; Truncus Arteriosus, Persistent
PubMed: 38884754
DOI: 10.1007/978-3-031-44087-8_52 -
Advances in Experimental Medicine and... 2024Integrated human genetics and molecular/developmental biology studies have revealed that truncus arteriosus is highly associated with 22q11.2 deletion syndrome. Other... (Review)
Review
Integrated human genetics and molecular/developmental biology studies have revealed that truncus arteriosus is highly associated with 22q11.2 deletion syndrome. Other congenital malformation syndromes and variants in genes encoding TBX, GATA, and NKX transcription factors and some signaling proteins have also been reported as its etiology.
Topics: Humans; Truncus Arteriosus, Persistent; Transcription Factors; Truncus Arteriosus; DiGeorge Syndrome; T-Box Domain Proteins; Genetic Predisposition to Disease
PubMed: 38884753
DOI: 10.1007/978-3-031-44087-8_51 -
Advances in Experimental Medicine and... 2024Truncus arteriosus (TA, also known as common arterial trunk) consists of only one great artery ("the truncus") with a semilunar valve (truncus valve) arising from the...
Truncus arteriosus (TA, also known as common arterial trunk) consists of only one great artery ("the truncus") with a semilunar valve (truncus valve) arising from the heart and an additional ventricular septal defect and (Fig. 50.1). This great artery is positioned above the ventricular septal defect and gives rise to the coronary arteries, the pulmonary arteries, and the aortic arch. Historically, TA has been classified by Collet and Edwards in three types, where in type I there was a common pulmonary artery truncus, in type II the left and right PA arise separately but close to each other, in type III both PA arise independently; in addition, there was a type IV that was later characterized as pulmonary atresia with VSD and major aortopulmonary collateral arteries arising from the descending aorta.
Topics: Humans; Pulmonary Artery; Pulmonary Atresia; Truncus Arteriosus; Truncus Arteriosus, Persistent
PubMed: 38884752
DOI: 10.1007/978-3-031-44087-8_50 -
Advances in Experimental Medicine and... 2024Tetralogy of Fallot (TOF) is the most common cyanotic heart defect. TOF consists of the combination of four anomalies (Fig. 35.1): (1) a large malalignment ventricular... (Review)
Review
Tetralogy of Fallot (TOF) is the most common cyanotic heart defect. TOF consists of the combination of four anomalies (Fig. 35.1): (1) a large malalignment ventricular septal defect, (2) an obstruction of the right ventricular outflow tract (usually infundibular and valvular pulmonary stenosis with a small pulmonary valve annulus and supravalvular stenosis, (3) an aorta that "overrides" the ventricular septal defect, and (4) right ventricular hypertrophy. TOF represents 4-8% of congenital heart defects. Specific variations of TOF include all forms of pulmonary atresia with VSD and absent pulmonary valve syndrome. In addition, the left and right main pulmonary arteries may be stenotic or hypoplastic. In these cases, there may be major aortopulmonary collateral arteries (MAPCAs) which are vessels arising from the aorta or the subclavian arteries that supply segments of the pulmonary arterial tree. Additional variations include an ASD (Pentalogy of Fallot), a right aortic arch, and coronary abnormalities.
Topics: Tetralogy of Fallot; Humans; Double Outlet Right Ventricle
PubMed: 38884737
DOI: 10.1007/978-3-031-44087-8_35 -
The Journal of Invasive Cardiology Jun 2024
PubMed: 38875169
DOI: 10.25270/jic/24.00169 -
The American Journal of Case Reports Jun 2024BACKGROUND Persistent truncus arteriosus is a rare congenital cyanotic heart defect characterized by a single ventricular outflow tract. Without surgical intervention,...
BACKGROUND Persistent truncus arteriosus is a rare congenital cyanotic heart defect characterized by a single ventricular outflow tract. Without surgical intervention, it has a poor prognosis in infancy. Here, we report an adult female patient with uncorrected truncus arteriosus type I, who presented with acute-onset abdominal pain due to torsion of a small bowel gastrointestinal stromal tumor (GIST). CASE REPORT A 41-year-old woman came to our Emergency Department with acute-onset lower abdominal pain for 2 days. Congenital heart disease, truncus arteriosus, had been diagnosed at birth, and there had been no surgical intervention. Abdominal computed tomography revealed a 10×9×12-cm mixed-density mass in the pelvic capacity. Transthoracic echocardiography revealed a 33-mm ventricular septal defect. The ascending aorta originated mainly from the right ventricle, and the pulmonary artery originated from the beginning of the aorta (type I truncus arteriosus, according to Collett and Edwards classification). After a quick and detailed preoperative workup, the patient underwent tumor resection by open surgery with general anesthesia. CONCLUSIONS This is the first case to report emergency surgery for a patient with uncorrected persistent truncus arteriosus due to torsion of a small bowel GIST. A multidisciplinary team with deep understanding of the disease entity was crucial. By considering the fixed hemodynamic and respiratory physiology, overtreatment and unrealistic goals were avoided. Eventually, the patient was discharged after being hospitalized for 2 weeks.
Topics: Humans; Female; Adult; Gastrointestinal Stromal Tumors; Torsion Abnormality; Truncus Arteriosus, Persistent; Intestine, Small
PubMed: 38833428
DOI: 10.12659/AJCR.943604 -
Sultan Qaboos University Medical Journal May 2024
Topics: Humans; Aortopulmonary Septal Defect; Male; Female; Heart Defects, Congenital; Echocardiography
PubMed: 38828251
DOI: 10.18295/squmj.3.2024.026