Truncal valve leaflet reconstruction with autologous pericardium in a neonate

A boy diagnosed with persistent truncus arteriosus and severe truncal valve regurgitation had uncontrollable heart failure. Thus, truncal valve repair, ventricular septal defect closure, and right ventricular outflow tract reconstruction were performed on the second day of life. We report about a neonate with severe truncal valve regurgitation who was successfully treated with autologous pericardial truncal valve leaflet reconstruction.

Fetal Blood Flow and Genetic Mutations in Conotruncal Congenital Heart Disease

In congenital heart disease, the presence of structural defects affects blood flow in the heart and circulation. However, because the fetal circulation bypasses the lungs, fetuses with cyanotic heart defects can survive in utero but need prompt intervention to survive after birth. Tetralogy of Fallot and persistent truncus arteriosus are two of the most significant conotruncal heart defects. In both defects, blood access to the lungs is restricted or non-existent, and babies with these critical conditions need intervention right after birth. While there are known genetic mutations that lead to these critical heart defects, early perturbations in blood flow can independently lead to critical heart defects. In this paper, we start by comparing the fetal circulation with the neonatal and adult circulation, and reviewing how altered fetal blood flow can be used as a diagnostic tool to plan interventions. We then look at known factors that lead to tetralogy of Fallot and persistent truncus arteriosus: namely early perturbations in blood flow and mutations within VEGF-related pathways. The interplay between physical and genetic factors means that any one alteration can cause significant disruptions during development and underscore our need to better understand the effects of both blood flow and flow-responsive genes.

Low-Dose Dual-Source Computed Tomography for Evaluating Persistent Truncus Arteriosus Associated with Cardiovascular Anomalies: Comparison with Transthoracic Echocardiography

BackgroundTo assess the morphological features of persistent truncus arteriosus (PTA) on low-dose dual-source computed tomography (DSCT) and compare its diagnostic value for associated cardiovascular anomalies with that of transthoracic echocardiography (TTE).Methods:Twenty-four PTA patients were enrolled in this retrospective study. The types of PTA, diameters of the truncus artery (TA), main pulmonary artery (MPA), right pulmonary artery (RPA), left pulmonary artery (LPA), and ventricular septal defect (VSD) on DSCT were recorded. Besides, all associated cardiovascular abnormalities were assessed. The diagnostic performance of DSCT and TTE for associated anomalies were compared. The effective doses of DSCT were calculated.Results:Four types were found: type A1(n=13/24, 54.17%), type A2(n=7/24, 29.16%), type A3（n=3/24, 12.50%）and type A4(n=1/24,4.17%). The mean diameter of VSD, TA, MPA, RPA, and LPA was 1.47±0.56cm, 3.92±1.56cm, 2.27±1.65cm, 1.48±0.74cm and 1.38±0.66cm, respectively. 78 associated cardiovascular anomalies were confirmed. The most common associated abnormalities were VSD (100%), right-sided aortic arch (33.33%) and aortopulmonary collateral vessels (29.17%). Although TTE was better at diagnosing intracardiac anomalies (accuracy:99.17% vs. 95%; sensitivity: 97.06% vs. 88.24%; specificity: 100% vs. 97.67%), DSCT had an advantage in diagnosing the associated cardiovascular abnormalities (accuracy: 98.61% vs. 96.07%; sensitivity: 94.87% vs. 82.05%; specificity: 99.44% vs. 99.15%). The estimated mean effective doses was 0.98±0.37mSv (＜1mSv). Conclusions:Low-dose DSCT could accurately confirm the morphological features of PTA. Compared to TTE, low-dose DSCT is a better diagnostic tool for associated cardiovascular abnormalities. Combining with TTE will be beneficial to provide more accurate information for clinical interventions.

Truncus Arteriosus Type 1: A Prenatal Diagnosis, Case Report, and Literature Review

Common arterial trunk (persistent truncus arteriosus) is a rare, congenital heart anomaly and characterized by Ventricular Septal Defect (VSD), single truncal valve, and a common ventricular outflow tract. We reported a case of truncus arteriosus type 1 in the fetus of a 28 years-old G2-P1-L1 pregnant female at 24 weeks of gestational age with large sub truncul VSD, truncal overriding, and main pulmonary artery bifurcation to the right and left pulmonary arteries.

Late follow-up of Persistent Truncus Arteriosus, after one-stage Repair, with Right Ventricular Remodeling.

We report long-term outcome after one-stage, surgical repair, in a two months-old girl with persistent truncus arteriosus type I, II. The operation was carried out with the remodeling of the right ventricle, using a swine bicuspid pulmonary prosthesis. Twenty-six years later, the patient is in excellent clinical condition, CF I (NYHA), with normal peripheral oxygen saturation. Recent invasive and not invasive imaging show: absence of intracardiac shunt and growing of the right ventricle outlet tract and discrete double lesion of the pulmonary valve. The pulmonary flow directed uniformly for both lungs. In selected cases, the long-term prognosis of patients with persistent truncus arteriosus, undergoing early surgical repair, avoiding the use of valved conduit, makes for an excellent evolution, without new interventions. Endovascular procedures, now well standardized, for the implantation of a pulmonary valve stent, through a catheter, will allow an effective approach, in the presence of late obstructions, in patients who have undergone right ventricular remodeling, without the use of valved conduits.

A case of computed tomography diagnosis of truncus arteriosus type IV

ABSTRACT Persistent truncus arteriosus is a rare congenital heart disease with four variants, and the last being the rarest. The prognosis without surgical intervention is poor. In such cases, an echocardiography is not sufficient hence computed tomography (CT) imaging is required. We report a 26-year-old female with difficulty in breathing since childhood with cyanosis. Her echocardiography showed a ventricular septal defect (VSD) and the CT showed a single arterial trunk overriding the interventricular septum with a VSD, and the descending aorta giving rise to the pulmonary arteries suggestive of pseudo truncus, known as truncus arteriosus type IV.

Silent Type Ii Truncus Arteriosus Diagnosed at the Age of 18-years in a Ugandan Male

Background: Persistent truncus arteriosus (TA) is a rare congenital heart condition with reported incidence of 1.2% among all congenital heart malformations. Majority of patients with TA die early in childhood due to complications such as heart failure. Case presentation: An 18-year-old adult with unsuspected congenital heart disease since birth, not detected in childhood. Six months prior to his presentation, he complained of easy fatigability and paroxysmal nocturnal dyspnea. Chest radiography demonstrated normal heart size and electrocardiogram showed biventricular hypertrophy. Echocardiograph showed truncus arteriosus type II with large outlet ventricular septal defect. The patient was put on medical therapy including bed rest, fluid restriction and then surgical correction was done. The patient’s symptom improved after the surgical correction.Conclusion: TA is a rare, serious congenital heart defect that can be detected in prenatal period by routine ultrasound screening. It is also very rare for TA to be silent and reach adulthood without clinical manifestation and a diagnosis.