Truncus

PURPOSE: This report reviews our experience with right lower sleeve lobectomy and describes our technique and approach to perioperative patient management. METHODS: We retrospectively reviewed 11 patients who underwent right lower sleeve lobectomy for lung cancer. Surgical techniques and perioperative management were also investigated. RESULTS: Bronchoplasty was performed using 4-0 absorbable monofilament sutures. The deepest portion was anastomosed using continuous sutures; interrupted sutures were used for the more superficial portions...

22q11.2 deletion syndrome (22q11.2DS) shows significant clinical heterogeneity. This study aimed to explore the association between clinical heterogeneity in 22q11.2DS and the parental origin of the deletion. The parental origin of the deletion was determined for 61 individuals with 22q11.2DS by genotyping DNA microsatellite markers and single-nucleotide polymorphisms (SNPs). Among the 61 individuals, 29 (47.5%) had a maternal origin of the deletion, and 32 (52.5%) a paternal origin. Comparison of the frequency of the main clinical features between individuals with deletions of maternal or paternal origin showed no statistically significant difference...

Dysphagia lusoria is a rare pediatric condition caused by extrinsic compression of the esophagus by an abnormal subclavian artery. The most common congenital abnormality in aortic arch development is an aberrant right subclavian artery. The retroesophageal right subclavian artery is typically symptomatic in 10-33% of cases. The patient, an 8-month-old girl with a history of early dysphagia and stridor, was diagnosed with an abnormal right subclavian artery. She was admitted to the pneumology service multiple times due to stridor, vomiting, and failure to thrive...

No abstract text is available yet for this article.

Partial heart transplantation is the first clinically successful approach to deliver growing heart valve implants. To date, 13 clinical partial heart transplants have been performed. However, turning partial heart transplantation into a routine procedure that is available to all children who would benefit from growing heart valve implants poses formidable logistical challenges. Firstly, a supply for partial heart transplant donor grafts needs to be developed. This challenge is complicated by the scarcity of donor organs...

Background: Truncus arteriosus is a rare congenital heart defect resulting from the failure of the truncus arteriosus to divide during fetal development. It leads to a single outflow tract from the heart and, if left untreated, can be fatal. Late presentation and repair can also increase the risk of pulmonary hypertensive crises, which can lead to morbidity and mortality after repair. Methods: We performed a retrospective study examining outcomes of late-presenting patients who were repaired for this anomaly at our institution...

CONTEXT: Osteopathic treatments regulate the neurovegetative system through joint mobilizations and manipulations, and myofascial and craniosacral techniques. Despite the growing body of research, the precise impact of osteopathic medicine on the autonomic nervous system (ANS) is not yet fully elucidated. As to Kuchera's techniques, the stimulation of the sympathetic trunk and prevertebral ganglia contributed to harmonization of the sympathetic activity. However, potential relationships between the harmonization of the sympathetic nervous system (SNS) and the hypothalamic-pituitary-adrenal (HPA) axis largely remain uncertain and warrant further exploration...

Although the molecular mechanisms underlying congenital heart disease (CHD) remain poorly understood, recent advances in genetic analysis have facilitated the exploration of causative genes for CHD. We reported that the pathogenic variant c.1617del of TMEM260, which encodes a transmembrane protein, is highly associated with CHD, specifically persistent truncus arteriosus (PTA), the most severe cardiac outflow tract (OFT) defect. Using whole-exome sequencing, the c.1617del variant was identified in two siblings with PTA in a Japanese family and in three of the 26 DNAs obtained from Japanese individuals with PTA...

Truncus Arteriosus (TA) is a congenital heart disease characterized by a single common blood vessel emerging from the right and left ventricles instead of the main pulmonary artery and aorta. TA accounts for 4% of all critical congenital heart diseases. The most common cause of TA is 22q11.2 deletion syndrome, accounting for 12-35% of all TA cases. However, no major causes of TA other than 22q11.2 deletion have been reported. We performed whole-genome sequencing of 11 Japanese patients having TA without 22q11...

BACKGROUND: Neurogenic Thoracic Outlet Syndrome (nTOS) describes a complex of symptoms caused by the compression of neural structures at the upper thoracic outlet. Typical symptoms include pain, numbness and motor weakness of the affected extremity. The incidence of nTOS is 2-3 per 100,000 and is highest between the ages of 25 and 40. There are only a few studies evaluating the surgical outcomes of nTOS in adolescent patients. In particular, there is a lack of long-term data. MATERIALS AND METHODS: In a retrospective study of nTOS cases receiving surgical treatment in our clinic between 2002 and 2021, eight patients between 15 and 18 years of age were included...

BACKGROUND: We provide updated crude and adjusted prevalence estimates of major birth defects in the United States for the period 2016-2020. METHODS: Data were collected from 13 US population-based surveillance programs that used active or a combination of active and passive case ascertainment methods to collect all birth outcomes. These data were used to calculate pooled prevalence estimates and national prevalence estimates adjusted for maternal race/ethnicity for all conditions, and maternal age for trisomies and gastroschisis...

Truncal valve regurgitation and interrupted aortic arch increase the need for reintervention and mortality rates in neonates undergoing truncus arteriosus repair. This featured case report presents a surgical video demonstrating the tricuspidization of a quadricuspid truncal valve during repair of truncus arteriosus and interrupted aortic arch in a 9-day-old girl. The patient had a quadricuspid and dysplastic truncal valve with moderate to severe regurgitation. Tricuspidization was successfully performed. The Institutional Review Board committee of Children's National approved the study protocol and publication of data (RB Number: Pro00015566, Initial Approval: 7/1/2021)...

BACKGROUND: Persistent hiccups lasting more than 48 hours are rare and have numerous possible causes that require further investigation. CASE PRESENTATION: We present a man in his seventies who was admitted to hospital after 11 days of hiccups. The hiccups were preceded by abdominal pain that spontaneously receded after a few hours. At admission he had no abdominal pain during examination, but a CT scan later suggested that the cause was a perforated cholecystitis with an intra-abdominal abscess...

Dysphagia is a common condition in clinical practice; however, an unusual type of dysphagia due to compression of the esophagus by an abnormal right subclavian artery may be discovered in a rare subset of patients. The prognosis and treatment will depend on the severity of the symptoms and the compromise of surrounding structures. We present the case of an 18-year-old female who presented with gradually progressive dysphagia. At first, it was treated as gastroesophageal reflux disease; nonetheless, the dysphagia became severe, and after a thorough evaluation, an aberrant right subclavian artery that compressed the esophagus was discovered along with a truncus bicaroticus...

IMPORTANCE: The treatment of neonates with irreparable heart valve dysfunction remains an unsolved problem because there are no heart valve implants that grow. Therefore, neonates with heart valve implants are committed to recurrent implant exchanges until an adult-sized valve can fit. OBJECTIVE: To deliver the first heart valve implant that grows. DESIGN, SETTING, AND PARTICIPANTS: Case report from a pediatric referral center, with follow-up for more than 1 year...

The quadratus lumborum (QL) block is an ultrasound-guided locoregional anesthesia technique which aims to provide analgesia to the abdomen. The main objective of this study was to assess a modified ultrasound-guided dorsal QL block in cat cadavers. For this purpose, a volume of 0.4 mL kg-1 of a mixture of iopromide and methylene blue was administered between the psoas minor muscle and the vertebral body (VB) of the first lumbar vertebra, and its distribution was assessed in thirteen cat cadavers. We hypothesized that this injection point would be feasible, offering a more cranial distribution of the injectate and a more consistent staining of the truncus sympathicus...

BACKGROUND: The crocodilian heart is unique among reptiles with its four-chambered structure and complete intracardiac separation of pulmonary and systemic blood flows and pressures. Crocodiles have retained two aortic arches; one from each ventricle, that communicate via Foramen of Panizza, immediately distally from the aortic valves. Moreover, crocodiles can regulate vascular resistance in the pulmonary portion of the right ventricular outflow tract (RVOT). These unique features allow for a complex regulation of shunting between the pulmonary and systemic circulations...

No abstract text is available yet for this article.

The DGCR8 gene, encoding a critical miRNA processing protein, maps within the hemizygous region in patients with 22q11.2 deletion syndrome. Most patients have malformations of the cardiac outflow tract that is derived in part from the anterior second heart field (aSHF) mesoderm. To understand the function of Dgcr8 in the aSHF, we inactivated it in mice using Mef2c-AHF-Cre. Inactivation resulted in a fully penetrant persistent truncus arteriosus and a hypoplastic right ventricle leading to lethality by E14.5...

Persistent pneumothorax is a life-threatening complication that can occur after congenital cardiac surgery. Traditional treatment such as chest tube drainage may not be effective in managing this condition. This study presents a new minimally invasive method for treating persistent pneumothorax using platelet-rich plasma-fibrin glue (PRP-FG). The method has been successful in treating postoperative chylothorax in previous studies, and its use has decreased morbidity, mortality, and hospital stay in chylothorax patients...