complete AV canal

Assessment of fetal ventricular function is mostly subjective, and currently, for the objective assessment left ventricular shortening fraction is obtained. However, this by itself is not very reliable. Hence, more tools that can provide an objective assessment are needed to increase the confidence of functional assessment. Speckle tracking imaging can provide one such tool. In this study we sought to establish the normative value of global longitudinal and circumferential strain for our fetal patients and for two major forms of congenital heart diseases, namely atrioventricular canal defects (AVC) and uncorrected dextro-transposition of the great arteries (dTGA) to act as a benchmark...

To estimate if there is an association between partial AVSD with chromosomal abnormalities, cardiac and extracardiac malformations, and to report the outcomes of prenatally diagnosed AVSD in a large, contemporary cohort. This is a retrospective cohort study of 190 prenatally diagnosed fetal AVSD between 2014 and 2023. Type of AVSD (complete vs partial), additional cardiac findings, extracardiac findings, presence of a heterotaxy, results of prenatal karyotype, and pregnancy outcomes were documented and analyzed...

OBJECTIVES: Parachute left atrioventricular valve (LAVV) complicates atrioventricular septal defect (AVSD) repair. We evaluate outcomes of AVSD patients with parachute LAVV and identify risk factors for adverse outcomes. METHODS: We evaluated all patients undergoing repair of AVSD with parachute LAVV from 2012-2021. The primary outcome was a composite of time-to death, LAVV reintervention, and development of ≥ moderate LAVV dysfunction (≥ moderate LAVV stenosis [LAVVS] and/or LAVV regurgitation [LAVVR])...

OBJECTIVES: Outcome data in tetralogy of Fallot (ToF) and complete atrioventricular canal (CAVC) are limited. We report our experience for over 40 years in this patient population. METHODS: Single center, retrospective analysis of patients who underwent surgical repair with diagnosis of ToF-CAVC from 1979 to 2022, divided in two different periods and compared. RESULTS: A total of 116 patients were included: 1979-2007 (n = 61) and 2008-2021 (n = 55)...

OBJECTIVE: To assess risks and benefits of cardiac intervention in adults with Down syndrome (DS). PATIENTS AND METHODS: A retrospective review was conducted using data from a study we published in 2010. Patients aged 18 years or older with DS who underwent cardiac operation or percutaneous intervention from February 2009 through April 2022 (new cohort) were compared with patients in the previous study (January 1969 through November 2007; remote cohort) at Mayo Clinic...

Complex congenital heart disease (CHD) in each of dichorionic diamniotic (DiDi) twin pairs is extremely rare and has not been well characterized. Four DiDi twin pairs were included in this multi-institutional case series. The congenital cardiac abnormalities noted included tetralogy of Fallot (ToF) with pulmonary atresia and collaterals (n = 1), ToF with absent pulmonary valve (n = 1), ToF (n = 2), discontinuous right pulmonary artery (RPA) (n = 1), tricuspid atresia (TA) with normally related great arteries and pulmonary valve stenosis or atresia (n = 2) and coarctation of aorta (CoA) with bicuspid aortic valve (BAV) and borderline left-sided structures (n = 1)...

BACKGROUND/AIM: Surgical repair techniques and management of patients with atrioventricular septal defect (AVSD) have progressed over the last few decades. Early and definitive interventions have become the choice of treatment for these patients. Based on this background, we aimed to review the early and mid-term outcomes of primary AVSD repair. METHODS: A total of 53 patients with a mean age of 3.45 ± 5.67 years underwent definitive repair for AVSD between January 2014 and June 2021...

OBJECTIVE: Patients with complete atrioventricular canal have a variable clinical course prior to repair. Many patients balance their circulations well prior to elective repair. Others manifest clinically significant pulmonary over circulation early in life and require either palliative pulmonary artery banding or complete repair. The objective of this study was to assess anatomic features that impact the clinical course of patients. METHODS: In total, 222 patients underwent complete atrioventricular canal repair between 2012 and 2022 at a single institution...

PURPOSE/OBJECTIVE(S): Low anterior resection syndrome (LARS) describes disordered bowel function including tenesmus, frequent, clustered, incomplete, urgent or incontinent bowel movements. The impact of clinical and radiation dosimetric factors on LARS score is unknown. We aimed to evaluate the radiation plans for patients who received long course chemoradiation (LC-CRT) to identify potential dosimetric predictors of LARS. MATERIALS/METHODS: We identified patients with rectal cancer treated with LC-CRT (50...

BACKGROUND: Some patients with pulmonary atresia with an intact ventricular septum (PA/IVS) or a left ventricle dominant atrioventricular canal defect (LDAVC) with a hypoplastic right ventricle (RV) and univentricular (1 V) circulation may be candidates for conversion to either a complete biventricular (2 V) repair or a one-and-a-half ventricle repair (1.5 V). We sought to identify pre-operative cardiovascular magnetic resonance (CMR) findings associated with successful conversion from 1V to 1...

This case report presents a 30-year-old male patient with a history of autism spectrum disorder who experienced a drastic decline in physical activity in recent years. Upon examination, the patient was found to be relatively bedridden, lethargic, and unable to maintain an upright gait, accompanied by severe clubbing. Transthoracic echocardiography revealed a massive 4 cm complete atrioventricular canal (CAVC) defect. Although the patient was asymptomatic throughout most of his life, the CAVC defect appeared to have progressively impacted his health and activity tolerance...

OBJECTIVE: To examine the incidence and phenotypes of congenital heart disease (CHD) in a large cohort of patients with Hirschsprung's disease (HSCR). STUDY DESIGN: Retrospective data review of children with HSCR between 2003 and 2020 was conducted at the Provincial Key Laboratory for Structural Birth Defects in Guangzhou, Guangdong, China. HSCR was confirmed by pathological diagnosis. CHD was defined as a gross structural abnormality of the heart or intrathoracic great vessels that is of functional significance...

BACKGROUND: Atrioventricular septal defects (AVSD) represent 4-7% of congenital cardiac malformations. Definitive early repair is favored over prior pulmonary artery banding and delayed definitive repair in many centers. The aim of this study was to analyze long-term outcomes following AVSD repair over a 21-year period. METHODS: A total of 202 consecutive patients underwent surgical AVSD correction between June 1999 and December 2020. Surgery was performed using the double-patch technique...

INTRODUCTION AND IMPORTANCE: Brain abscess is an uncommon but potentially fatal infection of the brain parenchyma that can affect 5 % to 18.7 % of people with uncorrected complex congenital heart defects. In management of patients with complex cardiac defects, the main concern is that they are prone to develop perioperative complications. Hence such cases are a real challenge for surgeons and anesthesiologists. In this study we have reported a well-managed awake craniotomy (Awake-Asleep-Awake) for drainage of cerebral abscess in a patient with complex cardiac defect...

BACKGROUND: Patients with unbalanced common atrioventricular canal can be difficult to manage. Surgical planning often depends on pre-operative echocardiographic measurements. We aimed to determine the added utility of cardiac MRI in predicting successful biventricular repair in common atrioventricular canal. METHODS: We conducted a retrospective cohort study of children with common atrioventricular canal who underwent MRI prior to repair. Associations between MRI and echocardiographic measures and surgical outcome were tested using logistic regression, and models were compared using area under the receiver operator characteristic curve...

BACKGROUND: Cerebrospinal fluid (CSF) rhinorrhea with meningoencephalocele (MEC) associated with Sternberg's canal is rare. We treated two such cases. CASE DESCRIPTION: A 41-year-old man and a 35-year-old woman presented with CSF rhinorrhea and mild headache worsening with standing posture. Head computed tomography showed a defect close to the foramen rotundum in the lateral wall of the left sphenoid sinus in both cases. Head magnetic resonance (MR) imaging and MR cisternography revealed that brain parenchyma had herniated into the lateral sphenoid sinus through the defect of the middle cranial fossa...

We present a case of a 61-year-old male who presented with an 8-month history of left hypacusis, tinnitus, and gait imbalance. MRI showed a vascular lesion in the left internal auditory canal (IAC). Angiogram showed a vascular lesion filling from the ascending pharyngeal and anterior inferior cerebellar artery (AICA) with drainage into the sigmoid sinus suggestive of either a dural arteriovenous malformation (dAVF) vs arteriovenous malformation (AVM) of the IAC. The decision was made to operate to prevent risk of future hemorrhage [1-5]...

OBJECTIVE: Acute outcomes after atrioventricular canal defects (AVCD) repair in the current era are excellent, yet despite surgical advances, ∼15% of patients require future left atrioventricular valve (LAVV) repair. Among AVC patients who undergo LAVV repair after primary AVC repair, we sought to characterize the durability of these repairs. Specifically, we aimed to determine predictors for re-intervention following a LAVV repair in repaired AVCD patients, with a focus on postoperative transesophageal echocardiography (TEE)...

Infective endocarditis (IE) is common in patients with corrected congenital heart disease (CHD) with a residual lesion, but is rarely found on surgical patches used to close atrial septal defects (ASDs). This is also reflected in the current guidelines that do not recommend antibiotic therapy for patients with a repaired ASD with no residual shunt six months after closure (percutaneous or surgical). However, the situation could be different in the case of mitral valve endocarditis, which causes leaflet disruption with severe mitral insufficiency and could seed the surgical patch...

BACKGROUND: Dural arteriovenous fistulas (dAVFs) occurring near the hypoglossal canal are rare. Detailed evaluation of vascular structures can identify shunt pouches at the jugular tubercle venous complex (JTVC) in the bone near the hypoglossal canal. Although the JTVC has several venous connections, including the hypoglossal canal, there have been no reports of transvenous embolization (TVE) of a dAVF at the JTVC using an approach route other than the hypoglossal canal. This report describes the first case of complete occlusion with targeted TVE using an alternative approach route in a 70-year-old woman presenting with tinnitus diagnosed with dAVF at the JTVC...