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Congenital diaphragmatic hernia (CDH) patients often have suspected isolated aortic arch anomalies (IAAA) on imaging. The purpose of this work was to describe the incidence and outcomes of CDH + IAAA patients.Cardiovascular data were collected for infants from the CDH Study Group born between 2007 and 2019. IAAA were defined as coarctation of aorta, hypoplastic aortic arch, interrupted aortic arch, and aortic aneurysmal disease on early, postnatal echocardiography. Patients with major cardiac malformations and/or chromosomal abnormalities were excluded. Primary outcomes included the rate of aortic intervention, rates of extracorporeal life support (ECLS) utilization, and mortality.Of 6357 CDH infants, 432 (7%) were diagnosed with a thoracic aortic anomaly. Of these, 165 were diagnosed with IAAA, most commonly coarctation of the aorta (n = 106; 64%) or hypoplastic aortic arch (n = 58; 35%). CDH + IAAA patients had lower birthweights (3 kg vs. 2.9 kg) and Apgar scores (7 vs. 6) than patients without IAAA (both χIAAA in CDH are associated with increased mortality. This often simply reflects severity of the defect and thoracic anatomic derangement, as opposed to unique aortic pathology, given few CDH + IAAA patients undergo aortic intervention.

BACKGROUND Borderline small ventricular size or technical issues precluding the use of both ventricles may lead to single ventricle palliation. Fontan complications have led some centers to look for alternatives to the traditional pathway. The objective of this study is to evaluate the essential philosophy and outcomes of a new biventricular (BiV) conversion program. METHODS The prospectively collected Children's Memorial Hermann Heart Institute Society of Thoracic Surgeon's Database was retrospectively reviewed between July 2017 and July 2020. RESULTS Thirteen patients met inclusion criteria and underwent BiV conversion during that time. The most frequent diagnosis was malposed great arteries and a ventricular septal defect (VSD) in 4 (31%) patients. Seven (54%) patients were in the first interstage, and 1 (8%) patient had already undergone a Fontan operation before their BiV conversion operation. One or more risk factors for single ventricle palliation (genetic syndrome ≥ moderate atrioventricular valve regurgitation ≥ moderate ventricular dysfunction, presence of signs of Fontan failure) were present in 3 (23%) patients. The median left ventricular end diastolic pressure increased from 5.5 mmHg (4-10 mmHg) to 10 mmHg (6-20 mmHg) postoperatively (p

Total anomalous pulmonary venous connection (TAPVC) is a major risk factor in infants with single ventricle (SV). Exact definition of TAPVC anatomy is crucial for surgical planning.To evaluate the role of cardiac computed tomography (CT) in this setting.Retrospective review of 13 infants who underwent TAPVC repair associated with SV from May 2016 to October 2021. Anatomy, incidence, and mechanisms of pulmonary venous obstruction (PVO) were described. Cardiac CT diagnostic yield was compared to echocardiography (echo).Of 13 infants, median age and weight were 24 days (range 2-303 days) and 3.2 (range 2.6-9.1) kg, 8 (62%) were male, 4 (31%) premature, and 11 (85%) had heterotaxy syndrome. All infants had pre- and postoperative echo; 13 had preoperative and 8 (62%) had postoperative cardiac CT. Type of TAPVC: six (46%) supracardiac, two (15%) intracardiac, one (8%) infracardiac, and four (31%) mixed, with pulmonary veins draining in1 confluence in nine (69%). PVO was present in 6/13 (46%) preoperatively and 5/13 (31%) postoperatively. Mechanisms of PVO: 9/11 (82%) stenosis, 1/9 (9%) membrane formation, and 1/9 (9%) external compression. The sensitivity to diagnose PVO was 45.5% for echo and 100% for cardiac CT, the specificity was 100% for both. No discrepancy was found between cardiac CT and intraoperative findings, but echo had a complete preoperative diagnosis in 1/13 (8%) (p .00001, Fisher exact test).Cardiac CT is essential to evaluate pre- and postoperative TAPVC in SV for surgical decision making and long term follow up.

Objectives The aim of this study is to describe clinical utility of low dose cardiac computed tomography (CT) in the evaluation of single ventricle physiology before and after Stage I palliation. Background Despite the increased utilization of CT imaging and advancement of CT technology, there are limited studies describing the routine clinical use of cardiac CT and radiation dose parameters in the single ventricle Stage I palliation. Methods This single center, retrospective study included 57 infants with single ventricle physiology who underwent cardiac CT scans between January 1, 2016 and November 30, 2020. Patients' demographic information, diagnosis, indication, total dose length product (DLP), computed tomographic dose index volume (CTDIvol), cardiac CT findings and intraoperative or intraprocedural findings were reviewed. Estimated effective radiation dose was calculated using a previously published conversion rate. Results The studies were performed using different generations of CT scanners over the 4 years period: Somatom AS 128, Somatom definition edge, Somatom Force (Siemens Medical Solutions). The studies performed with dual source scanner with prospective gated technique have lower radiation dose exposure with median effective radiation dose of 0.32 mSv. Conclusion Pre- and post-operative cardiovascular CT in Stage I single ventricle palliation using newer generation scanners with prospective gated technique can be done with minimal radiation exposure and good image quality. Cardiac CT is a powerful imaging modality for better management planning in this group of patients.

BACKGROUND Un-roofing is the most common technique utilized for repair of anomalous aortic origin of a coronary artery (AAOCA). There are very few publications directly comparing un-roofing to another surgical technique, like reimplantation. METHODS The prospectively collected Children's Memorial Hermann Heart Institute Society of Thoracic Surgeon's Database was retrospectively reviewed from 2007 to 2021. Surgical patients were included if they underwent un-roofing or reimplantation of the AAOCA. The primary outcomes of this study were operative characteristics and postoperative outcomes. Secondary outcomes included angiographic outcomes, aortic regurgitation incidence, ventricular function, and symptom relief. RESULTS From 2007 to 2021, there were 12 patients who underwent either a reimplantation (n = 9, 73%) or un-roofing (n = 3, 27%) for an AAOCA. The hospital length of stay was a median of 1.8 days longer for reimplantation compared to un-roofing. The last follow-up echocardiogram was a median of 52.2 days later in the reimplantation group. There was one patient (11%) in the reimplantation group that had more than or equal to mild aortic regurgitation and mild systolic ventricular dysfunction. Outpatient follow-up was incomplete and there was no postoperative computed tomographic angiography in the un-roofing cohort. CONCLUSIONS Coronary artery reimplantation is a valuable alternative surgical technique to un-roofing for the repair of AAOCA. There are still some concerns with the creation of aortic regurgitation or incomplete symptom relief with any surgical technique. Longer-term follow-up and prospective studies will be needed to show an effective reduction of myocardial ischemia and risk of sudden cardiac death.

Infective endocarditis is a significant cause of morbidity and mortality in hospitalized patients, especially with the increasing use of indwelling catheters in critically ill children. Surgical excision is sometimes essential to relieve valvar dysfunction and reduce burden of infection. Here we present a preterm infant who developed refractory infective endocarditis and right heart failure with tricuspid valve vegetation likely related to an indwelling umbilical venous catheter. Infective endocarditis resolved after resection of the vegetation and tricuspid valve repair, followed by a 6-week course of antibiotics.

Hypoplastic left heart syndrome (HLHS) was a uniformly fatal diagnosis before 1983, when surgical treatment was first undertaken with the Norwood I operation as the first of 3-staged operations. Since then, operative survival rate of stage I has risen from 53% to over 90% in the current era, not only thanks to technical advances in surgery but also through prenatal diagnosis and imaging, enhanced cardiopulmonary bypass technology, better perioperative intensive care, and closer interstage monitoring. The improvements in patient outcomes achieved through rigorous multidisciplinary teamwork have come at a tremendous cost in manpower and resources, making HLHS still a challenge to all congenital heart programs, established or emerging. We review the various surgical steps to treat HLHS and their current expected outcomes, and put into perspective cost considerations compared to other more "simple" congenital heart defects.

Interruption of the aortic arch, aortopulmonary window, and anomalous origin of the right pulmonary artery from the ascending aorta are very rare congenital anomalies. It is even rarer to have all three anomalies in the same setting. We present a case of a newborn who was diagnosed with these lesions and describe the primary repair of these anomalies.

Relative contraindications for transanal endoscopic microsurgery include high, anterior-based lesions for full-thickness excisions because of worries about entering the peritoneal cavity. Concerns exist regarding safety and oncological outcome.We examined the outcomes of transanal endoscopic microsurgery excisions with entry into the peritoneal cavity and compared them with those that did not to address our hypothesis that entry is safe with no ill infectious or oncological consequences.This single-institution retrospective review uses a prospectively maintained database.This study was conducted at a tertiary colorectal surgery referral center.From 1997 to 2012, we identified 303 patients who underwent transanal endoscopic microsurgery resections, with 26 patients having entrance into the peritoneal cavity.Perioperative data, postoperative morbidities, delayed morbidities, and oncological outcomes were the primary outcomes measured.Of 26 patients, there were 8 women with a mean age of 67.5 years. Mean BMI was 31 kg/m, and ASA class was III or IV in 69%. Mean superior border of the lesion was 10.4 cm (4.5-16). Forty-eight percent had anterior-based lesions. Anterior location, level from anorectal ring, and diagnosis of cancer were significantly higher in the peritoneal entry group (p = 0.003, p = 0.007, and p = 0.007). Preoperative diagnoses included 16 adenocarcinomas, 8 polyps, and 2 carcinoid tumors. Thirteen patients had preoperative chemoradiation. Median estimated blood loss was 15 mL (5-400), and 3 patients underwent diversions. Median time to discharge was 3 days (2-10). There were no perioperative mortalities. Median follow-up time was 21.0 months. There was 1 local recurrence (3.8%), and there was no development of carcinomatosis.This review was limited by its retrospective nature.High anterior location rectal lesions should be considered candidates for transanal endoscopic microsurgery excision in experienced hands. After obtaining considerable transanal endoscopic microsurgery experience, our use of transanal endoscopic microsurgery in a high-risk patient population allowed us to definitively treat 88% of patients without an abdominal operation and the need for a temporary or permanent colostomy. Theoretic concerns of abscess or carcinomatosis were not experienced (see Video, Supplemental Digital Content 1, http://links.lww.com/DCR/A154).

Since the first successful intervention for hypoplastic left heart syndrome (HLHS) was undertaken by Norwood in 1983, there have been many advancements in the pre-, intra-, and postoperative care of these children for a diagnosis that just 25 years ago was almost certainly a fatal one. This paper aims to describe the most recent trends and perspectives on the treatment of HLHS. In particular, we will discuss the five current options for HLHS, including Norwood stage I as the beginning to 3-stage palliation, transplant, true hybrid, hybrid-bridge-to-Norwood, and compassionate care.

Pheochromocytoma is an uncommon tumor of the adrenal glands that can present with headaches, sweating, palpitations, and paroxysmal hypertension. Pheochromocytoma crisis can lead to cardiomyopathy, pulmonary edema, and even total circulatory collapse. We describe a patient with hypoxic respiratory failure requiring extracorporeal membrane oxygenation to stabilize until the pheochromocytoma was discovered and treated.

Complete thymectomy is the procedure of choice in the treatment of thymomas and in treating selected patients with myasthenia gravis. Transsternal thymectomy is the gold standard for most patients. Robot-assisted thymectomy has emerged as an alternative to open transsternal surgery. The goal of this study was to compare perioperative outcomes in patients who underwent transsternal or robot-assisted thymectomy.We performed a retrospective review of all patients who underwent robot-assisted or transsternal thymectomy at our institution from February 2001 to February 2010. Data are presented as mean ± SD. Significance was set as P 0.05.Fifty patients underwent either transsternal (n = 35) or robot-assisted (n = 15) thymectomy. Patient demographics and the incidence of myasthenia gravis were similar between groups. There were no intraoperative complications or conversions to open surgery in the robot-assisted group. Intraoperative blood loss was significantly higher in the transsternal group (151.43 vs. 41.67 ml, P = 0.01). There were 20 postoperative complications and 1 postoperative death in the transsternal group and 1 postoperative complication in the robot-assisted group (P = 0.001). Hospital length of stay was 4 days (range 2-27 days) in the transsternal group and 1 day (range 1-7 days) in the robot-assisted group (P = 0.002).Robot-assisted thymectomy is superior to transsternal thymectomy, reducing intraoperative blood loss, postoperative complications, and hospital length of stay. Further investigation of the long-term oncologic results in thymoma patients and long-term remission rates in patients with myasthenia gravis who underwent robot-assisted thymectomy is warranted.