Stephens, Elizabeth H.

We describe the anesthetic and operative techniques utilized for a tracheal tumor resection in a pediatric patient with 95% tracheal occlusion. In prior tracheal tumor cases that dictated complete resection, our team had been able to comfortably bypass a tumor with an endotracheal tube. In this case, we could not intubate past the tumor. A rigid bronchoscope was able to be placed past the tumor, so we continued with sternotomy and dissection before cardiopulmonary bypass while ventilating through that bronchoscope as our definitive airway.

Introduction: While valve-sparing repair is ideal for Tetralogy of Fallot (TOF), it’s durability and which patients may benefit from a transannular patch remains unclear. To this end, we reviewed our experience with valve-sparing TOF repair. Methods: Retrospective review was performed of all primary TOF operations at our institution from 1/2008 to 12/2018. Standard demographic, operative, and echo data were collected, along with clinical outcomes. Transannular patch and valve-sparing repair groups were then compared. Results: Sixty-eight patients underwent TOF repair with a mean age of 4.1±2.2 months and weight of 5.7±1.8 kg. There was no difference in age or weight between patients who underwent a transannular patch repair and valve-sparing repair (Table). There was also no difference in the frequency of hypercyanotic spells or beta-blocker use. As expected the pre-operative pulmonary valve size and z-score were significantly different between groups. Bypass times were longer in the transannular patch group (176±40 vs. 144±40 minutes, p=0.005). There were no differences in post-operative complications. At last follow-up (median 41.5 months) there was a trend of a higher peak pulmonary valve gradient (p=0.07) in the valve-sparing group, but no difference in pulmonary valve annulus z-scores. Additionally, the pulmonary valve z-scores in the valve-sparing group decreased from -2.3±1.0 on pre-discharge echocardiogram to -1.2±1.6 on last follow-up, with the peak gradient on pre-discharge 20 (33) mmHg stable on last follow-up at 18 (29) mmHg and degree of pulmonary regurgitation stable. There was one reoperation in the cohort: a pulmonary valve replacement in a patient who had undergone a transannular patch repair 6 years prior. Conclusions: Valve-sparing TOF patients demonstrated stable repairs with pulmonary valve growth, acceptable gradients, minimal regurgitation, and high freedom from re-intervention during follow-up.

Ebstein anomaly has a breadth of presentations, including “typical” and “atypical,” and can be confused with congenital tricuspid dysplasia. We summarize how to differentiate within this spectrum of disease. Both typical and atypical Ebstein have an underlying failure of delamination, but atypical Ebstein does not have ≥8mm/m 2 apical septal leaflet displacement. In congenital tricuspid dysplasia, delamination is normal, while the leaflets and subvalvar apparatus are abnormal. To summarize, the sine qua non feature of Ebstein anomaly, present in both typical and atypical, is the failure of delamination. These are distinct from congenital tricuspid valve dysplasia in which the pathology is in the leaflet itself.

Left ventricular outflow tract (LVOT) obstruction is a component of many forms of congenital heart disease, including hypertrophic cardiomyopathy, membranous subaortic stenosis, tunnel subaortic stenosis, and outflow tract obstruction related to atrioventricular septal defects. We have gained a particularly extensive experience with the diagnosis and treatment of hypertrophic cardiomyopathy, having performed septal myectomy in over 3,800 patients. In the setting of this review of LVOT obstruction, we use hypertrophic cardiomyopathy as a template by which other pathologies causing LVOT obstruction can be understood. We review important surgical issues in patient selection, diagnostic evaluation, interpretation of imaging, and operative management. To this end, the review focuses on obstructive hypertrophic cardiomyopathy and then broadens to discuss other pathologies causing LVOT obstruction, with important similarities and differences in their management. These other pathologies share some similar presentations and operative techniques, and at times can be confused with hypertrophic cardiomyopathy, but also have important distinctions of which the surgeon should be aware.

The coronavirus disease 2019 (COVID-19) pandemic currently gripping the globe is impacting the entire health care system with rapidly escalating morbidities and mortality. Although the infectious risk to the pediatric population appears low, the effects on children with congenital heart disease (CHD) remain poorly understood. The closure of congenital heart surgery programs worldwide to address the growing number of infected individuals could have an unintended impact on future health for COVID-19-negative patients with CHD. Pediatric and congenital heart surgeons, given their small numbers and close relationships, are uniquely positioned to collectively assess the impact of the pandemic on surgical practice and care of children with CHD. We present the results of an international survey sent to pediatric and congenital heart surgeons characterizing the early impact of COVID-19 on the care of patients with CHD.

As recovery of congenital heart surgery programs begins during this COVID-19 pandemic, we review key considerations such as screening, protection of patients and health care workers (HCWs), case prioritization, barriers to reactivation, redesign of patient care teams, contribution of telemedicine, modification of trainees’ experiences, preparation for potential resurgence, and strategies to maintain HCW wellness. COVID-19 has tested the resolve and grit of our specialty and we have an opportunity to emerge more refined.

The world as we once knew it has been drastically altered secondary to coronavirus disease 2019 (COVID-19). The impact of these changes, particularly for those practicing in the medical profession, extends beyond the physical to the psychological, emotional, and spiritual. We discuss the factors that contribute to these stresses, way to manage them, and how we as leaders of our teams can inspire resilience and help our colleagues endure these most difficult times.

With the increasing number of congenital heart disease patients living into adulthood and requiring reoperations, learning a safe and efficient reoperative sternotomy is essential. This article provides insight into the successful reoperative sternotomy, including preoperative evaluation, assessing risk for cardiac injury and preparations to take depending on the level of risk, safeguards taken before and during the sternotomy, and pearls and pitfalls in managing untoward events.