Optimizing evaluation in pediatric and young adult patients with Marfan syndrome: Novel longitudinal metrics to track growth of aortic structures.

Objective: Surveillance metrics in pediatric and young adult Marfan syndrome (pMFS) are challenging. We evaluated the utility of aortic root cross-sectional area/height index (CSA/Ht) on echocardiogram among pMFS patients as a risk stratification and surgical triage metric.

Methods: Genotype or phenotype positive pMFS patients aged 25 years or younger seen at our center from 2001 to 2020 were identified.

Hybrid Transcarotid Exclusion of Postoperative Ascending Aortic Dissection in an Infant.

Aortic dissection is very rare in pediatric patients, and associated risk factors include congenital heart disease, previous cardiac surgery, and vasculopathies. Acute postoperative aortic dissection in pediatric patients can be life-threatening. We performed a novel hybrid transcarotid covered stent exclusion of a postoperative ascending aortic dissection in an infant.

Sex disparities in the current era of pediatric heart transplantation in the United States.

Background: While sex-related differences in transplant outcomes have been well characterized amongst adults, there are no sex-specific pediatric heart transplant studies over the last decade and none evaluating waitlist outcomes. In a contemporary cohort of children undergoing heart transplantation in the United States, this analysis was performed to determine if there were sex disparities in waitlist and/or post-transplant outcomes.

Methods: Retrospective review of Scientific Registry of Transplant Recipients database from December 16, 2011 to February 28, 2019 to compare male and female children after listing and after transplant.

Life saved and a lesson learned-anomalous origin of the left coronary artery from pulmonary artery in pregnancy.

Anomalous origin of the left coronary artery from pulmonary artery constitutes 0.5% of all CHD (Boutsikou M, Shore D, Li W, et al, Int J Cardiol 261: 49-53, 2018). Fifteen percent survive into adulthood undiagnosed and 90% present with sudden death (Yau JM, Singh R, Halpern EJ, Fischman D, Clin Cardiol 34: 204-210, 2011; Alexi-Meskishvili V, Berger F, Weng Y, Lange PE, Hetzer R, J Card Surg 10: 309-315, 1995).

Racial and Ethnic Disparities Persist in the Current Era of Pediatric Heart Transplantation.

Background: Previous studies have demonstrated that children in the United States who were of racial and ethnic minorities have inferior waitlist and post-heart transplant (HT) outcomes. Whether these disparities still exist in the contemporary era of increased ventricular assist device use remains unknown.

Methods: All children (age <18 years) in the Scientific Registry of Transplant Recipients database listed for HT from December 2011 to February 2019 were included and were separated into 5 races/ethnicities: Caucasian, African American, Hispanic, Asian, and Other.

Outcomes of treatment pathways in 240 patients with congenitally corrected transposition of great arteries.

Objective: Congenitally corrected transposition of the great arteries (ccTGA) encompasses a diverse morphologic cohort, for which multiple treatment pathways exist. Understanding surgical outcomes among various pathways and their determinants are challenged by limited sample size and follow-up, and heterogeneity. We sought to investigate these questions with a large cohort of ccTGA patients presenting at different ages and representing the full therapeutic spectrum.

Unusual coronary artery arrangement in D-transposition of the great arteries with ventricular septal defect.

A term female with prenatally diagnosed D-Transposition of the great arteries, large membranous ventricular septal defect with inlet extension, moderate secundum atrial septal defect, and large patent ductus arteriosus (Fig 1) was born by scheduled caesarean section. Transthoracic echocardiogram confirmed the anatomy with both coronary arteries arising from a single sinus with separate ostia. The right coronary artery arose from right posterior facing sinus (Fig 2).

Selective Coronary Artery Angiography in Hypoplastic Left Heart Syndrome.

Coronary artery disease in palliated hypoplastic left heart syndrome is uncommon. Myocardial infarction from a coronary thrombus, serving as a substrate for ventricular arrhythmia in Fontan physiology, is under-reported despite known hypercoagulopathic state. Traditional risk factors for coronary artery occlusion include intracardiac thrombi, hyperlipidemia, and hypertension.

Ruptured Sinus of Valsalva Aneurysm Mimicking Infective Endocarditis in DiGeorge Syndrome.

Sinus of Valsalva aneurysm (SOVA) is rare and can occur in diseases with progressive aortic dilation. We report an adolescent male with DiGeorge syndrome who presented with fever and wide pulse pressure mimicking endocarditis. A timely diagnosis of ruptured SOVA using echocardiography enabled optimal treatment.

Streamlining Pediatric Emergency Medicine at a Tertiary-care Hospital of a Low- to Middle-income Country.

The factors of integral importance to run any pediatric emergency department efficiently are the ability to process a high volume of patients quickly and a sensitive triage system that identifies the sickest children. Achieving these aims in a low- to middle-income country setting is more complex as a result of scarce resources and data on which to base systems. In this article, we discuss existing models of streamlining pediatric emergency department services that are most applicable to resource-limited countries, and present suggestions for streamlining pediatric emergency care in such countries.

Imaging and intervention for coronary artery disease following irradiation of malignant thymoma.

Thymomas are rare malignant epithelial growths, constituting 20% of mediastinal tumours. Resection followed by irradiation may be employed in all thymomas except for stage 1 thymomas. Mediastinal irradiation is associated with coronary artery disease.

Benign granulomatous polyp obstructing the bronchus.

Inflammatory endobronchial polyps are rarely encountered. We report a case of a 14 years old girl with a benign granulomatous polyp originating in the bronchus. Computed tomography scan showed an intraluminal soft tissue opacity while fiberoptic bronchoscopy revealed a large soft tissue mass completely occluding the left lower lobe.

The clinical, endoscopic and histological spectrum of the solitary rectal ulcer syndrome: a single-center experience of 116 cases.

Background: Solitary rectal ulcer syndrome (SRUS) is an uncommon although benign defecation disorder. The aim of this study was to evaluate the variable endoscopic manifestations of SRUS and its association with other diseases.

Methods: All the patients diagnosed with SRUS histologically from January 1990 to February 2011 at The Aga Khan University, Karachi were included in the study.

A US-based survey on ventriculostomy practices.

Background: The incidence of ICP monitoring has increased over the years and the indications for placement have expanded. Although ventriculostomy and ICP monitor placement are among the most commonly performed neurosurgical procedures, the current practice patterns have rarely been studied.

Methods: A 10-question survey was sent to 2006 neurosurgeons and 1060 neurosurgery residents in the US.