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). We describe an enigmatic case of a 29-year-old female who presented after an aborted cardiac arrest and was diagnosed with anomalous origin of the left coronary artery from pulmonary artery.

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. Differences in clinical characteristics and survival among children of different racial/ethnic groups were compared at listing and at HT. RESULTS: The waitlist cohort consisted of 2134 (52.2%) Caucasian, 840 (20.5%) African American, 808 (19.8%) Hispanic, 161 (3.9%) Asian, and 146 children of Other races (3.6%). At listing, Asian children mostly had cardiomyopathy (70.8%), whereas Caucasian children had congenital heart disease (58.7%). African American children were most likely to be listed as Status 1A and to have renal dysfunction and hypoalbuminemia at listing. African American and Hispanic children were most likely to be on Medicaid. After multivariable analysis, it was found that only African American children were at increased risk for waitlist mortality as compared to Caucasian children (adjusted hazard ratio = 1.25; P = 0.029). Post-HT, there were no disparities in early and midterm graft survival among groups, but African American children had increased numbers of rejection episodes compared to Caucasian and Hispanic children. CONCLUSION: African American children continue to experience increased waitlist mortality and have increased rejection episodes post-HT. Studies exploring barriers to health care access and implicit bias as reasons for these disparities need to be conducted.

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). The left coronary artery arose anomalously from the same sinus adjacent to the right coronary artery ostium, coursing posterior to the aorta, with brief intramural and interarterial course before bifurcating into the left anterior descending and left circumflex coronary arteries (Figs 3 and 4). As a result of this unique coronary pattern, she underwent unroofing of the intramural left coronary artery noted on opening the aortic root to the coronary ostium. Both coronary buttons were harvested and this large button was then divided into two buttons. The left coronary artery button was implanted with a trapdoor technique, right coronary artery button was implanted, and the remainder of the arterial switch procedure along with LeCompte maneuver was completed uneventfully, with closure of the atrial and ventricular septal defects. The post-operative course was uneventful and the patient was discharged on the seventh post-operative day. At discharge, the patient had normal biventricular systolic function, no residual intracardiac shunt, and robust antegrade flow in the reimplanted coronary arteries. The patient was growing well at the fourth month post-operative visit with normal biventricular function, patent coronaries, and outflow tracts.

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. The medical records were reviewed retrospectively to evaluate the clinical spectrum of the patients along with the endoscopic and histological findings. RESULTS: A total of 116 patients were evaluated. The mean age was 37.4 ± 16.6 (range: 13-80) years, 61 (53%) of the patients were male. Bleeding per rectum was present in 82%, abdominal pain in 49%, constipation in 23% and diarrhea in 22%. Endoscopically, solitary and multiple lesions were present in 79 (68%) and 33 (28%) patients respectively; ulcerative lesions in 90 (78%), polypoidal in 29 (25%), erythematous patches in 3 (2.5%) and petechial spots in one patient. Associated underlying conditions were hemorrhoids in 7 (6%), hyperplastic polyps in 4 (3.5%), adenomatous polyps in 2(2%), history of ulcerative colitis in 3 (2.5%) while adenocarcinoma of colon was observed in two patients. One patient had previous surgery for colonic carcinoma. CONCLUSION: SRUS may manifest on endoscopy as multiple ulcers, polypoidal growth and erythematous patches and has shown to share clinicopathological features with rectal prolapse, proctitis cystica profunda (PCP) and inflammatory cloacogenic polyp; therefore collectively grouped as mucosal prolapse syndrome. This may be associated with underlying conditions such as polyps, ulcerative colitis, hemorrhoids and malignancy. High index of suspicion is required to diagnose potentially serious disease by repeated endoscopies with biopsies to look for potentially serious underlying conditions associated with SRUS.

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. The mean duration of presentation of post-irradiation coronary artery disease is 16 years (range 3-29 years). In our patient coronary artery disease was found only a year post irradiation. A 55 year old male who presented with complaints of dyspnoea, retrosternal chest pain and heaviness since one year underwent resection for malignant thymoma followed by radiotherapy. He presented with coronary artery disease a year after undergoing mediastinal irradiation. On follow-up, patient was treated successfully by coronary artery bypass graft. This case is an unusual occurrence and suggests that mediastinal irradiation may result in significant coronary artery disease as early as within one year.

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. (Level of Difficulty: Advanced.).

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. Demographic, clinical characteristics and outcomes were compared unadjusted and after 1:1 propensity matching for selected covariates. RESULTS: Of 4089 patients, 2299 (56%) were males. At listing, males were more likely to be older, have congenital heart disease (58% vs 48%), renal dysfunction (49% vs 44%) and implantable cardioverter defibrillator (9% vs 7%). At transplant, males were more likely to have renal (42 % vs 35%) and liver dysfunction (13% vs 10%), PRA >10% (29% vs 22%) and ischemic time >3.5 hours (p < 0.05 for all). There were no significant sex differences found in unadjusted rates of transplant or mortality. After propensity matching, females had increased waitlist mortality (HR 1.3, 95%CI 1.04-1.5; p =0.019) compared to males. There were no significant differences in post-transplant morbidity or mortality (HR 1.2, 95% CI 0.93-1.5; p = 0.18) between groups. CONCLUSION: In a contemporary pediatric cohort, females have inferior heart transplant waitlist survival compared to propensity-matched males despite lower acuity of illness at listing and similar rates of transplantation. There were no sex-disparities noted in post-transplant outcomes.

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. Time-related transition to surgery was modeled using parametric methods. Predictive utility of CSA/Ht compared with aortic root diameter (ARd) and root Z score (ARz) were modeled using nonlinear multivariable parametric and nonparametric longitudinal regression models. RESULTS: Seventy-nine patients (43% female) presented at median age of 5.8 years (15th-85th percentile, 0.75-17 years) with median follow-up of 4.4 years (range, 0-18.5 years). Baseline echocardiography data were: CSA/Ht, 3.9 ± 1.4 cm2/m; ARd, 2.4 ± 0.89 cm; and ARz, 2.4 ± 1.7. CSA/Ht tracked ARd better compared with ARz (r = 0.91 vs 0.24). Eighteen patients underwent surgery. Surgical procedures included at least 2 components in 17 (aortic, mitral, tricuspid, aortic root, and arch procedures) and isolated mitral valve procedures in 1 patient. Time-related transition to surgery showed a prominent early phase to 1 year post presentation, followed by a slowly increasing late phase. CSA/Ht had a more linear correlation versus ARz during periods of rapid somatic growth in surgical patients. Surgical repair occurred at CSA/Ht between 5 and 7 cm2/m. CONCLUSIONS: CSA/Ht tracked ARd well over time. CSA/Ht between 5 and 7 cm2/m might be a promising metric for surgical triage in pMFS patients. CSA/Ht surgical threshold values in pMFS patients occurred at lower than current accepted "surgical" threshold values for CSA/Ht in adult Marfan syndrome patients.

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. METHODS: Retrospective review of 240 patients diagnosed with ccTGA from Cleveland Clinic coupled with prospective cross-sectional follow-up. Forty-six patients whose definitive procedure was completed elsewhere were excluded. Time-related survival was described among treatment pathways using actuarial, time-varying covariate, and competing risks analyses. Temporal trends in longitudinal valve and ventricular function were assessed using nonlinear mixed-effects models. RESULTS: Median follow-up was 10 years. Seventy-nine patients with ccTGA underwent anatomic repair, 45 physiologic repair, 24 Fontan palliation, and 6 primary transplant. Forty patients managed expectantly had excellent long-term survival when considered from time of presentation, but benefited from failures captured following transition to physiologic repair or transplant. Morphologic right ventricular dysfunction after physiologic repair increased from 68% to 85% after 5 years, whereas morphologic left ventricular function was stable in anatomic repair, especially with early surgery. Transplant-free survival at 15 years for anatomic and physiologic repair was 80% and 71%, respectively. CONCLUSIONS: Early anatomic repair may be preferable to physiologic repair for select ccTGA patients. Late attrition after physiologic repair represents failure of expectant management and progressive tricuspid valve and morphologic right ventricular dysfunction compared with anatomic repair, where morphologic left ventricular function is relatively preserved.

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. Baffle leaks and abnormal ventriculocoronary fistulae found in these patients are contributing factors. We sought to assess and describe coronary artery involvement in this complex patient population. Our research highlights both the need to assess distal coronary vasculature and to thoroughly evaluate hemodynamics and biventricular function with new-onset troponin leak or ventricular arrhythmias.

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. Our case highlights the need to assess for this rare condition in this high-risk population.

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.

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. Rigid bronchoscopy under general anaesthesia identified a fleshy mass in the left main stem bronchus which was excised via bronchoscopy. The histopathology showed benign polyp with non-specific inflammation. The patient's symptoms improved subsequently and no recurrence was reported till two years of follow-up.

A radiographic analysis of ventricular trajectories.

BACKGROUND: The prevalent method of ventriculostomy placement is via freehand insertion to cannulate the ventricle at a 90° angle to the skull to get ideal placement. Our goal was to test the validity of this practice in patients without midline shift and with normal ventricular size. METHODS: This study was a virtual radiographic analysis of 3-dimensional data of skull and ventricular anatomy. Data were collected using thin-cut (1-mm) computed tomography scans of 101 randomly selected patients with normal ventricular anatomy. Virtual ventriculostomy trajectories were determined for entry from the right and left sides separately, going in at a 90° angle to the skull. Three-dimensional multiplanar reconstructions were performed using Osirix software to see where the catheter would end up within the brain. RESULTS: In our patient population, the mean bicaudate index was 0.14. Of the 202 perpendicular lines created from Kocher's point into the brain, 67.8% (137) of the virtual lines passed through the ipsilateral frontal horn of the lateral ventricle, 20.8% (42) passed through the contralateral ventricle, and 10.4% (21) did not pass through a ventricular space. A lower bicaudate index also leads to a greater misplacement even with a perpendicular trajectory. Pushing a catheter beyond an entry length of 6.5 cm if no cerebrospinal fluid flow has been obtained will not result in ipsilateral ventricular catheterization. CONCLUSIONS: Our study concludes that not all catheters passed through Kocher's point using a perpendicular trajectory will end up in the ipsilateral frontal horn, and almost 10% of these catheters will be in a nonventricular space. In the instance in which a freehand pass fails to cannulate a ventricle, the safest alternative would be to make only minor adjustments to the perpendicular angle.

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. Demographic information and data regarding estimated success rates of ventriculostomies, the steps taken in failure and use of technological aids used was sought. RESULTS: 479 neurosurgeons and 108 residents responded to our survey (response rates 23.9% and 10.2%, respectively). No catheter misplacements were reported by 19.8% respondents in the previous year whereas 2.2% reported misplacing more than 30%. With regards to ventriculostomy for patients with slit ventricles, image guidance was used by 51.7%; freehand technique was preferred by 41.6% and the Ghajar guide was used by 6.7% of respondents. We found that 56.9% of respondents abandoned free-hand placement after 3 failed passes. After abandoning free-hand cannulation, respondents used an ICP bolt or similar intra-parenchymal pressure monitoring device in trauma patients. Other approaches included leaving the catheter in place and readjusting it after repeating a CT scan. CONCLUSIONS: This survey sheds light on the current practice of ventriculostomy placement. Both residents and neurosurgeons admit to multiple attempts and frequent catheter misplacement. In order to consider a change in practice, respondents cited an increase in available data about guidance systems and ability to accommodate abnormal ventricular anatomy as primary requirements. A prospective study could help establish true evidence based practice for this common neurosurgical procedure.