Search

Although established, transcatheter pulmonary valve replacement is in its infancy compared with surgical pulmonary valve replacement. Extended clinical experience and follow-up have identified new challenges; however, careful evaluation of data through clinical trials has facilitated effective evolution of responses to these challenges. The limited patient population has resulted in less interest in new valve design, but having been the older sibling to transcatheter aortic valve replacement, transcatheter pulmonary valve replacement is likely to benefit in the future from design modifications to the more popular and commercially viable transcatheter aortic valve revolution. Improving valve longevity and applying the technology to native outflow tracts remain the short-to-medium term goals.

We studied conduit-related risk factors for mortality, conduit reintervention, conduit replacement, and pulmonary artery (PA) reinterventions after truncus repair. Patients who underwent truncus repair at our institution between 1995 and 2019 were studied. Cox proportional hazards modeling evaluated variables for association with mortality, time to conduit reintervention, time to conduit replacement, and time to PA reintervention. Truncus was repaired in 107 patients at median age of 17 days (IQR 9-45). Median follow-up time was 7 years. Aortic homografts were implanted in 57 (53%) patients, pulmonary homograft in 40 (37%), and bovine jugular conduit in 10 (9%). Median conduit size was 11 mm (IQR 10-12) and median conduit Z-score was 1.71 (IQR 1.08-2.34). At 5 years, there was 87% survival, 21% freedom from conduit reinterventions, 37% freedom from conduit replacements, and 55% freedom from PA reinterventions. Conduit size (HR 0.7, 95%CI 0.4-1.4, p=.41) and type (aortic homograft reference; bovine jugular vein graft HR 0.6, 95% CI 0.08-5.2, p=.69; pulmonary homograft HR 0.7, 95% CI 0.2-2.3, p=.58) were not associated with mortality. On multivariate analysis, the hazard for conduit reintervention, conduit replacement, and PA reintervention decreased with increasing conduit Z-score values of 1 to 2.5 (non-linear relationship, p.01), with little additional reduction in hazard beyond this range. Implantation of a larger conduit within Z-score values of 1 and 2.5 is associated with a decreased hazard for conduit reintervention, conduit replacement, and PA reintervention after truncus repair. The type and size of the conduits did not impact mortality.

Cestode infestations in animals are the most important parasite of livestock and humans because most of these parasites are zoonotic causing cysticercosis and hydatidosis in man and it causes economic and production losses in livestock. Diagnosis of Taenia Spp by microscopic observation lack sensitivity and specificity and detection by enzyme-linked immunosorbent assay (ELISA) technique form cross-reaction. The molecular diagnostic can be best to detect in adult and larval stage in definitive and intermediate host based on the amplification of deoxyribonucleic acid (DNA) of target gene with the primer using a different technique of polymerase chain reaction (PCR) such as multiplex PCR. Conventional PCR, real-time PCR, nested PCR, and PCR-restriction fragment length polymorphism (RFLP) are highly sensitive for the diagnosis of cestode and metacestode. Those diagnoses are used for differentiation of Taenia species and differentiation of Taenia and Echinococcus species. As compared to other diagnostic techniques most molecular methods have higher sensitivity and specificity but due to the relatively higher cost, few are commercially available. Most of the molecular diagnostic tests developed to date are generally applicable for laboratory research purposes. The developments in the genomic and proteomic analysis should be used for further understanding of parasite-animal host interaction to find additional targets for diagnosis.

Background We evaluated the range of prosthetic size-to-weight ratio to optimize valve survival in small children. Methods A single-institution retrospective review of mechanical mitral valve replacements from 1995 to 2019 was performed. Prosthetic valve size-to-weight ratio was calculated as the prosthetic valve diameter divided by the patient’s operative weight in children less than or equal to 35 kg. Patient death or reoperation on the valve was analyzed by size-to-weight ratio. Identifying a U-shaped distribution of events, patients were stratified as being in the nadir of the distribution or on the edges. Results Mechanical mitral valve replacements were performed in 56 (75%) children weighing less than or equal to 35 kg. Median follow-up time was 3.7 (interquartile range, 0.46-12) years. Median size-to-weight ratio was 1.5 (interquartile range, 1.0-2.0). A second replacement was required in 15 (27%) patients. Death occurred in 6 (11%) patients, including 3 after reoperation. The nadir of U-shaped distribution of events by size-to-weight ratio was bounded by a ratio from 1 to 2, which included 29 (52%) patients. A size-to-weight ratio from 1 to 2 provided optimal outcomes regardless of patient age. Reoperation-free survival at 5 years was 96% for patients with a ratio from 1 to 2 and 46% for patients with a ratio less than 1 or greater than 2. Patients with size-to-weight ratio 1 to 2 had longer reoperation-free survival than patients with a ratio less than 1 or greater than 2 (P Conclusions Regardless of patient age, in patients less than or equal to 35 kg, optimal reoperation-free survival after prosthetic mitral valve replacement can be obtained by placing a prosthetic valve whose diameter is between 1 and 2 times the patient’s weight in kilograms.

Multiple techniques are available for repair of supracardiac partial anomalous pulmonary venous return (PAPVR); however, most series fail to compare the techniques in contemporary cohorts. This study aimed to describe outcomes of the Warden procedure with a single-patch repair cohort to serve as a control.A retrospective cohort analysis of all patients at a single institution (Texas Children's Hospital, Houston, TX) included patients undergoing either the Warden procedure or single-patch repair from 1996 to 2019 for PAPVR. Reintervention was defined as any catheter or surgical procedure on the superior vena cava (SVC) or pulmonary veins. Subgroup analysis was performed within the Warden cohort to evaluate for association between an SVC patch and reintervention-free survival.In total, 158 patients (122 in the Warden group and 36 in the single-patch group) were identified. The median age at operation was younger for patients in the Warden cohort (5.4 years; interquartile range, 3.3 to 10.2 years) compared with patients in the single-patch cohort (13.3 years; interquartile range, 6.5 to 18.7 years; P.001). One patient in each cohort died. One patient required reoperation after the Warden procedure for dehiscence of the intracardiac patch. Ten patients required transcatheter reinterventions. Reintervention-free survival was not different between patients in the Warden cohort and patients in the single-patch cohort (P = .54) or within the Warden cohort in patients with an SVC patch (P = .27). When controlling for repair type, older age at repair was associated with longer reintervention-free survival (hazard ratio, 0.81; 95% confidence interval, 0.71 to 0.93; P = .002).The Warden procedure is a viable option for younger patients requiring supracardiac PAPVR repair, although these younger patients are likely at greatest risk for reintervention regardless of surgical technique.

Background Multiple techniques exist for the repair of supravalvular aortic stenosis (SVAS), but given the lesion’s rarity, analyses comparing the efficacy of each repair have been limited. Methods A retrospective review of all children at a single institution who underwent repair of SVAS from June 1995 to May 2019 was performed. Anatomic and physiologic measurements across time points were compared between 2 predominant surgical techniques. Time-to-event outcomes were compared using the log-rank test. Results SVAS was repaired in 89 patients, by using a single-patch in 31 (35%) and the Doty repair in 58 (65%). Median age at operation was 2.5 years (interquartile range [IQR], 1.0 to 6.8 years), with median follow-up of 5.8 years (IQR, 1.8 to 10.7 years). Reoperation was required in 8 (9%) patients at a median of 1.5 years postoperatively (IQR, 0.3 to 4.8 years). There was 1 death after multiple reinterventions. The change from the preoperative to the postoperative sinotubular junction z-score was greater for patients after Doty repair (median change +2.5; IQR, 1.5, 4.1) than for patients after single-patch repair (median change +0.8; IQR, −0.1, 2.1; P = .001). Freedom from reoperation was longer for patients after Doty repair than after the single-patch technique (P = .008). Conclusions The Doty repair provides longer freedom from reoperation after supravalvular aortic stenosis repair compared with a single-patch technique, likely through a greater increase in the sinotubular junction at the time of initial operation.

Introduction: Cardiac defects that result in shunting are the most common types of congenital heart anomalies. Although these lesions can be simple, they can cause significant hemodynamic changes a...

BACKGROUND Covered stent correction of sinus venosus ASDs (SVASD) is a relatively new technique. Challenges include anchoring a sufficiently long stent in a nonstenotic superior vena cava (SVC) and expanding the stent at the wider SVC-RA junction without obstructing the anomalous right upper pulmonary vein (RUPV). The 10-zig covered Cheatham-platinum (CCP) stent has the advantage of being available in lengths of 5-11 cm and dilatable to 34 mm in diameter. METHODS An international registry reviewed the outcomes of 10-zig CCP stents in 75 patients aged 11.4-75.9 years (median 45.4) from March 2016. Additional stents were used to anchor the stent in the SVC or close residual shunts in 33/75. An additional stent was placed in 4/5 (80%) with 5/5.5 cm CCPs, 18/29 (62%) with 6 cm CCPs, 5/18 (28%) with 7 cm CCPs, 5/22 (23%) with 7.5/8 cm CCPs and 0/1 with an 11 cm CCP. A "protective" balloon catheter was inflated in the RUPV in 17. RESULTS Early stent embolization in two patients required surgical removal and defect repair and tamponade was drained in one patient. The CT at 3 months showed occlusion of the RUPV in one patient. Follow up is from 2 months to 5.1 years (median 1.8 years). QP:QS has reduced from 2.5 ± 0.5 to 1.2 ± 0.36 (p

INTRODUCTION: The optimal management of scimitar syndrome remains incompletely defined. We (1) evaluated the impact of aortopulmonary collateral (APC) occlusion, (2) compared outcomes according to surgical approach for patients who underwent surgery, and (3) identified anatomic factors associated with longer survival time without scimitar vein repair. METHODS: We conducted a single center, retrospective study of 61 patients diagnosed with scimitar syndrome between 1995 and 2019. Right pulmonary artery to total pulmonary artery cross-sectional area (RPA:PA CSA) quantitatively assessed right pulmonary artery size. Anatomical features were analyzed for association with longer survival time without scimitar vein repair. RESULTS: Median follow-up time was 6 years (Q1-Q3, 2-12), with 96% 5-year survival. Twenty-three patients underwent APC occlusion, which significantly decreased symptoms of overcirculation (100%-46%; p = .001) and systolic pulmonary artery pressure (median, 34-29 mmHg; p = .004). Twenty-three patients underwent scimitar vein repair; 5-year freedom from scimitar vein stenosis was 90% among patients who underwent a reimplantation compared with 42% in patients with baffle repair (p = .1). Three patients underwent surgery before the first year of age, with lower 5-year freedom from scimitar vein stenosis (0% vs. 84%; p < .001). On multivariate analysis, a lower RPA:PA CSA was associated with longer survival time without scimitar vein repair (p = .003). CONCLUSIONS: APC occlusion improves the clinical status of young and hemodynamically unstable patients. Repair at an early age is associated with an increased risk of scimitar vein stenosis. Scimitar vein repair might be avoided in patients with a smaller right pulmonary artery.

Given pulmonary artery interventions following the Norwood procedure can recur, the average number of occurrences per patient over time is likely more informative than the crude percentage of patients who required an intervention. Pulmonary artery intervention was defined as any surgical or catheter-based procedure after the Norwood procedure. The number of pulmonary artery interventions for patients with hypoplastic left heart syndrome were compared between patients with modified Blalock-Taussig Shunts (MBTS) and right ventricle-to-pulmonary artery conduits (RVPA) at a single institution from 2011 to 2018. The comparison was replicated using data from the Single Ventricle Reconstruction Trial (SVR), a nonoverlapping dataset. The mean number of pulmonary artery interventions per patient over time (mean cumulative function, MCF) is described using Nelson-Aalen estimates and compared using the pseudo-score test. The number of patients requiring intervention was compared using the chi-square test. Using our institutional dataset, the Norwood operation was performed on 117 patients (59 MBTS, 58 RVPA). In total, 73 patients had a pulmonary artery intervention, including 32 of 58 (55%) after MBTS and 41 of 59 (69%) after RVPA (P= 0.11). The MCF did not vary between cohorts (P = 0.55). Using the SVR trial dataset, 140 of 549 patients required pulmonary artery intervention, including 55 (21%) after MBTS and 85 (30%) after RVPA (P = 0.0090). The MCF did not vary between cohorts (P = 0.067). Although more patients with RVPA than MBTS require pulmonary artery interventions after the Norwood procedure, the MCFs are not different, which may be of greater importance to patients and families.

The foundation of wisdom is rooted in experience, and thus we reflexively call upon our senior leaders, mentors, coaches, and family members for guidance in our personal and professional lives. Witnessing the weathered perspectives of others allows for an internal audit of one's own strengths and deficiencies, which ultimately inspires personal growth. This experience is heightened when both the mentor and the mentee, for example, share a common goal. The field of congenital interventional cardiology, with its constant evolution and diverse technical approaches, requires a lifetime of learning, as well as safe passage of knowledge to the next generation. While there are published recommendations for what to consider when completing this task, hearing the sentiments of those with experience may be more profitable for future fellows and current interventionalists. In part one of a series, we hope to accomplish this goal by presenting an opportunity to learn from our experienced colleagues on the topic of congenital interventional cardiology training. Specifically, we aim to share expert opinions on how to succeed as a congenital interventional fellow, illustrate the diversity of teaching styles and expectations in various healthcare systems, and for the mid-career interventionalists, provide insight into the character traits of a successful mentor of interventional fellows.

Recent reports have suggested that the number of adults with congenital heart disease (CHD) has surpassed pediatric numbers and that approximately 1 in 150 adults has some form of CHD (Marelli et al., Circulation 115:163–172, 2007; Warnes et al., Circulation 118:2395–2451, 2008). Significant rates of intervention and re-intervention are required in this cohort of patients with one report demonstrating 20 % of young adults with CHD requiring cardiovascular surgery (Zomer et al., Circulation 124:2195–201, 2011). Increasingly, transcatheter alternatives to surgery exist in this population particularly in the field of transcatheter pulmonary valve replacement (tPVR), and recommendations for catheter intervention in adults with CHD have been published in the context of overall care of these patients (Warnes et al., Circulation 118:2395–2451, 2008). Case complexity will vary according to the underlying diagnosis ranging from more straightforward interventions such as atrial septal defect closure to complex stenting interventions for interatrial baffle leak in patients following atrial switch surgery for transposition of the great arteries. Many of the more complex patients may have had multiple previous interventions, and detailed review of previous data as well as sensible use of pre-procedural imaging techniques including cardiac CT and MRI is essential. This chapter will outline some of the procedural techniques and the diagnostic imaging tools used to optimize patient selection and procedural success. Outcome data where available will also be discussed in relation to the range of transcatheter interventions seen in this patient group.

Although aortopulmonary collaterals (APCs) frequently develop in patients with single ventricle palliation, there is a lack of understanding of pathophysiology, natural history, and outcomes with no universal guidelines for management and interventional practice. We conducted a study to assess the views held by interventional congenital cardiologists regarding the hemodynamic impact of APCs in patients with single ventricle palliation, and their embolization practice. An electronic survey using the Pediatric Interventional Cardiology Symposium (PICS) mailing list was conducted between February and March 2019 with one reminder sent 2 weeks after initial invitation for participation. Of the 142 interventional cardiologist respondents, 95 (66.9%) reside in North America and 47 (33.1%) worldwide. We elected to exclude the data from interventionalists outside North America in this analysis as it was not representative of worldwide practice. Hypoxemia was considered to be the most common trigger for development of APCs by 56 (58.9%) respondents. After completion of total cavopulmonary connection, 30 (31.6%) respondents reported the APC burden stays the same while 31 (32.6%) feel it decreases. In evaluating the burden of APC flow, only 4 (4.2%) reported measuring oxygen saturation at different pulmonary artery segments, 21 (22.1%) perform segmental aortic angiograms, and 18 (19%) perform selective bilateral subclavian artery angiograms. A majority of respondents, 71 (74.7%), occlude the feeder vessel at different locations, while 10 (10.5%) occlude only the origin of the vessel. Our study demonstrates significant variation in the understanding of the cause and prognosis of APCs in patients with single ventricle palliation. Furthermore, there is variation in the approach for diagnosis and management among interventional cardiologists. Further studies are required to improve understanding of APCs and develop universal management guidelines.

BACKGROUND AND OBJECTIVES: Polymer-based bioresorbable scaffolds (PBBS) have been assessed for coronary revascularization with mixed outcomes. Few studies have targeted pediatric-specific scaffolds. We sought to assess safety, efficacy, and short-term performance of a dedicated drug-free PBBS pediatric scaffold compared to a standard low profile bare metal stent (BMS) in central and peripheral arteries of weaned piglets. METHODS: Forty-two devices (22 Elixir PLLA-based pediatric bioresorbable scaffolds (6x18 mm) and 20 control BMS Cook Formula 418 (6x20 mm)) were implanted in the descending aorta and pulmonary arteries (PA) of 14 female Yucatan piglets. Quantitative measurements were collected on the day of device deployment and 30 and 90 days post implantation to compare device patency and integrity. RESULTS: The bioresorbable scaffold (BRS) has a comparable safety profile to the BMS in the acute setting. Late lumen loss (LLL) and percent diameter stenosis (%DS) were not significantly different between BRS and BMS in the PA at 30 days. LLL and %DS were greater for BRS vs BMS in the aorta at 30 days post implantation (LLL difference: 0.96 ± 0.26; %DS difference: 16.15 ± 4.51; p

Background This single-institution study assessed the midterm outcomes of patients undergoing complete vascular ring (CVR) repair and the need for reintervention. Methods The study included all patients who underwent surgical repair of an isolated CVR from 1996 to 2018 at our institution. Patients who underwent concomitant intracardiac repair were excluded. Data analysis included demographics, type of anomaly, other congenital heart disease, clinical symptomatology, operative technique, perioperative outcomes, reoperation rates, and mortality. Results CVR repair through open thoracotomy was performed in 148 patients (80 boys [54%]), median age, 1.04 years (interquartile range, 0.4-5.2 years), and median weight, 12.8 kg (interquartile range, 7.5-26.5 kg). The cohort included 72 patients with double aortic arch (DAA), 69 with right aortic arch (RAA) with aberrant left subclavian artery and left ligamentum arteriosum (LLA), 5 with RAA with left descending aorta and LLA, and 2 with RAA with mirror-image branching and LLA. There was 1 outpatient perioperative death (0.7%) 15 days postoperatively. Perioperative complications occurred in 20 patients (14%): 18 (12%) with chylothorax (3 required reintervention), 1 pneumothorax, and 1 vocal cord paresis. Two of 36 patients (5.5%) without primary diverticulum resection required reoperation and subclavian reimplantation at 3 and 4 years, and 1 patient required aortic translocation 9 years later for persistent symptoms. Conclusions Freedom from reoperation after CVR repair was 93% at 5 years and 86% at 10 years. A small proportion of patients who do not undergo diverticulum resection and aberrant left subclavian artery reimplantation at the time of CVR repair will require reintervention in the future.

Background Pain is one of the common and devastating symptoms that affects millions of cancer patients globally. Despite published guidelines and education on the assessment and management of cancer-related pain, underestimated or undertreated pain continues to be a considerable worldwide public health concern among cancer patients. In this study, we aimed to assess physicians’ adherence to the World Health Organization (WHO) guidelines in the management and assessment of pain in oncology patients based on the available score of pain in the Princess Noorah Oncology Center (PNOC) at the King Abdulaziz Medical City in Jeddah. Methodology This cross-sectional, retrospective chart review study studied 451 patients (selected through computerized random sampling) who were admitted to the PNOC during the study period. Results The pain was assessed using the Brief Pain Inventory in almost all patients (n = 450, 99.8%). The pain was categorized as mild in 386 (85.6%) patients, moderate in 46 (10.2%) patients, and severe in 19 (4.2%) patients. Opioid prescriptions were significantly higher among patients with moderate (76.1%) and severe pain (89.5%) compared to those with mild pain (39.1%; p < 0.0001). Conclusions The practice of pain documentation for cancer patients was adequate as indicated by reporting the pain scores of 99.8% of inpatients. Patients with moderate and severe pain were more likely to receive opioids and a combination of opioids plus non-opioid analgesics, whereas the prescription of analgesics was predicted by experiencing moderate cancer pain.

Background: Transcatheter pulmonary valve replacement (TPVR) is now an established modality for pulmonary valve replacement in suitable candidates. We aim to describe our experience with TPVR in adults. Methods: This is a descriptive study of all TPVR performed in adults with congenital heart disease at a single institution from 2010–2020. All adult patients (defined as 18 years old or older at TPVR) were included. Time-to-event outcomes were described using Kaplan-Meier estimates with 95% confidence intervals (CIs). Results: Out of a total of 200 patients that had undergone TPVR, 81 patients (57% male) met the inclusion criteria, with a median age and weight of 26 years (IQR 21–37) and 71.0 kg (IQR 54.6–89.0), respectively. In the cohort, 45 (56%) patients had tetralogy of Fallot. While 53 (65%) patients received a Melody valve, a Sapien valve (S3 in 20, XT in eight) was implanted in the rest. Pre-stenting was performed in 49 (52%) patients. One patient died of severe heart failure a year following TPVR. One patient had a second TPVR performed 2.2 years following initial TPVR for severe pulmonary regurgitation. Valve survival at 2.2 years was 94% (95% CI: 87–100%). Four patients developed endocarditis. Endocarditis-free survival was 89% (95% CI: 80–100%) at three years. Conclusions: Our experience suggests favorable results of TPVR in adults with congenital heart disease. Additional research would be warranted with a focus on total valve longevity and patient reported outcomes, in order to improve the understanding of TPVR in this population and further refine this technology.

Background Hydatid disease is an endemic disease in many countries of the world including the Middle East. It mainly affects the liver and lungs. Intramuscular hydatid disease is rarely reported in children. Such uncommon localization of hydatid cyst may pose difficulties in the clinical and radiological diagnosis; hence affecting patient’s management and outcome even in endemic areas. Case presentation We herein describe intramuscular hydatid cysts in 2 different children. The first case is a 5-year-old boy who presented with a painless palpable lump over the right lumbar paraspinal region. His history was remarkable for sheep contact. His laboratory results revealed a mild increase in white blood cell (WBC) count and C-reactive protein. The lesion showed typical features of a hydatid cyst on ultrasound. Further imaging including ultrasound of the abdomen and CT of the chest, abdomen, and pelvis showed infestation of the liver and lung as well. The lesions were resected surgically without complications. The patient received Albendazole preoperatively and after surgery for 3 months. No evidence of recurrence was seen during follow-up. The second case is a 6-year-old girl who presented with an incidental palpable lump in her left thigh during her hospital admission for recurrent meningitis. Ultrasound and MRI imaging were performed demonstrating a unilocular cystic lesion in the left proximal rectus femoris muscle. A provisional diagnosis of hematoma vs. myxoma was given. Biopsy was performed and yielded blood products only. The lesion was resected surgically with a postoperative diagnosis of hydatid cyst. Blood tests performed afterward showed a positive titer for Echinococcus. The patient received Albendazole for 3 months. No evidence of recurrence was seen during follow-up. Conclusions Despite its rarity; skeletal muscle hydatid cyst should always be considered in the differential diagnosis of cystic muscle lesions in children in endemic areas even if imaging studies did not show any of the typical signs. This will improve patient outcome by preventing unnecessary cystic puncture which might lead to serious complications, such as anaphylaxis and local dissemination.

Introduction: As with any bioprosthetic valve, bioprosthetic valves in the pulmonary position have a finite life span and patients with bioprosthetic pulmonary valves require lifetime management to...

We aimed to describe the short- and long-term outcomes of patients after an arterial switch operation (ASO) at a single institution during a 23-year period. A retrospective chart review of all patients18 months of age who underwent an ASO between January 1995 and March 2018 at Texas Children's Hospital, Houston, TX was performed. Primary endpoints include mortality and reintervention. Perioperative mortality was defined as mortality occurring in-hospital and/or30 days after surgery. Survival and freedom-from-reintervention were analyzed using Kaplan-Meier method, log-rank tests, and Cox regression models. The cohort included 394 patients. Diagnoses included 204 patients (52%) with intact ventricular septum, 137 (35%) with a ventricular septal defect, 17 (4%) with a ventricular septal defect and left ventricular outflow tract obstruction (LVOTO), and 36 (9%) with Taussig-Bing anomaly. Median age at surgery was 8 days (range: 1 day to 17 months) and median weight was 3.4 (range: 0.8-12.0) kg. Overall perioperative mortality was 1.3% (n = 5), 0.3% (n = 1) since 1999. Overall survival at 5, 10, and 15 years was 98.2%, 97.8%, and 97.8%, respectively. Perioperative morality was associated with prematurity (P = 0.012),2.5 kg (P0.001), and longer circulatory arrest (P = 0.024) after univariate analysis. Reintervention was associated with a longer cross-clamp time (P0.001),2.5 kg (P = 0.009), LVOTO resection (P = 0.047), and genetic syndrome (P= 0.011) after multivariable analysis. Current ASO expectations should include a perioperative mortality risk of1% and good long-term survival. Reinterventions are more frequent in patients2.5 kg, concomitant LVOTO resection, a genetic syndrome, and longer cross-clamp time.

Femoral head avascular bone necrosis (AVN) is the loss of blood supply to the bone tissue of femoral head that results in cellular death. This condition causes a significant limitation in patient daily life activities and has a poor functional outcome. Long-term steroid intake was established as a cause of AVN. However, few cases reported femoral head AVN post-single steroid intra-articular injection. We review all cases of AVN that results from single intra-articular steroid injection and present a case of femoral head AVN developed in a 78-year-old male. The patient, who was not known to have any medical illness, presented complaining of mild left hip pain for 4 months with long distant ambulation and weight standing. He was diagnosed to have left hip joint osteoarthritis for which he received intra-articular steroid injection 2 months prior visiting our orthopedics center. MRI of the pelvis revealed AVN of the femoral head. He underwent total hip arthroplasty. The pathological examination confirmed the diagnosis of AVN. To best of our knowledge, this is the fifth case of AVN of femoral head AVN after single intra-articular steroid injection. We reviewed all cases of AVN of femoral head after single steroid injection. Intra-articular steroid injection can cause femoral head AVN, and the patient receiving these injections should be aware about this rare but significant complication that results in poor functional outcome and significant morbidity.

Objective: Basal cell carcinoma (BCC) is the most common malignancy in humans and represents a growing public health care problem. The major etiological factors contributing to BCC development are exposure to ultraviolet radiation and genetic alterations. BCC is primarily caused by dysregulation of sonic Hedgehog (HH) signaling pathway in basal cells of the skin. BCC can be classified into low risk non-aggressive and high risk aggressive subtypes. BCC subtypes differentiation is essential for prognosis and for better disease management and treatment strategies. The aim of this study was to assess the correlation between PTCH1 protein expression level and the aggressiveness of BCC histopathology. Methods: Archival paraffin embedded blocks containing BCC were retrieved from a cohort of 101 patients. Immunohistochemistry staining was performed to assess the expression level of PTCH1 which is a key component of Hedgehog pathway. Results: 101 paraffin embedded samples were evaluated and classified as high risk and low risk BCC subtypes by histopathological finding. High risk BCC subtypes were found in 40 samples (39.6%) and low risk subtypes were identified in 61 samples (60.4%). Nodular was the most frequent subtype which was found in (56/ 101), followed by infiltrative (22/101) and micronodular (14/ 101) subtypes. Positive PTCH1 expression was found highest in nodular subtypes (46.5%). Conclusion: In this study, the correlation between low risk or high risk BCC subtypes and PTCH1 expression level was not statistically significant (p>0.05), but the frequency of positive PTCH1 expression was found to be higher in low risk subtypes than high risk BCC subtypes.

Step changes in angiographic imaging are not commonplace. Since the move from analogue to digital and flat detector plates, two-dimensional imaging technology has certainly evolved but not jumped forward. Of all the routine imaging techniques used in cardiology, angiography has been the last modality to embrace the third dimension. Although the development of rotational angiography was initially for the benefit of neuroimaging and fusion of cross sectional datasets was aimed at the treatment of descending aortic pathology, interventional physicians in congenital and structural cardiology have immersed themselves in this technology over the last 10 years. Like many disruptive technologies, its introduction has divided opinion. We aimed to explore the mindset of those in the field of interventional cardiology who are driving imaging forward. These structured interviews recorded during the 21st Pediatric and Adult Interventional Cardiac Symposium illustrate the challenges and sticking points as well as giving an insight into the direction of travel for three-dimensional imaging and fusion techniques. Covering a wide range of career development, seniority and experience, the interviewees in this article are probably responsible for the majority of the published literature on invasive three-dimensional imaging in congenital heart disease.

The range and number of educational and networking events that are available for fellows, trainees, and junior faculty to attend grows every year. Each meeting useful in its own way; each adding value to the development and the growth of an interventionist. Within paediatric, congenital, and structural heart disease, three of the standout meetings are: Pediatric and Interventional Cardiac Symposium (PICS-AICS), Congenital and Structural Interventions (CSI), and International Workshop on Interventional Pediatric and Adult Congenital Cardiology (IPC). All of these were started by leaders in our field; people known to be passionate educators and innovators. International congresses focusing more broadly on congenital cardiac disease in children and adults are rare. These forums allow more interdisciplinary discussions between the interventionist, surgeon, and non-invasive specialists. Purely interventional meetings are essential to allow colleagues to debate and explore the nuances and intricacies of technique and approach, developing concepts to be challenged in wider forums. During the recent 21st PICS-AICS meeting Prof. Ziyad M. Hijazi, Shakeel A. Qureshi, Mario Carminati, and Dr Damien Kenny shared their time to engage in frank, recorded conversations which provide a unique insight in to the process and concepts behind three of our most important educational congresses.

The prevalence of metabolic syndrome (MetS) increases in parallel with the increasing incidence of obesity. Hence, bariatric surgery is potentially curative in obese patients with MetS. We aim to measure the effect of bariatric surgery as well as the potential factors leading to MetS resolution in the Saudi population. A retrospective review of three tertiary care centers in Saudi Arabia was done. We identified 386 patients who have MetS and underwent bariatric surgery during the period between January 2016 and December 2018. After exclusion of patients with insufficient follow-up, data from 275 patients was analyzed. MetS diagnosis, persistence, and resolution were determined using the International Diabetes Federation consensus worldwide definition. MetS resolution was achieved in 78%, 79%, 73%, and 82% at 1, 2, 3, and 4 years after bariatric surgery, respectively. Accumulatively, 80% achieved MetS resolution with an average follow-up period of two and a half years. Patients who experienced resolution had younger age, lower BMI, lower systolic blood pressure, lower triglycerides, lower fasting plasma glucose, and lower HbA1c preoperatively in addition to a higher percentage of excess weight loss after bariatric surgery when compared to those who had persistence of MetS. More than three fourths of patients with MetS achieved resolution after bariatric surgery. Certain patient characteristics, such as young age and higher excess weight loss postoperatively, were associated with a higher chance of MetS resolution.

Patient: Male, 13-year-old Final Diagnosis: Post traumatic hemosiderotic synovitis Symptoms: Knee pain • swelling and limitation in movements Medication: — Clinical Procedure: — Specialty: Radiology Objective: Rare disease Background: Hemosiderotic synovitis (HS) is a rare proliferative synovial disorder with incompletely understood pathophysiology. It mainly affects the knee joint. It can be confused with pigmented villonodular synovitis (PVNS), both clinically and radiologically. HS has not been previously reported in children, and this rare incidence may lead to difficulties in early clinical and radiological diagnosis, possibly affecting the patient’s outcome. Case Report: A 13-year-old boy presented with progressive right knee pain, swelling, and limitation of movement 2 months after a traumatic injury in a soccer game. His past medical and family history was unremarkable. His physical exam showed right knee effusion, fullness in the popliteal fossa, and painful restriction in active and passive knee motion. Laboratory tests showed a mildly elevated erythrocyte sedimentation rate. Knee X-rays showed joint effusion. Knee MRI showed large knee joint effusion with diffuse low signal intensity villous synovial hypertrophy in all sequences in addition to a large popliteal cyst with the same imaging characteristics, consistent with diffuse-form PVNS. Total arthroscopic synovectomy was performed without complications. Intraoperatively, there was a large effusion containing yellow clots. The histopathological diagnosis was post-traumatic HS with no evidence of PVNS or malignancy. The patient showed significant clinical and radiological improvement postoperatively with no evidence of recurrence. Conclusions: Post-traumatic HS is a very rare entity that should always be considered in the differential diagnosis of synovitis in active, non-hemophilic children. Radiologists’ familiarity with this rare condition is important to guide correct and early diagnosis, and to avoid unnecessary invasive intervention.

To assess extraspinal findings (ESFs) prevalence in lumbar spine MRI, including clinically significant findings using a systematic approach, and to determine their reporting rate. Lumbar spine MRI scans were retrospectively reviewed over 18 months by two radiologists. Reading discrepancies were resolved by consensus. ESFs were classified according to the involved system, clinical diagnosis, and clinical significance. The reporting rate was estimated by referring to the original report. There were 1509 ESFs in 1322/4250 patients with a substantial agreement between the two radiologists (kappa = 0.8). Almost half (621/1322) were in the 45–60 age group. Females represented 56.6% (748/1322). 74.2% (1120/1509) of the ESFs involved the urinary system among which 79.6% (892/1120) were renal cysts. Clinically significant findings represented 8.7% (131/1509) among which hydronephrosis represented 23% (30/131). First time detected malignant lesions represented 4.6% (6/131). ESFs reporting rate was 47.3%. 58.8% of the clinically significant ESFs were not reported. ESFs prevalence was 31.1%. The Urinary system was the most commonly involved. Most ESFs were benign warranting no further workup. However, clinically significant ESF were not infrequently detected. More than half of the clinically significant findings were not reported. A systematic review of MRI images is highly recommended to improve patient’s outcome.

The aim of the present study was to investigate the molecular characteristics of hereditary multiple osteochondromas (HMO) in a subset of Jordanian patients with a focus on the genetic variants of exostosin (EXT1)/(EXT2) and their protein expression. Patients with HMO and their family members were included. Recorded clinical characteristics included age, sex, tumors number and location, joint deformities and associated functional limitations. Mutational analysis of EXT1 and EXT2 exonic regions was performed. Immunohistochemical staining for EXT1 and EXT2 was performed manually using two different commercially available rabbit anti-human EXT1 and EXT2 antibodies. A total of 16 patients with HMO from nine unrelated families were included, with a mean age of 13.9 years. A total of 75% (12/16) of the patients were male and (69%) (11/16) had a mild disease (class I). EXT mutation analysis revealed only EXT1 gene mutations in 13 patients. Seven variants were detected, among which three were novel: c.1019G>A, p. (Arg340His), c.962+1G>A and c.1469del, p. (Leu490Argfs*9). Of the 16 patients, 3 did not harbor any mutations for either EXT1 or EXT2. Immunohistochemical examination revealed decreased expression of EXT1 protein in all patients with EXT1 mutation. Surprisingly, EXT2 protein was not detected in these patients, although none had EXT2 mutations. The majority of Jordanian patients with HMO, who may represent an ethnic group that is infrequently investigated, were males and had a mild clinical disease course; whereas most patients with EXT1 gene mutations were not necessarily associated with a severe clinical disease course. The role of EXT2 gene remains a subject of debate, since patients with EXT1 mutations alone did not express the non-mutated EXT2 gene.

Background:The rate of bleeding complications following arterial switch operation is too low to independently justify a prospective randomised study for benefit from recombinant factor VIIa. We aimed to evaluate factor VIIa in a pilot study.Methods:We performed a retrospective cohort study of patients undergoing arterial switch operation from 2012 to 2017. Nearest-neighbour propensity score matching on age, gender, weight, and associated cardiac defects was used to match 27 controls not receiving recombinant factor VIIa to 30 patients receiving recombinant factor VIIa. Fisher’s exact test was performed to compare categorical variables. Wilcoxon’s rank-sum test was used to compare continuous variables between cohorts.Results:Post-operative thrombotic complications were not associated with factor VIIa administration (Odds Ratio (OR) 0.28, 95% CI 0.005–3.77, p = 0.336), nor was factor VIIa administration associated with any re-explorations for bleeding. No intraoperative transfusion volumes were different between the recombinant factor VIIa cohort and controls. Post-operative prothrombin time (10.8 [10.3–12.3] versus 15.9 [15.1–17.2], p < 0.001) and international normalised ratio (0.8 [0.73–0.90] versus 1.3 [1.2–1.4], p < 0.001]) were lower in recombinant factor VIIa cohort relative to controls.Conclusions:In spite of a higher post-bypass packed red blood cell transfusion requirement, patients receiving recombinant factor VIIa had a similar incidence of bleeding post-operatively. With no difference in thrombotic complications, and with improved post-operative laboratory haemostasis, a prospective randomised study is warranted to evaluate recombinant factor VIIa.

Background: Anomalous aortic origin of the left coronary artery (AAOLCA) confers a rare, but significant, risk of sudden cardiac death (SCD) in children. Surgical intervention is recommended for AAOLCA with an interarterial course, with other subtypes considered benign. We aimed to determine the clinical characteristics and outcomes of AAOLCA in a prospective cohort following a standardized approach. Methods: All patients with AAOLCA Results: A total of 47 patients (32 male, 68%) with AAOLCA were enrolled, median age 12 [IQR 6.0;15.0] years. Of these, 20 had exertional symptoms (42.5%) and 5 had aborted SCD (10.5%). Thirty-one patients (79%) were classified high-risk, 28% low-risk, and 3 (6%) work-up is ongoing. Of patients with completed work-up, 16/43 (37%) had inducible ischemia and, of these, 2/16 (13%) presented without symptoms and one with aborted SCD as the first symptom. Two patients in our cohort had AAOLCA arising close to the noncoronary sinus, just above the left and noncoronary commissure, resulting in ostial stenosis: one presented with aborted SCD and another with left ventricular dysfunction. To date, 17 patients have undergone repair, median age 10 years [IQR 6.6;15.7]. Median follow-up was 3.9 [IQR 1.3;6.3] years with no significant symptoms and all but 4 patients are cleared from exercise restrictions. Conclusion: AAOLCA may present with diverse anatomic configuration and risk stratification is essential to determine management. AAOLCA from the noncoronary sinus may also present with myocardial ischemia and, thus, be classified as high-risk. A multimodality imaging approach is necessary to adequately risk stratify this population. All patients are alive at last follow-up and the majority free from exercise restriction.

Introduction: Surgical repair in anomalous aortic origin of a coronary artery (AAOCA) aims at mitigating the risk of sudden cardiac death in a subset of patients (pts). The pattern and behavior of electrocardiogram (ECG) changes in a large cohort of these pts is lacking. We aim to describe post-operative (post-op) ECG changes in this population with medium term follow up (f/u). Methods: Observational study evaluating ECGs before and after surgery to describe peri-operative changes through f/u. All patients Results: A total of 64 pts were identified (median age 13.7 yrs, 64% male). Two patients had evidence of STE and TWI pre-op and were not included. Of 62 pts: 47 (75%) had new STE early post-op and all but 1 had resolution at 90-day f/u; 50 (81%) had no evidence of new TWI or q waves early post-op and 96% of these remained stable at 90-day f/u. Conversely, 12 (19%) had new TWI in the early post-op: 8 had isolated changes (75% resolved at 90 days) and 4 associated with new q waves (50% resolved at 90 days). Including all ECG changes in the total cohort, 92% resolved completely at 90-day f/u. In those pts (8%) with persistent changes, none required late reintervention and all but 1 were cleared for return to exercise. All patients were alive at medium f/u of 4 yrs (IQR 2-6) with normal ventricular function. Conclusions: New STE is a common finding in the early post-op period following repair of AAOCA and is not correlated with persistent ECG changes at 90-day follow up. TWI is infrequently seen but less likely to resolve if associated with new q waves. Despite these changes in the early post-op, clinical outcomes medium-term are excellent.

Introduction: Anomalous aortic origin of a right coronary artery (AAORCA) may lead to myocardial ischemia (ISCH) and sudden death in the young. Current guidelines deem asymptomatic patients (pts) low-risk if normal exercise stress test (EST). Hypothesis: The frequency of ISCH in AAORCA pts is seen more commonly on stress perfusion imaging (sPI) compared to EST. Method: Pts 7 yrs and in younger pts if concern for ISCH. sPI included nuclear imaging initially and dobutamine stress cardiac MRI since 06/2014. High-risk features included intramural length (IML), abnormal ostium, symptoms or evidence of myocardial ISCH. Shared decision-making directed medical/surgical management. Results: Of 220 pts (male 135, 61%) enrolled at median age of 12 [IQR 6-15] yrs, 168 (76%) had no/non-exertional symptoms (Group-1) and 52 (24%) had exertional chest pain/syncope (Group-2). Coronary CTA was available in 180 and EST in 164 pts. EST was positive in 2/164 (1%), both had positive sPI. Of 162 negative EST, inducible ISCH was seen in 10/96 (10%) in Group-1 and 8/42 (19%) in Group-2. No difference in IML was noted between pts with/without ISCH (5.7±2.4 vs 6.0±2.1 mm, p=0.6). Surgery was indicated in 67 (30%) pts with high-risk features, not indicated in 143 (65%), and 10 (5%) are undergoing evaluation. In 51 operated pts (38 unroofing; 13 translocation), 3 await post-operative assessment, 48 on normal physical activities; median follow-up of 4.6 [IQR 2.3-6.3] yrs. All patients were alive at last follow-up; median of 2.9 [IQR 1.2-5.1] yrs. Conclusions: AAORCA pts can present with reversible ISCH on sPI regardless of symptoms or IML. EST may be a poor predictor of ISCH and caution should be given to determine low-risk. Most high-risk patients return to exercise and remain well at medium-term follow-up.

Anomalous aortic origin of a coronary artery (AAOCA) can be associated with myocardial ischemia and sudden cardiac arrest. We describe and compare the management and surgical techniques for patients with AAOCA. Patients presenting to the Coronary Artery Anomalies Program are evaluated and managed following a standardized approach. Our approach and data were compared to other single-center and multi-institutional data and results. Patients with AAOCA present as an incidental finding approximately 50% of the time. Advanced axial imaging is essential to define the anatomic characteristics of this lesion. Preoperative and postoperative assessment of myocardial perfusion with provocative testing is feasible and contributes to risk stratification. The surgical techniques for AAOCA repair include coronary unroofing, transection and reimplantation, and neo-ostium creation, among others. In general, surgical repair of AAOCA can mitigate the risk of ischemia with low mortality. The specific morbidities and complications of each different technique should be considered during the surgical planning. Surgical repair of AAOCA can mitigate the risk of ischemia with a low associated mortality but with clinically relevant morbidities. Long-term follow-up is necessary to accurately balance the risks of repaired and unrepaired AAOCA.

Objective:Review a single-centre experience with pulmonary artery sling repair and evaluate risk factors for re-intervention.Methods:Patients with surgically repaired pulmonary artery sling at a single institution between 1996 and 2018 were retrospectively reviewed. A univariate Cox regression analysis was used to evaluate variables for association with freedom from re-intervention.Results:Eighteen patients had pulmonary artery sling repair. At operation, median age and weight were 6.9 months (interquartile range 4.1–18.1) and 9.5 kg (interquartile range 6.5–14.5), respectively. A median hospital length of stay was 12 days (interquartile range 5.8–55.3). Twelve patients (67%) had complete tracheal rings, of whom six (50%) underwent tracheoplasty (five concurrently with pulmonary artery sling repair). Airway re-intervention was required in five (83%) of the six patients who underwent tracheoplasty. One patient had intraoperative diagnosis and repair of pulmonary artery sling during unrelated lesion repair and required tracheoplasty 24 days post-operatively. One patient died 55 days after pulmonary artery sling repair and tracheoplasty following multiple arrests and re-interventions. Median post-operative follow-up for surviving patients was 6.3 years (interquartile range 11 months–13 years), at which time freedom from re-intervention was 61%. When controlling for patient and tracheal size, initial tracheoplasty was associated with decreased freedom from re-intervention (hazard ratio 21.9, 95% confidence interval 1.7–284.3, p = 0.018).Conclusions:In patients with pulmonary artery sling, tracheoplasty is associated with decreased freedom from re-intervention. In select patients with pulmonary artery sling and complete tracheal rings, conservative management without tracheoplasty is feasible. Further study is necessary to delineate objective indications for tracheoplasty.

Background:We aimed to describe our experience with patients with sickle cell trait (SCT) and undergoing surgery on cardiopulmonary bypass (CPB).Methods:Data on all patients with SCT or sickle-α thalassemia who underwent surgery on CPB were collected (1996-2017).Results:Overall, 46 patients were included, 37 (80%) had SCT and 9 (20%) had sickle-α thalassemia. A total of 4 (9%) developed a potential sickle cell–related complication. Patients with sickle cell-related complications were significantly older (median 14 years vs 14 months, P = .037) and heavier (median 54 kg vs 9 kg, P = .041). Complications occurred, although without statistical significance, in patients who underwent longer median CPB times (249 minutes vs 137 minutes, P = .069), lower median temperature (31.7 °C vs 33.3 °C, P = .094), and a higher percentage underwent deep hypothermic circulatory arrest (50% vs 7%, P = .053). A total of 30 (65%) patients underwent exchange transfusion (ET) pre-bypass. Patients who underwent ET were significantly older (median 4 years vs 7 months, P = .003) and heavier (median 16 kg vs 6 kg, P = .015) than patients who did not undergo ET. The incidence of complications was comparable between patients who underwent ET (10%) and those who did not (6%).Conclusions:In this retrospective, single-center study, it has been shown that cardiac surgery requiring CPB in patients with SCT and sickle-α thalassemia had a low risk for sickle cell-associated complications. In this cohort of patients, older age, longer CPB times, lower median temperature, and the utilization of deep hypothermic circulatory arrest appear to play an important role in the development of complications.

Background: Diabetic neuropathy is a condition that is prevalent among type 2 diabetic patients. Some physicians prescribe vitamin B12 or vitamin B complex supplements to improve symptoms, but studies have shown that there is little to no evidence of vitamin B12 being an effective treatment for diabetic neuropathy. Thus, this study aims to investigate local physicians’ knowledge and tendency to prescribe vitamin B12 or vitamin B complex for the treatment or prevention of diabetic peripheral neuropathy. Methods: It was a cross-sectional study, conducted between May and November of 2019, in several primary healthcare centers in different cities of Saudi Arabia. A total of 412 physicians with a minimum of three years of experience answered a three-part questionnaire on their demographic information, their prescribing behavior, and their knowledge of the relationship between vitamin B12 or vitamin B complex and diabetic neuropathy. Results: The study found that only 42% of the physicians believed that vitamin B12 supplementation did not prevent diabetic neuropathy, while only 52.7% found it to be an ineffective treatment for this condition. Moreover, 58.7% stated that they had indeed prescribed vitamin B12 or multivitamins as a form of treatment or prevention of diabetic neuropathy. 47.8% of the patients requested a vitamin B12 prescription 1-6 times from their physicians, while 31.6% of them requested it ≥ 7 times, with 42.5% of physicians agreeing that their prescriptions of vitamin B12 had been a result of patient demand more than clinical justification. Likewise, 43% of respondents were aware that vitamin B12 levels should be tested annually. Furthermore, a higher proportion of consultants chose not to prescribe vitamin B12 to prevent or treat diabetic neuropathy than any other rank. Conclusion: The findings of this study indicate a tendency of unnecessarily prescribing vitamin B12 supplementation for the prevention or treatment of diabetic neuropathy as well as a lack of knowledge on the matter among doctors in primary care hospitals in Saudi Arabia. The study has also shown that it is patients who often request this prescription, adding pressure on their physicians to comply. Future studies should investigate hospitals in a greater number of Saudi cities and include less experienced residents and medical students.

Objective Anomalous aortic origin of a coronary artery (AAOCA) can be associated with myocardial ischemia and sudden cardiac arrest. We compared outcomes data of patients who underwent transection and reimplantation (TAR) and patients who underwent an unroofing. Methods Patients who presented to the Coronary Artery Anomalies Program were evaluated and managed following a standardized approach. Anatomy was determined using computed tomography angiography, myocardial perfusion using advanced stress imaging, and surgical intervention according to anatomic features. Results Sixty-one patients underwent surgical repair of AAOCA between 2012 and 2019: 16 (26%) patients underwent TAR of the anomalous coronary without an aortic button and 45 (74%) patients underwent coronary unroofing. Compared with patients who underwent an unroofing, patients who underwent TAR had similar intramural length (5 mm with interquartile range of 4-7.7 vs 6 mm with interquartile range of 5-7; P = .6). One patient with an anomalous right coronary underwent coronary artery bypass grafting after TAR because of persistent postoperative ischemic changes. One patient with unroofing of an anomalous left coronary artery presented with recurrent aborted sudden cardiac death and underwent subsequent TAR, without further events. At last follow-up, 15 of 16 patients (94%) who underwent TAR and 42 of 45 (93%) patients who underwent an unroofing were released to unrestricted exercise activities. Conclusions Coronary artery TAR is a useful surgical alternative for AAOCA when there is a course below the commissure, when unroofing does not relocate the ostium to the appropriate sinus, or when unroofing results in compression by the intercoronary pillar.

Epic Supra valves have been used off-label in the pulmonary position. We aim to evaluate the durability of Epic valves in the pulmonary position. We performed a retrospective review of all Epic valves placed in the pulmonary position from October 2008 to May 2019. Time-to-event analysis was performed using Kaplan-Meier estimates to evaluate freedom from valve intervention, moderate pulmonary regurgitation, and peak velocity greater than 3.5 m/s. Valve dysfunction was a composite of all 3 end points. A total of 79 patients had Epic valves implanted in the pulmonary position. Median age was 18.5 years (15th–85th percentile 11.2–41.0). In total, 1 (1%) 19 mm valve, 4 (5%) 21 mm valves, 8 (10%) 23 mm valves, 23 (29%) 25 mm valves, and 43 (54%) 27 mm valves were implanted. There were no deaths or transplants. Median follow-up was 3.1 years (interquartile range 1.0–5.5). At 5 years, freedom from valve intervention was 95%, freedom from valve dysfunction was 68%, freedom from moderate pulmonary regurgitation was 73%, and freedom from peak velocity greater than 3.5 m/s was 82%. Epic Supra valves provide an acceptable valve replacement in the pulmonary position for children and adults. Longer follow-up is needed to determine valve durability through the entirety of the valve life expectancy.

Atrial septal defect (ASD) is one of the most common congenital heart diseases (CHD) in children and adults. This group of malformations includes several types of atrial communications allowing shunting of blood between the systemic and the pulmonary circulations. Early diagnosis and treatment carries favorable outcomes. Patients diagnosed during adulthood often present with complications related to longstanding volume overload such as pulmonary artery hypertension (PAH), atrial dysrhythmias, and right (RV) and left (LV) ventricular dysfunction.This article intended to review all aspects of ASD; anatomy, pathophysiology, clinical presentation, natural history, and indication for treatment. Also, we covered the transcatheter therapy in detail, including the procedural aspect, available devices, and outcomes.There has been a remarkable improvement in the treatment strategy of ASD over the last few decades. Transcatheter closure of ASD is currently accepted as the treatment of choice in most patients with appropriately selected secundum ASDs. This review will focus on the most recent advances in diagnosis and treatment strategy of secundum ASD.

Congenitally corrected transposition of the great arteries (ccTGA) is a lesion that rarely occurs in isolation. The presenting physiology of ccTGA is predominantly secondary to the concurrent cardiac lesions; however, as the child ages, unrepaired ccTGA results in progressive failure of the morphologic right ventricle under the strain of maintaining a systemic pressure. Repair of ccTGA was initially focused on rectification of the underlying physiologic aberrations, but in recent years, the focus of repair has shifted toward anatomic correction to avoid failure of the morphologic right ventricle. This anatomic repair is commonly associated with improved long-term mortality at the cost of increased short-term mortality. Key preoperative considerations such as morphologic left ventricular pressure, tricuspid valve competency, and out flow tract obstructions can assist in determining the optimal repair for individual patients. An alternative, single ventricle, pathway has been proposed for any patient without optimal preoperative anatomy to improve long-term survival. Adjunctive repair options including pulmonary artery banding and one-and-a-half ventricle repairs have also been proposed to augment the survival curves.

Background: Congenital kyphosis results from the failed formation of the vertebrae during the embryonic period and may be associated with cardiac, urogenital, or spinal cord anomalies. Surgical treatment is the best choice through anterior, posterior, or both approaches. Objectives: This study aims to evaluate the effect of posterior gradual correction using the “in situ” bender to correct severe thoracolumbar congenital kyphosis with or without osteotomy or excision of the vertebra. Methods: Twenty-five patients with an age range of 2–23 (mean ± SD = 12.58 ± 6.03) years with severe thoracolumbar kyphosis were treated surgically at our institution between 2004 and 2013. Pedicle screwing, osteotomy, and gradual “in situ” bending through the single posterior approach were the choices of treatment. Cobb’s angle, patient’s height, and SRS-22r were used to evaluate the patients preoperatively and postoperatively. Follow-up periods were 35–136 months. Result: Cobb’s angle pre-surgery range was 35–180 (81.48 ± 39.1) degrees improved post-surgery to 0–45 (21.72 ± 13.47) degrees ( p-value Conclusion: Gradual correction with the “in situ” bender with or without osteotomy through the single posterior approach can give satisfying clinical (patients’ standing height and SRS-22r scores) and radiological (Cobb’s angle) results to treat severe congenital thoracolumbar kyphosis.

Previous studies have demonstrated increased early mortality and pulmonary vein reintervention for patients with total anomalous pulmonary venous connection (TAPVC) and heterotaxy syndrome (HTX+) compared with patients with TAPVC without heterotaxy syndrome (HTX-). We aimed to evaluate the longitudinal risk of pulmonary vein reintervention and mortality in HTX + patients.A retrospective review was performed to identify longitudinal interventions in patients with TAPVC seen at a single center from 1995 to 2019. The mean cumulative interventions were described for all patients using the Nelson-Aalen estimator. Survival with TAPVC was described using Kaplan-Meier estimates.A total of 336 patients were identified with TAPVC, of whom 118 (35%) had heterotaxy syndrome. Functional single ventricles were identified in 106 of these 118 HTX + patients (90%) and in 14 of 218 HTX- patients (6%) (P .001). Obstructed TAPVC (OBS+) was present in 49 of 118 HTX + patients (42%) and in 87 of 218 HTX- patients (40%) (P = .89). The median duration of follow-up was 6.5 years. Five-year survival was 69% for HTX+/OBS + patients, 72% for HTX+/OBS- patients, 86% for HTX-/OBS + patients, and 95% for HTX-/OBS- patients (P .0001, log-rank test). The mean number of pulmonary vein interventions at the median follow-up time was greater in the HTX+/OBS + patients compared with HTX+/OBS- patients (mean, 2.0 vs 1.1; P = .030), HTX-/OBS + patients (mean, 1.3; P = .033), and HTX-/OBS- patients (mean, 1.3; P = .029).Among the 4 cohorts, HTX+ was associated with a higher rate of mortality, and HTX+/OBS+ was associated with a greater number of pulmonary vein interventions. This may be due in part to the high prevalence of single ventricle physiology in the HTX + cohort.

AIMS The aim of this study was to compare the efficacy and safety of the Occlutech Figulla Flex II Occluder (OFFII) with the Amplatzer Septal Occluder (ASO) in patients > 8kg undergoing transcatheter ASD closure. METHODS AND RESULTS Randomized, controlled, multi-center prospective clinical trial with randomization 2:1 in favor of the OFFII. Primary efficacy endpoint was the rate of successful device placement and defect closure without major complications at hospital discharge. All data were assessed through a core laboratory. Interim analysis was performed when 70% of the patients were treated to evaluate for noninferiority. From a total of 176 randomized subjects, interim analysis was performed on the first 158 patients (65.2% female) (107 OFFII/51 ASO) undergoing device closure at a median weight of 42 kg (range 13-125 kg). Seventy-six percent (120 patients) completed 6-month follow-up. Successful device placement (first attempt) was achieved in 99.1% of the OFF group vs 90.2% of the ASO group (P

Intracardiac echocardiography has historically been used to guide a limited number of transcatheter cardiac interventions. However, the tremendous advances in structural heart disease interventions in the last decade led to a growing interest in intracardiac echocardiography as a potential alternative to transesophageal echocardiography that mitigates the need for endotracheal intubation. Nonetheless, the scarcity of data, the imperfection of the current probes, and the limited experience among operators prevented a wider adoption of this technology. This review summarizes the contemporary relevant evidence and provides the structural interventionalist with an illustrative guide on the use of intracardiac echocardiography to guide various structural heart interventions.

Objectives We performed a systematic review and meta-analysis of randomized controlled trials to assess the effect of closure of a patent foramen ovale (PFO) compared with medical therapy for the prevention of stroke in patients with prior cryptogenic stroke. Background The role of PFO closure in reducing risk of stroke in patients with prior cryptogenic stroke has been controversial. Methods We searched PubMed, Embase, CINAHL, and CENTRAL for randomized trials investigating PFO closure versus medical therapy. We assessed trial bias and the quality of evidence for main outcomes was rated using GRADE. The primary outcome of interest was the occurrence of stroke. Estimates of effect were pooled with a random-effects model. This study is registered with PROSPERO (CRD42017081579). Results We included five trials, comprising data for 3,440 adults randomized to receive PFO closure (n = 1,829) or medical therapy (n = 1,611). Mean follow-up ranged from 2 years to 5.4 years across the trials. Patients treated with PFO closure had a lower risk of stroke (RR 0.39, 95% CI 0.18-0.88, I2 = 57%, P = 0.02) compared with those treated with medical therapy. Subgroup analyses showed that the beneficial effect of PFO closure on a composite outcome of cerebrovascular events is more pronounced in patients with a large PFO shunt (RR 0.25, 95% CI 0.12-0.54, I2 = 0%, P = 0.0004), male patients (RR 0.34, 95% CI 0.15-0.75, I2 = 36%, P = 0.07), and those aged ≤45 years (RR 0.35, 95% CI 0.15-0.79, I2 = 0%, P = 0.01). Conclusions PFO closure reduced risk of stroke compared with medical therapy. PFO closure is a therapeutic option that should be offered to adults with cryptogenic stroke.

A 3-dimensional printed model derived from a computed tomography dataset was obtained in a patient with tetralogy of Fallot and major aortopulmonary collaterals. The virtual and solid 3-dimensional heart models provided intuitive representation of such complex and highly individual pathologies and allowed comprehensive spatial conceptualization of the cardiac structures. (Level of Difficulty: Advanced.), Graphical abstract, A 3-dimensional printed model derived from a computed tomography dataset was obtained in a patient with tetralogy of Fallot and major aortopulmonary…

We describe the case of a 32-year-old man with history of patent ductus arteriosus (PDA) closed with an Amplatzer device 12 years earlier. Imaging investigations revealed a persistent large PDA and the device migrated in the right pulmonary artery. A new transcatheter PDA occlusion was attempted with optimal post-procedural results. (Level of Difficulty: Advanced.), Graphical abstract, We describe the case of a 32-year-old man with history of patent ductus arteriosus (PDA) closed with an Amplatzer device 12 years earlier…

Covered stent correction of sinus venosus ASDs (SVASD) is a relatively new technique. Challenges include anchoring a sufficiently long stent in a non-stenotic SVC and expanding the stent at the wider SVC-RA junction without obstructing the anomalous right upper pulmonary vein (RUPV). The 10-zig covered CP (CCP) stent has the advantage of being available in lengths of 5–11 cm and dilatable to 34 mm in diameter. Methods An international registry reviewed the outcomes of 10-zig CCP stents in 75 patients aged 11.4–75.9 years (median 45.4) from March 2016. Additional stents were used to anchor the stent in the SVC or close residual shunts in 33/75. An additional stent was placed in 4/5 (80%) with 5/5.5 cm CCPs, 18/29 (62%) with 6 cm CCPs, 5/18 (28%) with 7 cm CCPs, 5/22 (23%) with 7.5/8 cm CCPs and 0/1 with an 11 cm CCP. A “protective” balloon catheter was inflated in the RUPV in 17. Results Early stent embolisation in two patients required surgical removal and defect repair and tamponade was drained in one patient. The CT at 3 months showed occlusion of the RUPV in one patient. Follow up is from 2 months to 5.1 years (median 1.8 years). QP:QS has reduced from 2.5 + 0.5 to 1.2 + 0.36 (P Conclusions 10-zig CCPs of 7–8 cm appear to provider reliable SVASD closure with a low requirement for additional stents. Careful selection of patients and meticulous attention to detail is required to avoid complications.