Ebstein's anomaly with total anomalous pulmonary venous connection in an adult.

Ebstein's anomaly is a rare congenital cardiac disease which is often associated with various other cardiac anomalies. However, its association with total anomalous pulmonary venous connection is extremely rare with only one case reported so far in the English literature. We report the first successful surgical correction of both Ebstein's anomaly and total anomalous pulmonary venous connection in an adult patient. Such complex scenarios may pose unique challenges in management which require a judicious approach. Supplementary information: The online version contains supplementary material available at 10.1007/s12055-023-01664-8.

Anomalous origin of branch pulmonary artery from the aorta: Current challenges in the management.

Anomalous origin of a branch pulmonary artery from the aorta is a rare congenital anomaly that requires early surgery to prevent pulmonary vascular disease. The rate of reintervention after surgery remains high. Many aspects of the management could be improved such as assessment of operability in late presenters, selection of suitable surgical technique in each case, and prevention of anastomotic complications. We report the series of 10 patients who were operated for this anomaly. We aim to focus on the current challenges in the management of this condition.

Congenital systemic arteriovenous fistulas: Interventional strategies and embryological perspectives.

Background: Data on congenital systemic arteriovenous fistulas are largely based on individual case reports. A true systemic arteriovenous fistula needs to be differentiated from other vascular malformations like capillary or venous hemangiomas, which are far more common. Objectives: We sought to identify the varied symptoms, diagnostic challenges, describe interventional treatment options, and postulate an embryological basis for this uncommonly described entity. Methods: This is a descriptive study of a cohort of systemic arteriovenous fistulas seen in the department of pediatric cardiology at a tertiary cardiac institute from 2010 to 2020, with prospective medium-term follow-up. A total of seven cases were identified. The diagnosis was confirmed by computed tomographic imaging, magnetic resonance angiography, or conventional angiography. Results: All were successfully closed using duct occluders or embolization coils with no recurrence in six cases over a median duration of follow-up of 48 months (interquartile range: 16; 36-52 months). Four of the seven cases underwent follow-up imaging using echocardiography or ultrasound. Conclusion: The incidence of congenital systemic arteriovenous fistulas is low and accounted for 0.009% of pediatric outpatients seen over 10 years at our institute. The spectrum of clinical presentation varies from an innocuous swelling or a pulsating mass to frank heart failure. Strong clinical suspicion and advanced imaging modalities have helped identify some hitherto undescribed connections. Large malformations with multiple communications may persist or recur despite transcatheter closure.

Axillary artery: An unanticipated choice of access in the cath lab!

Ventricular septal defects are increasingly being closed by transcatheter technique, with lesser morbidity and shorter hospital stay compared to open heart surgery. We report a case of embolization of a duct occluder deployed in a posterior muscular septal defect. The rare site of embolization necessitated an unusual approach for retrieval prior to subsequent closure using a double-disc device.

"Double Trouble": Severe Meningoencephalitis Due to Borrelia burgdorferi and Powassan Virus Co-Infection Successfully Treated with Intravenous Immunoglobulin.

BACKGROUND Powassan virus (POWV) is an emerging tick-borne flavivirus transmitted to humans by ticks. While infection is asymptomatic in some people, others develop life-threatening encephalitis with high mortality rates. Co-infection between POWV and Borrelia burgdorferi is rare despite the fact that both pathogens can be transmitted through the same tick vector, Ixodes scapularis. It is unclear if co-infection leads to more severe clinical presentation and worse outcome. CASE REPORT A 76-year-old Wisconsin man was admitted for meningoencephalitis complicated by hypoxemic and hypercapnic respiratory failure requiring endotracheal intubation. The patient had no known tick bites but lived in a heavily wooded area. Extensive work-up for infectious, autoimmune, and paraneoplastic causes was positive for Borrelia burgdorferi and Powassan virus infection (POWV). Following treatment with ceftriaxone for neuroborreliosis and supportive care for POWV infection, the patient failed to improve. Intravenous immunoglobulins (IVIG) were started empirically, and the patient attained gradual neurological improvement and was successfully extubated. CONCLUSIONS Treatment for POWV infection is supportive, and at this time there are no approved targeted antivirals for this disease. At this time, it remains unclear if co-infection with 2 pathogens leads to a more severe clinical presentation and higher mortality. In the absence of contraindications, IVIG might be beneficial to patients with POWV infection who are not improving with supportive care.

Deep learning for detection and segmentation of artefact and disease instances in gastrointestinal endoscopy.

The Endoscopy Computer Vision Challenge (EndoCV) is a crowd-sourcing initiative to address eminent problems in developing reliable computer aided detection and diagnosis endoscopy systems and suggest a pathway for clinical translation of technologies. Whilst endoscopy is a widely used diagnostic and treatment tool for hollow-organs, there are several core challenges often faced by endoscopists, mainly: 1) presence of multi-class artefacts that hinder their visual interpretation, and 2) difficulty in identifying subtle precancerous precursors and cancer abnormalities. Artefacts often affect the robustness of deep learning methods applied to the gastrointestinal tract organs as they can be confused with tissue of interest. EndoCV2020 challenges are designed to address research questions in these remits. In this paper, we present a summary of methods developed by the top 17 teams and provide an objective comparison of state-of-the-art methods and methods designed by the participants for two sub-challenges: i) artefact detection and segmentation (EAD2020), and ii) disease detection and segmentation (EDD2020). Multi-center, multi-organ, multi-class, and multi-modal clinical endoscopy datasets were compiled for both EAD2020 and EDD2020 sub-challenges. The out-of-sample generalization ability of detection algorithms was also evaluated. Whilst most teams focused on accuracy improvements, only a few methods hold credibility for clinical usability. The best performing teams provided solutions to tackle class imbalance, and variabilities in size, origin, modality and occurrences by exploring data augmentation, data fusion, and optimal class thresholding techniques.

A Fatal Case of Kaposi Sarcoma Immune Reconstitution Syndrome (KS-IRIS) Complicated by Kaposi Sarcoma Inflammatory Cytokine Syndrome (KICS) or Multicentric Castleman Disease (MCD): A Case Report and Review.

BACKGROUND Kaposi Sarcoma Inflammatory Cytokine Syndrome (KICS) is a relatively new syndrome described in patients co-infected with Human Immunodeficiency Virus (HIV) and Kaposi Sarcoma (KS) Herpes Virus (KSHV). KICS clinically resembles Multicentric Castleman disease (MCD) and both present with various degrees of lymphadenopathy, pancytopenia, HIV and KSHV viremia, and signs of systemic inflammatory syndrome (SIRS). KICS has higher mortality than MCD and is rarely recognized. Lymph node, bone marrow, or splenic biopsy can help differentiate between the 2 entities. CASE REPORT We present a case of a 28-year-old African American man with advanced acquired immunodeficiency syndrome (AIDS) who was diagnosed with disseminated pulmonary and cutaneous KS. Following initiation of combined antiretroviral therapy (cART), rapid immunologic recovery occurred followed by rapid clinical deterioration (IRIS) with multiorgan failure, overwhelming SIRS, and ultimately death. The patient's symptoms, signs, and laboratory findings during this episode could not be solely explained by KS-IRIS, and MCD versus KICS was diagnosed. CONCLUSIONS SIRS in patients with uncontrolled HIV viremia and CD4 lymphopenia has a broad differential diagnosis, including infectious and noninfectious causes. It encompasses sepsis due to common bacterial pathogens, various HIV-specific opportunistic infections, immunological conditions such as hemophagocytic lymphohistiocytosis (HLH), and IRIS, malignancies such as primary effusion lymphoma (PEL) and MCD, and finally KCIS. Clinicians involved in treatment of these patients should have a high index of suspicion for less-known and recently described syndromes such as KICS to recognize it early and initiate timely treatment, which might improve the high mortality associated with KICS.

Transcatheter closure of ruptured sinus of Valsalva: Different techniques and mid-term follow-up.

OBJECTIVES: The aim of the present study was to study the feasibility and approaches to device closure of ruptured sinus of Valsalva (RSOV). We have compared the advantages and disadvantages of different devices and techniques and discussed complications which might occur during or after the procedure. BACKGROUND: There are only a few case series of transcatheter closure of RSOV, with most cases being traditionally referred for surgery. We have employed different devices used for treatment of congenital shunt lesions to achieve closure. Reasons for failure and need for surgical referral have also been analyzed. METHODS: Transcatheter closure was attempted in a total of 25 patients of RSOV who presented to us over a period of 4 years. Different devices were deployed using antegrade and retrograde approaches. All patients have been on regular follow-up. RESULTS: The procedural success rate was 84%. Two patients presenting in cardiogenic shock were effectively treated by device closure. There was one case of device embolization and two patients had residual leak. Three patients were referred for surgery. There was one case of device induced severe aortic regurgitation. Ventricular septal defect and RSOV were closed simultaneously in one patient. CONCLUSIONS: Different approaches and devices may be used for transcatheter closure of RSOV. We prefer antegrade closure using duct occluders in most cases while muscular VSD occluders may be used in select situations. ADO II devices should hardly ever be used on their own for device closure of RSOV.

Dilated cardiomyopathy being the presenting manifestation of Takayasu arteritis and treated with renal angioplasty.

Dilated cardiomyopathy (DCM) is an uncommon complication of Takayasu arteritis (TA) with a prevalence of about 6%. We report a case of 14-year-old girl who presented with dyspnea, bipedal edema, loss of weight, and easy fatigability for three months. She was being treated for DCM for the same duration. Clinical examination revealed absence of both upper limb pulses. Echocardiography revealed features of DCM with severe biventricular dysfunction (ejection fraction 30%). Computed tomography angiogram confirmed the diagnosis of TA and revealed the presence of bilateral renal artery stenosis. Bilateral renal angioplasty was done, and immunosuppressant therapy with oral prednisolone and weekly oral methotrexate was started.

d-Transposition of the great arteries in a 12-year-old child: is arterial switch still an option?

Arterial switch surgery for d-transposition of great arteries is usually performed in the first 2 weeks of life before the left ventricle regresses. The arterial switch surgery that helps achieve anatomic and physiologic correction of this condition has better long-term outcomes than other surgical approaches. The procedure may still be attempted at an older age where the left ventricle has not regressed. We report a rare case of a 12-year-old child with d-transposition of great arteries, a remote muscular ventricular septal defect and isolated valvar pulmonic stenosis, which was corrected by an arterial switch surgery.