Transcatheter closure of sinus venosus defect: First-in-human implant of a dedicated self-expanding VB stent system.

The paper describes the first-in-human use of a dedicated, self-expandable covered stent system (VB stent) for closure of sinus venosus defects.

Does a Change in Device Design Alter Device Size Selection? A Comparison of Conventional and Occlutech Duct Occluder Designs.

AIM: The Occlutech duct occluder (ODO) with a novel design has been available for closure of patent ductus arteriosus (PDA) since 2011. Available data on initial experience with the ODO have shown that operators continue to choose device sizes based on their experience with conventional duct occluders (CDO). This study postulated that occlusion of the pulmonary arterial (PA) end of the ductus should achieve satisfactory PDA closure without additional complications. Accordingly, the size of the ODO was chosen such that the larger PA diameter in the new design exceeded the PA end of the ductus by 2-3 mm and 2-4 mm for normotensive and hypertensive ducts, respectively. It sought to examine the feasibility and safety of such an approach, and compared ODO and CDO devices with respect to device sizes deployed, to ascertain if the newer design had any advantages. METHODS: This prospective study enrolled 105 infants, children and a few adults with various duct morphologies and PA pressures for ductal closure using the ODO from 2018-2020. The control group consisted of 105 ducts closed with CDO. A comparison of the two groups with respect to duct diameter and implanted device sizes was performed using appropriate statistical software. RESULTS: The study found that 40% of the ducts had moderate-to-severe pulmonary hypertension. Most ducts measured between 3-4 mm in both groups. The mean weight of the subjects in both groups was 12 kg. Although there was no change in the way that duct occluders were chosen in both groups (2-3 mm over the pulmonary artery diameter), ODO could be significantly downsized in most ducts measuring between 2.5-6.4 mm in diameter (p<0.05). CONCLUSIONS: This study found successful closure of relatively larger PDA in infants and children using smaller ODO compared with CDO, without embolisation or aortic narrowing. It also demonstrated that a different device size selection technique may be safely employed with the ODO.

Off-label use of duct occluder devices to close hemodynamically significant perimembranous ventricular septal defects: A multicenter experience.

OBJECTIVE: We sought to evaluate the feasibility, technical aspects, and outcome of transcatheter perimembranous ventricular septal defect (pmVSD) closure using duct occluder devices with a single retention disc. BACKGROUND: Use of duct occluder devices to close pmVSD seems a promising alternative therapy. However, limited data exist on this technique. METHODS: From 2010 to 2016, 222 patients (female 47.7%) were identified from databases of five participating institutions in whom pmVSD closure was attempted using an Amplatzer Duct Occluder I or Lifetech duct occluder device. RESULTS: Patients ranged in age from 0.7 to 52 years (median, 7.0 years) and in weight from 4.0 to 70 kg (median, 18.0 kg). The mean size of the VSD was 6.8 ± 2.2 mm. A large defect (> 6 mm) was present in 137 patients (61.7%). Device closure was successful in 218 patients (98.2%). The 10/8 mm device was used in most patients (n = 85, 38.3%), and the vascular approach was from the femoral vein in 169 patients (76.1%). There were 18 early complications in 17/218 patients (7.8%). Three patients (1.4%) developed complete heart block (transient n = 2; requiring permanent pacing n = 1). Median follow-up was 6 months (6 months-6 years). A mild residual shunt was seen in 10 patients at 6 months follow-up. CONCLUSIONS: The immediate results of transcatheter pmVSD closure using a duct occluder device with a single retention disc are promising. It is an effective technique with a lower rate of complications than for other currently available devices.

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.

Spectrum of cyanotic congenital heart disease diagnosed by echocardiographic evaluation in patients attending paediatric cardiology clinic of a tertiary cardiac care centre.

BACKGROUND: Cyanotic CHD comprises up to 25% of cases of all causes of CHD. RATIONALE: There is lack of data about the present spectrum of congenital cyanotic heart disease in the paediatric age group. OBJECTIVE: The present study was undertaken to determine the spectrum of patients with congenital cyanotic heart disease in the paediatric age group in tertiary paediatric cardiac care clinic. DESIGN: Prospective observational study. SETTING: Paediatric cardiac clinic of a tertiary cardiac care centre. METHODS: All children aged 0-18 years with suspected cyanotic CHD were provisionally included in this study. They underwent a thorough echocardiographic evaluation, and those patients who had definitive diagnosis of congenital cyanotic heart disease were included for final analysis. RESULTS: A total of 119 children met the inclusion criteria. Tetralogy of Fallot and its variant were the most common congenital cyanotic heart disease with proportion of about 44%. Other common malformations were double outlet right ventricle (14%), pulmonary atresia with ventricular septal defect (8%), total anomalous pulmonary venous connection (7%), d-transposition of the great arteries (9%), tricuspid valve anomalies--tricuspid atresia and Ebstein's anomaly--hypoplastic left-heart syndrome, truncus arteriosus, and complex CHD such as single ventricle. CONCLUSION: Tetralogy of Fallot and its variants were the most common cyanotic heart disease diagnosed in our patients. As there were a significant proportion of cases with complex cyanotic CHD, paediatric cardiologists should be familiar with the diagnosis and management of all these complex congenital malformations of the heart.

Transcatheter closure of perimembranous ventricular septal defects with ductal occluders.

BACKGROUND: To study the feasibility and complications associated with the use of ductal occluders for closure of perimembranous ventricular septal defects. METHODS: A total of 126 patients, ranging from 1 to 41 years of age (median - 8 years), underwent closure of ventricular septal defects from August 2010 to April 2013. Small- and moderate-sized defects were closed using first-generation Patent ductus arteriosus occluders or Amplatzer Duct Occluder-II. Patients were followed up for the development of complications such as heart block, aortic regurgitation, and tricuspid regurgitation. RESULTS: Patent ductus arteriosus occluders were used in 81 patients, and the Amplatzer Duct Occluder-II device in 45 patients. The devices were successfully deployed in 99.2% of the cases. One patient had embolisation of an Amplatzer Duct Occluder-II device soon after deployment. There was one case of transient complete heart block (0.8%) needing temporary pacing, and two cases of isoarrhythmic atrioventricular dissociation (1.6%). One patient developed late-onset complete heart block 15 months after the procedure and underwent permanent pacemaker implantation. There were no instances of new-onset aortic regurgitation. New-onset mild tricuspid regurgitation was seen in two patients. Of the patients, three had small residual shunts on follow-up, without haemolysis. CONCLUSIONS: Duct occluders can be used to effectively close small- and moderate-sized ventricular septal defects. The incidence of complete heart block and valvular regurgitations are much less than reported with other devices, and they are cost-effective.

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.

Left sided Scimitar syndrome.

The images depict a rare case of Scimitar syndrome involving the left lower pulmonary vein.

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.

Deep Learning Algorithms to Detect Murmurs Associated With Structural Heart Disease.

Background The success of cardiac auscultation varies widely among medical professionals, which can lead to missed treatments for structural heart disease. Applying machine learning to cardiac auscultation could address this problem, but despite recent interest, few algorithms have been brought to clinical practice. We evaluated a novel suite of Food and Drug Administration-cleared algorithms trained via deep learning on >15 000 heart sound recordings. Methods and Results We validated the algorithms on a data set of 2375 recordings from 615 unique subjects. This data set was collected in real clinical environments using commercially available digital stethoscopes, annotated by board-certified cardiologists, and paired with echocardiograms as the gold standard. To model the algorithm in clinical practice, we compared its performance against 10 clinicians on a subset of the validation database. Our algorithm reliably detected structural murmurs with a sensitivity of 85.6% and specificity of 84.4%. When limiting the analysis to clearly audible murmurs in adults, performance improved to a sensitivity of 97.9% and specificity of 90.6%. The algorithm also reported timing within the cardiac cycle, differentiating between systolic and diastolic murmurs. Despite optimizing acoustics for the clinicians, the algorithm substantially outperformed the clinicians (average clinician accuracy, 77.9%; algorithm accuracy, 84.7%.) Conclusions The algorithms accurately identified murmurs associated with structural heart disease. Our results illustrate a marked contrast between the consistency of the algorithm and the substantial interobserver variability of clinicians. Our results suggest that adopting machine learning algorithms into clinical practice could improve the detection of structural heart disease to facilitate patient care.

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.

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.

Congenital portosystemic shunt with multiple splenic artery aneurysms: Reversing pulmonary hypertension and preventing aneurysm rupture.

We report an unusual occurrence of multiple splenic artery aneurysms and splenomegaly in a young woman with severe pulmonary hypertension, secondary to a congenital portosystemic shunt (CPS). The splenic artery was occluded using an Amplatzer Duct Occluder-II device, and closure of the large intrahepatic CPS was achieved using a muscular ventricular septal defect occluder. There was resolution of splenomegaly with normal pulmonary artery pressures, a few months after the procedure.

Transient Complete Blindness Due to Metformin-Associated Lactic Acidosis (MALA) Reversed with Hemodialysis.

BACKGROUND Metformin-associated lactic acidosis (MALA) is a relatively rare adverse effect of metformin therapy. It usually occurs in patients with metformin overdose or in those with underlying acute and/or chronic conditions resulting in impaired lactate metabolism. Among these, patients with acute kidney injury, heart failure, sepsis, and cirrhosis are the most vulnerable to MALA, even in the setting of appropriate therapy. The most common symptoms of MALA include nausea, vomiting, diarrhea, encephalopathy, hypothermia, respiratory failure, and hypotension. Blindness is a dramatic symptom that has been rarely reported with MALA. CASE REPORT We report a case of 78-year-old woman with history of type 2 diabetes mellitus with nephropathy for which she was treated with metformin and insulin. She developed nausea, non-bloody emesis, and watery diarrhea, which led to dehydration, anion gap metabolic acidosis due to hyperlactatemia, and acute kidney injury (AKI). She was hospitalized for i.v. hydration and further management when she suddenly developed blindness. The diagnostic work-up ruled out central causes and her symptoms resolved briefly after continuous renal replacement therapy (CRRT) was initiated, confirming the diagnosis of MALA. CONCLUSIONS By reporting this case, we wish to increase awareness about MALA symptoms, its diagnosis, and the importance of early recognition and initiation of treatment among clinicians involved in the care of patients with chronic kidney disease (CKD) who take metformin for diabetes mellitus. Although rare, this metformin adverse effect can present dramatically and can be distressing for both patient and treating team.

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.

Congenitally corrected transposition of the great arteries in a patient with cor triatriatum: a rare combination.

Congenitally corrected transposition of the great arteries is a rare anomaly that is thought to arise from a defect in looping of the primitive cardiac tube. Cor triatriatum is another rare congenital cardiac anomaly due to faulty incorporation of the common pulmonary vein into the left atrium. We present a rare case comprising both disorders in one patient.

Personnel scheduling during Covid-19 pandemic.

This paper addresses a real-life personnel scheduling problem in the context of Covid-19 pandemic, arising in a large Italian pharmaceutical distribution warehouse. In this case study, the challenge is to determine a schedule that attempts to meet the contractual working time of the employees, considering the fact that they must be divided into mutually exclusive groups to reduce the risk of contagion. To solve the problem, we propose a mixed integer linear programming formulation (MILP). The solution obtained indicates that optimal schedule attained by our model is better than the one generated by the company. In addition, we performed tests on random instances of larger size to evaluate the scalability of the formulation. In most cases, the results found using an open-source MILP solver suggest that high quality solutions can be achieved within an acceptable CPU time. We also project that our findings can be of general interest for other personnel scheduling problems, especially during emergency scenarios such as those related to Covid-19 pandemic.

Delayed presentation of partial anomalous systemic venous connection.

Anomalies of systemic venous connections are extremely rare. We describe the case of an asymptomatic 29-year-old woman who was found to have systemic desaturation in the peripartum period and referred to us for suspected cyanotic heart disease. She was diagnosed to have hemianomalous systemic venous connection of the inferior vena cava (IVC) into the left atrium (LA). Transesophageal echocardiogram with contrast diagnosed anomalous connection of the IVC to the LA, further confirmed by computed tomography and conventional angiography. The patient underwent successful surgical correction with an uneventful postoperative course.

"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.