Isolated ventricular inversion with hypoplastic right ventricle and pulmonary stenosis in an adolescent with situs solitus: a rare combination.

Isolated ventricular inversion with situs solitus is a severe and rare congenital cardiac malformation characterised by an atrioventricular discordance but with ventriculo-arterial concordance. Here, we present the rare case of an adolescent with isolated ventricular inversion and hypoplasia of the left-sided morphological right ventricle and pulmonary stenosis, a first of its kind to be reported in the literature.

Long-term outcome of variety of techniques used to stabilize left ventricular lead in difficult Coronary Sinus Anatomy- A single centre Experience.

INTRODUCTION: Difficult coronary sinus (CS) anatomy may lead to difficulty in optimal left ventricular (LV) lead placement and lead displacements leading to nonresponse to cardiac resynchronization therapy (CRT). METHODS: In this retrospective study, we studied the CRT parameters of devices implanted by single operator during the time period from January 2014 till December 2021, where different off-label techniques were used to place/stabilize LV lead. The technique used to stabilize LV lead, CRT parameters at baseline and follow up were noted for each patient. RESULTS: Out of 133 CRTs implanted during the study period, 23 patients (17.29%) required off-label techniques. Stylet and guidewire retaining techniques were used in 11/23 (47.82%) and 7/23 (30.43%) patients respectively. In two patients, LV lead was jailed using coronary stent to prevent displacement. Two patients had CS stenosis and required balloon dilation while one patient had tortuous posterolateral vein which was straightened using a coronary stent. There was technical failure of 6/23 LV leads (26.08%) with loss of capture, at a median follow up of 44 months (Range: 06 -114 months). Out of these 6 patients, stylet and guidewire retaining techniques were used in 4 and 2 patients respectively. CONCLUSION: Despite having acceptable parameters at implantation, these techniques particularly stylet and guidewire retention, may lead to non- capture of LV lead on long term follow ups. Better LV leads like active fixation leads and conduction system pacing (His Bundle /left bundle branch pacing) should be preferred in difficult CS anatomy.

Incidence of Kawasaki disease among children in Chandigarh, India during 2015-2019: a trend analysis.

Background: Only limited information exists regarding the epidemiology of Kawasaki disease (KD) in low-income and middle-income countries. The present study provides the incidence of KD during 2015-2019 in Chandigarh, north India. Our centre follows the largest KD cohort in India. Methods: Children with KD at Chandigarh diagnosed during January 2015-December 2019 were enrolled in the study. Annual incidence rates were determined using decadal growth rates of the National Census 2011. We computed the incidence of KD in children aged <5, and <15 years. We also undertook linear trend analysis using our incidence data from 1994 to 2019. Findings: During 2015-2019, 83 patients (66 males, 17 females) were diagnosed with KD in Chandigarh. Incidence rates during these 5 years were 5.64, 9.25, 9.11, 9.87, and 9.72/100,000 in children aged <5 years, and 2.65, 4.44, 3.86, 5.07, 4.74/100,000 in children aged <15 years. The median age at diagnosis was 48 months (range: 12 days to 15 years). Compared to previous data (2009-2014), there was a 53.1% increase in annual incidence of KD in children aged <5 years, and a 53.7% increase in children aged <15 years. Coronary artery abnormalities during acute phase were noted in 16.9%, and in 7.2% of patients at 6 weeks of follow-up. The trend analysis indicated a monthly rise of 0.002 cases per 100,000 children aged <5 years, and 0.0165 cases per 100,000 children aged <15 years. Interpretation: The incidence of KD has continued to show an upward trend in Chandigarh over the period 2015-2019. This may indicate a true rise in the occurrence of KD or may reflect better disease ascertainment as a result of greater awareness about KD amongst healthcare professionals. Funding: None.

Pericardial effusion in anti-complement factor H antibody-associated atypical hemolytic uremic syndrome: two case reports.

Hemolytic uremic syndrome (HUS), a cause of pediatric acute kidney injury (AKI), has a spectrum of extra-renal manifestations. While neurological and gastrointestinal system involvement is common, cardiac involvement is rare. This is more so with pericardial involvement, though it has been reported in a handful of HUS cases associated with shiga toxin-producing Escherichia coli (STEC HUS). However, this complication has scarcely been reported in atypical HUS (aHUS) where there is alternate complement abnormality or DKGE (diacylglycerol kinase epsilon) mutation. We describe two children diagnosed with anti-complement factor H (CFH) antibody-associated aHUS who had pericardial involvement. Two boys, one 10-year-old and another 8-year-old, presented with pallor, oliguria and hypertension. They both had microangiopathic haemolytic anemia, thrombocytopenia and AKI suggestive of HUS. Complement workup revealed elevated anti-CFH antibody titres. With a diagnosis of anti-CFH antibody aHUS, they were started on plasmapheresis, pulse methylprednisolone and cyclophosphamide. The first case developed cardiac tamponade during the second week of hospital stay for which he needed pigtail drainage and further immunosuppression with rituximab. He gradually improved and pigtail was removed. The second case presented with pericardial effusion which subsequently resolved during the course of treatment. Thus, our patients developed pericardial effusion, with one of them progressing to life-threatening cardiac tamponade. Therefore, it is prudent that we are aware of this complication while treating children with aHUS.

Mystery still unresolved: Untouched "Blue heart" presenting at 40yrs of age.

The modern-day surgical techniques and strategies have changed the outlook of patients with dextro-transposition of great arteries (d-TGA). The survival of an unrepaired d-TGA into late adulthood is difficult to explain. Even when large intracardiac shunts are present, it still remains a lethal cyanotic congenital heart disease if it is not surgically corrected soon after birth. Here, we report an extremely rare case of d-TGA presenting at 40 years of age, with moderately elevated pulmonary artery pressures and relatively stable symptoms.

Ventricular Septal Defect Pulmonary Atresia: Provisional Bifurcation Strategy for Confluence Stenosis During Patent Ductus Arteriosis Stenting.

Neonates with pulmonary atresia survive only if duct patency is maintained before staged surgical repair. Ductal stenting is an effective alternative to conventional shunt surgery, as it avoids thoracotomy. With today's generation of coronary stents having better profile, flexibility, and trackability, it may be achieved safely, with less difficulty than previously described. The strategy during patent ductus arteriosus (PDA) stenting with trifurcation narrowing is a real challenge. We present an underweight newborn with ventricular septal defect pulmonary atresia, restrictive PDA, and bilateral branch stenosis at the ostia. We performed a successful PDA stenting as well as addressed the ostia of branch pulmonary arteries.

Transcatheter closure of Patent ductus arteriosus in a child with IVC interruption through standard femoral access: a case report.

BACKGROUND: Portsmann and co. performed the first PDA device closure in 1967. The technique and the devices used have evolved since then and are the first choice in managing anatomically feasible patent ductus arteriosus (PDA) for the last 20 years. Though catheter-based closure of PDA is generally a simple procedure, there are instances when the interventionist faces challenges, especially in smaller children, with syndromic features and venous anomalies even when defects are small and pulmonary artery pressures are normal. Although the femoral vein is the relatively risk-free standard access, internal jugular vein, femoral artery, and transhepatic IVC can be used to close the PDA in different anomalies. The rare venous anomaly of infrahepatic interruption of the IVC with azygous continuation poses technical challenges when percutaneous closure of PDA was attempted through the standard femoral access. CASE PRESENTATION: We report a rare case of PDA device closure in a syndromic child with a short neck having interrupted IVC via femoral-azygous venous approach. CONCLUSION: Knowledge of the IVC course and its anomalies should be known to the operator before the percutaneous closure of PDA. Although other approaches are available, femoral vein approach can be used in case of interrupted IVC for percutaneous closure of PDA.

An Unusual Combination of Truncus Arteriosus, Interrupted Aortic Arch, and Hypoplastic Left Ventricle.

Truncus arteriosus (common arterial trunk) is known to be associated with interrupted aortic arch in 10% to 15% of cases. However, the association of either of these lesions with a hypoplastic left ventricle is rare. The combination of all three of these lesions along with an intact interventricular septum is virtually unknown with only two cases reported in the literature. We report such a case with its anatomical and surgical management aspects.