Anomalous origin of a pulmonary artery in a teenager with tetralogy of Fallot with associated double aortic arch and coronary arteriovenous fistula.

We report the case of a 14-year-old female who had tetralogy of Fallot along with anomalous origin of the left pulmonary artery from the ascending aorta with co-dominant double aortic arch forming a complete vascular ring compressing the oesophagus along with a left main coronary artery to right ventricular outflow tract fistula. She underwent surgical correction without conduit placement.

Hsp90 activator Aha1 drives production of pathological tau aggregates.

The microtubule-associated protein tau (MAPT, tau) forms neurotoxic aggregates that promote cognitive deficits in tauopathies, the most common of which is Alzheimer's disease (AD). The 90-kDa heat shock protein (Hsp90) chaperone system affects the accumulation of these toxic tau species, which can be modulated with Hsp90 inhibitors. However, many Hsp90 inhibitors are not blood-brain barrier-permeable, and several present associated toxicities. Here, we find that the cochaperone, activator of Hsp90 ATPase homolog 1 (Aha1), dramatically increased the production of aggregated tau. Treatment with an Aha1 inhibitor, KU-177, dramatically reduced the accumulation of insoluble tau. Aha1 colocalized with tau pathology in human brain tissue, and this association positively correlated with AD progression. Aha1 overexpression in the rTg4510 tau transgenic mouse model promoted insoluble and oligomeric tau accumulation leading to a physiological deficit in cognitive function. Overall, these data demonstrate that Aha1 contributes to tau fibril formation and neurotoxicity through Hsp90. This suggests that therapeutics targeting Aha1 may reduce toxic tau oligomers and slow or prevent neurodegenerative disease progression.

Transverse Sternal Split: a Safe Mini-invasive Approach for Perventricular Device Closure of Ventricular Septal Defect.

OBJECTIVE:: Perventricular device closure of ventricular septal defect through midline sternotomy avoids the cardiopulmonary bypass, however, lacks the cosmetic advantage. Perventricular device closure of ventricular septal defect with transverse split sternotomy was performed to add the cosmetic advantage of mini-invasive technique. METHODS:: Thirty-six pediatric patients with mean age 7.14±3.24 months and weight 5.00±0.88 kg were operated for perventricular device closure of ventricular septal defect through transverse split sternotomy in 4th intercostal space under transesophageal echocardiography guidance. In case of failure or complication, surgical closure of ventricular septal defect was performed through the same incision with cervical cannulation of common carotid artery and internal jugular vein for commencement of cardiopulmonary bypass. All the patients were postoperatively followed, and then discharged from hospital due to their surgical outcome, morbidity and mortality. RESULTS:: Procedure was successful in 35 patients. Two patients developed transient heart block. Surgical closure of ventricular septal defect was required in one patient. Mean duration of ventilation was 11.83±3.63 hours. Mean intensive care unit and hospital stay were 1.88±0.74 days and 6.58±1.38 days, respectively. There was no in-hospital mortality. A patient died one day after hospital discharge due to arrhythmia. No patients developed wound related, vascular or neurological complication. In a mean follow-up period of 23.3±18.45 months, all 35 patients were doing well without residual defect with regression of pulmonary artery hypertension as seen on transthoracic echocardiography. CONCLUSION:: Transverse split sternotomy incision is a safe and effective alternative to a median sternotomy for perventricular device closure of ventricular septal defect with combined advantage of better cosmetic outcomes and avoidance of cardiopulmonary bypass.

Human cyclophilin 40 unravels neurotoxic amyloids.

The accumulation of amyloidogenic proteins is a pathological hallmark of neurodegenerative disorders. The aberrant accumulation of the microtubule associating protein tau (MAPT, tau) into toxic oligomers and amyloid deposits is a primary pathology in tauopathies, the most common of which is Alzheimer's disease (AD). Intrinsically disordered proteins, like tau, are enriched with proline residues that regulate both secondary structure and aggregation propensity. The orientation of proline residues is regulated by cis/trans peptidyl-prolyl isomerases (PPIases). Here we show that cyclophilin 40 (CyP40), a PPIase, dissolves tau amyloids in vitro. Additionally, CyP40 ameliorated silver-positive and oligomeric tau species in a mouse model of tau accumulation, preserving neuronal health and cognition. Nuclear magnetic resonance (NMR) revealed that CyP40 interacts with tau at sites rich in proline residues. CyP40 was also able to interact with and disaggregate other aggregating proteins that contain prolines. Moreover, CyP40 lacking PPIase activity prevented its capacity for disaggregation in vitro. Finally, we describe a unique structural property of CyP40 that may permit disaggregation to occur in an energy-independent manner. This study identifies a novel human protein disaggregase and, for the first time, demonstrates its capacity to dissolve intracellular amyloids.

Transverse sternal split: a safe mini-invasive approach for perventricular device closure of ventricular septal defect

Abstract Objective: Perventricular device closure of ventricular septal defect through midline sternotomy avoids the cardiopulmonary bypass, however, lacks the cosmetic advantage. Perventricular device closure of ventricular septal defect with transverse split sternotomy was performed to add the cosmetic advantage of mini-invasive technique. Methods: Thirty-six pediatric patients with mean age 7.14±3.24 months and weight 5.00±0.88 kg were operated for perventricular device closure of ventricular septal defect through transverse split sternotomy in 4th intercostal space under transesophageal echocardiography guidance. In case of failure or complication, surgical closure of ventricular septal defect was performed through the same incision with cervical cannulation of common carotid artery and internal jugular vein for commencement of cardiopulmonary bypass. All the patients were postoperatively followed, and then discharged from hospital due to their surgical outcome, morbidity and mortality. Results: Procedure was successful in 35 patients. Two patients developed transient heart block. Surgical closure of ventricular septal defect was required in one patient. Mean duration of ventilation was 11.83±3.63 hours. Mean intensive care unit and hospital stay were 1.88±0.74 days and 6.58±1.38 days, respectively. There was no in-hospital mortality. A patient died one day after hospital discharge due to arrhythmia. No patients developed wound related, vascular or neurological complication. In a mean follow-up period of 23.3±18.45 months, all 35 patients were doing well without residual defect with regression of pulmonary artery hypertension as seen on transthoracic echocardiography. Conclusion: Transverse split sternotomy incision is a safe and effective alternative to a median sternotomy for perventricular device closure of ventricular septal defect with combined advantage of better cosmetic outcomes and avoidance of cardiopulmonary bypass.

Cervical Cannulation for Surgical Repair of Congenital Cardiac Defects in Infants and Small Children.

INTRODUCTION:: The biggest challenge faced in minimally invasive pediatric cardiac surgery is cannulation for cardiopulmonary bypass. Our technique and experience of cervical cannulation in infants and small children for repair of congenital cardiac defects is reported in this study. METHODS:: From January 2013 to June 2015, 37 children (22 males) with mean age of 17.97±8.63 months and weight of 8.06±1.59 kg were operated on for congenital cardiac defects through right lateral thoracotomy. The most common diagnosis was ventricular septal defect (18 patients). In all patients, right common carotid artery, right internal jugular vein and inferior vena cava were cannulated for institution of cardiopulmonary bypass and aorta was cross clamped through right 2nd intercostal space. RESULTS:: There were no deaths or any major complications related to cervical cannulation. Common carotid artery cannulation provided adequate arterial inflow while internal jugular vein with inferior vena cava provided adequate venous return in all patients. No patient required conversion to sternotomy or developed vascular, neurological or wound related complications. Three patients had residual lesions (small leak across ventricular septal defect patch-2, Grade II left atrio-ventricular valve regurgitation-1) and one patient had mild left ventricular dysfunction. At discharge, both common carotid artery and internal jugular vein were patent on color Doppler ultrasonography in all patients. In a mean follow-up period of 11.4±2.85 months, all patients were doing well. No patient had any wound related, neurological or vascular complication. No patient had residual leak across ventricular septal defect patch. CONCLUSION:: Cervical cannulation of common carotid artery and internal jugular vein is a safe, reliable, efficient and quick method for institution of cardiopulmonary bypass in minimally invasive pediatric cardiac surgery.

Cervical cannulation for surgical repair of congenital cardiac defects in infants and small children

Abstract INTRODUCTION: The biggest challenge faced in minimally invasive pediatric cardiac surgery is cannulation for cardiopulmonary bypass. Our technique and experience of cervical cannulation in infants and small children for repair of congenital cardiac defects is reported in this study. METHODS: From January 2013 to June 2015, 37 children (22 males) with mean age of 17.97±8.63 months and weight of 8.06±1.59 kg were operated on for congenital cardiac defects through right lateral thoracotomy. The most common diagnosis was ventricular septal defect (18 patients). In all patients, right common carotid artery, right internal jugular vein and inferior vena cava were cannulated for institution of cardiopulmonary bypass and aorta was cross clamped through right 2nd intercostal space. RESULTS: There were no deaths or any major complications related to cervical cannulation. Common carotid artery cannulation provided adequate arterial inflow while internal jugular vein with inferior vena cava provided adequate venous return in all patients. No patient required conversion to sternotomy or developed vascular, neurological or wound related complications. Three patients had residual lesions (small leak across ventricular septal defect patch-2, Grade II left atrio-ventricular valve regurgitation-1) and one patient had mild left ventricular dysfunction. At discharge, both common carotid artery and internal jugular vein were patent on color Doppler ultrasonography in all patients. In a mean follow-up period of 11.4±2.85 months, all patients were doing well. No patient had any wound related, neurological or vascular complication. No patient had residual leak across ventricular septal defect patch. CONCLUSION: Cervical cannulation of common carotid artery and internal jugular vein is a safe, reliable, efficient and quick method for institution of cardiopulmonary bypass in minimally invasive pediatric cardiac surgery.

Effect of Prime Blood Storage Duration on Clinical Outcome After Pediatric Cardiac Surgery.

BACKGROUND: In this study, we tested the hypothesis that in pediatric patients undergoing cardiac surgery using cardiopulmonary bypass (CPB) with blood prime, the storage duration of the packed red blood cells (PRBCs) used in prime led to differences in postoperative complications and metabolic profiles of the patients. METHODS: For this prospective observational study we included 400 pediatric patients undergoing cardiac operations using CPB and requiring PRBCs prime. To study the effect of storage duration of PRBCs on postoperative morbidity, mortality, and metabolic profile, patients were divided into four groups (based on storage duration of PRBCs used in prime). Group 1: ≤7 days, group 2: 8 to 14 days, group 3: 15 to 21 days, and group 4: >21 days. RESULTS: On univariate analysis, patients transfused with PRBCs stored >14 days had significantly higher incidence of postoperative complications, for example, liver dysfunction, hematological complications, sepsis, and multiorgan failure. However, after regression analysis and adjusting for the other confounder's effects, no significant association was found between storage duration of PRBCs and postoperative complications and mortality. Metabolic profile of PRBCs was observed to become deranged with increasing duration of storage. This, however, improved to near physiological range early after the initiation of CPB and remained normal one hour after weaning from CPB, irrespective of the storage duration. CONCLUSION: Storage duration of PRBCs used for priming the pediatric CPB circuit neither affects the metabolic profile of the patients on CPB or early after surgery, nor it has any association with postoperative complications and mortality.

Invasive pericardial hydatid cyst: Excision of multiple huge cysts.

Cardiac hydatid cyst is rare even in endemic countries, and poses a therapeutic challenge due to varying presentation and unpredictable pre-, peri-, and postoperative complications. We herein present a case of multiple, multifocal, huge pericardial hydatid cyst, with invasion into the left ventricle and main pulmonary artery in a young male patient, presented with atypical chest pain.

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Adulthood: Challenges and Outcomes.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is an extremely rare, potentially fatal, congenital anomaly with a high mortality rate in the first year of life. It occurs rarely in adulthood and may appear with malignant ventricular a rrhythmia or sudden death. We report a case of a 49-year-old woman with ALCAPA who presented with dyspnea on exertion. Management was coronary artery bypass grafting to the left anterior descending artery and obtuse marginal arteries, closure of the left main coronary artery ostium, and reestablishment of the dual coronary artery system.

Recurrent Left Atrial Myxoma in a Young Patient: A Rare Entity.

Recurrence of atrial myxoma arising from the site other than inter-atrial septum is quite rare, which is more common in familial than sporadic cases. We here in present a case of 15-year-old young female who presented with recurrence of left atrial (LA) myxoma from unusual site - posterior LA wall after 3 years without any constitutional symptoms, which is the hallmark of recurrence. Complete removal of underlying atrial septum with atrial wall for recurrence prevention is the dictum in primary operation for tumour removal.