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Interrupted aortic arch (IAA) is a congenital anomaly characterized by a discontinuity between the ascending and descending aorta. We present a 27-year-old male patient who had presented with Stanford Type A aortic dissection and was subsequently found to have IAA on computed tomography aortogram. The patient subsequently underwent emergency surgery for the repair of aortic dissection. Although few studies with IAA and coexistent Stanford type-B aortic dissection have been described, there is no reported case in literature on IAA with Stanford Type A aortic dissection.

Immediately after coronary artery bypass grafting (CABG) surgery, the left ventricular (LV) function measured by peak LV global longitudinal strain (GLS) is influenced by general anesthesia, positive pressure ventilation, myocardial protection technique used, the effect of myocardial revascularization, and the effect of inotropic and vasopressor agent used. Therefore, we intended to perform a study evaluating the change in the LV function, measured by peak LV GLS, from the immediate post-operative period to the immediate pre-hospital discharge period (7–10 days after surgery) in patients undergoing uneventful, on-pump CABG surgery and having an uneventful early post-operative course. In patients aged 30–65 years, having LV ejection fraction (LVEF) >50%, undergoing elective, multiple (>2) graft, on-pump CABG surgery by a single surgeon and having uneventful post-operative course, peak LV GLS was measured in the immediate post-operative period by TEE and at the time of hospital discharge (post-operative day 7–10) by transthoracic echocardiography. The association between the change in the peak LV GLS and the vasoactive inotropic score (VIS) in the immediate post-operative period was also checked. Analysis of data from 51 participants revealed a significant decline in peak LV GLS from −12.8 ± 3.8% (mean ± standard deviation [SD]) in the immediate post-operative period to −10.2±2.4% (mean ± SD) in the immediate pre-discharge period (P = 0.000). Heart rate and cardiac index decreased significantly, while mean arterial pressure and systemic vascular resistance index increased significantly from the immediate post-operative period to the immediate pre-discharge period. Low-risk patients with normal LVEF undergoing multivessel CABG surgery with uneventful intraoperative and early post-operative (during index hospital stay) course have a significant decline in peak LV GLS from the immediate intraoperative period. This decline is not significantly associated with either immediate pre-operative peak LV GLS or VIS in the immediate post-operative period.

Congenital absence of sternum is a rare malformation of the anterior chest wall that needs surgical correction to avoid life-threatening complications as a consequence of such defect. It results from either partial or complete failure of fusion of mesenchymal strip during in utero organogenesis. The absence of sternum entails the risk of trauma to the mediastinal structures and other life-threatening complications. This defect is evaluated by a thorough clinical examination and computed tomography imaging of the thoracic cage to plan the surgical procedure. Early repair of the defect when the thoracic cage is still compliant yields the best result.

Coarctation of the aorta (CoA) is a rare congenital malformation, the symptoms of which may remain subtle in childhood and appear at a later age. It can manifest only with symptoms of upper body hypertension. Various methods have been described for managing coarctation of the aorta in adults, including surgical or percutaneous balloon angioplasty with or without stent placement and medical therapy. Surgical approaches include an extra-anatomical bypass through a left lateral thoracotomy, a median sternotomy, or a combined median sternotomy and a laparotomy incision; all have their merit in overcoming the symptoms. We went ahead with an extra-anatomical tube graft between the ascending aorta and the descending thoracic aorta in a 24-year-old patient who presented to us with a diagnosis of coarctation of the aorta.

Empirical use of pharmacogenetic test(PGT) is advocated for many drugs, and resource-rich setting hospitals are using the same commonly. The clinical translation of pharmacogenetic tests in terms of cost and clinical utility is yet to be examined in hospitals of low middle income countries (LMICs). The present study assessed the clinical utility of PGT by comparing the pharmacogenetically(PGT) guided- versus standard of care(SOC)- warfarin therapy, including the health economics of the two warfarin therapies. An open-label, randomized, controlled clinical trial recruited warfarin-receiving patients in pharmacogenetically(PGT) guided- versus standard of care(SOC)- study arms. Pharmacogenetic analysis of CYP2C9*2(rs1799853), CYP2C9*3(rs1057910) and VKORC1(rs9923231) was performed for patients recruited to the PGT-guided arm. PT(Prothrombin Time)-INR(international normalized ratio) testing and dose titrations were allowed as per routine clinical practice. The primary endpoint was the percent time spent in the therapeutic INR range(TTR) during the 90-day observation period. Secondary endpoints were time to reach therapeutic INR(TRT), the proportion of adverse events, and economic comparison between two modes of therapy in a Markov model built for the commonest warfarin indication- atrial fibrillation. The study enrolled 168 patients, 84 in each arm. Per-protocol analysis showed a significantly high median time spent in therapeutic INR in the genotype-guided arm(42.85

Infected coronary aneurysms are rare in clinical practice and represent a catastrophic complication following percutaneous coronary interventions. A high index of suspicion is imperative in patients presenting with fever a few weeks after percutaneous coronary intervention. Early diagnosis and surgical repair are key to mitigating the morbidity and mortality associated with it.

BACKGROUND/AIM:Double-chambered right ventricle is a rare and progressive condition that is characterised by obstruction of the right ventricular tract. Double-chambered right ventricle is usually associated with ventricular septal defect. Early surgical intervention is recommended in patients with these defects. Based on this background, the present study aimed to review early and midterm outcomes of primary repair after double-chambered right ventricle.METHODS:Between January 2014 and June 2021, 64 patients with a mean age of 13.42 ± 12.31 years underwent surgical repair for double-chambered right ventricle. The clinical outcomes of these patients were reviewed and assessed retrospectively.RESULTS:An associated ventricular septal defect was present in all the recruited patients; 48 (75%) patients of sub-arterial type, 15 (23.4%) of perimembranous, and 1 (1.6%) patient of muscular type. The patients were followed up for a mean period of 46.73 ± 27.37 months. During their follow-up, a significant decrease in the mean pressure gradient from 62.33 ± 5.52 mmHg preoperatively to 15.73 ± 2.94 mmHg postoperatively was observed (p < 0.001). Notably, there were no hospital deaths.CONCLUSIONS:The development of double-chambered right ventricle in association with ventricular septal defect results in an increased pressure gradient within the right ventricle. The defect needs correction in a timely manner. In our experience, the surgical correction of double-chambered right ventricle is safe and shows excellent early and mid-term results.

Cardiac calcified amorphous tumor is a rare, non-neoplastic, pedunculated, intra-cavity mass, with very high preponderance of distal embolisation. Differentiation from calcified atrial myxoma or calcified thrombi is very difficult. Histo-pathologic examination is the mainstay of diagnosis. Treatment is emergency excision. A 46-year-old female presented with heart failure. On echocardiography, 1.9x1.7 cm pedunculated mobile mass in left ventricle attached to intraventricular septum was seen. On cardiac MRI lesion was isointense. The mass was excised. Histopathology revealed fibrin deposition with eosinophilic amorphous material in the centre with calcification in the periphery without any myxomatous tissue. A final diagnosis of CAT was established.

Objective To study the clinical features and laboratory parameters of neonatal lupus erythematosus (NLE) from India.Patients and methods We analyzed case records of children diagnosed with NLE in the Pediatric Rheumatology Clinic at tertiary care centre from North India during the period January 1999 - December 2023.Results Twenty-four babies are diagnosed with NLE during the study period. Median age at diagnosis was 60 days with a female predisposition (Male to female- 1:2). Cutaneous manifestations were reported in 14 (58%) patients. Hepatomegaly was noted in 15 (62.5%), and splenomegaly in 2 (8%). Elevated transaminases were noted in 14 (58%) patients, and cholestatic jaundice in one. Hematological manifestations were noticed in 20 (83.3%), and neurological manifestations were noticed in 8 patients. Most non-cardiac manifestations were self-limiting. Intravenous immunoglobulin was used for phrenic nerve palsy and autoimmune hemolytic anemia. Oral corticosteroid was used in a patient with refractory cytopenia, and four patients required transfusions. Cardiac involvement was reported in 13 (54%) patients - 3rd-degree heart block in 9 patients, and 6 were managed with epicardial pacemaker insertion. One patient required pacemaker reimplantation due to infective endocarditis. Congenital hydrops was seen in 3 patients, and required respiratory support and recovery was uneventful.Conclusion NLE is associated with significant morbidity. More than half had cardiac manifestation, of which 70% had 3rd-degree heart block requiring pacemaker insertion. Other rare clinical manifestations like phrenic nerve palsy and seizures are noticed in our cohort.

Myocardial ischemia after arterial switch operation is most commonly associated with imperfect translocation of coronary arteries to the neoaorta. Early post-operative myocardial ischemia is the main cause of morbidity and mortality in these patients. We present a rare case of intra-operative myocardial ischemia after ASO, detected with transesophageal echocardiography before electrocardiography changes.

OBJECTIVES:A study was conducted to develop and validate the warfarin pharmacogenetic dose optimization algorithm considering the clinical pharmacogenetic implementation consortium (CPIC) recommendations for the Asian ethnicity population.METHODS:The present prospective observational study recruited warfarin-receiving patients. We collected a three ml blood sample for VKORC1, CYP2C9*2, CYP2C9*3, and CYP4F2 polymorphism assessment during the follow-up visits. Clinical history, sociodemographic and warfarin dose details were noted.RESULTS:The study recruited 300 patients (250 in derivation and 50 in validation timed cohort) receiving warfarin therapy. The baseline characteristics were similar in both cohorts. BMI, presence of comorbidity, VKORC1, CYP2C9*2, and CYP2C9*3 were identified as covariates significantly affecting the warfarin weekly maintenance dose (p<0.001 for all) and the same were included in warfarin pharmacogenetic dose optimization algorithm building. The algorithm built-in the present study showed a good correlation with Gage (r=0.57, p<0.0001), and IWPC (r=0.51, p<0.0001) algorithms, widely accepted in western side of the globe. The receiver operating characteristic curve analysis showed a sensitivity of 73 %, a positive predictive value of 96 %, and a specificity of 89 %. The algorithm correctly identified the validation cohort's warfarin-sensitive, intermediate reacting, and resistant patient populations.CONCLUSIONS:Validation and comparisons of the warfarin pharmacogenetic dose optimization algorithm have made it ready for the clinical trial assessment.

Background/aim: Surgical repair techniques and management of patients with atrioventricular septal defect (AVSD) have progressed over the last few decades. Early and definitive interventions have become the choice of treatment for these patients. Based on this background, we aimed to review the early and mid-term outcomes of primary AVSD repair.Methods: A total of 53 patients with a mean age of 3.45 +/- 5.67 years underwent definitive repair for AVSD between January 2014 and June 2021. The clinical data including age, type of defect, associated co -anomalies, symptoms, pulmonary hypertension, etc. were collected and assessed retrospectively. Mitral regurgitation (MR) as a clinical outcome was assessed at 0, 1, 2, and 5 years.Results: Among the recruited patients, 35 (66.1%) were male and 18 (33.9%) were female. Of 53 patients, repair for the complete defect was done in 38 (71.69%) patients, repair for intermediate/partial defect was done in 15 (23.1%) patients, and one patient underwent repair for incomplete type. Other associated co -anomalies were anterior mitral leaflet (12 (22.6%)), atrial and ventricular septal defect (VSD) (30 (56.6%)), and patent ductus arteriosus (PDA) (11 (20.8%)). Different procedures for surgical repair included patch closure, cleft repair, and polytetrafluoroethylene (PTFE) VSD closure. After repair, the mean follow-up period was 46.73 +/- 27.37 months. Overall mortality was 3.78% (2/53), and two patients underwent reintervention due to symptomatic severe MR.Conclusions: A definitive and timely correction of AVSD shows satisfactory early and mid-term results.

Aortic transection following blunt trauma chest is one of the main life-threatening situations requiring early intervention. The endovascular approach is now preferred; however, in some conditions, an open approach is warranted. We describe our experience of dealing with acute traumatic transection in six such patients over the past 3 years.

Total anomalous pulmonary venous connection (TAPVC) is an uncommonly encountered cyanotic congenital heart disease characterized by anomalous drainage of the pulmonary veins. Concomitant venous anomalies are rarely found in these patients. We present a case of an infra-cardiac TAPVC with an intrahepatic portosystemic shunt, where a vascular channel was seen between the middle hepatic vein and the left branch of the portal vein with fusiform dilatation of the latter, diagnosed on computed tomography angiography.

Asymptomatic presentation is common in benign mature mediastinal tumours. Symptoms of the above diseases are sometimes life-threatening and can cause massive hemoptysis, recurrent pulmonary infection, hypoxia related to the pulmonary parenchymal hemorrhage, or pressure effect on or more of the major bronchi. A 16-year-old boy presented with frequent episodes of hemoptysis and recurrent fever unresponsive to antimicrobials. On investigation, it was found to be a benign mature mediastinal mass with cystobronchial connection to the right middle lobe. Resection of this mass resulted in the complete recovery of the individual. An anterior mediastinal benign teratodermoid tumour with intraparenchymal extension through cystobronchial connection is very rare. Surgical resection is challenging but offers the complete cure.

Subacute right ventricular perforation by a pacemaker lead is a rare complication. Although occasionally asymptomatic, complicated cases are usually evidently symptomatic. Here, we report a 70-year-old man presented with mild left-sided chest pain three days after permanent pacemaker implantation. Suspected of ventricular perforation by the pacemaker lead on chest X-ray, device interrogation revealed non-corroborative parameters. This warranted a computed tomography (CT) scan, which confirmed the diagnosis, detected hemopneumothorax, and helped plan surgical intervention. The patient underwent surgical management with the placement of an epicardial pacemaker lead and was discharged after five days. Our case illustrates a rare report of subtle clinical presentation in a patient with subacute right ventricular perforation by a pacemaker lead complicated by hemopneumothorax. It further recapitulates the role of CT scan in providing definitive diagnostic information in managing such a patient. Anticipation of such a presentation is essential for primary care physicians, who are often a first contact point for a patient in the community. This requires a high index of suspicion in such patients presenting with minimal symptoms. Early recognition and timely referral by a family physician may prevent untoward consequences of device-related complications.

Heparin is the most commonly used anticoagulant for patients undergoing cardiothoracic surgery requiring cardiopulmonary bypass (CPB). Apart from the intravenous route and intracardiac route as cardioplegia fluid, it is also used to coat CPB circuits. Various components of the CPB circuit cause hemolysis at a different rate. Heparin-coated polyvinyl chloride (PVC) tubing causes more hemolysis, but it is considered by some authorities as sublethal [1]. To date, there is no focused investigation on heparin-induced hemolysis. Particularly the interaction of heparin with erythrocyte membrane protein is an unknown area. Therefore, in the instant work, we investigated heparin-induced hemolysis and studied the binding of heparin with erythrocyte membrane proteins. We used Autodock 4.2 for predicting the interaction of heparin with different erythrocyte integral membrane proteins. The 3-D structure of proteins and heparin were obtained from the structural database (Protein Data Bank). The docking was performed using the Lamarckian genetic algorithm (LGA) by applying default parameters. The grid center for proteins was selected as mentioned in Table 1 (supplementary section). The details of the docking simulation protocol are mentioned in the supplementary section. We observed strong binding interactions of heparin with all the chosen erythrocyte menbrane proteins, namely band 3, Glucose transporter 1 (GLUT1), Urea Transporter 1, Aquaporin 1, Cluster of Differentiation 47 (CD 47), and Acetylcholinesterase (AChE) (Table 1; supplementary section mentions the docking score in the form of interaction energy). Among all the proteins, Band 3 showed the highest negative binding energy with heparin. Therefore, this protein was subjected to detailed docking (methods in supplementary section). The highest negative binding energy docked conformation of band3 protein with heparin was subjected to Molecular dynamic (MD) simulation following procedure as detailed in supplementary section for further analysis. MD was performed using the GROningen MAchine for Chemical Simulations (GROMACS) Software (v 2.1) (Supplementary Fig.1). Different parameters like potential energy, kinetic energy, root mean square deviation (RMSD), radius of gyration, and hydrogen bonds were checked with respect to time, and the representative graphs are shown (Fig. 1). The RMSD curves indicated the stability of the simulated complex after 3 ns. The radius of gyration was stable throughout the runtime of 5 ns. On average, the hydrogen bonds were between 8 and 11 in number throughout the runtime. This signifies interaction between heparin and the protein. Pymol tool was used to analyze the binding interactions. Most of the residues involved in hydrogen bonds formation were similar before and after MD simulations. However, change in interacting residues from Lys539 and Lys851 (before MD simulation) to Thr727 and Thr728 (after MD simulation) was observed. This signifies that even though there is change in interaction residues, molecular interactions are not affected as hydrogen bonds are formed following MD simulation. In silico analysis revealed that heparin interacts with the critical residues of Band 3 protein via hydrogen bonds. Some of the interacting residues of hydrogen bonds are