Successful Management of a Very Unusual Coronary Pattern in Transposition of the Great Arteries.

Coronary artery anatomy is the key to a successful arterial switch operation in transposition of the great arteries. We came across an unusual coronary pattern in a child with transposition in which the three major coronary arteries were seen arising from all three aortic sinuses. This coronary pattern is the first of its kind in transposition, and this case report emphasizes the difficulty in translocating such a rare coronary pattern while performing an arterial switch operation.

"Y" incision and rectangular patch enlargement of aortic annulus and valve reconstruction with autologous pericardium in congenital aortic stenosis.

Surgical aortic valve replacement in children who have undergone prior balloon or surgical valvuloplasty or both is a formidable challenge. The aortic annulus is small, there is no ideal prosthesis and lifelong anticoagulation is highly undesirable. A "Y" incision and rectangular patch enlargement of the aortic annulus introduced by Dr. Bo Yang in 2020 combined with aortic valve reconstruction introduced by Dr. Shigeyuki Ozaki in 2011, is feasible, as described in this case.

Acquired cyanosis secondary to RVOT obstruction due to pacemaker lead prolapse and vegetation in a child with ASD.

Pacemaker implantation in the paediatric population is associated with significant perioperative complications. We report a child with atrial septal defect who developed cyanosis following pacemaker implantation. An 8-year-old male child presented to us with fever of 2 months, along with cyanosis and clubbing. He underwent endocardial transvenous pacemaker implantation for congenital complete heart block. His echocardiography revealed right atrial vegetation (1 × 1 cm), moderate tricuspid regurgitation, and a prolapsed lead loop extending into the right ventricular outflow tract and pulmonary valve causing severe obstruction of pulmonary valve. He was started on empirical treatment followed by sensitive antibiotics based on culture report; however due to recurrent ventricular tachycardia he was taken for emergency surgery. He underwent closure of atrial septal defect, removal of pulse generator, removal of endocardial leads/vegetation, and pulmonary valve repair. Postoperative echocardiography revealed mild tricuspid regurgitation and resolution of pulmonic stenosis. This case presents a rare complication of severe right ventricular outflow tract obstruction following pacemaker implantation. .

Changing spectrum of infective endocarditis in India: An 11-year experience from an academic hospital in North India.

OBJECTIVE: Several studies have demonstrated a shift in the spectrum of infective endocarditis (IE) in the developed world. We aimed to investigate whether demographic and microbiologic characteristics of IE have changed in India. DESIGN: A retrospective analysis of patients with in north India between 2010 and 2020. METHODS: The clinical and laboratory profiles of 199 IE admitted to an academic hospital patients who met the modified Duke criteria for definite IE were analysed. RESULTS: The mean age was 34 years, and 84% were males. The main predisposing conditions were injection drug use (IDU) (n = 71, 35.7%), congenital heart disease (n = 46, 21.6%), rheumatic heart disease (n = 25, 12.5%), and prosthetic device (n = 19, 9.5%). 17.1% of patients developed IE without identified predispositions. Among 64.3% culture-positive cases, the most prevalent causative pathogens were Staphylococcus aureus (46.1%), viridans streptococci (7.0%), enterococci (6.0%), coagulase-negative staphylococci (5.5%), gram negative bacilli (5.5%), polymicrobial (5.5%), and Candida (1.0%). The tricuspid (30.3%), mitral (25.6%), and aortic (21.6%) valves were the most common sites of infection, and 60.3% had large vegetations (>10 mm). Systemic embolization occurred in 55.3% of patients at presentation. Cardiac surgery was required for 13.1%. In-hospital mortality was 17.1% and was associated with prosthetic devices (p-value, 0.001), baseline leucocytosis (p-value, 0.036) or acute kidney injury (p-value, 0.001), and a microbial etiology of gram negative bacilli or enterococci (p-value, 0.005). CONCLUSION: IDU is now the most important predisposition for IE in India, and S. aureus has become the leading cause of native valve endocarditis with or without IDU.

Short-term outcome of polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of right ventricular outflow tract in tetralogy of fallot: a randomized controlled trial

Abstract Introduction: Reconstruction of right ventricular outflow tract during primary repair of tetralogy of Fallot often requires the placement of a transannular patch which results in pulmonary regurgitation (PR). We compared the short-term outcomes of bicuspid polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of the right ventricular outflow tract. Methods: Thirty consecutive patients undergoing primary repair of tetralogy of Fallot were randomly allocated to two groups - polytetrafluoroethylene valve (PTFEV) group (n=15) and transannular pericardial patch (TAP) group (n=15). The two groups had similar preoperative demographic characteristics. We compared the short-term clinical and echocardiographic outcomes between these groups. The transthoracic echocardiographic follow-up was performed at one week, one month and six months after surgery. Results: The PTFEV group had significantly lower central venous pressure in the immediate postoperative period compared to the TAP group (7.60±2.06 vs. 10.13±1.73, P=0.002). Extubation time was significantly shorter in the PTFEV group compared to the TAP group (12.93±7.55 hrs vs. 22.23±15.11 hrs, P=0.04). PR in the PTFEV group was absent in five patients at 24 hours post-surgery. At the study endpoint, PR was absent in six, trivial in one and mild in eight patients in the PTFEV group compared to TAP group, where all 15 patients had severe PR. Conclusion: The bicuspid polytetrafluoroethylene membrane valves significantly decrease the central venous pressure in the immediate postoperative period, facilitate early extubation and, thus, prevent ventilator-related comorbidities. They achieve a high degree of pulmonary competence and do not increase the right ventricular outflow tract gradient in short-term follow-up.

Short-term outcome of Polytetrafluoroethylene Membrane Valve versus Transannular Pericardial patch Reconstruction of Right Ventricular Outflow Tract in Tetralogy of Fallot: a Randomized Controlled Trial.

INTRODUCTION: Reconstruction of right ventricular outflow tract during primary repair of tetralogy of Fallot often requires the placement of a transannular patch which results in pulmonary regurgitation (PR). We compared the short-term outcomes of bicuspid polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of the right ventricular outflow tract. METHODS: Thirty consecutive patients undergoing primary repair of tetralogy of Fallot were randomly allocated to two groups - polytetrafluoroethylene valve (PTFEV) group (n=15) and transannular pericardial patch (TAP) group (n=15). The two groups had similar preoperative demographic characteristics. We compared the short-term clinical and echocardiographic outcomes between these groups. The transthoracic echocardiographic follow-up was performed at one week, one month and six months after surgery. RESULTS: The PTFEV group had significantly lower central venous pressure in the immediate postoperative period compared to the TAP group (7.60±2.06 vs. 10.13±1.73, P=0.002). Extubation time was significantly shorter in the PTFEV group compared to the TAP group (12.93±7.55 hrs vs. 22.23±15.11 hrs, P=0.04). PR in the PTFEV group was absent in five patients at 24 hours post-surgery. At the study endpoint, PR was absent in six, trivial in one and mild in eight patients in the PTFEV group compared to TAP group, where all 15 patients had severe PR. CONCLUSION: The bicuspid polytetrafluoroethylene membrane valves significantly decrease the central venous pressure in the immediate postoperative period, facilitate early extubation and, thus, prevent ventilator-related comorbidities. They achieve a high degree of pulmonary competence and do not increase the right ventricular outflow tract gradient in short-term follow-up.

Benefits of perioperative sildenafil therapy in children with a ventricular septal defect with pulmonary artery hypertension on early surgical outcomes.

OBJECTIVES: Pulmonary hypertension is a common association in children with nonrestrictive ventricular septal defect. It increases perioperative mortality and morbidity. Oral sildenafil is an effective pulmonary vasodilator. In this study, we assessed effects of perioperative oral sildenafil therapy on pulmonary artery pressure and early surgical outcomes. METHODS: This was a single centre, prospective randomized control study. Thirty children with nonrestrictive ventricular septal defects with pulmonary hypertension were divided into two groups. In the sildenafil group (n = 15, mean age 23.3 months), oral sildenafil was administered two weeks before surgery. In the control group (n = 15, mean age 36 months), preoperative sildenafil was not given. Sildenafil was continued postoperatively in both groups, provided the postoperative pulmonary artery pressure was over 50% of systemic pressure. RESULTS: There was no perioperative mortality, pulmonary hypertensive crisis and there were no intolerable side effects related to sildenafil in either group. Mean pulmonary artery pressure showed a reduction in both groups. Sildenafil group showed statistically significant improvement in duration of cardiopulmonary bypass (100.27 ± 21.09 min vs. 125.40 ± 26.83 min, p = .008), mechanical ventilation requirement (22.79 ± 17.13 h vs. 30.53 ± 13.05 h; p = .04), epinephrine requirement (22% patients vs. 48% patients; p = .03) and hospital stay (6.13 ± 1.40 days vs. 7.53 ± 1.92 days; p = .05). CONCLUSION: Oral Sildenafil therapy is an inexpensive and well-tolerated method for reducing pulmonary hypertension secondary to non-restrictive ventricular septal defect. It has noteworthy advantages regarding early surgical outcomes like reduced cardiopulmonary bypass time, improved mechanical ventilation time, lower inotrope requirement and shorter hospital stay if used preoperatively in select patient population.

Outcomes of transcatheter closure of patent ductus arteriosus with the off-label use of large occluders (≥16 mm).

OBJECTIVE: Transcatheter closure is the first-choice strategy for the management of appropriate patients with patent ductus arteriosus (PDA). The management of large PDAs is challenging due to the limited available sizes of approved devices and the inherent risks of surgical ligation, especially in adults with calcified PDAs. This study aimed to assess the outcomes of the off-label use of large occluders at a tertiary center. METHODS: This retrospective review included patients who underwent transcatheter PDA closure with large occluders (≥16 mm) over 16 years. The baseline patient data, procedural details, angiograms, and immediate outcomes were recorded and patients were followed up at 3, 6, 12 months after the intervention and annually thereafter. RESULTS: Of the 685 patients who underwent transcatheter PDA closure, 36 patients (mean age 16.6 ± 12.5 years) needed occluders ≥ 16 mm in size. Cocoon duct occluder, Cera duct occluder, Amplatzer atrial septal occluder (ASO), and Cera muscular ventricular septal defect occluders were used for PDA closure. There was no device embolization, one patient in whom ASO was used had residual shunt with intravascular hemolysis requiring surgery, and one patient had mild left pulmonary artery narrowing after the intervention, which was managed conservatively. No patient had residual shunt and one patient had persistent pulmonary hypertension at an intermediate duration of follow-up. CONCLUSION: Transcatheter PDA closure with the use of large devices, which are available in Asia and Europe, is an effective and safe method, especially in adolescents and adults. However, a close follow-up of these patients is mandatory.

Coagulation F13A1 V34L, fibrinogen and homocysteine versus conventional risk factors in the pathogenesis of MI in young persons.

BACKGROUND: The pathogenesis of myocardial infarction (MI) involves environmental and genetic risk factors, with the latter putatively playing significant roles in younger patients. Genetic variability in coagulation factors comprises one such group. The coagulation factor 13 subunit A (F13A1) Val34Leu polymorphism (rs5985) has yielded variable findings in literature, with no prior South Asian data. METHODS: We studied the frequency of this polymorphism using the amplification-created restriction-enzyme site (ACRES) polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) in 101 MI patients aged below 40 years and 103 controls along with plasma fibrinogen and serum homocysteine levels. RESULTS: The distribution of Val/Val, Val/Leu and Leu/Leu genotypes was similar among cases (72.3%, 26.7% and 1.0%) and controls (78.6%, 19.4% and 1.9%, respectively). Val and Leu allele frequencies were 85.6% and 14.4% among patients and 88.3% and 11.7% among controls, respectively (p = .416). Mean plasma fibrinogen was higher in patients vis-à-vis controls (3.1 versus 3.7 g/l; p < .001) but homocysteine was elevated in both patients (52%) and controls (67%) (p = .225). Multivariate analysis revealed hypertension (p < .001, OR 6.16) and smoking (p < .001, OR 5.48) to impart strongest risk followed by positive family history, plasma fibrinogen levels and male gender. CONCLUSIONS: Despite its small sample size, this first South Asian study suggests neither protective nor deleterious effects of the F13A1 Val34Leu polymorphism on the risk of MI in young persons. The Leu allele frequency is intermediate to that reported from the West and the Far East. Traditional risk factors contribute greatly to risk even in younger MI patients in South Asia.

Variant origin and course of left circumflex coronary artery.

Variant origin of left circumflex coronary artery (LCx) from right aortic sinus is a well-recognized coronary variation, usually without any clinical consequences. However, the variant origin and trajectory of the artery may have major implications during percutaneous coronary intervention, coronary artery surgery, aortic and mitral valve replacement procedures. We observed a variant LCx in a heart specimen belonging to 45-year-female with no history of hypertension, diabetes mellitus and coronary artery disease. The artery arose along with the right coronary artery from a common ostium in right aortic sinus and depicted a retroaortic course. The vessel was located at the level of aortic annulus and 6.6 mm above mitral valve annulus. The degree of luminal stenosis in variant LCx was higher than that in right coronary artery (RCA) and left anterior descending artery (LAD). Appropriate anatomical knowledge of the location and course of variant LCx is important for successful coronary interventions and valve replacement procedures.

Vitamin D regulates the production of vascular endothelial growth factor: A triggering cause in the pathogenesis of rheumatic heart disease?

Rheumatic heart disease (RHD) remains a major cause of cardiac related mortality and morbidity in the developing countries due to poor diagnosis and lack of proper therapeutics. The definite reason of heart valve injury during RHD is poorly understood. Valvular endothelial cells play an important role in pathogenesis of different cardiovascular diseases. Besides, the regulation of vitamin D (calciferol) and VEGF (vascular endothelial growth factor) results in the functional changes in endothelial cells. However, the crosstalk between vitamin D and VEGF in the pathogenesis of RHD is not yet unfurled. Evidences in the concerned fields are documented by searching through Google Scholar and Pubmed. Literature based survey has revealed that vascular endothelium, especially endothelial cells play important roles in valvular remodelling during cardiovascular diseases. Endothelial cell dysfunction leads to heart valve remodelling, which furthermore initiates the pathogenesis of valvular heart disease. Vitamin D has the potential to maintain the concentration of VEGF in the circulation and induce the function of endothelial cells. Hence, we hypothesize that vitamin D and VEGF homeostasis can alter the function of endothelial cells, which may subsequently trigger the valvular remodelling or even damage of heart valves during the progression of RHD pathogenesis. Our hypothesis shed light on the evidence based knowledge translation of plausible cellular phenomena due to vitamin D/VEGF homeostasis during valvular vandalism in RHD.

Perioperative echocardiography-derived right ventricle function parameters and early outcomes after tetralogy of Fallot repair in mid-childhood: a single-center, prospective observational study.

BACKGROUND: Right ventricular (RV) function alterations are invariably present in all patients after tetralogy of Fallot (TOF) repair. Unlike the developed world where most of the patients with TOF are corrected in infancy, average age of presentation and thus surgery for these patients in the developing world may be higher. We aimed to study the correlation between RV function parameters such as tricuspid annular peak systolic excursion (TAPSE), fractional area change (FAC), and tricuspid annular peak systolic velocity (S') with early outcome variables after intracardiac repair for TOF. MATERIALS AND METHODS: Fifty patients with a preoperative diagnosis of tetralogy of Fallot scheduled for corrective surgery were included in this single-center, prospective observational study. A preoperative transthoracic echocardiogram was performed to measure RV function parameters (FAC0, TAPSE0, S'0). Transthoracic echocardiography was repeated postoperatively to measure FAC1, TAPSE1, S'1 (day 1) and FAC2, TAPSE2, and S'2 (day 3). The relationship between preoperative and postoperative RV function parameters with in-hospital mortality, duration of mechanical ventilation, and intensive care unit stay was studied. RESULTS: The median age of patients was 6 years (range 1-14 years). Multiple stepwise logistic regression analysis showed RV FAC as best predictor of clinical outcome. Area under the receiver operating characteristic curve for postoperative RV function parameters, that is, FAC, TAPSE, and S' to predict early or delayed recovery was 0.944, 0.875, and 0.655, respectively. CONCLUSIONS: Among the RV function parameters studied, RV FAC best predicted the early outcome variables after TOF repair, followed by TAPSE while lateral tricuspid annular velocity S' being the least predictive.

Infliximab is the new kid on the block in Kawasaki disease: a single-centre study over 8 years from North India.

This was a single-centre study to evaluate the usefulness of tumour necrosis factor-α (TNF-α) blocker, infliximab (IFX), for treatment of Kawasaki disease (KD) in children in Northern Indian. The study was carried out in the Paediatric Allergy-Immunology Unit, Advanced Paediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh. The study period was January 2007 to March 2015. Review of records of 23 children with KD who had received IFX was carried out. Median age at presentation was 2 years (range 2 months to 12 years). Indications for using IFX were intravenous immunoglobulin (IVIg) resistance (12/23 patients); severe KD especially when coronary artery abnormalities (CAAs) had developed in spite of IVIg (9/23 patients); retinal vasculitis in association with KD (1 patient) and economic reasons (1 patient). Twenty one (21/23) patients had received IVIg (2 g/kg) as first line therapy. A dose of IFX was 5-7 mg/kg given intravenously. Screening tests for tuberculosis (chest xray, Tuberculin test, QuantiFERON-TB Gold test) were not carried out prior to IFX infusion in any patient. Duration of follow-up was 0-20 months in 13 patients; 21-40 months in 5 patients and >40 months in 6 patients. Mean follow-up was 28.78±25.49 months, range 1-84 months. Eleven of 12 patients (11/12) who had IVIg resistance showed prompt resolution with IFX. Nineteen patients (19/23) in the cohort had CAAs. Of these, 12 showed improvement over mean follow-up of 28.78±25.49 months (range 1-84 months) and 4 showed normalisation. No adverse reactions were noted during infusion of IFX. On follow-up, none of these patients has developed tuberculosis or any other significant infection over a cummulative follow-up of 662 months. IFX can be considered as a useful adjunct in treatment of children with KD.

Endovascular transluminal stent grafting: Treatment of choice for post lumbar spine surgery iliac arterio-venous fistulae.

Iliac vessels are prone to injury during lumbar spine surgery due to their proximity to the lumbar spine. Arterio-venous fistula formation during lumbar spine surgery is an uncommon complication and can present as an asymptomatic incidental finding to rapidly deteriorating hemodynamics leading to cardiopulmonary collapse. We have reported three patients who had symptomatic iliac arterio-venous fistula detected soon after lumbar spine surgery. All these patients were successfully treated by endovascular transluminal stent grafting. © 2013 Wiley Periodicals, Inc.

Perioperative Follow-Up of Patients With Severe Pulmonary Artery Hypertension Secondary to Left Heart Disease: A Single Center, Prospective, Observational Study.

OBJECTIVE: A substantial portion of the Indian cardiac surgery population experiences rheumatic valve disease that progresses to severe pulmonary artery hypertension (PAH) in a few patients. Right ventricular (RV) function, particularly in the perioperative period, has been studied sparsely. The authors describe serial RV function and clinical variables in the perioperative period in patients with severe PAH secondary to left heart disease. DESIGN: Prospective, observational study. SETTING: University hospital. PARTICIPANTS: Patients with PAH. INTERVENTIONS: The study comprised consecutive patients referred for open cardiac surgery from January 2012 to June 2013 who also had an estimated right ventricular systolic pressure≥50 mmHg on referral echocardiogram. Composite echocardiographic assessment of right ventricular size and linear/two-dimensional tissue Doppler systolic function and diastolic function analysis were performed at predetermined intervals. Data from right heart catheterization, inotrope use, fluid requirements, mechanical ventilation logs, and intensive care stay also were acquired. MEASUREMENTS AND MAIN RESULTS: A complete dataset was obtained in 20 of 22 patients enrolled in the study. Serial comparison of most RV echocardiographic function variables were noted to be abnormal at baseline, deteriorating further in the immediate postoperative period and trending to a partial recovery at discharge from the intensive care unit, particularly for longitudinal assessment of the RV. Fractional area change, although abnormal, was noted to be preserved. Pulmonary artery systolic pressures registered significantly declined after intervention. The clinical course was largely uneventful. CONCLUSION: Although linear echocardiographic RV function was grossly abnormal in the perioperative period in this patient subset with PAH, there was apparent disjunction with the clinical course.

Prospective study to develop surface landmarks for blind axillary vein puncture for permanent pacemaker and defibrillator lead implantation and compare it to available contrast venography guided technique.

OBJECTIVE: To develop surface landmarks for blind axillary vein puncture for pacemaker lead implantation. METHODS AND RESULTS: Patients for routine coronary angiography were counseled for participating in our study. 20 patients who gave consent were taken up for axillary venogram after proper positioning at the time of coronary angiogram. The venograms of these 20 patients, were reviewed and the landmarks were used to develop a blind axillary puncture technique. Success rate of 100% was achieved with surface landmark guided axillary vein puncture. The implantation time while using surface landmark guided axillary puncture was not significantly longer than when venography based approach was used. Another interesting observation made from the study was that increasing BMI had a positive correlation with the time taken for venous access, the fluoroscopic time and the volume of contrast used, all the associations being statistically significant. Thus, the surface landmark guided technique is more safe and expeditious in non obese patients and probably in pediatric patients as well. Moreover, the new surface landmark guided approach is a significant safety step in terms of reducing the unwanted and avoidable radiation exposure to the hands. CONCLUSION: The results of this study demonstrate that placement of endocardial permanent pacemaker and ICD leads via the developed surface landmarks is effective and safe and is devoid of the harmful effects of radiation and contrast exposure.

Cardiovascular Causes of Pediatric Airway Compression: A Pictorial Review.

Airways compression by vascular structures is one of the important comorbidities of congenital heart disease with incidence of approximately 1%-2% in children. Airways compression is a consequence of abnormal configuration of the great vessels producing a vascular ring with enlargement of normal structures (pulmonary arteries or cardiac chambers) or because of surgery. A high index of suspicion for vascular airway compression is important in children with recurrent respiratory complaints. Early diagnosis and management are essential, as chronic airway compression causes significant morbidity. As the underlying anatomical patterns tend to be highly complex, presurgical imaging assessment is essential.

Post Blalock-Taussig shunt mediastinal mass - a single shadow with two different destinies.

The modified Blalock-Taussig shunt is a synthetic shunt between the subclavian and pulmonary artery, used in the treatment of congenital cyanotic heart diseases with pulmonary hypoperfusion. Delayed complications include progressive failure of the shunt, serous fluid leak, and pseudoaneurysm formation. We report two different and rare mediastinal vascular complications following modified BT shunt surgery in this case report. The first one is a seroma, due to serous fluid leakage through the shunt graft, which is a relatively benign complication. The second one is a pseudoaneurysm, arising from the shunt, a frequently fatal complication. Generally, X-ray chest is used for screening in these patients. CT angiography plays a vital role in the diagnosis of both these conditions. Management in pseudoaneurysm should be aggressive, as timely intervention may be life saving, while in seroma the management is most often conservative occasionally requiring surgical intervention.