Surgery compared to fibrinolytic therapy for symptomatic left-sided prosthetic heart valve thrombosis (SAFE-PVT): Rationale and design of a randomized controlled trial.

BACKGROUND: Left-sided mechanical prosthetic heart valve thrombosis (PVT) occurs because of suboptimal anticoagulation and is common in low-resource settings. Urgent surgery and fibrinolytic therapy (FT) are the two treatment options available for this condition. Urgent surgery is a high-risk procedure but results in successful restoration of valve function more often and is the treatment of choice in developed countries. In low-resource countries, FT is used as the default treatment strategy, though it is associated with lower success rates and a higher rate of bleeding and embolic complications. There are no randomized trials comparing the two modalities. METHODS: We performed a single center randomized controlled trial comparing urgent surgery (valve replacement or thrombectomy) with FT (low-dose, slow infusion tissue plasminogen activator, tPA) in patients with symptomatic left-sided PVT. The primary outcome was the occurrence of a complete clinical response, defined as discharge from hospital with completely restored valve function, in the absence of stroke, major bleeding or non-CNS systemic embolism. Outcome assessment was done by investigators blinded to treatment allocation. The principal safety outcome was the occurrence of a composite of in-hospital death, non-fatal stroke, non-fatal major bleed or non-CNS systemic embolism. Outcomes will be assessed both in the intention-to-treat, and in the as-treated population. We will also report outcomes at one year of follow-up. The trial has completed recruitment. CONCLUSION: This is the first randomized trial to compare urgent surgery with FT for the treatment of left-sided PVT. The results will provide evidence to help clinicians make treatment choices for these patients. (Clinical trial registration: CTRI/2017/10/010159).

Is menstruation a valid reason to postpone cardiac surgery?

Background: Cancellation of any scheduled surgery is a significant drain on health resources and potentially stressful for patients. It is frequent in menstruating women who are scheduled to undergo open heart surgery (OHS), based on the widespread belief that it increases surgical and menstrual blood loss. Aims: The aim of this study was to evaluate blood loss in women undergoing OHS during menstruation. Settings and Design: A prospective, matched case-control study which included sixty women of reproductive age group undergoing OHS. Patients and Methods: The surgical blood loss was compared between women who were menstruating (group-M; n = 25) and their matched controls, i.e., women who were not menstruating (group-NM; n = 25) at the time of OHS. Of the women in group M, the menstrual blood loss during preoperative (subgroup-P) and perioperative period (subgroup-PO) was compared to determine the effect of OHS on menstrual blood loss. Results: The surgical blood loss was comparable among women in both groups irrespective of ongoing menstruation (gr-M = 245.6 ± 120.1 ml vs gr-NM = 243.6 ± 129.9 ml, P value = 0.83). The menstrual blood loss was comparable between preoperative and perioperative period in terms of total menstrual blood loss (gr-P = 36.8 ± 4.8 ml vs gr-PO = 37.7 ± 5.0 ml, P value = 0.08) and duration of menstruation (gr-P = 4.2 ± 0.6 days vs gr-PO = 4.4 ± 0.6 days, P value = 0.10). Conclusion: Neither the surgical blood loss nor the menstrual blood loss is increased in women undergoing OHS during menstruation.

Neurodevelopmental outcomes in children with cyanotic congenital heart disease following open heart surgery.

Background: Neurodevelopmental abnormalities are common in congenital heart disease (CHD), more so in cyanotic CHDs. Perioperative factors have been known to affect neurodevelopmental outcomes. Aim: We aimed to determine the neurodevelopmental outcomes following open-heart surgery in cyanotic CHD. Methods: In this prospective observational study, eligible infants and children ≤21 months with cyanotic CHD planned for open-heart surgery underwent preoperative neurodevelopmental assessment using Developmental Assessment Scale for Indian Infants (DASII) to look for any motor and/or mental delay. A second neurodevelopmental assessment was performed after 9 months ± 2 weeks of cardiac surgery. Follow-up DASII was conducted through interactive video conferencing in 23 of 60 patients due to COVID-19 pandemic. The univentricular and biventricular repair groups were compared in terms of their neurodevelopmental outcomes. Perioperative factors were compared between neurodevelopmental "delay" and "no delay" groups. Results: Of the 89 children enrolled, preoperative motor and mental delay were present in 29 and 24 children, respectively. Follow-up DASII could be performed in 60 children. At follow-up, motor delay was present in seven and mental delay in four children. Overall, there was a significant improvement in both motor and mental developmental quotient at follow-up. There was no significant difference in either motor or mental domains between univentricular and biventricular groups. Among the perioperative variables, only the postoperative length of stay in intensive care unit was significantly different between neurodevelopmental "delay" and "no delay" groups (P = 0.04). Conclusion: Neurodevelopmental delay occurred substantially among unoperated children with cyanotic CHD. The neurodevelopmental status improved significantly following open-heart surgery among the survivors. Delay was associated with length of stay in intensive care following cardiac surgery.

A rare pediatric case of pedunculated cardiac hemangioma of the left ventricle.

A 12-year-old male child with atypical chest pain and fatigue since past 3 months underwent cardiac magnetic resonance (CMR) imaging for suspicious globular mobile mass detected in the left ventricle on a transthoracic echocardiography. CMR revealed peripheral vascular blush on the first pass perfusion images with centripetal progressive intensely bright enhancement on late gadolinium-enhanced images. Further confirmation and histological diagnosis of the tumor was made through transaortic aortic resection of the mass.

Incidence and predictors of de novo late-onset persistent atrial fibrillation in postoperative rheumatic heart disease patients (LOAF-RHD study).

PURPOSE: Late-onset atrial fibrillation (LOAF) after valve surgery for degenerative mitral valve disease often with underlying mitral valve prolapse is a known phenomenon. However, there is no similar data for postoperative rheumatic heart disease (RHD) patients. We sought to assess the incidence and predictors of LOAF during postoperative follow-up in RHD patients. METHODS: This single-center retrospective case-control study included a total of 384 RHD patients with normal sinus rhythm (NSR) who underwent rheumatic valve surgery between 1st July 2008 and 30th June 2013. Patients detected with de novo persistent atrial fibrillation (AF) after 2 months of valve surgery were diagnosed as having LOAF. Presurgical demographic and echocardiographic parameters were compared between the LOAF and NSR groups to identify risk factors for LOAF. RESULTS: The incidence of de novo LOAF after rheumatic valve surgery was 9.63% at an average of 2.67 ± 1.32 years follow-up. Age ≥ 32 years [OR 2.4 (95% CI 1.2-5.1); P = 0.01] and left atrial (LA) size ≥ 51 mm [OR 5.9 (95% CI 2.8-12.4); P < 0.0001] were the most significant and independent predictors of LOAF. Moreover, significant mitral valve disease was associated with a higher risk of LOAF than significant aortic valve disease (P = 0.037). LA size ≥ 51 mm at surgery showed a fair discriminative power [AUC = 0.75; sensitivity = 68%, specificity = 70%] to identify patients at high risk for LOAF. CONCLUSIONS: Late-onset AF develops in almost a tenth of the RHD patients postoperatively following corrective valve surgery. Preoperative LA size can be used to identify patients at high risk for LOAF.

An extremely rare association of coarctation of aorta with double chambered right ventricle: double-trouble causing bi-ventricular failure in a child.

Double chambered right ventricle is a rare cardiac defect characterised by an obstructive hypertrophied muscle bundle in the right ventricle. The common associated lesions are ventricular septal defect followed by sub-aortic membrane. We report a child who had coarctation of aorta in association with double chambered right ventricle. This case is being reported for its rarity and challenges in management.

Unique Association of Aortopulmonary Window With Anomalous Origin of Left Coronary Artery From Pulmonary Artery in an Infant: A Blessing in Disguise?

Aortopulmonary window (APW) seen in association with anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is extremely uncommon. When faced with this combination, ALCAPA usually goes undetected since most of the clinical and echocardiographic features of ALCAPA, including ventricular dysfunction are absent due to co-existent pulmonary hypertension. Herein we report a 5-month-old child with a large APW in whom a preoperative computed tomography angiogram facilitated the diagnosis of ALCAPA. The case is described for its rarity and the challenges faced during management. Also, it is extremely crucial that this coronary anomaly is identified and corrected during surgery, since failure to do so results in unforeseen postoperative ventricular dysfunction.

Concomitant Transthyretin Amyloidosis and Severe Aortic Stenosis in Elderly Indian Population: A Pilot Study.

BACKGROUND: Prevalence of both degenerative severe aortic stenosis (AS) and transthyretin cardiac amyloidosis (ATTR-CA) increases with age. Dual disease (AS+myocardial ATTR-CA) occurs in significant proportion of patients undergoing surgical aortic valve replacement (SAVR). OBJECTIVES: This study aimed to determine the prevalence of ATTR-CA in severe AS in the Indian population, identify noninvasive predictors of its diagnosis, and understand its impact on prognosis. METHODS: Symptomatic severe AS patients aged ≥65 years undergoing SAVR were enrolled. ATTR-CA diagnosis was based on preoperative 99m-technetium pyrophosphate (PYP) scan and intraoperatively obtained basal interventricular septum biopsy for myocardial ATTR-CA, and excised native aortic valve for isolated valvular ATTR-CA. Primary amyloidosis was excluded by serum/urine protein electrophoresis with serum immunofixation. RESULTS: SAVR was performed in 46 AS patients (age 70 ± 5 years, 70% men). PYP scan was performed for 32 patients, with significant PYP uptake in 3 (n = 3 of 32, 9.4%), suggestive of myocardial ATTR-CA. On histopathological examination, none of the interventricular septum biopsy specimens had amyloid deposits, whereas 33 (71.7%) native aortic valves showed amyloid deposits, of which 19 (57.6%) had transthyretin deposition suggestive of isolated valvular amyloidosis. Noninvasive markers of dual disease included low myocardial contraction fraction (median [interquartile range], 28.8% [23.8% to 39.1%] vs 15.3% [9.3% to 16.1%]; P = 0.006), deceleration time (215 [144 to 236] ms vs 88 [60 to 106] ms; P = 0.009) and global longitudinal strain (-18.7% [-21.1% to -16.9%] vs -14.2% [-17.0% to -9.7%]; P = 0.030). At 1-year follow-up, 2 patients died (4.3%); 1 each in myocardial ATTR-CA negative and positive groups (3.4% vs 33.3%; P = 0.477). CONCLUSIONS: Dual disease is not uncommon in India. Isolated valvular amyloidosis in severe AS is much more common.

Detecting sub-clinical disease activity in patients with chronic rheumatic valvular heart disease.

OBJECTIVE: Valve disease progression in rheumatic heart disease(RHD) is generally attributed to recurrent attacks of acute rheumatic fever(ARF). However, persistence of chronic sub-clinical inflammation remains a plausible but unproven cause. Non-invasive means to identify sub-clinical inflammation may facilitate research efforts towards understanding its contribution to disease progression. METHODS: Patients with chronic RHD, without clinical evidence of ARF, undergoing elective valve surgery were enrolled. Sub-clinical inflammation was ascertained by histological evaluation of left atrial appendage and valve tissue excised during surgery. We assessed the diagnostic utility of Gallium-67 scintigraphy imaging, and inflammatory biomarkers, hsCRP, IL-2, IL-6, Tumor Necrosis Factor-Alpha(TNF-α), Interferon-gamma(IFN-γ), and Serum Amyloid A(SAA), in identifying patients with sub-clinical inflammation. RESULTS: Of the 93 RHD patients enrolled(mean age 34 ± 11 years, 45% females), 86 were included in final analysis. Sub-clinical inflammation was present in 27 patients(31.4%). Patients with dominant regurgitant lesions were more likely to have sub-clinical inflammation compared to those with stenotic lesions, though this association was not statistically significant(dominant regurgitant lesions vs isolated mitral stenosis: OR 3.5, 95%CI 0.68-17.96, p = 0.133). Inflammatory biomarkers were elevated in the majority of patients: hsCRP, IL-2, IL-6, TNF-α, and IFN-γ in 44%, 89%, 90%, 79%, and 81% patients, respectively. However, there was no significant association between biomarker elevation and histologically ascertained sub-clinical inflammation. Ga-67 imaging was unable to identify inflammation in the 15 patients in whom it was performed. CONCLUSION: Sub-clinical inflammation is common in RHD patients. Conventional inflammatory markers are elevated in the majority, but aren't discriminatory enough to identify the presence of histologic inflammation.

Ivabradine Versus Amiodarone in the Management of Postoperative Junctional Ectopic Tachycardia: A Randomized, Open-Label, Noninferiority Study.

OBJECTIVES: This study sought to compare the efficacy of ivabradine and amiodarone in the management of postoperative junctional ectopic tachycardia (JET) after cardiac surgery in children. BACKGROUND: JET is a serious arrhythmia occurring in children after cardiac surgery and requires aggressive management. Amiodarone has been conventionally used in its treatment. Recent studies have reported the utility of ivabradine in this regard. METHODS: In this open-label randomized controlled trial, 94 children (age ≤18 years) who developed postoperative JET were allocated to receive either amiodarone or ivabradine. The primary endpoint was restoration of normal sinus rhythm. RESULTS: Sinus rhythm was achieved in 43 out of the 46 patients (93.5%) in the amiodarone group and 46 out of the 48 patients (95.8%) in the ivabradine group (mean difference of treatment effect: 2.3%; 95% confidence interval: -6.7% to 11.5%). The median (interquartile range) time taken to achieve sinus rhythm conversion was similar in both the groups: 21.5 (17-30.2) hours versus 22 (13.4-38.5) hours (p = 0.36)]. The time taken to rate control of JET was significantly less in the amiodarone group: median 7.0 (5.5-9.5) hours versus 8.0 (5.8-10.8) hours (p = 0.02)]. No drug-related adverse events were observed in the ivabradine group. CONCLUSIONS: Oral ivabradine is not inferior to intravenous amiodarone in converting postoperative JET to sinus rhythm. There was no difference in time taken to sinus rhythm conversion between the groups, although the rate control was earlier in patients who received amiodarone. Monotherapy with ivabradine may be considered as an alternative to amiodarone in the management of postoperative JET. (Comparison of Two Drugs, Ivabradine and Amiodarone, in the Management of Junctional Ectopic Tachycardia, an Abnormality in Cardiac Rhythm in Patients Under 18 years Who Undergo Cardiac Surgery: CTRI/2018/08/015182).

Surgical correction of rare anomaly of scimitar syndrome with tetralogy of Fallot.

We present a 9-year-old male with a history of cyanosis diagnosed as scimitar syndrome with tetralogy of Fallot with left anterior descending coronary artery crossing right ventricle outflow tract. He underwent reimplantation of scimitar vein into right atrium baffled into left atrium along with intracardiac repair for tetralogy of Fallot. Postoperative recovery was uneventful and the patient was discharged on postoperative Day 8 and was asymptomatic at follow-up at 6 months.

Pulmonary root translocation in corrected transposition of great arteries with ventricular septal defect and pulmonary stenosis with dextrocardia.

We present 13 years old with history of cyanosis and diagnosis of congenitally corrected transposition of great arteries with ventricle septal defect and pulmonary stenosis with dextrocardia. He underwent modified Senning procedure with pulmonary root translocation. Postoperative recovery was uneventful and was extubated on postoperative day 1. Patient remained in sinus rhythm. Postoperative echocardiography showed good biventricular function with no baffle obstruction and mild pulmonary regurgitation with no significant left and right outflow tract obstruction. Patient was discharged on postoperative day 6 and remained asymptomatic on follow up at 3 months.

Combined Rastelli procedure with supra-coronary ascending aorta replacement.

We present a 5-year-old child with pulmonary atresia palliated at infancy with midline shunt now for complete repair with aneurysmally dilated aorta. Patient underwent a combined Rastelli procedure with supra-coronary ascending aorta replacement with hemi-arch repair. Patient was discharged on post-operative day 10 and was asymptomatic on follow-up.

Tetralogy of Fallot with congenital diaphragmatic hernia with right lung aplasia-a rare entity.

A combination of tetralogy of Fallot with diaphragmatic hernia represents a very rare entity. We present a case of 18-month-old child presenting late with cyanosis and respiratory distress. Chest X-ray and computed tomography angiography showed tetralogy of Fallot with small left pulmonary artery with right lung aplasia with congenital diaphragmatic hernia with liver and bowel loops in right chest cavity. The patient was managed with central shunt on cardiopulmonary bypass and was discharged uneventfully.

Arduous redo made simple by "lateral approach"-pulmonary valve replacement via mini left thoracotomy.

We present a follow-up case of total anomalous pulmonary vein repair and pulmonary valvotomy done 21 years back presented with severe pulmonary regurgitation. Magnetic resonance imaging shows the right ventricle end-diastolic volume and end-systolic volume being 185 mL/m2 and 80 mL/m2 , respectively. In addition to it the patient had had severe kyphoscoliosis causing severe pulmonary restriction. The patient underwent mechanical pulmonary valve replacement through a mini left thoracotomy. The patient had an uneventful recovery was discharged on postoperative day 6, and was in class 1 on follow-up at 3 and 6 months of surgery.

Indian Guidelines for Indications and Timing of Intervention for Common Congenital Heart Diseases: Revised and Updated Consensus Statement of the Working Group on Management of Congenital Heart Diseases. Abridged Secondary Publication.

JUSTIFICATION: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. PROCESS: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA. OBJECTIVES: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. RECOMMENDATIONS: Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

Clarifying the anatomy of common arterial trunk: a clinical study of 70 patients.

AIMS: Anatomic variations in hearts with common arterial trunk are well-known, although there is no large study of living patients. Detailed knowledge of the origins of the pulmonary and coronary arteries is vital for surgical management. We sought to clarify the variations using computed tomography. METHODS AND RESULTS: We prospectively studied 70 consecutive patients using echocardiography and computed tomography. In 63 (90%) patients, there was aortic dominance, while 7 (10%) had dominance of the pulmonary component. In 27 (43%) patients with aortic dominance, part of the pulmonary segment arose from a truncal valvar sinus. A long confluent pulmonary channel was more common in patients with sinusal origin compared to those with non-sinusal origin of the pulmonary segment (19 vs. 0; P = 0.0005). Close proximity between the orifices of the coronary arteries and the pulmonary component was also more frequent with sinusal origin (21 vs. 6; P < 0.001) with 5 (19%) patients having pulmonary flow obstructed by a truncal valvar leaflet. CONCLUSION: Sinusal origin of the pulmonary component is common with aortic dominance, frequently in association with a long confluent pulmonary segment, which may be in close proximity to the origin of a coronary artery. One-fifth of patients with sinusal origin of pulmonary component have a truncal valvar leaflet obstructing the pulmonary orifice. These morpho-anatomic findings have important implications for management.