Pulmonary arterial compliance in patients of CHD with increased pulmonary blood flow.

INTRODUCTION: Pulmonary arterial compliance, the dynamic component of pulmonary vasculature, remains inadequately studied in patients with left to right shunts. We sought to study the pulmonary arterial compliance in patients with left to right shunt lesions and its utility in clinical decision-making. MATERIALS AND METHODS: In this single-centre retrospective study, we reviewed cardiac catheterisation data of consecutive patients of left to right shunt lesions catheterised over one year. In addition to the various other parameters, pulmonary arterial compliance was calculated, as indexed pulmonary flow (Qpi) / (Heart rate × pulse pressure in the pulmonary artery). RC time was also calculated, as the product of pulmonary arterial compliance and pulmonary vascular resistance index. Patients were divided into "operable," "borderline," and "inoperable" based on the decision of the treating team, and the pulmonary arterial compliance values were evaluated in these groups to study if it can be utilised to refine the operability decision. RESULTS: 298 patients (Median age 16 years, 56% <18 years) with various acyanotic shunt lesions were included. Overall, the pulmonary arterial compliance varied with Qpi, pulmonary artery mean pressure, and pulmonary vascular resistance index, but did not vary with age, type of lesion, or transpulmonary gradients. The median pulmonary arterial compliance in patients with normal pulmonary artery pressure (Mean pulmonary artery pressure less than 20 mmHg) was 4.1 ml/mmHg/m2 (IQR 3.2). The median pulmonary arterial compliance for operable patients was 2.67 ml/mmHg/m2 (IQR 2.2). Median pulmonary arterial compliance was significantly lower in both inoperable (0.52 ml/mmHg/m2, IQR 0.34) and borderline (0.80 ml/mmHg/m2, IQR 0.36) groups when compared to operable patients (p < 0.001). A pulmonary arterial compliance value lower than 1.18 ml/mmHg/m2 identified inoperable patients with high sensitivity and specificity (95%, AUC 0.99). However, in borderline cases, assessment by this value did not agree with empirical clinical assessment.The median RC time for the entire study population was 0.47 S (IQR 0.30). RC time in operable patients was significantly lower than that in the inoperable patients (Median 0.40 IQR 0.23 in operable, 0.73 0.25 in inoperable patients (p < 0.001). CONCLUSIONS: Addition of pulmonary arterial compliance to the routine haemodynamic assessment of patients with shunt lesions may improve our understanding of the pulmonary circulation and may have clinical utility.

A reassessment of the anatomical features of multiple ventricular septal defects.

BACKGROUND: Over the course of time, new developments associated with the embryogenesis of the murine heart have served to clarify the developmental processes observed in the human heart. This evidence allows for the creation of a developmental framework for many congenital cardiac defects. AIMS: We aim to solidify the framework related to the categorization of both solitary and multiple ventricular septal defects. MATERIALS AND METHODS: Mice having genetic perturbation of the Furin enzyme have demonstrated perimembranous and juxta-arterial ventricular septal defects, permitting the inference to be made that these defects can co-exist with defects occurring within the apical muscular septum. RESULTS: Basis of developmental evidence, furthermore, all interventricular communications can be placed into one of three groups, namely those which are perimembranous, juxta-arterial, and muscular. All of the defects are described based on their borders as seen from the morphologically right ventricle. Our focus here will be on those defects within the muscular ventricular septum, recognizing that such defects can co-exist with those that are perimembranous. We discuss the differentiation of multiple discrete defects from those referred to as the "Swiss cheese" variant. CONCLUSIONS: As we show, appropriate surgical management requires an understanding of the specific terminology, as the surgical approach may differ depending on the combination of the individual defects. Data from the Society for Thoracic Surgeons revealed that both mortality and morbidity were increased in the setting of multiple as opposed to solitary ventricular septal defects.

Changing Pattern of Congenital Heart Disease Care During COVID-19 Pandemic.

OBJECTIVE: To study the impact of coronavirus disease 2019 (COVID-19) pandemic on the utilization of pediatric cardiac care services and to determine the role of teleconsultation services in delivering healthcare in this subset of population. METHODS: It was a retrospective, observational study. All children who attended pediatric cardiology outpatient/teleconsultation services or were admitted to pediatric cardiology ward between April 1, 2019 to July 31, 2019 and April 1, 2020 to July 31, 2020, were recruited in the study. Data for patients who underwent surgery or catheter intervention for congenital heart disease were also recorded and analyzed. Comparisons were drawn between the statistics during the two time-periods. RESULTS: Physical outpatient services were discontinued and were replaced by teleconsultations from April 2020. Inpatient admissions during COVID-19 pandemic (n = 66) decreased by two-thirds as compared to the admissions during similar period in 2019 (n = 189). Similarly, the percentage decrease during these 4 mo of pandemic were 84% for catheter interventions, 90% for total congenital heart disease (CHD) surgeries, and 40% for emergency CHD surgeries. The number of patients availing successful teleconsultation was 1079, which was only 15% of the total number of patients attending physical outpatient services (n = 7176) during the corresponding period in the year 2019. During the pandemic, systematic teleconsultation and local evaluation and investigations aided in better management of patients with CHD. CONCLUSIONS: The utilization of cardiovascular services for CHD has reduced significantly during COVID-19 pandemic, for both out- and inpatient care. Teleconsultation services have streamlined the follow-up care to some extent and have helped in noncontact triaging of these patients for further care.

Effect of acute lower respiratory tract infection on pulmonary artery pressure in children with post-tricuspid left-to-right shunt.

We sought to examine the influence of clinically severe lower respiratory tract infection on pulmonary artery pressure in children having CHD with post-tricuspid left-to-right shunt, as it may have physiological and clinical implications. In a prospective single-centre observational study, 45 children with post-tricuspid left-to-right shunt and clinically severe lower respiratory tract infection were evaluated during the illness and 2 weeks after its resolution. Pulmonary artery systolic pressure was estimated non-invasively using shunt gradient by echocardiography and systolic blood pressure measured non-invasively.Median pulmonary artery systolic pressure during lower respiratory tract infection was only mildly (although statistically significantly) elevated during lower respiratory tract infection [60 (42-74) versus 53 (40-73) mmHg, (p < 0.0001)]. However, clinically significant change in pulmonary artery systolic pressure defined as the increase of >10 mmHg was present in only 9 (20%) patients. In the absence of hypoxia or acidosis, only a small minority (9%, n = 4) showed significant pulmonary artery systolic pressure rise >10 mmHg. In the absence of hypoxia or acidosis, severe lower respiratory tract infection in patients with acyanotic CHD results in only mild elevation of pulmonary artery systolic pressure in most of the patients.

Characteristics of Children with Acute Rheumatic Carditis from a High-Incidence Region: Importance of Unexplained Worsening of Functional Class.

BACKGROUND: Acute rheumatic fever (ARF) and acute rheumatic carditis (ARC) continue to be a major public health problem in developing countries. OBJECTIVE: To study the characteristics of children with ARC being treated at a tertiary centre. METHODS AND RESULTS: We studied 126 children (mean age 10.4 ± 2.3 years, range 5-15 years, 60% males) diagnosed with ARC by treating cardiologists. Most had lower socio-economic status. Fifty of 126 (40%) presented with a first episode of ARC. Joint symptoms were present in 29% and fever in 25%. Only 2.4% had subcutaneous nodules and none had erythema marginatum or chorea. Fifty-one percent presented in NYHA class II and 29% in NYHA class III or IV. Tachycardia and heart failure were present in 53% and 21%, respectively. Recent worsening of NYHA class (dyspnoea) was the commonest feature (48%). Laboratory investigations showed raised antistreptolysin O titres (>333 units) in only 36.7% of patients. Raised C-reactive protein (CRP) was present in 70%, while raised erythrocyte sedimentation rate was found in only 37% of patients. On the basis of above findings, the modified Jones criteria (2015) for the diagnosis of ARF were satisfied only in 46% of children. Echocardiography showed mitral valve thickening in 77% and small nodules on the tip of the leaflets in 43% (27 and 8%, respectively for aortic valve). Left ventricular ejection fraction was <50% in only 3 patients. The dominant valve lesion was mitral regurgitation (MR) (present in 95% of patients; severe in 78%, moderate in 15%), while aortic regurgitation was present in 44% (severe in 14%). CONCLUSIONS: The criteria are often not satisfied by patients being treated for ARC. Recent unexplained worsening of dyspnoea, young age, significant MR, echocardiographic nodules, and elevated CRP are important indicators.

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.

Surgical repair for aortopulmonary window with interrupted aortic arch in late childhood.

Aortopulmonary window with interrupted aortic arch is rarely reported beyond infancy. Pre-operative assessment and surgical repair are challenging. We report successful surgical repair of aortopulmonary window with interrupted aortic arch in a 6-year-old girl with near-normal pulmonary artery pressure immediately following 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.

A number of guidelines are available for the management of congenital heart diseases (CHD) 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 CHD, as often these patients present late in the course of the disease and may have coexisting morbidities and malnutrition. Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on August 10 and 11, 2018, at the All India Institute of Medical Sciences. The meeting was supported by Children's HeartLink, a nongovernmental organization based in Minnesota, USA. The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common CHD; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for CHD; and (iii) indications for use of pacemakers in children. Evidence-based recommendations are provided for indications and timing of intervention in common CHD, 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 CHD (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein's anomaly, and others). In addition, protocols for follow-up of postsurgical patients are also described, disease wise. Guidelines are also given on indications for implantation of permanent pacemakers in children.

Guidelines for the management of common congenital heart diseases in India: A consensus statement on indications and timing of intervention.

INTRODUCTION: 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 the 10th and 11th of August, 2018 at the All India Institute of Medical Sciences. OBJECTIVES: The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases and (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, obstructive lesions, and cyanotic congenital heart diseases. In addition, protocols for follow-up of postsurgical patients are also described.

Right superior caval vein to the left atrium in a child with vein of Galen malformation.

Vein of Galen malformation (VGM) is a rare intracranial vascular malformation. High output heart failure is common in infancy and is characterized by dilatation of all the cardiac chambers. We report an unusual case of VGM in a 3-month-old infant without dilatation of the right-sided cardiac chambers. We then demonstrate importance of comprehensive evaluation in detecting rare coexistence of anomalous right superior caval vein connection to the left atrium.

Aortic atresia with normal sized left ventricle.

Aortic atresia with an associated ventricular septal defect and adequate sized left ventricle is extremely rare. We present two cases in which an alternate diagnosis was suggested on echocardiography because the hypoplastic aortic trunk was missed due to its small caliber. The final diagnosis was, however, clinched on dual source computed tomography, which not only showed the thin aortic trunk but also clearly depicted the coronary artery origins from the hypoplastic aortic root. To the best of our knowledge, use of multi-detector computed tomography in aortic atresia with well developed left ventricle has not been reported in literature till date.

Clarifying the anatomy of hearts with concordant ventriculo-arterial connections but abnormally related arterial trunks.

Hearts in which the arterial trunks arise from the morphologically appropriate ventricles, but in a parallel manner, rather than the usual spiralling arrangement, have long fascinated anatomists. These rare entities, for quite some time, were considered embryological impossibilities, but ongoing experience has shown that they can be found in various segmental combinations. Problems still exist about how best to describe them, as the different variants are often described with esoteric terms, such as anatomically corrected malposition or isolated ventricular inversion. In this review, based on our combined clinical and morphological experience, we demonstrate that the essential feature of all hearts described in this manner is a parallel arrangement of the arterial trunks as they exit from the ventricular mass. We show that the relationship of the arterial roots needs to be described in terms of the underlying ventricular topology, rather than according to the arrangement of the atrial chambers. We then discuss the importance of determining atrial arrangement on the basis of the morphology of the appendages, following the precepts as set out in the so-called "morphological method" and distinguished according to the extent of the pectinate muscles relative to the atrioventricular junctions as opposed to basing diagnosis on the venoatrial connections. We show that, when approached in this manner, the various combinations can be readily diagnosed in the clinical setting and described in straightforward way.

Perforating the atretic pulmonary valve with CTO hardware: Technical aspects.

OBJECTIVES: To review the success and technical aspects of pulmonary valve (PV) perforation using chronic total occlusion (CTO) hardware in patients with pulmonary atresia and intact ventricular septum (PA-IVS). BACKGROUND: Interventional therapy is possible in selected patients with PA-IVS. Among the various interventional options available, radiofrequency and laser assisted perforation may be more successful, but require expertise and may be substantially costly. METHODS: We describe the technique of mechanical catheter PV perforation using currently available coronary hardware meant for coronary CTO in nine cases with PA-IVS. After complete echocardiographic evaluation and informed parental consent was obtained, patients were electively intubated, mechanically ventilated, adequately heparinized and were placed on intravenous prostaglandin infusion. Basic steps involved were-localizing the atretic segment and accomplishing coaxial alignment of catheters using biplane fluoroscopy, crossing the atretic segment with the soft end of perforating guidewire, stabilizing the assembly and performing graded balloon dilatation with the balloon size never exceeding 130% of pulmonary annulus diameter. For crossing the atretic PV, a retrograde approach was used in one patient where the antegrade approach was not possible. RESULTS: The procedure was successful in 8/9 cases (89%). Valve opening was achieved in all eight patients with immediate fall in right ventricular (RV) systolic pressures. One neonate died following surgery after catheter induced RV perforation. All surviving cases were discharged from the hospital in good general condition with no evidence of heart failure and a room air oxygen saturation of >85%. No patient required an additional pulmonary irrigation procedure. CONCLUSION: With appropriate patient and hardware selection, PV perforation using readily available coronary hardware is feasible in PA-IVS. © 2014 Wiley Periodicals, Inc.

Saline Contrast Echocardiography in the Era of Multimodality Imaging--Importance of "Bubbling It Right".

Saline contrast echocardiography is an established imaging modality. Logical interpretation of a carefully performed study is vital to realize its diagnostic potential. In this review, we discuss utility of saline contrast echocardiography in evaluation of various pathologies within and outside the heart other than a patent foramen ovale.

A report on 5(th) congress of Asia Pacific Pediatric Cardiac Society, New Delhi, India, 6-9 March 2014.

The 5(th) Congress of Asia Pacific Pediatric Cardiac Society was held in New Delhi from 6-9 March 2014. This article describes the journey of preparing and hosting one of the largest international events in the specialty of Pediatric Cardiac Care ever held in India. A total of 938 delegates, including 400 from outside India, participated. The scientific program was inclusive keeping in mind the diverse background of delegates from the member nations. Large numbers of research papers were presented, mostly by fellows in training.

How frequent is the fifth arch artery?

Although usually shown in embryology textbooks, the presence of the fifth pair of pharyngeal arch arteries has long been controversial. To the best of our knowledge, six pairs of bilaterally symmetrical arteries developing within the pharyngeal arches are yet to be found in any mammalian or avian species. Collateral channels between the distal ends of the fourth and sixth arch arteries, in contrast, have been found in up to half of all developing mouse embryos. In only one human embryo, again to the best of our knowledge, has a channel been found that extends from the aortic sac to the dorsal aorta, and hence qualifies as an arch artery. Despite these confounding factors in terms of the developmental heritage of the fifth arch arteries, the purported channels are invoked with increasing frequency to describe various lesions discovered in the setting of the congenitally malformed heart. Persistence of the artery of the fifth arch was initially proposed to explain double-barrelled aorta. It was subsequently proposed to account for various systemic-to-pulmonary channels feeding the pulmonary circulation in the setting of pulmonary atresia. It has also been claimed to persist so as to explain abnormal branching of the brachiocephalic arteries from the aortic arch. In the light of the ongoing doubts concerning the existence of the arteries of the fifth arch themselves, we have reviewed the various descriptions of purported fifth arch arteries within the world literature. We have then sought to validate the descriptions on the basis of our own understanding of development, for this purpose providing images of the remoulding arch arteries in the mouse so as to substantiate our conclusions. While accepting that our own interpretations are speculative, we suggest that more convincing alternative explanations can be advanced to account for the majority of lesions currently interpreted on the basis of persistence of the arteries of the fifth arches. Although the interpretations do not necessarily change the therapeutic approaches to the channels, appropriate description is important in terms of their classification.

Evaluation of patient satisfaction after non-vital bleaching in traumatized discolored intact anterior teeth.

AIM: This study evaluated patient satisfaction and clinical effectiveness of intracoronal bleach in traumatized anterior discolored intact teeth. Assessment of awareness and knowledge of the patient regarding treatment modalities for such cases was also performed. MATERIAL AND METHOD: Forty-one patients were included in the study. Walking bleach was performed using sodium perborate (tetrahydrate) after root canal treatment and proper cervical seal. Patient was recalled after 1 week for evaluation. The bleaching procedure was repeated again till the patient agreed for it. On each recall visit, shade evaluation was performed using the Vita Classic shade guide. On completion of treatment, the patients were questioned as to their satisfaction level. Statistical analysis was carried out using anova. RESULTS: 87.8% of the subjects were highly satisfied with the results obtained; 7.32% were satisfied, while 4.9% were not satisfied. Average number of appointments needed for highly satisfied group was 2.75, and average shade change obtained was 11.97. Significantly more number of appointments were needed for the older patients (P < 0.01) or patients with older trauma (P < 0.05)/discoloration (P < 0.05). Severity of discoloration did not affect the number of appointments (P > 0.05). Most of the participants (97.6%) were not aware of bleaching procedure. The aspect of this treatment, which satisfied them the most, was improvement of the tooth coloration followed by conservation of tooth structure while the most perplexing thing was the unpredictability of final shade achieved and lack of color stability. CONCLUSIONS: High level of patient satisfaction after non-vital tooth bleach treatment strongly supports this treatment modality to be a conservative treatment alternative for traumatized discolored anterior tooth.