CSI clinical practice guidelines for dyslipidemia management: Executive summary.

Dyslipidemias are the most important coronary artery disease (CAD) risk factor. Proper management of dyslipidemia is crucial to control the epidemic of premature CAD in India. Cardiological Society of India strived to develop consensus-based guidelines for better lipid management for CAD prevention and treatment. The executive summary provides a bird's eye-view of the 'CSI: Clinical Practice Guidelines for Dyslipidemia Management' published in this issue of the Indian Heart Journal. The summary is focused on the busy clinician and encourages evidence-based management of patients and high-risk individuals. The summary has serialized various aspects of lipid management including epidemiology and categorization of CAD risk. The focus is on management of specific dyslipidemias relevant to India-raised low density lipoprotein (LDL) cholesterol, non-high density lipoprotein cholesterol (non-HDL-C), apolipoproteins, triglycerides and lipoprotein(a). Drug therapies for lipid lowering (statins, non-statin drugs and other pharmaceutical agents) and lifestyle management (dietary interventions, physical activity and yoga) are summarized. Management of dyslipidemias in oft-neglected patient phenotypes-the elderly, young and children, and patients with comorbidities-stroke, peripheral arterial disease, kidney failure, posttransplant, HIV (Human immunodeficiency virus), Covid-19 and familial hypercholesterolemia is also presented. This consensus statement is based on major international guidelines (mainly European) and expert opinion of lipid management leaders from India with focus on the dictum: earlier the better, lower the better, longer the better and together the better. These consensus guidelines cannot replace the individual clinician judgement who remains the sole arbiter in management of the patient.

Impact of COVID-19 pandemic on pediatric cardiac services in India.

BACKGROUND: COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID.19 on pediatric cardiac care in India. AIMS: The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. SETTINGS AND DESIGN: This is a retrospective, multicentric, observational study. METHODS: We collected monthly data on the number and characteristics of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries and major hospital statistics, over a period of 5 months (April to August 2020), which coincided with the first wave of COVID-19 pandemic in India and compared it with data from the corresponding months in 2019. RESULTS: The outpatient visits across the 24 participating pediatric cardiac centers decreased by 74.5% in 2020 (n = 13,878) as compared to the corresponding period in 2019 (n = 54,213). The reduction in the number of hospitalizations, cardiac surgeries, and catheterization procedures was 66.8%, 73.0%, and 74.3%, respectively. The reduction in hospitalization was relatively less pronounced among neonates as compared to infants/children (47.6% vs. 70.1% reduction) and for emergency surgeries as compared to elective indications (27.8% vs. 79.2%). The overall in-hospital mortality was higher in 2020 (8.1%) as compared to 2019 (4.8%), with a higher postoperative mortality (9.1% vs. 4.3%). CONCLUSIONS: The current COVID-19 pandemic significantly impacted the delivery of pediatric cardiac care across India with two-third reduction in hospitalizations and cardiac surgeries. In an already resource-constrained environment, the impact of such a massive reduction in the number of surgeries could be significant over the coming years. These findings may prove useful in formulating strategy to manage subsequent waves of ongoing COVID-19 pandemic.

Early multicenter experience of Melody valve implantation in India.

BACKGROUND: Transcatheter valves provide a safe and effective alternative to surgery for treating dysfunctional right ventricular outflow tracts (RVOTs). We present our early multicenter experience of percutaneous pulmonary valve implantation (PPVI) using Melody valve (Medtronic Inc., Minneapolis, MN). METHODS: Patients with stenosed conduits or degenerated bioprosthetic valves in RVOT with combined stenosis and regurgitation were evaluated for suitability of Melody valve implantation. After undergoing an initial structured training, PPVI using Melody transcatheter pulmonary valve (TPV) was guided by an approved proctor. Conduits were serially dilated and prestented with careful coronary interrogation, and bioprosthetic valves were dilated with high-pressure balloons. Clinical and echocardiographic follow-up was performed at 6 monthly intervals. RESULTS: Fifteen patients (three females) aged 23.1 ± 9.5 years in NYHA Class II-III underwent Melody TPV implantation in four Indian centers. The underlying anatomy comprised surgically implanted bioprosthetic valves for pulmonary regurgitation (n= 5), conduit repair for pulmonary atresia (n = 4), Rastelli repair (n = 3), truncus (n = 1), and Ross procedure (n = 2). Twelve patients had more than one previous surgery. Doppler gradient decreased from 74.2 ± 21.5 mmHg to 10.2 ± 4.5 mmHg after the PPVI. At a median follow-up of 14 months (1-39 months), all the patients were in NYHA Class I with echocardiographic gradients of 8 ± 5.7 mmHg with no evidence of pulmonary regurgitation. There were no major procedural adverse events or deaths. CONCLUSIONS: Our early experience shows encouraging results of the PPVI program in India with proctored case selection and meticulous planning. It also confirms the safety and efficacy of Melody TPV for treating dysfunctional RVOT in postoperative patients.

Percutaneous paravalvular leak closure with their outcomes: A single center experience.

Background: Transcatheter paravalvular leak (PVL) closure in recent times has emerged as a safe and effective alternate to redo-surgical repair. We sought to examine the clinical efficacy and safety of percutaneous PVL closure at our center. Methods and Results: A retrospective study from August 2012 to December 2019 of 19 patients who underwent 21 procedures for PVL closure. The mean age was 49.25 ± 14.72 years. The target valve was mitral in 11 (57%) and aortic in 7 (36%) cases. One (5%) patient had prosthetic valve in left atrioventricular valve with congenitally corrected transposition of great arteries. Majority of the cohort presented with heart failure without hemolysis (89%), with most of them being in NYHA functional class III (57%) or class IV (21%). A procedural success of 85% was achieved. Post procedure severity of regurgitation reduced from severe in thirteen patients and moderate in six patients to moderate in two patients and mild in fourteen patients. Symptomatic improvement was observed in all cases who had successful closure with NYHA function class improving from 3 ± 0.64 to 1.6 ± 0.94. The mean follow-up duration was 21 ± 13 months (median 24 months). There was one (4.7%) mortality with cumulative survival from all-cause mortality of 95%. Conclusion: The results of percutaneous PVL closure appear encouraging in our series with modest number of patients and offers a promising alternative to redo-surgery in this high-risk cohort.

A systematic approach to epicardial echocardiography in pediatric cardiac surgery: An important but underutilized intraoperative tool.

Intraoperative echocardiography is an integral component of the peri-operative management of pediatric heart disease. It confirms the adequacy of surgery, identifies residual lesions, and can provide useful hemodynamic data. It, therefore, helps to decide on the need for revision of repair and guides the postoperative management strategy. Intraoperative echocardiography is done with the use of either an epicardial probe or a transesophageal probe. Epicardial echocardiography is a simple, useful modality and has the ability to perform imaging in cases where transesophageal echocardiography cannot be easily performed, for example, in low birth weight babies. We attempt to describe in detail the technique of epicardial echocardiography and the various views that we have found useful for a complete postsurgical evaluation. The limitations of the technique are also discussed in detail.

Isolated absent right pulmonary artery in an infant with 22q11 deletion.

DiGeorge syndrome is a rare spectrum of disorder affecting structures derived from third and fourth pharyngeal pouches characterised by aplasia or hypoplasia of thymus and parathyroid glands, and conotruncal anomalies. Presentation includes infants with hypocalcemic seizures, CHD, or recurrent infection. This case report illustrates a unique combination of proximal interruption of right pulmonary artery and aberrant right subclavian artery in a 3-month-old infant who was subsequently diagnosed as DiGeorge syndrome. This constellation of vascular anomalies in an infant with DiGeorge syndrome has not been previously reported in the literature.

Clinical profile of Kawasaki disease in children admitted at a tertiary care hospital of North India and their short-term follow-up.

Aim: The aim of this study was to evaluate presenting symptoms, clinical features, and laboratory tests for the diagnosis of Kawasaki disease (KD) in children and their short-term follow-up at a tertiary care hospital of North India from April 2017 to March 2020. Materials and Methods: A total of 31 children (23 boys and 8 girls) up to 10 years of age were included in this study. The diagnosis of KD was made as per the American Heart Association 2017 guidelines. Clinical features, laboratory parameters, and coronary involvement were compared between the complete and incomplete KD groups. Results: The incidence of complete versus incomplete KD was 19 (61.2%) versus 12 (38.7%) children, respectively. Change in extremities and oral mucosal changes were more encountered in the complete KD group as compared to the incomplete KD group (100% vs. 58.3%, P = 0.004, and 78.9% vs. 33.3%, P = 0.002, respectively). Coronary artery aneurysm was seen in 54% of the patients on echocardiography which was greater in the incomplete KD group (83.3%) as compared to the complete KD group (36.8%). The median time from the onset of symptoms to intravenous immunoglobulin infusion was <10 days in 84.2% of the patients with complete KD versus 41.7% with incomplete KD which was statistically significant. Fifty percent of the children with coronary ectasia and small aneurysm had normal coronaries at follow-up of 6 months. Conclusion: KD is probably underdiagnosed in most developing countries, like that of ours, and requires a high index of suspicion.

Double outlet of both ventricles: morphological, echocardiographic and surgical considerations.

OBJECTIVES: To describe the morphology, echocardiographic features and surgical management of the entity appropriately described as 'double outlet of both ventricles'. METHODS: Seven patients (5 males, age 0.5-7.5 months) were diagnosed to have a unique form of subarterial ventricular septal defect (VSD) and ventriculo-arterial connection, where a muscular outlet septum straddled the crest of the ventricular septum in a cruciate manner, such that both great arteries were equally committed to both ventricles. Diagnosis was established by echocardiography, with 6 patients submitted to surgical repair by means of intracardiac routing of the left ventricle to the aorta using 2 patches. RESULTS: Surgical repair was successful in all 6 patients in whom it was attempted. In addition, 1 patient underwent concomitant repair of aortic coarctation, and 2 had closure of multiple VSDs. We lost 1 patient to follow-up after diagnosis. Follow-up with a range from 3 months to 8 years in the remaining patients revealed all to be clinically well with satisfactory growth of both outflow tracts. CONCLUSIONS: We describe a series of patients with the ventriculo-arterial connection best described as 'double outlet of both ventricles'. Diagnosis is readily established by echocardiography. Good early and midterm results can be expected subsequent to surgical repair using 2 patches for interventricular septation.

Mid-Term Follow-Up of Neonatal Neochordal Reconstruction of Tricuspid Valve for Perinatal Chordal Rupture Causing Severe Tricuspid Valve Regurgitation.

BACKGROUND: Papillary muscle rupture in the perinatal period is a rare event that leads to severe mitral or tricuspid insufficiency due to a flail leaflet. Neonatal tricuspid chordal reconstruction for this condition is rarely reported. Early recognition and treatment have the potential to be lifesaving. We present our surgical experience with five such patients, along with their midterm follow-up. METHODS: Between August 2010 and November 2012, five neonates (aged 1-30 days) underwent surgery for severe atrioventricular valve regurgitation. All neonates had severe tricuspid regurgitation due to ruptured chordae. In addition, two had moderate mitral regurgitation; one due to ruptured chordae of the posterior mitral leaflet and the other due to prolapse of the anterior mitral leaflet. All underwent emergent surgery where the ruptured chordae to the anterior tricuspid leaflet were replaced with neochordae made with expanded polytetrafluoroethylene (ePTFE) suture. The mitral valve was repaired in two patients. RESULTS: All patients survived surgery without the need for postoperative mechanical circulatory assist. Predischarge echocardiograms showed good coaptation of tricuspid and mitral leaflets with minimal regurgitation in all. At follow-up between 75 months to 102 months, four patients had excellent outcomes with less than mild tricuspid regurgitation. One child with flail tricuspid and mitral leaflets developed progressive tricuspid and mitral regurgitation requiring surgical re-repair at 20 months following the initial surgery. CONCLUSION: Repair of chordal rupture of the tricuspid valve in neonates using ePTFE neo-chordae can provide acute salvage and gratifying midterm results in the management of this potentially fatal condition.

Multiple diffuse coronary cameral fistulas from the left anterior descending artery and right coronary artery to both the right and left ventricle associated with left ventricular noncompaction: A rare combination.

Diffuse and multiple coronary cameral fistulas are very rare and with very few case reports of its association with left ventricular noncompaction are published. Here, we report a 6-year-old child of multiple diffuse coronary cameral fistulas to both the right and left ventricle in association with the left ventricular noncompaction. A possible common embryological link between the two uncommon entities is also discussed.

Non-obstructive membrane of left atrial appendage orifice: first paediatric report.

Left atrial appendage membrane is a rare anomaly. Its implications on clinical course are unknown. We report the first paediatric case in association with a congenital heart disease.

Rare Combination of Pathologies Causing Mitral Stenosis and Mitral Regurgitation: A Case Report.

A supramitral ring is a rare cause of mitral stenosis, while an isolated mitral valve cleft is a rare cause of congenital mitral regurgitation. Fortunately, both the lesions are known to have good outcomes after surgical correction. Although each is known to be associated with a variety of other structural heart defects, their coexistence has not been reported previously. We report a case of a three- and half-year-old boy detected to have a rare combination of supramitral ring producing severe mitral stenosis with a coexisting cleft in the anterior leaflet of mitral valve causing severe mitral regurgitation. The patient underwent successful surgical repair with resolution of both mitral stenosis and regurgitation.

Atypical Left Ventricular Myxoma: Unusual Echocardiographic and Histopathological Features.

Cardiac myxomas are the most common primary cardiac tumors and are typically attached to the interatrial septum. Left ventricular myxomas are exceedingly rare and presentation in children is all the more uncommon. We report a case of left ventricular myxoma with very atypical cystic appearance raising an initial suspicion of a hydatid cyst. Subsequently, cardiac magnetic resonance imaging was done, which ruled out the diagnosis of hydatid cyst. Complete surgical excision was done through transaortic and transmitral route. Histopathological examination revealed it to be a cardiac myxoma with vascular proliferation, which on echocardiography had appeared as a polycystic lesion. This is a very unusual histopathological presentation of cardiac myxoma.

Left Superior Vena Cava Draining Into Left Atrium in Tetralogy of Fallot-Four Cases of a Rare Association.

Persistent left superior vena cava is a common congenital anomaly of the thoracic venous system. Left superior vena cava draining into left atrium is a malformation of sinus venosus and caval system. The anomaly may be a cause of unexplained hypoxia even in adults. It may give rise to various diagnostic and technical challenges during cardiac catheterization and open-heart surgery. It is often detected serendipitously during diagnostic workup. Isolated left superior vena cava opening into left atrium is very commonly associated with other congenital heart defects. But tetralogy of Fallot is very rarely associated with persistent left superior vena cava which drains into left atrium. We report four such cases who underwent surgical correction successfully.

Transcatheter closure of right pulmonary artery to left atrium fistula in an infant: technical consideration and possible closure techniques.

Congenital right pulmonary artery to left atrium fistula is rare cause of cyanosis. It is an abnormal fistulous connection between right pulmonary artery and left atrium representing a direct communication between a pulmonary artery and vein with absence of capillary network connecting between these two. Cardiovascular examination usually remains normal. High index of suspicion on clinical examination and subsequent transthoracic echocardiography is needed to suspect this entity. Bubble contrast echocardiography usually confirms the diagnosis. Cardiac catheterization is used as diagnostic as well as therapeutic modality. Cardiac catheterization is useful in classifying the fistula and helps in transcatheter closure by embolization devices. Choice of devices depends on type of fistula, vascular access, and presence of atrial communication. Here, we are reporting 8-month-old girl presenting with type 2 right pulmonary artery to left atrium fistula, which underwent successful transcatheter closure by 6 mm/4 mm duct occluder (Heart R, Lifetech Scientific, Shenzhen, China). Early closure in this young age will prevent complications of cyanosis. The technical consideration, possible access, and closure techniques are discussed in this young infant.

Dual Communication of Confluence of Total Anomalous Pulmonary Venous Connection: An Interesting but a Rare Entity.

Here, we describe the case of a 9-month-old male child who was diagnosed with a very unusual and rare variety of mixed total anomalous pulmonary venous connection.

Dual Communication of Confluence of Total Anomalous Pulmonary Venous Connection: An Interesting but a Rare Entity.

Here, we describe case of a 9-month-old male child who was diagnosed with a very unusual and rare variety of mixed total anomalous pulmonary venous connection.

Surgical Options for Uhl's Anomaly.

BACKGROUND: Uhl's anomaly is an extremely rare congenital heart defect characterized by a near total absence of the myocardium of the parietal wall of the right ventricle. Few reports of surgical management exist in literature. We present three patients with this anomaly who were managed with different surgical strategies. PATIENTS AND METHODS: Patient 1: This 43-month-old girl had maternal rubella syndrome with speech and hearing deficits and gross right heart failure. Diagnosis was made on echocardiography and magnetic resonance imaging. She underwent partial excision and plication of the right ventricular parietal wall and total cavopulmonary connection. Patient 2: This 19-month-old boy presented with progressive cyanosis and features of right heart failure. Diagnosis was confirmed on echocardiography. He underwent right ventricular exclusion by tricuspid valve closure and free wall plication followed by a bidirectional Glenn procedure. Patient 3: This 21-year-old male presented with right heart failure and cyanosis. Diagnosis was established with transesophageal echocardiography and magnetic resonance imaging. As the hemodynamics were not suitable for a Fontan conversion, a one and a half ventricle repair was done along with plication of the right ventricular free wall and tricuspid valve annuloplasty. RESULTS: All three patients were extubated within 24 hours. Patients 2 and 3 were discharged relatively uneventfully, whereas patient 1 had a more prolonged stay due to transient hepatic failure. All were symptomatically and clinically improved on short-term follow-up. CONCLUSION: Uhl's anomaly is an extremely rare condition with varied clinical presentation. Surgical exclusion of the right ventricle yields gratifying results; however, surgical technique has to be adapted to the individual patient.

Off-pump midline repair of coarctation of aorta when associated with intracardiac defects: an alternate approach for single-stage correction.

OBJECTIVES: Strategies for the optimal surgical management of coarctation of aorta with associated intracardiac defects continue to be debated upon. We describe a previously unreported surgical technique for the management of this combination of defects and present our early results. METHODS: Thirty-one patients (median age: 73 days and median weight: 3.9 kg) underwent repair of coarctation of aorta with associated cardiac defects, in a single-stage, through a midline sternotomy. Twenty-eight patients had associated distal arch hypoplasia and 1 had a type I interruption. The coarctation and when necessary, the distal arch, was repaired prior to the institution of cardiopulmonary bypass. Cardiopulmonary bypass was subsequently instituted for the repair of the associated intracardiac defects. RESULTS: All patients had successful repair of coarctation and distal arch hypoplasia without the need for rescue cardiopulmonary bypass. In addition, 29 patients had closure of one or more ventricular septal defects, 3 patients had an arterial switch procedure and 1 had repair of double outlet right ventricle. Repair of supramitral ring and subaortic membrane was performed in another 3 patients. One patient underwent concomitant mitral valve repair. There was one in-hospital mortality (3.22%) due to fulminant sepsis. The median follow-up was 29.5 months (range: 1-58 months). There was no late mortality. Two patients required balloon dilatation for recurrent coarctation. Overall event-free survival rate was 88.5% at both 2 and 3 years of follow-up after surgery. However, intervention-free survival at 2 and 3 years of follow-up was 91.7% among the survivors. CONCLUSION: Repair of coarctation and distal arch hypoplasia or type I arch interruption is feasible prior to the institution of cardiopulmonary bypass without the use of hypothermic circulatory arrest or regional cerebral perfusion, with acceptable and reproducible results.

Fetal left ventricular myocardial performance index: Defining normal values for Indian population and a review of literature.

OBJECTIVE: The aim of this study was to determine normal values for fetal left ventricular (LV)-myocardial performance index (MPI) in Indian population and to assess its relation to advancing gestation and fetal heart rate (FHR). MATERIALS AND METHODS: Two hundred pregnant women without any pregnancy-related complications and whose fetuses were shown to have structurally normal hearts were enrolled in this study. Doppler waveform involving simultaneous display of mitral inflow and LV outflow was obtained in all. Various intervals including isovolumetric contraction time (IVCT), isovolumetric relaxation time (IVRT), and ejection time (ET) were measured and then the MPI was calculated using the formula IVCT + IVRT/ET. Also the correlation between MPI and gestation age and FHR was assessed. We also reviewed the literature on the use of MPI for the assessment of fetal LV function. RESULTS: The normal MPI in second and third trimester fetuses of Indian population was 0.42 ± 0.03. The mean IVCT was 33 ± 4 milliseconds (ms), mean IVRT was 39 ± 5 ms, and mean ET was 169 ± 9 ms. The mean heart rate was 148 ± 8 bpm and the mean PR interval was 111 ± 10 ms. There was no significant association of LV-MPI with either FHR or advancing gestation. CONCLUSION: MPI is a useful parameter for the assessment global cardiac function. MPI has the advantage of not being affected by FHR, ventricular size, and geometry or image quality. The review of literature shows its significant importance in monitoring complicated pregnancies.