Early and Long-Term Clinical Outcomes of Ductal Stenting Versus Surgical Aortopulmonary Shunt Among Young Infants with Duct-Dependent Pulmonary Circulation.

Surgical aortopulmonary shunting (SAPS) and ductal stenting (DS) are the main palliations in infants with cyanotic congenital heart diseases (CHD). We aimed to study the safety and efficacy of DS and to compare it with SAPS as a palliative procedure in infants with CHD and duct-dependent pulmonary circulation. Retrospective institutional clinical data review of consecutive infants aged < 3 months who underwent DS or SAPS over 5 years. The primary outcome was procedural success which was defined as event-free survival (mortality, need for re-intervention, procedural failure) at 30 days post-procedure. The secondary outcome was defined by a composite of death, major adverse cardiovascular events, or need for re-intervention at 6 months and on long-term follow-up. We included 102 infants (DS, n = 53 and SAPS, n = 49). The median age at DS and SAPS was 4 days (IQR 2.0-8.5) and 8 days (IQR 4.0-39.0), respectively. The median weight at intervention was 3.0 kg (IQR 3.0-3.0) and 3.0 kg (IQR 2.5-3.0) in the two respective arms. Tetralogy of Fallot with pulmonary atresia was the most common indication for DS and SAPS. The 30-day mortality was significantly higher in SAPS group as compared with DS group (p < 0.05). However, 30-day major adverse cardiac events (MACE) rates were similar in both groups (p = 0.29). DS was associated with shorter duration of mechanical ventilation, duration of stay in the intensive care and hospital stay than with SAPS. At 6 months, there was no significant difference in terms of mortality or event-free survival. Long-term MACE-free survival was also comparable (p = 0.13). DS is an effective and safer alternative to SAPS in infants with duct-dependent pulmonary circulation, offering reduced procedure-related mortality and morbidity than SAPS. Careful study of ductal anatomy is crucial to procedural success. However, long-term outcomes are similar in both procedures.

Clinical profile and outcomes of pediatric hypertrophic cardiomyopathy in a South Indian tertiary care cardiac center: a three decade experience.

INTRODUCTION: Although much research has been done on adult hypertrophic cardiomyopathy, data on pediatric hypertrophic cardiomyopathy is still limited. METHODS AND RESULTS: The study enrolled all patients with cardiomyopathy who presented to us between 1990 to 2020 and were younger than 18 yrs. During the thirty-year study period, we identified 233 cases of pediatric cardiomyopathy. Sixty-three cases (27%) had hypertrophic cardiomyopathy. Out of the 63 HCM cases, 12% presented in the neonatal period and 37% presented in the first year of life. The median age of presentation was 7 yrs (Range 0.1-18 yrs). Sixteen patients had proven syndromic, metabolic, or genetic disease (25%). LV outflow obstruction was present in 30 patients (47%). Noonan syndrome was present in 9 of the 63 patients (14%). Dyspnea on exertion was the most common mode of presentation. Cardiac MRI was done in 28 patients, out of which 17 had late gadolinium enhancement (LGE). Mid myocardial enhancement was the most common pattern. Four patients had LGE of more than 15%. Over a mean follow-up period of 5.6 years (0.1-30 years), twenty-one were lost to follow-up (33%). Among the patients whose outcome was known, eleven died (26%), and thirty-one (73%) were alive. The 5-year survival rate of HCM patients was 82%, and the 10-year survival rate was 78%. Seven died of sudden cardiac death, three from heart failure, and one from ventricular arrhythmias. Sustained ventricular arrhythmias were seen in three patients and atrial arrhythmias in two. First-degree AV block was seen in 10 patients (15%) and bundle branch blocks (BBB) in five (8%). Eight patients required ICD or transplant (12.7%). Two patients underwent ICD for primary prevention, and one underwent PPI for distal AV conduction disease. Among the various clinical, echocardiographic, and radiological risk factors studied, only consanguinity showed a trend towards higher events of death or ventricular arrhythmias (P-value 0.08). CONCLUSION: More than one-third of our HCM cohort presented in infancy. LV outflow tract obstruction is common (47%). Mid myocardial enhancement was the most common pattern of late gadolinium enhancement. SCD was the most common cause of death. The outcome in our HCM cohort is good and similar to other population cohorts. Only Consanguinity showed a trend towards higher events of death or ventricular arrhythmias.

A Comparative Study of Invasive Modalities for Evaluation of Pulmonary Arteriovenous Fistula after Bidirectional Glenn Shunt.

Development of pulmonary AV fistula (PAVF) after bidirectional glenn shunt (BDG) results in significant cyanosis, impaired exercise performance, and increased morbidity and mortality. We attempted to detect and quantify PAVF in post-BDG patients by saline contrast transesophageal echocardiography (TEE) and compare with pulmonary angiography and pulmonary vein oximetry. This was a prospective study done between 2017 and 2018. Twenty-five children who underwent BDG and planned for cardiac catheterization prior to Fontan completion were included in the study. All patients underwent pulmonary angiography, oximetry, and saline contrast TEE at the time of cardiac catheterization. Twenty-two patients had undergone unilateral BDG surgery and three were palliated by bilateral BDG. The mean oxygen saturation was 80 ± 5.2%. Thirteen patients (52%) had preserved antegrade pulmonary blood flow. Eighteen patients (72%) had PAVF by angiography and oximetry, while 19 (76%) had PAVF identified by contrast echocardiography. There was moderate correlation between the degree of pulmonary venous desaturation and grading of PAVF by contrast echocardiography. PAVF was predominantly located in the lower zones of the lungs. Higher grades of PAVF were not seen in patients with preserved antegrade flow after BDG. Angiographically detected PAVF showed a steady increase with increasing delay to cardiac catheterization from BDG. Significant reduction in systemic saturation was limited to advanced grades of PAVF in patients after BDG. Saline contrast TEE, pulmonary venous oximetry, and pulmonary angiography equally identified PAVF in patients after BDG. Prognostic utility of the same needs to be assessed by long-term follow-up of these subjects.

Haemodynamic consequences following closure of an Abernethy malformation in a patient following a total cavopulmonary shunt.

A 17-year-old girl with situs ambiguous, hypoplastic right ventricle with a large ventricular septal defect, and severe pulmonary stenosis had undergone Kawashima operation 10 years back. She had significant desaturation because of a large Abernethy malformation, with reverse shunting from the inferior caval vein to the portal vein. It was closed with a vascular plug, with improvement in oxygen saturation. She developed extensive inferior caval vein thrombus following the procedure, which was managed conservatively by anti-coagulation.

Imaging the elusive anomalous origin of left coronary artery from pulmonary artery.

A 7-year-old girl with severe mitral regurgitation and pulmonary artery hypertension with good ventricular function was planned for mitral valve repair, but was subsequently diagnosed to have anomalous left coronary artery from pulmonary artery. We describe the pitfalls in the diagnosis of this rare anomaly, particularly in the presence of severe pulmonary artery hypertension and absence of circumstantial evidence of coronary steal and ventricular dysfunction.

Surgery for Congenital Tricuspid Valve Cleft: Tricuspid Valve Repair with Neochordae and Annuloplasty.

BACKGROUND AND AIM OF THE STUDY: Congenital abnormalities of the tricuspid valve (TV), including dysplasia, straddling, and those associated with other congenital heart disease, are rare causes of tricuspid regurgitation (TR). In congenital TV anomalies there can be varying levels of abnormalities of leaflet and subvalvular structures. Herein is reported a case of TV cleft with absent chordae, and a technique of TV repair. METHODS: A 14-year-old boy was found to have severe TR due to dysplasia of the anterior TV leaflet. Intraoperatively he was noted to have dysplasia of the TV with a cleft in the anterior leaflet of the TV and an absence of chordae supporting the anterior two-thirds of the anterior leaflet. The anterior papillary muscle was hypoplastic, with chordae to the posterior leaflet and small chordae partly to the anterior leaflet. The cleft was repaired and a neochordae placed onto the anterior leaflet with attachment to the papillary muscle, followed by an annuloplasty. RESULTS: Intraoperative and postoperative echocardiographic assessment showed good mobility of the anterior tricuspid leaflet at six months and two-year follow up. CONCLUSION: Chordal replacement is a useful technique for repairing congenital dysplastic TV with absent chordae. The same technique for mitral valve repair with neochordae can be applied to chordal anomalies of the TV, with excellent outcome.

Aorto-Left Ventricular Tunnel-An Uncommon Entity.

The aorto-left ventricular tunnel is an extracardiac communication that has a specific morphological feature. It is important to differentiate this entity from other diagnoses because the treatment options differ significantly and better outcomes are obtained with this entity.

Cor triatiratum dexter: a rare cause of isolated right atrial enlargement.

Cor triatriatum sinistrum (division of the left atrium) is a recognized clinical and surgical entity. Division of the right atrium, also known as cor triatriatum dexter, is an extremely rare congenital abnormality in which persistence of the right valve of the embryonic systemic sinus venosus divides the right atrium into two chambers. Typically, the right atrial partition is due to exaggerated fetal eustachian and thebesian valves, which together form an incomplete septum across the lower part of the atrium. This septum may range from a reticulum to a substantial sheet of tissue. Cor triatriatum dexter can be diagnosed at any age, especially if it is incidentally discovered. Usually, this anomaly is recorded at necropsy. This report describes the case of a divided right atrium evaluated for nonspecific symptoms and unexplained cardiomegaly with right atrial enlargement.

Acute ST-elevation myocardial infarction with Guillain-Barre syndrome in Fontan circulation.

Total cavopulmonary connection, commonly referred to as the Fontan procedure, is the established destination therapy for univentricular hearts. While the procedure permits a longer survival of these patients, this circulation involves several compromises from normal human circulation and poses several challenges with increasing age after surgery. We present an instance of acute ST-elevation myocardial infarction with Guillain-Barre syndrome in an adult after Fontan palliation and discuss the challenges in management.

Tandem Saphenous Vein Graft Aneurysms with Right Atrial Fistula: Evaluation of a "Rarest of a Rare Complication" Using CT Coronary Angiography.

Saphenous vein graft (SVG) aneurysm after coronary artery bypass grafting (CABG) is a rare complication. A fistula between an SVG aneurysm and a cardiac chamber is even rarer. Herein, we report a middle-aged man who underwent CABG with five grafts 13 years prior presenting with multiple aneurysms in the venous graft with a fistula between the aneurysm and the right atrium. The computed tomographic angiogram findings and the subsequent treatment of the patient are addressed in the report.

A Giant Coronary Aneurysm in a Suspected Kawasaki Disease Causing Asymptomatic Myocardial Ischemia.

Coronary involvement in Kawasaki disease is not uncommon; however, giant coronary aneurysm exceeding 50 mm is extremely rare. In this article, we presented a case of giant coronary aneurysm involving right coronary artery with associated asymptomatic myocardial ischemia as evident by multimodality imaging.

Utility of 3D Echocardiography for Device Sizing During Transcatheter ASD Closure: A Comparative Study.

BACKGROUND: Two-dimensional (2D) transesophageal echocardiography (TEE) is commonly used for assessing patients undergoing transcatheter atrial septal defect (ASD) device closure. 3D TEE, albeit providing high resolution en-face images of ASD, is used in only a fraction of cases. We aimed to perform a comparative analysis between 3D and 2D TEE assessment for ASD device planning. METHODS: This was a prospective, observational study conducted over a period of one year. Patients deemed suitable for device closure underwent 2D and 3D TEE at baseline. Defect characteristics, assessed separately in both modalities, were compared. Using regression analysis, we aimed to derive an equation for predicting device size using 3D TEE parameters. RESULTS: Thirty patients were included in the study, majority being females (83%). The mean age of the study population was 40.5 ± 12.05 years. Chest pain, dyspnea and palpitations were the common presenting complaints. All patients had suitable rims on 2D TEE. A good agreement was noted between 2D and 3D TEE for measured ASD diameters. 3D TEE showed that majority of defects were circular in shape (60%). The final device size used had high degree of correlation with 3D defect area and circumference. An equation was devised to predict device size using 3D defect area and circumference. The mean device size obtained from the equation was similar to the actual device size used in the study population (p = 0.31). CONCLUSIONS: Device sizing based on 3D TEE parameters alone is equally effective for transcatheter ASD closure as compared to 2D TEE.

Culprit Lesion Morphology on Optical Coherence Tomography in ST-elevation Myocardial Infarction vs Non ST-elevation Myocardial Infarction - A Systematic Review of 7526 Patients.

Background: Patients with STEMI are postulated to have different culprit lesion morphology compared to NSTEMI. The use of OCT in ACS can help delineate lesion morphology. The aim of this systematic review was to analyze the available data on culprit plaque morphology in ACS patients. Methods: The available literature was systematically screened for studies on culprit lesion morphology in ACS patients. Data was extracted from the selected studies and analyzed for baseline characteristics as well as culprit lesion morphology on OCT. Lesion characteristics between STEMI and NSTEMI groups were compared. Results: A total of 32 studies were selected for the final analysis. The average age of the study population was 62.4 years. Majority of patients (66.6%) had STEMI on presentation. NSTEMI patients had a higher prevalence of diabetes compared to STEMI. Both STEMI and NSTEMI patients had similar prevalence of thin-cap fibroatheroma (44.9%). The mean fibrous cap thickness was 84.2 µm in the study. STEMI patients had higher prevalence of lipid plaques, macrophages and luminal thrombus as compared to NSTEMI patients. Plaque rupture was the predominant culprit lesion morphology in both STEMI and NSTEMI groups, with higher prevalence in STEMI patients. Plaque erosion was also more common in STEMI patients (34.4% vs 13.2%). Conclusion: Plaque rupture is the predominat culprit lesion morphology in both STEMI and NSTEMI patients, despite having differences in baseline characteristics. Use of OCT to determine plaque morphology in ACS patients can help guide management strategy in select cases. [PROSPERO CRD42021249742].

Natural history of coronary stents: 14 year follow-up of drug eluting stents versus bare metal stents.

BACKGROUND: Several randomized trials have shown the effectiveness of drug-eluting stents (DES) over bare metal stents (BMS) in terms of repeat revascularization at 1 year; however long term data in this context is conflicting. AIM: To assess the long term clinical outcomes after coronary artery stenting with drug-eluting stents and bare metal stents. METHODS: This is a retrospective cohort study, including 100 consecutive patients with Coronary Artery Disease who underwent successful percutaneous intervention (PCI) with implantation of DES and contemporary 100 patients who underwent PCI with implantation of BMS in the years 2005 and 2006 at our center. RESULTS: Over a median follow-up of 14 years, the primary composite outcome of major adverse cardiovascular and cerebrovascular events (MACCE) was found to be similar in both the groups [DES-37; BMS-36 (p value = 0.88)]. At 1 year of follow-up, the incidence of MACCE was significantly lower with DES group than BMS group [DES-3; BMS-10, P value = 0.04]; but the benefit was not seen at 5 years, 10 years and 14 years follow-up. The incidence of very late stent thrombosis in our study population was similar in either of the groups (p value = 0.13). Obesity and creatinine of >1.4 mg/dl were found to be the predictors of all-cause death. CONCLUSION: In patients with coronary artery disease, the composite endpoint of MACCE for the first year after stenting was significantly lower in patients receiving DES than those receiving BMS; however, at very long term follow-up, the event rates were similar.

Clinical profile and outcomes of childhood dilated cardiomyopathy - A single-center three-decade experience.

Introduction and Aims: Dilated cardiomyopathy (DCM) is an important cause of heart failure (HF) among children. Research on pediatric DCM remains surprisingly scarce. The primary objective of the study was to evaluate the clinical profile and outcomes of pediatric DCM and the secondary objective was to study the predictors of outcome. Methods and Results: We enrolled all patients with cardiomyopathy who presented to us between 1990 and 2020 and were younger than 18 years. During the 30-year study period, we identified 233 cases of pediatric cardiomyopathy. One hundred and nineteen (51%) cases had DCM. This retrospective cohort was analyzed to study their outcome and the possible predictors of outcome. Nearly, 8% presented in the neonatal period, and 37% in infancy. The most common mode of presentation was dyspnea on exertion (71%). Ninety-three patients presented in heart failure (78%). The median left ventricular dimension z-score in diastole was 4.3 (range 2.5-9.06). The median left ventricle (LV) ejection fraction was 31%. Seventy-two percent of this cohort were on angiotensin-converting-enzyme inhibitors, 40% on aldosterone antagonists, and 47% on beta-blockers. One-third had syndromic, metabolic, genetic, or any secondary cause identified. Twenty-seven patients satisfied the three-tiered clinical classification for the diagnosis of probable acute myocarditis. Over a mean follow-up of 3.29 years, 27% were lost to follow-up. Among the remaining patients who were on follow-up (n = 86), 39 (45%) died, 31 (36%) recovered, and 16 (18%) had persistent LV dysfunction. Heart Failure was the most common cause of death. Eight patients in this cohort (4.2%) had thromboembolic phenomena. Nine had sustained ventricular arrhythmias and six had atrial/junctional arrhythmias. Among the various risk factors studied, only infantile onset had a significant relationship with death or ventricular arrhythmias (P value- 0.05). The 5-year survival rate of DCM patients was 59%. Conclusion: A reasonably good percentage of our population showed recovery of the left ventricular function (36%). Only infantile onset had a significant relationship with death or ventricular arrhythmias. The outcome in our DCM cohort is similar to other population cohorts.

Comparative study on safety, efficacy, and midterm results of balloon mitral valvotomy performed with triple lumen and double lumen mitral valvotomy catheters.

BACKGROUND: The triple lumen Inoue balloon is routinely used for balloon mitral valvotomy (BMV) in India. Its major limitation is the high cost. The double lumen Accura balloon is less expensive, making it an attractive alternative in the developing countries. The study was meant to assess the safety, efficacy and midterm results of Accura balloon with respect to the Inoue balloon. PATIENTS AND METHODS: 816 consecutive patients, who underwent elective BMV in this Institute from 1997 to 2003, were included in the study. The data of 487 patients who underwent BMV with Accura balloon was compared with 329 patients who underwent BMV with Inoue balloon. The clinical, echocardiographic, and hemodynamic data of these patients were analyzed retrospectively to assess the safety and efficacy of Accura balloon with respect to the Inoue balloon. RESULTS: Immediate procedural success (93.9% in Inoue group and 91.6% in Accura group p. NS) and complications (6.6% in Inoue group and 5.6% in Accura group p. NS) were comparable between the study groups. The two study population had similar restenosis rate and events at 1 year after BMV. Both balloons could be reused multiple times without compromising on the safety and effectiveness. Accura balloons were less costly than Inoue balloon. The reusability with Accura was slightly more and found to be more cost-effective. CONCLUSIONS: Both Accura and Inoue balloon mitral valvotomy balloons are effective in providing relief from hemodynamically significant mitral stenosis in terms of gain in valve area and reduction in trans mitral gradient. Both groups have similar procedural success and complication rates, restenosis, and follow-up events at 1 year. Both balloons could be reused multiple times and Accura balloon is found to be more cost effective.

Technical considerations of coarctation stenting in double-barreled aorta - A persistent fifth arch mimic.

The double-barreled aorta connecting the ascending aorta and descending aorta caudal to the normal fourth aortic arch has fascinated the interests of cardiac morphologists for over a century. This condition is commonly associated with coarctation. While the controversies surrounding the embryology of the double-barreled aorta have settled down, we present a case-based illustration of the technical aspects of coarctation stenting peculiar to this condition.

Outcomes of Venus P-valve for dysfunctional right ventricular outflow tracts from Indian Venus P-valve database.

BACKGROUND: Balloon-expandable pulmonary valves are usually not suitable for dilated native outflow tracts. METHODS: Indian Venus P-valve registry was retrospectively analyzed for efficacy, complications, and midterm outcomes. Straight valve was used in prestented conduits in patients with right ventricular pressure above two-thirds systemic pressure and/or right ventricular dysfunction. Flared valve 1-4 mm larger than balloon waist was used in native outflow in symptomatic patients, large ventricular volumes, and ventricular dysfunction. OBJECTIVES: A self-expanding porcine pericardial Venus P-valve is available in straight and flared designs.. RESULTS: Twenty-nine patients were included. Straight valve was successful in all seven conduits, reducing gradients significantly, including one patient with left pulmonary artery (LPA) stent. Flared valve was successfully implanted in 20 out of 22 native outflow tracts. Sharp edges of the older design contributed to two failures. Complications included two migrations with one needing surgery, endocarditis in one, insignificant wire-frame fractures in three, and groin vascular complication in one patient. There were no deaths or valve-related reinterventions at a mean follow-up of 47.8 ± 24.5 months (1-85 months). Modifications of technique succeeded in three patients with narrow LPA. There was significant improvement in symptoms, right ventricular volume, and pulmonary regurgitant fraction. CONCLUSION: Straight and flared Venus P-valves are safe and effective in appropriate outflow tracts. Straight valve is an alternative to balloon-expandable valves in stenosed conduits. Flared valve is suitable for large outflows up to 34 mm, including patients with LPA stenosis. Recent design modifications may correct previous technical failures. Studies should focus on durability and late complications.