Left ventricular apical aneurysm in Takayasu arteritis and chronic active Epstein-Barr virus infection.

BACKGROUND: Takayasu arteritis (TA) is a chronic inflammatory disease of unknown etiology characterized by a large vessel vasculitis involving the aorta and its branches. Myocardial involvement is extremely unusual in TA and is mostly in the form of myocarditis, ventricular hypertrophy, and ventricular dysfunction secondary to coronary ischemia. Submitral aneurysms have been reported in TA and has been attributed to the chronic inflammatory process in TA. CASE PRESENTATION: We report a novel instance of left ventricular apical aneurysm in a 37-year-old lady with TA and normal epicardial coronaries. She was diagnosed with a left ventricular apical aneurysm, moderate aortic regurgitation, and moderate pericardial effusion. The coronary arteries were normal. The patient had concomitant chronic active Epstein-Barr virus infection complicating patient outcome. CONCLUSIONS: Left ventricular apical aneurysm with normal epicardial coronaries is a rare cause of heart failure in Takayasu arteritis. Concomitant chronic active Epstein-Barr virus infection can potentially accentuate the inflammatory process in Takayasu arteritis and complicate management and patient outcomes.

Markedly dilated right atrium in an infant: what is the diagnosis?

An 18-month-old boy presented with recurrent episodes of irritability and documented fast heart rate suggestive of supraventricular tachycardia. Cardiovascular examination revealed significant cardiomegaly, normal heart sounds and no murmurs. The differential diagnosis of marked right atrial dilatation and management principles of idiopathic dilatation of the right atrium are described.

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.

All that flows across the ventricular septum are not ventricular septal defects.

Coronary cameral fistula is a rare congenital anomaly and clinical presentation depends on the location of the defect, degree of shunting, and associated complications. We present a case of coronary cameral fistula where segmental analysis by echocardiogram helped us to avoid misdiagnosis as a ventricular septal defect.

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.

Pancycle aortic incompetence and double alternans in pediatric heart failure.

A 12-year-old boy presented with bicuspid aortic valve, severe aortic regurgitation, and dilated dysfunctional left ventricle in heart failure. He underwent aortic valve replacement with a 23 mm TTK Chitra heart valve prosthesis (tilting disk). He was gradually weaned off milrinone and noradrenaline in the intensive care. Echocardiography showed severe left ventricular dysfunction with an ejection fraction of 24%. The radial pulse was regular and of normal volume but exactly half that of the heart rate. Evaluation of the rhythm and echocardiography revealed an interesting hemodynamic phenomenon with double alternans.

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.

Paediatric quality of life in toddlers and children who underwent arterial switch operation beyond early neonatal period.

OBJECTIVES: The aim of this study was to evaluate the quality of life (QOL) of children who underwent the arterial switch operation (ASO) for Transposition of Great Arteries in our population and, specifically, to explore early modifiable factors and the influence of parental and socioeconomic factors on the QOL of these children. METHODS: Cross-sectional study using Paediatric Quality of Life Inventory™ 3.0 Cardiac Module was carried out on 3- to 12-year-old children who had undergone ASO between the years 2012-2018. Socioeconomic status was calculated using the modified Kuppuswamy scale (2019). Other clinical factors with possible bearing on the outcome were also analysed. RESULTS: Immediate survival after surgery was 196 out of 208 (94.2%) with an attrition of 19 patients (9.6%) over the follow-up period. Most surviving children (98.9%) had started formal schooling in age-appropriate classes. Two children had severe neuromotor impairment. The median cumulative health-related QOL score of the children was 97.9 (interquartile range 4.2) at 5.6 ± 1.27 years of life. The median scores each of the health-related QOL parameters, viz, heart problem symptoms, treatment compliance, perceived physical appearance, treatment-related anxiety, cognitive problems, and communication was 100 with negative skewing. CONCLUSIONS: Excellent QOL was observed in most children after ASO with the median total paediatric QOL scores in all domains of 97.9. Social factors did not show a statistically significant influence on the QOL parameters in the current cohort. The gradually declining trend across the age groups emphasizes the need for continued follow-up for early identification of possible correctable factors and initiating intervention to ensure good QOL into teenage and adulthood.

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.

Safety of drug-eluting stents for stenting patent arterial duct in neonates.

OBJECTIVES: The primary objective was to evaluate the trend of blood sirolimus concentrations in neonates following ductal stenting. The long-term outcomes and incidence of infections were also evaluated. METHODS: Prospective open-label observational study in a tertiary referral centre over a 1-year period. Serum sirolimus levels were estimated at 1 hour and 24hrs post-stent insertion followed by 7 days in neonates who underwent ductal stenting. The trend in sirolimus levels, incidence of infections, complications and outcomes following ductal stenting were studied. RESULTS: Seven neonates with duct-dependent pulmonary circulation underwent ductal stenting at median age of 8.5 days and weight of 2.83kg. The average stent size was 3.5±0.4 mm, and average stent length was 16.3±5.1 mm. The mean sirolimus concentrations at 1 hour, 24 hours and 7 days were 41.3±6.9ng/ml, 15.4±7.1ng/ml and 3.1±0.85ng/ml respectively. Levels fell below therapeutic range for all patients by 7 days. Three patients had sepsis or necrotising enterocolitis, but responded well to antibiotics; 1 patient had aspiration related sudden death. There were no further events at a mean follow-up of 207 days, and 4 patients underwent elective surgery at 238 ± 81 days after ductal stenting. CONCLUSIONS: This study demonstrates applicability of drug-eluting stents for ductal stenting in newborns. Drug-eluting stents with abluminal drug delivery are associated with high sirolimus levels in initial hours but rapidly taper to negligible levels within a week of implantation. Neonates with high pre-procedure likelihood of infection developed sepsis but responded well to conservative management. The patency of drug-eluting ductal stents is preserved over long-term follow-up.

Influence of age at surgery on left ventricular strain in patients with anomalous origin of left coronary artery from pulmonary artery.

OBJECTIVES: Myocardial strain abnormalities are described after surgical repair of anomalous left coronary artery from pulmonary artery (ALCAPA) even after recovery of ventricular function. The factors that predispose to the presence of these strain abnormalities in the presence of normal ventricular function are unknown. The aim of this study was to find out whether the age at repair influences the presence of global and regional strain abnormalities on follow-up. METHODS: Repaired ALCAPA patients from a single centre (n = 40) with good ventricular ejection fraction on follow-up were recruited. Baseline and follow-up data were collected from electronic records. Global and regional myocardial strain assessment was done by speckle tracking echocardiography prospectively. The association between age at repair and strain abnormalities on follow-up was analysed. RESULTS: The patients who presented earlier had significantly worse ventricular function pre-operatively compared to older patients (P < 0.0005). Global longitudinal strain was abnormal in 40% of patients with normal ventricular ejection fraction on follow-up. Presence of longitudinal strain abnormalities was more in patients who underwent repair at older age than in those who were repaired earlier (P < 0.0005). The probability of having normal longitudinal strain on follow-up was 81.6% if surgery was done before 7.8 months of age. If operated before 6 months, the odds of having normal myocardial strain was 11 times higher. Regional strain abnormalities of varying severity were present in all patients in the left and in some patients in the right coronary artery territories. CONCLUSIONS: Older age at ALCAPA repair is associated with increased incidence of myocardial strain abnormalities. Regional strain abnormalities were found in both left and right coronary artery territories.

Outcomes of repair of anomalous origin of pulmonary artery branch from aorta ascendens with autologous tissue: a rare condition revisited.

BACKGROUND: Anomalous origin of pulmonary artery branch from ascending aorta (APA) in the presence of two separate semilunar valves is an uncommon entity necessitating early diagnosis and surgery to prevent development of irreversible pulmonary vascular disease. We evaluated our experience with the technique and outcome of 11 patients with this condition. METHODS: Between January 2000 and December 2019, 11 patients were diagnosed with APA. Echocardiographic data were collected from the records, including the site of origin of the anomalous pulmonary artery, additional defects, pulmonary artery pressures, and biventricular function. Intraoperative charts were reviewed for the details of the surgical procedure and cardiopulmonary bypass. Post-operative data included survival, ventilatory support, and duration of hospital stay. After discharge, children were reviewed at 1 month, 3 months, 6 months, and then at yearly intervals. RESULTS: Of the 11 patients, females were more than males (7:4) with a median age of 6 months (15 days-28 years) and median weight of 5.7 kg (1.8-40 kg). Nine patients underwent direct re-implantation of anomalous pulmonary artery branch to main pulmonary artery. The survival rate was 88.8% in our series. On follow-up, no re-operations or re-interventions were required and all surgically corrected patients were in stable clinical condition. CONCLUSION: Early- and midterm outcomes of children who underwent surgery for APA is convincing. Early direct re-implantation of the anomalous branch pulmonary artery to main pulmonary artery without any graft material is the optimal surgical strategy for these patients.

Chylotamponade in Pediatric Primary Mediastinal Large B-Cell Lymphoma.

Chylotamponade involves rapid accumulation of chyle in the pericardium elevating the pericardial pressures above normal right heart filling pressures, and is extremely rare. A 12-y-old boy presented to the emergency with complaints of facial puffiness for 1 mo and breathing difficulty for 1 wk. The neck veins were distended, and the heart sounds were muffled. A chest CT demonstrated a large anterior mediastinal mass with pleural and pericardial effusions. Echocardiography confirmed cardiac tamponade. Pericardiocentesis revealed chylopericardium. He was placed on a chyle leak diet, and the drain was removed after 48 h. Biopsy of the mediastinal mass revealed a primary mediastinal B-cell lymphoma. He was successfully managed with chemotherapy. The index case demonstrates how prompt identification and management of chylotamponade and treatment of the underlying cause can lead to good clinical outcomes.