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.

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.

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.

Left cardiac sympathetic denervation in children with Jervell Lange-Nielsen syndrome and drug refractory torsades - A case series.

INTRODUCTION: Long QT syndrome is an inherited malignant channelopathy which leads to life-threatening arrhythmia, with multiple genotypes. Jervell and Lange-Nielsen syndrome (JLNS) is an autosomal recessive subtype of this disease, characterized by congenital sensorineural deafness and a high incidence of sudden cardiac death (SCD). METHODOLOGY: We prospectively followed up six children who underwent left cardiac sympathetic denervation (LCSD) for JLNS in view of high-risk features despite being on maximally tolerated doses of oral propranolol. RESULTS: Mean age at diagnosis was 2.75 ± 0.39 years, with a significant delay between onset of symptoms and diagnosis (mean 7.2 ± 3.5 months). All had sensorineural hearing loss, conforming to the JLNS phenotype. Mean QTc interval was 603 ± 93 ms, with T wave alternans (TWA) seen in all cases. All were started on propranolol and subsequently subjected to LCSD, and 3 underwent AAI permanent pacemaker implantation. Over a mean follow-up of 20 months, there was a significant reduction in QTc (603 ± 93 ms to 501 ± 33 ms, p = .04), which was persistent on follow-up (525 ± 41 ms) and only two out of six had persistent T wave alternans on ECG (p < .01). None of these children had presyncope, syncope, seizures, torsades de pointes, cardiac arrest or death on follow up following LCSD. CONCLUSION: Jervell Lange-Nielsen syndrome is a subtype of LQTS with high-risk features. LCSD, an effective therapeutic option for those having symptoms despite being on propranolol, results in significant reduction of QTc interval and amelioration of symptoms.

Interesting phenomenon during positioning of the 3830 lead deep inside the septum by rapid rotations. What is the mechanism?

A 66-year-old lady having complete AV block with narrow QRS escape had undergone left bundle branch pacing (LBB) with 4.1-F diameter 3830 Select SecureTM pacing lead (Medtronic Inc., Minneapolis, MN, USA). The pacing lead was placed with the inter-ventricular septum, 2 cm inferior to the distal His region towards the right ventricular apex, by 5 rapid turns. During the rapid turns, an interesting phenomenon was noted [Fig. 1A & B]. What is the mechanism?

Study of atrial arrhythmias after surgical or device closure of atrial septal defect.

Background Device closure of atrial septal defect (ASD) has emerged as a treatment modality for the past 3 decades and has changed the natural history of ASD compared to that of surgical closure. Early intervention in ASD retards the geometrical and electrical remodelling of the atrium that contributes to the development of atrial tachyarrhythmias. We studied the incidence of atrial arrhythmias in patients undergoing surgical and device closure of ASD. Methods We did this retrospective observational study at a tertiary referral centre, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala. Patients of all age groups undergoing surgical and device closure of ASD between 1 January 2003 and 31 December 2008 were included to compare the incidence and nature of atrial arrhythmias and also analyse the difference in new-onset atrial arrhythmias between the two arms. Results Of 277 patients, 144 with surgical closure and 133 with device closure were followed up for 10-15 years. A larger number of men underwent surgical closure (41.7%) compared to device closure (25.6%). The mean (SD) follow-up was 12.6 (3.7) years in the surgical closure group and 10.9 (2.6) years in the device closure arm. There were a larger number of patients with atrial tachyarrhythmias in the surgical closure group (6.3%) compared to the device closure group (0.8%) at baseline (p=0.02). A significantly larger number of patients had atrial fibrillation in the surgical closure group (5.6%) compared to the device closure group (0.7%) at baseline (p=0.003). Pulmonary hypertension at baseline was present in 38.9% of patients in the surgical closure group and in 23.3% of patients in the device closure group (p=0.006). New-onset atrial arrhythmias occurred in patients ≥30 years of age (p=0.006) and exclusively in patients with pulmonary hypertension in the surgical group (3.7%) and in the device closure group (6.6%). This was statistically significant in the device closure group (p=0.05) but not in the surgical closure group (p=0.13). The incidence of new-onset arrhythmias was not statistically significant in both groups. Conclusions Atrial arrhythmias were significantly more common in patients who underwent surgical or device closure at ≥ 30 years of age and in patients with pulmonary hypertension. There was no difference in new-onset atrial arrhythmias between the surgical and device closure groups. Our study results suggest that surgical or device closure before 30 years of age and before the development of atrial arrhythmias may be beneficial with respect to the development of atrial arrhythmias.

Postoperative Aortic Regurgitation in Outflow Ventricular Septal Defect: Determinants of Outcome.

Aortic cusp prolapse is an acquired complication and usually precedes the development of aortic regurgitation (AR) in unoperated outflow ventricular septal defect (VSD). However, its impact on postoperative AR-progression is unknown. 161 patients with outflow-VSD and AR who underwent surgery between 2006 and 2012 were studied retrospectively. 31 patients without prolapse (group-I), 87 with only right coronary cusp (RCC) (group-II), 43 with noncoronary cusp (NCC) prolapse (group-III: 23 only NCC (IIIa), 20 both NCC-RCC (IIIb)) were followed postoperatively for a mean 6.05 ± 2.4 years (range 3-12 years). Moderate or severe-AR was present in 4.2%, 36.8%, 52.2% and 80% preoperatively; in 3.2%, 10.3%, 39.1% and 30% patients at follow-up in group-I, II, IIIa, and IIIb, respectively. Although freedom from significant-AR (moderate or severe AR) or aortic valve replacement (AVR) at 10 years was lesser in subaortic-VSD than subpulmonic-VSD (64.3 ± 7.5% vs 87.9 ± 3.6%; P = 0.02), the difference was not significant when compared within prolapse groups (80 ± 8% vs 88.7 ± 4.0%, P = 0.28 in group-II; 40.7 ± 11.8 vs 70 ± 14.5%, P = 0.48 in group-III). The significant-AR or AVR free survival in patients with trivial or mild preoperative-AR was not significantly different between prolapse groups (98.2 ± 1.8% vs 75 ± 21.7% in group-II and III respectively; P = 0.85). However, in those with moderate or severe preoperative-AR it was significantly lesser in group-III than II (30.1 ± 9.8% vs 65.6 ± 8.4%, respectively; P = 0.04). Group-III, compare to group-II, had 3.28 and 5.24-time risk of development of significant-AR or requirement of AVR, respectively. Prolapse of NCC alone or in addition to RCC prolapse has unfavourable impact on the postoperative outcomes, especially in subaortic-VSD after development of more than mild AR preoperatively.

Outflow Ventricular Septal Defect With Aortic Regurgitation: Optimal Timing of Surgery?

BACKGROUND: Ideal time of surgery still remains controversial in outflow ventricular septal defect (VSD) with aortic regurgitation (AR). We aimed to identify the prevalence and predictors of postoperative AR progression. METHODS: A total of 154 patients with outflow VSD and AR who underwent VSD surgery between 2006 and 2012 were studied retrospectively. RESULTS: Eighty patients with subpulmonic VSD and 74 with subaortic VSD were followed up for mean 6.32 ± 2.27 years (range, 3-12 years). Of these, 100 had trivial to mild (group A) and 54 had moderate to severe preoperative AR (group B). At follow-up, there was no significant worsening of mean residual AR grade in group A (P = .16) and subpulmonic VSD of group B (P = .083). However, AR grade worsened significantly in subaortic VSD (1.85 ± 0.87 vs 2.21 ± 1.08, P = .005) of group B. Only 2 (both had subaortic VSD) patients of group A developed moderate AR and none required aortic valve replacement (AVR), while 23 (42.60%) of group B patients developed moderate or severe AR and 7 (30.4%) of them required AVR. Moreover, all who needed AVR had subaortic VSD and had undergone valvuloplasty during VSD closure. The 10 years freedom from moderate or severe AR was significantly lower in group B than group A in both VSDs (subaortic VSD 42.5% ± 10.7% vs 89.3% ± 8.1%, P < .01; subpulmonic VSD 66.7% ± 10.3% vs 100%, P< .01). On multiple regression analysis, postoperative residual AR was the only predictor of AR progression (standardized coefficient, 0.48; P < .001) at follow-up. CONCLUSIONS: Mild preoperative AR rarely progressed after VSD repair. However, worsening of AR could not be prevented effectively, even with valvuloplasty, after the development of moderate or severe AR. Mild or more postoperative residual AR requires close follow-up, especially in subaortic VSD.

"All that glitters is not gold".

A 63-year-old gentleman presented to the emergency room (ER) with complaints of sudden onset of palpitation for the last 1 hour. He denied any presyncope or syncope. He was tachycardic with pulse rate of 120 beats per minute. His blood pressure was 110/70 mm Hg. A 12-lead electrocardiogram (ECG) was taken. What is the likely diagnosis?

Management of a complicated 'pacemaker pocket' site infection.

Pocket site infection after implantation of a pacemaker is a dreaded complication which requires removing the device and reimplanting it at a contralateral site. Difficulties arise when the patient is dependent on pacing and when there are issues with venous access at the contralateral site. We report a patient with pacemaker pocket site infection with congenital complete heart block managed with explantation of the device, semi-permanent pacing during antibiotic treatment, reimplantation of the device at the contralateral site and management of subtotal subclavian vein stenosis noted during reimplantation.

Narrow QRS Tachycardia: What Is the Mechanism?

A 45-year-old man was referred for radiofrequency catheter ablation of narrow QRS tachycardia that terminated with intravenous adenosine. A 12-lead electrocardiogram showed no baseline pre-excitation. The echocardiogram was essentially normal. The electrophysiological study showed a normal atrial-His interval of 110 ms and a His-ventricular interval of 44 ms during sinus rhythm. An anterograde study demonstrated no dual atrioventricular nodal physiology. Atrial pacing protocols easily and reproducibly induced a narrow QRS tachycardia. What is the mechanism of the tachycardia?

Cardiac magnetic resonance imaging in right ventricular outflow tract arrhythmia: a retrospective analysis from a tertiary care centre in South India.

INTRODUCTION: Arrhythmia arising from right ventricular outflow tract (RVOT) is the most common cause of idiopathic ventricular arrhythmia. Previous studies involving cardiac magnetic resonance imaging (MRI) in these patients are known to show abnormalities in up to 60% of patients. METHODOLOGY: This was a retrospective descriptive analysis of patients having idiopathic RVOT arrhythmia who underwent cardiac MRI between January 2010 and December 2020. Clinical and demographic details were recorded from the electronic medical records and cardiac MRI of all patients were reviewed. RESULTS: Among 214 patients with RVOT arrhythmia, 64 underwent cardiac MRI. A total of 41 patients, who did not have any abnormality on baseline echocardiogram were included in the study. There was male preponderance (56.1%), with median age of 43 years. About 43.9% had syncope. Twenty-four-hour Holter study revealed a premature ventricular complex (PVC) burden of 26.3 ± 11.7%. MRI showed structural abnormalities in 51.2% of patients, which commonly included RV sacculations and aneurysms. Late gadolinium enhancement (LGE) was seen in six patients, which was mostly seen in RV free wall. Right ventricular (RV) systolic dysfunction was evident in 29.3%. About 9.8% fulfilled the criteria for arrhythmogenic RV cardiomyopathy (ARVC). Out of 23 patients with baseline abnormalities on ECHO, 87% had structural abnormalities on MRI, and 43.5% fulfilled the criteria for ARVC. CONCLUSIONS: Additional imaging by cardiac MRI helps to identify structural abnormalities in 51.2% of patients with RVOT arrhythmias, even with normal baseline echocardiogram and electrocardiogram (ECG). It is useful tool to rule out ARVC in this subset of patients, and can help in increasing the diagnostic yield in the early stages.

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.

Interesting Response with the Delivery of Right Ventricular Extrastimulus of Increasing Prematurity.

A 45-year-old man with no structural heart disease underwent an electrophysiology study for recurrent episodes of palpitation. There was no evidence of pre-excitation on the baseline electrocardiogram. Baseline intervals, including the A-H and H-V intervals, were within the normal limits and remained so during the electrophysiology study. An interesting response was observed with the delivery of ventricular extrastimuli with increasing prematurity from the right ventricular apex, triggering consideration of the possible mechanism.

Tricuspid annular PVCS: Radiofrequency ablation by subtricuspid retrograde catheter approach.

Tricuspid annular PVCs constitute 8% of idiopathic PVCs and 5% of RV PVCs. Although a rare entity to encounter in routine clinical practice, it can be a prime culprit behind major arrhythmic burden in few individuals. Electroanatomic approach with sub tricuspid retrograde catheter technique can target those annular PVCs and decrease the arrhythmia burden to normal or near normal range. Although procedural approach for parahisian PVCs requires a close look to avoid injury to native conduction system, TA PVCs are a real challenge due to inherent catheter instability and contact issue in sub tricuspid retrograde approach.