Long-term, real world experience of ventricular tachycardia and granulomatous cardiomyopathy.

BACKGROUND: Granulomatous cardiomyopathy (GCM) is relatively uncommon in patients presenting with ventricular tachycardia (VT). Sarcoidosis and tuberculosis are the most common causes of GCM with VT. The aim of study was to evaluate their clinical characteristics and the long-term outcomes. METHODS: We retrospectively analyzed patients from March 2004 to January 2020, presenting with VT and subsequently diagnosed to have GCM. Patients were divided into three groups (sarcoid, tuberculosis and indeterminate) based on serologic tests, imaging and histopathology. The response to anti-arrhythmic and disease specific therapy on long-term follow-up were analyzed. RESULTS: There were 52 patients, comprising 27 males and 25 females, age 40 ± 10 years. The follow-up period was 5.9 ± 3.9 years. Sarcoidosis was diagnosed in 20 (38%); tuberculosis (TB) in 15(29%) and 17(33%) patients were indeterminate. Left ventricular ejection fraction (LVEF) of the entire cohort was 0.45 ± 0.14. Erythrocyte Sedimentation Rate(ESR) was found to be significantly higher in TB(43.6 ± 18.4) patients vs sarcoid(18.9 ± 6.7)p < 0.0001, but not the indeterminate group (36.2 ± 21.1), p = 0.3. Implantable Cardioverter Defibrillator (ICD) implantation was performed in 12/20(60%) patients in the sarcoid group, in 4/15(27%) patients in the TB group and in 10/17(59%) patients in the indeterminate group. At a mean follow-up of six years, VT recurrences were noted in 6, 2, and 7 patients in the sarcoid, TB and indeterminate groups respectively. CONCLUSION: Despite the advances in diagnostic modalities for tuberculosis and sarcoidosis, in real-world practice, almost one-third of the patients with VT and GCM have uncertain etiology. Long term outcomes of patients presenting with GCM and VT with mild left ventricle dysfunction treated appropriately seems favorable.

Unilateral Breast Uptake of 99mTc-MIBI in a Lactating Woman During Myocardial Perfusion Imaging.

ABSTRACT: A 34-year-old woman, who was 11 months postpartum, underwent 99mTc-MIBI myocardial perfusion SPECT imaging for atypical symptomatology with normal baseline electrocardiogram and 2-dimensional echocardiography. She was lactating on and off, preferentially from the right breast. Analysis of the raw images revealed unilateral intense tracer uptake in the right breast region that persisted in the delayed spot views (24-hour postinjection). Although bilateral breast uptake of 99mTc-MIBI may be seen in postpartum scenario, unilateral breast uptake can also occur in patients with preferential lactation from 1 breast as seen in this case and should not be mistaken for pathology.

Arrhythmia spectrum and outcome in children with myocarditis.

INTRODUCTION: Myocarditis remains an under-diagnosed entity among children. We evaluated the spectrum of electrocardiogram (ECG) changes and arrhythmias in children with myocarditis. METHODS: A single-center prospective observational study was conducted over a period of 18 months at a public university hospital, which included all cases with myocarditis from the ages of 1 month to 12 years. Myocarditis was diagnosed according to standard criteria. Arrhythmias were detected by 12-lead ECG or by multiparameter monitors. RESULTS: There were 63 children with myocarditis. Sinus tachycardia remained the most important ECG finding (61, 96.8%) followed by ST-T changes (30, 47.6%), low voltage QRS complexes (23, 36.5%), and premature complexes (11, 17.4%). Sustained arrhythmias were seen in 14/63 (22.2%) of the children (Group A), while the remaining 49 patients were designated as Group B. There were 11 (17.5%) cases with sustained tachyarrhythmias, comprising 5 with supraventricular tachycardia, 4 with ventricular tachycardia, and 2 with atrial flutter/fibrillation. Bradyarrhythmias were seen in 3 patients, including 2 children with atrioventricular block and 1 with severe sinus bradycardia. A longer hospital stay of 18.5 (4.75) days vs. 13 (4) days, P = 0.001), and more ST-T changes [12 (85.7%) vs. 18 (36.73%), P = 0.003] were seen in Group A. Multivariate regression analysis found only the presence of ST-T changes as predictors for arrhythmia. CONCLUSIONS: A variety of arrhythmias and other ECG changes were commonly seen in children with myocarditis. Sustained arrhythmias were seen in one-fifth of the patients, being associated with ST-T changes and a longer hospital stay.

A Rare Case of Transposition of Great Arteries Uncovered With Progressive Rheumatic Aortic Stenosis.

The authors describe a case of transposition of great arteries with a large atrial septal defect with fusion of tricuspid valve leaflets and severe aortic stenosis. The latter two were likely rheumatic in etiology. The patient's condition improved after atrial switch and aortic valve replacement surgery. (Level of Difficulty: Intermediate.).

Is left atrial fibrosis an independent determinant of atrial fibrillation in mitral stenosis?

We prospectively studied whether left atrial (LA) fibrosis is a determinant of atrial fibrillation (AF) in mitral stenosis in patients who underwent balloon mitral valvotomy. There were 2 groups: Group A (n = 16), with AF and Group B (n = 27), without AF. Fibrosis was assessed by MRI. Patients underwent cardioversion before MRI. There were 27 females and 16 males, aged 29 ± 6 years. The LA areas in Groups A and B were 54.3 ± 4.4 mm2 and 39.4 ± 2.3 mm2 (p < 0.05) and the LA volume index was 46.2 ± 2.9 ml/m2 vs 33 ± 3 ml/m2 respectively (p < 0.0001). The presence of LA scarring was not statistically different in the two groups.

A case report of successful complex percutaneous coronary intervention for acute coronary syndrome in a paediatric patient with familial hypercholesterolaemia.

BACKGROUND: Familial hypercholesterolaemia (FH) is a primary genetic dyslipidaemia characterized by elevation in serum low-density lipoprotein cholesterol and its deposition in systemic arteries, which causes premature atherosclerosis. CASE SUMMARY: A 10-year-old girl presented with severe symptomatic coronary artery disease. She demonstrated characteristic morphological features of FH. Despite aggressive medical management and lipid-lowering therapy, her symptoms were not relieved and she had dynamic electrocardiogram changes. Coronary angiography showed a distal left main coronary artery lesion along with significant lesions in ostio-proximal and mid-left circumflex artery which were managed by provisional left main coronary artery to left circumflex artery stenting technique, with good immediate- and short-term results and angina relief. DISCUSSION: To the best of our knowledge, this is the first reported case of a paediatric patient with FH and acute coronary syndrome treated with percutaneous coronary intervention to left main coronary artery and left circumflex artery using provisional stenting technique. Revascularization strategies for symptomatic coronary artery disease in paediatric patients with FH have multiple unique challenges and remain an unexplored and under-reported subject.

Study of the Prevalence of Congenital Coronary Anomalies of Origin from the Opposite or Non-coronary Sinus in patients undergoing Invasive Coronary Angiography.

OBJECTIVES: 1. To study the incidence and clinical features of patients with Congenital Coronary Anomalies of Origin from Oppossite or Non-coronary Sinus; 2. To study the course of these Anomalous Coronary Artery Origins with CT Coronary Angiography. METHODS: A Cross Sectional Observation study was conducted at Department of Cardiology, in a tertiary Hospital for a period of 3 years. Patients undergoing Coronary Angiography (CAG) were screened and patients having Anomalous Coronary Origin from the Opposite or Non-Coronary Sinus of Valsalva were selected. CT Cronary Angiography was done in all such patients and the course of the anomalous artery was studied. RESULTS: During the 3 years period of study, total of 5241 CAGs were conducted of which 42 (0.8%) patients were found to have Anomalous Coronary Artery Origin as per the inclusion criteria. Out of the 42 cases, Anomalous origin of RCA from Left Corornary Cusp was the commonest with 23(0.43%) cases, followed by Anomalous Origin of LCX from Right Coronary or RCC with 17 (0.32%) cases. Other anomalies of origin were quite rare (n=2) which included Anomalous origin of Left Main from RCA (n=1) and Dual origin of LAD (n=1). CT Coronary Angiography showed that of the 23 patients with Anomalous Origin of RCA from Left side,27.4% had Inter-Arterial course and 56.1% had Retro-Aortic course. Of the 17 patients with Anomalous Origin of LCX from Right sinus, 88.23% had Retro-Aortic course. CONCLUSIONS: This study showed that anomalous origin of Coronary Artery from the opposite sinus is quite rare with RCA from Left Sinus and LCX from Right sinus comprised the vast majority of cases. Most of these anomalous Coronaries have on benign course as defined on CT Coronary Angiography. However, it is important to recognize to delineate the origin and course as it can have significant clinical, therapeutic and prognostic implications.

A Study of Prehospital Delay Patterns in Acute Myocardial Infarction in an Urban Tertiary Care Institute in Mumbai.

OBJECTIVE: There is now increasing awareness about the need for early diagnosis in patients presenting with chest pain. Pre-hospital delay remains a major hurdle in the institution of early reperfusion therapy, which is crucial in salvaging 'at-risk' myocardium and reducing adverse cardiovascular events following ST elevation myocardial infarction (STEMI). This study aims to determine the incidence and the determinants of delayed presentation STEMI and the potential impact of such delay on adverse cardiovascular outcomes. METHODS: We prospectively evaluated all patients who were admitted in the emergency department of our hospital with STEMI from March 2014 to February 2016. Data was collected sequentially at the time of admission, discharge and during follow-up. Patients were evaluated with serial ECGs, continuous ECG monitoring and echocardiography. RESULTS: Out of 1386 patients with STEMI, delayed presentation was seen in 1148 (> 2 hrs) and 805 (> 4 hrs) patients. The duration from onset of symptoms to the presentation in the emergency room (pre-hospital delay) was 228 ± 341minutes.The door to needle time was 34 ± 24 minutes. The major factors for pre-hospital delay were misinterpretation of symptoms (45%) and transportation problems (27%). CONCLUSIONS: The problem of pre-hospital delay continues to remain a major hurdle in initiating timely reperfusion therapy in patients with acute STEMI. Lack of awareness and poor transportation facilities are the major contributors. It should be the goal of STEMI care programmes of the future to make a concerted effort to addressing these factors, in order to optimize the benefit of reperfusion therapy for this high risk group of patients.

Prevalence and predictors of ventriculo-atrial conduction in structurally normal hearts.

BACKGROUND: The prevalence of ventriculo-atrial (VA) conduction varies from 20% to 90%, depending on the population studied (Militianu et al., 1997; Inoue et al., 1985; Kazmierczak et al., 1993; Ciemniewski et al., 1990; Hayes and Furman, 1983; Westveer et al., 1984). This wide range is mostly based on studies done in patients with implanted devices or impaired atrioventricular conduction. However, the prevalence of VA conduction in structurally normal heart has not been well documented till date. OBJECTIVE: To study the prevalence and identify predictors of retrograde conduction via the His-Purkinje system and AV node in structurally normal hearts. METHODS: We included 54 consecutive adults without structural heart disease who underwent electrophysiological (EP) study for various tachycardias. The basic parameters including PR, AH and HV intervals, atrioventricular Wenckebach point (AVWP) and anterograde effective refractory period (ERP) of atrioventricular node (AVNERP), were measured after ablation. The VA conduction was assessed basally and if absent, after isoprenaline. The VA Wenckebach point (VAWP) and retrograde ERP(VAERP) were recorded in patients showing VA conduction. RESULTS: The mean age was 37.1±12.6years. Twenty five (46%) of the patients were men. VA conduction was present in 30 (55%) patients at baseline. Of the remaining 24 patients, 18 (34%) showed VA conduction after isoprenaline. Only 6 (11%) patients failed to reveal VA conduction even after adequate response to isoprenaline. Amongst all clinical and EP variables analysed, only the HV interval was shorter (p<0.01) in patients with VA conduction. CONCLUSION: In structurally normal hearts, VA conduction was present at baseline in 55% of patients. Isoprenaline unmasked VA conduction in an additional 34% of the subjects. The HV interval was longer in patients without VA conduction.

Percutaneous balloon valvuloplasty with Inoue balloon catheter technique for pulmonary valve stenosis in adolescents and adults.

BACKGROUND: Percutaneous balloon pulmonary valvuloplasty is the procedure of choice for uncomplicated severe or symptomatic pulmonary stenosis. The present study describes our experience in balloon pulmonary valvuloplasty using the Inoue balloon catheter in adolescent and adult patients. AIMS: To assess the immediate and mid-term outcomes of percutaneous balloon valvuloplasty with Inoue balloon catheter in adolescent and adult patients. METHODS AND RESULTS: Between June 2010 and July 2015, we performed percutaneous balloon pulmonary valvuloplasty with Inoue balloon catheter in 32 patients (59.37% females) aged 8 to 54 years (mean 23.6±11.5). Following the procedure, the mean right ventricular systolic pressure and the pulmonary valvular peak-to-peak systolic gradient decreased from (121.6±42.4 to 61.19±24.5mmHg, p=0.001) and (100.9±43.3 to 36.4±22.5mmHg, p=0.001), respectively. Twenty patients (Group A) showed immediate optimal results with post-procedure peak systolic gradient <36mmHg while 12 patients (Group B) had suboptimal results. An increase in pulmonary regurgitation by one grade was detected in 17 patients (53.2%). Twenty-three patients available for follow-up (mean duration, 2.75 years [range 0.25-5 years]) had a mean residual peak gradient of 23.6±2.51mmHg on Doppler echocardiography with attenuation of reactive RVOT stenosis in all Group B patients. There was no further increase in grade of pulmonary regurgitation or restenosis on mid-term follow-up. CONCLUSION: Percutaneous Inoue balloon technique is an attractive alternative with excellent mid-term results for adolescents and adults with isolated pulmonary stenosis.

Long-term cardiac (valvulopathy) safety of cabergoline in prolactinoma.

BACKGROUND: Clinical relevance of association of cabergoline use for hyperprolactinemia and cardiac valvulopathy remains unclear. OBJECTIVE: The aim of the study was to determine the prevalence of valvular heart abnormalities in patients taking cabergoline for the treatment of prolactinoma and to explore any associations with the cumulative dose of drug used. DESIGN: A cross-sectional echocardiographic study was performed in patients who were receiving cabergoline therapy for prolactinoma. RESULTS: Hundred (61 females, 39 males) prolactinoma cases (81 macroprolactinoma and 19 microprolactinoma) were included in the study. The mean age at presentation was 33.9 ± 9.0 years (range: 16-58 years). The mean duration of treatment was 53.11 ± 43.15 months (range: 12-155 months). The mean cumulative dose was 308.6 ± 290.2 mg (range: 26-1196 mg; interquartile range: 104-416 mg). Mild mitral regurgitation was present in one patient (cumulative cabergoline dose 104 mg). Mild tricuspid regurgitation was present in another two patients (cumulative cabergoline dose 52 mg and 104 mg). Aortic and pulmonary valve functioning was normal in all the cases. There were no cases of significant valvular regurgitation (moderate to severe, Grade 3-4). None of the patients had morphological abnormalities such as thickening, calcification, and restricted mobility of any of the cardiac valves. CONCLUSION: Cabergoline appears to be safe in patients with prolactinoma up to the cumulative dose of ~300 mg. The screening for valvulopathy should be restricted to those with higher cumulative cabergoline exposure.

Cirrhotic cardiomyopathy is less prevalent in patients with Budd-Chiari syndrome than cirrhosis of liver.

BACKGROUND AND AIM: Cirrhotic cardiomyopathy (CCM) is associated with high mortality after transjugular intrahepatic portosystemic shunt (TIPS) and liver transplantation in patients with cirrhosis. There is no data about the prevalence or impact of CCM in Budd-Chiari syndrome (BCS). We assessed the prevalence of CCM in patients with BCS and its impact on outcome after radiological intervention. METHODS: Thirty-three consecutive patients with BCS (15 men) and 33 controls with hepatitis B-related cirrhosis (18 men, matched for Child-Pugh score) were evaluated with baseline electrocardiography (ECG), echocardiography (ECHO) and dobutamine stress ECHO, and ECG (DSE). The two groups were compared for prevalence of CCM. Patients with BCS with and without CCM were assessed for development of heart failure, duration of intensive care unit (ICU) stay, and in-hospital mortality immediately after radiological intervention. RESULTS: Fewer patients with BCS had CCM (7/21 vs. 21/33; p = 0.001, OR-0.16, CI [0.05-0.5]), diastolic dysfunction (DD) (0/33 vs. 6/33; p = 0.01, OR-0.06, CI [0.00-1.1]), and prolonged QTc interval (5/33 vs.17/33; p = 0.001, OR-0.16, CI [0.05-0.5]) despite correction for age. Patients with BCS had lower end-systolic and end-diastolic volumes of left and right ventricles. None of the 19 patients (five with CCM) with BCS undergoing radiological intervention (12 TIPS, 4 inferior vena cava, and 3 hepatic vein stenting) developed heart failure or had prolonged ICU stay. There was no in-hospital mortality. CONCLUSION: Patients with BCS have lower frequency of CCM as compared to patients with cirrhosis. CCM may not adversely affect outcomes after radiological interventions.

Brothers with constrictive pericarditis - A novel mutation in a rare disease.

Familial constrictive pericarditis is extremely rare. We report a case of two brothers both suffering constrictive pericarditis along with having multiple painless joint deformities. Genetic workup confirmed the clinical diagnosis of camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome CACP syndrome and also revealed a rare mutation in the causative gene.

Echocardiographic parameters in clinical responders to surgical pericardiectomy - A single center experience with chronic constrictive pericarditis.

BACKGROUND: Chronic constrictive pericarditis (CCP) is the end result of chronic inflammation of the pericardium. Developing countries continue to face a significant burden of CCP secondary to tuberculous pericarditis. Surgical pericardiectomy offers potential cure. However, there is paucity of echocardiography data in post-pericardiectomy patients vis-a-vis their clinical status. We studied the changes in multiple echocardiographic parameters in these patients before and after pericardiectomy. METHODS: Twenty-three patients (14 men, 9 women) who underwent pericardiectomy for CCP in the last 5 years (from January 2009 to December 2014) were subjected to detailed clinical and echocardiographic evaluation during the study period (between June 2013 and December 2014). Patients with residual symptoms of NYHA class II and below were considered as 'responders'. The data thus obtained were compared to the pre-operative parameters. RESULTS: After pericardiectomy, the incidence of vena caval congestion decreased from 100% to 15% (p<0.001). There was significant reduction in the mean left atrial size from 39.33±10.52mm to 34.45±10.08mm (p<0.001) and also the ratio of left atrium to aortic annulus from 1.93 to 1.69 (p<0.001) among 'responders' to pericardiectomy. Septal bounce was observed to persist in 5 (25%) patients after pericardiectomy. There was significant respiratory variation of 39.23±15.11% in the mitral E velocity before pericardiectomy. After pericardiectomy, this variation reduced to 14.43±7.76% (p<0.001). There was also significant reduction in the respiratory variation in tricuspid E velocities from 31.33±18.81% to 17.35±16.26% (p<0.001). After pericardiectomy, the mean ratio of mitral annular velocities, medial e': lateral e', reduced from 1.08 to 0.87 (p<0.03). The phenomenon of 'annulus reversus' was found to persist in 6 'responders', thereby reflecting a 50% reduction in its incidence after pericardiectomy (p<0.001). The ratio of mitral E to medial e' (E/e') increased from 4.21±1.35 before pericardiectomy to 6.91±2.62 after pericardiectomy (p=0.001). CONCLUSION: Among clinical responders to surgical pericardiectomy, echocardiographic assessment revealed a significant reduction in vena caval congestion, LA size, ratio of LA to aortic annulus, septal bounce, respiratory variation in mitral and tricuspid E velocities, mitral annular medial e' and the phenomenon of annulus reversus. Also, there was a significant rise in minimum tricuspid and mitral E velocities and the E/e' ratio.

Cardiac manifestations in mucopolysaccharoidosis.

Through this case, we want to highlight, that in patients with mucopolysaccharoidosis (MPS), coronary arteries need to be screened in patients with LV dysfunction. Further, CT scan may not be a good modality and newer diagnostic modalities like IVUS must be offered to delineate concentric obstruction. Also, meticulous screening for hypertension must be carried out in all MPS patients.

Giant aneurysm of the vertical vein in a case of supracardiac total anomalous pulmonary venous connection.

A 17-year-old female presented with mild cyanosis. Imaging showed supracardiac total anomalous pulmonary venous connections with a vertical vein travelling between the left pulmonary artery and the left bronchus with significant obstruction. There was a huge post-stenotic aneurysm of the vertical vein.