Functional tricuspid stenosis: a rare presentation of suspected rhabdomyoma as congenital cyanotic heart disease.

Cardiac tumours in newborns are often asymptomatic and can be sporadically detected on routine screening unless they result in intractable arrhythmias or haemodynamically significant obstructions causing heart failure. Their presentation as a cause of congenital cyanosis is never anticipated. We report a rare case of a newborn presenting with congenital cyanosis consequent to suspected cardiac rhabdomyoma causing tricuspid inflow obstruction. Our experience with this patient with two large cardiac masses illustrates the significance of its inclusion in the differential diagnosis of perinatal cyanosis, as early detection and surgical management might be the only lifesaving options, if performed well in time.

Mitral atresia with transposed great arteries and normal semilunar valves: a rare combination.

Mitral atresia is commonly seen as a part of the spectrum of hypoplastic left heart syndrome, and it is usually associated with multiple levels of systemic outflow tract obstruction. Isolated mitral atresia with a normal aortic valve is extremely rare. We report the rare combination of mitral atresia, transposition of the great arteries, and unobstructed systemic and pulmonary blood flow.

Coronary sinus filling time: a novel method to assess microcirculatory function in patients with angina and normal coronaries.

OBJECTIVE: Dysfunction of the coronary microcirculation is considered as one of the factors responsible for symptoms and abnormal stress tests in patients with angina and normal coronaries (syndrome X). We sought to evaluate the usefulness of coronary sinus filling time (CSFT) to assess coronary microcirculation in this group of patients. METHODS: We compared the CSFT of patients having definite angina or atypical angina with positive treadmill electrocardiography test (angina group), with that of patients undergoing coronary angiogram (CAG) prior to balloon mitral valvuloplasty (control group). During CAG, coronary sinus was visualized in appropriate views and CSFT in seconds was derived from frame count. Thrombolysis In Myocardial Infarction (TIMI) flow grade, corrected TIMI (cTIMI) frame count, TIMI Myocardial Perfusion grade (TMP) were assessed. RESULTS: There were 41 patients in angina group and 16 in control group. Among the angina group 68.8% were females as against 81.8% in the control group. 87.8% (n = 36) had typical angina. Mean CSFT was 4.25 ± 0.72 s and 3.46 ± 0.99 s in the angina group and control group respectively (p = 0.001). No significant differences were found between the groups with respect to TMP (p = 0.68) & cTIMI frame count (p = 0.22). CONCLUSION: CSFT is a simple method to assess the transit time through coronary microcirculation. CSFT was significantly delayed in patients with angina and normal coronaries. TMP and cTIMI frame count were not significantly different between groups.

Clinical profile and one-year outcomes of patients with mural infective endocarditis: - A tertiary care centre study based on data from a seven-year registry.

BACKGROUND: Infective endocarditis patients present very rarely with vegetations on the mural endocardium. Only very few studies are available comparing Mural infective endocarditis with commoner valvular or device related infective endocarditis. AIM: To analyse the clinical features, microbiological profile and clinical course of mural endocarditis in comparison to valvular endocarditis. METHODS: This was a retrospective analysis of data from a registry of infective endocarditis. Patients enrolled between April 2012 and April 2019 were included. Patients who were reported to have vegetations on the mural endocardial surface were taken as a group and compared with rest of the patients. Clinical profile, laboratory parameters including culture and outcomes were compared between the two groups. RESULTS: Out of 278 patients in the study, 15 (5.38%) had vegetations on the mural endocardium. Of them, only 4 patients had structural heart diseases. All the patients with mural endocarditis were NYHA class II or below at presentation. Ventricles were the commonest sites of vegetations. Inflammatory markers like ESR and CRP were low in mural endocarditis compared to rest. Culture positivity was high in mural endocarditis and Staphylococcus Aureus was the commonest organism. Mural endocarditis patients had similar in hospital mortality to rest of the patients. Cardiac complications were not reported in mural endocarditis, but they had similar incidence of embolic complications including neurological events. CONCLUSION: Mural endocarditis is a rare clinical entity with similar morbidity and mortality to that of endocarditis with valvular vegetation.

3D speckle tracking echocardiographic strain pattern in Hypertrophic Cardiomyopathy and its relation with Sudden Cardiac Death risk markers.

CONTEXT: Sudden cardiac death (SCD) predictability for assessing the need for primary insertion of Implantable Cardioverter Defibrillator (ICD) in patients with Hypertrophic cardiomyopathy (HCM) is difficult though there are several conventional risk markers. The role of deformation indices in predicting SCD in HCM is less addressed. OBJECTIVES: To analyse the 3D speckle tracking echocardiographic strain parameters of HCM patients and its relation with SCD risk markers. DESIGN AND STUDY METHODOLOGY: It was a cross-sectional observation study done over a period of one year with a follow up period of one year. Fifty HCM patients were included after screening eighty-two patients. Their global LV strain parameters, Global Longitudinal Strain (GLS), Global Circumferential Strain (GCS), Global Radial Strain (GRS) and Global area strain (GAS) were analysed with respect to their age and gender-matched controls. The various strain parameters were correlated with the conventional SCD risk markers and the ESC SCD risk score among these HCM patients. RESULTS: All the global strain parameters were significantly low in HCM patients compared to their controls {GLS -7.30 ± 3.424 vs -18.78 ± 2.342, p < 001; GCS -11.26 ± 2.754 vs -25.08 ± 3.542, p < 001; GRS 20.56 ± 8.929 vs 39.70 ± 7.546, p < 001}. On subgroup analysis of HCM patients with LV thickness >30 mm, abnormal exercise test, family history of SCD, LVOT gradients >30mmHgand more than one SCD risk marker had significantly low values for all global deformation parameters, when compared with their control HCM cohort. The ESC risk score also had significant inverse correlation with all deformation parameters (GLS 0.496, p < 0.001; GCS 0.491, p < 0.001; GRS -0.529, p < 0.001; GAS 0.519, p < 0.001). On follow up, only one event was recorded in this cohort. CONCLUSION: There exists a possible linear correlation between conventional SCD risk markers and 3D deformation parameters, which may be utilized for risk stratification and SCD predictability in HCM patients after confirmation with further large prospective studies.

Strain patterns in primary mitral regurgitation due to rheumatic heart disease and mitral valve prolapse.

OBJECTIVE: Left atrial (LA) and left ventricular (LV) remodelling are the adaptive changes that occur in primary mitral regurgitation (MR) and are related to its clinical outcomes. Despite the pathophysiological differences in MR in rheumatic heart disease (RHD) and mitral valve prolapse (MVP), whether the pattern of LV and LA remodelling is different between the two conditions remains unknown. Hence, we compared the LA and LV strain pattern in MR due to RHD, the predominant etiology in developing countries topatients with MVP and age and sex-matched controls. METHODS: A total of 50 patients of severe MR which included 30 MVP MR and 20 RHD MR were assessed by strain imaging by speckle tracking echocardiography (STE) and were compared with age and sex-matched controls. 2D STE was used for LA and 3D STE was used for LV strain analysis. LA and LV strain parameters were compared between MVP MR and RHD MR groups. RESULTS: 30 patients with MVP and 20 with RHD were studied. 60% (n = 30) were symptomatic. Mean GLS was -17.2 ± 4.4% compared to -20 ± 3.2% among controls and mean LA strain was 17.35 ± 10.3% compared to 51.34 ± 11.5% among controls which were significantly lower (both p < 0.01). No significant difference in LA strain and GLS was found between MVP and RHD subgroups (LA strain 20.45 ± 11.9% and 14.63 ± 8.85%; p = 0.08; GLS - 18.25 ± 4.3% and-16.2 ± 4.6%; p = 0.12). PALS in the RHD group was lower compared to MVP(p = 0.08) which showed a trend towards significance. LV strain parameters showed no significant difference among the MVP and RHD groups. CONCLUSION: LA and LV strain parameters showed no significant difference in MR due to either RHD or MVP. There was a trend towards lower LA strain in RHD which needs validation with large multicentric studies. The current strain parameters from MVP with the prognostic value may be applied to MR of RHD etiology, pending confirmation of our results by other groups.

One-year clinical outcome of percutaneous coronary intervention with very long (≥ 40mm) drug-eluting stent.

OBJECTIVES: The aim of this study was to assess the clinical outcome of patients with diffuse coronary lesions treated with very long drug-eluting coronary stents (DES) (≥ 40mm) over a period of one year. METHODS: This single-center prospective study enrolled a total of 343 consecutive patients (376 long stents) who underwent percutaneous coronary stent implantation with very long DES. One year clinical outcomes were analyzed. A subgroup analysis of diabetic patients was also performed. RESULTS: One year follow up data was available for 314 patients (91.5%). All-cause mortality was 5 (1.6%). Eleven (3.5%) patients had non-ST-elevation myocardial infarction. Definite / probable stent thrombosis was reported in 7 (2.2%) patients. Over one year, 3 (1%) patients underwent target lesion revascularization (TLR). The total number of target lesion failure was 9 (2.9%). The rate of target lesion failure at one year was 2.6% using one vessel per patient analysis. Two patients had ischemic stroke. Any major adverse cardiac event (MACE) was observed in 19 (6%) patients. The event rates between sirolimus and everolimus stent groups were compared - cardiac death (1.7% vs 1.5%; p=0.911), stent thrombosis (2.5% vs 1.7%; p=0.612), TLR (1% vs 0.8%; p=0.878), any MACE (7% vs 4.1%; p=0.284). Exertional dyspnea was reported by 47 (15%) patients at the end of one year. Dual antiplatelet adherence rate was 96% (n=301 of 314). CONCLUSION: Use of very long stents (≥40mm) for diffuse coronary lesions is safe and effective with acceptably low event rates. No significant differences in event rates were observed between the types of DES used in this study (Sirolimus Vs. everolimus).

Pulmonary hypertension registry of Kerala, India (PRO-KERALA) - Clinical characteristics and practice patterns.

BACKGROUND: Epidemiological data on pulmonary hypertension (PH) are scarce from developing countries including India. METHODS: We established a multi-center registry of PH, the PRO-KERALA registry, in Kerala, India. Fifty hospitals enrolled consecutive adult (>18 years) patients for one year. Echocardiographic criteria (right ventricular systolic pressure - RVSP > 50 mmHg) or invasively obtained mean pulmonary artery pressure > 25 mmHg was the criteria for entry. RESULTS: There were 2003 patients (52% Women, mean age 56 ±â€¯16.1 years) enrolled. The mean RVSP was 68.2 (SD = 17.9) mmHg. Majority of the study participants (59%) belonged to group 2 of the WHO Nice Classification 2013 (PH secondary to left heart disease). One-fifth (21.2%) belonged to group 1, while 13.3%, 3.8% and 2.4% of the study population belonged to groups 3, 4 and 5 respectively. More than a quarter (27%) reported PH due to left heart disease with valvular disease etiology; while 20.7% had coronary artery disease. The other common etiological factors were chronic obstructive pulmonary disease (10.6%), congenital heart disease (14.6%), idiopathic pulmonary hypertension (5.8%), and chronic thromboembolic pulmonary hypertension (3.8%). Only one of two patients with pulmonary artery hypertension was receiving PH specific therapies. The use of combination therapy was negligible and PH-specific therapies were prescribed off-label to a small proportion of patients too. CONCLUSION: PRO-KERALA is the first PH registry from South Asia and the second largest globally. Left heart diseases attribute to three fifths of patients with PH. Utilization rates of PH specific drug therapies are remarkably lower than the Western population.

'Caterpillar right ventricle': unusual manifestation of a rare disease.

A 66-year-old man presented with abdominal distension and pedal oedema for the past 10 years. He had history of right heart failure on several occasions in the past, and one of these admissions prompted referral to a cardiac specialist. On examination, he had markedly elevated jugular venous pressure with prominent 'y-descent', a tricuspid regurgitation murmur, gross ascites and pedal oedema. A full blood count, routine biochemical screen and serum bicarbonate levels were normal. Right ventricular angiogram revealed a 'caterpillar'-like aneurysm of the right ventricle (RV) apex (figure 1 arrow) with a dilated right ventricular outflow tract and significant tricuspid regurgitation with a dilated right atrium (see online supplementary video 1). Figure 1 Acine angiographic frame of the right ventricle in posteroanterior view, showing a 'caterpillar'-like right ventricle aneurysm (arrow). 10.1136/heartasia-2017-010957.supp3Supplementary file 3. QUESTION: On the basis of the clinical and right ventriculography features, what is the most likely diagnosis for this patient?Arrhythmogenic right ventricular cardiomyopathy (ARVC)Right ventricular endomyocardial fibrosis (RV-EMF) with an RV aneurysmCardiac sarcoidosisCongenital diverticulum of the right ventricle.

Demographical and clinicopathological characteristics in heart failure and outcome predictors: a prospective, observational study.

AIMS: The aims of the study were to study the demographical and clinicopathological characteristics of patients presenting with heart failure and evaluate the 1 year outcomes and to identify risk predictors if any. METHODS AND RESULTS: A prospective observational study was conducted in consecutive patients of systolic heart failure. The study was divided into two parts-an initial 6 month enrolment phase followed up for 1 year for major adverse cardiovascular events. All patients were treated according to the Institutional Heart Failure Protocol. Demographical and clinicopathophysiological characteristics were studied, and results were analysed. A total of 143 patients were enrolled. The mean age of subjects was 56.4 years with male subjects constituting almost two-thirds of the study population. The commonest aetiology of heart failure was ischemic with valvular heart disease being the commonest cause of non-ischemic heart failure. Bendopnea, a recently described symptom of heart failure, was found in a significant number of subjects. By univariate analysis, male sex (P = 0.042) and cardiomegaly (P = 0.035) were predictors of rehospitalization, whereas the univariate predictors of mortality were ischemic aetiology (P = 0.000), age > 50 years (P = 0.007), hypertension (P = 0.012), worsening NYHA class (P = 0.003), diabetes mellitus (P = 0.009), and hypokalaemia (P = 0.006). Multivariate analysis performed showed age > 50 years [P = 0.007; OR (CI) = 13.547 (2.034-90.238)], NYHA class [P = 0.002; OR (CI) = 32.300 (3.733-276.532)], and hypokalaemia [P = 0.031; OR (CI) = 7.524 (1.208-46.862)] as significant predictors of mortality during long-term follow-up. CONCLUSIONS: The study will definitely help us to throw more light in identifying risk predictors of heart failure and help in improving clinical outcomes.

Prevalence and prognostic significance of left ventricular myocardial late gadolinium enhancement in severe aortic stenosis.

BACKGROUND: Myocardial fibrosis occurs in aortic stenosis (AS) as part of the hypertrophic response. It can be detected by LGE, which is associated with an adverse prognosis in the form of increased mortality and morbidity. OBJECTIVES: To assess the prevalence of LGE patterns using cardiac magnetic resonance (CMR) in severe AS patients and to study its prognostic significance. METHODS: Patients enrolled into the study from June 2012 to November 2014. All the patients underwent CMR and various patterns of LGE studied. These patients if symptomatic were advised AVR and others were managed conservatively. All patients were followed up and watched for outcomes like mortality, heart failure/hospitalization for cardiovascular cause, fall in left ventricular ejection fraction (LVEF) ≥20% and arrhythmia. RESULTS: A total of 109 patients (mean age-57.7±12.5yrs) underwent CMR with 63 males. These patients were followed up for a mean of 13 months. Among 38 patients who underwent AVR, 6 died (5-cardiovascular cause, 1-non cardiovascular). 71 patients were managed conservatively out of which 18 died (17-cardiovascular cause, 1-non cardiovascular cause). LGE patterns were seen in 46 patients (43%); mid myocardial enhancement was seen in 31.1% of cases (33 patients). No LGE pattern was seen in 57%(63 patients). Basal and mid regions were maximally involved with mid myocardial enhancement in 66% & 68.3% respectively. LV ejection fraction (p=0.002), peak aortic systolic velocity (p=0.01) and peak aortic systolic gradient (p=0.02) were the main predictors of LGE. Main predictors of primary outcome were NYHA class [OR- 13.4(2.8-26.1), p≤0.001], age- 62± 9.6yrs(p=0.001), EF simpson-50.9±13%(p≤ 0.001), LGE[OR 2.8 (1.27-6.47),p=0.01], number of segments involved [2.37±2.1,P≤0.001] & CMR LV mass (151.73±32gms, p=0.007). LGE predicted heart failure/hospitalization for cardiovascular cause [OR- 3.8(1.2-11.9), p=0.01] and fall in LVEF [OR- 5.8(1.5-22.5), p=0.005]. Patients with LGE had 2.87 times risk of adverse outcomes and patients with more than 3 segment LGE involvement had again increased chances for adverse outcomes. CONCLUSIONS: LGE was detected by CMR in 43% of patients with severe AS. It predicted recurrent heart failure, hospitalization for cardiovascular cause and fall in LV ejection fraction. Our study has laid a path to larger prospective studies with long term follow up to assess the prognostic impact of CMR in patients with severe AS.

Bilateral variant origin of subclavian artery branches.

Subclavian artery branching patterns have been studied in cadaveric series and frequencies of the many variations have been documented. However, such variations have been seldom noticed antemortem. Here, we present the case of a very rare type of bilaterally different branching pattern of the subclavian artery.

Effect of balloon mitral valvotomy on left ventricular function in rheumatic mitral stenosis.

AIM: Mitral stenosis (MS) is found to produce left ventricular (LV) dysfunction in some studies. We sought to study the left ventricular function in patients with rheumatic MS undergoing balloon mitral valvotomy (BMV). Ours is the first study to analyze effect of BMV on mitral annular plane systolic excursion (MAPSE), and to quantify prevalence of longitudinal left ventricular dysfunction in rheumatic MS. METHODS: In this prospective cohort study, we included 43 patients with severe rheumatic mitral stenosis undergoing BMV. They were compared to twenty controls whose distribution of age and gender were similar to that of patients. The parameters compared were LV ejection fraction (EF) by modified Simpson's method, mitral annular systolic velocity (MASV), MAPSE, mitral annular early diastolic velocity (E'), and myocardial performance index (MPI). These parameters were reassessed immediately following BMV and after 3 months of procedure. RESULTS: MASV, MAPSE, E', and EF were significantly lower and MPI was higher in mitral stenosis group compared to controls. Impaired longitudinal LV function was present in 77% of study group. MAPSE and EF did not show significant change after BMV while MPI, MASV, and E' improved significantly. MASV and E' showed improvement immediately after BMV, while MPI decreased only at 3 months follow-up. CONCLUSIONS: There were significantly lower mitral annular motion parameters including MAPSE in patients with rheumatic mitral stenosis. Those with atrial fibrillation had higher MPI. Immediately after BMV, there was improvement in LV long axis function with a gradual improvement in global LV function. There was no significant change of MAPSE after BMV.

Correlation of pulmonary capillary wedge pressure with left atrial pressure in patients with mitral stenosis undergoing balloon valvotomy.

AIMS: We sought to evaluate the correlation between PCWP and LAP and to compare transmitral gradients obtained with LAP and PCWP in MS, before and after balloon mitral valvotomy (BMV). METHODS: Consecutive patients with MS for BMV were included in this prospective cohort study. Simultaneous PCWP and LAP were recorded followed by simultaneous left atrium-left ventricular (LA-LV) and pulmonary capillary wedge pressure-left ventricular (PCWP-LV) gradients before and after BMV. RESULTS: There were 30 patients with a mean age of 41 yrs (males 10 (33.3%), females 20 (66.7%)). There was no significant difference between mean LAP and mean PCWP before BMV (21.3mmHg and 22.3mmHg, respectively) or after BMV (15.3mmHg and 17.3mmHg, respectively). There was excellent correlation between mean PCWP and mean LAP before BMV (r=0.95) (p<0.001) and after BMV (r=0.85) (p<0.001). The phasic components of the pressures (a and v waves) of LAP and PCWP also showed good correlation before and after BMV. Further, transmitral gradients assessed by LA-LV and PCWP-LV pressures showed excellent correlation before BMV (r=0.95) (p<0.001) and after BMV (r=0.95) (p<0.001). CONCLUSION: In patients with MS undergoing balloon valvotomy, PCWP shows good correlation with LAP. Transmitral gradients obtained with PCWP and LAP also correlate well after correction of phase lag in PCWP tracing. Hence, PCWP can be used for reliable measurement of transmitral gradient.

A comparative study of ivabradine and atenolol in patients with moderate mitral stenosis in sinus rhythm.

BACKGROUND: Beta-blockers are frequently used in patients with mitral stenosis to control the heart rate and alleviate exercise-related symptoms. The objective of our study was to examine whether ivabradine was superior to atenolol for achieving higher exercise capacity in patients with moderate mitral stenosis in sinus rhythm. We also evaluated their effects on left ventricular myocardial performance index (MPI). METHODS AND RESULTS: Eighty-two patients with moderate mitral stenosis in sinus rhythm were randomized to receive ivabradine (n=42) 5mg twice daily or atenolol (n=40) 50mg daily for 6 weeks. Transthoracic echocardiography and treadmill test were performed at baseline and after completion of 6 weeks of treatment. Mean total exercise duration in seconds markedly improved in both study groups at 6 weeks (298.57±99.05s vs. 349.12±103.53s; p=0.0001 in ivabradine group, 290.90±92.42s vs. 339.90±99.84s; p=0.0001 in atenolol group). On head-to-head comparison, there was no significant change in improvement of exercise time between ivabradine and atenolol group (p=0.847). Left ventricular MPI did not show any significant change from baseline and at 6 weeks in both drug groups (49.8%±8% vs. 48.3%±7% in ivabradine group, 52.9%±10% vs. 50.9%±10% in atenolol groups; p=0.602). CONCLUSION: Ivabradine or atenolol can be used for heart rate control in patients with moderate mitral stenosis in sinus rhythm. Ivabradine is not superior to atenolol for controlling heart rate or exercise capacity. Left ventricular MPI was unaffected by either of the drugs.

Device closure of pulmonary arteriovenous malformation using Amplatzer vascular plug II in hereditary hemorrhagic telangiectasia.

Pulmonary arteriovenous malformations (AVM) are very rare and carry the risk of cerebral thrombo-embolism, brain abscess or pulmonary hemorrhage. The Amplatzer vascular plug II (AVP II) is a new device, used for embolization of the pulmonary AVMs. We report a case of pulmonary AVM successfully managed by using AVP II in a patient with hereditary hemorrhagic telangiectasia (HHT).

Double ventricular rupture after acute myocardial infarction: A rare case report.

A previously asymptomatic 53-year-old male presented 5 days after an acute anterior wall myocardial infarction, who was fibrinolytic naïve, with worsening dyspnea. Transthoracic echocardiographic evaluation revealed rupture of the interventricular septum and pseudoaneurysm of the left ventricle, confirmed by angiography. Coronary angiogram revealed multivessel disease. The patient underwent successful closure of ventricular septal rupture with repair of pseudoaneurysm and saphenous vein grafts to posterior descending branch of right coronary artery and obtuse marginal branch of left circumflex artery. Double ventricular ruptures following acute myocardial infarction are very rare with a reported incidence of 0.3% from various series in the revascularization era. They are also associated with exceedingly high mortality rates reaching up to 50%, even when intervened emergently.

Hammock mitral valve: a rare cause of congenital mitral regurgitation--a case report.

Hammock valve, also known as anomalous mitral arcade is a rare mechanism for congenital mitral insufficiency. We report a case of a two-week-old neonate who presented with features of heart failure and an apical systolic murmur. Echocardiogram showed severe mitral regurgitation and abnormal mitral valve with direct attachment of mitral leaflets to papillary muscle without intervening chordae tendinae, typical of hammock valve. Heart failure was controlled with ionotrpes and diuretics. The literature on the hammock mitral valve is reviewed.