Isolation of the left subclavian artery: a rare aortic arch anomaly.

Isolation of the left subclavian artery is a rare anomaly associated with acyanotic CHDs and right aortic arch. This condition can be asymptomatic or can present with neurological and left upper-limb ischaemic symptoms; it does not form a vascular ring. Treatment options include implantation of the subclavian artery to the aortic arch vessels and closure of the patent ductus arteriosus to prevent pulmonary run off. Here we describe a child who was incidentally detected to have this condition during evaluation of atrial septal defect for device closure.

Percutaneous device closure of Abernethy malformation--a treatable cause of hepatopulmonary syndrome.

An eight-year-old girl was evaluated for unexplained cyanosis. A contrast echo was suggestive of pulmonary arteriovenous fistula. Further evaluation revealed Abernethy malformation (type 2) leading to hepatopulmonary syndrome. Percutaneous device closure of Abernethy malformation was done after anatomical and physiological evaluation of the portal circulation. Prior to closure, it is important to ensure adequate portal radicals into the liver and normal portal pressure after test balloon occlusion. Subcostal echocardiography can diagnose and guide device closure of Abernethy malformation, a treatable cause of hepatopulmonary syndrome.

Voices of care: unveiling patient journeys in primary care for hypertension and diabetes management in Kerala, India.

Background: Diabetes and hypertension are leading public health problems, particularly affecting low- and middle-income countries, with considerable variations in the care continuum between different age, socio-economic, and rural and urban groups. In this qualitative study, examining the factors affecting access to healthcare in Kerala, we aim to explore the healthcare-seeking pathways of people living with diabetes and hypertension. Methods: We conducted 20 semi-structured interviews and one focus group discussion (FGD) on a purposive sample of people living with diabetes and hypertension. Participants were recruited at four primary care facilities in Malappuram district of Kerala. Interviews were transcribed and analyzed deductively and inductively using thematic analysis underpinned by Levesque et al.'s framework. Results: The patient journey in managing diabetes and hypertension is complex, involving multiple entry and exit points within the healthcare system. Patients did not perceive Primary Health Centres (PHCs) as their initial points of access to healthcare, despite recognizing their value for specific services. Numerous social, cultural, economic, and health system determinants underpinned access to healthcare. These included limited patient knowledge of their condition, self-medication practices, lack of trust/support, high out-of-pocket expenditure, unavailability of medicines, physical distance to health facilities, and attitude of healthcare providers. Conclusion: The study underscores the need to improve access to timely diagnosis, treatment, and ongoing care for diabetes and hypertension at the lower level of the healthcare system. Currently, primary healthcare services do not align with the "felt needs" of the community. Practical recommendations to address the social, cultural, economic, and health system determinants include enabling and empowering people with diabetes and hypertension and their families to engage in self-management, improving existing health information systems, ensuring the availability of diagnostics and first-line drug therapy for diabetes and hypertension, and encouraging the use of single-pill combination (SPC) medications to reduce pill burden. Ensuring equitable access to drugs may improve hypertension and diabetes control in most disadvantaged groups. Furthermore, a more comprehensive approach to healthcare policy that recognizes the interconnectedness of non-communicable diseases (NCDs) and their social determinants is essential.

Percutaneous closure of a moderate to large tubular or elongated patent ductus arteriosus in children younger than 3 years: is the ADO II appropriate?

Protrusion of the Amplatzer duct occluder (ADO) II device into the aortic isthmus or the pulmonary artery causing obstruction and residual flow has been reported, but the same has not been widely studied in small children with a patent ductus arteriosus (PDA) anatomy not considered suitable for closure with the ADO I device. This study aimed to report the safety and efficacy of the ADO II device in children younger than 3 years with a tubular or elongated PDA and to analyze the possible reasons for residual flow in children with such a PDA. In this study, 17 children younger than 3 years (mean age, 10.3 ± 7 months; mean weight, 6 ± 3.6 kg) underwent attempted closure of a tubular or elongated PDA (mean diameter at the narrowest point, 4.1 ± 1.1 mm) with the ADO II device between July 2010 and July 2012. Of the 17 patients, 16 (2 boys and 14 girls) completed the follow-up evaluation. A complete echocardiographic evaluation was performed on all the patients before PDA closure and at the follow-up visit, and the results were compared with those of previous published studies. Of the 16 patients, the 15 who completed the follow-up evaluation had successful device closure (1 device embolization). Residual flow was present in six patients immediately after deployment, which was reduced to three patients at the last follow-up visit. Five of nine patients closed with a 6-mm-long device had residual flow compared with only one of seven patients closed with a 4-mm-long device. After device closure, significant elevations of the left and right pulmonary artery velocities occurred in three and two patients, respectively; in 12 patients, descending thoracic aortic (DTA) velocities increased mildly. There was trend toward a fall in the elevated pressures at the last follow-up visit, although one patient had an elevation in right pulmonary artery velocity at last the follow-up echocardiogram compared with the echocardiogram immediately after closure. Hence, in children younger than 3 years with or without pulmonary arterial hypertension, closure of a PDA not amenable to closure with the ADO I device is feasible using the ADO II device, with an increased incidence of clinically nonsignificant complications. Selection of device dimensions according to the manufacturer's recommendation may not be the optimal strategy.

Impact of comprehensive cardiovascular risk reduction programme on risk factor clustering associated with elevated blood pressure in an Indian industrial population.

BACKGROUND & OBJECTIVES: Cardiovascular risk factors clustering associated with blood pressure (BP) has not been studied in the Indian population. This study was aimed at assessing the clustering effect of cardiovascular risk factors with suboptimal BP in Indian population as also the impact of risk reduction interventions. METHODS: Data from 10543 individuals collected in a nation-wide surveillance programme in India were analysed. The burden of risk factors clustering with blood pressure and coronary heart disease (CHD) was assessed. The impact of a risk reduction programmme on risk factors clustering was prospectively studied in a sub-group. RESULTS: Mean age of participants was 40.9 ± 11.0 yr. A significant linear increase in number of risk factors with increasing blood pressure, irrespective of stratifying using different risk factor thresholds was observed. While hypertension occurred in isolation in 2.6 per cent of the total population, co-existence of hypertension and >3 risk factors was observed in 12.3 per cent population. A comprehensive risk reduction programme significantly reduced the mean number of additional risk factors in the intervention population across the blood pressure groups, while it continued to be high in the control arm without interventions (both within group and between group P<0.001). The proportion of 'low risk phenotype' increased from 13.4 to 19.9 per cent in the intervention population and it was decreased from 27.8 to 10.6 per cent in the control population (P<0.001). The proportion of individuals with hypertension and three more risk factors decreased from 10.6 to 4.7 per cent in the intervention arm while it was increased from 13.3 to 17.8 per cent in the control arm (P<0.001). INTERPRETATION & CONCLUSIONS: Our findings showed that cardiovascular risk factors clustered together with elevated blood pressure and a risk reduction programme significantly reduced the risk factors burden.

An unusual cause of heart failure in postoperative tetralogy of Fallot.

The long-term results of transatrial transpulmonary tetralogy of Fallot (TOF) repair have been excellent. Progressive pulmonary regurgitation and consequent right ventricular (RV) dilatation are the most common long-term sequel of definitive repair in childhood. Overt systemic venous congestion after TOF repair is limited to the rare setting where RV dysfunction sets in due to deferred surgery or progressive arrhythmia. Here, we report a unique case of right heart failure from an unexpected etiology, 28 years after TOF repair. Cardiac catheterization confirmed findings of elevated right heart pressures. Magnetic resonance imaging showed obliteration of the RV apex with late gadolinium enhancement of the right ventricular apical endomyocardium.

Efficacy of a family-based cardiovascular risk reduction intervention in individuals with a family history of premature coronary heart disease in India (PROLIFIC): an open-label, single-centre, cluster randomised controlled trial.

BACKGROUND: Coronary heart disease, a leading cause of death globally, is amenable to lifestyle interventions. The family environment can affect the ability or willingness of individuals to make lifestyle changes. We aimed to investigate the efficacy of a targeted family-based intervention for reduction of total cardiovascular risk in individuals with a family history of premature coronary heart disease. METHODS: We did an open-label, cluster randomised controlled trial (PROLIFIC) in the families (first-degree relatives and spouses, older than age 18 years) of individuals with coronary heart disease who had been diagnosed before age 55 years. Patients with coronary heart disease diagnosed within the past year were selected from a tertiary care speciality hospital that provides care for patients from Kerala, India. Family members of selected patients who were bedridden or terminally ill, and individuals with a history of established cardiovascular heart disease and stroke were excluded, as were families with fewer than two eligible family members. Simple randomisation with computer-generated random numbers was used to randomly assign families to intervention and usual care groups (1:1). Participants in the intervention group received a comprehensive package of interventions facilitated by non-physician health workers, consisting of: screening for cardiovascular risk factors; structured lifestyle interventions; linkage to a primary health-care facility for individuals with established chronic disease risk factors or conditions; and active follow-up for adherence. The usual care group received one-time counselling and annual screening for risk factors. We obtained data on lifestyle, clinical, and biochemical characteristics at baseline and annually during the 2-year follow-up. The primary outcome was achievement or maintenance of any three of the following: blood pressure lower than 140/90 mm Hg, fasting plasma glucose lower than 110 mg/dL, low-density lipoprotein cholesterol lower than 100 mg/dL, and abstinence from tobacco. The primary outcome was analysed in all participants available for follow-up at the relevant timepoint. This trial is registered with Clinicaltrials.gov, NCT02771873. FINDINGS: From Jan 1, 2015, to April 30, 2017, 980 patients with coronary heart disease were assessed for eligibility and 230 were excluded primarily due to lack of evidence of coronary artery disease (n=199), or a diagnosis of coronary heart disease more than 1 year previously (n=29). Of the 750 remaining families, 368 (with 825 participants) were assigned to the intervention group and 382 (with 846 participants) were assigned to the usual care group. At the 2-year follow-up, data from 803 (97%) of 825 participants in the intervention group and 819 (97%) of 846 participants in the usual care group were available. Of the 1671 participants, 1111 (66·5%) were women, and 560 (33·5%) were men. The mean age of the study population was 40·8 years (SD 14·2). At the 2-year follow-up, the primary outcome was achieved by 514 (64%) of 803 participants in the intervention group and 379 (46%) of 819 in the usual care group. After adjustment for clustering and baseline risk factors, the odds of achieving the primary outcome at the 2-year timepoint was two times higher in the intervention group than in the usual care group (odds ratio 2·2, 95% CI 1·7-2·7; p<0·0001). INTERPRETATION: The reduction of total cardiovascular risk observed after the intervention could have a substantial public health impact by preventing future cardiovascular events. FUNDING: The Wellcome Trust and Department of Biotechnology, Government of India, and India Alliance.

Natural history of aortic valve disease following intervention for rheumatic mitral valve disease.

BACKGROUND AND AIM OF THE STUDY: A significant proportion of patients who require interventions for rheumatic mitral valve (MV) disease have coexisting aortic valve (AV) disease. To date, little is known of the natural history of AV disease in these patients. METHODS: The details of a cohort of 200 patients (146 females, 54 males; mean age at MV intervention 30.3 +/- 9.9 years) with rheumatic heart disease were retrospectively reviewed. The patients had undergone an index MV intervention (either closed or balloon mitral valvotomy) or MV replacement between 1994 and 1996, and received long-term regular follow up examinations. The clinical and echocardiographic data at entry and at follow up were noted. Patients were allocated to two groups, based on whether the AV disease was absent (group I, n=98) or present (group II, n=102) at baseline. The AV disease was categorized as thickening only (group IIA), isolated aortic regurgitation (AR) (group IIB), or combined aortic stenosis (AS) and AR (group IIC). No patient had isolated AS at baseline. RESULTS: The mean follow up period was 9.3 +/- 1.07 years; during which 10 patients in group I developed new AV disease, which included AV thickening only (n=2), trivial-mild AR (n=7) and mild AS with trivial AR (n=1). Of 16 patients in group IIA, 11 developed isolated AR, and one patient progressed to have mild AS and AR. Among 69 patients in group IIB, 22 (31.9%) developed AS, and all had either mild (n=8) or moderate (n=14) AR with mild AS. Group IIC included 17 patients with mild combined AV disease at baseline, except for moderate AS and moderate AR in one patient each. Among 16 patients with mild AS in group IIC, six progressed to moderate AS and two to severe AS. AR became moderate in 10 patients and severe in one patient. The two patients who progressed to severe AS requiring AV replacement had mild AS at baseline. No patient who developed new combined AV disease had lesions with severity more than mild AS or moderate AR. On logistic regression analysis of the variables predisposing to progression of AV disease, such as age, gender, history of rheumatic fever (RF) and recurrence, and interval from RF episode to symptom onset, only the initial AV gradient was identified as being statistically significant (beta coefficient 0.528, SE = 0.17, p < 0.0001). CONCLUSION: Patients with no or mild AV disease at the time of MV intervention rarely develop severe AV disease, and seldom require AV surgery over the long-term follow up. The presence of mild AS at baseline is predictive in the minority of cases where AV disease will progress relatively more rapidly.

Doppler echocardiographic assessment of TTK Chitra prosthetic heart valve in the mitral position.

AIMS: TTK Chitra heart valve prosthesis (CHVP), a tilting disc mechanical heart valve of low cost and proven efficacy, has been in use for the last 15 years. Although various studies substantiating its long-term safety and efficacy are available, no study had assessed its echocardiographic characteristics. The purpose of this study was to determine the normal Doppler parameters of CHVP in the mitral position and to assess whether derivation of mitral valve area (MVA) using the continuity equation (CE) and more commonly used pressure half-time (PHT) method is comparable in the functional assessment of this tilting disc mitral prosthesis. METHODS AND RESULTS: Doppler echocardiography was performed in 40 consecutive patients with CHVP in the mitral position. All patients were clinically stable, without evidence of prosthetic valve dysfunction such as significant obstruction or regurgitation, endocarditis, left ventricular dysfunction (ejection fraction <40%), or significant aortic regurgitation. Valve sizes studied included 25, 27, and 29 mm. Mitral valve area was derived both by the PHT method and by the CE, using the stroke volume measured in the ventricular outflow tract divided by the time-velocity integral of CHVP jet. The peak Doppler gradient ranged from 5 to 21 (mean 11.0) mmHg, and the mean gradient ranged from 1.7 to 9.2 (mean 4.1) mmHg. Mean gradient negatively correlated with an increase in the actual orifice area (AOA) derived from the valve orifice diameter given by the manufacturer (r = -0.45, P = 0.004). Mitral valve area calculated by both PHT and CE increased significantly with an increase in the AOA (r = 0.42, P = 0.007 and r = 0.32, P = 0.046, respectively). Mitral valve area by the CE averaged 1.55 +/- 0.36 cm(2) (range 0.85 cm(2) for a 25 mm valve to 2.41 cm(2) for a 29 mm valve) and was smaller than by PHT (mean 2.04 +/- 0.41 cm(2), range 1.40-3.14 cm(2); P = 0.0001; t-test), irrespective of whether PHT is less than or >110 ms. CONCLUSION: The Doppler parameters obtained with CHVP in the mitral position are comparable with those obtained with the different prosthetic valves in common use. In the selected group of patients with CHVP, assessment of MVA by the PHT method is comparable with that by the CE. Areas by both methods were smaller than the AOA provided by the manufacturer, as seen in other similar design valves.

The outcome of mitral regurgitation after the repair of anomalous left coronary artery from the pulmonary artery in infants and older children.

OBJECTIVES: Mitral regurgitation (MR) in the majority of infants with an anomalous left coronary artery from the pulmonary artery (ALCAPA) has been shown to improve without concomitant mitral valve repair. However, the outcome of MR in older children with ALCAPA is unclear. The purpose of this study was to compare the outcome of MR following the ALCAPA repair in infants and older children. METHODS: Forty-six patients (34 were younger than 1 year and 12 were aged 1-12 years) underwent the repair for ALCAPA in our institution from June 2006 to June 2016. Baseline and follow-up data were collected from records, and the latest echocardiogram was performed for all surviving patients from June 2017 to August 2017. RESULTS: Of the 34 infants, 82% had moderate-to-severe ventricular dysfunction, and 47% had significant MR at presentation. Fifty-seven percent of the older age group had MR, though none of them had ventricular dysfunction. Two infants and 3 older children underwent concomitant mitral valve repair. There were 2 early deaths in the infantile group. On follow-up, ventricular function normalized in 88% of infants; 12.5% of the infants had moderate-to-severe MR on follow-up, and 50% of the older children had moderate-to-severe MR on follow-up. CONCLUSIONS: The ALCAPA repair has excellent survival outcomes in all age groups. MR improves in the majority of infants with revascularization alone. MR worsens or persists in a significant number of older children following the ALCAPA repair, and they are more likely to require reinterventions for the mitral valve on follow-up.

Unusual association of aortic valve stenosis with ventricular septal defect and pulmonary atresia: Differentiation from truncus arteriosus with truncal valve stenosis.

Stenosis of systemic semilunar valve in cyanotic congenital heart defects is rare. It can happen in truncus arteriosus with truncal valve stenosis and the very rare anomaly of tetralogy of fallot with aortic valve stenosis. Here we describe a neonate with pulmonary atresia, ventricular septal defect and associated aortic valve stenosis and discuss the points of differentiation from truncus arteriosus.

Persistent pulmonary artery hypertension in patients undergoing balloon mitral valvotomy.

Pulmonary artery pressure (PAP) is known to regress after successful balloon mitral valvotomy (BMV). Data of persistent pulmonary artery hypertension (PPAH) following BMV is scarce. We analyzed the clinical, echocardiographic, and hemodynamic data of 701 consecutive patients who have undergone successful BMV in our institute from 1997 to 2003. Data of 287 patients who had PPAH (defined by pulmonary artery systolic pressure [PASP] of ≥ 40 mmHg at one year following BMV) were compared to the data of 414 patients who did not have PPAH. Patients who had PPAH were older (39.9 ± 9.9 years vs. 29.4 ± 10.1; P < 0.001). They had higher prevalence of atrial fibrillation (AF; 21.9 vs. 12.1%, P < 0.05), moderate or severe pulmonary artery hypertension (PAH) defined as PASP more than 50 mmHg (43.5 vs. 33.8%, P = 0.00), anatomically advanced mitral valve disease as assessed by Wilkin's echocardiographic score > 8 (33.7 vs. 23.2%, P < 0.001), and coexistent aortic valve disease (45.6 vs. 37.9%, P < 0.001) at the baseline. Those patients with PPAH had comparatively lower immediate postprocedural mitral valve area (MVA). On follow-up of more than five years, the occurrence of restenosis (39.3 vs. 10.1%, P = 0.000), new onset heart failure (14% vs. 4%, P < 0.05) and need for reinterventions (9.5% vs. 2.8%, P < 0.05) were higher in the PPAH group. Patients with PPAH were older, sicker, and had advanced rheumatic mitral valve disease. They had higher incidence of restenosis, new onset heart failure, and need for reinterventions on long term follow-up. PPAH represents an advanced stage of rheumatic valve disease and indicates chronicity of the disease, which may be the reason for the poorer prognosis of these patients. Patients with PPAH requires intense and more frequent follow-up.

Association between gender, process of care measures, and outcomes in ACS in India: results from the detection and management of coronary heart disease (DEMAT) registry.

BACKGROUND: Studies from high-income countries have shown that women receive less aggressive diagnostics and treatment than men in acute coronary syndromes (ACS), though their short-term mortality does not appear to differ from men. Data on gender differences in ACS presentation, management, and outcomes are sparse in India. METHODS AND RESULTS: The Detection and Management of Coronary Heart Disease (DEMAT) Registry collected data from 1,565 suspected ACS patients (334 women; 1,231 men) from ten tertiary care centers throughout India between 2007-2008. We evaluated gender differences in presentation, in-hospital and discharge management, and 30-day death and major adverse cardiovascular event (MACE; death, re-hospitalization, and cardiac arrest) rates. Women were less likely to present with STEMI than men (38% vs. 55%, p<0.001). Overall inpatient diagnostics and treatment patterns were similar between men and women after adjustment for potential confounders. Optimal discharge management with aspirin, clopidogrel, beta-blockers, and statin therapy was lower for women than men, (58% vs. 65%, p = 0.03), but these differences were attenuated after adjustment (OR = 0.86 (0.62, 1.19)). Neither the outcome of 30-day mortality (OR = 1.40 (0.62, 3.16)) nor MACE (OR = 1.00 (0.67, 1.48)) differed significantly between men and women after adjustment. CONCLUSIONS: ACS in-hospital management, discharge management, and 30-day outcomes did not significantly differ between genders in the DEMAT registry, though consistently higher treatment rates and lower event rates in men compared to women were seen. These findings underscore the importance of further investigation of gender differences in cardiovascular care in India.

Role of stents in congenital heart disease.

For more than 10 years, balloon-expandable intravascular stents have become an integral part of treatment for obstructive vascular lesions in children and adults with congenital heart disease. The initial problems with stents, such as sharp edges, rigid frame and unacceptable shortening when over-expanded have been overcome to a large extent with the newer designs. The problems related to delivery of stents, such as stent migration, balloon rupture, flaring of the edges of the stent, rupture of vessels and milking of the stent off the balloon, have also been overcome by newer designs of balloons. The failure of growth of balloon-expandable stents with the growth of the child is managed by redilation with or without additional stenting and newer growth stents. Self-expanding stents are not appropriate for use in growing children due to the limitation of their maximum diameters. The development of biodegradable stents may overcome these disadvantages in the future. Various new developments have recently occurred. Covered stents are ideal for treating acute vessel rupture and in isolating vascular aneurysms. Drug-eluting stents may prevent restenosis, but have not been used in children. Valved stents are a recent innovation for the treatment of regurgitant pulmonary valves.

Dilated cardiomyopathy presenting during fetal life.

OBJECTIVES: To describe the echocardiographic features, underlying causes, and outcome of fetuses with dilated cardiomyopathy. DESIGN: A retrospective observational study between 1983 and 2003 at a tertiary centre for fetal cardiology. PATIENTS: Affected fetuses were identified using a computerised database. We included fetuses with dilation and reduced systolic function of either the right ventricle, left ventricle, or both. We excluded fetuses with abnormal cardiac connections, arrhythmias, or stenosis of the aortic or pulmonary valves. In all, we identified 50 fetuses, born to 46 mothers. Of the fetuses, 24 had biventricular cardiomyopathy, 17 had isolated right ventricular cardiomyopathy, and 9 had isolated left ventricular cardiomyopathy. Two-thirds of the fetuses (32) were hydropic at some point during gestation. MAIN OUTCOMES: A cause of cardiomyopathy was identified in 37 cases (74 per cent). This was genetic or metabolic in 11 fetuses; infective in 11; fetal anaemia, without proven parvovirus infection, in 5; of cardiac origin in 5; and an association with renal disease in 5. In 10 cases (20 per cent), the pregnancy was terminated. Based on an intention to treat, the survival to delivery was 25 of 40 (62.5 per cent, 95 per cent confidence intervals from 46 to 77 per cent), at 28 days was 17 of 40 (42.5 per cent, 95 per cent confidence intervals from 27 to 59 per cent), and at 1 year was 15 of 40 (37.5 per cent, 95 per cent confidence intervals from 23 to 54 per cent). The overall survival of non-hydropic fetuses was 9 of 18 (50 per cent), compared to 6 of 32 (18 per cent) hydropic fetuses. CONCLUSIONS: Genetic, metabolic, infective, and cardiac diseases may present with dilated cardiomyopathy during fetal life. There is a high rate of spontaneous intra-uterine and early neonatal death. The prognosis is particularly poor for hydropic fetuses.