Decentralization and Integration of Advanced Cardiac Care for the World's Poorest Billion Through the PEN-Plus Strategy for Severe Chronic Non-Communicable Disease.

Rheumatic and congenital heart disease, cardiomyopathies, and hypertensive heart disease are major causes of suffering and death in low- and lower middle-income countries (LLMICs), where the world's poorest billion people reside. Advanced cardiac care in these counties is still predominantly provided by specialists at urban tertiary centers, and is largely inaccessible to the rural poor. This situation is due to critical shortages in diagnostics, medications, and trained healthcare workers. The Package of Essential NCD Interventions - Plus (PEN-Plus) is an integrated care model for severe chronic noncommunicable diseases (NCDs) that aims to decentralize services and increase access. PEN-Plus strategies are being initiated by a growing number of LLMICs. We describe how PEN-Plus addresses the need for advanced cardiac care and discuss how a global group of cardiac organizations are working through the PEN-Plus Cardiac expert group to promote a shared operational strategy for management of severe cardiac disease in high-poverty settings.

Correction: Decentralization and Integration of Advanced Cardiac Care for the World's Poorest Billion Through the PEN-Plus Strategy for Severe Chronic Non-Communicable Disease.

[This corrects the article DOI: 10.5334/gh.1313.].

Rheumatic heart disease in school-attending Nepalese children: A descriptive analysis of the national heart screening database.

OBJECTIVE: Rheumatic Heart Disease (RHD) remains a significant public health problem with high morbidity and mortality in children and young adults from lower-middle income countries like Nepal. However, a nation-wide database of the disease is lacking for designing effective future prevention and control programmes and strategies. The aim of our study is to estimate the prevalence of RHD in school-attending Nepalese children. METHODS: We performed a cross-sectional descriptive analysis of a nationally representative database of Nepal Heart Foundation (NHF) national RHD screening programme which included school-attending Nepalese children between five and sixteen years of age. The screening was conducted between May 2015 and March 2020 in 236 schools, representing all seven provinces, across all three ecological zones of Nepal. Transthoracic two-dimensional echocardiography was performed in all eligible children with more than grade one murmur on cardiac auscultation. We estimated the prevalence of RHD among school-attending children as the number of RHD cases per 1000 school-attending children with a 95% confidence interval. RESULTS: The database included a total of 107,340 children who were screened clinically, of whom 10,600 (9·9%) underwent transthoracic two-dimensional echocardiography. The overall prevalence of RHD was 2.22 cases per 1000 school-attending children (95% CI:1·94 - 2·50). The highest prevalence was observed among children living in the southern Terai ecological zone (2·89 per 1000, 95% CI (2·32-3·46)) of Nepal. Among the provinces, Karnali had the highest prevalence of RHD (3·45 per 1000, 95% CI (2·42-4·48)). Among the districts screened, Kalikot had the highest RHD prevalence (5.47 per 1000, 95% CI (3.02-7.92)). CONCLUSION: Primordial, primary and secondary prevention programmes should pay special attention to southern Terai zone, particularly the under-privileged children from remote districts.

Quality of Life, Anxiety and Depression among Clients with Ostomy Attending Selected Stoma Clinics.

BACKGROUND: Ostomy is a part of treatment among patients which has detrimental effects in patient's life. The main aim is to identify the quality of life, anxiety and Depression in clients with an ostomy. METHODS: Descriptive exploratory study design was used. Total of 116 clients with ostomy, aged 18 years and above were selected from stoma clinics. The modified version of City of Hope and Beckman Research Institute, Quality of Life Questionnaire for Patient with Ostomy and Hospital Anxiety and Depression Scale was adopted for data collection using an interview technique. RESULTS: The overall mean ± S.D quality of life score was 5.89 ± 1.34. Majority (59.5%) of respondents possessed low Quality of life. Among the quality of life domains, the least and most affected domains were physical (5.96±1.52) and social (4.71±1.44) respectively. Duration of having an ostomy (p<0.001), problem in clothing (p<0.002) and change in clothing style (p= 0.002) were significantly associated with the level of quality of life. Almost two-thirds of the respondents were in the borderline and abnormal level of anxiety and depression. The level of anxiety has significant association with suicidal consideration/attempt (p=.04). CONCLUSION: Presence of ostomy affects patient's quality of life by increasing financial burden, adjustment difficulties, sexual and psychological problems (anxiety, depression, suicidal consideration). Sexual and psychological counselling, ostomy support groups and free health services to ostomates may improve their quality of life.

First-episode versus recurrent acute rheumatic fever: is it different?

BACKGROUND: Recurrent episodes of acute rheumatic fever (RF) can lead to rheumatic heart disease with considerable disability and mortality in children. RF can recur in the absence of secondary prophylaxis. The differences in clinical manifestations and outcome between first-episode and recurrent RF have been less studied. METHODS: A cross-section of patients under 14 years was studied for 2 years (2003-2005) in order to compare the clinical, laboratory, echocardiographic profile and outcome of first-episode RF with recurrent attacks, and risk factors for recurrence and mortality. Patients without a previous history of RF and/or mitral stenosis (MS) and/or aortic stenosis (AS) were defined as first-episode patients, and patients with previous history of RF and/or MS and/or AS, were defined as recurrent RF patients based on the Jones criteria. RESULTS: Of 51 patients in total, 26 had first-episode RF and 25 had recurrent RF. Arthritis occurred in a significantly higher number of first-episode patients (P = 0.047) whereas shortness of breath (SOB; P = 0.003), palpitation (P = 0.034), and aortic regurgitation (AR; P = 0.001) occurred in a significantly higher number of recurrent RF patients. Audible murmur of corresponding echocardiographic regurgitation was present in all recurrent RF patients whereas audible murmur was present in 61.5% and echocardiographic regurgitation in 81% in first-episode patients (P = 0.007). Palpitation, SOB, audible murmur, thrill, age and AR on admission were independent predictors of recurrence. Palpitation, age and AS on admission were independent predictors of mortality. CONCLUSIONS: Subclinical carditis occurred only in the first-episode patients, which requires further evaluation for clinical significance. Because all deaths occurred in recurrent RF group (P = 0.02), secondary prophylaxis and management of sore throat need re-emphasis.

Primary surgical repair of coarctation of the aorta in adolescents and adults: intermediate results and consequences of hypertension.

OBJECTIVES: Coarctation of the aorta is known to present with hypertension in older patients; we reviewed our experience and assessed the outcome of hypertension following surgical correction. METHODS: From April 2004 to date, 43 patients above the age of 12 underwent coarctation of the aorta repair. The mean age was 20.4 + 9.7 years (maximum 56 years); 21 (48.8%) were older than 18 years and 28 (65.1%) were men. Thirty (69.8%) patients had hypertension. Fourteen (32.6%) had a bicuspid aortic valve; 11 (25.6%) had patent ductus arteriosus; 6 (14%) had myxomatous mitral valve; 4 (9.3%) had ascending aortic aneurysms; and 2 (4.7%) had descending aneurysms. RESULTS: Surgical correction included resection and interposition of a tube graft in 31 (72.1%), an end-to-end anastomosis in 6 (14%) and patch aortoplasty in 3 (7%). Three (7%) patients required an extra-anatomical bypass: 1 had a long segment coarctation of the aorta, and 2 had a Bentall procedure with an ascending-to-descending aortic bypass. Staged procedures were done for concomitant disease in 4 (9.3%). There was 1 death: a 56-year-old woman died of refractory ventricular fibrillation during surgery. Thirty (69.8%) patients were discharged with antihypertensive medication. At a follow-up of 2.8 ± 2.2 years (maximum 9.2 years), the number of hypertensive patients decreased (17/36; 47.2%) (P = 0.042). Univariable predictors for persistence of hypertension revealed the use of an interpositional tube graft for repair (odds ratio 13.855, confidence interval 0.000-0.001; P = 0.001) as an indicator, whereas there were no independent predictors for persistence of hypertension. CONCLUSIONS: Surgical intervention is warranted irrespective of age and helps correct and control hypertension better; however, significant numbers of patients still require antihypertensive medication and regular monitoring. Intervention using an interposition tube graft may affect the prevalence of hypertension.

Lipoblastoma: an unusual tumour of the left ventricle.

Lipoblastoma is a rare benign tumour of infancy originating from white foetal adipose tissue. Most commonly located in the extremities, intrathoracic and mediastinal involvement of this tumour is rare, and an intracardiac location is even rarer, with only one reported case. Herein, we present a 2-month old asymptomatic boy diagnosed with an echogenic mass in the left ventricular outflow tract. The patient underwent surgical excision and histopathological evaluation revealed a lipoblastoma.

Percutaneous transvenous mitral commissurotomy in juvenile mitral stenosis.

BACKGROUND: Percutaneous transvenous mitral commissurotomy (PTMC) is a valid alternative to surgical therapy in selected patients with mitral stenosis. Juvenile mitral stenosis (JMS) varies uniquely from adult rheumatic heart disease (RHD). We aimed to evaluate the efficacy of PTMC in JMS patients. METHODS: It was a single centre, retrospective study conducted between July 2013 to June 2015 in Shahid Gangalal National Heart Centre, Kathmandu, Nepal. Medical records of all consecutive patients aged less than 21 years who underwent PTMC were included. Mitral valve area (MVA), left atrial pressure and mitral regurgitation (MR) were compared pre and post procedure. RESULTS: During the study period 131 JMS patients underwent PTMC. Seventy (53.4%) were female and 61 (46.6%) were male. Among the 131 patients, 40 (30.5%) patients were below the age of 15 years. Patient age ranged between 9 to 20 years with the mean of 16.3±2.9 years. Electrocardiography (ECG) findings were normal sinus rhythm in 115 (87.7%) patients and atrial fibrillation in 16 (12.3%) patients. Left atrial size ranged from 2.9 to 6.1 cm with the mean of 4.5±0.6 cm. The mean MVA increased from 0.8±0.1 cm(2) to 1.6±0.2 following PTMC. Mean left atrial pressure decreased from their pre-PTMC state of 27.5±8.6 to 14.1±5.8 mmHg. Successful results were observed in 115 (87.7%) patients. Suboptimal MVA <1.5 cm(2) in 11 (8.4%) patients and post-procedure MR of more than moderate MR in 5 (3.8%) patients was the reason for unsuccessful PTMC. CONCLUSIONS: PTMC in JMS is safe and effective.

Clinical, laboratory and echocardiographic profile of acute rheumatic fever in Nepali children.

BACKGROUND: Acute rheumatic fever (RF) is a common, preventable health problem in developing countries. Sporadic outbreaks and the prevalence in some indigenous populations have renewed interest in RF in developed countries also. AIMS: To describe the clinical, laboratory and echocardiographic features, outcome and value of echocardiography in detecting valvular disease in RF. METHODS: A prospective, cross-sectional study was conducted over 2 years. Patients under 14 years admitted to the cardiology unit of Kanti Children's Hospital, Kathmandu with RF using the Jones criteria were recruited consecutively. RESULTS: The median age (range) of the 51 patients was 11 (5-14) years, the male:female ratio was 1.6:1 and 39% had a history of a sore throat. Clinical and laboratory features detected were as follows: carditis 92%, arthritis 33%, chorea 8%, subcutaneous nodules 4%, fever 51%, arthralgia 37%, elevated antistreptolysin O titre 94%, elevated CRP 78%, prolonged PR interval 45%, pericardial effusion 22% and cardiac failure 28%. In total, 36 patients (71%) complained of joint pains. A murmur on auscultation was significantly associated with underlying diseased valves confirmed by echocardiography (p=0.001). A murmur was audible in 78.4% and diseased valves were confirmed by echocardiography in 88.2%. The mitral valve was the most commonly involved valve (82%) and mitral regurgitation the commonest lesion (24%). A thickened mitral valve predicted carditis (p=0.007). Five (10%) patients died. CONCLUSION: Inclusion of echocardiographic evidence of carditis and possibly arthralgia as major criteria would improve case detection.