Spectrum of cyanotic congenital heart disease diagnosed by echocardiographic evaluation in patients attending paediatric cardiology clinic of a tertiary cardiac care centre.

BACKGROUND: Cyanotic CHD comprises up to 25% of cases of all causes of CHD. RATIONALE: There is lack of data about the present spectrum of congenital cyanotic heart disease in the paediatric age group. OBJECTIVE: The present study was undertaken to determine the spectrum of patients with congenital cyanotic heart disease in the paediatric age group in tertiary paediatric cardiac care clinic. DESIGN: Prospective observational study. SETTING: Paediatric cardiac clinic of a tertiary cardiac care centre. METHODS: All children aged 0-18 years with suspected cyanotic CHD were provisionally included in this study. They underwent a thorough echocardiographic evaluation, and those patients who had definitive diagnosis of congenital cyanotic heart disease were included for final analysis. RESULTS: A total of 119 children met the inclusion criteria. Tetralogy of Fallot and its variant were the most common congenital cyanotic heart disease with proportion of about 44%. Other common malformations were double outlet right ventricle (14%), pulmonary atresia with ventricular septal defect (8%), total anomalous pulmonary venous connection (7%), d-transposition of the great arteries (9%), tricuspid valve anomalies--tricuspid atresia and Ebstein's anomaly--hypoplastic left-heart syndrome, truncus arteriosus, and complex CHD such as single ventricle. CONCLUSION: Tetralogy of Fallot and its variants were the most common cyanotic heart disease diagnosed in our patients. As there were a significant proportion of cases with complex cyanotic CHD, paediatric cardiologists should be familiar with the diagnosis and management of all these complex congenital malformations of the heart.

Large unruptured sinus of valsalva aneurysm dissecting into interventricular septum and presenting as a complex myocardial cystic mass.

Unruptured aneurysm of sinus of Valsalva (SOV) is extremely rare. They may be asymptomatic and diagnosed incidentally or can present with either fatal outcome following rupture or manifest acutely with compression of surrounding structures. Dissection of unruptured aneurysm of left SOV into interventricular septum (IVS) and presenting as intramyocardial mass is not reported in the literature. We are reporting a case of 21-year-old man who was referred to us following a seizure episode and was incidentally diagnosed with aneurysm of left SOV dissecting into IVS. Multimodality imaging confirmed the diagnosis. He successfully underwent surgical repair and was asymptomatic during follow-up.

Persistent left superior vena cava draining into left atrium as a cause of persistent systemic desaturation after surgery.

Persistent left superior vena cava (LSVC) is a rare congenital anomaly which usually produces no physiologic derangements if it drains into the right atrium via the coronary sinus, but it may cause significant desaturation when it drains into the left atrium (LA). Failure to diagnose LSVC communicating with the LA preoperatively may lead to serious consequences. We are describing an interesting case of a boy who presented with systemic desaturation due to an undetected LSVC after having undergone corrective surgery for atrioventricular canal defect. We have demonstrated that echocardiography with agitated saline contrast is a simple, accurate, and inexpensive diagnostic modality.

An atypical presentation of retinal astrocytic hamartoma with co-occurring SEGA in a tuberous sclerosis patient.

Tuberous sclerosis complex (TSC) is a genetic disorder that causes noncancerous growths and tumors in various body systems. This case report discusses an unusual eye condition called retinal astrocytic hamartoma (RAH) in a TSC patient, along with a brain tumor called subependymal giant cell astrocytoma (SEGA). These conditions, linked to TSC gene mutations, can be atypical in size and cause complications like vitreous hemorrhage. Surgical treatment is generally effective. Recognizing similarities between SEGA and RAH can help with early detection and comprehensive care for TSC patients.

Incidence of Coronary Artery Disease After Permanent Pacemaker Implantation: A Hospital-based Study from East India.

Bradyarrhythmias, characterized by heart rates of <60 bpm due to conduction issues, carry risks of sudden cardiac death and falls. Pacemaker implantation is a standard treatment, but the interplay between bradyarrhythmias, coronary artery disease (CAD), and patient attributes requires further exploration. This study was a retrospective hospital record-based study that analyzed data from 699 patients who underwent pacemaker implantation for symptomatic bradyarrhythmias between February 2019 and February 2022. Clinical parameters, coronary angiography (CAG) findings, ejection fraction, and indications for pacemaker implantation were documented. The relationship between CAD severity, specific bradyarrhythmias, and ejection fraction was explored. Statistical analysis included chi-squared tests and t tests. The mean age of the study population (n = 699) was 66.75 years (male:female ratio, 70:30), with 77.2% having type 2 diabetes and 61.6% being hypertensive. The majority of patients had minor or non-obstructive CAD (61.8%), followed by normal CAG findings (25.75%) and obstructive CAD (12.45%). Complete heart block (CHB) was the primary indication for pacemaker implantation (55.2%), followed by sick sinus syndrome (22.3%). The results did not show any association between ejection fraction and CAG findings. Patients who presented with CHB had a higher incidence of obstructive CAD, indicating greater severity. This study sheds light on the intricate interplay between severe bradyarrhythmias, CAD, and patient characteristics. Our analysis revealed no statistical significance between obstructive CAD and the need for a permanent pacemaker. This makes us question our practice of maintaining a low threshold for coronary angiography during pacemaker implantation. The observed low yield and anticoagulation protocol reassure us of the choice to delay this diagnostic intervention. These insights can guide tailored management strategies, enhancing clinical care approaches for patients with severe bradyarrhythmias necessitating pacemaker implantation.

An adult patient of double-chambered right ventricle with ventricular septal defect incidentally diagnosed after presentation with acute myocardial infarction.

The double-chambered right ventricle (DCRV) is a rare congenital heart disease caused by hypertrophic anomalous muscle bands that divide the ventricular cavity, resulting in the formation of a high-pressure proximal chamber and other low-pressure distal one. Because of its evolving nature, its diagnosis is usually made during childhood/adolescence and very rarely during adulthood. It accounts for approximately 0.5-1% of patients with congenital heart disease. We report a case of a 50-year-old man who presented to us with acute inferior wall myocardial infarction (MI). Echocardiography revealed the features of DCRV and ventricular septal defect. Coronary angiogram revealed significant stenosis in right coronary artery. This is the first case where an adult patient with DCRV incidentally diagnosed after presentation with acute MI and had undergone successful coronary angioplasty.

A Rare Case of Trifoliate Gallbladder in an Adult Male and Its Management: A Comprehensive Case Report and Literature Review.

Triple gallbladder, a rare congenital abnormality resulting from the incomplete regression of rudimentary bile ducts, often goes undetected until incidentally discovered during imaging studies. This report presents the case of a 38-year-old male with intermittent abdominal pain, food intolerance, and nausea. Physical examination findings included tenderness in the right hypochondrium and a positive Murphy's sign. Laboratory tests revealed leukocytosis and elevated liver enzymes, while abdominal ultrasound identified three fluid-filled sacs in the expected gallbladder fossa location, with one showing cholelithiasis and mild wall thickening in all three. Further evaluation via magnetic resonance cholangiopancreatography (MRCP) unveiled three incompletely separated tubular cystic structures draining into a solitary cystic duct, confirming the diagnosis as trifoliate gallbladder type 3. This case highlights the importance of considering rare entities like triple gallbladder in the differential diagnosis of biliary pathologies, necessitating a high index of suspicion and comprehensive imaging to ensure accurate diagnosis and appropriate management, as exemplified by successful laparoscopic cholecystectomy.

Acute Psychosis and Mania: An Uncommon Complication of Dengue Fever.

Dengue fever is primarily known as a mosquito-borne viral infection that causes a range of physical symptoms, ranging from myalgia to bleeding tendencies. However, there is growing recognition of its potential to trigger psychiatric manifestations, although such cases remain relatively rare. We report a case of acute dengue fever in a 25-year-old male who developed mania and psychotic symptoms after one week of infection. A comprehensive diagnostic workup, including laboratory tests, including cerebrospinal fluid analysis, and neuroimaging, confirmed the absence of organic causes for his psychiatric symptoms, except for his prior exposure to the dengue virus. The patient was initiated on mood stabilizers and antipsychotic medications, leading to a gradual improvement in his mental health.

Factors in the Development of Somatoform Disorders Among Children: A Case-Control Study.

Introduction Somatoform disorders (SFDs) are a spectrum of diseases mainly manifesting with physical symptoms of no recognizable etiology. These disorders are believed to be primarily influenced and exacerbated by psychological factors. Given the connection between parental sociodemographics and psychological factors and SFDs, there is a pressing need to investigate this area further, particularly concerning parents and their children affected by somatoform disorders. Aims and objectives The aims and objectives of this study are as follows: i) study the determinants of SFDs, namely, parent handling of child, parent-child relationship, parenting with respect to attending to the needs of children, and intelligence quotient (IQ) of parents, and ii) compare host factors to the factors matched in control subjects. Materials and methods We adopted purposive sampling in our case-control study. The study sample was obtained from the psychiatry department of the Ganesh Shankar Vidyarthi Memorial (GSVM) Medical College, Kanpur, India, from September 2020 to December 2022, once a week, every Monday. Children manifesting SFD manifestations that are among the chief complaints were included in our study. Results A total of 115 cases were included in our study based on inclusion criteria. The study compared sociodemographic characteristics, intelligence quotient (IQ), parental characteristics, parental handling, parent-child relationships, and parenting practices between a patient group and a control group. The results showed no significant differences in sex, religion, occupation, domicile, and socioeconomic status in both control and patient groups. However, significant differences were found in parental characteristics, such as lower mean age, education, and IQ, in the patient group. This difference between the patient and control groups with respect to the parental handling questionnaire was statistically significant for the domain of "praise," "talk," "feel better," "comes to you," "unduly strict" items, "frequently reprimanded," "tried to control everything," creative activities, protectiveness, education, neglecting, objective punishment, housing, medical care, demanding, symbolic reward, loving, objective reward, food, parent-to-child communication, clothing, support, routine, recreation, social activities, rules, managing problem behavior, guidance for career, and security. Conclusion Parents were deficient in terms of years of education, occupational status, IQ, parental handling, parent-child relationship, and parenting with respect to the children's needs. These findings offer insights into the sociodemographic and psychological factors contributing to the patient group's condition.

Neurological Wilson's disease with refractory rickets.

Wilson's disease rarely presents with isolated neurological complaints without any hepatic involvement. Refractory rickets with Wilson's disease has been infrequently reported in literature. We are reporting a case of isolated neurological Wilson's disease associated with refractory rickets which on complete evaluation was diagnosed as familial hypophosphatemic rickets.

Idiopathic Fanconi's syndrome with nephrogenic diabetes insipidus in a child who presented as vitamin D resistant rickets--a case report and review of literature.

Fanconi's syndrome is a complex of multiple tubular dysfunctions of proximal tubular cells occurring alone or in association with a variety of inherited (primary) or acquired (secondary) disorders. It is characterized by aminoaciduria, normoglycemic glycosuria, tubular proteinuria without hematuria, metabolic acidosis without anion gap and excessive urinary excretion of phosphorous, calcium, uric acid, bicarbonate, sodium, potassium and magnesium. Diabetes insipidus is a disease of collecting tubules and a child mainly presents with dehydration and hypernatremia. We report the first case of idiopathic Fanconi's syndrome along with nephrogenic diabetes insipidus (NDI) in a child who presented to us as resistant rickets. Medline search did not reveal any case of nephrogenic diabetes insipidus associated with idiopathic Fanconi's syndrome. We hypothesized that the NDI may be due to severe hypokalemia induced tubular dysfunction. The child was treated for hypophosphatemic rickets with severe metabolic acidosis and the treatment for NDI was also given. Now he has healed rickets and normal blood pH, sodium and osmolarity.

Diagnostic modalities for gastro-esophageal reflux in infantile wheezers.

OBJECTIVE: To compare the diagnostic value of Gastro-intestinal (GI) scintiscan with 24-h pH study in detecting gastro-esophageal reflux (GER) among infantile wheezers. SUBJECTS: Fifty-two children < 2 years of age. METHODS: All patients, irrespective of symptom underwent study to evaluate for GER. RESULTS: GER studies were positive in almost 45% of cases. The agreement between positivity of these two tests is best among children between 7 and 12 months of age (κ = 0.591, p = 0.002). Overall GI scintiscan was a better test with higher sensitivity and specificity as compared to 24-h pH study when compared with the history suggestive of reflux and clinical response with anti-reflux treatment as standard (p ≤ .001). CONCLUSION: Both GER scan and 24-h pH study are complimentary to each other, however, if both the tests are available then GI scintiscan is better as a single test for GER in these early wheezers.

Percutaneous coronary intervention in chronic total occlusion of anomalous right coronary artery: an onerous journey.

Chronic total occlusion (CTO) of coronary artery is a subset where cardiologists confront technical challenges most of the time during percutaneous coronary intervention (PCI). They experience an additional impediment when CTO intervention is performed in presence of anomalous coronary artery. Here we report a 58 years old gentleman with Diabetes mellitus and hypertension who admitted with Canadian Cardiovascular Society class III angina for 5 months. Coronary angiography revealed dual vessel coronary artery disease with CTO of right coronary artery (RCA) originating from left sinus of Valsalva. PCI with stenting has been done successfully to RCA lesion. Patient got relieved of angina after PCI and remains stable clinically since then.

Prevalence of Cardiovascular Risk Factors among Executive and Nonexecutive Workers in an Urban Public Sector Office Setting: A Cross-Sectional Epidemiological Study from Eastern India.

AIM: The aim of the study is to determine the prevalence of cardiovascular risk factors among executive and nonexecutive workers in an urban public sector office setting. METHODS: A prospective, cross-sectional survey of employees in a public sector office in Eastern India was done using a structured questionnaire to collect data on demographic and lifestyle details and health conditions. Clinical examination, anthropometric measurements, blood sugar, and lipid levels were measured. The employees were divided as executives and nonexecutives based on whether they held gazetted or nongazetted posts. RESULTS: A total of 502 participants were surveyed - 140 executives and 362 nonexecutives; majority were male (88.23%). The executive group had a significantly greater number of participants with older age, hypertension (57.9% vs. 39%), and overweight (40% vs. 30.6%) than the nonexecutive group. Significantly, more nonexecutives had a physically active lifestyle and relatively less presence of conventional cardiovascular risk factors such as tobacco use, hypertension, diabetes, hypercholesterolemia, and weight. The prevalence of ≥3 cardiovascular risk factors was significantly high in executives (27.9%) as compared to nonexecutives (14.1%). CONCLUSION: A higher prevalence of mostly lifestyle-related modifiable cardiovascular risk factors was seen among the executives in an urban public sector office setting in Eastern India.

The emerging role of gut microbiota in cardiovascular diseases.

There is mounting evidence which suggests the involvement of gut microbiota dysbiosis in the pathogenesis of various cardiovascular diseases (CVD) and associated risk states such as hypertension, type 2 diabetes, obesity and dyslipidaemia, atherosclerosis, heart failure and atrial fibrillation. The current review comprehensively summarizes the various pathogenetic mechanisms of dysbiosis in these conditions and discusses the key therapeutic implications. Further deeper understanding of the pathogenetic links between CVD and gut microbiota dysbiosis can aid in the development of novel microbiota-based targets for the management of CVDs.

Left bundle branch pacing in hypertrophic cardiomyopathy-a novel approach.

Symptomatic bradycardia attributed by sick sinus syndrome in hypertrophic cardiomyopathy (HCM) is not commonly seen. Dual chamber pacing with right ventricular apical lead placement is conventional strategy in such scenario. Now physiological pacing which includes left bundle branch (LBB) pacing emerging as new technology for pacemaker implantation. Use of this technique is difficult in HCM due to septal hypertrophy. There is no such case reported so far in the literature where LBB pacing was performed in adult HCM for sick sinus syndrome. Here we present a novel approach of treating irreversible, symptomatic sinus node dysfunction in non-obstructive HCM with implementation of left bundle pacing strategy. Pacing parameters remain stable after 3 months of follow-up.

Cardiac catheterization through ipsilateral radial and ulnar artery access during the same procedure.

We evaluated the safety and feasibility of ipsilateral radial and ulnar artery cannulation during the same catheterization procedure. Crossover from radial to femoral was done in 122 patients. Both ipsilateral radial and ulnar catheterization were performed in 16 patients without any complications, which was further supported by Doppler ultrasonography.

Case series of neonatal hypocalcemia due to pseudohypoparathyroidism.

Four children; all between 10-40 days of age, were admitted with multiple episodes of multifocal seizure. After admission blood investigation was carried out and they were found to have hypocalcemia, hyperphosphatemia; kidney function tests were normal. Serum parathyroid hormone was highly elevated and serum magnesium level was found to be normal in these patients. The infants did not have any other features of pseudohypoparathyroidism. The seizure was initially resistant to intravenous calcium therapy and later responded to oral calcitriol and calcium therapy. The diagnosis of pseudohypoparathyroidism was made according to biochemical investigations. The infants were symptom-free during follow-up and the biochemistry reports were also normalized. One of the infants was also suffering from neonatal hepatitis, due to CMV infection.

Ischemic mitral regurgitation: the way ahead is a step back.

Ischemic mitral regurgitation (IMR) has a profound negative effect on survival of patients following myocardial infarction. It occurs when the closing forces are overpowered by the tethering forces as a consequence of ventricular remodeling. Surgeons sought to correct moderate and severe IMR by mitral annuloplasty. Though short-term results were encouraging, survival after 2 years were not so. Higher recurrence rates were also noted with severe mitral regurgitation (MR). Parameters defining severity of IMR were initially formulated in 2003. These were revised enabling intervention in moderate MR in 2014. With the lack of positive medium and long-term evidence, 2017 guidelines have raised the bar, discouraging intervention in moderate IMR. Current guidelines have taken a conservative stance in advocating repair only for severe MR and very symptomatic patients. Till emergence of fresh evidence surgical enthusiasm for repair of IMR has to be restrained.