Unravelling supravalvular aortic stenosis in a young patient: look beyond the valve.

A 16-year-old adolescent girl was referred to our tertiary care center for management of a failed aortic valve balloon dilatation procedure for congenital valvular aortic stenosis. The medical records revealed a residual peak-to-peak gradient of 80 mm Hg after 2 dilations with 16 x 6-mm Tyshak balloon (NuMed Inc.).

Impasse sign in balloon mitral valvotomy.

A 33-year-old female presented to our out-patient department with worsening shortness of breath over the past 2 years.

Ballerina foot in heart.

A 67-year-old woman with no known risk factors for coronary artery disease presented to the outpatient department with complaints of exertional fatigue and angina New York Heart Association class III.

Transcatheter management of acquired atresia of aorta.

A 24-year-old man was referred to our tertiary care center for the management of uncontrolled hypertension secondary to severe coarctation of aorta.

Bladder indentation sign: A stitch in time can save mine.

A 70-year-old woman with diabetes presented with recurrent episodes of rest angina the previous month. She had undergone coronary angiography, which was suggestive of triple vessel disease.

Recanalized vein of marshall following bidirectional glenn surgery.

A 6-year-old boy with a case of double outlet right ventricle with large non-routable ventricle septal defect and severe pulmonary stenosis was deemed unsuitable for biventricular repair on a prior evaluation. Hence, a bidirectional Glenn (BDG) shunt was performed at 3 years of age following cardiac catheterization.

Novel homozygous leptin receptor mutation in an infant with monogenic obesity.

Monogenic obesity can be caused by a mutation in one of the single genes involved in hunger and satiety. The most common mutations affect melanocortin 4 (MC4) followed by the leptin gene and its receptor. Leptin receptor (LEPR) gene mutation is an extremely rare endocrine disease characterized by early-onset obesity, hyperphagia in addition to pituitary hormone deficiency, and metabolic abnormalities. We report the case of a 12-month-old male infant born of a non-consanguineous marriage. He presented to us with rapid weight gain from 2 months of age along with hyperphagia. Biochemistry revealed a deranged lipid profile, elevated transaminases, and markedly raised serum leptin levels. On genetic analysis, a novel mutation was detected, which was a homozygous variation In exon 12 of the LEPR gene (chr1:g.65608901G>A) that resulted in the synonymous amino acid change of lysine at codon 584 proximal to donor splice site (p.Lys584). The in silico prediction of the variant was 'damaging' by MutationTaster2. The mutation was classified as a 'variant of uncertain significance' due to a lack of published literature and had to be correlated carefully with the clinical symptoms. It was recommended to do Sanger sequencing of the parents and other family members. However, due to financial constraints, the family could not afford the same. At the time of writing, funds were being arranged for procuring setmelanotide, which is a novel and effective therapy for monogenic obesity due to LepR mutation.

Epigenetics of the Pathogenesis and Complications of Type 2 Diabetes Mellitus.

Epigenetics of type 2 diabetes mellitus (T2DM) has widened our knowledge of various aspects of the disease. The aim of this review is to summarize the important epigenetic changes implicated in the disease risks, pathogenesis, complications and the evolution of therapeutics in our current understanding of T2DM. Studies published in the past 15 years, from 2007 to 2022, from three primary platforms namely PubMed, Google Scholar and Science Direct were included. Studies were searched using the primary term 'type 2 diabetes and epigenetics' with additional terms such as 'risks', 'pathogenesis', 'complications of diabetes' and 'therapeutics'. Epigenetics plays an important role in the transmission of T2DM from one generation to another. Epigenetic changes are also implicated in the two basic pathogenic components of T2DM, namely insulin resistance and impaired insulin secretion. Hyperglycaemia-i nduced permanent epigenetic modifications of the expression of DNA are responsible for the phenomenon of metabolic memory. Epigenetics influences the development of micro-and macrovascular complications of T2DM. They can also be used as biomarkers in the prediction of these complications. Epigenetics has expanded our understanding of the action of existing drugs such as metformin, and has led to the development of newer targets to prevent vascular complications. Epigenetic changes are involved in almost all aspects of T2DM, from risks, pathogenesis and complications, to the development of newer therapeutic targets.

Long term safety and efficacy of the Yukon Choice Flex sirolimus-eluting coronary stent-a real-world data from India.

In-stent restenosis and stent thrombosis are the major concerns while choosing a coronary stent. This single-centre, retrospective study evaluated the one and three-year clinical outcomes following implantation of Yukon Choice Flex (YCF) sirolimus-eluting stent. A total of 168 consecutive patients with 217 lesions underwent stenting with YCF stent. The presentation was with acute coronary syndrome in 158 (94%) patients. At 3 years, 9 (5.3%) patients died due to cardiac cause. Myocardial infarction, and definite stent thrombosis occurred in 10 (6%) and 4 (2.4%) patients respectively. Redo stenting and coronary artery bypass surgery was performed in 3 (1.8%) and 1 (0.6%) patient respectively. The use of YCF sirolimus eluting stent was associated with a favourable safety and efficacy profile at one and three-years of follow-up in a high-risk population.

Balloon Rupture During Balloon Mitral Valvotomy.

The reuse of sterilized Inoue catheters is practiced widely in developing countries to bring down the procedure cost. However, blood can enter the space between the latex layers and become embedded in the mesh layer, which is difficult to clean when sterilizing the catheters. This is a common cause of rupture. Proper meticulous inspection of reused Inoue balloons for deformity or leakage through the small holes is necessary to prevent such complications.

Stent Embolization in Spontaneous Coronary Artery Dissection and Its Deployment at the Right Radial Artery.

Spontaneous coronary artery dissection (SCAD) is an unusual but important cause of acute coronary syndrome and is often underdiagnosed. The first clue to the diagnosis is the angiographic appearance of the lesion, and, in certain cases, intravascular imaging is needed to confirm it. Conservative management is the preferred treatment strategy for the majority of cases. However, revascularization is needed in the presence of high-risk features, including hemodynamic instability, ongoing ischemia, and left main dissection. We report a case of a 43-year-old man who presented with acute inferior wall myocardial infarction. Angiogram revealed SCAD of the right coronary artery (RCA). In view of ongoing chest pain, we proceeded with direct stenting. However, during the stent delivery, the stent got embolized and laid unexpanded in the proximal RCA. The stent was successfully retrieved and was deployed at the right radial artery. Subsequently, after the troubleshoot, we again secured wire access across the RCA, and this time after pre-dilatation, successful stenting across the SCAD segment was achieved. Percutaneous coronary intervention (PCI) in SCAD is technically challenging with lower success and higher complication rates compared to atherosclerotic disease. Stent embolization is a potential complication during PCI of SCAD and to the best of our knowledge has never been reported before. Though, in general, the SCAD lesion is soft and one may proceed with direct stenting with long stents, occasionally adequate pre-dilatation may be necessary in order to facilitate the smooth passage of stent across the lesion. Though stent embolization in SCAD is a rare event, the operator must be aware of such a possibility and the potential bailout strategies if faced with such a scenario.

Sudden unexpected improvement in the atrioventricular conduction. What is the mechanism?

The 12-lead electrocardiogram (ECG) of a 79-year-old male patient with recurrent pre-syncope showed irregular sinus rhythm with constant PR interval and left bundle branch block (LBBB) with intermittently blocked P waves. The beat following the blocked P wave had a narrower QRS with a shorter PR interval. The phenomenon of bilateral bundle branch block explains the sudden improvement in the atrioventricular conduction.

Radial Artery Pseudoaneurysm Following Percutaneous Coronary Intervention.

Radial artery pseudoaneurysm is a very rare complication. In the presented case, computed tomography 3 months post percutaneous coronary intervention demonstrated contrast extravasation, suggestive of radial artery pseudoaneurysm. This complication was managed with thrombin infusion at the distal radial artery.

Proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors in aortic stenosis - Is this the light at the end of the tunnel for patients with aortic stenosis?

The exploratory analysis of FOURIER trial has offered a ray of hope for patients with nonrheumatic aortic stenosis (AS). At present, the only definitive treatment of severe AS is aortic valve replacement (AVR). Despite transaortic valvular replacement revolutionizing the treatment of AS, it still remains a progressive condition, with no disease-modifying pharmacotherapy. Angiotensin-converting-enzyme inhibitors, angiotensin receptor blockers, eplerenone, nitrates and statins all have been tried previously but failed to slow down the progression of aortic stenosis. Recently, there has been an emerging role of lipoprotein A [Lp(a)] in the pathogenesis of AS. This raises the possibility that long-term therapy with specific emphasis on Lp(a) reduction may reduce or slow the progression of AS.

Cardiovascular manifestations of COVID-19: An evidence-based narrative review.

The recent outbreak of coronavirus disease 2019 (COVID-19) was declared a pandemic by the World Health Organization on March 11, 2020. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the causative agent of COVID-19, primarily involves the respiratory system with viral pneumonia as a predominant manifestation. In addition, SARS-CoV-2 has various cardiovascular manifestations which increase morbidity and mortality in COVID-19. Patients with underlying cardiovascular diseases and conventional cardiovascular risk factors are predisposed for COVID-19 with worse prognosis. The possible mechanisms of cardiovascular injury are endothelial dysfunction, diffuse microangiopathy with thrombosis and increased angiotensin II levels. Hyperinflammation in the myocardium can result in acute coronary syndrome, myocarditis, heart failure, cardiac arrhythmias and sudden death. The high level of cardiac troponins and natriuretic peptides in the early course of COVID-19 reflects an acute myocardial injury. The complex association between COVID-19 and cardiovascular manifestations requires an in-depth understanding for appropriate management of these patients. Till the time a specific antiviral drug is available for COVID-19, treatment remains symptomatic. This review provides information on the cardiovascular risk factors and cardiovascular manifestations of COVID-19.

An Interesting Case of Acute Asymptomatic Lead Perforation of a Permanent Cardiac Pacemaker.

Acute complications of pacemaker implantation such as lead dislodgement, pneumothorax, and myocardial perforation are not uncommon. Management of these usually requires reintervention. We herein describe lead perforation after a single chamber pacemaker implantation, which was successfully managed conservatively. This case underscores that vigilant monitoring post lead perforation can avoid a redo procedure.

Accidental Cannulation of the Cardiac Venous System During Pericardiocentesis.

A 52-year-old woman presented to another hospital with progressive dyspnea of 10-day duration. The patient was diagnosed with a massive pericardial effusion and underwent pericardiocentesis. However, the patient's symptoms did not improve and she was referred to our hospital with a pericardial sheath in situ. On evaluation, the patient had a large pericardial effusion and evidence of cardiac tamponade, but no fluid could be aspirated from the sheath. This case underscores the importance of image-guided pericardiocentesis.

Giant Aortic Root Aneurysm in Marfan's Syndrome.

A 25-year-old man came to our clinic with the complaints of exertional palpitations and dyspnea. He had skeletal features suggestive of Marfan's syndrome. Contrast-enhanced computed tomography confirmed aneurysmal dilation of the aortic root and the proximal part of the ascending aorta. The patient was advised to undergo Bentall procedure for replacement of the aortic valve, aortic root, and ascending aorta. Marfan's syndrome is a connective tissue disorder with autosomal-dominant inheritance. Patients have a predisposition for progressive aortic root and ascending aortic dilation, and should undergo periodic echocardiographic monitoring.