Neurogenic myocardial dysfunction post craniopharyngioma resection: A diagnostic dilemma.

Endoscopic transsphenoidal resection of craniopharyngioma is a commonly used technique. Cerebral vasospasm may occur in nearly 10% of cases leading to adverse neurological outcomes. Cardiopulmonary dysfunction may be seen in patients with severe vasospasm. The literature describing the occurrence of neurogenic stunned myocardium following craniopharyngioma resection in pediatric patients is very sparse. Here, we describe such a case managed with a combination of milrinone (to relieve vasospasm and improve cardiac pump function), noradrenaline (to obtain target blood pressure), and vasopressin (to control urine output). This case report proposes the treatment plan of neurogenic stunned myocardium following vasospasm in pediatric patients.

Cirrhotic cardiomyopathy influences clinical outcomes and enhances performance of conventional risk prediction models in acute-on-chronic liver failure with severe sepsis.

BACKGROUND: Point-of-care echocardiography (POC-Echo) is an essential intensive care hemodynamic monitoring tool. AIMS: To assess POC-Echo parameters [i.e., cardiac index (CI), systemic vascular resistance index (SVRI) and cirrhotic cardiomyopathy (CCM) markers] and serum biomarkers in predicting circulatory failure (need for vasopressors) and mortality in patients with acute-on-chronic liver failure (ACLF) having sepsis-induced hypotension. METHODS: We performed serial POC-Echo within 6 hours (h) of presentation and subsequently at 24, 48 and 72 h in patients with ACLF and sepsis-induced hypotension admitted to our liver intensive care unit. Clinical data, POC-Echo data and serum biomarkers were collected prospectively. RESULTS: We enrolled 120 patients [59% men, aged 49 ± 12 years, 56% alcohol-related disease and median MELDNa of 30 (27-32)], of whom 68 (56.6%) had circulatory failure, with overall mortality of 60%. CCM was present in 52.5%. The predictors of circulatory failure were CI (aHR -1.5; p = 0.021), N-terminal brain natriuretic peptide (aHR -1.1; p = 0.007) and CCM markers; e' septal mitral velocity (aHR -0.5; p = 0.039) and E/e' ratio (aHR -1.2; p = 0.045). Reduction in CI by 20% and SVRI by 15% at 72 h predicted mortality with a sensitivity of 84% and 72%, and specificity 76% and 65%, respectively (p < 0.001). The MELD-CCM model and CLIF-CCM model were computed as MELDNa + 1.815 × E/e' (septal) + 0.402 × e' (septal) and CLIF-C ACLF + 1.815 × E/e' (septal) + 0.402 × e' (septal), respectively, based on multivariable logistic regression. Both scores outperformed MELDNa (z-score = -2.073, p = 0.038) and CLIF-C ACLF score (z score = -2.683, p-value = 0.007), respectively, in predicting 90-day mortality. CONCLUSION: POC-Echo measurements such as CCM markers (E/e' and e' velocity) and change in CI reliably predict circulatory failure and mortality in ACLF with severe sepsis. CCM markers significantly enhanced the CLIF-C ACLF and MELDNa predictive performance.

Percutaneous coronary intervention of anomalous right coronary arteries arising from the left sinus of Valsalva: a single-centre experience from Northern India.

Background: An anomalous right coronary artery (ARCA) arising from the left sinus of Valsalva is an uncommon congenital anomaly. The unusual location, take-off and intramural courses of ARCA pose a considerable technical challenge during percutaneous coronary intervention (PCI). Aims: We sought to report our experience of PCI of ARCA in 35 cases of atherosclerotic occlusion. Methods: The PCI database of 35 cases of ARCA was retrospectively analysed. The details about demography, clinical presentation, PCI procedure and clinical follow-up were noted. Results: The mean age was 56.7±13.5 years. The clinical presentation included stable angina in 45.7%, unstable angina in 20% and acute myocardial infarction in 34.3% of patients. Vascular access was transradial in 37.1% of cases, transfemoral in 60% of cases, and transbrachial in 1 case (2.9%). Judkins left and Amplatz left were commonly used guide catheters. Two patients with balloon-uncrossable, calcified lesions required rotablation-assisted PCI. Intravascular imaging was performed in 12 patients (34.3%). Optical coherence tomography demonstrated a proximal intramural course in 3 patients, and a slit-like orifice in 4 patients. The mean radiation dose and fluoroscopy time were 877±687.3 mGy and 18.8±11.6 minutes, respectively. Twenty-eight (80%) patients had an asymptomatic median follow-up of 49 (interquartile range: 29.0-97.5) months. Conclusions: We performed successful PCI in a cohort of 35 patients with ARCA, with favourable long-term clinical outcomes. The selection of an appropriate guide catheter and technical skills were important factors in achieving favourable results.

Strut Inversion During Valve-in-Valve Transcatheter Aortic Valve Replacement: An Unknown Complication?

A 74-year-old man presented with failure of a bioprosthetic aortic valve implanted 7 years earlier, with a mean gradient of 44 mm Hg across the aortic valve. During valve-in-valve transcatheter aortic valve replacement, we came across an unusual complication of strut inversion at the lower end of the valve. (Level of Difficulty: Advanced.).

Cardiac MRI and Echocardiography for Early Diagnosis of Cardiomyopathy Among Boys With Duchenne Muscular Dystrophy: A Cross-Sectional Study.

Background: Cardiomyopathy is an important cause of morbidity and mortality in boys with Duchenne muscular dystrophy (DMD). Early diagnosis is a prerequisite for timely institution of cardioprotective therapies. Objective: We compared cardiac MRI (CMRI) with transthoracic echocardiography (TTE) including tissue Doppler imaging (TDI) and speckle tracking echocardiography (STE) for diagnosis of cardiomyopathy in early ambulatory boys with DMD. Methodology: This cross-sectional study was conducted between June 2018 and December 2020. Consecutive boys between 7 and 15 years of age with DMD were enrolled. Percentage ejection fraction (EF), fractional shortening, wall motion abnormalities, early diastolic mitral annulus velocity (Ea), medial mitral annulus ratio (E/Ea), and global strain were measured with STE. CMRI-derived EF, segmental hypokinesia, and late gadolinium enhancement (LGE) were studied and compared. Results: A total of 38 ambulatory boys with DMD were enrolled. The mean age was 8.8 ± 1.6 years, and none had clinical features suggestive of cardiac dysfunction. In the TTE, EF was ≤55% in 5 (15%), FS was ≤28% in 3 (9%), and one each had left ventricular wall thinning and wall hypokinesia. In TDI, none had diastolic dysfunction, and STE showed reduced global strain of < 18% in 3 (9%) boys. CMRI-derived EF was ≤55% in 20 (53%) boys and CMRI showed the presence of left ventricular wall hypokinesia in 9 (24%) and LGE in 4 (11%) boys. Conclusion: Cardiomyopathy remains clinically asymptomatic among early ambulatory boys with DMD. A significantly higher percentage of boys revealed early features of DMD-related cardiomyopathy in CMRI in comparison with echocardiography.

Ortner's Syndrome Due to Giant Thoracic Aortic Aneurysm.

Ortner's syndrome is hoarseness of the voice caused by enlarged cardiac structures compressing the left recurrent laryngeal nerve (LRLN). The LRLN is vulnerable for impingement by cardiac structures due to its anatomical course. LRLN is longer and it hooks below the arch of the aorta, posterior to the ligamentum arteriosum before ascending toward the tracheo- esophageal groove. Ortner's syndrome has been commonly described with left atrial enlargement due to mitral stenosis; however, other causes like aortic aneurysm, pulmonary artery aneurysm, and aortic dissection have been described. Although rare, hoarseness of the voice could be the only presenting complaint in aortic aneurysms as in the case described herein.

Abnormal blood supply to a normal lung: left circumflex artery branch supplying the right lung.

A middle-aged woman with hypertension presented with atypical chest pain of 1 month duration and had a positive exercise stress test. She underwent diagnostic coronary angiography which demonstrated an anomalous branch arising from the proximal part of the left circumflex artery supplying the right lung. She had atherosclerotic plaques in the right coronary artery and left anterior descending artery. Stress myocardial perfusion imaging did not reveal any inducible ischaemia in the left circumflex artery territory. She was started on medical therapy for coronary artery disease and is doing well on follow-up.

Kounis syndrome secondary to nimesulide ingestion: a case report.

BACKGROUND: Kounis syndrome, also known as "allergic myocardial infarction," is a rare co-occurrence of acute coronary syndrome (ACS) in the setting of hypersensitivity reaction to any agent. Non-steroidal anti-inflammatory drugs (NSAIDs) like are often implicated in causing allergic reactions. Here, we present a case of anterior wall myocardial infarction (AWMI) occurred following angioedema secondary to intake of Nimesulide, not described earlier in literature. CASE PRESENTATION: A 45-year-old female developed generalized pruritic, erythematous maculopapular rash, facial puffiness, oral ulcers and hoarseness of voice within few hours following consumption of Nimesulide for fever and body-ache. Due to development of hypotension, electrocardiogram (ECG) was done, which revealed ST elevation in V2-V6, with marked elevation of troponin (TnI) and creatine kinase (CK-MB). He had no chest pain or shortness of breath. Echocardiography showed regional wall motion (RWMA) abnormality in left anterior descending artery (LAD) territory with an ejection fraction of 25%. Coronary angiography showed a complete thrombotic cutoff of LAD, for which Tirofiban infusion was started to decrease thrombus burden. Repeat angiography on next day showed 80% lesion in proximal LAD for which she underwent revascularization with a drug-eluting stent. The patient later showed improvement in cardiac function at 8 months of follow-up. CONCLUSIONS: The occurrence of ACS requiring percutaneous coronary intervention (PCI) in the setting of allergic reactions is rarely reported in the literature. One should be aware of the rare possibility of Kounis syndrome in the setting of hypersensitivity reaction when accompanying features of symptoms suggestive of coronary artery disease co-exists. When indicated, ECG monitoring and cardiac biomarkers in patients with allergic responses help to identify this rare and treatable condition.

Submitral aneurysm of varied aetiologies: a case series.

BACKGROUND: Submitral aneurysm is a rare disease initially described in the African population. It is usually considered congenital in origin, due to a defect in the posterior portion of the mitral annulus. However, it can be seen in other diseases like ischaemic heart disease, rheumatic heart disease, infective endocarditis, tuberculosis, and syphilis. CASE PRESENTATION: Case 1 was a 29-year-old female, hypertensive undergoing maintenance haemodialysis for chronic kidney disease and on anti-tubercular therapy. She was found to have a large submitral aneurysm with severe mitral regurgitation, moderate left ventricular dysfunction, and pericardial effusion on echocardiogram. Case 2 was a 58-year-old gentleman presented with inferior wall ST-elevation myocardial infarction and was thrombolyzed with streptokinase for the same. Echocardiogram done 6 months later for evaluation of dyspnoea showed a large inferobasal aneurysm. Case 3 was a 56-year-old hypertensive presented with dyspnoea on exertion and echocardiogram showed a large posterolateral region with transmural late gadolinium enhancement. Case 4 was a 13-year-old boy presented with fever and cerebrovascular accident. Echocardiogram revealed vegetation in the mitral valve and a small submitral aneurysm with vegetation inside it. DISCUSSION: Submitral aneurysm is usually considered congenital in origin. However, it can be due to ischaemic heart disease, rheumatic heart disease, Takayasu arteritis, and tuberculosis. Top dimensional echocardiogram is the investigation of choice. Cardiac magentic resonance imaging helps in identifying the underlying aetiology and delineating the surrounding structures.

The Expanding Spectrum of Dystrophinopathies: HyperCKemia to Manifest Female Carriers.

Background: X-linked dystrophinopathies have a wide spectrum of manifestation. The most common forms are severe Duchenne muscular dystrophy (DMD) and Becker's muscular dystrophy (BMD). However, less common manifestations are isolated cardiomyopathy, myalgia, cramps, rhabdomyolysis, hyperCKemia, and manifest female carriers. Materials and Methods: This case series is a part of an ongoing long-term prospective cohort of children with DMD and BMD from the year 2013. The clinical details are maintained in the clinic files and standard management protocols are followed. For this case series, clinical details were collected from the clinic files and recorded on a case record proforma. Details of cardiology, radiology, and genetic investigations were collected. Results: We report cases of classical DMD, BMD, manifest female carrier with proximal pelvic girdle weakness, a female carrier with isolated dilated cardiomyopathy, and infantile-onset asymptomatic hyperCKemia. We also report less common but notable clinical presentations of DMD, autism, intellectual disability, epilepsy, and asymptomatic transaminitis. Conclusions: It is important for clinicians to be aware of these less common clinical presentations for prompt diagnosis, and to avoid unnecessary investigations. Here, we report the clinical spectrum of dystrophinopathies seen in pediatric neuromuscular clinic and emphasize the variability and expanding knowledge about different manifestations of dystrophinopathies.

Large arteriotomies closure using a combination of vascular closure devices during TEVAR/EVAR: A single centre experience.

In this case series, we share our experience of total percutaneous closure of large arteriotomies using combination of vascular closure devices (VCD). A total of six patients with seven sites for endovascular repair were taken for total percutaneous endovascular aortic repair. Ten femoral arteriotomies (26 French (F) = 2, 24 F = 1, 22 F = 3, 20 F = 1, 18 F = 1 &16 F = 2) were successfully closed with 26 Perclose™ and 07 Angio-seal™ devices. There were no local site complications or VCD failure in any of our patients.

Bailout subclavian artery stenting in a sick child with iatrogenic subclavian artery perforation: a case report.

BACKGROUND: Subclavian artery injury during internal jugular vein catheterization is a rare yet potentially life-threatening complication leading to hemothorax and exsanguination. The percutaneous endovascular approach offers a less invasive and effective alternative to the high-risk surgical repair in emergent situations. CASE PRESENTATION: We present a case of a 6-year-old child suffering from hemolytic uremic syndrome requiring urgent hemodialysis, for which IJV (internal jugular vein) cannulation was attempted. This procedure led to iatrogenic subclavian arterial perforation causing massive hemothorax with hemodynamic compromise. CT angiogram showed a through and through perforation in the first part of right subclavian artery between common carotid and vertebral artery. A definitive assessment of the extent of ongoing leak was made through an invasive angiogram in the catheterization laboratory. The perforation was successfully closed percutaneously with a covered stent without compromising any branch vessels. CONCLUSION: Arterial injury although rare is a potentially life-threatening complication of IJV cannulation which warrants immediate attention and corrective measures. Ultrasound guidance can reduce the risk of such life-threatening complications. Percutaneous management offers a less invasive, less time consuming, and effective alternative in critically ill patients in emergency situations.

Left Atrial Myxoma from Anterior Mitral Valve.

Primary cardiac tumors are relatively rare, and myxoma, the most common variety, is found predominantly in the left atrium. Clinical presentation varies from asymptomatic incidental masses to serious life-threatening cardiovascular complications. Some cases are difficult to diagnose, as symptoms can be nonspecific. We present a case of a young female with 4 months of history of dyspnea, weight loss, and night sweats, eventually diagnosed as a case of large left atrial myxoma arising from the anterior mitral valve through transthoracic echocardiography.

Cardiac changes in children hospitalized with severe acute malnutrition: A prospective study at tertiary care center of northern India.

Severe acute malnutrition (SAM) may affect cardiac structure and function. Cardiac changes in sick children with SAM have received little attention in the literature. Children aged 6-60 months with SAM were cases, and age and sex matched children were controls. Cardiac biomarker levels were measured by the quantitative the Enzyme- linked immunosorbent assay (ELISA) method, and echocardiography was used to assess cardiac changes in all children. The study included 76 children in each group. Children with SAM had less left ventricular mass and increased myocardial performance index as compared with controls (p < 0.0001). Cardiac biomarker levels were increased in children with SAM (p < 0.0001). Cardiac changes and biomarker levels were comparable in children with edema and children without edema except creatine kinase-MB (p = 0.01).

Renal autotransplantation for the management of renal artery in-stent restenosis in an adult patient with Takayasu arteritis.

Renovascular hypertension is a common clinical presentation in Takayasu arteritis (TA), when the renal arteries are involved. Although most of the patients respond to optimal antihypertensive drug therapy, certain patients with TA require percutaneous or surgical renal artery revascularisation to manage renovascular hypertension. We, hereby, present a 45-year-old woman, who had resistant hypertension secondary to in-stent restenosis (ISR) of renal artery stent in a single functioning kidney. She had successful renal autotransplantation following a failed endovascular attempt to treat ISR. Endovascular and surgical interventions related to renal artery stenosis in TA are discussed in the article.

Bleeding Heart and Coronary Cameral Fistulae.

This letter addresses an article regarding coronary-cameral fistula after septal myomectomy published in the December 2017 issue of the Journal of Invasive Cardiology and authored by Walters D, et al.

Evaluation of cardiac biomarkers in children with acute severe bronchial Asthma-A prospective study from tertiary care center in northern India.

OBJECTIVES: During the attacks of acute severe bronchial asthma there are marked cardiopulmonary changes leading to hypoxia. The study aims to find the incidence of myocardial dysfunction in patients of acute severe bronchial asthma based on cardiac enzyme levels at admission and see whether the myocardial damage is transient or persistent even after stabilization of the patient based on enzyme levels at discharge. MATERIALS AND METHODS: This prospective, case control study was done at Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University between October 2016 to May 2018. Sixty pediatric patients of acute severe bronchial asthma were taken as cases and 15 age and sex matched children served as controls. Blood samples were collected in Ethylene diamine tetra acetic acid vials before the start of treatment, for measurement of cardiac biomarkers Troponin I (TnI), Brain natriuretic peptide (BNP) and Creatine Kinase-muscle/brain (CK-MB) and repeat samples were taken before discharge. RESULTS: Fifty percent of the cases had abnormal TnI levels, 15% had abnormal CK-MB levels and 8.3% had abnormal BNP levels at admission. At discharge, only 1 (1.7%) case had abnormal levels of CK-MB, whereas the levels of TnI and BNP normalized in all. The level of cardiac biomarkers were significantly raised at admission when compared to discharge values (p value < 0.001). CONCLUSIONS: The raised cardiac biomarkers suggest myocardial stress during acute exacerbation of bronchial asthma. Though, the present study showed that the changes are of transient nature, larger follow up studies are required to document any permanent damage to myocardium.

Clinical Profile with Angiographic Correlation in Naïve Acute Coronary Syndrome.

INTRODUCTION: Despite cardiovascular diseases having grown to epidemic proportions, there are few studies from India pertaining to Acute Coronary Syndrome (ACS), more so from the region of Purvanchal which is less developed with more poverty. Our study is first of its kind in this region of patients presenting for the first time with ACS. AIM: The present study was undertaken to study the clinical and angiographic characteristics of ACS patients of Purvanchal. MATERIALS AND METHODS: This was a prospective cohort study of 100 patients admitted with ACS. Patients were excluded if they had prior cardiac pathology like valvular heart disease, cardiomyopathy, pericardial disease, cor pulmonale, ischaemic heart disease or cardiac revascularisation. Patients who did not undergo angiography were excluded. Patients were divided into ST Elevation Myocardial Infarction (STEMI) and non STEMI (NSTEMI). Presentation delays as well as clinical characteristics analysed in each group were age, gender, presence or absence of diabetes mellitus, hypertension, dyslipidaemia, smoking, Body Mass Index (BMI), family history, duration of chest pain, and treatment received. RESULTS: Mean age of patients was 58.9 years with 27% below 50 years. Of the total 75% were males. Patients with STEMI were 65%. Median time to reach hospital was 24 hours with only 27% patients reaching hospital within 6 hours. Among patients with STEMI only 43% received fibrinolytic therapy. 23% patients had diabetes, 21% were hypertensive, 16% were smokers, family history of cardiovascular disease present in 11% and 21% had body mass index more than 30. Mean LDL was 115mg/dl and HDL 39mg/dl with 54% of patients having at least one risk factor. Factors favouring triple vessel involvement were female sex, higher age, smoking, presence of diabetes and NSTEMI. CONCLUSION: Indians develop ACS at earlier age. Precious time is lost before seeking treatment. There is a need for aggressive risk factor modification which along with health awareness will be the key to prevent premature cases of ACS and limit morbidity and mortality due to delayed treatment.