Journal of The Indian Academy of Echocardiography & Cardiovascular Imaging最新文献

{"title":"Case Report of Absent Pulmonary Valve with Intact Interventricular Septum","authors":"Deendayal Nagar, Touseef Ul Ayoub, Satish Koirala, N. Awasthy","doi":"10.4103/jiae.jiae_62_23","DOIUrl":"https://doi.org/10.4103/jiae.jiae_62_23","url":null,"abstract":"\u0000 Absent pulmonary valve syndrome is a rare cardiac malformation, usually associated with tetralogy of Fallot. Its presence with intact interventricular septum is even less common with case reports or very small series in the literature. We describe a case who presented with symptoms of right heart failure due to an absent pulmonary valve with severe pulmonary regurgitation and small atrial septal defect. Diagnostic evaluation revealed grossly dilated main pulmonary artery and rudimentary leaflets of the pulmonary valve with severe regurgitation. Ventricular septum was intact and there was no patent ductus arteriosus. Later, the patient underwent surgical prosthetic pulmonary valve implantation.","PeriodicalId":175565,"journal":{"name":"Journal of The Indian Academy of Echocardiography & Cardiovascular Imaging","volume":"85 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140236412","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Scimitar Syndrome with Acquired Cardiac Disease: A Once-in-a-blue-moon Occurrence","authors":"I. Tammiraju, Meghana Bhupathi","doi":"10.4103/jiae.jiae_68_23","DOIUrl":"https://doi.org/10.4103/jiae.jiae_68_23","url":null,"abstract":"\u0000 Scimitar syndrome (SS) is a rare congenital anomaly characterized by various abnormalities observed in both children and adults, primarily involving partial anomalous pulmonary venous drainage from the right lung to the inferior vena cava and right lung hypoplasia. It is also known by alternative names such as congenital venolobar syndrome, Halasz syndrome, mirror-image lung syndrome, hypogenetic lung syndrome, and vena cava bronchovascular syndrome. The clinical presentation of this syndrome varies widely, ranging from asymptomatic cases to severe forms. Surgical intervention is typically required for severe cases, and the diagnosis relies predominantly on imaging studies. We present two instances of SS: one involving a patient with underlying rheumatic heart disease scheduled for valve replacement surgery and the other as an incidental finding in an asymptomatic individual, managed through medical means.","PeriodicalId":175565,"journal":{"name":"Journal of The Indian Academy of Echocardiography & Cardiovascular Imaging","volume":"89 4","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140236190","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Giant Coronary Artery Aneurysm Associated with Extraluminal Migration of Stent: Role of Multimodal Imaging","authors":"Madhu Shukla, J. Mohan","doi":"10.4103/jiae.jiae_69_23","DOIUrl":"https://doi.org/10.4103/jiae.jiae_69_23","url":null,"abstract":"\u0000 Extraluminal migration of a drug-eluting intracoronary stent into pericardial space with or without aneurysm formation is a rare complication. It is usually symptomatic although rare asymptomatic cases have been described. Herein, we report a 49-year-old male who presented with atypical chest pain 9 years after intracoronary stent implantation in the right coronary artery and the ramus intermediate branch. Echocardiographic examination showed a 7.4 cm × 3.8 cm large oblong cystic mass along the lateral wall of the left ventricle with inward bulging of the adjacent segments. Computed tomography and invasive coronary angiography showed this mass to be a partly thrombosed giant oblong coronary aneurysm arising from the bifurcation of the left main coronary artery with extraluminal migration of the stent from the ramus branch.","PeriodicalId":175565,"journal":{"name":"Journal of The Indian Academy of Echocardiography & Cardiovascular Imaging","volume":" 18","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140391083","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Congenital Aneurysmal Tunnel of the Trabecular Muscular Interventricular Septum or a Partially Closed Ventricular Septal Defect? A Case Report with Review of Literature","authors":"Madhu Shukla, J. Mohan","doi":"10.4103/jiae.jiae_67_23","DOIUrl":"https://doi.org/10.4103/jiae.jiae_67_23","url":null,"abstract":"\u0000 Isolated congenital aneurysm-like deformity of the muscular interventricular septum is rare and may be associated with other congenital heart defects, ventricular dysfunction, conduction disturbances, and chromosomal abnormalities. It may be a partial or near-total defect. We report a tunnel-like congenital anomaly of the mid-muscular ventricular septum without other congenital anomalies or conduction disturbance in a 17-year-old asymptomatic girl. The partial ventricular septal defect was lined on the outer side by the right ventricular endocardium and showed partial obliteration during systole.","PeriodicalId":175565,"journal":{"name":"Journal of The Indian Academy of Echocardiography & Cardiovascular Imaging","volume":"12 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140236302","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cardiac Amyloidosis: Mutimodality Imaging for Diagnosis and Prognosis","authors":"N. Burkule","doi":"10.4103/jiae.jiae_65_23","DOIUrl":"https://doi.org/10.4103/jiae.jiae_65_23","url":null,"abstract":"\u0000 Cardiac amyloid cardiomyopathy (CM) is a rapidly progressive disease that is frequently underrecognized and frequently diagnosed late in a significant number of individuals suffering from heart failure. Cardiac amyloid infiltration resulting in myocardial hypertrophy and restrictive CM is primarily caused by the misfolding of precursor proteins such as transthyretin, light chain immunoglobulin, and apolipoprotein AI-IV. The utilization of echocardiography, cardiac magnetic resonance, and bone avid radiotracer scintigraphy are essential in establishing a reliable diagnosis and prognosis of cardiac amyloidosis in the majority of patients. However, in certain clinical scenarios, the use of cardiac or extracardiac biopsy is necessary. There are significant developments in the understanding of the pathobiology of amyloid formation, which has paved the way for the development of new-targeted therapies, specifically for transthyretin cardiac amyloidosis. Imaging techniques are developing to monitor quantitatively the progression and regression of cardiac and systemic amyloid infiltration.","PeriodicalId":175565,"journal":{"name":"Journal of The Indian Academy of Echocardiography & Cardiovascular Imaging","volume":"53 9","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140237208","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prominent Transverse Sinus of Pericardium is Uncommon Diagnostic Pitfall on Echocardiography: Clues to Diagnosis","authors":"M. K. Angjushev, D. Angjushev, A. Dikić","doi":"10.4103/jiae.jiae_63_23","DOIUrl":"https://doi.org/10.4103/jiae.jiae_63_23","url":null,"abstract":"\u0000 Transverse sinus (TS) is a normal structure of pericardium, usually visualized on transesophageal echocardiography (TEE). Rarely, when it is prominent, it may be visualized on transthoracic echocardiography (TTE), as a mobile circumscribed mass in the parasternal short-axis view. Due to its location adjacent to left atrial appendage (LAA), it can be misdiagnosed as an LAA thrombus. A 41-year-old woman presented with heart failure signs. TTE showed a circumscribed mass (1 cm × 2 cm), in or adjacent to the LAA, suggestive of an LAA thrombus. Additional maneuvers with the transducer made it obvious that it was a prominent TS, adjacent to the LAA, while the appendage was empty. TEE confirmed the diagnosis.","PeriodicalId":175565,"journal":{"name":"Journal of The Indian Academy of Echocardiography & Cardiovascular Imaging","volume":"93 S2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140235915","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Study of the Usefulness of Tissue Doppler Imaging for the Diagnosis of Coronary Artery Disease in Patients with Left Bundle Branch Block","authors":"Syed Imamuddin, Ravi Srinivas, K. Parvathareddy, Praveen Nagula, K. Suneetha, M. Ganesh","doi":"10.4103/jiae.jiae_39_23","DOIUrl":"https://doi.org/10.4103/jiae.jiae_39_23","url":null,"abstract":"\u0000 \u0000 \u0000 In patients with left bundle branch block (LBBB), the diagnosis of ischemia by noninvasive modalities is cumbersome. Most of them such as stress tests, nuclear imaging, and magnetic resonance imaging have limitations in the detection of ischemia in this subset. The postsystolic motion (PSM) during the isovolumetric relaxation period on tissue Doppler imaging (TDI) is a sensitive and specific marker of ischemia. We aimed to see whether the TDI parameters can detect coronary artery disease (CAD) in patients with LBBB.\u0000 \u0000 \u0000 \u0000 Patients with LBBB (n = 64) who underwent coronary angiography were divided into two groups. Group A (n = 30) included patients with left anterior descending (LAD) artery stenosis of ≥70%, and group B (n = 34) included patients without LAD stenosis. All patients underwent TDI and various myocardial tissue velocity parameters were analyzed to detect the presence of CAD. P < 0.05 was considered significant.\u0000 \u0000 \u0000 \u0000 The TDI of the mid-interventricular septum showed a higher delayed amplitude of PSM (>100 ms after aortic valve closure), lower myocardial systolic (Sm), and early diastolic (Em) velocities, and a higher late diastolic (Am) velocity in group A when compared to group B (all were significant, P < 0.0001). Both the ratios Sm/PSM and Em/Am were significantly lower in group A compared to group B (P < 0.0001). On receiver operating characteristic curve analysis to predict the presence of significant LAD stenosis, the value of Sm/PSM ratio <0.8 showed the best combination of sensitivity (78%) and specificity (96%) with an area under the curve of 0.936.\u0000 \u0000 \u0000 \u0000 TDI, a noninvasive imaging modality, is reliable and effective in identifying myocardial ischemia in patients with LBBB.\u0000","PeriodicalId":175565,"journal":{"name":"Journal of The Indian Academy of Echocardiography &amp; Cardiovascular Imaging","volume":" 5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139788508","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cardiac Involvement in Pediatric Mucopolysaccharidoses Patients and their Genetic Profile: A Retrospective Single-center Study","authors":"Shruti Irene Varghese, Joshua Abishek Christudoss, Rekha Aaron, S. Danda, Jesu Krupa","doi":"10.4103/jiae.jiae_61_23","DOIUrl":"https://doi.org/10.4103/jiae.jiae_61_23","url":null,"abstract":"\u0000 \u0000 \u0000 Mucopolysaccharidoses (MPS) are rare metabolic disorders of monogenic inheritance, with varying degrees of cardiac involvement due to undegraded glycosaminoglycans getting aggregated in the spongiosa of the cardiac valves, the myocardium, and myointima of the coronary arteries. This study is an effort to understand the cardiac involvement and genetic pattern of pediatric MPS patients at a tertiary care center in South India.\u0000 \u0000 \u0000 \u0000 This descriptive study was done by examining records in our hospital database for MPS patients in the age group of 0–15 years, diagnosed between January 2014 and January 2023. Of these patients, 37 patients with echocardiographic loops available in the hospital system were reevaluated by a single pediatric cardiologist to avoid observer bias. The underlying genetic spectrum was also reviewed.\u0000 \u0000 \u0000 \u0000 The cohort comprised 67.5% of males and a median age of 9.8 years (interquartile range: 6.5–14.9 years) with a distribution of 37.8% MPS I, 32.4% MPS II, 13.5% MPS IV, and 10.8% MPS VI. Early cardiac involvement in the form of valvar thickening, predominantly left sided (mitral [92%] >aortic [38%]), was seen across all MPS subtypes. We also noted an increased involvement of the tricuspid valve (38%) in our cohort. Moderate–severe valvar involvement was seen in 16% of the cohort, mostly MPS I, II, and VI patients, and more in the second half of the age group (>6 years), suggesting an age-associated worsening of cardiac lesions. Genetic analysis done in 68% of the cohort detected 61% of pathogenic variants, with a predominance of missense mutation (43%). However, no genotype–cardiac phenotype correlation could be ascertained.\u0000 \u0000 \u0000 \u0000 We identified a high proportion of cardiac abnormalities in our pediatric MPS cohort, which ranged from isolated valvar thickening to a more severe clinical picture, including moderate-to-severe valvular insufficiency and/or stenosis and left ventricular hypertrophy. Cardiac pathology is an early manifestation and a known cause of mortality in many MPS subtypes. Echocardiography is a key diagnostic technique for early detection, careful monitoring, and guidance in the initiation of a timely intervention that will help curb its progression. Hence, the need arises to raise awareness among the primary caretakers of this subset of patients on the need for early cardiac evaluation with a detailed echocardiography.\u0000","PeriodicalId":175565,"journal":{"name":"Journal of The Indian Academy of Echocardiography &amp; Cardiovascular Imaging","volume":" 11","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139790634","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Study of the Usefulness of Tissue Doppler Imaging for the Diagnosis of Coronary Artery Disease in Patients with Left Bundle Branch Block","authors":"Syed Imamuddin, Ravi Srinivas, K. Parvathareddy, Praveen Nagula, K. Suneetha, M. Ganesh","doi":"10.4103/jiae.jiae_39_23","DOIUrl":"https://doi.org/10.4103/jiae.jiae_39_23","url":null,"abstract":"\u0000 \u0000 \u0000 In patients with left bundle branch block (LBBB), the diagnosis of ischemia by noninvasive modalities is cumbersome. Most of them such as stress tests, nuclear imaging, and magnetic resonance imaging have limitations in the detection of ischemia in this subset. The postsystolic motion (PSM) during the isovolumetric relaxation period on tissue Doppler imaging (TDI) is a sensitive and specific marker of ischemia. We aimed to see whether the TDI parameters can detect coronary artery disease (CAD) in patients with LBBB.\u0000 \u0000 \u0000 \u0000 Patients with LBBB (n = 64) who underwent coronary angiography were divided into two groups. Group A (n = 30) included patients with left anterior descending (LAD) artery stenosis of ≥70%, and group B (n = 34) included patients without LAD stenosis. All patients underwent TDI and various myocardial tissue velocity parameters were analyzed to detect the presence of CAD. P < 0.05 was considered significant.\u0000 \u0000 \u0000 \u0000 The TDI of the mid-interventricular septum showed a higher delayed amplitude of PSM (>100 ms after aortic valve closure), lower myocardial systolic (Sm), and early diastolic (Em) velocities, and a higher late diastolic (Am) velocity in group A when compared to group B (all were significant, P < 0.0001). Both the ratios Sm/PSM and Em/Am were significantly lower in group A compared to group B (P < 0.0001). On receiver operating characteristic curve analysis to predict the presence of significant LAD stenosis, the value of Sm/PSM ratio <0.8 showed the best combination of sensitivity (78%) and specificity (96%) with an area under the curve of 0.936.\u0000 \u0000 \u0000 \u0000 TDI, a noninvasive imaging modality, is reliable and effective in identifying myocardial ischemia in patients with LBBB.\u0000","PeriodicalId":175565,"journal":{"name":"Journal of The Indian Academy of Echocardiography &amp; Cardiovascular Imaging","volume":"346 1-3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139848339","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cardiac Involvement in Pediatric Mucopolysaccharidoses Patients and their Genetic Profile: A Retrospective Single-center Study","authors":"Shruti Irene Varghese, Joshua Abishek Christudoss, Rekha Aaron, S. Danda, Jesu Krupa","doi":"10.4103/jiae.jiae_61_23","DOIUrl":"https://doi.org/10.4103/jiae.jiae_61_23","url":null,"abstract":"\u0000 \u0000 \u0000 Mucopolysaccharidoses (MPS) are rare metabolic disorders of monogenic inheritance, with varying degrees of cardiac involvement due to undegraded glycosaminoglycans getting aggregated in the spongiosa of the cardiac valves, the myocardium, and myointima of the coronary arteries. This study is an effort to understand the cardiac involvement and genetic pattern of pediatric MPS patients at a tertiary care center in South India.\u0000 \u0000 \u0000 \u0000 This descriptive study was done by examining records in our hospital database for MPS patients in the age group of 0–15 years, diagnosed between January 2014 and January 2023. Of these patients, 37 patients with echocardiographic loops available in the hospital system were reevaluated by a single pediatric cardiologist to avoid observer bias. The underlying genetic spectrum was also reviewed.\u0000 \u0000 \u0000 \u0000 The cohort comprised 67.5% of males and a median age of 9.8 years (interquartile range: 6.5–14.9 years) with a distribution of 37.8% MPS I, 32.4% MPS II, 13.5% MPS IV, and 10.8% MPS VI. Early cardiac involvement in the form of valvar thickening, predominantly left sided (mitral [92%] >aortic [38%]), was seen across all MPS subtypes. We also noted an increased involvement of the tricuspid valve (38%) in our cohort. Moderate–severe valvar involvement was seen in 16% of the cohort, mostly MPS I, II, and VI patients, and more in the second half of the age group (>6 years), suggesting an age-associated worsening of cardiac lesions. Genetic analysis done in 68% of the cohort detected 61% of pathogenic variants, with a predominance of missense mutation (43%). However, no genotype–cardiac phenotype correlation could be ascertained.\u0000 \u0000 \u0000 \u0000 We identified a high proportion of cardiac abnormalities in our pediatric MPS cohort, which ranged from isolated valvar thickening to a more severe clinical picture, including moderate-to-severe valvular insufficiency and/or stenosis and left ventricular hypertrophy. Cardiac pathology is an early manifestation and a known cause of mortality in many MPS subtypes. Echocardiography is a key diagnostic technique for early detection, careful monitoring, and guidance in the initiation of a timely intervention that will help curb its progression. Hence, the need arises to raise awareness among the primary caretakers of this subset of patients on the need for early cardiac evaluation with a detailed echocardiography.\u0000","PeriodicalId":175565,"journal":{"name":"Journal of The Indian Academy of Echocardiography &amp; Cardiovascular Imaging","volume":"16 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139850559","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}