Identification and Analysis of Atrial-Bronchial-Abdominal Disharmony in Patients with Isomeric Atrial Appendages.

Ideally, the morphology of atrial appendages should solely be used to identify and differentiate patients with isomeric right and left atrial appendages. However, in clinical practice, the segregation is often indirectly based on the arrangement of thoraco-abdominal structures. The correlation between thoraco-abdominal arrangement and atrial appendages, however, is imperfect. In this study, we sought to clarify the cardiovascular malformations in patients with isomeric atrial appendages with an emphasis on atrial-thoracic-abdominal disharmony. A retrospective review of all patients who underwent cardiac CT angiography between January 2014 and June 2023 and identified to have isomeric atrial appendages was performed. Of the 366 cases (median age: 2 years [interquartile range: 11 months-7 years]), 247 (67.5%) patients had isomeric right atrial appendages while 119 (32.5%) patients had isomeric left atrial appendages. In 316 (86.3%) patients, the thoraco-abdominal arrangement was as per atrial appendage morphology while the remaining 50 (13.6%) patients had disharmonious patterns. Compared to isomeric left atrial appendages, the disharmonious pattern was more frequent with isomeric right atrial appendages (5.9% vs. 17.4%; p 0.003). Irrespective of the type of isomerism, disharmony was mostly confined to the level of the abdomen. Not all patients with isomeric atrial appendages have a harmonious thoraco-abdominal arrangement. The atrial-bronchial-abdominal disharmony is more frequent with isomeric right atrial appendages and is mostly present at the level of the abdomen. A detailed sequential segmental analysis with an independent description of each organ system is, therefore, essential for the complete evaluation of patients with isomeric atrial appendages.

Role of Iron Therapy in Heart Failure: A Consensus Statement from India.

Iron deficiency (ID) with or without anemia is frequently observed in patients with heart failure (HF). Uncorrected ID is associated with higher hospitalization and mortality in patients with acute HF (AHF) and chronic HF (CHF). Hence, in addition to chronic renal insufficiency, anemia, and diabetes, ID appears as a novel comorbidity and a treatment target of CHF. Intravenous (IV) ferric carboxymaltose (FCM) reduces the hospitalization risk due to HF worsening and improves functional capacity and quality of life (QOL) in HF patients. The current consensus document provides criteria, an expert opinion on the diagnosis of ID in HF, patient profiles for IV FCM, and correct administration and monitoring of such patients.

Angiotensin Receptor-Neprilysin Inhibitor Therapy and Cardiac Remodeling in Heart Failure: Consensus Statement from India.

Adverse cardiac remodeling refers to progressive structural and functional modifications in the heart because of increased wall stress in the myocardium, loss of viable myocardium, and neurohormonal stimulation. The guideline-directed medical therapy for Heart failure (HF) includes Angiotensin receptor-neprilysin inhibitor (ARNI) (sacubitril/valsartan), ß-blockers, sodium-glucose co-transporter 2 (SGLT2) inhibitors, and mineralocorticoid receptor antagonists (MRA). ARNI is under-prescribed in India despite its attractive safety and efficacy profile. Therefore, the consensus discusses objectives and topics related to ARNI in the management of cardiac remodeling, and experts shared their views on the early timely intervention of effective dosage of ARNI to improve the diagnosis and enhance mortality and morbidity benefits in cardiac reverse remodeling (CRR).

A meta-analysis of the diagnostic performance of quiescent-interval-single-shot magnetic resonance angiography in peripheral arterial disease.

OBJECTIVES: To evaluate by meta-analysis the diagnostic accuracy of non-contrast quiescent-interval-single-shot (QISS) magnetic resonance angiography (MRA) in patients with peripheral arterial disease (PAD) using digital subtraction angiography (DSA) or contrast-enhanced magnetic resonance angiography (CE-MRA) as reference standard. METHODS: This study was performed and reported according to the Preferred Reporting Items for Systematic reviews and Meta-analysis guidelines. A systematic literature search of MEDLINE, Embase and Scopus was done for studies reporting the diagnostic accuracy of QISS in PAD published up to 31 May 2021. The pooled sensitivity, specificity and diagnostic accuracy of QISS were calculated on a per-segment basis for the entire arterial tree. RESULTS: Seventeen studies including 459 patients were found eligible for the meta-analysis. There was significant heterogeneity among studies as depicted by chi-square test (p = 0.02) and moderate heterogeneity by I2 statistic (I2: 69 [95% CI: 30-100]). The pooled sensitivity and specificity of QISS on a per-segment basis with DSA/CE-MRA as reference standard was 0.88 (95% CI: 0.85-0.91) and 0.94 (95% CI: 0.92-0.96) respectively. The area under hierarchical summary receiver-operating characteristic reflected a high accuracy of 0.96 (95% CI: 0.94-0.98). There was a low likelihood of publication bias as indicated by Deeks' funnel plot. CONCLUSIONS: The present meta-analysis has consolidated the evidence that QISS has high accuracy for identifying as well as excluding arterial stenosis/occlusions in patients with symptoms of PAD. It can thus be considered the test of choice in patients with renal failure and in "at-risk patients" including pregnant women and patients with contrast allergy. KEY POINTS: • The pooled sensitivity and specificity of QISS magnetic resonance angiography on a per-segment basis with DSA or contrast-enhanced MRA as reference standard are 88% and 94% respectively. • The diagnostic accuracy of QISS in patients with peripheral arterial disease as reflected by area under hierarchical summary receiver-operating characteristic is high (0.96 (95% CI: 0.94-0.98)). • There is moderate to significant heterogeneity among studies as depicted by I2 statistic and chi-square test.

Efficacy and Safety of Venous Sac Embolization With or Without Feeding Artery Embolization Versus Feeding Artery Embolization Alone in the Management of Pulmonary Arteriovenous Malformations: A Systematic Review and Meta-analysis.

PURPOSE: To evaluate by meta-analysis the safety and efficacy of venous sac embolization (VSE) with or without feeding artery embolization versus feeding artery embolization (FAE) alone in the management of pulmonary arteriovenous malformations (PAVMs). METHODS: This systematic review and meta-analysis was performed and reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A systematic literature search was performed in MEDLINE, Embase, and Scopus till August 31, 2021 to identify studies comparing the safety and efficacy of VSE with or without FAE versus FAE alone in PAVMs. The success of treatment was assessed by comparing the number of PAVMs with ≥70% decrease in the size of draining vein/sac between the 2 groups. The pooled odds ratio (OR) and 95% confidence interval (95% CI) were calculated using the random-effects inverse-variance model and were used to compare pooled therapeutic efficacy between the groups. RESULTS: Three studies were found eligible for the meta-analysis. The included studies had a total of 169 patients (119 females and 50 males). The total number of PAVMs was 298, and hereditary hemorrhagic telangiectasia was present in 119 patients. The success of treatment was higher with VSE as compared to FAE (OR=3.54, 95% CI=1.66-7.56). The minor complications were similar in both groups, and one major complication occurred in the FAE group. More coils were used in the VSE group. CONCLUSIONS: The present systematic review and meta-analysis has consolidated the evidence that VSE with or without FAE is more efficacious than FAE alone in the management of PAVMs without any increase in the associated risk of sac rupture or coil migration. CLINICAL IMPACT: Until high-quality data from a prospective, multicentric, randomized controlled trial becomes available, the evidence consolidated by the present systematic review and meta-analysis showing the efficacy of venous sac embolization (with or without feeding artery embolization) over feeding artery embolization alone, can be used for clinical decision-making in the management of pulmonary arterio-venous malformations.

Origin of the left common carotid artery from ascending aorta in association with tetralogy of Fallot and hypoplastic right cervical aortic arch.

We present a case of a 6-year-old girl with tetralogy of Fallot (TOF) and hypoplastic aortic arch with anomalous origin of left common carotid artery from the ascending aorta. The case highlights the importance of screening for abnormalities of aortic arch and arch vessels in TOF patients for accurate presurgical planning and to predict the postoperative outcomes.

Anomalous great cardiac vein draining into juxtaposed right atrial appendage.

We present a case of a 2-year-old boy with a double outlet right ventricle and juxtaposed atrial appendages with anomalous drainage of the great cardiac vein into the aneurysmally dilated juxtaposed right atrial appendage. The case highlights the role of computed tomography (CT) angiography in depicting anomalous coronary venous anatomy and guiding therapeutic interventions involving the coronary venous approach.

Incidentally detected dual right coronary artery in patient with constrictive pericarditis.

We report a case of a 29-year-old man with constrictive pericarditis where CT angiography incidentally demonstrated a dual right coronary artery (RCA). The present case highlights the diagnostic criterion for dual RCA as well as the potential clinical implications of the anomaly.

Proximal interruption of the right pulmonary artery: A rare anomaly.

We report a case of a 21-year-old woman with cyanosis where computed tomography (CT) angiography revealed a complete absence of the mediastinal part of the right pulmonary artery with the normal intrapulmonary vascular network, receiving supply from multiple collateral vessels. The case also highlights the various differential diagnoses and the role of CT angiography in arriving at the correct diagnosis.

Juxtaposition of atrial appendages: Evaluation of cardiovascular morphology and associated anomalies on multidetector computed tomography angiography.

AIM: To evaluate the morphology and associated cardiovascular abnormalities in patients with juxtaposition of atrial appendages (JAA) on multidetector computed tomography (MDCT) angiography. MATERIALS AND METHODS: We performed the retrospective study at a tertiary referral center to identify patients diagnosed with JAA on MDCT angiography using dual-source CT scanner between January 2014 and April 2021. The various imaging abnormalities evaluated included the type of JAA, morphological and positional classification of JAA, atrial situs, cardiac position, atrioventricular and ventriculoarterial connections, great vessel relationship, and other associated cardiovascular anomalies. RESULTS: We identified 129 patients with JAA. Left-sided juxtaposition was seen in 124 (96.1%) patients while right-sided juxtaposition was seen in 5 (3.9%) patients. Among patients with left-sided juxtaposition, frequent cardiovascular associations included outflow tract malformations (100%), hypoplastic right ventricle (40.3%), tricuspid atresia (32.2%), and right-sided heart (19.4%). The most frequent outflow tract malformation was double outlet right ventricle (DORV; 60.5%) followed by transposition of great arteries (20.1%) and transposed aorta with pulmonary atresia (18.5%). In patients with right-sided juxtaposition, outflow tract malformation was seen in three (60%) patients with one (20%) patient each showing DORV, transposed aorta with pulmonary atresia, and tetralogy of Fallot, respectively. CONCLUSION: The identification of JAA on MDCT angiography suggests coexistence of various complex congenital heart diseases, especially outflow tract malformations. A meticulous search is imperative to identify this anomaly before interventional or surgical procedures to avoid complications.

Anomalous origin of one pulmonary artery from aorta: Evaluation on computed tomography angiography.

AIM: The present study sought to evaluate the morphology and associated cardiovascular anomalies in patients with anomalous origin of one pulmonary artery from aorta (AOPA) on CT angiography. MATERIALS AND METHODS: A retrospective search of the departmental database from January 2014 to September 2021 was conducted to identify patients diagnosed with AOPA on CT angiography. The subtype of AOPA, site of origin, and associated cardiovascular abnormalities were assessed. RESULTS: AOPA was identified in 27 patients (19 males, mean age: 8.8 years). Anomalous origin of the right pulmonary artery from aorta (AORPA) was observed in 17/27 (63%) patients, while anomalous origin of the left pulmonary artery (AOLPA) was seen in 10/27 (37%) patients. The proximal and distal subtypes were observed in 24/27 (88.9%) and 3/27 (11.1%) patients respectively. AOPA was associated with other cardiac anomalies in 92.6% (25/27) patients. Patent arterial duct (11/17; 64.7%) was the most frequently anomaly associated with AORPA, while tetralogy of Fallot (10/10; 100%) was the most commonly anomaly associated with AOLPA. The anomalous pulmonary artery was contralateral to the aortic arch in 23/27 (85.2%) patients. Right-sided aortic arch was observed in only 2/17 (11.8%) patients with AORPA and 8/10 (80%) patients with AOLPA. In 2/10 (20%) patients, AORPA was associated with aortopulmonary window and aortic arch anomaly. CONCLUSION: AOPA is rare and frequently associated with other cardiovascular anomalies like patent arterial duct, tetralogy of Fallot, and right-sided aortic arch. CT angiography helps in providing exact anatomical delineation and identifying associated anomalies, thus aiding preoperative planning of surgical management.

A rare case of tetralogy of Fallot-pulmonary atresia with absent right pulmonary artery and anomalous origin of left pulmonary artery from ascending aorta.

We report a case of a 5-year-old child with tetralogy of Fallot-pulmonary atresia with anomalous origin of left pulmonary artery from ascending aorta and right lung supplied by collateral arteries. This case highlights the role of preoperative CT angiography in differentiating this entity from a common arterial trunk with discontinuous pulmonary arteries.

Double aortic arch with partially atretic left arch detected on CT angiography in a case of double-chambered right ventricle: A rare association.

We report an 11-year-old child with double-chambered right ventricle detected on echocardiography in whom computed tomography (CT) angiography revealed a rare arch anomaly double aortic arch with atretic left arch. This case highlights the ability of CT angiography in identifying such cardiovascular anomalies which have clinical and surgical implications.

Imaging characteristics and associations of left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers on multidetector CT.

AIM: To study the imaging characteristics and the associations of left atrioventricular valve atresia with patent aortic root and lateralized atrial chambers on multidetector computed tomography (CT) angiography. MATERIALS AND METHODS: We retrospectively evaluated all the CT angiography studies performed for the evaluation of various congenital heart diseases at our center from January 2014 to December 2021. Cases with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers were identified. The types of cardiovascular involvement as identifiable on CT angiography were studied and compiled. RESULTS: A total of 38 patients with left atrioventricular valve atresia and patent aortic root were included (median age: 5 months, males: 31). A total of 89.5% had usual atrial arrangement and 10.5% had mirror imagery of atria. Normal drainage of right and left superior caval veins and inferior caval vein into systemic venous atrium was seen in 77.8%, 61.5%, and 86.6%, respectively. Anomalous pulmonary venous drainage was seen in 3 (7.9%) patients. Atrial and ventricular septal defects were the most common associations. Imperforate left atrioventricular membrane was seen in 9 (23.7%) patients and absent left atrioventricular connection in 27 (71.1%). Discordant right atrioventricular connection was seen in 5 (13.1%) patients. While a double outlet right ventricle (76.3%) was the most common ventriculoarterial connection, discordant ventriculo-arterial connection (transposition) in 4 (10.5%). Most patients had good sized pulmonary arteries. Patent arterial duct and right aortic arch were seen in 29% and 21%, respectively. Aberrant right subclavian artery was the most common arch vessel anomaly. Coronary anomalies were seen in 7 patients (19%); single coronary artery being the most common. CONCLUSIONS: Majority of the patients with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers have usual atrial arrangement, normal right atrioventricular connections and a double outlet configuration of the right ventricle. Absent left atrioventricular connection is a more common than imperforate left atrioventricular valve membrane. Atrial and ventricular septal defects are the most common associations. Comprehensive CT based evaluation of vascular and nonvascular thoracic structures is imperative before surgery or interventions in patients with mitral atresia.

Imaging characteristics and associations of left atrioventricular valve atresia and patent aortic root with isometric atrial appendages on multidetector CT.

AIM: The present study sought to study the imaging associations of left atrioventricular valve (mitral) atresia and patent aortic root in patients with isomerism of atrial appendages as detected on multidetector computed tomography (CT) angiography MATERIALS AND METHODS: The CT angiography studies performed at a tertiary medical center from January 2014 to December 2021 were retrospectively evaluated to identify patients with left atrioventricular valve atresia and patent aortic root with isometric atrial chambers. The morphology of the atrial appendage and extent of pectinate muscles was used to identify the type of isomeric atrial chambers. A comprehensive assessment of discordant arrangement of organs and the variations in venoatrial connections was done. RESULTS: Left atrioventricular valve atresia in the presence of patent aortic root and isomeric atrial appendages was identified in eight patients (four males). The mean age at diagnosis was 7 years. Right isomerism of the atrial appendages was seen in five patients while three patients had left isomerism. Disharmonious patterns of abdominal visceral arrangement were seen in two patients. Atrial and ventricular septal defects were seen in all patients. The most common pattern of ventricular arterial connection was DORV (double outlet right ventricle) (n = 4). While five patients had normally related great arteries, three had aorta anterior to the pulmonary trunk. Right ventricular outflow obstruction was seen in seven patients. Right aortic arch with mirror image branching was seen in four patients. Coronary artery anomalies were seen in four patients. CONCLUSIONS: The presence of left atrioventricular valve atresia and patent aortic root with isomeric atrial appendages is a rare congenital anomaly frequently associated with anomalous systemic and pulmonary drainage, conotruncal anomalies, and right ventricular outflow obstruction. Hence, comprehensive CT-based evaluation is indispensable in providing detailed anatomy and aids in presurgical planning.

Evaluation of aortopulmonary window using virtual dissection of multidetector computed tomography angiography data sets.

AIM: The present study sought to evaluate the morphology and associated cardiovascular anomalies in patients with aortopulmonary window on virtual dissection of multidetector computed tomography (CT) angiography data sets. MATERIAL AND METHODS: We conducted a retrospective search of our departmental database from January 2014 to September 2021 to identify patients with aortopulmonary window and relevant information was extracted from the electronic case records and from routine examination as well as virtual dissection of CT data sets. RESULTS: An aortopulmonary window was observed in 26 patients (20 males; 6 females). Based on location of the defect, a distal aortopulmonary window was the most common subtype, seen in 13/26 (50%) patients followed by a proximal, complete and intermediate subtypes seen in 7/26 (27%), 5/26 (19%) and 1/26 (4%) patients respectively. Associated ventricular septal defect was observed in 9/26 (34.6%) patients while an interrupted aortic arch was present in 5/26 (19.2%) patients. Tetralogy of Fallot was seen in 5/26 (19.2%) patients. Anomalous origin of right pulmonary artery from ascending aorta and crossed pulmonary arteries were seen in 2/26 (7.6%) patients each. An isolated aortopulmonary window without any simple/complex congenital anomaly was seen in 10/26 (38.5%) patients. CONCLUSION: Aortopulmonary window is associated with a wide gamut of cardiovascular lesions, with ventricular septal defect being the commonest associated anomaly followed by tetralogy of Fallot and interrupted aortic arch respectively. Virtual dissection of multidetector CT angiography allows detailed anatomical evaluation of aortopulmonary window, allowing a clear visualization of the defect and associated cardiovascular anomalies.

Bicuspid aortic valve and anomalous origin of the right ventricular branch in a patient with congenitally corrected transposition of great arteries: A rare association.

We hereby present a case of congenitally corrected transposition of great arteries association of bicuspid aortic valve morphology and anomalous separate origin of the right ventricular branch of the right coronary artery. We aim to highlight the role of computed tomography angiography in identifying these rare associations.

Intrathymic course of left brachiocephalic vein: A hitherto undescribed anatomical variant in tetralogy of Fallot.

We hereby present a case of a unique intrathymic course of left brachiocephalic vein in a patient with Tetralogy of Fallot and aberrant right subclavian artery deciphered on computed tomography angiography.

A rare pediatric case of pedunculated cardiac hemangioma of the left ventricle.

A 12-year-old male child with atypical chest pain and fatigue since past 3 months underwent cardiac magnetic resonance (CMR) imaging for suspicious globular mobile mass detected in the left ventricle on a transthoracic echocardiography. CMR revealed peripheral vascular blush on the first pass perfusion images with centripetal progressive intensely bright enhancement on late gadolinium-enhanced images. Further confirmation and histological diagnosis of the tumor was made through transaortic aortic resection of the mass.

Isolated left common carotid artery: multidisciplinary management is the key to success.

An isolated carotid artery is a rare aortic arch anomaly. Instead of connecting to the aorta, the isolated carotid artery connects anomalously to the pulmonary artery. Chronically altered cerebral circulation poses the risk of cerebral hyperaemia following surgical reimplantation. We describe successful reimplantation of the isolated left common carotid artery in a child, highlighting the importance of a multidisciplinary approach for good clinical and neurological outcomes. We also briefly discuss the embryologic basis of this rare arch anomaly.