Clarifying the Anatomy of Tetralogy of Fallot with S-shaped Ascending Aorta.

We recently encountered several cases of tetralogy of Fallot with an abnormally oriented S-shaped ascending aorta. In this retrospective study, we sought to clarify morphology of this unusual under-recognized variant. Databases were reviewed to identify all patients with tetralogy of Fallot having an S-shaped ascending aorta. Computed tomographic angiography was used for the assessment of cardiac morphology. Out of the 21 patients, 18 (86%) had a right aortic arch, 2 (9%) had a left aortic arch, and the remaining patient (5%) had a double aortic arch. Patients with a right aortic arch, compared to age and sex-matched patients with a right aortic arch but normally oriented ascending aorta, had lesser aortic override (29.3 ± 14% vs 54.8 ± 13.2%; p = 0.0001) and a wider ascending aorta (25.2 ± 6.9 vs 18.0 ± 3.2 mm; p = 0.0003). The S-shaped ascending aorta was located posteriorly, with a higher sterno-aortic distance (25.5 ± 7.7 vs 9.9 ± 4.5 mm; p = 0.0001). The ascending aorta among patients with tortuosity was longer (4.12 ± 1.7 vs 3.07 ± 0.82, p = 0.03) but with similar tortuosity index (1.22 ± 0.19 vs 1.15 ± 0.17, p = 0.23). Of the cases with right aortic arch and S-shaped ascending aorta, 16 (89%) had extrinsic compression of the right pulmonary artery (p = 0.0001), while 7 (39%) had crossed pulmonary arteries (p = 0.008), with no such findings among those with normally oriented ascending aorta. Tetralogy of Fallot with an S-shaped ascending aorta is a variant with lesser aortic override and a more posteriorly located ascending aorta. Compression of the right pulmonary artery and crossed pulmonary arteries is frequent in the presence of a right-sided aortic arch. These findings have important implications for optimal diagnosis and surgical repair.

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.

Pulmonary arterial compliance in patients of CHD with increased pulmonary blood flow.

INTRODUCTION: Pulmonary arterial compliance, the dynamic component of pulmonary vasculature, remains inadequately studied in patients with left to right shunts. We sought to study the pulmonary arterial compliance in patients with left to right shunt lesions and its utility in clinical decision-making. MATERIALS AND METHODS: In this single-centre retrospective study, we reviewed cardiac catheterisation data of consecutive patients of left to right shunt lesions catheterised over one year. In addition to the various other parameters, pulmonary arterial compliance was calculated, as indexed pulmonary flow (Qpi) / (Heart rate × pulse pressure in the pulmonary artery). RC time was also calculated, as the product of pulmonary arterial compliance and pulmonary vascular resistance index. Patients were divided into "operable," "borderline," and "inoperable" based on the decision of the treating team, and the pulmonary arterial compliance values were evaluated in these groups to study if it can be utilised to refine the operability decision. RESULTS: 298 patients (Median age 16 years, 56% <18 years) with various acyanotic shunt lesions were included. Overall, the pulmonary arterial compliance varied with Qpi, pulmonary artery mean pressure, and pulmonary vascular resistance index, but did not vary with age, type of lesion, or transpulmonary gradients. The median pulmonary arterial compliance in patients with normal pulmonary artery pressure (Mean pulmonary artery pressure less than 20 mmHg) was 4.1 ml/mmHg/m2 (IQR 3.2). The median pulmonary arterial compliance for operable patients was 2.67 ml/mmHg/m2 (IQR 2.2). Median pulmonary arterial compliance was significantly lower in both inoperable (0.52 ml/mmHg/m2, IQR 0.34) and borderline (0.80 ml/mmHg/m2, IQR 0.36) groups when compared to operable patients (p < 0.001). A pulmonary arterial compliance value lower than 1.18 ml/mmHg/m2 identified inoperable patients with high sensitivity and specificity (95%, AUC 0.99). However, in borderline cases, assessment by this value did not agree with empirical clinical assessment.The median RC time for the entire study population was 0.47 S (IQR 0.30). RC time in operable patients was significantly lower than that in the inoperable patients (Median 0.40 IQR 0.23 in operable, 0.73 0.25 in inoperable patients (p < 0.001). CONCLUSIONS: Addition of pulmonary arterial compliance to the routine haemodynamic assessment of patients with shunt lesions may improve our understanding of the pulmonary circulation and may have clinical utility.

Outcomes of multisystem inflammatory syndrome in children temporally related to COVID-19: a longitudinal study.

To study the clinical, laboratory characteristics and outcomes of multisystem inflammatory syndrome in children (MIS-C) temporally related to coronavirus disease 2019 (COVID-19) in a resource-limited setting. All children meeting the World Health Organization case definition of MIS-C were prospectively enrolled. Baseline clinical and laboratory parameters were compared between survivors and non-survivors. Enrolled subjects were followed up for 4-6 weeks for evaluation of cardiac outcomes using echocardiography. The statistical data were analyzed using the stata-12 software. Thirty-one children with MIS-C were enrolled in an 11-month period. Twelve children had preexisting chronic systemic comorbidity. Fever was a universal finding; gastrointestinal and respiratory manifestations were noted in 70.9% and 64.3%, respectively, while 57.1% had a skin rash. Fifty-eight percent of children presented with shock, and 22.5% required mechanical ventilation. HSP like rash, gangrene and arthritis were uncommon clinical observations.The median duration of hospital stay was 9 (6.5-18.5) days: four children with preexisting comorbidities succumbed to the illness. The serum ferritin levels (ng/ml) [median (IQR)] were significantly higher in non-survivors as compared to survivors [1061 (581, 2750) vs 309.5 (140, 720.08), p value = 0.045]. Six patients had coronary artery involvement; five recovered during follow-up, while one was still admitted. Twenty-six children received immunomodulatory drugs, and five improved without immunomodulation. The choice of immunomodulation (steroids or intravenous immunoglobulin) did not affect the outcome. Most children with MIS-C present with acute hemodynamic and respiratory symptoms.The outcome is favorable in children without preexisting comorbidities.Raised ferritin level may be a poor prognostic marker. The coronary outcomes at follow-up were reassuring.

A rare case of infective endocarditis in association with ventricular septal defect complicated by mycotic pulmonary artery aneurysm.

We present a case of a 3-year-old child with mycotic aneurysm of left descending pulmonary artery secondary to infective endocarditis in the setting of ventricular septal defect. The case highlights the role of CT angiography in the diagnosis and characterization of the aneurysm and in demonstrating the extent of thrombo-embolic complications in distal pulmonary arteries and lung parenchyma.

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.

Evaluation of cardiovascular morphology and associated anomalies in patients with crossed pulmonary arteries on multidetector computed tomography angiography.

AIM: The present study sought to study morphology and associated cardiovascular anomalies in patients with crossed pulmonary arteries on multidetector computed tomography (CT) angiography. MATERIALS AND METHODS: We retrospectively evaluated all CT angiography studies performed at a tertiary medical center from January 2014 to December 2021 to identify patients with crossed pulmonary arteries. The associated cardiovascular anomalies as identified on CT angiography were evaluated. RESULTS: Out of 4773 patients who had undergone CT angiography for evaluation of congenital heart diseases, we identified 24 (0.5%) patients (18 males; mean age: 7.7 years) with crossed pulmonary arteries. Tetralogy of Fallot was associated in seven (29.17%) patients, double outlet right ventricle in one (4.17%) patient, and common arterial trunk in four (16.67%) patients. An aortopulmonary window was seen in three (12.5%) patients while atrial septal defect and ventricular septal defect were seen in two (8.33%) and 16 (66.67%) patients, respectively. Aortic arch anomalies were present in 16 (66.67%) patients including interrupted aortic arch and coarctation of aorta with hypoplastic aortic arch seen in two (8.33%) patients each. A double aortic arch with an atretic left arch was seen in one (4.17%) patient. Coronary artery anomalies were seen in three (12.5%) patients. CONCLUSION: Crossed pulmonary arteries are a rare anomaly and their presence suggests the coexistence of a variety of cardiovascular anomalies, including aortic arch anomalies and outflow tract malformations. Comprehensive CT angiography-based evaluation of cardiovascular morphology is imperative in the presence of crossed pulmonary arteries to facilitate presurgical planning.

Left pulmonary veins draining into persistent left superior caval vein in presence of unroofed coronary sinus.

We report a case of 5-year-old girl with double outlet right ventricle with a rare combination of partial anomalous pulmonary venous drainage into a persistent left superior caval vein in the presence of an unroofed coronary sinus while highlighting its possible embryological origins and therapeutic implications.

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.

Total transseptal course of anomalous anterior interventricular artery arising from the right coronary aortic sinus.

We report a case of a 4-year-old girl with tetralogy of Fallot where computed tomography angiography revealed an anomalous origin of anterior interventricular artery from the right coronary aortic sinus with total transseptal course up to its termination.

Common arterial trunk with crossed pulmonary arteries.

We report a case of a 27-day-old girl with an unusual association of crossed pulmonary arteries with common arterial trunk showing pulmonary dominance with an interruption of aortic arch between the origin of the left common carotid artery and left subclavian artery. This case highlights the potential airway-related implications of this variant pulmonary arterial anatomy in the setting of the common arterial trunk and the role of computed tomography angiography in providing not only accurate and three-dimensional visualization of complex cardiovascular anatomy, but also a comprehensive evaluation of associated airway abnormalities with great precision.

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.

Aberrant origin of right vertebral artery from right common carotid artery in a right-sided aortic arch.

We present a case of a 2-year-old girl with incidentally detected aberrant origin of right vertebral artery from right common carotid artery in a right-sided aortic arch. The case highlights the role of computed tomography angiography in detecting anomalies of the aortic arch and arch vessels in the setting of complex congenital heart diseases while also discussing the potential clinical implications.

Separate origin of all three coronary arteries from a single sinus of Valsalva.

We present a case of a 6-year-old boy with a double outlet right ventricle where the independent origin of all three coronary arteries from a single sinus of Valsalva was incidentally detected on CT angiography. The case highlights the role of CT angiography in identifying coronary arterial anomalies in the setting of complex congenital heart diseases.

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.

An unusual case of aortopulmonary window with crossed pulmonary arteries.

We report a case of a 4-year-old boy with a distal type of aortopulmonary window with crossed arrangement of the pulmonary arteries. This case highlights the potential clinical implications of this variant pulmonary arterial anatomy in the setting of the aortopulmonary window and the role of computed tomography angiography in accurately defining vascular relationships in patients with complex congenital cardiac defects.