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.

A Phantom Lesion in the Left Main Coronary Artery: Concertina Effect.

Classically described in tortuous coronaries, the concertina effect is a type of pseudostenosis induced by guidewire and is extremely rare in the left main coronary artery because of its short length. However, concertina effect in the left main coronary artery after percutaneous intervention should be considered in the appropriate setting to avoid confounding management dilemmas and unwarranted interventions.

Aneurysmal and obstructive lesions of the left ventricular outflow: evaluation on multidetector computed tomography angiography.

The left ventricular outflow is an anatomically complex region situated between the anterior leaflet of the mitral valve and the left ventricular aspect of the muscular and membranous interventricular septum. It gives rise to the aorta, provides support to the aortic valvular cusps, and houses important components of the conduction system. The left ventricular outflow handles high pressures and pressure variations and is subsequently affected by a variety of aetio-pathological conditions. Diseases involving the left ventricular outflow can be intraluminal, mural, or extramural, and the consequent complications of the lesions can be local, loco-regional, or even systemic. Appropriate evaluation requires comprehensive multimodality imaging with each modality contributing to assessment of different aspects of diagnosis, lesion characterization, local extension, prognostication for systemic complications and mortality, and the decision for the approach and type of intervention and aggressive follow-up in case non-interventional management is decided. In this review, we briefly describe the relevant anatomy and the gamut of structural abnormalities pertaining to the left ventricular outflow on multidetector computed tomography angiography.

Stenotic lesions of aorta: Imaging evaluation using multidetector computed tomography angiography.

Aortic involvement can be secondary to various pathologic disease processes. These may result in stenotic or aneurysmal aortic lesions with a varied spectrum of imaging findings including intra-luminal, mural, and periaortic changes along with associated loco-regional or distal changes, depending on the etiology. Multidetector computer tomography with its recent advances has become the frontline imaging modality for the evaluation of aortic pathologies. Comprehensive evaluation of the aortic pathology with simultaneous evaluation of lungs, bones, and visceral organs is possible with a single multidetector computer tomography acquisition. It allows accurate primary diagnosis, identifies important anatomic landmarks and relationships, and identifies associated cardiovascular anomalies. Moreover, it serves as an adjunct in diagnosis of various complications, helps in treatment planning and detection of disease progression during follow-up.

Supravalvar aortic stenosis: Imaging characteristics and associations on multidetector computed tomography angiography.

AIM: To evaluate the imaging features and associations in patients with supravalvar aortic stenosis on multidetector computed tomography (CT) angiography. MATERIALS AND METHODS: We retrospectively reviewed all CT angiography studies performed for evaluation of congenital heart diseases at our institution through the period from January 2014 to June 2020. Cases with supravalvar aortic stenosis were identified and classified as syndromic and nonsyndromic based on history, physical examination, and relevant investigations. The type and extent of vascular involvement and associated cardiovascular abnormalities were characterized. RESULTS: Supravalvar aortic stenosis was identified in 26/3926 (0.66%) patients (22 males and 4 females; Age range: 2 months to 20 years). Discrete stenosis was seen in 14/26 (53.8%) patients, while diffuse involvement of the ascending aorta to varying degrees was seen in the remaining 12 (46.2%) patients. About 15/26 (57.7%) patients had pulmonary involvement at some level, namely, infundibular, valvar, supravalvar, or peripheral pulmonic stenosis while 15/26 (57.7%) patients had coronary arterial involvement either in the form of stenosis, occlusion, or ectasia. Aortic valvular abnormality including thickening, partial fusion, and adhesion of leaflet edges to the sinutubular junction causing reduced coronary inflow was seen in 15/26 (57.7%) patients. Associated ventricular septal defect, patent ductus arteriosus, and mitral valvular prolapse were seen in four (15.4%), five (19.2%), and two (7.7%) patients respectively. CONCLUSION: Supravalvar aortic stenosis is a rare abnormality showing associated pulmonary arterial involvement, coronary arterial involvement, aortic valvular abnormalities, and associated congenital cardiac defects in the majority of cases, which may influence surgical outcomes.

Randomized, Double-Blind, Placebo-Controlled Trial to Evaluate Safety and Therapeutic Efficacy of Angiogenesis Induced by Intraarterial Autologous Bone Marrow-Derived Stem Cells in Patients with Severe Peripheral Arterial Disease.

PURPOSE: To evaluate safety and efficacy of angiogenesis induced by intraarterial autologous bone marrow-derived stem cell (BMSC) injection in patients with severe peripheral arterial disease (PAD). MATERIALS AND METHODS: Eighty-one patients with severe PAD (77 men), including 56 with critical limb ischemia (CLI) and 25 with severe claudication, were randomized to receive sham injection (group A) or intraarterial BMSC injection at the site of occlusion (group B). Primary endpoints included improvement in ankle-brachial index (ABI) of > 0.1 and transcutaneous pressure of oxygen (TcPO2) of > 15% at mid- and lower foot at 6 mo. Secondary endpoints included relief from rest pain, > 30% reduction in ulcer size, and reduction in major amputation in patients with CLI and > 50% improvement in pain-free walking distance in patients with severe claudication. RESULTS: Technical success was achieved in all patients, without complications. At 6 mo, group B showed more improvements in ABI of > 0.1 (35 of 41 [85.37%] vs 13 of 40 [32.50%]; P < .0001) and TcPO2 of > 15% at the midfoot (35 of 41 [85.37%] vs 17 of 40 [42.50%]; P = .0001] and lower foot (37 of 41 [90.24%] vs 19 of 40 [47.50%]; P < .0001). No patients with CLI underwent major amputation in group B, compared with 4 in group A (P = .0390). No significant difference was observed in relief from rest pain or > 30% reduction in ulcer size among patients with CLI or in > 50% improvement in pain-free walking distance among patients with severe claudication. CONCLUSIONS: Intraarterial delivery of autologous BMSCs is safe and effective in the management of severe PAD.

Isolation of arch vessels: Imaging characteristics and associations on multidetector computed tomography angiography.

AIM: To evaluate the imaging characteristics and associations in patients with isolation of arch vessels on multidetector computed tomography angiography (CTA). MATERIALS AND METHODS: We retrospectively reviewed all multidetector CTA studies performed for the evaluation of congenital heart diseases (CHDs) at our institution from January 2014 to June 2020. Cases with isolation of arch vessels were identified. The isolated arch artery and its relationship with patent arterial duct, pulmonary artery, and aortic arch were characterized in addition to other associated intra- and extracardiac anomalies. RESULTS: Isolation of arch vessels was seen in 14/3926 (0.36%) patients. Left subclavian artery (SCA) was the commonest isolated arch vessel, involved in 7/14 (50%) cases. Isolation of right SCA, left brachiocephalic artery, and left common carotid artery was seen in 4 (28.6%), 2 (14.3%), and 1 (7.1%) patient, respectively. The isolated arch vessel was seen associated with right aortic arch in 10/14 (71.4%) cases and was on the opposite side of aortic arch in all 14 (100%) patients. Right-sided nonrestrictive patent arterial duct was seen in 3/14 (21.4%) cases, left-sided nonrestrictive patent arterial duct was seen in 1/14 (7.1%) while a left-sided restrictive patent arterial duct was seen in 3/14 (21.4%) cases. Tetralogy of Fallot (ToF) was the commonest associated anomaly seen in 8/14 (57.1%) patients. CONCLUSION: Isolation of aortic arch branch vessels is rare, seen most commonly associated with ToF. Left SCA is the commonest involved vessel. CTA is useful not only in the diagnosis of isolation of arch vessels, but also in the presence or absence of associated anomalies which may impact the symptomatology, prognosis, and surgical management.

Imaging characteristics and associations in twisted atrioventricular connections on multidetector computed tomography angiography.

AIM: To evaluate the imaging characteristics and associations in patients with twisted atrioventricular connections on multidetector computed tomography (CT) angiography. MATERIAL AND METHODS: We retrospectively reviewed 2605 CT angiography studies performed for suspected congenital heart diseases in our institution from January 2014 to December 2018. Twisted atrioventricular connections were diagnosed in 12 patients. Segmental sequential approach developed by Anderson et al was employed to characterize the complex congenital heart disease. Ventricular topology was also determined. CTA was also assessed to look for any associated intra- and extracardiac anomalies. RESULTS: Out of 12 patients with twisted atrioventricular connections, usual viscero-atrial arrangement was seen in nine patients, two patients had mirror-imaged viscero-atrial arrangement and one patient had mirror-imaged atrial arrangement. Right-sided heart was seen in four patients. Two patients had discordant atrioventricular connections, seven had concordant atrioventricular connections while three showed double-inlet right ventricle. Ventriculoarterial connections were concordant in two patients, discordant in one patient while nine patients had double-outlet right ventricle. Superior-inferior ventricular morphology with near-horizontal interventricular septum was seen in four patients. Left-sided aortic arch with normal branching pattern was observed in nine patients. No coronary anomaly was seen in any patient. Other associations included ventricular and atrial septal defects, pulmonic stenosis, hypoplasia/atresia of tricuspid valve, straddling of mitral valve, and duplicated superior caval veins. CONCLUSION: CT angiography is useful in diagnosis of twisted atrioventricular connections with accurate identification of viscero-atrial arrangement, atrioventricular and ventriculoarterial connections, and orientation and presence or absence of associated anomalies.

Thoracoatrial discordance in a case of twisted atrioventricular connections: A unique configuration of visceroatrial arrangement.

We present a case of a 12-year-old boy with discordance between the atrial arrangement and the thoracoabdominal arrangement in the setting of twisted atrioventricular connections. This case highlights the importance of a separate description of all visceral organs and venous drainage for an accurate description of visceroatrial arrangement in cases of congenital heart defects.

Anomalous origin of left vertebral artery from descending thoracic aorta: A rare variant.

We present a case of a 4-year-old child operated previously for cyanotic congenital heart disease where the follow-up computed tomography (CT) angiography revealed anomalous origin of the left vertebral artery from the descending thoracic aorta. This case also highlights the potential implications of this variant and the value of CT angiography in diagnosing this anomaly.

An exceedingly rare association of an isolated right subclavian artery with transposition of great vessels.

Isolation of the right subclavian artery (RSCA), defined as loss of continuity between the RSCA and aorta, is a rare anomaly and its association with dextrotransposition of great arteries (D-TGA) is exceedingly unusual. We present the case of a 5-day-old neonate with cyanosis from birth and a transthoracic echocardiographic diagnosis of D-TGA, where on computed tomography angiography, the RSCA was seen arising from the proximal part of the ipsilateral pulmonary artery via a patent right ductus arteriosus.

Incidence of systemic venous anomalies in tetralogy of Fallot on multidetector CT angiography: A retrospective single-center study of 973 patients.

OBJECTIVES: The present study sought to estimate the incidence and evaluate the patterns and associations of systemic venous anomalies in patients with tetralogy of Fallot on multidetector computed tomography (CT) angiography. METHODS: We retrospectively reviewed CT angiographies of 973 pediatric age group patients diagnosed with tetralogy of Fallot, performed at our institution between 1st January 2015 and 31st December 2019, for systemic venous drainage patterns. We also proposed a structured classification and reporting system on CT angiography which would simplify the characterization, classification, and communication of these anomalies. RESULTS: Variations in systemic venous drainage patterns were observed in 146 (15.01%) patients. The most commonly observed anomaly was persistence of left superior caval vein, which was seen in 112 (11.51%) patients, with the most prevalent pattern being the presence of bilateral superior caval veins with no intercommunicating vein (n = 85). The second most common anomaly observed was presence of anomalous brachiocephalic vein (39/973 [4.01%]). Patients with an anomalous course of brachiocephalic vein had significantly higher prevalence of a right aortic arch than those without (23/39 [58.97%] vs 188/934 [20.13%]; P < .0001). Similarly, a higher prevalence of pulmonary atresia was noted in patients with an anomalous course of brachiocephalic vein compared to those without (8/39 [20.51%] vs 80/934 [8.57%]; P = .0109). CONCLUSION: There is a high incidence of anomalies of superior caval veins and brachiocephalic vein in the presence of tetralogy of Fallot. With advancements in corrective cardiac surgery and use of intraoperative cardiopulmonary bypass, the presence of systemic venous anomalies assumes greater significance making preoperative identification of these anomalies imperative.

Correction to: Etiology, Diagnosis and Management of Aortitis.

In the original article, the section "Fact Sheet" was not published. This section should give the reader an overview on the most important take-home messages on aortitis. Please see below the missing section.

Absent left common carotid artery in a right aortic arch: Separate origins of left internal and external carotid arteries from brachiocephalic artery.

We present a case of cyanotic congenital heart disease with left common carotid artery agenesis in the setting of the right aortic arch highlighting the potential implications in management.

Etiology, Diagnosis and Management of Aortitis.

Aortitis includes conditions with infectious or non-infectious etiology, characterized by inflammatory changes in one or more layers in aortic wall. Age at onset, geographic predilections, distribution and pattern of involvement in aorta, its branches and pulmonary arteries, and systemic associations provide a clue to etiology. Clinical presentations are often non-specific. An integrated approach including clinical, laboratory and imaging assessment is essential to confirm diagnosis and plan treatment. Assessment of disease activity is the key as it influences timing and outcome of treatment. Markers of activity include clinical, laboratory and imaging. Medical management remains the first-line therapy. Revascularization is indicated in the presence of hemodynamically significant stenosis and inactive disease. In the presence of flash pulmonary edema, left ventricular dysfunction or hypertensive encephalopathy, revascularization is performed irrespective of disease activity. Endovascular management is favored over surgery due to its high success and low restenosis rates. Symptomatic aneurysmal disease is usually managed by surgery.

A two-vessel aortic arch: Rare configuration with bilateral brachiocephalic arteries.

We present a case of type B aortic dissection with a rare aortic arch branching variation whereby two separate brachiocephalic trunks arise from the arch. This case also highlights the potential implications of this variant in the management of thoracic aortic dissections and aneurysms.