Techniques and pitfalls of coronary arterial reimplantation in anatomical correction of transposition.

BACKGROUND AND AIM: We assessed the anatomical variations in coronary arterial patterns relative to the techniques of reimplantation in the setting of the arterial switch operation, relating the variations to influences on outcomes. METHODS: We reviewed pertinent published investigations, assessing events reported following varied surgical techniques for reimplantation of the coronary arteries in the setting of the arterial switch procedure. RESULTS: The prevalence of reported adverse events, subsequent to reimplantation, varied from 2% to 11%, with a bimodal presentation of high early and low late incidence. The intramural pattern continues to contribute to mortality, with some reports of 28% fatality. The presence of abnormal course relative to the arterial pedicles in the setting of single sinus origin was associated with a three-fold increase in mortality. Abnormal looping with bisinusal origin of arteries was not associated with increased risk. CONCLUSION: The techniques of transfer of the coronary arteries can be individually adapted to cater for the anatomical variations. Cardiac surgeons, therefore, need to be familiar with the myriad creative options available to achieve successful repair when there is challenging anatomy. Long-term follow-up will be required to affirm the superiority of any specific individual technique. Detailed multiplanar computed-tomographic scanning can now reveal all the variants, and elucidate the mechanisms of late complications. Coronary angioplasty or surgical revascularization may be considered in selected cases subsequent to the switch procedure.

A rare configuration of single coronary artery: Anomalous anterior interventricular artery arising from right coronary artery with absent left circumflex artery.

We report a case of a 49-year-old woman planned mitral valve replacement surgery where computerized tomography angiography incidentally detected a rare configuration of a single coronary artery resulting from a combination of anomalous origin of anterior interventricular artery from right coronary artery and absent left circumflex artery.

A reassessment of the anatomical features of multiple ventricular septal defects.

BACKGROUND: Over the course of time, new developments associated with the embryogenesis of the murine heart have served to clarify the developmental processes observed in the human heart. This evidence allows for the creation of a developmental framework for many congenital cardiac defects. AIMS: We aim to solidify the framework related to the categorization of both solitary and multiple ventricular septal defects. MATERIALS AND METHODS: Mice having genetic perturbation of the Furin enzyme have demonstrated perimembranous and juxta-arterial ventricular septal defects, permitting the inference to be made that these defects can co-exist with defects occurring within the apical muscular septum. RESULTS: Basis of developmental evidence, furthermore, all interventricular communications can be placed into one of three groups, namely those which are perimembranous, juxta-arterial, and muscular. All of the defects are described based on their borders as seen from the morphologically right ventricle. Our focus here will be on those defects within the muscular ventricular septum, recognizing that such defects can co-exist with those that are perimembranous. We discuss the differentiation of multiple discrete defects from those referred to as the "Swiss cheese" variant. CONCLUSIONS: As we show, appropriate surgical management requires an understanding of the specific terminology, as the surgical approach may differ depending on the combination of the individual defects. Data from the Society for Thoracic Surgeons revealed that both mortality and morbidity were increased in the setting of multiple as opposed to solitary ventricular septal defects.

A review of the therapeutic management of multiple ventricular septal defects.

BACKGROUND AND AIM: We showed in our anatomical review, ventricular septal defects existing as multiple entities can be considered in terms of three major subsets. We address here the diagnostic challenges, associated anomalies, the role and techniques of surgical instead of interventional closure, and the outcomes, including reinterventions, for each subset. METHODS: We reviewed 80 published investigations, noting radiographic findings, and the results of clinical imaging elucidating the location, number, size of septal defects, associated anomalies, and the effect of severe pulmonary hypertension. RESULTS: Overall, perioperative mortality for treatment of residual multiple defects has been cited to be between 0% and 14.2%, with morbidity estimated between 6% and 13%. Perioperative mortality is twice as high for perimembranous compared to muscular defects, with the need for reoperation being over four times higher. Perventricular hybrid approaches are useful for the closure of high anterior or apical defects. Overall, the results have been unsatisfactory. Pooled data reveals incidences between 2.8% and 45% for device-related adverse events. Currently, however, outcomes cannot be assessed on the basis of the different anatomical subsets. CONCLUSIONS: We have addressed the approaches, and the results, of therapeutic treatment in terms of coexisting discrete defects, the Swiss-cheese septum, and the arrangement in which a solitary apical muscular defect gives the impression of multiple defects when viewed from the right ventricular aspect. Treatment should vary according to the specific combination of defects.

Surgical management of hearts with isomeric atrial appendages.

BACKGROUND AND AIM: On the basis of previously published accounts, coupled with our own experience, we have assessed the surgical approaches to patients with isomeric atrial appendages. METHODS: We reviewed pertinent published studies on surgical treatment of individuals with isomeric atrial appendages, with the pertinent surgical details provided by most of the manuscripts. RESULTS: Half of patients with right isomerism, and two-thirds of those with left isomerism have bilateral superior caval veins. Azygos extension of the inferior caval vein is reported in three-quarters of those with left isomerism. The coronary sinus is universally absent in right isomerism, along with totally anomalous pulmonary venous connection, and is absent in two-fifths of those with left isomerism. Univentricular atrioventricular connections are expected in up to three-quarters of those with right isomerism. Atrioventricular septal defect is reported in up to four-fifths, more frequently in right isomerism, with such patients typically having discordant ventriculoatrial connections or double outlet right ventricle. Reported mortalities extend to 85% for those with right, and 50% for those with left isomerism. In right isomerism, mortality is up to 54% for systemic-to-pulmonary arterial shunting, up to 75% for univentricular repair, and up to 95% for repair of totally anomalous pulmonary venous connection itself. No more than one-quarter had undergone Fontan completion, with reported mortalities of 21%. CONCLUSION: Early surgical results are satisfactory in patients with left isomerism, but disappointing for those with right. Recent advances in cardiac and liver transplantation may offer improved survival.

Surgical management of lesions encountered in the setting of the retroaortic left brachiocephalic vein.

BACKGROUND AND AIM: Although the retroaortic left brachiocephalic vein in isolation is of no clinical importance, its recognition in the setting of associated lesions is important. We sought to address issues concerning the influence of isomerism, the establishment of diagnosis, and its importance in various surgical and interventional procedures. METHODS: A total of 80 published clinical and necropsy studies in the setting of a retroaortic left brachiocephalic vein described 250 patients. Clinical presentation, radiographic, ultrasonographic findings, contrast echocardiography, computed-tomographic angiocardiography, magnetic resonance imaging, and angiocardiography provided the diagnostic information prior to considering the surgical approach to the associated cardiac anomalies. RESULTS: Among 250 reported cases, three-quarters had associated congenitally malformed hearts. Of these 189 patients, all but seven had usual atrial arrangement. Right isomerism was reported in five patients and two patients having left isomerism. Almost two-thirds had tetralogy of Fallot or its variants, over four-fifths had malformations involving the outflow tract, two-thirds had a right aortic arch with two patients having a cervical aortic arch, and onepatient had double aortic arch. Various innovative individualized surgical procedures were employed with an overall perioperative mortality of 3.4%. CONCLUSIONS: Although the retroaortic left brachiocephalic vein is asymptomatic, its recognition during clinical investigation should raise the possibility of an association with other malformations, especially right aortic arch, ventricular septal defect, and anomalies of the outflow tracts. We submit that an increased appreciation of this venous anomaly may facilitate surgical planning, endovascular procedures, placement of central venous lines, and transvenous pacemakers.

Surgical management of divided atrial chambers.

BACKGROUND AND AIM: The morphological variations when one, or both, of the atrial chambers is subdivided, are many and varied. We sought to address clinical presentations, potentially misdiagnosed cases, diagnostic modalities, surgical approaches, and outcomes of this "family" of uncommon lesions. METHODS: A total of 193 published investigations were synthesized. Diagnostic information was provided by clinical presentation, and multimodality imaging studies. RESULTS: Almost three-quarters of patients with divided atrial chambers present during infancy with severe pulmonary hypertension and cardiac failure. Associated cardiac and extra-cardiac defects are present in between half and nine-tenths of cases. Acquired division of the left atrium has been reported after the Fontan operation, orthotopic cardiac transplantation, and complicated aortic valvar infective endocarditis. Surgery under cardiopulmonary bypass remains the definitive treatment. Balloon dilation may be considered in anatomically compatible variants in the setting of cardiac failure and pregnancy as a bridge todefinitive treatment. Overall, mortality has been cited between nil to 29%. Presentation during infancy, associated congenital anomalies, pulmonary hypertension, and surgery in the previous era, have been the reported causes of death. The operative survivors have long-term favourable outcomes, with near normal cardiac dimensions and low risk of recurrence. While asymptomatic patients with division of the right atrium do not need treatment, surgical resection of the dividing partition under cardiopulmonary bypass is recommended in symptomatic patients with complex anatomy, the spinnaker malformation, or associated cardiac anomalies. Balloon dilation may be considered in uncomplicated patients with less obstructive lesions. Hybrid intervention and endoscopic robotic correction also have been performed. CONCLUSIONS: Resection of the dividing shelf allows the survivors to regain near normal dimensions with a low risk of recurrence. We submit that an increased appreciation of the anatomic background to division of the atrial chambers will contribute to improved surgical management.

Surgical management of the scimitar syndrome.

BACKGROUND AND AIM: We sought to address the varied anatomical details, the diagnostic challenges, associated cardiopulmonary anomalies, the techniques, and outcomes of management, including re-interventions of scimitar syndrome. METHODS: A total of 92 published investigations of scimitar syndrome were reviewed. Diagnostic information was provided by clinical presentations, radiographic findings, transthoracic and transesophageal echocardiography, computed-tomographic angiography, magnetic resonance imaging, angiocardiography, and ventilation/perfusion scans. These investigations served to elucidate the origin, course, and termination of the scimitar vein, the intracardiac anatomy, the presence of associated defects, and the patterns of any accompanying pulmonary lesions prior to surgical intervention. RESULTS: Of the patients described, up to four-fifths presented during infancy, with cardiac failure, increased pulmonary flow, and pulmonary hypertension. Associated cardiac and extracardiac defects, particularly hypoplasia of the right lung, are present in up to three-quarters of cases. Overall operative mortality has been cited between 4.8% and 5.9%. Mortality was highest in patients with preoperative pulmonary hypertension, and those undergoing surgery in infancy. Despite timely surgical intervention, post-repair obstruction of the scimitar vein, intra-atrial baffle obstruction, or stenosis of the inferior caval vein were reported in up to two-thirds of cases. The venous obstruction could not be related to any particular surgical technique. On long term follow-up, one sixth of patients reported persistent dyspnoea and recurrent respiratory infections. CONCLUSIONS: Any infants presenting with heart failure, right-sided heart, and hypoplastic right lung should be evaluated to exclude the syndrome. An increased appreciation of variables will contribute to improved surgical management.

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.

Pulmonary-to-systemic venous collateral in obstructed supracardiac totally anomalous pulmonary venous connection: Blood finds a way!

We describe a rare variant of obstructed supracardiac totally anomalous venous connection in a 7-day-old boy where the vertical vein was occluded and the entire pulmonary venous return was draining through a large tortuous pulmonary-systemic venous collateral. The case highlights the importance of computed tomography angiography in demonstrating such complex anatomies in congenital heart diseases.

Anomalous branch of right pulmonary artery supplying the left lung: A "pseudo-pulmonary sling".

We report a case of a 9-month-old boy with supracardiac total anomalous pulmonary venous connection showing a small aberrant vessel arising from the right pulmonary artery and traversing below the left main bronchus to supply the anteromedial segment of the left lower lobe.

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.

An unusual pediatric case of tuberculosis-associated mediastinal fibrosis with concomitant pulmonary arterial and venous occlusion.

We present a case of a 4-year-old boy with tuberculosis-associated mediastinal fibrosis with concomitant pulmonary arterial and venous occlusion, highlighting the role computed tomography angiography in identification of the disease process, coexistent vascular abnormalities and associated complications.

Cardiac Magnetic Resonance Imaging in Coronavirus Disease 2019 (COVID-19): A Systematic Review of Cardiac Magnetic Resonance Imaging Findings in 199 Patients.

OBJECTIVE: Cardiac magnetic resonance imaging (CMR) with its new quantitative mapping techniques has proved to be an essential diagnostic tool for detecting myocardial injury associated with coronavirus disease 2019 (COVID-19) infection. This systematic review sought to assess the important imaging features on CMR in patients diagnosed with COVID-19. MATERIALS AND METHODS: We performed a systematic literature review within the PubMed, Embase, Google Scholar, and WHO databases for articles describing the CMR findings in COVID-19 patients. RESULTS: A total of 34 studies comprising 199 patients were included in the final qualitative synthesis. Of the CMRs 21% were normal. Myocarditis (40.2%) was the most prevalent diagnosis. T1 (109/150; 73%) and T2 (91/144; 63%) mapping abnormalities, edema on T2/STIR (46/90; 51%), and late gadolinium enhancement (LGE) (85/199; 43%) were the most common imaging findings. Perfusion deficits (18/21; 85%) and extracellular volume mapping abnormalities (21/40; 52%), pericardial effusion (43/175; 24%), and pericardial LGE (22/100; 22%) were also seen. LGE was most commonly seen in the subepicardial location (81%) and in the basal-mid part of the left ventricle in inferior segments. In most of the patients, ventricular functions were normal. Kawasaki-like involvement with myocardial edema without necrosis/LGE (4/6; 67%) was seen in children. CONCLUSION: CMR is useful in assessing the prevalence, mechanism, and extent of myocardial injury in COVID-19 patients. Myocarditis is the most common imaging diagnosis, with the common imaging findings being mapping abnormalities and myocardial edema on T2, followed by LGE. As cardiovascular involvement is associated with poor prognosis, its detection warrants prompt attention and appropriate treatment.

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.

Separate arch origin of the left external carotid artery with common trunk giving rise to the left internal carotid artery and left subclavian artery.

We present a case of a 3-year-old boy with tetralogy of Fallot having the direct origin of the left external carotid artery from the aorta with a common trunk giving rise to the left internal carotid artery and left subclavian artery, in a right-sided aortic arch. We also highlight the potential implications in management.

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.

Unilateral pulmonary aplasia and congenital diaphragmatic hernia associated with tetralogy of Fallot: a rare trifecta.

We present a case of a 7-month-old boy with tetralogy of Fallot associated with unilateral pulmonary aplasia and herniation of the liver and small bowel loops in the right hemithorax.