Idiopathic focal fibrosing mediastinitis with unilateral pulmonary arterial and venous involvement.

We report a case of a 1-year-old girl where computed tomography (CT) angiography demonstrated presence of ill-defined heterogenous soft tissue thickening obliterating the mediastinal fat planes in right peri-hilar region and encasing the hilar right pulmonary artery and its segmental branches leading to marked narrowing with nonvisualization of the right-sided pulmonary veins. The case highlights the features of fibrosing mediastinitis as well as the role of CT angiography in the diagnosis and accurate depiction of the extent of involvement of the mediastinal vasculature and airways.

Superior sinus venosus defect with partial anomalous pulmonary venous return in a patient with tetralogy of Fallot.

We report a case of a 2-year-old boy with tetralogy of Fallot-pulmonary atresia where CT angiography additionally demonstrated the presence of superior sinus venosus defect with partial anomalous pulmonary venous return. Our case highlights the role of CT angiography in the preoperative evaluation in patients with tetralogy of Fallot in identifying such uncommon anomalies, which would help avoid postoperative complications and re-exploration in these patients.

Separate origin of left external and internal carotid arteries directly from aortic arch in a patient with double outlet right ventricle.

We report a case of a 1-year-old boy with double outlet right ventricle where computed tomography (CT) angiography incidentally demonstrated the separate origin of the left external and internal carotid arteries directly from the left-sided aortic arch with absence of a left common carotid artery. The case highlights the underlying embryological hypothesis as well as the importance of CT angiography in identifying anomalies of the aortic arch and arch vessels in the setting of complex congenital heart defects.

Levoatriocardinal vein in a patient with transposition of great arteries and pulmonary stenosis.

We report a case of a 3-month-old boy transposition of great arteries, ventricular septal defect, and pulmonary stenosis where computed tomography (CT) angiography detected a "levoatriocardinal vein" connecting the left superior pulmonary vein and the left brachiocephalic vein with a preserved normal connection between the left superior pulmonary vein and left atrium. This case highlights the hemodynamic implications of a "levoatriocardinal vein" as well as the role of CT angiography in detecting such associated cardiovascular anomalies in patients with complex congenital heart 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.

A reappraisal of the sinus venosus defect.

OBJECTIVES: The phenotypic features and morphogenesis of the 'sinus venosus defect' remain controversial. The phenotypic features are anomalous systemic connections of 1 or more pulmonary veins that retain their left atrial connection, usually associated with a biatrial connection of the superior caval vein. Cases with these features, however, have not always been described as sinus venosus defects. METHODS: We reviewed the findings documented in the literature from 11 patients with a biatrial connection of the superior caval vein, most reported following an autopsy examination. We compared these findings with the anatomical details of 50 patients undergoing surgical correction in our centre, paying particular attention to the override of the superior caval vein. RESULTS: In only two-thirds of those undergoing surgery did the superior caval vein override the rims of the oval fossa, with the degree of override >50% in only 2 individuals. It is only these latter 2 cases that are directly comparable to the reported cases of biatrial connection of the superior caval vein. CONCLUSIONS: Our comparisons provide new insights into the developmental background and phenotypic features of the superior sinus venosus defect. The defects exist because of the anomalous systemic connection of the pulmonary veins that retain their left atrial connections but not always in association with a biatrial connection of the superior caval vein. In extreme cases, nonetheless, they can underscore the connection of the caval vein to the morphologically left atrium, frequently described previously as a 'biatrial connection'. The sinus venosus defect is better considered a venovenous malformation than a septal defect.

Extremely tortuous vertical vein in supracardiac totally anomalous pulmonary venous connection.

We present a case of a 2-month-old boy with supracardiac totally anomalous pulmonary venous connection where computed tomography angiography demonstrated an extremely tortuous course of the vertical vein before its drainage into the left brachiocephalic vein.

Diphtheritic myocarditis: An unusual and reversible cause of heart failure.

Diphtheria is a life-threatening infectious disease caused by Corynebacterium diphtheriae. Although the disease is seen infrequently in the postvaccination era, sporadic cases continue to occur. Cardiac involvement, in the form of myocarditis, is the most serious manifestation of diphtheria and is the most common cause of mortality in these patients. The features of diphtheritic myocarditis on cardiac magnetic resonance imaging (MRI) have not been reported previously. In this brief report, we describe the cardiac MRI and histopathologic features on endomyocardial biopsy of a patient with acute heart failure who was later diagnosed to be a case of diphtheritic myocarditis.

Unique Association of Aortopulmonary Window With Anomalous Origin of Left Coronary Artery From Pulmonary Artery in an Infant: A Blessing in Disguise?

Aortopulmonary window (APW) seen in association with anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is extremely uncommon. When faced with this combination, ALCAPA usually goes undetected since most of the clinical and echocardiographic features of ALCAPA, including ventricular dysfunction are absent due to co-existent pulmonary hypertension. Herein we report a 5-month-old child with a large APW in whom a preoperative computed tomography angiogram facilitated the diagnosis of ALCAPA. The case is described for its rarity and the challenges faced during management. Also, it is extremely crucial that this coronary anomaly is identified and corrected during surgery, since failure to do so results in unforeseen postoperative ventricular dysfunction.

Interventricular membranous septal aneurysm is seen on multidetector computed tomography in postoperative child.

BACKGROUND: Ruptured interventricular septal aneurysms are rare. We present a case of an interventricular membranous septal aneurysm in a 7-year-old boy who presented with features of congestive heart failure, 2 years after undergoing a patch closure of perimembranous ventricular septal defect and tricuspid valve repair. Material and methods and results: Transthoracic echocardiography suggested the presence of an aneurysm that has ruptured into the right ventricle (RV), however, the precise origin of the aneurysm could not be identified on echocardiography. Subsequently, a computed tomography (CT) angiography was done which revealed that the aneurysm was originating from the membranous interventricular septum with evidence of rupture into the RV inflow. DISCUSSION: Due to its close proximity to the aortic annulus, an aneurysm of the membranous interventricular septum may be confused with a sinus of Valsalva aneurysm, especially if the former is associated with aortic regurgitation. CONCLUSION: This case highlights the importance of CT in the precise differentiation of these two abnormalities.

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.

Right aortic arch with brachio-bicephalic trunk mimicking an incomplete double aortic arch with distal left arch atresia.

We report a case of a 6-month-old girl with double outlet right ventricle where a right aortic arch with an abnormally posterior brachio-bicephalic trunk on the left side closely resembled an incomplete double-aortic-arch with distal left arch atresia on computed tomography (CT) angiography. The case highlights the role of preoperative CT angiography in demonstrating aortic arch and arch vessel anatomy in patients with complex congenital heart diseases.

All that attaches to atrial septum is not myxoma: deception is everywhere!

Preterm neonates - especially those with prolonged duration of intensive care stay - are prone to develop fungal endocarditis. Majority of these children have a stormy course, however, a few may be relatively asymptomatic. Occasionally these vegetations may be large and pedunculated, originating from the atrial septum, mimicking a cardiac myxoma on echocardiography.

A unique arrangement of "kissing" atrial appendages.

We report a case of a 2-month-old boy with tricuspid and pulmonary atresia with a unique configuration of "kissing" atrial appendages. The case highlights the importance of computed tomography angiography in identifying such anomalies associated with complex congenital heart defects.

"Dual Drainage" in the mixed variety of totally anomalous pulmonary venous connection.

We report a case of 6-week-old boy with a mixed variety of totally anomalous pulmonary venous connection where the common channel had a dual drainage into the coronary sinus and left brachiocephalic vein respectively. The case also highlights the role of preoperative computed tomography angiography in demonstrating the exact pulmonary venous anatomy, course and identifying obstruction, when present.

Ivabradine Versus Amiodarone in the Management of Postoperative Junctional Ectopic Tachycardia: A Randomized, Open-Label, Noninferiority Study.

OBJECTIVES: This study sought to compare the efficacy of ivabradine and amiodarone in the management of postoperative junctional ectopic tachycardia (JET) after cardiac surgery in children. BACKGROUND: JET is a serious arrhythmia occurring in children after cardiac surgery and requires aggressive management. Amiodarone has been conventionally used in its treatment. Recent studies have reported the utility of ivabradine in this regard. METHODS: In this open-label randomized controlled trial, 94 children (age ≤18 years) who developed postoperative JET were allocated to receive either amiodarone or ivabradine. The primary endpoint was restoration of normal sinus rhythm. RESULTS: Sinus rhythm was achieved in 43 out of the 46 patients (93.5%) in the amiodarone group and 46 out of the 48 patients (95.8%) in the ivabradine group (mean difference of treatment effect: 2.3%; 95% confidence interval: -6.7% to 11.5%). The median (interquartile range) time taken to achieve sinus rhythm conversion was similar in both the groups: 21.5 (17-30.2) hours versus 22 (13.4-38.5) hours (p = 0.36)]. The time taken to rate control of JET was significantly less in the amiodarone group: median 7.0 (5.5-9.5) hours versus 8.0 (5.8-10.8) hours (p = 0.02)]. No drug-related adverse events were observed in the ivabradine group. CONCLUSIONS: Oral ivabradine is not inferior to intravenous amiodarone in converting postoperative JET to sinus rhythm. There was no difference in time taken to sinus rhythm conversion between the groups, although the rate control was earlier in patients who received amiodarone. Monotherapy with ivabradine may be considered as an alternative to amiodarone in the management of postoperative JET. (Comparison of Two Drugs, Ivabradine and Amiodarone, in the Management of Junctional Ectopic Tachycardia, an Abnormality in Cardiac Rhythm in Patients Under 18 years Who Undergo Cardiac Surgery: CTRI/2018/08/015182).

Aetiological agents for pulmonary exacerbations in children with cystic fibrosis: An observational study from a tertiary care centre in northern India.

Background & objectives: Pulmonary disease is the main cause of morbidity and mortality in cystic fibrosis (CF). The infection occurs with a unique spectrum of bacterial pathogens that are usually acquired in an age-dependent fashion. The objective of this study was to find out the aetiological agents in respiratory specimens from children with CF during pulmonary exacerbation and relate with demographic variables. Methods: In this observational study, airway secretions from children (n=104) with CF presenting with pulmonary exacerbations were collected and tested for bacteria, fungi, mycobacteria and viral pathogens using appropriate laboratory techniques. The frequencies of isolation of various organisms were calculated and associated with various demographic profiles. Results: Bacteria were isolated in 37 (35.5%) and viral RNA in 27 (29.3%) children. Pseudomonas was the most common bacteria grown in 31 (29.8%) followed by Burkholderia cepacia complex (Bcc) in three (2.8%) patients. Among viruses, Rhinovirus was the most common, identified in 16 (17.4%) samples followed by coronavirus in four (4.3%). Fungi and mycobacteria were isolated from 23 (22.1%) and four (3.8%) children, respectively. Aspergillus flavus was the most common fungus isolated in 13 (12.5%) children. Interpretation & conclusions: Pseudomonas was the most common organism isolated during exacerbation. Non-tuberculous mycobacteria were not isolated, whereas infection with Bcc and Mycobacterium tuberculosis was observed, which could probably have a role in CF morbidity. Polymicrobial infections were associated with severe exacerbations.