Hitherto Unreported Pattern of Complex Obstructive Partial Anomalous Pulmonary Venous Drainage with Dual Drainage of Accessory Pulmonary Veins.

Partial anomalous pulmonary venous drainage is a congenital cardiac disorder characterized by abnormal drainage of one or more pulmonary veins into the systemic circulation. It can be isolated or associated with other congenital cardiac anomalies, most commonly atrial septal defect and patent ductus arteriosus. The clinical presentation is variable and depends on the degree of shunting and associated cardiac anomalies. Many patients usually remain asymptomatic until late in life. In this article, we presented a complex case of obstructive partial anomalous pulmonary venous drainage with dual drainage of bilateral accessory pulmonary veins with intact interatrial septum in conjunction with a patent ductus arteriosus and a ventricular septal defect. This pattern is incredibly rare and to the best of our knowledge has not been previously reported. Computed tomography played a pivotal role in precisely elucidating the intricate anatomy in this case with a complex pattern of anomalous pulmonary venous drainage.

Complete Duplication of Inferior Vena Cava Coexisting with Double Superior Vena Cava In Situ Solitus: Hitherto Unreported Pattern.

Congenital anomalies of inferior vena cava are increasingly being recognized with the technical advancements and increased utilization of cross-sectional imaging techniques. Duplication of inferior vena cava classically involves duplication of the infrarenal segment, where both inferior vena cava ascend on either side of the abdominal aorta until they form a confluence at the level of the renal veins. It has been extensively described in literature with few reports of more complex variation in the form of duplicated infrarenal inferior vena cava with azygos or hemiazygos continuation. This article describes extremely rare complete duplication of inferior vena cava involving both suprarenal and infrarenal segments. Moreover, the complete duplication of inferior vena cava is seen in association with concomitant double superior vena cava, in a patient with visceroatrial situs solitus and associated congenital heart disease, which to the best of our knowledge, has not been reported so far in literature. This study also highlights the utility of multidetector computed tomography in accurate identification of such anomalies.

Refractory supraventricular fetal tachycardia as a cause of non-immune hydrops: management conundrum.

Supraventricular tachyarrhythmia (SVT) is the most common form of fetal tachyarrhythmias. The presentation can vary from ill-defined, non-sustained episodes of tachyarrhythmia to frank non-immune hydrops. The standard of care is transplacental therapy by treating the mother with oral antiarrhythmic drugs, followed by direct fetal therapy in refractory cases. We report a case of primigravida in her late 20s, who presented at 28.1 weeks of gestation with fetal hydrops and SVT. She was initially managed with oral digoxin and flecainide, but due to worsening hydrops, risk of fetal demise and extreme prematurity, further management by direct fetal therapy was given in terms of intramuscular digoxin and intraperitoneal flecainide. Following which, the fetus had a favourable outcome. This case highlights the possible role of direct fetal therapy in refractory cases of SVT.

Right Aortic Arch with Bicarotid Trunk and Isolated Left Subclavian Artery: Hitherto Unreported Pattern.

Variant arch anatomy may be seen associated with many congenital heart diseases. Its accurate preoperative identification is of paramount importance in optimal surgical planning of such cases. This case describes one such variant arch anatomy with two vessel right aortic arch, comprising of bicarotid trunk (giving rise to bilateral common carotid arteries) and right subclavian artery with isolation of the left subclavian artery, in a patient with tetralogy of Fallot. Right aortic arch with isolated left subclavian artery has already been described in association with tetralogy of Fallot. However, to the best of our knowledge, present arch pattern consisting of right aortic arch with bicarotid trunk and isolated left subclavian artery has not been reported in literature so far. Moreover, this case highlights the utility of multidetector computed tomography in accurate identification of variant arch anatomy in addition to delineation of cardiac and extracardiac details.

Profile of cardiac lesions among laboratory confirmed congenital rubella syndrome (CRS) infants: a nationwide sentinel surveillance, India, 2016-22.

Background: The phenotypical profile of cardiovascular malformations in patients with congenital rubella syndrome (CRS) is varied. We aimed to describe the profile of cardiac defects among CRS patients detected in the sentinel CRS surveillance in India during 2016-22. Methods: Sentinel sites enrolled infants with suspected CRS based on presence of cardiac defects, hearing impairment, eye signs, or maternal history of febrile rash illness. Suspected CRS cases underwent detailed systemic examination, including echocardiography and serological investigation for rubella. Cardiac defects were categorized as 'Simple' or 'Complex' as per the National Heart, Lung, and Blood Institute classification. We compared the distribution of cardiac defects among laboratory confirmed CRS cases and seronegative discarded cases. Findings: Of the 4578 suspected CRS cases enrolled by 14 sites, 558 (12.2%) were laboratory confirmed. 419 (75.1%) laboratory confirmed cases had structural heart defects (simple defects: n = 273, 65.2%, complex defects: n = 144, 34.4%), with ventricular septal defect (42.7%), atrial septal defect (39.4%), patent ductus arteriosus (36.5%), and tetralogy of Fallot as the commonest defects (4.5%). Laboratory confirmed CRS cases had higher odds of left to right shunt lesions (OR = 1.58, 95% CI: 1.15-2.17). This was mainly on account of a significant association of PDA with CRS (OR = 1.77, 95% CI: 1.42-2.21). Mortality was higher among CRS patients with complex heart defects (HR = 2.04, 95% CI: 1.26-3.30). Interpretation: Three-fourths of the laboratory confirmed CRS cases had structural heart defects. CRS patients with complex cardiac defects had higher mortality. Detecting CRS infection early and providing timely intervention for cardiovascular defects is critical for the management of CRS patients. Funding: Ministry of Health and Family Welfare, Govt of India, through Gavi, the Vaccine Alliance.

Simultaneous Cesarean Section and Maternal Cardiac Surgery: Outcomes and Feasibility from a Tertiary Care Hospital in India.

INTRODUCTION: Cardiovascular disease is the leading cause of pregnancy-related mortality, and it has gradually increased over time; this rise has been attributed to numerous reasons including the growing number of women with congenital heart disease who are surviving to childbearing age. Valve surgery during pregnancy is a high risk, with a fetal and maternal mortality rate of 35% and 9%, respectively. Prior knowledge about the cardiovascular disease opens up a host of options for the mother even during pregnancy, but presentation in the 3rd trimester puts both the mother and the baby at risk. Simultaneous caesarean section and maternal cardiac surgery is a suitable option for this subset of patients, and with this study we aim to assess its outcomes and feasibility. METHODS: This is a retrospective study of five pregnant patients who presented with predominant symptoms of heart failure in the 3rd trimester between June 2019 and June 2021. Intraoperative and postoperative intensive care unit charts of all the patients were reviewed. RESULTS: All five patients underwent simultaneous cesarean section and maternal cardiac surgery successfully with no fetal or maternal mortality and are doing well in the follow-up period. CONCLUSION: Cesarean section followed by definitive maternal cardiac surgery in the same sitting is a safe and feasible approach in the management of such patients. A well-prepared team is pivotal for a safe delivery with a cardiopulmonary bypass machine on standby. Specialized multidisciplinary care in the antepartum, peripartum, and postpartum period is essential to improve outcomes.

Surgical outcomes of double-orifice mitral valve repair in patients with atrioventricular canal defects: a systematic review and meta-analysis.

INTRODUCTION: Double-orifice mitral valve or left atrioventricular valve is a rare congenital cardiac anomaly that may be associated with an atrioventricular septal defect. The surgical management of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect is highly challenging with acceptable clinical outcomes. This meta-analysis is aimed to evaluate the surgical outcomes of double-orifice mitral valve/double-orifice left atrioventricular valve repair in patients with atrioventricular septal defect. METHODS AND RESULTS: A total of eight studies were retrieved from the literature by searching through PubMed, Google Scholar, Embase, and Cochrane databases. Using Bayesian hierarchical models, we estimated the pooled proportion of incidence of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect as 4.88% in patients who underwent surgical repair (7 studies; 3295 patients; 95% credible interval [CI] 4.2-5.7%). As compared to pre-operative regurgitation, the pooled proportions of post-operative regurgitation were significantly low in patients with moderate status: 5.1 versus 26.39% and severe status: 5.7 versus 29.38% [8 studies; 171 patients]. Moreover, the heterogeneity test revealed consistency in the data (p < 0.05). Lastly, the pooled estimated proportions of early and late mortality following surgical interventions were low, that is, 5 and 7.4%, respectively. CONCLUSION: The surgical management of moderate to severe regurgitation showed corrective benefits post-operatively and was associated with low incidence of early mortality and re-operation.

A Rare Case of Giant Atypical Patent Ductus Arteriosus.

A broad window-like patent ductus arteriosus (PDA) arising from the transverse aortic arch proximal to the left subclavian artery and without any associated cardiac anomalies in a one-year-old child is rare. This case is reported for the atypical location and unusually large size of the PDA.

Major Aortopulmonary Collaterals From Coronary Arteries and Dual Left Anterior Descending Artery in an Adult With Tetralogy of Fallot.

Major aortopulmonary collaterals (APCs) are more common with tetralogy of Fallot (TOF) with pulmonary atresia. Collateral arteries, when present, arise most commonly from the descending thoracic aorta, less commonly from the subclavian arteries, and rarely from the abdominal aorta or its branches or from the coronary arteries. Collaterals arising from the coronary arteries can cause myocardial ischemia due to coronary steal phenomenon. They can be addressed either by endovascular interventions like coiling or surgical ligation during the intracardiac repair. Coronary anomalies are seen in 5%-7% of TOF patients. In approximately 4% of TOF patients, the left anterior descending artery (LAD) or an accessory LAD takes origin from the right coronary artery or right coronary sinus and crosses the right ventricular outflow tract in its course toward the left ventricle. Presence of such anomalous coronary anatomy poses certain challenges during intracardiac repair of TOF.

Left ventricular diverticulum in association with bicuspid aortic valve and pseudocoarctation: Hitherto unreported association.

Left ventricular diverticulum is a rare cardiac abnormality characterised by focal protrusion of the ventricular wall containing all the three layers with synchronous contractility. They have been described more commonly in paediatric patients and may be seen associated with congenital midline thoracoabdominal abnormalities. There are few reports of left ventricular diverticulum in cases of patent ductus arteriosus and coarctation with subaortic diverticula in the bicuspid aortic valve. Here, we report a case of left ventricular diverticulum in association with bicuspid aortic valve and pseudocoarctation in a 53-year-old female patient with a pertinent review of the literature.

Main pulmonary artery continuing as right pulmonary artery with trifurcation at its origin and aberrant origin of left pulmonary artery from ascending aorta in a case of tetralogy of Fallot.

Anomalous origin of the left pulmonary artery from the ascending aorta is a rare cardiac malformation described with tetralogy of Fallot. Trifurcation of right pulmonary artery after origin from the main pulmonary artery has not been reported in literature yet.

Simultaneous Cesarean Section and Maternal Cardiac Surgery: Outcomes and Feasibility from a Tertiary Care Hospital in India

ABSTRACT Introduction: Cardiovascular disease is the leading cause of pregnancy-related mortality, and it has gradually increased over time; this rise has been attributed to numerous reasons including the growing number of women with congenital heart disease who are surviving to childbearing age. Valve surgery during pregnancy is a high risk, with a fetal and maternal mortality rate of 35% and 9%, respectively. Prior knowledge about the cardiovascular disease opens up a host of options for the mother even during pregnancy, but presentation in the 3rd trimester puts both the mother and the baby at risk. Simultaneous caesarean section and maternal cardiac surgery is a suitable option for this subset of patients, and with this study we aim to assess its outcomes and feasibility. Methods: This is a retrospective study of five pregnant patients who presented with predominant symptoms of heart failure in the 3rd trimester between June 2019 and June 2021. Intraoperative and postoperative intensive care unit charts of all the patients were reviewed. Results: All five patients underwent simultaneous cesarean section and maternal cardiac surgery successfully with no fetal or maternal mortality and are doing well in the follow-up period. Conclusion: Cesarean section followed by definitive maternal cardiac surgery in the same sitting is a safe and feasible approach in the management of such patients. A well-prepared team is pivotal for a safe delivery with a cardiopulmonary bypass machine on standby. Specialized multidisciplinary care in the antepartum, peripartum, and postpartum period is essential to improve outcomes.

Aortopulmonary window with anomalous ascending aortic origin of left pulmonary artery: Hitherto unreported pattern.

Anomalous ascending aortic origin of left pulmonary artery in association with aortopulmonary window has not been reported so far in literature and is the main highlight of the present case.

Clinico-Laboratory Profile, Intensive Care Needs and Short-Term Outcome of Multisystem Inflammatory Syndrome in Children (MIS-C): Experience during First and Second Waves from North India.

OBJECTIVES: To describe the clinico-laboratory profile, intensive care needs and outcome of multisystem inflammatory syndrome in children (MIS-C) during the first and second waves. METHODOLOGY: This retrospective study was conducted in the paediatric emergency and paediatric intensive care unit (PICU) of a tertiary care teaching hospital in North India involving 122 children with MIS-C admitted during the first wave (September 2020-January 2021, n = 40) and second wave (February 2021-September 2021, n = 82) of coronavirus disease 2019 (COVID-19). RESULTS: The median (interquartile range) age was 7 (4-10) years and 67% were boys. Common manifestations included fever (99%), abdominal symptoms (81%), rash (66%) and conjunctival injection (65%). Elevated C-reactive protein (97%), D-dimer (89%), procalcitonin (80%), IL-6 (78%), ferritin (56%), N-terminal pro B-type natriuretic peptide (84%) and positive severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) antibody (81%) were common laboratory abnormalities. Cardiovascular manifestations included myocardial dysfunction (55%), shock (48%) and coronary artery changes (10%). The treatment included intensive care support (57%), non-invasive (33%) and invasive (18%) ventilation, vasoactive drugs (47%), intravenous immunoglobulin (IVIG) (83%), steroids (85%) and aspirin (87%). The mortality was 5% (n = 6). During the second wave, a significantly higher proportion had positive SARS-CoV-2 antibody, contact with COVID-19 and oral mucosal changes; lower markers of inflammation; lower proportion had lymphopenia, elevated IL-6 and ferritin; lower rates of shock, myocardial dysfunction and coronary artery changes; lesser need of PICU admission, fluid boluses, vasoactive drugs and IVIG; and shorter hospital stay. CONCLUSION: MIS-C is a febrile multisystemic disease characterized by hyperinflammation, cardiovascular involvement, temporal relationship to SARS-CoV-2 and good outcome with immunomodulation and intensive care. During the second wave, the severity of illness, degree of inflammation, intensive care needs, and requirement of immunomodulation were less as compared to the first wave.

Successful endovascular repair with coil embolization of ruptured right internal thoracic artery aneurysm.

Internal thoracic artery aneurysms (ITAAs) are rare with wide variation in clinical presentation and a high risk of rupture. Endovascular techniques are increasingly being used for treatment of such aneurysms over surgical repair in recent times. A 34-year-old male presented with progressive swelling of the right anterior chest wall for 2 weeks and was diagnosed with right internal thoracic artery aneurysm with contained rupture. He underwent successful endovascular repair with coil embolization of ruptured right ITAA. Post intervention computed tomography (CT) angiography confirmed sealing of the ruptured aneurysm with no residual filling of the sac. At six months follow-up he is doing well with complete resolution of hematoma. This case demonstrates that an endovascular approach with coil embolization is a feasible and safe option for treating the rare ruptured ITAAs.

Os aneurismas da artéria torácica interna (ITAAs) são raros, com ampla variação na apresentação clínica e alto risco de ruptura. As técnicas endovasculares têm sido cada vez mais utilizadas para o tratamento desses aneurismas em relação ao reparo cirúrgico. Um homem de 34 anos de idade apresentou edema progressivo da parede torácica anterior direita por 2 semanas e foi diagnosticado com aneurisma da artéria mamária interna direita com ruptura contida. Ele foi submetido a reparo endovascular bem-sucedido, com embolização de ITAA direito roto. A angiotomografia computadorizada (angioTC) pós-intervenção confirmou o selamento do aneurisma rompido, sem enchimento residual do saco. No seguimento de 6 meses, ele estava bem, com resolução completa do hematoma. Este caso demonstra que a abordagem endovascular com embolização com molas é uma opção viável e segura no tratamento dos raros ITAAs rotos.

Congenitally corrected transposition with absent pulmonary valve: Hitherto unreported association.

Absent pulmonary valve has usually been described in association with tetralogy of Fallot. Present case highlights its association with congenitally corrected transposition which has not been reported so far in literature.